Volume 17, Number 1
Dr Swati Y Bhave
Dr KK Aggarwal
Editor
Group Editor-in-Chief
Online Submission
IJCP Group of Publications
Asian Journal of
Paediatric Practice
Dr Sanjiv Chopra Prof. of Medicine and Faculty Dean Harvard Medical School Group Consultant Editor
Volume16, 17,No. No.4,1,2013 2013 Volume
Dr Deepak Chopra Chief Editorial Advisor
from the desk of the editor
Padma Shri and Dr BC Roy National Awardee
Dr KK Aggarwal
Group Editor-in-Chief
Dr Veena Aggarwal
5
Swati Y Bhave
MD, Group Executive Editor Anand Gopal Bhatnagar Editorial Anchor
AJPP Speciality Board Chief Editor Dr Swati Y Bhave Editorial Board National Dr Alagiriswamy Parthasarathy Dr Ajay Kalra Dr K Nedunchelian Dr Yagnesh Popat Dr Chhaya Prasad Dr Atul Agarwal Dr Anoop Verma Dr Vijay Zawar Dr J S Tuteja Dr Surekha Joshi Editorial Board International Dr Professor Antonio An Tung Chuh Dr Jay E Berkelhamer Dr Neil Wigg Professor Andreas Konstantopoulos Ahmaduddin Maarij Professor Leyla Namazova-Baranova Dr Angelo Neeneo Dr Yoshikatsu Eto Dr Peter Cooper
from the desk of the group editor-in-chief 6 Harvard Commonly Held Myths About End-of-life Issues
KK Aggarwal
REVIEW ARTICLE 7 Evaluation of Anemia in Children
Jennifer Janus, Sarah K. Moerschel
IJCP Editorial Board Obstetrics and Gynaecology Dr Alka Kriplani Dr Thankam Verma, Dr Kamala Selvaraj Cardiology Dr Praveen Chandra, Dr SK Parashar Paediatrics Dr Swati Y Bhave Diabetology Dr CR Anand Moses, Dr Sidhartha Das Dr A Ramachandran, Dr Samith A Shetty ENT Dr Jasveer Singh, Dr Chanchal Pal Dentistry Dr KMK Masthan, Dr Rajesh Chandna Gastroenterology Dr Ajay Kumar, Dr Rajiv Khosla Dermatology Dr Hasmukh J Shroff, Dr Pasricha, Dr Koushik Lahiri Nephrology Dr Georgi Abraham Neurology Dr V Nagarajan, Dr Vineet Suri Journal of Applied Medicine & Surgery Dr SM Rajendran, Dr Jayakar Thomas Orthopedics Dr J Maheshwari Advisory Bodies Heart Care Foundation of India Non-Resident Indians Chamber of Commerce & Industry World Fellowship of Religions
CLINICAL STUDY 18 Septoplasty with Adenoidectomy: A Combined Procedure for Nasal Obstruction in Children
K Mallikarjuna Swamy, KP Basavaraju
21 Vitamin E Level in Normal Indian Children
Geeta Gathwala, Subrika Yadav, Ojawanai, G Lal
case report 24 Lichen Sclerosus et Atrophicus in a Young Girl
YS Marfatia, Sonia Jain
case report
Published, Printed and Edited by Dr KK Aggarwal, on behalf of IJCP Publications Ltd. and Published at E - 219, Greater Kailash, Part - 1 New Delhi - 110 048 E-mail: editorial@ijcp.com
26 Massive Fetomaternal Hemorrhage
Kannan Venkatnarayan, Rajeev Thapar, Himanshu Sharma
Printed at VM Prints, Chennai Š Copyright 2013 IJCP Publications Ltd. All rights reserved. The copyright for all the editorial material contained in this journal, in the form of layout, content including images and design, is held by IJCP Publications Ltd. No part of this publication may be published in any form whatsoever without the prior written permission of the publisher.
29 An Interesting Case of Meckel-Gruber Syndrome
Sangeeta Arya, K Pandey, A Verma, RK Singh, S Chauhan
Editorial Policies The purpose of IJCP Academy of CME is to serve the medical profession and provide print continuing medical education as a part of their social commitment. The information and opinions presented in IJCP group publications reflect the views of the authors, not those of the journal, unless so stated. Advertising is accepted only if judged to be in harmony with the purpose of the journal; however, IJCP group reserves the right to reject any advertising at its sole discretion. Neither acceptance nor rejection constitutes an endorsement by IJCP group of a particular policy, product or procedure. We believe that readers need to be aware of any affiliation or financial relationship (employment, consultancies, stock ownership, honoraria, etc.) between an author and any organization or entity that has a direct financial interest in the subject matter or materials the author is writing about. We inform the reader of any pertinent relationships disclosed. A disclosure statement, where appropriate, is published at the end of the relevant article.
photo quiz 31 A Bubble Under the Tongue of a Child
RESEARCH REVIEW 33 From the Journals...
Note: Asian Journal of Paediatric Practice does not guarantee, directly or indirectly, the quality or efficacy of any product or service described in the advertisements or other material which is commercial in nature in this issue.
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from the desk of THE editor
Dr Swati Y Bhave
Chief Editor Executive Director, AACCI (Association of Adolescent and Child Care in India) Senior Visiting Consultant Indraprastha Apollo Hospitals, New Delhi
We are very happy to bring out this issue of AJPP of July-September 2013. Anemia is a very common problem in children. Although a vast majority of anemia is due to nutritional deficiency, it is important to keep other causes of anemia in mind and do tests where there are clinical indications so that other important causes are not missed out. This issue contains a well-written review article on ‘Evaluation of Anemia in Children’. Young children are often brought for snoring, mouth breathing, rhinorrhea and sleep disturbances. The commonest causes are adenoid hypertrophy and deviated nasal septum. Surgery is generally recommended by physicians only if symptoms are severe. We have a good surgical article that enlightens us about adverse effects of not performing surgery for nasal septal deviation. It also helps us to understand that surgery for deviated septum should be combined with adenoidectomy for best results. Vitamin E has antioxidant properties and is useful for many important functions in the human bodylike signaling process in platelets and delaying disease process in many conditions such as neurogenerative disorders, coronary arteriosclerosis and others. Normal vitamin E levels in children and adults range from
5 to 20 µg/ml and are slightly lower in infants (3-10 µg/ml). It is important to know the levels in normal Indian children. We have a clinical study that was conducted to determine the ‘Vitamin E Level in Normal Indian Children’. There are three interesting case reports. The first describes a case of ‘Lichen Sclerosus et Atrophicus in a Young Girl’. This condition can be cosmetically distressing and also can be associated with sexual abuse and trauma; hence, the management is multipronged. Fetomaternal hemorrhage (FMH) can occur with minimal symptoms as in 98% of cases blood loss is usually <0.1 ml. Massive FMH i.e., blood loss of >150 ml is rare and we have a very interesting case report of this condition in this issue. The third case report is of Meckel-Gruber syndrome that is characterized by neurological malformations, polydactyly and cystic dysplasia of the kidneys. A photo quiz of ‘A Bubble Under the Tongue of a Young Child’ should be useful in clinical practice. It has a list of differential diagnoses and requires surgical excision. Last but not the least are journal reviews about zinc and its role in diarrhea as well as its supplementation in full-term normal infants and approach to fever of unknown origin. Happy Reading
Dr Swati Y Bhave Address for correspondence IJCP Group of Publications E - 219, Greater Kailash, Part - 1, New Delhi - 110 048
Asian Journal of Paediatric Practice, Vol. 17, No. 1, 2013
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from the desk of THE group editor-in-chief Dr KK Aggarwal
Padma Shri and Dr BC Roy National Awardee Sr. Physician and Cardiologist, Moolchand Medcity, New Delhi President, Heart Care Foundation of India Group Editor-in-Chief, IJCP Group and eMedinewS National Vice President Elect, IMA Member, Ethics Committee, MCI Chairman, Ethics Committee, Delhi Medical Council Director, IMA AKN Sinha Institute (08-09) Hony. Finance Secretary, IMA (07-08) Chairman, IMA AMS (06-07) President, Delhi Medical Association (05-06) emedinews@gmail.com http://twitter.com/DrKKAggarwal Krishan Kumar Aggarwal (Facebook)
Harvard Commonly Held Myths About End-of-life Issues Myth: More care is always better. Truth: Not necessarily. Sometimes more care prolongs the dying process without respect for quality-of-life or comfort. It is important to know what interventions are truly important. It is often impossible to know that in advance. That is where the advice of a healthcare team is invaluable. Myth: Refusing life support invalidates your life insurance because you are committing suicide. Truth: Refusing life support does not mean that you are committing suicide. Instead, the underlying medical problem is considered to be the cause of death. Myth: If medical treatment is started, it cannot be stopped. Truth: Not starting a medical treatment and stopping a treatment are the same in the eyes of the law. So, you or your healthcare agent can approve a treatment for a trial
period that you think may be helpful without fear and then you cannot change your mind later. However, be aware that stopping treatment can be more emotionally difficult than not starting it in the first place. Myth: If you refuse life-extending treatments, you are refusing all treatments. Truth: No matter what treatments you refuse, you should still expect to receive any other care you need or want, especially the pain and symptom management, sometimes called intensive comfort care. Myth: Stopping or refusing artificial nutrition and hydration causes pain for someone who is dying. Truth: Unlike keeping food or water away from a healthy person, for someone who is dying, declining artificial nutrition or intravenous hydration does not cause pain.
mmmmm
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Asian Journal of Paediatric Practice, Vol. 17, No. 1, 2013
REVIEW ARTICLE
Evaluation of Anemia in Children JENNIFER JANUS, SARAH K. MOERSCHEL
ABSTRACT Anemia is defined as a hemoglobin level of less than the 5th percentile for age. Causes vary by age. Most children with anemia are asymptomatic, and the condition is detected on screening laboratory evaluation. Screening is recommended only for high-risk children. Anemia is classified as microcytic, normocytic, or macrocytic, based on the mean corpuscular volume. Mild microcytic anemia may be treated presumptively with oral iron therapy in children six to 36 months of age who have risk factors for iron deficiency anemia. If the anemia is severe or is unresponsive to iron therapy, the patient should be evaluated for gastrointestinal blood loss. Other tests used in the evaluation of microcytic anemia include serum iron studies, lead levels, and hemoglobin electrophoresis. Normocytic anemia may be caused by chronic disease, hemolysis, or bone marrow disorders. Workup of normocytic anemia is based on bone marrow function as determined by the reticulocyte count. If the reticulocyte count is elevated, the patient should be evaluated for blood loss or hemolysis. A low reticulocyte count suggests aplasia or a bone marrow disorder. Common tests used in the evaluation of macrocytic anemias include vitamin B12 and folate levels, and thyroid function testing. A peripheral smear can provide additional information in patients with anemia of any morphology. Keywords: Anemia, mean corpuscular volume, iron supplementation
A
n estimated 20 percent of American children will have anemia at some point in their childhood.1 Anemia is defined as a hemoglobin (Hgb) concentration or red blood cell (RBC) mass less than the 5th percentile for age. Hgb levels vary by age, and many laboratories use adult norms as references; therefore, the patient’s Hgb level must be compared with age-based norms to diagnose anemia2 (Table 1).3
Anemia is usually classified based on the size of RBCs, as measured by the mean corpuscular volume (MCV). Anemia can be microcytic (MCV typically less than 80 μm3 [80 fL]), normocytic (80 to 100 μm3 [80 to 100 fL]), or macrocytic (greater than 100 μm3 [100 fL]). The RBC distribution width is a measure of the size variance of RBCs. A low RBC distribution width suggests uniform cell size, whereas an elevated width (greater than 14 percent) indicates RBCs of multiple sizes. Etiology Although some studies have suggested a decline in the prevalence of anemia,4,5 the most recent Pediatric JENNIFER JANUS, MD, FAAP, is an internist and pediatrician with Johns Hopkins Community Physicians, part of the Johns Hopkins Health System, in Hagerstown, Md. At the time this article was written, she was a clinical assistant professor in the Departments of Family Medicine, Internal Medicine, and Pediatrics at the West Virginia University Robert C. Byrd Health Sciences Center Eastern Division, Harpers Ferry. SARAH K. MOERSCHEL, MD, FAAP, is a clinical assistant professor in the Departments of Family Medicine and Pediatrics at the West Virginia University Robert C. Byrd Health Sciences Center Eastern Division. Source: Adapted from Am Fam Physician. 2010;81(12):1462-1471.
