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Anaemia affects approximately 69 % of Indian children aged 6–12 months, with Fe deficiency (ID) being a common cause. The effectiveness of micronutrient-fortified infant cereal in improving Fe status and neurodevelopment was evaluated in non-anaemic and mildly anaemic Indian infants. An intervention group (IC) enrolled at age 6 months consumed 50 g/d of rice-based cereal providing 3·75 mg Fe/d as ferrous fumarate for 6 months (n80) and was compared with a matched static cross-sectional control group (CG) without intervention enrolled at age 12 months (n80). Mean Hb was higher in IC (118·1 (sd10·2) g/l)v.CG (109·5 (sd16·4) g/l) at age 12 months (adjusted mean difference: 9·7 g/l; 95 % CI 5·1, 14·3;P< 0·001), while geometric mean serum ferritin tended to be higher (27·0 (–1sd13·4, +1sd54·4)v.20·3 (–1sd7·5, +1sd55·0) ng/ml);P= 0·085) and soluble transferrin receptor was lower (1·70 (–1sd1·19, +1sd2·43)v.2·07 (–1sd1·29, +1sd3·33) mg/l;P= 0·014). Anaemia (23v.45 %;P= 0·007) and ID (17v.40 %;P= 0·003) were lower in ICv.CG. Bayley Scales of Infant and Toddler Development Third Edition scores for language (P= 0·003), motor development (P= 0·018), social-emotional (P= 0·004) and adaptive behaviour (P< 0·001), but not cognitive development (P= 0·980), were higher in ICv.CG. No significant difference in anthropometricZ-scores was observed between the groups. Consuming a micronutrient-fortified infant cereal daily for 6 months during complementary feeding promoted better Fe status while reducing the risk for anaemia and ID and was associated with superior neurodevelopmental scores.

Introduction: Lipoprotein a {Lp(a)} increases in Nephrotic Syndrome (NS). Although the majority of paediatric NS are steroid sensitive, relpase and steroid dependence are commonly seen in this cases. Lp(a) is an LDL-like lipoprotein that consists of an LDL particle to which the glycoprotein apolipoprotein(a) {apo(a)} is attached.Aim: To evaluate the potential of Lp(a), measured on admission, for the prediction of relapse/steroid dependency.Materials and Methods: Children (n=36) with first episode NS were recruited in this prospective observational case-control study and followed up for one year. They were tested at presentation for Lp(a) (mg/dL) and standard tests such as haemoglobin, albumin, protein, cholesterol, triglyceride, and urine protein. Children received standard therapy for NS, and were followed for a period of one year from diagnosis to record days to initial remission, relapse episodes, steroid dependence etc. Patients were categorised as: no relapse (NR), Infrequent Relapse (IFR), frequent relapse (FR) and Steroid Dependent (SD) as per standard definitions. Fifteen healthy volunteers were also tested for lipid profile and Lp(a) levels.Results: Of 36 cases (median age 3 years, 19 males), there were 15NR, 7IFR, 2FR and 12SD. The mean Lp(a) of the NS group (165.2±120.4 mg/dL) was higher than controls (30.52±21.9 mg/dL) (p

Background/objectives Langerhans cell histiocytosis (LCH), a rare neoplasm of hematopoietic myeloid precursor cells, is clinically characterized by spontaneously resolving lesions to a progressive life-threatening multisystem disorder. Diagnosing LCH in children is challenging as it mimics other skin disorders. This study describes the varied clinical presentation and disease course in children less than 18 years diagnosed with LCH. Methods We performed a retrospective observational study of all cases diagnosed with LCH presenting to a children's hospital in the last 26 years. Data on history, cutaneous and systemic examination, and laboratory evaluation performed, were recorded. Results A total of 126 children diagnosed with LCH were included in the study. There were 68% cases limited only to skin, and 32% children with multisystem involvement at the initial presentation. Scaly papules were the most common morphologic finding in skin. The skeletal system was the second most common organ system to be affected. Failure to thrive was a common symptom. Progression of skin to systemic involvement was seen in 27.9%. In 76.7%, skin lesions cleared over a period of 2 to 4 years. Complete remission was seen in 56.9% of children over a period of 3 to 7 years, while 8.1% children died of complicationsand 31.8% were lost to follow-up. Conclusions Long-term follow-up in this study has shown cutaneous LCH without systemic involvement has a good prognosis. Skin involvement,along with failure to thrive, was the most common clinical presentation in our study. The skeletal system was the second most common organ system involved.

