Pediatric Education and Research

Tyrosenemia type 1 is a rare inherited metabolic disorder attributable to a deficiency of enzyme fumarylacetoacetate hydrolase. It has an autosomal recessive pattern of inheritance. The accumulation of tyrosine and its toxic metabolites succinyl acetone and succinyl acetoacetate in various tissues leads to the charecteristic hepatic failure, renal dysfunction and neurological crisis. Here we report 21 months old child presented with signs and symptoms of hepatic failure. This case report highlights that clinical examination has not lost its significance even in this new era of advanced extensive investigations.

Background: Convulsive status epilepticus (CSE) is the most common neurological emergency in children and often associated with poor outcome. A prospective cross sectional study was done on 193 north Indian children with CSE, aged one month to 15 years presented in pediatric emergency with primary objective was to record the time taken to control seizure and secondary objective was to find out etiology, laboratory changes, neuroimaging findings. Methods: Children (1 month to 180 months) were recruited in study as per protocol. Seizure was managed as per hospital and Indian Academy of paediatrics protocol. Data was entered in predesigned proforma and analyzed for outcomes. Results: The mean age of children was 56.7±47.1 months, where as male: female ratio was 2.3:1. Seventy four (38.3%) children belonged to the age group 61-180 months, followed by 13-60 months (34.7%) and 1-12 months (26.9%). Fever; 87 (45.1%) was most common associated symptom followed by vomiting (29.5%), headache (4.7%), and diarrhoea (3.1%). Seizure was clinically controlled (Group-I) in 149 (77.2%), partially controlled (Group-II) in 25 (12.95%), and 19 (9.84%) patients had died (Group-III). The mean duration of CSE at presentation was significantly higher in Group-II and group-III. The mean time taken to control seizure in group-I was 49.7±54.1 minutes, groupII; 135.8±77.7 minutes and in group-III; 118.21±97.5 minutes respectively. The most common CT-scan finding was meningoencephalitis 43 (22.3%), followed by tubercular meningitis 34 (17.6%), bacterial meningitis 33 (17.1%), neurocysticercosis 19 (9.8%) and cerebral infarction (5.2%). CSE was controlled with lorazepam and phenytoin in most of children. Conclusion: The duration of CSE at presentation, number of seizure episodes, time taken to control status epilepticus and etiology were the important factors for outcome of status epilepticus. 

Ninety eight (98) school going children of both sexes (56 boys and 42 girls) aged between 6 to 14 years. 5(5%) were 6 years, 7 (7.1%) were 7 years. 8 (8.1%) were 8 years, 10 (10.2%) were 9 years, 12 (12.2%) were 10 years, 11 (11.2%) were 11 year 14 (14.1%) were 12 years. 13 (13.3%) were 13 years 18 (18.3%) were 14 years. The clinical manifestation was 11(11.2%) were asthmatic with obesity 13 (13.2%), had onset of asthma more than 2 years, 11 (11.2%) had relief with bronchodilators, 9 (9.1%) had nocturnal exacerbation of asthma, 13 (13.2%) had seasonal exacerbation, 8 (8.1%) had exercise induced asthma, 12 (12.2%) had trigger induced attacks, 11 (11.2%), a febrile episode 10 (10.2%), had family history asthma / atophy. The risk factors of prevalence of bronchial asthma 3 (3.06%), was cat 5 (5.10%), was dog 10 (10.2%) both (cat and dog), 80 (81.6%), had none. 38 (38.7%) had Smoke exposure, 60 (61.2%) had Dust exposure, 19 (19.3%) used electricity for cooking, 47 (47.9%) used LPG gas for cooking, 32 (32.6%) used open? for cooking. 38 (38.7%) were staying in open atmosphere, 60 (61.2%) were staying in crowd area, 20 (20.4%) were upper, 35 (35.7%) middle, 43 (43.8%) had lower socio-economic status. The study of bronchial asthma in children with different age group and different socio-economic status will certainly help the pediatrician to evaluate the different causes, risk factors and treat efficiently to prevent morbidity of mortality