Nutrition Surveillance System Report showed an increase among low-income children, from 13 percent in 2002 to 15 percent in 2007.6 The causes of anemia vary by age (Table 2).2,7 Anemia should not be considered a diagnosis, but a finding that warrants further investigation.8 In children, it is usually caused by decreased RBC production or increased RBC turnover.2 Iron deficiency commonly causes decreased RBC production. Risk factors include prematurity, poor diet, consumption of more than 24 oz of cow’s milk per day, and chronic blood loss.9 Other causes of decreased RBC production include inflammation from chronic infection or other inflammatory conditions, renal failure, medication use, viral illnesses and bone marrow disorders (Table 3).2,10 Increased RBC turnover may be a result of blood loss, mechanical destruction of RBCs, or hemolysis. Hemolysis may result from inherited defects in RBCs; therefore, sex, ethnicity, and family history are potential risk factors. Medications may cause anemia because of immune-mediated hemolysis or oxidative stress. Mechanical destruction may occur in persons with mechanical valves or splenomegaly. RBC loss may also be a result of acute bleeding.2 DIAGNOSIS
Clinical Diagnosis Most children with mild anemia have no signs or symptoms. Some may present with irritability or pica
Asian Journal of Paediatric Practice, Vol. 17, No. 1, 2013
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review article Table 1. Age-Specific Normative Red Blood Cell Values Age
Hemoglobin (g per dL)
Hematocrit (%)
Mean corpuscular volume (fL)
Mean
2 SDs below mean
Mean
2 SDs below mean
Mean
2 SDs below mean
26 to 30 weeks’ gestation
13.4
11.0
41.5
34.9
118.2
106.7
28 weeks’ gestation
14.5
NA
45
NA
120
NA
32 weeks’ gestation
15.0
NA
47
NA
118
NA
Full term (cord sample)
16.5
13.5
51
42
108
98
1 to 3 days
18.5
14.5
56
45
108
95
2 weeks
16.6
13.4
53
41
105
88
1 month
13.9
10.7
44
33
101
91
2 months
11.2
9.4
35
28
95
84
6 months
12.6
11.1
36
31
76
68
6 months to 2 years
12.0
10.5
36
33
78
70
2 to 6 years
12.5
11.5
37
34
81
75
6 to 12 years
13.5
11.5
40
35
86
77
12 to 18 years (male)
14.5
13.0
43
36
88
78
12 to 18 years (female)
14.0
12.0
41
37
90
78
Adult (male)
15.5
13.5
47
41
90
80
Adult (female)
14.0
12.0
41
36
90
80
NA = Not available; SD = Standard deviation.
(in iron deficiency), jaundice (in hemolysis), shortness of breath, or palpitations. Physical examination may show jaundice, tachypnea, tachycardia, and heart failure, especially in children with severe or acute anemia. Pallor has poor sensitivity for predicting mild anemia, but correlates well with severe anemia.11-13 One study showed that physical examination findings of pallor of the conjunctiva, tongue, palm, or nail beds is 93 percent sensitive and 57 percent specific for the diagnosis of anemia in patients with an Hgb level of less than 5 g per dL (50 g per L).14 The sensitivity decreases to 66 percent when the Hgb level is 5 to 8 g per dL (50 to 80 g per L).14 Chronic anemia may be associated with glossitis, a flow murmur, and growth delay, although these conditions are rare in developed countries.1
Diagnostic Tests
days previously. Because reticulocytes survive in the periphery for only one or two days, reticulocyte hemoglobin content (RHC) is a more accurate “realtime” measurement of bone marrow iron status.15 Alternatively, many cases of anemia in children are not caused by iron deficiency. Therefore, measurement of a single Hgb level may result in unnecessary treatment and retesting.16 Measurement of RHC may help avoid this issue. In a study of infants nine to 12 months of age, an Hgb level of less than 11 g per dL (110 g per L) was only 26 percent sensitive in detecting iron deficiency (as measured by a transferrin saturation of less than 10 percent), whereas an RHC of less than 27.5 pg was 83 percent sensitive in detecting iron deficiency.17 RHC is not available in all laboratories, and more studies are needed to determine whether screening with this test is clinically useful and cost-effective.
Laboratory tests used in the diagnosis of anemia include measurement of ferritin, which reflects iron stores, and transferrin or total iron-binding capacity, which indicates the body’s ability to transport iron for use in RBC production.
Approach to the Child with Anemia: Illustrated Case Studies
Hgb measurement fails to detect many cases of early or mild iron deficiency because the life span of RBCs reflect bone marrow iron content from up to 120
A full-term infant is delivered with the use of forceps; the pregnancy and delivery were otherwise uncomplicated. The initial examination is normal, but on the second hospital
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Asian Journal of Paediatric Practice, Vol. 17, No. 1, 2013
Anemia in a Newborn
review article Table 2. Age-Specific Causes of Anemia Cause
Etiology and epidemiology
Presentation
Indices and other laboratory testing
Neonatal7 Blood loss
Isoimmunization
Hemorrhage (placental abruption, Tachypnea, pallor, and mental status subgaleal, traumatic); maternal change (irritability, poor feeding); > 20 percent loss of blood volume results in fetal and twin-twin transfusion Accounts for 5 to 10 percent of all shock and cardiopulmonary collapse
cases of severe neonatal anemia ABO incompatibility, Jaundice and mild anemia; infants with Positive Coombs test; elevated bilirubin severe isoimmunization (e.g., untreated level; normocytic anemia with elevated Rh incompatibility Rh incompatibility occurs in 10.6 Rh incompatibility) may present with reticulocyte count per 10,000 live births; 50 percent hydrops fetalis
of these infants develop anemia Congenital Spherocytosis, G6PD deficiency Hyperbilirubinemia and moderate hemolytic anemia jaundice
Congenital infection DiamondBlackfan syndrome
Anemia with normal indices; reticulocyte count is initially normal, then increases; positive Kleihauer- Betke test in maternalfetal hemorrhage
Parvovirus B19, human immunodeficiency virus, syphilis, rubella, sepsis Congenital pure red cell aplasia resulting from increased apoptosis in erythroid precursors Affects 7 per 1 million live births Increased susceptibility of progenitor cells in bone marrow leads to increased apoptosis, progressing to pancytopenia
Pallor, irritability, and other findings associated with infection (e.g., deafness) Neonatal pallor progressing to symptomatic anemia; average age of diagnosis is 3 months; about 30 percent have other abnormalities
Low enzyme activity; with hemolysis, smear may show poikilocytosis, reticulocytosis, Heinz bodies, and bite cells (in G6PD deficiency) or spur cells (in pyruvate kinase deficiency) Normocytic anemia with low reticulocyte count Macrocytic anemia with low reticulocyte count
Average age of diagnosis is 8 years, but associated congenital abnormalities may facilitate early diagnosis (e.g., cafĂŠ-au-lait spots; microsomy; low birth weight; thumb, renal, skeletal, and eye abnormalities)
Microcytic anemia and reticulocytopenia, thrombocytopenia, or leukopenia; DNA sequencing can detect genetic mutations for Fanconi anemia complementation groups
Usually asymptomatic; severe cases can present with fatigue, pallor, or dyspnea; rarely occurs before 6 months of age; highest risk is at 6 to 36 months of age
Microcytic anemia with elevated RBC distribution width; peripheral smear shows hypochromic microcytes and may show target cells; iron and ferritin levels and iron saturation are low; transferrin level is elevated
Prevalence is 8 to 15 percent Bacterial or viral infection leading to cytokine-mediated decrease in iron utilization and RBC production
Presenting symptoms usually result from infectious process
Blood loss
Trauma, gastrointestinal bleeding
Tachypnea, tachycardia, pallor, hypotension
Normocytic or mildly microcytic, low/ normal serum iron level with low transferrin level; ferritin level may be elevated because it is an acute phase reactant Hgb levels may initially be normal, followed by anemia with normal indices
Disorder of Hgb structure or synthesis
Thalassemia, sickle cell disease Anemia in thalassemia may range from mild and asymptomatic to severe, depending on number of heme chains affected; sickle cell disease presents with hemolysis, pain crises, dactylitis, and aplastic crisis; symptoms are rarely present at birth but typically develop in the first year
Fanconi anemia
Infancy to toddlerhood2 Iron deficiency
Concurrent infection
Inadequate dietary intake, chronic occult blood loss (excessive cowâ&#x20AC;&#x2122;s milk consumption, inflammatory bowel disease, Meckel diverticulum, parasites)
Microcytic anemia, low RBC distribution width, and low Mentzer index in thalassemia; Hgb electrophoresis may show Hgb F; smear with basophilic stippling; hemolysis, reticulocytosis, and Hgb S on electrophoresis in sickle cell disease
Continued... Asian Journal of Paediatric Practice, Vol. 17, No. 1, 2013
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review article Table 2. Age-Specific Causes of Anemia (Continued) Cause
Etiology and epidemiology
Infancy to
Presentation
Indices and other laboratory testing
toddlerhood2 (Continued) Neonatal hyperbilirubinemia and hemolytic anemia when exposed 10 percent of the black population to oxidative stress has G6PD deficiency
RBC enzyme defects
G6PD deficiency, pyruvate kinase deficiency
RBC membrane defects
Spherocytosis, elliptocytosis
Acquired hemolytic anemias
Antibody-mediated hemolysis, Jaundice, fatigue, dyspnea drug-induced hemolysis, hemolytic uremic syndrome, disseminated intravascular coagulation
Transient erythroblastopenia of childhood
Transient immune reaction against Anemia after toxin ingestion or erythroid progenitor cells viral illness, usually in children 6 months to 3 years of age
Leukemia, myelofibrosis
Usually spontaneous, but rates Anemia causes pallor, fatigue, and are increased in patients with prior dyspnea; patients with leukemia radiation exposure or chemotherapy may present with petechiae, lowgrade fever, nonspecific bone pain, gum swelling, or rash Risk factors include young age, In addition to anemia, patients living in a home built before 1970 or may present with abdominal in areas where soil is contaminated, pain, altered mental status, renal disease, and hypertension and pica (as in iron deficiency)
Lead poisoning
Hyperbilirubinemia, splenomegaly, gall bladder disease, and aplastic crisis; autosomal dominant, so family history is positive in about 75 percent of patients
Low enzyme activity; with hemolysis smear may show poikilocytosis, reticulocytosis, Heinz bodies, and bite cells (in G6PD deficiency) or spur cells (in pyruvate kinase deficiency) Macrocytosis, reticulocytosis, elevated bilirubin and lactate dehydrogenase levels; spherocytes or elliptocytes on smear; osmotic fragility test is commonly done but not specific Positive Coombs test and spherocytes visible on smear in antibody-mediated hemolysis; schistocytes visible on smear in hemolytic uremic syndrome or disseminated intravascular coagulation Normocytic anemia, initially with reticulocyte count of 0; anemia resolves within 2 months Normocytic anemia with decreased reticulocyte count; leukopenia, leukocytosis, or thrombocytopenia; peripheral smear shows blast cells Microcytic anemia may be concurrent with iron deficiency; peripheral smear may show basophilic stippling; hemolysis may be present
Late childhood and adolescence2 Iron deficiency
Second peak in iron deficiency Pallor, fatigue, dyspnea occurs in adolescence because of growth spurt, menstruation, and poor dietary iron intake
Chronic disease
Renal disease, liver disease, hypothyroidism, other chronic illnesses
Blood loss
Same as for infants and toddlers, above
Disorders of Hgb synthesis or RBC membrane defects
Usually mild and asymptomatic
Menstruation in adolescent girls Same as for infants and toddlers, above
Acquired hemolytic anemias
Same as for infants and toddlers, above
Leukemia and other bone marrow disorders
Same as for infants and toddlers, above
Note: Causes listed in decreasing order of approximate prevalence. G6PD = Glucose-6-phosphate dehydrogenase; Hgb = Hemoglobin; RBC = Red blood cell. Information from references 2 and 7.
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Asian Journal of Paediatric Practice, Vol. 17, No. 1, 2013
Same as for infants and toddlers, above
Normocytic or mildly microcytic, low/ normal serum iron level with low transferrin level; ferritin level may be elevated because it is an acute phase reactant
review article Table 3. Risk Factors for Anemia Etiology
Risk factor
Comment
Decreased RBC production
Chronic disease
Renal disease can result in anemia because of decreased erythropoietin levels; hypothyroidism can result in macrocytic anemia because of impaired RBC production; chronic inflammation (as in chronic infection or rheumatologic disease) can lead to cytokinemediated suppression of erythropoiesis; inflammatory bowel disease or celiac disease can result in anemia because of inflammation and nutrient malabsorption
Iron deficiency10
Pica induced by iron deficiency increases risk of lead ingestion, and lead is absorbed more readily in the presence of iron deficiency; iron levels should be tested in patients with lead poisoning
Poor diet
Inadequate nutrient intake can cause deficiencies in iron, folate, and vitamins A, B12, and D
Prematurity
Decreased iron stores and increased demand for catch-up growth can cause iron deficiency; rarely occurs before birth weight is doubled
Drug use
Primaquine, sulfamethoxazole, and nitrofurantoin can lead to hemolysis; this is more pronounced in patients with G6PD deficiency but can occur in any patient; phenytoin can cause megaloblastic anemia
Ethnicity
African ancestry in sickle cell disease; Mediterranean, Asian, or African ancestry in thalassemia; Sephardic Jewish, Filipino, Greek, Sardinian, or Kurdish ancestry in G6PD deficiency
Family history
Thalassemia, spherocytosis, and sickle cell disease; family history may include gallstones and jaundice in addition to anemia
Mechanical heart valves
Mechanical destruction by the valve can cause hemolysis
Sex
G6PD deficiency and pyruvate kinase deficiency are X-linked and therefore more common in males
Splenomegaly
Sequestration and increased destruction of RBCs can cause hemolysis
Infection
Infection can precipitate immune-mediated hemolytic anemia or cause hemolytic crises in patients with inherited enzyme defects and sickle cell disease; can cause RBC aplasia (as in parvovirus B19 infection) or result in transient erythroblastopenia of childhood
Increased RBC turnover
Both
G6PD = Glucose-6-phosphate dehydrogenase; RBC = Red blood cell. Information from references 2 and 10.
day, he is pale and fussy. The reticulocyte count and bilirubin level are normal, and the Hgb level is 9 g per dL (90 g per L). Repeat physical examination reveals an increased head circumference. Causes of anemia in the newborn are blood loss, decreased RBC production, and increased RBC turnover. Blood loss during delivery can result from a ruptured umbilical cord, placenta previa, and abruptio placentae. Maternal-fetal transfusion occurs in 50 percent of all pregnancies, but usually does not cause significant loss of blood volume.7 The patient’s history eliminates most of these causes. A normal reticulocyte count confirms that the infant’s bone marrow is functional. This rules out causes of decreased RBC production, including Fanconi anemia, Diamond-Blackfan syndrome, and congenital infections.
