Background: Wilson disease (WD) is an inherited autosomal recessive disorder with varied manifestations. Objective: The present study was aimed to know about the types and range of clinical and laboratory features of this disease in pediatric patients as observed during a period of three years. Material and Methods: Fourteen patients suffering from Wilson disease attending the pediatric ward of this hospital during a period of three years, between July, 2009 and June, 2012, were included in the present study. The diagnosis was based on established clinical and laboratory criteria. All the cases were labeled on the basis of features, as those with hepatic, neurologic or mixed manifestations. Results: The most frequent clinical manifestations were splenomegaly, jaundice, hepatomegaly and neurological findings, mostly in the form of dystonia or gait disturbances; K-F ring was detected in all patients. Findings of the study show that though, WD presented mainly with features of liver disease in childhood, a significant number were found with neurologic manifestations. Conclusion: On the basis of results, Wilson disease should be considered in all young patients of chronic liver disease with negative viral markers. Neurologic WD, mostly with extrapyramidal manifestations occurs frequently in childhood unlike in the West.

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Objective The present study evaluated the pattern of drug use and cost of management of acute diarrhea in childhood. Method Questionnaire was designed factoring patients? demographic profile, history, disease severity, prescription regimen, nutritional advice and cost of therapy. Result A total of 102 prescriptions {70% out-patient department (OPD) and 30% (in patient department (IPD)} for acute childhood diarrhea were analyzed. Maximum children were in the age group of 6 months to 1 year (47.4%). Male were 60% and female 40%. The average number of stools per day at the time of presentation was 4. Of them, 80 % had watery diarrhea and 20 % had presence of mucus in stool. Tenesmus was reported in 25 % of cases. Clinical diagnosis was made as „Viral diarrhea? in 75% cases and as „Bacterial diarrhea? in 10 % of them. 15% were labeled as „Loose motion? pending laboratory confirmation. 60% had some dehydration and 15% had severe dehydration and were hospitalized. Nutritional advice was given to 92 % cases and 72 % were advised to continue more frequent breastfeeding on the prescription in writing. The average number of drugs per prescription was 4. Total cost of therapy per prescription was Rs 66/- for OPD and Rs 264/- for hospitalized cases. The percentage of drug prescription by generic name was 99 %. Zinc was prescribed in 98 % and oral rehydration solution (ORS) also in 98 % cases. Injectables, including intravenous fluid, were given to 30% (All IPD cases). Antibiotics were prescribed in 22% patients, the most common drug being Cefixime for OPD cases and Ceftriaxone for IPD cases by brand names. Antibiotic use correlated well with 25% invasive (Bacterial) diarrhea. There was no use of probiotics, antisecretory or antimotility agents. Most common concomitant medications prescribed were paracetamol and domperidone. Conclusion The prescribing pattern in childhood acute diarrhea by and large was found to be in conformity with the WHO guideline in the teaching hospital. However, Poly pharmacy, prescription by brand names and high average cost per prescription (Excluding the hospital related charges other than medications for IPD cases) are still matters of concern.

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Pediatric sarcoidosis is a rare multisystem disease with varied presentation mimicking a number of common chronic granulomatous diseases like tuberculosis. Herein, a case of pediatric sarcoidosis is presented with failure to thrive, profound anorexia, uveitis and multisystem involvement treated successfully with corticosteroids.

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Physiological intracranial calcification noted in 0.3-1.5% of cases, is asymptomatic, incidentally detected by neuroimaging. Pathological basal ganglia calcification is due to various causes, such as: metabolic disorders, infectious and genetic diseases. Hypoparathyroidism and pseudo-hypoparathyroidism are one of the most common causes of pathological basal ganglia calcification, adequate treatment of hypoparathyroidism may lead to marked clinical improvement, serum Ca, P and PTH is to be determined in all cases of basal ganglia calcification to rule out hypoparathyroidism.

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Crigler-Najjar syndrome type II (CN-II) is caused by a severely reduced hepatic activity of bilirubin UDP-glucuronyl transferase (UGT). Recently, by the analysis of the genetic background of CN-II patients, it has been clarified that the patients carry homozygous missense mutations or nonsense plus missense mutations on the gene for UGT, and CN-II was inherited as an autosomal recessive trait. We present an 8 month old female baby with waxing waning neonatal hyperbilirubinemia which responded well to oral phenobarbitone therapy.

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[ABSTRACT]   Full text not available  [PDF]

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Full text not available  [PDF]