Congenital anomalies of kidney and urinary tract in siblings: An uncommon condition

Congenital anomalies of kidney and urinary tract (CAKUT) are important causes of chronic kidney disease (CKD) in childhood. Most do not have a definite identifiable genetic defect and occur in isolation. Rarely, familial occurrence of CAKUT has been reported. The burden of CKD to a family in a developing country is enormous, and if more than one child is afflicted with the condition, the situation is almost catastrophic. We present here two families with siblings having upper and lower urinary tract obstruction

Adrenocortical suppression in children with nephrotic syndrome treated with low-dose alternate day corticosteroids

Corticosteroids form the mainstay of therapy for all forms of nephrotic syndrome. The long-term use of this medication is associated with serious side effects including adrenocortical suppression. The primary objective of this study was to identify adrenocortical suppression (assessed by single morning serum cortisol levels) in children with nephrotic syndrome on treatment with low-dose alternate day steroids. This cross-sectional study was conducted in the Department of Pediatrics in a tertiary care hospital from January 2014 to January 2015. Seventy children (1–18 years) with nephrotic syndrome (steroid sensitive and resistant) who were in remission and on low-dose alternate day steroids for at least 8 weeks or had received steroids of 2 mg/kg/d for at least 2 weeks in the last 1 year (infrequent relapsers) were enrolled. Relevant history was taken, clinical examination was done and blood samples were drawn for serum cortisol, lipid profile, kidney function tests, fasting blood sugar, glycated hemoglobin (HbA1c), and serum albumin. Forty percent (28/70) children had adrenocortical suppression as assessed by low morning serum cortisol levels. The mean serum cortisol levels were 188 nmol/L and were significantly lower in frequently relapsing individuals (85.9 nmol/L) as compared to other types of nephrotic syndrome (P = 0.05). The prevalence of adrenocortical suppression was higher in steroid-resistant patients (57%) as compared to 28% in frequently relapsing and 11% in steroid-dependent patients. Fifty-seven percent of patients with adrenocortical suppression had short stature while 50% had obesity. All individuals had normal serum HbA1clevels. The cumulative steroid doses and total duration of corticosteroid therapy were significantly higher in patients with adrenocortical suppression. Children with nephrotic syndrome treated with low-dose alternate day steroids have a high prevalence of adrenocortical suppression on screening with single morning cortisol sample. Those with frequently relapsing or steroid-resistant diseases are at a higher risk of suppression

Megacystis, microcolon, intestinal hypoperistalsis syndrome and bilateral streak gonads

Megacystis, microcolon, intestinal hypoperistalsis is an uncommon condition presenting in neonatal age with features of intestinal obstruction and bladder evacuation abnormalities. We present here an infant girl with the diagnosis consistent with this entity