Cranial hemorrhages are often associated with birth trauma, including vacuum and forceps delivery. In particular, subgaleal bleeds can be of sufficient volume to cause shock. Physical examination findings may include mental status changes, jaundice, tachycardia or tachypnea, and increased head circumference.7 In this patient, a computed tomography scan confirms a subgaleal hemorrhage, and the infant is transferred to a neonatal intensive care unit for transfusion and monitoring. In newborns, an elevated bilirubin level in association with anemia suggests hemolysis. If this infant’s bilirubin level had been elevated, further testing would have included a Coombs test to evaluate for isoimmunization (as in ABO or Rh incompatibility) and a peripheral smear to evaluate for spherocytosis or other RBC membrane defects. Testing for glucose-
Asian Journal of Paediatric Practice, Vol. 17, No. 1, 2013
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review article Table 4. Comparison of Recommendations for Screening for Anemia Organization
Recommendations
High-risk groups
American Academy of Pediatrics
Screening is recommended at 9 to 12 months of age and again 6 months later for all infants in populations with high rates of iron deficiency, or (in populations with a rate of 5 percent or less) in infants with medical risks or whose diet puts them at risk of iron deficiency
Premature infants
Screening is recommended for children from low-income or newly immigrated families between 9 and 12 months of age, then 6 months later, then annually from 2 to 5 years of age
Infants fed non–iron-fortified formula or cow’s milk before 12 months of age
Centers for Disease Control and Prevention
Screening should be considered for preterm and low–birth-weight infants before 6 months of age if they are not fed iron-fortified formula Infants and young children with risk factors should be assessed at 9 to 12 months of age, and again 6 months later
Low–birth-weight infants Infants fed low-iron formula Breastfed infants older than 6 months who are not receiving iron supplementation
Breastfed infants older than 6 months without adequate iron supplementation Children who consume more than 24 oz of cow’s milk per day Children with special health care needs (e.g., medications that interfere with iron absorption, chronic infection, inflammatory disorders, blood loss)
Beginning in adolescence, all nonpregnant women should be screened every 5 to 10 years U.S. Preventive Services Task Force
No recommendation for or against screening for iron deficiency anemia in asymptomatic children 6 to 12 months of age
Premature infants
Screening at 9 to 12 months of age is recommended for high-risk infants
Recent immigrants
Low–birth-weight infants
Adolescent girls who follow fad diets or who are obese Adult females
Information from references 9, 18, and 19.
6-phosphate dehydrogenase (G6PD) deficiency should be considered if the patient’s ethnicity or family history is a risk factor.
Microcytic Anemia in an Infant A 12-month-old boy of Mediterranean descent presents for a health maintenance examination. He consumes 32 oz of whole milk daily. The medical history and review of systems are normal. On physical examination, the patient is found to have an elevated weight for length. No other abnormalities are noted. Laboratory testing shows that the patient’s Hgb level is 9.8 g per dL (98 g per L). The MCV is low (70 μm3 [70 fL]), and the RBC distribution width is elevated (18 percent). The RBC count is 5.0 × 106 per mm3 (5.0 × 1012 per L). The child is presumptively treated with oral iron therapy, and after one month, the Hgb level is 11.2 g per dL (112 g per L). After another month of iron therapy, the Hbg level has normalized at 13 g per dL (130 g per L). Neither the Centers for Disease Control and Prevention, the American Academy of Pediatrics, nor the U.S.
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Asian Journal of Paediatric Practice, Vol. 17, No. 1, 2013
Preventive Services Task Force recommends universal screening for anemia. Instead, children at risk should be identified and then undergo evaluation between nine and 12 months of age (Table 4).9,18,19 This child’s excessive milk consumption and weight are risk factors for anemia20-22; therefore, evaluation is justified. Iron deficiency is characterized by microcytosis with an elevated RBC distribution width. Because the anemia is mild and the history and laboratory values are consistent with iron deficiency, it is appropriate to treat presumptively with oral iron therapy and repeat testing in one month23 (Figure 1). Treatment for mild anemia is 3 to 6 mg of elemental iron per kg per day.24 Once-daily dosing results in similar improvement as two- or threetimes-daily dosing and does not significantly increase adverse effects.25 An Hgb increase of more than 1 g per dL (10 g per L) after iron therapy has been started confirms the diagnosis of iron deficiency. If the Hgb level does not increase or if the initial anemia is severe, further evaluation should include a complete blood count (CBC), peripheral blood
review article Table 5. Calculation of the Mentzer Index Example patient
MCV (fL)
RBC count (× 106 per mm3)
5-year-old black child with pallor
64
5.3
12
Mentzer index < 13 suggests thalassemia
2-year-old child who drinks 30 oz of cow’s milk daily
72
4.8
15
Mentzer index > 13 suggests iron deficiency
Mentzer index Comments (MCV/RBC count)
Note: Although commonly used, the Mentzer index and other indices used to differentiate iron deficiency from thalassemia are not uniformly reliable.26 MCV = Mean corpuscular volume; RBC = Red blood cell. Information from references 26 and 27.
smear, iron studies, and fecal occult blood testing. Lead testing should also be considered. Patients with thalassemia typically have a Mentzer index of less than 13 (Table 5)26,27 and may be of African, Asian, or Mediterranean descent. In patients with thalassemia, Hgb electrophoresis may show an increase in levels of Hgb A or F. Sideroblastic anemia, which is rare, results in a high RBC distribution width with normal or elevated iron levels; diagnosis requires bone marrow aspiration. Iron is utilized by tissues other than bone marrow, including the brain. Studies show an association between iron deficiency and impaired neurocognitive performance.28-33 The association is not definitively causal, and studies do not show an immediate improvement in psychomotor development or cognitive performance after treatment has commenced. However, long-term studies are few and conflicting.34 Until further studies provide clarity, iron deficiency should be treated until one month after normalization of Hgb levels. The total treatment course is typically three months. If a longer course is needed, further investigation should include a CBC, peripheral blood smear, iron studies, and fecal occult blood testing.23
Normocytic Anemia in an Older Child A previously healthy eight-year-old boy of Filipino descent presents with increasing fatigue for the past five days. He has low-grade fever and nonspecific musculoskeletal pain. He has had no symptoms of upper respiratory infection. Physical examination shows pallor, pale conjunctivae, scattered facial petechiae, tachycardia, and a flow murmur. There is no scleral icterus. A CBC shows an Hgb level of 7.8 g per dL (78 g per L) and an MCV of 90 μm3 (90 fL). The white blood cell count is 14,000 per mm3 (14.00 × 109 per L), and the platelet count is 368 × 103 per mm3 (368 × 109 per L). The reticulocyte count is 0.21 percent (normal range in an eight-year-old is 0.5 to 1.0 percent). The peripheral smear shows 21 percent lymphoblasts.
This is normocytic anemia in a previously healthy child. Although normocytic anemia commonly results from early iron deficiency or chronic disease, this patient has findings suggesting an acute process (pallor, tachycardia, and flow murmur). Hemoglobinopathies, enzyme defects, RBC membrane defects, and other hemolytic anemias result in normocytic anemia. Given his sex and ethnicity, G6PD deficiency is in the differential diagnosis. However, he has no history and is not jaundiced, which makes hemolysis unlikely. In a child who otherwise appears well and has had a recent viral infection, transient erythroblastopenia of childhood (TEC) should be considered. This condition usually occurs in children six months to three years of age after a viral infection or exposure to toxic agents. It is the result of an immune reaction against erythroid progenitor cells. In patients with TEC, the initial reticulocyte count is zero, but slowly increases as the patient recovers, which typically occurs within two months of onset.35 This child’s age, ill appearance, and lack of viral symptoms make TEC less likely. The first step in evaluation of normocytic anemia is determination of the reticulocyte count (Figure 2) to distinguish cases of increased RBC turnover, such as hemolysis, from bone marrow disorders. The low reticulocyte count suggests bone marrow hypofunction. Leukemia and aplastic anemia reduce RBC production. Because leukemia is a consideration in the differential diagnosis for this patient, a peripheral smear is ordered, which confirms the diagnosis of leukemia. If the diagnosis had been less clear, further evaluation would have included a careful history and testing of iron levels and liver, kidney, and thyroid function to assess for chronic disease. Low iron saturation suggests early iron deficiency. Normal or elevated iron saturation in the presence of low serum iron levels suggests infection or chronic disease.
Asian Journal of Paediatric Practice, Vol. 17, No. 1, 2013
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review article Evaluation of Low Hemoglobin Levels Low Hgb level Confirm level and add indices; evaluate MCV
Low MCV
Normal MCV
Microcytic anemia
High MCV
Normocytic anemia (see Figure 2)
Macrocytic anemia (see Figure 3)
Is anemia mild, and are history and indices consistent with iron deficiency? Yes Test presumptively; retest in one month Hgb increased by > 1.0 g per dL (10 g per L)? Yes Diagnosis confirmed; counsel about cowâ&#x20AC;&#x2122;s milk consumption; continue treatment for an additional one to two months
No
No
Iron deficiency (not responsive to oral therapy)
Iron studies, Hgb electrophoresis, lead level
Anemia of chronic disease
Thalassemia
No cause found
Treat underlying disease
Counsel or refer as needed
Refer to pediatric hematologist
Test for gastrointestinal bleeding; refer to pediatric gastroenterologist
Figure 1. Algorithm for evaluation of low hemoglobin (Hgb) levels in children. (MCV = Mean corpuscular volume.)
Other Considerations Macrocytic anemia is rare in children. The initial workup is a peripheral smear (Figure 3).36 The presence of hypersegmented neutrophils signals a megaloblastic anemia, which is caused by folate or vitamin B12 deficiency or other disorders of DNA synthesis. Nonmegaloblastic causes of macrocytosis include alcoholism, hemolysis, hemorrhage, hepatic disease, bone marrow disorders (e.g., aplastic anemia, myelodysplasia, sideroblastic anemia), and hypothyroidism. Subsequent testing is based on peripheral smear findings.36
should receive a blood transfusion while evaluation for the underlying cause is undertaken. Transfusion is typically given at a volume of 10 mL per kg, infused at a rate of no more than 5 mL per kg per hour. The patient should be monitored for signs of heart failure during transfusion.
Treatment and Prevention
The U.S. Food and Drug Administration recommends adequate iron intake to prevent iron deficiency anemia (Table 69). One half of American toddlers do not receive the recommended daily intake of iron.37 However, it is not clear whether iron supplementation reduces the incidence of anemia. Studies in countries outside the United States have had promising results. However, a randomized study in the United States demonstrated that high-risk, six-month-old infants who received 10 mg of supplemental iron per day did not have a reduced incidence of anemia or abnormal indices indicative of iron deficiency.38
Iron deficiency is treated orally; otherwise, treatment is geared toward the underlying cause of anemia. Symptomatic patients and those with severe anemia
In the first four to six months of life, full-term infants use hepatic stores of iron in addition to dietary iron in formula or breast milk; iron supplementation is
Older children and adolescents are also at risk of anemia. The combination of a growth spurt and the onset of menstruation leaves adolescent girls at particularly high risk of iron deficiency anemia.
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Asian Journal of Paediatric Practice, Vol. 17, No. 1, 2013
review article Evaluation of Normocytic Anemia Normocytic anemia Review patient history for underlying disease; obtain reticulocyte count and peripheral smear
Reticulocyte count low (indicating bone marrow hypofunction)
Reticulocyte count high (indicating increased red blood cell turnover) Laboratory testing for hemolysis (bilirubin, lactate dehydrogenase, and haptoglobin levels
Medical disease suspected
Laboratory testing for renal, liver, or thyroid disease
Underlying inflammation
Abnormal smear
Consider iron studies Consider bone for diagnosis of marrow disorders anemia (e.g., leukemia, of chronic disease myelofibrosis)
Positive
Negative
Consider enzyme defects, autoimmune disorders, hemoglobinopathies, or membrane disorders; test accordingly
Consider blood loss, hypersplenism, or mixed disorder
Refer to pediatric hematologist
Cause unknown
Cause unknown
Figure 2. Algorithm for evaluation of normocytic anemia in children.
Evaluation of Macrocytic Anemia Macrocytic anemia Order peripheral smear to evaluate for hypersegmented neutrophils (indicating megaloblastic anemia)
Nonmegaloblastic anemia
Megaloblastic anemia
Obtain reticulocyte count
Test folate and vitamin B12 levels
Vitamin B12 level low
Folate level low
Treat and retest; consider treating for pernicious anemia or ileal disease
Treat and retest; provide dietary counseling
No improvement
Both levels low or normal
Low Evaluate for alcoholism, hypothyroidism, or hepatic disease
Refer to pediatric hematologist for consideration of bone marrow disorders
High Evaluate for hemolysis or hemorrhage
Cause unknown
Figure 3. Algorithm for evaluation of macrocytic anemia in children.
Asian Journal of Paediatric Practice, Vol. 17, No. 1, 2013
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review article Table 6. Daily Iron Requirements for Infants and Young Children Age
Daily iron requirement
Source
7. Bizzarro MJ, Colson E, Ehrenkranz RA. Differential diagnosis and management of anemia in the newborn. Pediatr Clin North Am. 2004;51(4):1087-1107.