Hemophagocytic lymphohistiocytosis (HLH) is an inflammatory condition that may run a rapid fatal course and calls for prompt diagnosis. Early intervention with steroids and other immunosuppressive drugs can contain the disease process and favours positive outcome. Ferritin ≥500 ng/ml is a HLH diagnostic criterion. We evaluated the diagnostic potential of admission ferritin, in children with HLH. Pediatric patients of a referral teaching hospital from Feb 2010–Oct 2013 having been investigated for ferritin on admission were included. HLH was confirmed when patients had clinical features and met 5/8 diagnostic criteria of the revised 2004 HLH guidelines. Ferritin was estimated on Cobas e411 by electrochemiluminiscence, with a measuring range of 0.5–2000 ng/ml. Dilutions were made when linearity exceeded and absolute values were reported. 905 on-admission ferritin investigations were reviewed out of which 346 values ≥500 ng/ml. Hyperferritinemia was seen in HLH/MAS (macrophage activation syndrome) [HLH group, median age 4 year 4 month, 59% male] and in systemic lupus erythematosus, sepsis, juvenile idiopathic arthritis, impending HLH, haemolytic anemias and malignancy [non-HLH group, median age 4 year 6 month, 60% male]. Of 346, 72 cases of hyperferritinemia were diagnosed with secondary HLH while one patient had primary HLH. 13/73 patients expired. The median ferritin level of the HLH group was significantly higher [6556 (2402–11,734) ng/ml] compared to non-HLH group [median 1175 (943–2000) ng/ml] (p

Drug rash, eosinophilia, and systemic symptoms (DRESS) syndrome is a severe systemic hypersensitivity reaction that usually occurs within 6 weeks of exposure to the offending drug. Diagnosis is usually straightforward in patients with pyrexia, skin rash, hepatitis, and eosinophilia with a preceding history of exposure to agents often associated with DRESS syndrome, such as aromatic anticonvulsants and sulfa drugs, but diagnosis of DRESS may still be a challenge. We report a 4-year-old child with probable DRESS syndrome complicated by multiple hematologic complications that developed 1 month after exposure to fluoxetine, a drug not known to be associated with such severe reactions.

Vici syndrome is a neurodevelopmental disorder of the autophagy pathway. Almost all cases reported have the cardinal features of agenesis of corpus callosum, cataract, cardiomyopathy, immunodeficiency and hypopigmentation.8-month-old boy with developmental delay, myoclonic jerks, repeated respiratory infections, coarse facial features, cataract and hypopigmented hair. Echocardiography revealed dilated cardiomyopathy and magnetic resonance imaging of brain suggested agenesis of corpus callosum. Exome sequencing detected a novel homozygous nonsense mutation in the EPG5 gene.Establishing a definite diagnosis helped in proper prognostication, providing genetic counseling and prenatal diagnosis to the family.Though uncommon, presence of the characteristic features makes Vici syndrome a clinically recognizable cause of developmental delay.

Evaluation of tolerability, safety and immunogenicity of a two-dose series of a quadrivalent meningococcal polysaccharide diptheria toxoid conjugate (ACYW-D) vaccine in Indian and Russian infants/toddlers. Open-label, single-arm, phase III multi-national trial. 300 children aged 9–17 months, previously unvaccinated against meningococcal disease from four sites each in India (n=200) and the Russian Federation (n=100). Two 0.5 mL doses of ACYW-D by intramuscular injection, 3–6 months apart. Meningococcal antibody titers to serogroups A, C, W-135 and Y, determined using a serum bactericidal assay in the presence of human complement before vaccination and 28 days after the second vaccination. Titers ≥1:8 against either/all of the A, C, W-135 or Y were considered seroprotective. After dose 2, 95.7–99.5% and 92.9–99.0% of infants/ toddlers achieved seroprotection across the four serogroups in India and the Russian Federation, respectively. No immediate adverse events were reported after any dose of ACYW-D. Solicited reactions were reported in 49.2% of participants, and were mainly of Grade 1 severity, and resolved within three days. Unsolicited adverse events were reported in 19.1% of infants: one event (Grade 3 diarrhea, resolving within one day) was considered related to study vaccine. No non-serious adverse events led to premature withdrawal from the study. Four serious adverse events were reported; none were considered related to study vaccine. No deaths occurred during the study. A two-dose series of ACYW-D vaccine in Indian and Russian children (9-17 month) was well-tolerated with no safety concerns, and induced robust bactericidal antibody responses against the meningococcal serogroups contained in the vaccine.