Objective: To compare the incidence of thrombocytopenia in breastfed term and preterm neonates with unconjugated hyperbilirubinemia receiving phototherapy. Material and Methods: A prospective study was done over a period of 5 months in NICU of a tertiary care hospital; 64 breastfed term and preterm (≥34 - <37 week) neonates with unconjugated hyperbilirubinemia requiring phototherapy were selected for the study after applying inclusion and exclusion criteria. Serum bilirubin and platelet count were sent on admission, after 24 hours and 48 hours of phototherapy. Phototherapy was administered according to AAP criteria, using LED phototherapy
(10 bulbs) positioned within 15 - 20 cm of the patient’s body. It was interrupted only for feeding and nursing for around 20 minutes every two to three hours. Platelet count and total serum bilirubin levels, before and after 24 and 48 hours of phototherapy were estimated. Results: After 24 to 48 hours of phototherapy, neonates showed statistically significant decrease in the platelet count; term (2.38 to 2.07 x 109/L, p = 0.0245) and preterm (2.27 to 1.96 x 109/L, p=0.0177), along with expected decrease of serum bilirubin levels. There was no association in the incidence of thrombocytopenia between the gestational age (term vs preterm, 6/32 vs 9/32 babies, p= 0.4741). Conclusion: Thrombocytopenia does occur following phototherapy in neonates. However it is clinically insignificant and asymptomatic. Gestational age of the neonate is not a factor associated with the incidence of thrombocytopenia after phototherapy in neonates with unconjugated hyperbilirubinemia.

Background: An estimated 30 per cent of the world’s population is anaemic, with the global prevalence among 6-12 years children to be 36 percent. Anaemia prevalence is higher in developing countries than developed countries. Objectives: Our aim was to estimate haemoglobin levels in tribal and non-tribal school going children [5-10 years] of Mysore and to compare the prevalence and severity of anemia between them. Study design and settings: This is a cross sectional community based study. Methods: School going children aged 5-10 years belonging to the tribal (Jenukuruba) and non-tribal belt of H.D. Kote Taluk, Mysore, were included. The sample size was estimated to be a minimum of 457 in each group. Hemoglobin estimation, clinical examination and anemia grading was done in all. Results: Out of 497 Jenukuruba tribal children, 89.5% of them were diagnosed as anemic, while only 63.6% were anemic among the Non tribal children [p<0.0001]. The mean Hemoglobin of tribal children was 8.62g/dl while that of non-tribal children was 10.94g/dl [p<0.0001]. Prevalence of anaemia was more among the girls than boys in tribal children., whereas anemia was more among boys than in girls in non-tribal children. While 22.54% of tribal-children had severe anemia, only 2.8% were severely anemic among non-tribal children. There is a correlation between the BMI and Hemoglobin. Conclusions: The prevalence of anemia in the present study subjects was higher than the national prevalence. There is need to improve the nutritional status of the children. Good training should be provided for the health workers to detect pallor and to take appropriate action.

Context: Severe acute malnutrition (SAM) is a major cause of morbidity and mortality in children under 5 years of age, especially in developing countries. Co-morbid conditions such as infections, micronutrient deficiencies and parasitic infestation are mainly responsible for this high mortality in SAM. Aims: To describe anemia and vitamin deficiency in children admitted with SAM at two tertiary level hospitals. Settings and Design: Observational cross sectional study, conducted at two centers. One centre is the Department of Pediatrics, Institute of Medical Sciences, Banaras Hindu University, and Varanasi and another is Department of Pediatrics, Shyam Shah Medical College Rewa MP. Methods and Material: Children between 6-60 months fulfilling the WHO criteria of SAM were enrolled. Cases were divided into edematous and non edematous groups. We collected data on demography, anthropometry, history and clinical examination including sign of vitamin deficiency. Investigations included CBC, general blood picture, serum electrolytes and calcium, serum albumin, blood sugar. Children were managed as per WHO SAM protocol. Statistical analysis used: The SPSS version 18 was used for data analysis. A p value <0.05 was considered significant. Results: Hundred four patients from MP group and 109 patients from UP group were finally enrolled in the study in which 98 (94.2%) patient out of 104 from MP and 77 (60%) patients out of 109 from UP less than 36 months. In this study male are more affected than female in UP group, male to female ratio is 1.79:1 as (64.2% verse 35.7%). In MP group SAM is more prevalent in lower middle class 51% as compared to upper lower class 31.7%. However in UP groups under nutrition is more common in upper lower 34.8% than lower middle 28.4%. We found out that, Vitamin B deficiency was most common (45.87%) followed by Vitamin A (16.5%) and most common deficiency sign were cheliosis, angular stomatitis and conjunctival xerosis in UP group while in MP group also most common Vitamin B deficiency (18.2%) followed Vitamin A deficiency (8.6%). Conclusions: Anemia and dermatitis are significantly coexist with edematous SAM children at both centers and vitamin deficiencies was frequently seen in edematous SAM children in UP.