Up to 4 to 6 months 0.27 mg (full-term infants)
Breast milk or ironfortified formula
8. Olhs RK, Christensen RD. Diseases of the blood. In: Behrman RE, Kliegman R, Jenson HB, eds. Nelson Textbook of Pediatrics. 17th ed. Philadelphia, Pa.: Saunders; 2004:1604-1634.
4 to 6 months to 1 year (full-term infants)
Breast milk or formula plus iron-rich foods*
9. Pediatric Nutrition Handbook. 6th ed. Elk Grove Village, Ill.: American Academy of Pediatrics; 2009:403-422.
11 mg
1 month to 1 year 2 to 4 mg per kg (premature or lowâ&#x20AC;&#x201C; birth-weight infants)
1 to 3 years
7 mg
Iron-fortified preterm formula or iron supplementation (2 mg per kg per day) plus breast milk and iron-rich foods Iron-rich foods
*If a full-term breastfed infant cannot consume adequate iron after 6 months of age, supplementation is necessary (1 mg per kg per day). Information from reference 9.
not required in these children. Preterm infants do not have adequate hepatic iron stores and require larger amounts of iron for catch-up growth. These infants should receive supplemental iron. Starting at four to six months of age, infants require an additional source of iron.39 One half cup of iron-fortified cereal contains 90 percent of the recommended daily intake of iron for a six- to 12-month-old infant. Lean meats, beans, ironfortified whole grains, tofu, and spinach are other ironrich options for infants who consume solid foods. REFERENCES 1. Irwin JJ, Kirchner JT. Anemia in children. Am Fam Physician. 2001;64(8):1379-1386. 2. Oski FA, Brugnara C, Nathan DG. A diagnostic approach to the anemic patient. In: Nathan and Oskiâ&#x20AC;&#x2122;s Hematology of Infancy and Childhood. 6th ed. Philadelphia, Pa.: Saunders; 2003:409-418. 3. Robertson J, Shilkofski N, eds. The Harriet Lane Handbook. 17th ed. Philadelphia, Pa.: Mosby; 2005:337. 4. Cusick SE, Mei Z, Freedman DS, et al. Unexplained decline in the prevalence of anemia among US children and women between 1988-1994 and 1999-2002. Am J Clin Nutr. 2008;88(6):1611-1617. 5. Oken E, Rifas-Shiman SL, Kleinman KP, Scanlon KS, Rich-Edwards JW. Trends in childhood anemia in a Massachusetts health maintenance organization, 19872001. MedGenMed. 2006;8(3):58. 6. Borland EW, Dalenius K, Grummer-Strawn L, Mackintosh H, Polhamus B, Smith BL. Pediatric Nutrition Surveillance: 2007 Report. Atlanta, Ga.: Centers for Disease Control and Prevention; 2009.
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10. Wright RO, Tsaih SW, Schwartz J, Wright RJ, Hu H. Association between iron deficiency and blood lead level in a longitudinal analysis of children followed in an urban primary care clinic. J Pediatr. 2003;142(1):9-14. 11. Stoltzfus RJ, Edward-Raj A, Dreyfuss ML, et al. Clinical pallor is useful to detect severe anemia in populations where anemia is prevalent and severe. J Nutr. 1999;129(9):1675-1681. 12. Montresor A, Albonico M, Khalfan N, et al. Field trial of a haemoglobin colour scale: an effective tool to detect anaemia in preschool children. Trop Med Int Health. 2000;5(2):129-133. 13. Strobach RS, Anderson SK, Doll DC, Ringenberg QS. The value of the physical examination in the diagnosis of anemia. Correlation of the physical findings and the hemoglobin concentration. Arch Intern Med. 1988;148(4):831-832. 14. Luby SP, Kazembe PN, Redd SC, et al. Using clinical signs to diagnose anaemia in African children. Bull World Health Organ. 1995;73(4):477-482. 15. Mast AE, Blinder MA, Lu Q, Flax S, Dietzen DJ. Clinical utility of the reticulocyte hemoglobin content in the diagnosis of iron deficiency. Blood. 2002;99(4):1489-1491. 16. White KC. Anemia is a poor predictor of iron deficiency among toddlers in the United States: for heme the bell tolls. Pediatrics. 2005;115(2):315-320. 17. Ullrich C, Wu A, Armsby C, et al. Screening healthy infants for iron deficiency using reticulocyte hemoglobin content. JAMA. 2005;294(8):924-930. 18. Centers for Disease Control and Prevention. Recommendations to prevent and control iron deficiency in the United States. MMWR Recomm Rep. 1998;47(RR-3):1-29. 19. U.S. Preventive Services Task Force. Screening for iron deficiency anemia, including iron supplementation for children and pregnant women: recommendation statement. Rockville, Md.: Agency for Healthcare Research and Quality; 2006. AHRQ publication no. 060589. http://www.ahrq.gov/clinic/uspstf/uspsiron.htm. Accessed February 18, 2010. 20. Brotanek JM, Gosz J, Weitzman M, Flores G. Iron deficiency in early childhood in the United States: risk factors and racial/ethnic disparities. Pediatrics. 2007;120(3):568-575. 21. Nead KG, Halterman JS, Kaczorowski JM, Auinger P, Weitzman M. Overweight children and adolescents: a risk group for iron deficiency. Pediatrics. 2004;114(1):104-108.
review article 22. American Academy of Pediatrics Committee on Nutrition. The use of whole cowâ&#x20AC;&#x2122;s milk in infancy. Pediatrics. 1992;89(6 pt 1):1105-1109.
31. Grantham-McGregor S, Ani C. A review of studies on the effect of iron deficiency on cognitive development in children. J Nutr. 2001;131(2S-2):649S-666S.
23. Segel GB, Hirsh MG, Feig SA. Managing anemia in pediatric office practice: part 1. Pediatr Rev. 2002;23(3): 75-84.
32. McCann JC, Ames BN. An overview of evidence for a causal relation between iron deficiency during development and deficits in cognitive or behavioral function. Am J Clin Nutr. 2007;85(4):931-945.
24. Lexi-Comp, American Pharmaceutical Association. Pediatric Dosage Handbook. 12th ed. Hudson, Ohio: LexiComp; 2005:623-626. 25. Zlotkin S, Arthur P, Antwi KY, Yeung G. Randomized, controlled trial of single versus 3-times-daily ferrous sulfate drops for treatment of anemia. Pediatrics. 2001;108(3):613-616. 26. Mentzer WC Jr. Differentiation of iron deficiency from thalassemia trait. Lancet. 1973;1(7808):882. 27. Demir A, Yarali N, Fisgin T, Duru F, Kara A. Most reliable indices in differentiation between thalassemia trait and iron deficiency anemia. Pediatr Int. 2002;44(6):612-616. 28. Walter T, De Andraca I, Chadud P, Perales CG. Iron deficiency anemia: adverse effects on infant psychomotor development. Pediatrics. 1989;84(1):7-17. 29. Lozoff B, Jimenez E, Hagen J, Mollen E, Wolf AW. Poorer behavioral and developmental outcome more than 10 years after treatment for iron deficiency in infancy. Pediatrics. 2000;105(4):e51. 30. Halterman JS, Kaczorowski JM, Aligne CA, Auinger P, Szilagyi PG. Iron deficiency and cognitive achievement among school-aged children and adolescents in the United States. Pediatrics. 2001;107(6):1381-1386.
33. Beard JL. Why iron deficiency is important in infant development. J Nutr. 2008;138(12):2534-2536. 34. Logan S, Martins S, Gilbert R. Iron therapy for improving psychomotor development and cognitive function in children under the age of three with iron deficiency anaemia. Cochrane Database Syst Rev. 2001;(2):CD001444. 35. Walters MC, Abelson HT. Interpretation of the complete blood count. Pediatr Clin North Am. 1996;43(3): 599-622. 36. Davenport J. Macrocytic anemia. Am Fam Physician. 1996;53(1):155-162. 37. U.S. Department of Agriculture. Supplementary data tables: USDAâ&#x20AC;&#x2122;s 1994-1996 continuing survey of food intakes by individuals. http://www.ars.usda.gov/P2UserFiles/ Place/12355000/pdf/Supp.pdf. Accessed September 3, 2008. 38. Geltman PL, Meyers AF, Mehta SD, et al. Daily multivitamins with iron to prevent anemia in highrisk infants: a randomized clinical trial. Pediatrics. 2004;114(1):86-93. 39. Chaparro CM. Setting the stage for child health and development: prevention of iron deficiency in early infancy. J Nutr. 2008;138(12):2529-2533.
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Asian Journal of Paediatric Practice, Vol. 17, No. 1, 2013
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CLINICAL STUDY
Septoplasty with Adenoidectomy: A Combined Procedure for Nasal Obstruction in Children K Mallikarjuna Swamy*, KP Basavaraju**
ABSTRACT Nasal obstruction in children is caused by numerous and diverse factors but the symptoms are essentially snoring, mouth breathing, sleep disturbances and rhinorrhea. The commonest causes of nasal obstruction in children are septal deviation and adenoid hypertrophy. Nasal septal deviation in children is usually due to some form of injury. Performing septoplasty alone in this age group without addressing adenoids may lead to recurrence of symptom, that is, nasal obstruction may lead to failure of procedure. Both procedures can be combined in a single sitting. We present herein a study of combined septoplasty with adenoidectomy for relief of nasal obstruction in children aged 9-15 years. Keywords: Septoplasty, adenoidectomy, nasal obstruction, children, combined procedure
T
he commonest causes of nasal obstruction in children are septal deviation and adenoid hypertrophy. Adenoids may be implicated in upper respiratory tract disease due to partial or complete obstruction of the nasal choanae. Nasal obstruction in children is caused by numerous and diverse factors but the symptoms are essentially snoring, mouth breathing, sleep disturbances and rhinorrhea.
During the development years of the child, mouth breathing may lead to severe physical developmental disorder (facial, oral, nasal and thoracic), which may lead to cognitive impairment.1 Nasal septal deviation in children is usually due to some form of injury. There is much debate as to whether septal surgery is appropriate in the growing nose.2 The main growth center of the nose is the contact area between quadrangular cartilage and vomer1 and even a minor disruption here can lead to significant problems with final midfacial contour. Furthermore, evidence is available which states that not performing surgery on children affected by nasal septal deviation can lead to dental malocclusion, facial abnormalities and respiratory morbidity. Therefore, *Assistant Professor **Professor Dept. of ENT JJMMC, Davangere, Karnataka Address for correspondence Dr K Mallikarjuna Swamy Assistant Professor Dept. of ENT JJMMC, Davangere, Karnataka E-mail: drkmallikarjuna1980@gmail.com
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Asian Journal of Paediatric Practice, Vol. 17, No. 1, 2013
not performing septal surgery in children affected by septal deviation may be more detrimental.2 Generally, if symptoms are significant, a limited septoplasty with minimal removal of cartilage is acceptable.1 Performing septoplasty alone in this age group without addressing adenoids may lead to recurrence of symptom that is, nasal obstruction may lead to failure of procedure;1 so we should combine both procedures in single sitting. Only septoplasty with adenoidectomy done separately leads to recurrence or persistence of nasal obstruction in children. Objective This is a study of combined septoplasty with adenoidectomy for relief of nasal obstruction in children aged 9-15 years. Material and methods This study included 20 children between the ages of 9-15 years undergoing adenoidectomy with septoplasty for their obstructive symptoms in Chigateri General District Hospital, Davangere, Karnataka, from November 2010 to February 2012. Among this group, there were 12 boys and eight girls. Informed written consent was taken from parents/guardians. Children included in this study met the following criteria: ÂÂ
Continuous nasal obstruction for at least three months due to deviated nasal septum (DNS) (Fig. 1).
ÂÂ
Children without allergic rhinitis.
clinical study Exclusion Criteria ÂÂ
Isolated adenoid hypertrophy cases.
ÂÂ
Use of topical intranasal/systemic decongestants or steroids.
The patients had history of mouth breathing, snoring, headache and rhinorrhea. A lateral nasopharyngeal soft tissue X-ray was taken to evaluate the size of the adenoids (Fig. 3). All children had considerably enlarged adenoids. Adenoid facies and voice were evaluated. The ear was examined to look for eustachian tube dysfunction and its effects. X-ray paranasal sinuses (PNS) was taken to rule out associated sinus infection. Nasal endoscopy was also done if required to rule out other causes like nasal polyps (Fig. 4). Complete examination of ear, nose and throat examination was done. All children were operated under general anesthesia, the septoplasty procedure was performed with minimal removal of cartilage with septal repositioning was done
(upto 5 mm of inferior strip and posterior end of the cartilage was removed); nasal cavity was packed with Vaseline gauze. The children were then put in Rose position and traditional adenoidectomy was done, using the technique of curettage. Assessment of the adenoids was made digitally prior to curetting and hemostasis achieved with gauze tamponade. The relief of nasal obstruction was assessed subjectively by follow-up of the children or parents postoperatively. The children were discharged on third day of surgery and advised for follow-up once weekly for 2-3 weeks and once in a month upto six months. Results All the 20 children included in the study had nasal obstruction. Mouth breathing was seen in 18 children (90%). Snoring was the associated complaint in 16 children (80%). Twenty percent children had rhinorrhea
Figure 1. Gross DNS to the left with caudal dislocation in a 10-year-old patient.
Figure 2. Oblique view of the same patient as in Fig. 1.
Figure 3. X-ray of the nasopharynx showing adenoid hypertrophy.
