Deflazacort Versus Prednisolone: Randomized Controlled Trial in Treatment of Children With Idiopathic Nephrotic Syndrome.

BACKGROUND: Corticosteroids are the main therapy of nephrotic syndrome and goal of corticosteroid therapy is to obtain maximum clinical benefit with minimum adverse effects. Children are more vulnerable to side effects of corticosteroids related to growth and adrenal suppression, so a search for an alternative steroid with fewer side-effects is underway. Deflazacort is an oxazoline derivative and preliminary data suggest reduced osteoporosis, lesser growth retardation and weight gain with deflazacort. OBJECTIVES: This study was done to compare the effectiveness and safety of deflazacort in idiopathic nephrotic syndrome. PATIENTS AND METHODS: Twenty five children with age between 2 to 12 years, with idiopathic nephrotic syndrome were enrolled. They were randomly assigned to receive deflazacort (Group A, n = 12) or prednisolone (Group B, n = 13) and were followed up for six months. RESULTS: All children of group A and 11 of group B had remission. Two children from group B were steroid resistant. Mean time taken to induce remission was significantly (P = 0.012) less in group A (10.25 ± 2.41 days) than group B (12.55 ± 1.44 days). One patient in group A had relapse on follow up as compared to 3 in group B (P = 0.58). Statistically significant difference (P = 0.03) in change in mean height was found between group A (2.13 ± 0.50cm) and B (1.44 ± 0.45 cm), with group B gaining less height. CONCLUSIONS: Remission rate in both groups was comparable although time taken to induce remission was shorter in deflazacort group and there was a significant difference in change of mean height on follow up with prednisolone group gaining lesser height.

The acrocallosal syndrome in a neonate with further widening of phenotypic expression.

The presentation of the typical characteristics of the acrocallosal syndrome (ACLS) are hypoplasia/agenesis of corpus callosum, moderate to severe mental retardation, characteristic craniofacial abnormalities, distinctive digital malformation, and growth retardation in a neonate. An Indian neonate presented on day 1 of life (youngest in the literature to be reported) with combination of abnormalities consistent with the acrocallosal syndrome and some additional findings. The baby, born to non-consanguineous, healthy parents, presented with macrocephaly, prominent forehead, hypertelorism, polydactyly of the hands and feet, duplication of hallux, hypotonia, recurrent cyanotic episodes, rib anomalies, dextro-positioning of heart, and delayed fall of umbilical cord. As the mode of inheritance of ACLS is autosomal recessive, the risk of recurrence is 25%. Genetic counselling is of prime importance, Polydactyly, and central nervous system malformations can be detected by ultrasonography in the second trimester, but due to variability of presentation, prenatal diagnosis may not always be possible.

Comparison of 1 week versus 4 weeks of albendazole therapy in single small enhancing computed tomography lesion.

BACKGROUND: The appropriate duration of albendazole therapy in neurocysticercosis is uncertain. The observation in small uncontrolled randomized trials in children that short-course therapy (1 week) is as effective as the conventional regimen (4weeks) must be tested. OBJECTIVE: To compare the efficacy of 1 and 4 weeks of albendazole therapy in children with single small enhancing computed tomographic lesion (SSECTL). STUDY DESIGN: An open-labeled, randomized, clinical trial. MATERIALS AND METHODS: One hundred twenty children with SSECTLs presenting with seizure. INTERVENTION: The subjects were assigned to two groups using random tables: group A (n=58) received albendazole for 1 week and group B (n=62) for 4 weeks. All the subjects were followed up for 6 months. RESULTS: The proportions of subjects with complete resolution of lesion in the two groups were similar (group A 63.8% versus group B 51.6%). The proportion of subjects in the two groups in whom the lesion calcified on follow up (group A 19% versus group B 24.2%) also did not differ significantly. The incidence of seizure recurrence during the 6-month follow-up period was also similar in both the groups (group A 9.6% versus group B 3.4%, P > 0.05). CONCLUSION: One week of albendazole therapy is as effective as 4 weeks of therapy in children with SSECTLs.

Klippel-Trenaunay and Sturge-Weber overlap syndrome with phakomatosis pigmentovascularis.

Klippel-Trenaunay syndrome and Sturge-Weber syndrome are rare disorders with neurologic and cutaneous signs of vascular origin. Phakomatosis pigmentovascularis represents the association of widespread, aberrant, and persistent nevus flammeus and pigmentary abnormalities. We describe a case with features suggestive of overlap between them. A ten-month-old boy presented with seizures, developmental delay, skin lesions on face, trunk and legs, buphthalmos and right lower limb hypertrophy. CT scan of head showed atrophy of brain and calcification. Our case had overlap of Klippel-Trenaunay syndrome and Sturge-Weber syndrome with phakomatosis pigmentovascularis.