Allergic bronchopulmonary aspergillosis (ABPA) is a complex hypersensitivity reaction in patients with asthma or cystic fibrosis (CF), which is associated with bronchi colonized by the fungus Aspergillus species, most often Aspergillus fumigatus. ABPA is an important consideration for asthmatics that do not respond to asthma management or with recurrent chest infections and deteriorating lung function in children with cystic fibrosis. We present two cases of non CF bronchiectasis associated with ABPA who presented to our hospital with recurrent hospitalisations of undiagnosed aetiology.

Vi polysaccharide typhoid vaccines cannot be used in children

Purpose To assess the effect of combination probiotic Saccharomyces boulardii CNCM-I 3799 and Bacillus subtilis CU-1 in outpatient management of acute watery diarrhea in children. Methods A randomized double-blind placebo-controlled study was conducted in 180 participants aged six months to five years with acute mild to moderate diarrhea. All were enrolled from six centers across India and centrally randomized to receive S. boulardii CNCM-I 3799 and B. subtilis CU-1 or a placebo along with oral rehydration salts and zinc supplementation. Each participant was followed up for three months to assess recurrence of diarrhea. Results The mean duration of diarrhea in the probiotic and placebo groups were 54.16 hours and 59.48 hours, respectively. The difference in the duration of diarrhea in those administered with probiotic or placebo within 24 hours of diarrhea onset was 25.21 hours. Furthermore, the difference in duration of diarrhea was 13.84 hours (p

Worldwide, viral hepatitis continues to be a cause of considerable morbidity and mortality. Mass immunization with a single dose of live attenuated HAV has been shown to significantly reduce disease burden in the community. This was a phase IV, 5-year follow up study carried out at 4 centers (Kolkata, Delhi, Mumbai and Chennai) across India. The subjects with antibody titer 20 mIU/mL). The seroconversion rates considering seroprotection levels of anti-HAV antibody titer >20 mIU/mL, following vaccination starting from 6 weeks, 6 months, 12 months, 24 months, 36 months, 48 months and 60 months were 95.1%, 97.9%, 98.3%, 96.2%, 97.8%, 92.6% and 97.3%, respectively. The geometric mean concentration (GMC) over the years increased from 64.9 mIU/mL at 6 weeks to 38.1 mIU/mL and 135.2 mIU/mL at 6 months and 12 months, respectively and was maintained at 127.1 mIU/mL at 60 months. In conclusion, the result of this 5-year follow up study showed that the single dose of live attenuated vaccine is well tolerated and provides long-term immunogenicity in healthy Indian children.

Vitamin D deficiency is highly prevalent in Indian children of northern, western and southern states. Serum 25 hydroxy cholecalferol (ng/ml) was analyzed in 310 children and adolescents of pediatric hospital of Kolkata, India. Serum calcium (mg/dl), phosphorous (mg/dl) and alkaline phosphatase (IU/L) data was obtained. Median 25(OH)D was 19 ng/ml. 19.2 % of population had serum 25(OH)D 30 ng/ml (optimum). Deficiency was highest in adolescents (86.1 %), followed by school children (61.0 %), lowest in pre-school children (41.6 %). 25(OH)D concentrations was lowest in winters (P = 0.002) and spring (P = 0.03) compared to summer. There was no correlation with calcium (P = 0.99), phosphorous (P = 0.23) and ALP (P = 0.63). There is high prevalence of vitamin D deficiency in children and adolescents of eastern India. Prevalence was lower in younger subjects. 25(OH)D did not correlate with bone mineral markers.