Figure 4. Endoscopic view showing DNS with adenoid in 9-year-old patient.
Asian Journal of Paediatric Practice, Vol. 17, No. 1, 2013
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clinical study Table 2. Clinical Examination Sign
No. of Patients
Percentage (%)
Septal deviation
20
100
Adenoid facies
08
40
Rhinorrhea
16
80
Caudal dislocation
00
00
Table 1. Distribution of Symptoms Among Patients Symptoms Nasal obstruction
No. of Patients Percentage (%) 20
100
Mouth breathing
18
90
Snoring
16
80
Rhinorrhea and headache
4
20
and headache (Table 1). On clinical examination, all 20 children had septal deviation of various degrees. Rhinorrhea was seen in 16 children (80%), eight patients had adenoid facies (40%). None of the children had caudal dislocation (Table 2). Out of 20 children, three children could not be assessed for outcome of surgery since they dropped out of their follow-up; of the 17 patients, nine children showed improvement after one week of surgery. The remaining eight children showed relief of symptoms on the 2nd follow-up. Of these, four patients who complained of snoring and restless sleep, showed marked symptom reduction (as observed by the parents). Overall the combined procedure of septoplasty with adenoidectomy yielded good results in terms of nasal obstruction, mouth breathing and snoring. No major complications like postnasal bleeding, septal perforation or external deformity were seen. Discussion The empirical indication for adenoidectomy includes obstructive sleep apnea, recurrent rhinosinusitis and otitis media with effusion.2 The adenoids when diseased may act as a source of infection, supporting bacteria in a biofilm with resultant inflammatory changes in the mucosa of nose, nasopharynx PNS and middle ear. Surgery is recommended by American Association of Otolaryngology-Head and Neck Surgery (AAO-HNS) for infective causes including adenoiditis, where two courses of antibiotics have failed and for recurrent rhinorrhea on four occasions.3 A DNS alone is rarely significant enough to be the sole cause of obstructive breathing. Adenoidectomy appears helpful
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Asian Journal of Paediatric Practice, Vol. 17, No. 1, 2013
as a part of management of obstructive sleep apnea syndrome (OSAS) and sleep disordered breathing (SDB), but cross-sectional studies support the benefit of adenoidectomy and tonsillectomy performed together for OSAS and SDB.3 Therefore, a detailed examination is advised to exclude another cause or co-existing pathology, that is adenoid hypertrophy prior to considering septoplasty.2 During our clinical examination in outpatients, we have found adenoid hypertrophy even in children above 10 years of age. Septal deviations in children are very common and contribute significantly to nasal obstruction. In children who have septal deviations along with adenoid hypertrophy, either septoplasty or adenoidectomy alone may not give satisfactory results. Hence, septoplasty with adenoidectomy helps in relieving nasal obstruction in such children without any major complications. Conclusion We performed combined septoplasty with adenoidectomy in 20 children between 9 and 15 years of age with good results without any major complications. Subjective and clinical assessment in these children showed significant improvement in nasal obstruction and mouth breathing. There was also marked improvement in general health and scholastic performance after long-term follow-up. Septoplasty with adenoidectomy when done separately leads to recurrence or persistence of nasal obstruction in children. So, we conclude that combined procedure septoplasty with adenoidectomy is required for relief of nasal obstruction in children aged 9-15 years. References 1. Verucchi F, Caropreso CA. Indications and contraindications for septoplasty in children. VI IAPO Manual of Pediatric Otorhinolaryngol. Available at: http://www. iapo.org.br/manuals/VI_manual_en_Fulvio.pdf. 2. Lawrence R. Pediatric septoplasty: a review of the literature. Int J Pediatr Otorhinolaryngol 2012;76(8):1078-81. 3. Cervera Escario J, Castillo Martín FD, Gómez Campderá JA, Gras Albert JR, Pérez Piñero B, Villafruela Sanz MA. Indications for tonsillectomy and adenoidectomy: consensus document by the Spanish Society of ORL and the Spanish Society of Pediatrics. Acta Otorrinolaringol Esp 2006;57(2):59-65. 4. Adams DA, Cinnamon MJ. Pediatric otolaryngology. Volume 6. Scott-Brown’s Otorhinolaryngology: Head and Neck Surgery. 6th edition, Butterworth-Heinemann. 1997 nasal obstruction and rhinorrhea in infants and children, 6w/17/1-14.
CLINICAL STUDY
Vitamin E Level in Normal Indian Children Geeta Gathwala, Subrika Yadav, Ojawanai, G Lal
ABSTRACT Objective/Background: The study was conducted with an aim to determine vitamin E levels in normal Indian children. Vitamin E has positive effect on cardiovascular health through its ability to influence signaling process in platelets and also has significant role in neurodegenerative disorders. Vitamin C and vitamin E supplementation retards the progression of coronary arteriosclerosis during early stage following cardiac transplantation. It also mediates the generation and availability of superoxide and nitric oxide and is beneficial in preventing prostate cancer or delaying disease progression. Material and methods: Study included 30 healthy children of either sex, they were divided into three groups each of 10 children, that is, Group I (2-4 years) mean age 3.42 years, Group II (4-6 years) mean age 5.35 years and Group III (6-10 years) mean age eight years. Vitamin E levels were measured in their serum fluorometric micro methods for serum tocopherol as described by Hansen and Warwick. Result: In Group I, the mean ± SEM (standard error of mean) value of vitamin E was 2.74 ± 1.10 µg/ml, in Group II it was 2.82 ± 1.11 µg/ml and in Group III it was 2.93 ± 1.12 µg/ml. Conclusion: Serum vitamin E levels in healthy Indian children is 2.82 ± 1.11µg/ml and there is no sex predilection. Keywords: Vitamin E, healthy Indian children
V
itamin E was discovered by Even and Bishop. Vitamin E is indispensable for reproduction and prevents diseases associated with oxidative stress, such as cardiovascular disease, cancer, chronic inflammation and neurologic disorders.1-3 Vitamin E and ω-3 fatty acids are positive effectors of cardiovascular health through their ability to influence signaling process in platelets. Vitamin E has significant role in neurodegenerative disorders.4,5 Vitamin C and vitamin E supplementation retards progression of coronary arteriosclerosis during early stage following cardiac transplantation.6-8 Vitamin E may mediate the generation and availability of superoxide and nitric oxide.9 Vitamin E may be beneficial in preventing prostate cancer or delaying disease progression.10 At transcriptional level, several genes (CD36, a-tocopherol transfer protein [a-TTP], a-topomyosin and collagenase) are modulated by a-tocopherol. These effects result in inhabitation of smooth muscle cell proliferation, platelet aggregation and monocyte adhesion and thus protect atherosclerosis.11 Dept. of Pediatrics and Biochemistry Pt. BD Sharma Postgraduate Institute of Medical Sciences Rohtak, Haryana Maulana Azad Medical College, New Delhi Address for correspondence Dr Geeta Gathwala Professor and Head Dept. of Pediatrics 6J/8, Postgraduate Institute of Medical Sciences Rohtak, Haryana E-mail: g_gathwala@hotmail.com
Vitamin E has an additional positive effect on autoimmune disease by decreasing proinflammatory cytokines, lipid mediators and has important role in rheumatoid arthritis patients.12 Prolonged vitamin E deficiency leads to peripheral neuropathy, muscle weakness and ophthalmoplegia, which are irreversible. Fifty to 70% of children with chronic cholestasis are vitamin E deficient, even when consuming standard supplements of vitamin E.7 a-tocopherol is the most bioactive form of vitamin E followed in order by b, g and d tocopherol. The most commonly used pharmacologic form of a-tocopherol is a totally synthetic product and has both D and L stereoisomers now designated as racemic a-tocopherol. This synthetic form has considerably less bioactivity (approximately 75%) than the pure naturally occurring form. Vitamin E is absorbed into intestinal mucosa by nonsaturable, noncarrier-mediated passive diffusion process and absorption is maximal in median small intestine and none is absorbed in large intestine. Human body stores 40 mg/kg vitamin E, 77% of which is in adipose tissue, 20% in muscle and only 1% in liver. Since, there is paucity of Indian data, the study was planned to assess the serum vitamin E levels in normal Indian children. MATERIAL AND METHODS This study was conducted in 30 healthy children of either sex, who were divided into three groups each of 10 children, that is, Group I (2-4 years) mean age 3.42 years, Group II (4-6 years) mean age 5.35 years and Group III (6-10 years) mean age eight
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clinical study years. Vitamin E levels were measured in their serum by fluorometric micro method for serum tocopherol as described by Hansen and Warwick.13 RESULT In Group I, the mean ± SEM (standard error of mean) value of vitamin E was 2.74 ± 1.10 ± µg/ml, in Group II it was 2.82 ± 1.11 ± µg/ml, while in Group III it was 2.93 ± 1.12 ± µg/ml (Table 1). On inter group comparison, using students t-test the difference in values amongst the three groups was found to be nonsignificant. Since, there was no significant difference in serum vitamin E levels in different age groups of children and there was no specific gender predilection, we assumed the 60 children belonging to one group to report the serum vitamin E level in normal Indian children to be 2.82 ± 1.11 µg/ml. DISCUSSION Among the nutritional factors contributing to maintain health during aging, fat-soluble vitamins are crucial to protect against free radical generated degenerative processes or impaired efficiency of immune system.14,15 Antioxidant activity is provided by a number of naturally occurring substances including a-tocopherol (vitamin E) and b-carotene, whose effects are mediated by their capacity to quench ringlet oxygen, scavenge free radicals and prevent the formation of free radicals and is used to prevent ultraviolet-induced skin damage.16,17 Inadequate intake of several vitamins is linked to chronic diseases, including coronary heart disease, cancer and osteoporosis.18 Enterally fed premature infants are supplemented fat-soluble vitamins.19 The normal serum vitamin E levels in children and adults range from 5 to 20 µg/ml and are slightly lower in infants and young children, that is, 3-10 µg/ml. In our study also, vitamin E levels were found to increase with age, that is, mean ± SEM was 2.74 ± 1.10, 2.82 ± 1.11, 2.93 ± 1.12 in Groups I, II and III, respectively (Table 1). Although, this increment in vitamin E levels
Age (years)
Mean age (years)
Vitamin (mean ± SEM)
P value
I
2-4
3.42
2.74 ± 1.10
NS
II
4-6
5.35
2.82 ± 1.11
NS
III
6-10
8.00
2.93 ± 1.12
NS
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conclusion Serum vitamin E levels in healthy Indian children is 2.82 ± 1.11 µg/ml and there is no sex predilection. REFERENCES 1. Brigelius-Flohé R, Kelly FJ, Salonen JT, Neuzil J, Zingg JM, Azzi A. The European perspective on vitamin E: current knowledge and future research. Am J Clin Nutr 2002;76(4):703-16. 2. Marchioli R, Schweiger C, Levantesi G, Tavazzi L, Valagussa F. Antioxidant vitamins and prevention of cardiovascular disease: epidemiological and clinical trial data. Lipids 2001;36 Suppl:S53-63. 3. Ricciarelli R, Zingg JM, Azzi A. Vitamin E: protective role of a Janus molecule. FASEB J 2001;15(13):2314-25. 4. Butterfield DA, Castegna A, Drake J, Scapagnini G, Calabrese V. Vitamin E and neurodegenerative disorders associated with oxidative stress. Nutr Neurosci 2002;5(4):229-39. 5. Martin A, Youdim K, Szprengiel A, Shukitt-Hale B, Joseph J. Roles of vitamins E and C on neurodegenerative diseases and cognitive performance. Nutr Rev 2002;60(10 Pt 1):308-26. 6. Liu L, Meydani M. Combined vitamin C and E supplementation retards early progression of arteriosclerosis in heart transplant patients. Nutr Rev 2002;60(11):368-71. 7. Praticò D. Vitamin E: murine studies versus clinical trials. Ital Heart J 2001;2(12):878-81. 8. Soares KV, McGrath JJ. Vitamin E for neurolepticinduced tardive dyskinesia. Cochrane Database Syst Rev 2001;(4):CD000209. 9. Chow CK, Hong CB. Dietary vitamin E and selenium and toxicity of nitrite and nitrate. Toxicology 2002;180(2):195-207. 10. Fleshner NE. Vitamin E and prostate cancer. Urol Clin North Am 2002;29(1):107-13, ix.
Table 1. Effect of Age on Vitamin E Levels Group
was not found to be significant, which may be due to the minor differences in age of different groups. A ratio of <0.6 mg in children under 12 years and <0.8 mg total tocopherol per gram of lipid in older children and adults, represents vitamin E deficiency.1
11. Ricciarelli R, Zingg JM, Azzi A. Vitamin E 80th anniversary: a double life, not only fighting radicals. IUBMB Life 2001;52(1-2):71-6. 12. Tidow-Kebritchi S, Mobarhan S. Effects of diets containing fish oil and vitamin E on rheumatoid arthritis. Nutr Rev 2001;59(10):335-8.
clinical study 13. Hansen LG, Warwick WJ. A fluorometric micro method for serum tocopherol. Tech Bull Regist Med Technol 1966;36(6):131-6. 14. Sorg O, Tran C, Saurat JH. Cutaneous vitamins A and E in the context of ultraviolet- or chemically-induced oxidative stress. Skin Pharmacol Appl Skin Physiol 2001;14 (6):363-72. 15. Rock E, Winklhofer-Roob BM, Ribalta J, Scotter M, Vasson MP, Brtko J, et al. Vitamin A, vitamin E and carotenoid status and metabolism during ageing: functional and nutritional consequences (VITAGE PROJECT). Nutr Metab Cardiovasc Dis 2001;11 (4 Suppl):70-3.
