Bone mineral density in children with relapsing nephrotic syndrome: A hospital-based study.

This cross-sectional analytical study was conducted from January 2012 to November 2014 in the Department of Pediatric Nephrology, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh, to evaluate the bone mineral density (BMD) values in children with relapsing nephrotic syndrome (NS). Thirty relapsing nephrotic patients were enrolled in this study. They were divided into two groups: Group I - Frequent Relapse (FR) with 21 patients and Group II - Infrequent Relapse (IFR) with nine patients. Children included were both males and females aged between four and 15 years with relapsing NS with normal renal function. Steroid-resistant NS or those with abnormal renal functions or who were on cyclosporine and calcium supplement with Vitamin D or children with secondary NS were excluded from the study. All the study population underwent dual-energy X-ray absorptiometry scan to see the BMD value. Mean age of the patients of Group I (8.43 ± 2.61 years) was lower than that of Group II (9.41 ± 2.94 years (P = 0.4043). Mean BMD Z-scores of Group I was significantly lower than that of Group II (-2.70 ± 1.28 vs. -1.30 ± 1.54, respectively; P = 0.0317). A significantly higher cumulative dose of prednisolone was administered to Group I compared with Group II (P = 0.00003). On multivariate analysis, the total dose of prednisolone (P = 0.03693), body mass index (BMI) (P = 0.00703), and age of onset of disease (P = 0.03465) had a linear relationship with dependent variable BMD Z-score. On univariate regression analysis, statistically significant inverse relationship was observed between cumulative dose of prednisolone (in grams) (P = 0.049) and BMI (P = 0.00) with BMD Z-score, but no relation was observed with duration of illness. Children with relapsing NS, especially those receiving higher doses of steroids, were at risk for low BMD.

Posterior reversible encephalopathy syndrome in a 5-year-old boy with steroid-dependent nephrotic syndrome.

Posterior reversible encephalopathy syndrome (PRES) is a rare clinical and radiological phenomenon is encountered in children compared to adults. In our center, a 5-yearold boy with steroid-dependent nephrotic syndrome (SDNS) presented with headache and blurring of vision during relapse after a long course of immunosuppressive therapy. Evaluation by computed tomography scan of the brain showed that the child had hypodense areas throughout the occipital region of the brain. All signs of PRES, except papilledema, resolved after seven days of supportive treatment evidenced by subsequent radiological evaluation. PRES should be kept in mind in any nephrotic child who is on prolonged immunosuppressive therapy.

Renal transplantation: experience in Australia.

OBJECTIVE: Outcome of renal transplantation in children under the age of 15 years, who received a renal allograft at The New Children's Hospital between January 1983 to June 1997 was studied. METHODS: Retrospective review of patients records and access to data collected from Renal Registry of the Hospital. 64 renal transplants were undertaken in 57 children during this period. Prednisone and Azathioprine were the mainstay of the immunosuppressive regimen up to 1983, then Cyclosporine A was introduced. Median age of first grafts was 10 years (range 1 month-14 years). There were 41 living related and 23 cadaveric grafts. 37 (64%) children had a congenital disorder as the cause of renal failure. Among them 14 (37%) had congenital renal dysplasia/hypoplasia, 20 (36%) had primary glomerular disorder as the cause of renal failure. RESULTS: Survival analysis at 12 month, 5 yr and 10 yr showed functioning grafts in 85%, 67% and 64% case respectively. Longest surviving transplant was 14 years. 6 children had died. Cumulative patient survival was 92.9% at 1 year, 90% at 5 year and 87% at 10 year. Sixteen primary grafts were lost with most common cause being chronic rejection accounting for 68% of all transplant lost. Recurrence of primary disease was the second most common cause of graft failure. There was one malignancy in this series. 25 of the 29 children of school age with functioning transplant attended school full time in a class appropriate for their age and nine of 13 years older patients were working full time, two worked part time. CONCLUSION: Renal transplantation is a successful treatment of end stage renal failure in children with high survival and normally functioning life. Chronic rejection remains a major cause of graft loss.