An open-labelled, non-comparative study was conducted in 117 children aged 2–12 years to evaluate the efficacy and safety of azithromycin (20mg/ kg/day for 6 days) for the treatment of uncomplicated typhoid fever. Of the patients enrolled based on a clinical definition of typhoid fever, 109 (93.1%) completed the study. Mean (SD) of duration of fever at presentation was 9.1(4.5) days. Clinical cure was seen in 102 (93.5%) subjects, while 7 were withdrawn from the study because of clinical deterioration. Mean day of response was 3.45±1.97. BACTEC blood culture was positive for Salmonella typhi in 17/109 (15.5%) and all achieved bacteriological cure. No serious adverse event was observed. Global well being assessed by the investigator and subjects was good in 95% cases which was done at the end of the treatment. Azithromycin was found to be safe and efficacious for the management of uncomplicated typhoid fever.

Background: Dengue infection has been known to be endemic in India for centuries. In recent years, the disease has changed its course manifesting in its severe form as Dengue Hemorrhagic Fever (DHF) and Dengue Shock Syndrome (DSS), with increasing frequency of outbreaks. The objective of the present study was to study the clinical and epidemiological profile of dengue fever in children admitted in a tertiary care hospital.Methods: Clinical records of children aged 0-15 years with serologically confirmed dengue infection admitted at the Institute of Child Health, Kolkata during the Dengue outbreak (June 2016 to November 2016) were reviewed.Results: Out of 958 cases of Dengue fever majority were boys 565 (59%), with M: F ratio of 1.4:1, and among them majority (40%) were in the age group of 5-10 years. Maximum number of cases were seen in the month of September 282 (29.4%). 65.6% cases had dengue fever without warning sign, while 28.6% had dengue fever with warning sign and 5.7% had severe dengue. Among the severe dengue cases 72% were less than 5 years of age while only 28% were more than 5 years of age. Out of 958 cases we had 63 (6.6%) cases of dengue with atypical manifestations. Seizure was the most common atypical manifestation. Six children expired with a Case Fatality Rate of 0.6%. Dengue shock syndrome was the leading cause of death.Conclusions: Dengue affects all age groups, but children less than 5 years are more prone for developing severe dengue. Atypical manifestations are becoming more common in dengue fever, which can delay the diagnosis so careful observation from the clinician side is required to prevent any serious morbidity or mortality.

Seckel syndrome (Online Mendelian Inheritance in Man database Number 210600) is the classic prototype of primordial bird-headed dwarfism. In addition to the characteristic craniofacial dysmorphism and skeletal defects, abnormalities of the cardiovascular, hematopoietic, endocrine, and central nervous systems are described. The full phenotypic spectrum of this clinically and genetically heterogeneous syndrome is yet to be delineated. Presented herein is a boy 2 years and 5 months old, with Seckel syndrome, born to second-degree consanguineous Muslim parents. In addition to the classic phenotype of the disorder, this patient had both, an open and a closed lip schizencephaly detected on cranial computed tomography (CT) scan. To our knowledge, the association of schizencephaly and Seckel syndrome is not described previously in the English language literature. In addition, presented briefly is a review of the anatomical cerebral cortical malformations associated with this syndrome.

A 6-year-old boy presented with post-burn like cutaneous scars over the buttocks and the back of thighs, following skin ulceration. Cutaneous polyarteritis (CPA) was diagnosed based on the histopathological examination of the skin biopsy specimen from one of the scars. Such a presentation of childhood CPA, to our knowledge, is not documented in the English literature.

The association of viral hepatitis and acute pancreatitis is well described in the literature. Most of the cases occur in conjunction with fulminant hepatitis A and hepatitis B virus infections. The recent literature reports increasing number of cases of this complication secondary to hepatitis E virus (HEV) infection, mostly in young adults in regions endemic for the virus. Till date, to the authors' knowledge, there are 14 well-documented cases of HEV-associated acute pancreatitis in the literature. This study reports on a 7-year-old boy from India deficient in glucose 6 phosphate dehydrogenase (G6PD) with moderately severe pancreatitis, manifesting during the course of nonfulminant acute HEV infection. He developed extremely high serum bilirubin levels, probably attributed to the concomitant viral infection and his G6PD status. He recovered completely with conservative therapy. The present child is the youngest ever reported case till date with this complication secondary to HEV infection.

Tuberculosis in pregnancy though not uncommon, congenital tuberculosis continues to be a rare entity. A case of congenital tuberculosis where the mother manifested the disease 3 months after it was diagnosed in the newborn is reported considering its rarity.

