Multicystic Dysplastic Kidney: A Retrospective Study.

Objective: To report the renal structural and functional anomalies in children with multicystic dysplastic kidneys. Methods: Retrospective descriptive analysis of 47 children with multicystic dysplastic kidney seen in a pediatric nephrology unit over a period of 6 years. Results: Antenatal diagnosis of multicystic dysplastic kidney was made in 34 (72.3%) patients. On follow up of 31 children for more than 12 months, 21 (68%) had involution, 4 [13%] had non-regression, and 4 (13%) were nephrectomized. Vesico-ureteric reflux (n=13; 28%) was the commonest renal abnormality. The serum creatinine values were higher (P=0.006) in children with contralateral reflux. Sub-nephrotic proteinuria was noted in 9 (29%) and was significantly associated with complete involution (P=<0.023). None of the patients developed hypertension and 2 (6.4%) had renal failure. Conclusion: Close nephrological follow-up is needed in children with multicystic dysplasia of kidneys.

Efficacy of Levamisole in Children with Frequently Relapsing and Steroid-dependent Nephrotic Syndrome.

Objectives: To assess the efficacy of levamisole in frequently relapsing nephrotic syndrome and steroid-dependent nephrotic syndrome. Study Design: Retrospective analysis of hospital case records. Setting: Pediatric nephrology department of a tertiary referral pediatric hospital. Participants: 62 children with frequently relapsing nephrotic syndrome and 35 children with steroid-dependent nephrotic syndrome. Methods: Case records of children who were diagnosed as steroid-dependant or frequently-relapsing nephrotic syndrome from June 2004 to June 2011, were reviewed. Levamisole was given daily (2 mg/kg/d) along with tapering doses of alternate day steroids after remission on daily steroids. Results: Levamisole was effective in 77.3% children with a better (80.6%) efficacy in frequently relapsing nephrotic syndrome. A total of 34 children completed 1 year follow-up post levamisole therapy. The cumulative mean (SD) steroid dose 1-year before therapy was 4109(1154) mg/m2 and 1-year post therapy was 661 (11) mg/m2 (P<0.001). The relapses were also less during the period of post-levamisole therapy. Conclusion: Levamisole is an effective alternative therapy in frequently relapsing and steroid-dependent nephrotic syndrome.

Chronic Myeloid Leukemia in a Child with IgA Nephropathy.

We report an 11 year old boy with IgA nephropathy developing chronic myeloid leukemia on follow-up. This association suggests that a B cell defect might be involved in the pathogenesis of these two conditions.

Evaluation of osteopathy in thalassemia by bone mineral densitometry and biochemical indices.

Objective To evaluate osteopathy in thalassemia by bone mineral densitometry (BMD) and biochemical indices. Methods Prospective review analysis with no follow up from 2006 to 2007 of 42 regularly transfused thalassemics aged 10–25 years (27 boys, 15 girls) was done. Anthropometry, pubertal stage and symptomatology were noted. Urinary C–terminal cross–linked telopeptide of type–1 collagen (Crosslaps) by ELISA; serum 25–OH vitamin D and osteocalcin by RIA; parathyroid hormone (PTH) and ferritin by chemiluminescence and IGF–1 by Enzyme immunoassay were evaluated. Dual Energy X-ray Absorptiometry (DEXA) of lumbar spine and femur was done on Lunar prodigy system. Data was entered and analyzed using the SPSS for Windows software. Mean comparisons were done by ANOVA 1 and data was compared using Chi–square test and p value<0.05 was taken as significant. Results Of 42 patients, 81% had osteoporosis by Z–score of DEXA. Urinary crosslaps was high in 55%; 36% had increased osteocalcin; 62% had low vitamin D levels; 38% had high parathyroid levels and IGF–1 was low in 52%. Mean serum ferritin level was 5344±2855 ng/dl. There was statistical significance (p=0.046) between chronological age and BMD. All 42 cases were divided into two groups: Group–1 (Normal DEXA), Group–2 (Abnormal DEXA) and analysis of biochemical indices between two groups showed no significant difference in any of the biochemical parameters. Conclusion This study revealed majority of thalassemics with inadequate chelation have bone resorption with advancing chronological age and BMD should be evaluated regularly for early diagnosis to prevent morbidity.

L-carnitine in Beta Thalassemia.

This study was conducted to determine L-carnitine levels in regularly transfused and chelated beta thalassemia patients (n=40; mean age, 17.5±5.0 years).Ten age matched controls were also studied. The mean L-carnitine level in thalassemic patients was 23.71±7.3μM as compared to control 29.26±2.37μM (P<0.0001). Mean Carnitine was significantly lower (P=0.037) in those with ferritin greater than 2000ng/dL (22.80±6.97μM) in comparison to those with ferritin less than 2000ng/ dL (30.1±7.77μM). Although Carnitine levels in non vegetarians was higher (26.91 ±8.4μM) than in vegetarians (22.34±6.55μM), this difference was not statistically significant (P=0.072). We conclude that L-carnitine levels were found to be lower in thalassemics as compared to age matched controls.