Bardet-Biedl syndrome (BBS) is a rare cause of renal failure requiring renal replacement therapy. It is an autosomal recessive condition characterized by retinitis pigmentosa, postaxial polydactyly, central obesity, mental retardation, hypogonadism, and renal involvement. We report the first successful renal transplant in a case of BBS from India.

Background: In India there is scarcity of studies regarding medication non-adherence in CKD (chronic kidney disease) patients and there is lack of any prospective data. Objective: To study the prevalence and predictors of medication non-adherence in patients of CKD and outcomes of nonadherence prospectively over 2 years. Material and Methods: In this multi-centric, prospective observational study a total of 510 patients were included after obtaining consent. A validated scale of medication adherence, Morisky 8-item Medication Adherence Scale (MMAS-8) was used to calculate adherence. The primary outcome of the study was all-cause mortality and secondary outcome was composite of all-cause mortality and progression of CKD or occurrence of ESRD (end-stage renal disease). Results: Longitudinal evaluation showed increase in non-adherence over time, from 58% at baseline to 82% at two years. Overall, 18% patients were taking alternative medicines. The rate of all-cause death in low adherence population was numerically double as compared to patients with high adherence (1.4vs 0.7 per 100 patient-years), though the difference was statistically non-significant. The secondary outcome in low adherence population was 5.5 per 100 patient-years compared to 9.9 per 100 patient-years in high adherence population (hazard ratio [HR] = 1.53, 95% confidence interval [CI] = 1.21-1.74). Conclusion: Non adherence to medication is very prevalent among CKD patients in India, which further increases with duration of treatment. High use of alternative medicine was seen without physician's knowledge, especially in patients on haemodialysis. Moreover, we found that low medication adherence was significantly associated with composite of mortality and disease progression.

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