Vidya Arankalle,1 Monjori Mitra,2 Sheila Bhave,3 Apurba Ghosh,2 Sundaram Balasubramanian,4 Suparna Chatterjee,5 Jaydeep Choudhury,6 Amarjeet Chitkara,7 Ganesh Kadhe,8 Amey Mane,8 Sucheta Roy81Department of Virology, National Institute of Virology, Pashan, Pune, Maharashtra, India; 2Department of Pediatrics, Institute of Child Health, Kolkata, West Bengal, India; 3Department of Pediatrics, KEM Hospital and Research Center, Pune, Maharashtra, India; 4Department of Pediatrics, Kanchi Kamkodi Child Trust Hospital (KKCTH), Nungambakkam, Chennai, Tamil Nadu, India; 5Department of Pharmacology, Institute of Post Graduate Medical Education and Research (IPGMER) and Seth Sukhlal Karnani Memorial (SSKM) Hospital, Kolkata, West Bengal, India; 6Department of Pediatrics, Sri Aurobindo Seva Kendra (EEDF Hospital), Kolkata, West Bengal, India; 7Department of Pediatrics, Sarvodaya Childcare, Pitampura, Delhi, India; 8Medical Affairs Department, Wockhardt Ltd, Mumbai, Maharashtra, IndiaAbstract: Previous studies from India have observed an increased incidence of hepatitis A virus (HAV) infection in the adult and adolescent population compared with children, indicating a shift in epidemiology of HAV. However, no HAV seroprevalence study has been conducted in India over the past decade. This prospective, multicenter, cross-sectional study was conducted in 928 children (aged 18 months to 10 years), to estimate the age-related seroprevalence of HAV across different regions of India. The present study also evaluated the impact of various factors such as age, socioeconomic class (SEC), education, source of drinking water, and excreta disposal on HAV seroprevalence. Overall, 348 (37.5%) children were seropositive for anti-HAV antibodies. Seroprevalence of HAV in the 6- to 10-year age group (50.3%) was higher (P=0.000) than in the 18-month to 6-year age group (30.3%). SEC and educational status of the parents were significantly associated with HAV seropositivity (P=0.000 for both). An increase in seroprevalence was observed with an increase in age in all SECs (P

Ectodermal dysplasia (ED) is a genetic disorder affecting the skin, hair, nails, teeth and sweat glands. The clinical presentation is heterogenous; however, hypohidrotic (reduced sweat) ectodermal dysplasia (HED) being the commonest. Also known as anhidrotic ED, sweat glands are sparse or rudimentary, leading to dysregulation of body temperature and episodes of uncontrolled hyperthermia due to reduced sweating. Of the many aids to document hypohidrosis in HED, we present here the technique of pilocarpine iontophoresis to induce, collect and measure sweat. Evaluation of sweat generated (against normally obtained values) is a non-invasive alternative to establish hypohidrosis in disorders such as HED. This augments clinical decision levels to plan skin biopsy for confirmation of diagnosis and facilitates patient management and early discharge. We present two cases of HED that were primarily diagnosed with sweat gland dysplasia using pilocarpine iontophoresis, and later confirmed with skin biopsy findings.

Pediatric cardiac tumors are rare and usually benign. An infectious etiology like tuberculosis invading myocardium and presenting as infiltrative mass is extremely rare. We present a case of a 15 month old girl with clinical feature of cardiac failure who had infiltrative multiple myocardial masses in echocardiogram. Advanced cardiac imaging by Cardiac Magnetic resonance imaging (MRI ) helped in tissue delineation. Therapeutic trial of anti-tubercular drugs in view clinical suspicion of Tuberculosis resulted in complete remission of symptom and disappearance of the cardiac mass.

Sri Lanka Journal of Child Health , 2014; 43 (4): 242-243 (Key words: Campomelic dysplasia; pathological fracture) DOI: http://dx.doi.org/10.4038/sljch.v43i4.7773

Sri Lanka Journal of Child Health , 2013; 43 (3): 180-181 (Key words: Chronic relapsing purpura fulminans; child) DOI: http://dx.doi.org/10.4038/sljch.v43i3.7384

Sri Lanka Journal of Child Health , 2014; 43 (2): 112-113 (Key words: Nasal cerebral heterotopia; nasal glioma) DOI: http://dx.doi.org/10.4038/sljch.v43i2.7017

Sri Lanka Journal of Child Health , 2014; 43 (1): 63-64 (Key words: Congenital insensitivity to pain with anhidrosis; CIPA; hereditary sensory and autonomic neuropathy type IV; pain insensitivity; anhidrosis) DOI: http://dx.doi.org/10.4038/sljch.v43i1.6668

Isolated cranial nerve paresis in childhood hepatitis A virus infection is rare. The authors report an instance of concomitant right-hand side palatal and abducens palsy, developing in the course of an otherwise uncomplicated hepatitis A virus infection in a 5-year-old girl. The neurological complications were transient, and she recovered completely with supportive therapy.