16. Anstey AV. Systemic photoprotection with alphatocopherol (vitamin E) and beta-carotene. Clin Exp Dermatol 2002;27(3):170-6. 17. Podhaisky HP, Wohlrab W. Is the photoprotective effect of vitamin E based on its antioxidative capacity? J Dermatol Sci 2002;28(1):84-6. 18. F airfield KM, Fletcher RH. Vitamins for chronic disease prevention in adults: scientific review. JAMA 2002;287(23):3116-26. 19. Greer FR. Fat-soluble vitamin supplements for enterally fed preterm infants. Neonatal Netw 2001;20(5):7-11.
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A Road Map for Fever of Unknown Origin in Children Fever of unknown origin (FUO) in adults is conventionally defined by the occurrence of body temperatures above 38.3° C (101° F) for a period of three weeks without any identified etiology after a period of 1-week hospitalization. The issue of FUO in pediatrics is rather hazy and still represents a challenging diagnostic dilemma. Most of the available data are limited to nationwide cohorts of patients of any age. The major difficulty in establishing a diagnosis is that the characteristic features rendering specific disorders clinically recognizable are absent or subtle, hence only a painstaking questioning on family background may elicit the correct investigative path. No diagnostic algorithms are actually available and clinicians must rely on a very careful step-by-step evaluation of the single patient. The need for invasive diagnostic techniques should be closely taken into consideration when laboratory tests or simple imaging procedures fail to discern the origin of FUO. Fevers with no reasonable explanation and no localizing signs often conceal different common diseases in children, which tend to display an unusual or atypical pattern. The principal causes behind FUO in pediatric age remain infections, followed by collagen vascular diseases and neoplastic disorders, although most children with malignancies present other systemic signs or suggestive laboratory abnormalities. The possibility of auto-inflammatory syndromes, drug fever and factitious fever should also be taken into account. Source: Rigante D, Esposito S. Int J Immunopathol Pharmacol 2013;26(2):315-26. Adding mild hypothermia to neonatal extracorporeal membrane oxygenation (ECMO) in infants with very severe cardiorespiratory problems did not improve neurological outcomes at two years, a randomized trial reported online in Pediatrics. Infants randomized to receive ECMO with cooling showed a mean cognitive score of 88.0 (standard deviation [SD] 16.2) at two years compared with 90.6 (SD 13.1) for those who received ECMO alone, a nonsignificant difference. According to a new study reported at the American Thyroid Association meeting, newborns have sufficient iodine levels whether they’re breastfed or formula-fed. In a single-center study in the Boston area, urine iodine concentrations were similar and well above the required 100 µg/l for infants who received only breastmilk, only formula or a combination of the two.
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case report
Lichen Sclerosus et Atrophicus in a Young Girl YS Marfatia*, SoNIA JAIN**
ABSTRACT Lichen sclerosus et atrophicus is a chronic inflammatory dermatosis that results in white plaques and epidermal atrophy. The condition has both genital and extragenital presentations. Here we describe the case of a 12-year-old girl who presented to us with white plaques over her genitals and no manifestation of extragenital disease. Keywords: Lichen sclerosus et atrophicus, lichen albus, white spot disease
L
ichen sclerosus is a chronic inflammatory dermatosis that most commonly affects the anogenital region and leads to intractable pruritus and soreness. The condition is more common in females1 as in the present case also the patient is a young girl who presented with complaints of white plaques with itching over the genitals since her prepubertal years. The condition involves the risk of malignant transformation more so over the genital lesions but the precise incidence has not been defined. Pathophysiologically, the condition is associated with the presence of autoantibodies to glycoprotein extracellular matrix protein 1 (ECM-1).2 Several risk factors have also been proposed including autoimmune diseases, infections and genetic predisposition.3 There is evidence of its association with thyroid disease.4
Figure 1. Photograph showing labial atrophy.
CASE SUMMARY A 12-year-old young girl presented to us with depigmented patches over the genitals, which had an insidious onset and were gradually progressive over a period of one year (Fig. 1). She had moderate itching over the site and she had seen many doctors for her complaints but had no relief. On dermatological examination, labia majora showed atrophy along with depigmentation of the labia minora (Fig. 2). The depigmented patches extended from the fourchette to *Professor and Head Dept. of Skin and VD, Baroda Medical College SSG Hospital, Raopura, Vadodara, Gujarat **Professor Dept. of Skin and VD MGIMS, Sewagram, Wardha, Maharashtra Address for correspondence Dr Sonia Jain A-14, Dhanvantri Nagar MGIMS, Sewagram, Wardha, Maharashtra E-mail: soniapjain@rediffmail.com
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Figure 2. Photograph showing depigmented patch over the labia.
the vestibule and she had no oral or cutaneous lesions elsewhere. There was no history of sexual abuse or any high-risk behavior and none of the family members had a similar clinical picture. She had no urinary or bowel complaints. We performed a labial biopsy
case report also described the association of LSA with infection, autoimmunity, and trauma. There has been a case on the records wherein Virdi and Kanwar reported the co-existence of localized cutaneous morphea with LSA and submucosal fibrosis in a middle-aged man in his late-thirties.5 Another citation of a similar association between the aforesaid conditions has been mentioned by Prasad and Padmavathy et al., wherein they reported a case of a young male in his mid-twenties who presented with generalized morphea and LSA along with osteolytic bone changes.6 However, our patient showed no signs of morphea or any other systemic disease. Figure 3. Histology showing homogenization of the collagen and inflammatory infiltrate in the dermis.
from the depigmented patch after taking a written informed consent and the histopathological findings were consistent with Lichen sclerosus et atrophicus (LSA) showing homogenization of the collagen and inflammatory infiltrate in the dermis (Fig. 3). DISCUSSION Lichen sclerosus is also known as LSA, balanitis xerotica obliterans (BXO) in men, Csillag’s disease, White spot disease, Lichen albus and Krauosis vulvae. LSA was first described in 1887 by Dr Hallopeau. A case series of 42 children (all females) suffering from LSA has been reported by Shirley A Warrington and Camille de San Lazaro where they found a high incidence of sexual abuse associated with a higher incidence of LSA in pediatric population. Besides, they
REFERENCES 1. Tasker GL, Wojnarowska F. Lichen sclerosus. Clin Exp Dermatol 2003;28(2):128-33. 2. Chan I, Oyama N, Neill SM, Wojnarowska F, Black MM, McGrath JA. Characterization of IgG autoantibodies to extracellular matrix protein 1 in Lichen sclerosus. Clin Exp Dermatol 2004;29(5):499-504. 3. Yesudian PD, Sugunendran H, Bates CM, O’Mahony C. Lichen sclerosus. Int J STD AIDS 2005;16(7):465-73, test 474. 4. Birenbaum DL, Young RC. High prevalence of thyroid disease in patients with lichen sclerosus. J Reprod Med 2007;52(1):28-30. 5. Virdi SK, Kanwar AJ. Generalized morphea, Lichen sclerosis et atrophicus associated with oral submucosal fibrosis in an adult male. Indian J Dermatol Venereol Leprol 2009;75(1):56-9. 6. Prasad PV, Padmavathy L, Sethurajan S, Kumar P, Rao L. Generalised morphoea with Lichen sclerosus et atrophicus and unusual bone changes. Indian J Dermatol Venereol Leprol 1995;61(2):113-5.
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Children with medically unexplained functional abdominal pain (FAP) are vulnerable to developing anxiety disorder or depression in adolescence and young adulthood, even if the pain resolves. Hence, chronic or recurrent pain should be addressed by a multidisciplinary health team that can evaluate the physical, emotional/ mental and interpersonal aspects of pain in order to develop a treatment plan that addresses all the factors that contribute to pain, according to Lynn S Walker, PhD, from the Division of Adolescent and Young Adult Health, Monroe Carell Jr Children’s Hospital at Vanderbilt University in Nashville, Tennessee. The study is published online August 12 in Pediatrics. (Source: Medscape)
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case report
Massive Fetomaternal Hemorrhage Kannan Venkatnarayan*, Rajeev Thapar**, Himanshu Sharma
ABSTRACT Massive fetomaternal hemorrhage (FMH) is a rare entity and the diagnosis needs to be determined by performing a KleihauerBetke test (KBT) of the maternal blood. We present a case of massive FMH presenting as severe neonatal anemia. The varied presentations of FMH and its management are discussed. Keywords: Fetomaternal hemorrhage, Kleihauer-Betke test
T
he ability of fetal red cells passing the placental membrane was demonstrated by Chown in 1954.1 Fetal red cells in the maternal circulation can be detected with the Kleihauer acid-elution method by relative acid resistance from fetal hemoglobin to adult hemoglobin.2 About 40-50% of pregnancies usually in late gestation have fetal red cells in the maternal circulation.3 In 98% of the cases, the loss of blood is minimal, usually only < 0.1 ml.4 Fetomaternal hemorrhage (FMH) of a significant volume is rare with a frequency of about 0.2 per 1,000 pregnancies.5 Massive FMH has been defined as bleeding in which > 150 ml of fetal blood is found in maternal circulation.6 Diagnosis is difficult and is usually made postpartum. CASE PRESENTATION A male neonate weighing 2,700 g was born by normal spontaneous delivery to a 36-year-old gravida II para I mother at 36 weeks three days of gestation at a nursing home. Mother was a known case of hypothyroidism, on treatment for last six years. Ultrasound evaluation done in the second trimester showed a single fetus with evidence of a small fibroid over the anterior wall. Both parents had blood group O positive. However, there was no history of any trauma or any vaginal bleed occurring in any of the trimesters. The baby cried immediately after birth with Apgar scores of 7 *Assistant Professor Dept. of Pediatrics Command Hospital (Eastern Command), Alipore, Kolkata **Postdoctoral Fellow Dept. of Neonatology AIIMS, New Delhi Address for correspondence Dr Kannan Venkatnarayan Assistant Professor Dept. of Pediatrics Command Hospital (Eastern Command), Kolkata - 700 027 E-mail: venkatnarayan_kannan@yahoo.co.in
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and 8 at 1 and 5 minutes, respectively, and did not require any active resuscitation. Baby was noticed to have significant pallor immediately after delivery and advised treatment at a higher center equipped with neonatal intensive care unit (NICU) facility. The baby was brought to our hospital at around 12 hours of age. At presentation, there was noticeable pallor and mild generalized edema along with tachypnea with normal temperature and heart rate. Blood pressure was also found to be normal. The most significant finding was the oxygen saturation, which was very low, ranging 35-40% on room air. This responded dramatically to hood box oxygen of 6 L/min. Tissue perfusion as measured by capillary refill time was less than three second and evidence of any hematoma or bruise was lacking. Also, there was a Grade II/VI systolic murmur heard best in the apical area. There was presence of bilateral nontender, nonmatted axillary lymphadenopathy. Liver or spleen was not palpable. A complete blood count revealed that hemoglobin (Hb) was 6.6 g/dl, hematocrit 22.1%, white blood cell count 24 × 10³/mm3, differential cell count: Neutrophils 54%, lymphocytes 41, monocytes 03%, eosinophils 02%, corrected reticulocyte count 7.9%, platelets 280 × 10³/mm³. Peripheral smear showed marked anisocytosis with red blood cells (RBCs) being predominantly macrocytic and presence of polychromasia and nucleated RBCs (28 nRBC/100 WBC) few fragmented and dysmorphic RBCs were also noticed. Features of sepsis were present in the form of IT ratio of 22%, polymorphonuclear leucocytosis with some polymorphs showing cytoplasmic vacuolations and occasional toxic granules. Blood culture showed growth of coagulase-negative Staphylococcus aureus. Cerebrospinal fluid (CSF) study was normal. The baby’s blood group (type O Rh+) was the same as the mother’s. Both the direct Coombs’ test of the baby and the indirect Coombs’ test of the mother were negative. Ultrasonography of brain as well as abdomen was
case report unremarkable. 2D-echo done in view of the cardiac murmur was a normal study. The maternal peripheral blood smear with Kleihauer-Betke stain (Fig. 1) showed 13.34% fetal cells, which represented 620 ml of fetal blood loss in the maternal circulation on the basis of 55 kg of mother’s weight. This contrasted with no visible fetal cells on Kleihauer-Betke test (KBT) from a control, with the maternal RBCs appearing as ‘ghost cells’ (Fig. 2). Antibiotics were started on Day 1 in view of generalized edema and presence of features of sepsis on peripheral smear, which were continued for a period of two weeks.
Figure 1. Fetal red cells derived from leakage of the infant’s blood into the maternal circulation rich in hemoglobin F and stained darkly in the index case.
