Zinc Supplementation for One Year Among Children with Cystic Fibrosis Does Not Decrease Pulmonary Infection.

BACKGROUND: Children with cystic fibrosis may have a deficiency of micronutrients, including zinc, which may affect their susceptibility to infections. There is a paucity of data on zinc supplementation among children with cystic fibrosis. We hypothesized that a pharmacologic dose of zinc administered daily for 12 months would reduce the need for antibiotics by 50%. METHODS: This double-blind randomized placebo-controlled trial was conducted among children with cystic fibrosis to assess the effect of zinc supplementation on the need for antibiotics and pulmonary function tests. The children, age 5-15 y, of either sex, received either 30-mg zinc tablets or similar looking placebo tablets daily in addition to standard care. They were followed up every month for a period of 12 months and whenever they had pulmonary exacerbations. Their serum zinc was estimated at baseline and at 12 months of enrollment. During each visit, the children underwent a pulmonary function test and sputum culture. RESULTS: Of a total of 43 children screened, 40 were enrolled, and of them, 37 completed the study. The median (interquartile range) number of days of the administration of antibiotics over 12 months of follow-up among the children receiving zinc was 42 (14-97) d. In the placebo group, it was 38 (15-70) d (P = .79). There were no significant differences in the percent-of-predicted FEV1 or change in FEV1 values at 12 months (P = .44). The number of children in whose respiratory specimens Pseudomonas was isolated was similar for the 2 groups at different time intervals. The adverse events reported were similar in the 2 groups. CONCLUSION: We did not find any significant difference in the need for antibiotics, pulmonary function tests, hospitalization, colonization with Pseudomonas, or the need for antibiotics for children with cystic fibrosis receiving zinc supplementation of 30 mg/d.

Establishing National Neonatal Perinatal Database and birth defects registry network - need of the hour!

Early detection and prevention of birth defects is necessary to further reduce neonatal morbidity and mortality. A birth defect registry or surveillance system is necessary to assess the exact magnitude, profile and modifiable risk factors for birth defects. We review the existing efforts and suggest possible options for addressing this important issue. Connecting birth defects registry with the pre-existing programs such as National Neonatal Perinatal Database could be one of the option.

Acrodermatitis enteropathica-like skin lesions in a neonate.

A male neonate was born to a sixth-gravida mother with a history of four early-neonatal deaths. On day 21 of life, the patient was admitted for poor feeding, vomiting and encephalopathy. Final diagnosis of propionic acidaemica (propionylcarnitine, 17.67 µmol/L) was made. He was managed by peritoneal dialysis followed by protein-free and special lipid diet, sodium benzoate and multivitamins. On day 28 of life, he developed acrodermatitis enteropathica-like skin lesions on perioral and diaper area that did not respond to oral zinc or antimicrobials. A possibility of acrodermatitis acidaemica was kept and supplementation with essential amino acids started, following which the skin lesions regressed.

Peripheral neuropathy in cystic fibrosis: a prevalence study.

BACKGROUND: Information on peripheral neuropathy in children with cystic fibrosis is scanty. The etiology can be multifactorial (micronutrient deficiency, chronic hypoxia, impaired glucose tolerance, immunological, vasculopathic, critical illness). METHODS: Forty five cystic fibrosis children aged 1-18 years on vitamin E supplementation for at least 6 months underwent detailed neurological examination, serum vitamin E, vitamin B12, folate, copper levels and detailed nerve conduction studies. RESULTS: The mean age of the study population was 8.35 years (±4.9 years) with 62.2% being males. Overall 22 out of 45 (48.88%,CI: 33.7-64.2) had electrophysiological evidence of peripheral neuropathy which was predominantly axonal (86.4%), sensory (50%), and polyneuropathy (95.45%). There was no significant association between status of serum micronutrients and electrophysiological evidence of peripheral neuropathy. CONCLUSION: Patients with cystic fibrosis have electrophysiological evidence of peripheral neuropathy (predominantly axonal, sensory and polyneuropathy). There is significant association of higher chronological age with occurrence of peripheral neuropathy.

Blood ammonia levels in epileptic children on 2 dose ranges of valproic acid monotherapy: a cross-sectional study.

This cross-sectional study was planned to study the blood ammonia levels in epileptic children on 2 dose ranges of valproic acid monotherapy. A total of 60 epileptic children aged 3 months to 12 years, that were on valproic acid monotherapy for at least 3 months, were enrolled and divided into 2 groups, a low-dose group (dose, 20-39 mg/kg/d [n = 32]) and a high-dose group (dose, 40-60 mg/kg/d [n=31]). Measurements of blood ammonia, serum valproic acid levels, and liver and renal function tests were performed. Blood ammonia levels were significantly higher in the high-dose group (median, 79.2 µg/dL; range, 55.23-114.29 µg/dL) as compared with the low-dose group (median, 47.2 µg/dL; range, 20.62-73.25 µg/dL). Blood ammonia levels significantly correlated with both the dose (r = .77, P < .001) and serum levels (r = .88, P < .001) of valproic acid. All the children with hyperammonemia were asymptomatic. There were no significant differences in the other biochemical parameters between the 2 groups.

Lead encephalopathy in an infant mimicking a neurometabolic disorder.

We report the case of a 7-month-old child who presented with regression of milestones, seizures, altered sensorium, and vomiting. An elder sibling had died of similar complaints. Lead encephalopathy was considered because of presence of microcytic hypochromic anemia and dense metaphyseal bands on wrist radiogram. Magnetic resonance imaging (MRI) of the brain revealed diffuse dysmyelination involving both periventricular and subcortical white matter. Such diffuse changes have not been described previously. The child's father was operating an illicit lead-acid battery manufacturing unit at home. The child was subjected to chelation therapy, which was accompanied by environmental exposure source modification. He showed significant improvement. Our case highlights the importance of taking a detailed occupational history and considering lead poisoning in the differential diagnosis of encephalopathy of unidentifiable cause.