A 4-year-old male child with hepatitis A virus (HAV) infection is presented. His disease course was complicated by the development of pseudotumor cerebri (PC), as evidenced by symptoms and signs of increased intracranial pressure in the presence of normal cerebrospinal fluid examination and cranial magnetic resonance scan. The neurological examination was normal with the exception of the right-sided sixth cranial nerve paresis. His neurological course was uncomplicated with spontaneous recovery within three days. To our knowledge, this is the first report in the English literature of PC complicating the course of HAV in a child.

Sri Lankan Journal of Child Health , 2013; 42 : 224-225 (Key words: Richner Hanhart syndrome; tyrosinaemia type II; oculo-­cutaneous tyrosinaemia) DOI: http://dx.doi.org/10.4038/sljch.v42i4.6271

Sri Lanka Journal of Child Health , 2013; 42 : 226 (Key words: rhizomelic chondrodysplasia punctata) DOI: http://dx.doi.org/10.4038/sljch.v42i4.6272

Sri Lanka Journal of Child Health, 2012; 42 (3): 168-169 (Key words: Hypothyroidism; hypertrichosis; ovarian cyst; pituitary adenoma) DOI: http://dx.doi.org/10.4038/sljch.v42i3.6024

Sri Lanka Journal of Child Health , 2013; 42 (2): 97-98 (Key words: Valproate; pregnancy; teratogenicity; trigonocephaly) http://dx.doi.org/10.4038/sljch.v42i2.5632

Kawasaki disease(KD) is an acute self limiting vasculitis and probably the most common vasculitis in pediatric age group. It has become the commonest cause of acquired heart disease in west as well as in the east replacing the rheumatic fever. The diagnosis of Kawasaki disease is entirely clinical with none of the clinical features are path gnomic. It may present with many typical as well as with atypical features. Here we present a case of Kawasaki disease where a 3 years old boy presented with atypical KD with bilateral ptosis.

Sri Lanka Journal of Child Health , 2012; 41 (4): 201-202 (Keywords: Lujan Fryns syndrome; marfanoid habitus; mental retardation; attention deficit hyperactivity disorder) DOI: http://dx.doi.org/10.4038/sljch.v41i4.4987

Background: Hand, foot, and mouth disease (HFMD) is caused mostly by Coxsackievirus A16 (CA16) and enterovirus 71 (EV71). Epidemic of HFMD has occurred in India only once in Kerala in 2003. We report here a recent outbreak of HFMD in three districts of West Bengal, India. Materials and Methods: A case detection system developed with 1) three private clinics in three districts; two at Howrah and one at Hooghly, 2) Pediatrics Department of two medical colleges in Kolkata, 3) 12 practioners of these three districts with 4) a central referral center at Department of Dermatology, NRS Medical College, Kolkata where all cases from this system were confirmed by a single observer. Pediatric Dermatology unit of the Institute of Child Health, Kolkata was another independent unit. Results: A total of 38 cases of HFMD were reported till 08.10.07. Age group ranged from 12 months to 12 years (mean 40.76 months, SD 29.49). Males were slightly higher than females (M:F - 21:17). Disease was distributed mostly over buttocks, knees, hands, feet - both dorsum and palmar or the plantar surface and the oral mucosa. Highest severity noted over the buttocks and the knee. Healing time for skin lesions was 6-13 days (mean 9.13 days, SD 1.93). Oral lesions were found in 33 (86.8%) cases. Conclusion: This outbreak far away from the initial one confirmed regular outsourcing of the virus with possibilities of future epidemics. Also the fact that EV71 induced epidemic is on rise in this part of globe is alarming for India. We hope this early report will be of help for strategic planning for a better management of the disease and prevention of dreaded neurological complications in India.