Baby was transfused with 80 ml of packed RBCs (40 ml on Day 1 and 40 ml on Day 18) with improvement in the clinical status. Baby was discharged on Day 22 with normal physical and neurological examination. DISCUSSION Neonatal anemia can be induced by fetal hemorrhage (internal, external or intraplacental), fetal hemolysis or failure of RBC production. Anemia may be compensated at birth, or be only minimal if FMH has been <50 ml. De Almeida and Bowman defined massive FMH as fetal blood loss of 80-150 ml and reported an incidence of 0.2 per 1,000 pregnancies from a large cohort.7 Most of the case reports have not explained the causes of the massive FMH. The risk factors of FMH include, antepartum fetal death, cesarean delivery, abruptio placentae, placenta previa, manual removal of the placenta, intrapartum manipulation, antepartum genital bleeding, thirdtrimester trauma and third-trimester amniocentesis.5 Bowman and Pollock concluded that the risk of FMH of 20 ml or more in third-trimester amniocentesis was about 0.7%.8 Manifestation of FMH depends on the magnitude and the acuity of blood loss. Generally, the nonstress test and ultrasound are useless for early diagnosis of FMH except in unusual cases.9 However, sinusoidal heart rate pattern and decrease in fetal movement are considered important signs of FMH.10 Prenatal ultrasound studies could sometimes identify the presence of hydrops fetalis11 or fetal growth retardation with a low biophysical score.12 In fact, fetal anemia in these cases can be diagnosed by measuring the peak systolic velocity of the middle cerebral artery (MCA PSV) of the fetus. Diagnosis of FMH can be confirmed by fetal RBC cells in the maternal blood with KBT.2 Alternatively, the fetal RBCs can be detected by flow cytometry studies. However, flow cytometry studies are not easily available and in fact, are being currently used only in 4% of laboratories in the United States.7 Estimates of the actual amount of fetal blood loss into maternal circulation from KBT can be derived based on published formulae13,14 and generally correspond to 140 ml of FMH for every 3% KBT count.
Figure 2. Kleihauer-Betke acid-elution preparation of maternal postpartum peripheral blood. The maternal RBCs from a control appear as ‘Ghost cells’. (Courtesy: Dr Bhaskar Mukerjee)
In addition to KBT, elevated serum α-fetoprotein has also been found to be a surrogate marker of FMH. Although such an association is found in a number of pregnancies associated with complications like intrauterine growth retardation, preterm delivery, late vaginal bleeding, pre-eclampsia, abruptio placentae, fetal death, placenta sonolucencies and
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case report fetal malformations, especially neural tube defects, thus leading to high false-positive rates.12 In our case, the estimated volume of fetal blood in maternal circulation was approximately 620 ml, which represented a loss of triple amount of the infant’s entire blood volume. Although a great amount of blood was lost, the patient only demonstrated pallor with mild edema, which indicated a chronic FMH with good compensation. A similar case has been earlier reported by Tsai et al.15 Neonatal deaths due to FMH are associated with shock at birth or soon after. Massive FMH that occurs on a more chronic basis and that allows fetal hemodynamic compensation usually has a good prognosis. As for massive FMH, the rapidity of the hemorrhage is probably a more important prognostic factor than the amount of fetal blood loss to the maternal circulation. Treatment of the anemic newborn following FMH depends primarily on the presence or absence of signs of circulatory failure. For those cases with chronic massive FMH, partial exchange transfusion is a better option.16 In our case, in spite of the massive blood loss, the baby was born without shock and with normal Apgar scores. Since, the baby gradually developed edema and tachypnea, he was treated with packed RBC transfusion and the condition improved. As massive FMH accounts for a significant portion of unexplained fetal deaths,12 this condition needs to be considered when there is an anemic newborn without clues of hemolytic disease, obstetric hemorrhage, decreased RBC production, or neonatal hemorrhage. A Kleihauer-Betke stain should be performed for consideration of FMH. References
3. Clayton EM Jr, Feldhaus W, Phythyon JM, Whitacre FE. Transplacental passage of fetal erythrocytes during pregnancy. Obstet Gynecol 1966;28(2):194-7. 4. Jørgensen J. Feto-maternal bleeding. During pregnancy and at delivery. Acta Obstet Gynecol Scand 1977;56(5): 487-90. 5. de Almeida V, Bowman JM. Massive fetomaternal hemorrhage: Manitoba experience. Obstet Gynecol 1994;83(3):323-8. 6. Hoag RW. Fetomaternal hemorrhage associated with umbilical vein thrombosis. Case report. Am J Obstet Gynecol 1986;154(6):1271-4. 7. Wylie BJ, D’Alton ME. Fetomaternal hemorrhage. Obstet Gynecol 2010;115(5):1039-51. 8. Bowman JM, Pollock JM. Transplacental fetal hemorrhage after amniocentesis. Obstet Gynecol 1985;66(6):749-54. 9. Tsuda H, Matsumoto M, Sutoh Y, Hidaka A, Imanaka M, Miyazaki A. Massive fetomaternal hemorrhage: a case report. Int J Obstet Gynecol 1995;50:47-9. 10. Clark SL, Miller FC. Sinusoidal fetal heart rate pattern associated with massive fetomaternal transfusion. Am J Obstet Gynecol 1984;149(1):97-9. 11. Cardwell MS. Successful treatment of hydrops fetalis caused by fetomaternal hemorrhage: a case report. Am J Obstet Gynecol 1988;158(1):131-2. 12. D’Ercole C, Boubli L, Chagnon C, Nicoloso E, Leclaire M, Cravello L, et al. Fetomaternal hemorrhage: diagnostic problems. Three case reports. Fetal Diagn Ther 1995;10(1):48-51. 13. Kleihauer E. Beihefte zum. Arch Kinderheilk 1966;53: 234-5. 14. Creasy RK, Resnik R, Iams JD (Eds.). Maternalfetal medicine. 5th edition, Saunders: Philadelphia (PA) 2004:p.541.
1. Chown B. Anaemia from bleeding of the fetus into the mother’s circulation. Lancet 1954;266(6824):1213-5.
15. Tsai YG, Hung CH, Cheng SN, Hua YM, Yuh YS. Chronic massive fetomaternal hemorrhage: a case report. Clin Neonatol 1998;5(1):35-7.
2. Kleihauer E, Braun H, Betke K. Demonstration of fetal hemoglobin in erythrocytes of a blood smear. Klin Wochenschr 1957;35(12):637-8.
16. Moya FR, Perez A, Reece EA. Severe fetomaternal hemorrhage. A report of four cases. J Reprod Med 1987;32(3):243-6.
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CASE REPORT
An Interesting Case of Meckel-Gruber Syndrome Sangeeta Arya*, K Pandey**, A Verma†, RK Singh‡, S Chauhan¶
ABSTRACT Meckel-Gruber syndrome, also known as ‘Dysencephalia splanchnocystica,’ is a rare lethal autosomal recessive disorder consisting of central nervous system malformation mainly posterior encephalocele (80%), multicystic kidneys (95%) and polydactyly (75%). Besides the classic triad of neural tube defects, polydactyly and cystic dysplasia of the kidneys, other abnormalities can occur in association with the syndrome, which may be detected sonographically include micrognathia, cardiac abnormalities, syndactyly, clinodactyly and clubbed foot. We report a case of a 26-year-old woman G3P1L1A1 with previous lower-segment cesarean section (LSCS) referred from a private practitioner with abnormal ultrasonographic findings. She was diagnosed to have Meckel-Gruber syndrome. The woman and her husband were counseled regarding this lethal condition incompatible with life and after proper consent and information, pregnancy was terminated. Keywords: Prenatal diagnosis, Meckel-Gruber syndrome, trisomy 13, encephalocele
M
eckel-Gruber syndrome, also known as ‘Dysencephalia splanchnocystica,’ is a rare lethal autosomal recessive disorder consisting of central nervous system malformation mainly posterior encephalocele (80%), multicystic kidneys (95%), and polydactyly (75%). In some cases, hepatic developmental defects also occur along with pulmonary hypoplasia due to oligohydramnios.
24 weeks, liquor seemed to be clinically reduced. We repeated her ultrasound which showed gestational age corresponding to 24 weeks (BPD 21 weeks 5 days, HC 20 weeks 4 days, AC 24 weeks 5 days, FL 25 weeks 6 days), hypotelorism present, large occipital encephalocele present, normal anatomy of cervicovertebral junction, liquor reduced, with bilateral multicystic kidneys,
Case report A 26-year-old woman G3P1L1A1 with previous lowersegment cesarean section (LSCS) two years back (indication-severe pre-eclamptic toxemia [PET] with fetal distress) was referred from a private practitioner as her USG done at 26 weeks showed single live fetus corresponding to around 26 weeks with mild oligohydramnios, amniotic fluid index (AFI) = 7, placenta post-Grade I maturity, post part of skull showing gap of around 2.2 cm with herniation of brain tissue through it. Her fundal height corresponded to *Lecturer **Head of the Dept. Dept. of Obstetrics and Gynecology †Assistant Professor Dept. of Anesthesiology ‡Assistant Professor Dept. of Surgery ¶Junior Resident Dept. of Obstetrics and Gynecology GSVM Medical College, Uttar Pradesh Address for correspondence Dr Sangeeta Arya New Type IV/8, (Near CSA Gate) GSVM Medical College Campus Kanpur, Uttar Pradesh E-mail: sangeetanilverma@gmail.com
Figure 1. Baby showing syndactyly with postaxial polydactyly.
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case report DISCUSSION
Figure 2. Baby with large encephalocele.
polydactyly could not be seen due to oligohydramnios with no cystic lesions in lungs or liver. In presence of these findings, most probable diagnosis of MeckelGruber syndrome was made. The woman and her husband were counseled regarding this lethal condition incompatible with life. They wanted termination of pregnancy. After proper consent and information, pregnancy was terminated. She delivered a dead born female baby who had large occipital encephalocele with hypotelorism with postaxial polydactyly in both hands. Lower limbs had normal number of toes. Fetal kidney biopsies were taken, which showed multiple cysts. Histopathology of placenta showed thick-walled blood vessels, large areas of fibrinoid necrosis with signs of acute chorioamnionitis. Umbilical cord showed normal, three vessels.
Besides the classic triad of neural tube defects, polydactyly, and cystic dysplasia of the kidneys, other abnormalities can occur in association with the syndrome, which may be detected sonographically include micrognathia, cardiac abnormalities, syndactyly, clinodactyly and clubbed foot.1 Recurrence risk of a neural tube defect may be between 1% and 3% in a given family, whereas recurrence of MeckelGruber syndrome is as high as 25%, due to its autosomal recessive inheritance pattern. So, in a fetus with neural tube defect, other anomalies associated with Meckel-Gruber syndrome should be carefully sought for.1,2 It is estimated that Meckel-Gruber syndrome accounts for 5% of all neural tube defects.3,4 The most likely syndrome to be confused with MeckelGruber syndrome is trisomy 13, since 30% of fetuses with trisomy 13 will have enlarged, cystic kidneys and many of them will also have polydactyly and neural tube defects.2,3 Ideally, a karyotype should be done in fetuses with this constellation of physical findings. Fetal prognosis is same whether the fetus has, trisomy 13 or Meckel-Gruber syndrome but the rate of recurrence is indeed very different, trisomy 13 is mostly a sporadic event with a lower recurrence rate than Meckel-Gruber syndrome. References 1. Nyberg DA, Hallesy D, Mahony BS, Hirsch JH, Luthy DA, Hickok D. Meckel-Gruber syndrome. Importance of prenatal diagnosis. J Ultrasound Med 1990;9(12):691-6. 2. Karjalainen O, Aula P, Seppala M, Hartikainen-Sorri AL, Ryynanen M. Prenatal diagnosis of the Meckel syndrome. Obstet Gynecol 1981;57(6 Suppl):13S-5S. 3. Pardes JG, Engel IA, Blomquist K, Magid MS, Kazam E. Ultrasonography of intrauterine Meckels syndrome. J Ultrasound Med 1984;3(1):33-5. 4. Ahdab-Barmada M, Claasen D. A distinctive triad of malformations of the central nervous system in the Meckel-Gruber Syndrome. J Neuropathol Exp Neurol 1990;49(6):610-20.
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photo quiz
A Bubble Under the Tongue of a Child
A
n eight-year-old girl presented with swelling in the floor of her mouth that began 10 days prior. She denied any recent fever or illness. Physical examination revealed a nontender, bluish, fluctuant sublingual mass with no obvious extension into the neck (see accompanying figure). No cervical adenopathy was present.
Question Based on the patientâ&#x20AC;&#x2122;s history and physical examination, which one of the following is the most likely diagnosis? A. Dermoid cyst. B. Lipoma. C. Ranula. D. Soft tissue space abscess.
SEE THE FOLLOWING PAGE FOR DISCUSSION.
Source: Adapted from Am Fam Physician. 2011;84(4):441-442.
