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 HbA1c levels. 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.

Collapsing glomerulopathy associated with hepatitis B infection: A case report.

Collapsing glomerulopathy has been classified as a variant of focal segmental glomerulosclerosis. It is associated with infections, inflammations, and certain medications. While its association with human immunodeficiency virus has been well established its occurrence with hepatitis B has not been reported. We present here a case of collapsing glomerulopathy in a child with hepatitis B infection.

Xpert(®) MTB/RIF assay for the diagnosis of pulmonary tuberculosis in children.

SETTING: A tertiary care teaching hospital in New Delhi, India. OBJECTIVE: To determine the sensitivity and specificity of the Xpert(®) MTB/RIF assay in paediatric pulmonary tuberculosis (PTB) using MGIT™ culture as gold standard. METHODS: After ethical approval had been obtained, 50 patients aged 0-14 years with suspected PTB were enrolled. Sputum/induced sputum and gastric lavage from the participants were sent for direct smear, MGIT culture and Xpert testing. Chest X-ray and tuberculin skin test (TST) were also performed. PTB diagnosis was made without considering Xpert results according to the Revised National Tuberculosis Control Programme (RNTCP) algorithm. The sensitivity and specificity of Xpert were calculated using culture as gold standard. RESULTS: Of 50 individuals with suspected PTB, 23 (46%) were diagnosed with PTB based on the RNTCP algorithm. Sixteen children from the PTB group (69.5%) were Xpert-positive. None in the 'not PTB' group were Xpert-positive. With culture as gold standard, Xpert sensitivity and specificity were respectively 91.6% (95%CI 59.7-99.5) and 86.8% (95%CI 71.1-95.05). CONCLUSION: In almost 70% of PTB cases, a definitive diagnosis could be made within 2 h using Xpert, establishing its role as a sensitive and specific point-of-care test.

Non-response to the intensive phase of anti-tuberculosis treatment in children: evaluation and outcome [Short Communication].

The present study evaluates the causes for the persistence of symptoms and radiological signs after at least 2 months of intensive anti-tuberculosis treatment in children. In this prospective observational study, 26 paediatric patients with partial or no response to anti-tuberculosis treatment after the 2-month intensive phase were enrolled. After a detailed history and workup, it was found that 9 (34.6%) patients had a wrong initial diagnosis, while 12 (46.2%) had either received inadequate treatment or had complications requiring prolonged treatment; 5 (19.2%) failed to respond. Failure to respond to anti-tuberculosis treatment in paediatric tuberculosis seems to be over-diagnosed.

Post-infectious glomerulonephritis following infective endocarditis: Amenable to immunosuppression.

Glomerulonephritis develops in about 20% patients with infective endocarditis (IE), but is mostly asymptomatic. Heavy proteinuria or derangement of kidney functions is uncommon. We report here a child with IE and proliferative glomerulonephritis who manifested as significant proteinuria that recovered on treatment with immunosupressants.

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.

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.