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photo quiz Discussion The answer is C: ranula. Ranulas are mucus retention pseudocysts in the floor of the mouth. They can originate from trauma to or obstruction of the sublingual salivary gland. Less commonly, the submandibular gland or the minor salivary glands of the mouth may be involved. There are two main types of ranulas. Oral ranulas originate superior to the mylohyoid muscle. Cervical or plunging ranulas, which penetrate through the belly of the mylohyoid muscle, often produce an externally visible neck mass. Complete excision of the pseudocyst with the affected salivary gland is associated with the least likelihood of recurrence.1 Marsupialization of the cyst with packing can also be performed, and some success has been reported with placement of a single suture into the dome of the cyst.2 Watchful waiting may be an appropriate medical treatment for oral ranulas.3 Injections of onabotulinumtoxin A or sclerosing agents are considered experimental, and surgical management is usually preferred.4-6 Summary Table Condition
Characteristics
Dermoid cyst
Firm, slow-growing benign tumor containing mature skin cells; often possesses multiple epidermal appendages, such as hair follicles or sebaceous glands
Lipoma
Benign fatty tumor composed of mature fat cells, usually with a soft, rubbery consistency; slow-growing; yellow surface discoloration
Ranula
Localized collection of mucus underneath the tongue, usually arising from trauma to the sublingual salivary gland
Soft tissue space abscess
Localized collection of pus usually surrounded by inflamed tissue resulting from an infection; typically painful and may be associated with fever
Dermoid cysts are firm, slow-growing benign tumors that contain mature skin cells and are covered with a thick wall. They can contain multiple structures in the skin, including sebaceous glands, hair follicles, and other structures derived from the ectoderm. Dermoid cysts may occur in the skin, intracranially, intraspinally, paraspinally, or intra-abdominally.7 Most cysts found on the floor of the mouth occur in persons 10 to 30 years of age.7 Treatment is complete surgical excision. Lipomas are common benign fatty tumors composed of mature fat cells that can develop almost anywhere in the body. Lipomas represent about 1 to 5 percent of all neoplasms of the oral cavity.8 Superficial lipomas are slow-growing, soft, rubbery masses that may have a yellow surface discoloration. Treatment is surgical excision for symptomatic or rapidly enlarging lesions. An abscess is a localized collection of pus that usually forms in response to infection. The surrounding tissue is typically erythematous, inflamed, and painful. Fever may be present. Standard treatment is incision and drainage of the abscess. REFERENCES 1. Zhao YF, Jia J, Jia Y. Complications associated with surgical management of ranulas. J Oral Maxillofac Surg 2005;63(1):51-54. 2. Morton RP, Bartley JR. Simple sublingual ranulas: pathogenesis and management. J Otolaryngol. 1995;24(4): 253-254. 3. Pandit RT, Park AH. Management of pediatric ranula. Otolaryngol Head Neck Surg. 2002;127(1):115-118. 4. Fukase S, Ohta N, Inamura K, Aoyagi M. Treatment of ranula wth intracystic injection of the streptococcal preparation OK-432. Ann Otol Rhinol Laryngol. 2003; 112(3):214-220. 5. Roh JL, Kim HS. Primary treatment of pediatric plunging ranula with nonsurgical sclerotherapy using OK-432 (Picibanil). Int J Pediatr Otorhinolaryngol. 2008;72(9): 1405-1410. 6. Chow TL, Chan SW, Lam SH. Ranula successfully treated by botulinum toxin type A: report of 3 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2008;105(1):41-42. 7. Schwartz RA, Ruszczak Z. Dermoid cyst: overview. Medscape Reference Web site. http://emedicine. medscape.com/article/1112963-overview. Accessed September 21, 2010. 8. Fregnani ER, Pires FR, Falzoni R, Lopes MA, Vargas PA. Lipomas of the oral cavity: clinical findings, histological classification and proliferative activity of 46 cases. Int J Oral Maxillofac Surg. 2003;32(1):49-53.
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RESEARCH REVIEW
From the Journals...
Therapeutic Effects of Oral Zinc Supplementation on Acute Watery Diarrhea with Moderate Dehydration: A Double-blind Randomized Clinical Trial Background: To assess the therapeutic effects of oral zinc supplementation on acute watery diarrhea of children with moderate dehydration. Methods: All 9-month to 5-year-old children who were admitted with acute watery diarrhea and moderate dehydration to the Children Ward of Motahari Hospital, Urmia, Iran in 2008 were recruited. After the application of the inclusion and exclusion criteria, the patients were randomly allocated to two groups: One group to receive zinc plus oral rehydration solution (ORS) and the other one to receive ORS plus placebo. All the patients were rehydrated using ORS and then receiving ORS for ongoing loss (10 ml/kg after every defecation). Additionally, the patients in the intervention group received zinc syrup (1 mg/kg/day) divided into two doses. A detailed questionnaire was filled daily for each patient by trained pediatrics residents; it contained required demographic characteristics, nutrition and hydration status, and disease progression. The primary
outcome (frequency and consistency of diarrhea) and the secondary outcomes (duration of hospitalization and change in patients’ weight) were compared between the two groups. Results: The mean diarrhea frequency (4.5 ± 2.3 vs 5.3 ± 2.1; p = 0.004) was lower in the group receiving zinc + ORS; however, the average weight was relatively similar between the two groups (10.5 ± 3.1 vs 10.1 ± 2.3; p = 0.14). The qualitative assessment of stool consistency also confirmed earlier improvement in the treatment group in the first three days of hospitalization (p < 0.05). The mean duration of hospitalization was significantly lower in the patients receiving zinc supplements (2.5 ± 0.7 vs 3.3 ± 0.8 days; p = 0.001). Conclusion: Our results imply the beneficial effects of therapeutic zinc supplementation on disease duration and severity in patients with acute diarrhea and moderate dehydration in Iran. Source: Karamyyar M, Gheibi S, Noroozi M, et al. Iran J Med Sci 2013;38(2):93-9.
Zinc Supplementation for Treating Diarrhea in Children: A Systematic Review and Meta-analysis Objective: To update the available evidence about zinc use for treating diarrhea in children and to assess its effect on the malnourished population, a subgroup that has not been fully explored in previous analyses. Methods: A systematic review was performed of randomized clinical trials that assessed children upto five years old with acute diarrhea who received zinc supplementation. Controls received a placebo or oral rehydration therapy. After searching the main databases, without language restrictions, two independent reviewers selected eligible studies, extracted the data and assessed the risk of bias of included studies. Meta-analyses were calculated using mantel-haenszel or inverse variance random effects. Eighteen of 1,041 studies retrieved were included in the review (n = 7,314 children). Zinc was beneficial for reducing the duration
of diarrhea in hours (mean difference [MD] = −20.12, 95% confidence interval [CI] = −29.15 to -11.09, i² = 91%). The effect was greater in malnourished children (MD = −33.17, 95% CI = −33.55 to −27.79, i² = 0%). Diarrhea prevalence on Days 3, 5, and 7 was lower in the zinc group. The incidence of vomiting was significantly greater in the group that received zinc than in the control group. Included randomized controlled trials were of low-risk of bias in most domains assessed. Conclusions: Oral zinc supplementation significantly decreases diarrhea duration and has a greater effect on malnourished children. Zinc supplementation seems to be an appropriate public health strategy, mainly in areas of endemic deficiencies. Source: Galvao TF, Thees MF, Pontes RF, et al. Rev Panam Salud Publica 2013;33(5):370-7.
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reSEARCH review Effectiveness of Zinc Supplementation to Full-term Normal Infants: A Community-based Double-blind, Randomized, Controlled, Clinical Trial The study was aimed to test whether zinc supplementation, if initiated early, can prevent stunting and promote optimum body composition in full-term infants. For this, full-term pregnant women from low income urban community were enrolled and were followed-up for 24 months postpartum. Body mass index (BMI) was calculated from maternal weight and height that were collected one month after delivery. Infants’ weight, and length, head, chest and mid upper arm circumferences and skin fold thicknesses at triceps, biceps and subscapular area were collected at baseline (before randomization) and once in three months up till 24 months. Three hundred and twenty-four infants were randomized and allocated to zinc (163) or placebo (161) groups, respectively. Supplementation of zinc was initiated from four months of age and continued till children attained 18 months. The control (placebo) group of children received riboflavin 0.5 mg/ day, whereas the intervention (zinc) group received 5 mg zinc plus riboflavin 0.5 mg/day. When infants
were 18 months old, dietary intakes (in 78 children) were calculated by 24 hours diet recall method and hemoglobin, zinc, copper and vitamin A were quantified in blood samples collected from 70 children. The results showed prevalence of undernutrition (BMI <18.5) in 37% of the mothers. Mean ± SD calorie consumption and zinc intakes from diets in infants were 590 ± 282.8 kcal/day and 0.97 ± 0.608 mg/day, respectively. Multiple linear regression models demonstrated maternal weight as a strong predictor of infants’ weight and length at 18 months of age. As expected, diarrhea duration impacted infants’ linear growth and weight gain adversely. Zinc supplementation for a mean period of 190 days, starting from four months upto 18 months of age, in full-term normal infants, consuming an average energy of 590 kcal/day, had significant effect on the skin fold thicknesses, but not on their linear growth. Source: Radhakrishna KV, Hemalatha R, Geddam JJ, et al. PLoS One 2013;8(5):e61486.
Management of Children with Prolonged Fever of Unknown Origin and Difficulties in the Management of Fever of Unknown Origin in Children in Developing Countries This is Part II of a 2-part paper on fever of unknown origin (FUO) in children. It examines the etiology and management of prolonged FUO in children and the difficulties in the management of FUO in children in developing countries. Part I of this paper discussed acute FUO in children and was published in the March 2001 issue of Pediatric Drugs. Prolonged FUO is documented fever of more than 7-10 days, which has no apparent source and no apparent diagnosis after one week of clinical investigations. About 34% of cases of prolonged FUO are caused by infections, with bacterial meningitis and urinary tract infection accounting for about 6.5 and 11.4%, respectively, of cases attributable to infections. Chronic infections, particularly tuberculosis and ‘old’ disorders such as Kawasaki disease, cat-scratch disease and Epstein-Barr virus infection presenting with ‘new’ manifestations, collagen-vascular diseases and neoplastic disorders are the other issues of major concern in prolonged FUO. Overall, however, there is a trend towards an increased number of undiagnosed cases. This is due to advancements in diagnostic techniques, such that illnesses which were previously common among the causes of prolonged FUO are now diagnosed earlier, before the presentation becomes that of prolonged FUO. Clinical examination supplemented
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with laboratory tests to screen for serious bacterial infections should be the mainstay of initial evaluation of children with prolonged FUO. Use of scanning techniques (such as computerized tomography and ultrasound) as additional supplements to this clinical examination may allow for the earlier diagnosis of causes of prolonged FUO in children such as ‘occult’ abdominal tumors. A common error in management of children with prolonged FUO is the failure to perform a complete history and physical examination; repeated clinical examination and continued observation are of paramount importance in the diagnosis of difficult cases. Major difficulties in the management of FUO in children in developing countries include constraints in the availability and reliability of laboratory tests, cost, misuse of antibiotics and difficulties encountered in the diagnosis of malaria and typhoid fever. Malaria and typhoid fever are major etiological considerations in both acute and prolonged FUO in children in developing countries. The newer quinolones may hold great promise for the treatment of serious bacterial infections, including meningitis, which are associated with prolonged FUO in developing countries. Source: Akpede GO, Akenzua GI. Paediatr Drugs 2001;3(4):247-62.
Original Study
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Asian Journal of
Paediatric Practice Information for Authors Manuscripts should be prepared in accordance with the ‘Uniform requirements for manuscripts submitted to biomedical journals’ compiled by the International Committee of Medical Journal Editors (Ann. Intern. Med. 1992;96: 766-767). Asian Journal of Paediatric Practice strongly disapproves of the submission of the same articles simultaneously to different journals for consideration as well as duplicate publication and will decline to accept fresh manuscripts submitted by authors who have done so. The boxed checklist will help authors in preparing their manuscript according to our requirements. Improperly prepared manuscripts may be returned to the author without review. The checklist should accompany each manuscript. Authors may provide on the checklist, the names and addresses of experts from Asia and from other parts of the World who, in the authors’ opinion, are best qualified to review the paper.
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Results
– These should be concise and include only the tables and figures necessary to enhance the understanding of the text.
Discussion
– This should consist of a review of the literature and relate the major findings of the article to other publications on the subject. The particular relevance of the results to healthcare in India should be stressed, e.g. practicality and cost.
References These should conform to the Vancouver style. References should be numbered in the order in which they appear in the texts and these numbers should be inserted above the lines on each occasion the author is cited (Sinha12 confirmed other reports13, 14...). References cited only in tables or in legends to figures should be numbered in the text of the particular table or illustration. Include among the references papers accepted but not yet published; designate the journal and add ‘in press’ (in parentheses). Information from manuscripts submitted but not yet accepted should be cited in the text as ‘unpublished observations’ (in parentheses). At the end of the article the full list of references should include the names of all authors if there are fewer than seven or if there are more, the first six followed by et al., the full title of the journal article or book chapters; the title of journals abbreviated according to the style of the Index Medicus and the first and final page numbers of the article or chapter. The authors should check that the references are accurate. If they are not this may result in the rejection of an otherwise adequate contribution. Examples of common forms of references are:
Articles
Paintal AS. Impulses in vagal afferent fibres from specific pulmonary deflation receptors. The response of those receptors to phenylguanide, potato S-hydroxytryptamine and their role in respiratory and cardiovascular reflexes. Q. J. Expt. Physiol. 1955;40:89-111.
Books
Stansfield AG. Lymph Node Biopsy Interpretation Churchill Livingstone, New York 1985.
Articles in Books
Strong MS. Recurrent respiratory papillomatosis. In: Scott Brown’s Otolaryngology. Paediatric Otolaryngology Evans JNG (Ed.), Butterworths, London 1987;6:466-470.
– The legend must include enough information to permit interpretation of the figure without reference to the text.
Figures
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Please complete the following checklist and attach to the manuscript: 1. Classification (e.g. original article, review, selected summary, etc.)_______________________________ 2. Total number of pages ________________________ 3. Number of tables ____________________________ 4. Number of figures ___________________________ 5. Special requests ____________________________ 6. Suggestions for reviewers (name and postal address) Indian 1.___________ Foreign 1.____________ 2.___________ 2.____________ 3.___________ 3.____________ 4.___________ 4.____________ 7. All authors’ signatures________________________ 8. Corresponding author’s name, current postal and e-mail address and telephone and fax numbers __________________________________________
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For Editorial Correspondence: Dr KK Aggarwal Group Editor-in-Chief
Asian Journal of Paediatric Practice E- 219, Greater Kailash, Part - 1, New Delhi - 110 048, Tel: 40587513 E-mail: editorial@ijcp.com Website: www.ijcpgroup.com