The Effect of Parental Consanguinity on Clinical Course and Outcome of Children with Focal Segmental Glomerulosclerosis, a Report from Isfahan, Iran

Focal segmental glomerulosclerosis (FSGS) accounts for 20% of nephrotic syndromes among children as well as 75% of the steroid resistant nephrotic syndrome (SRNS). The aim of the present study was to evaluate the influence of parental consanguinity on clinical course and outcome of FSGS in children. Methods. This historical cohort was carried out on 69 children affected by steroid resistant FSGS. Patients’ data were recorded at the initial and the final analyses and response to therapeutic measures. Subjects were also questioned about the history of parental consanguinity.

Forty-four participants (63.8%) were male with a male to female proportion of 1.76:1. Mean baseline age was 5.69 ± 2.39 (range: 1 to 10). Fifty-one patients (73.9%) reported consanguinity. A more significant resistance to cyclosporine A and cyclophosphamide was observed in participants denoting parental consanguinity than those with no kinship. The average renal survival time obtained significantly lower among those reporting consanguinity compared to the others (8.33 vs. 10.44 years, P < .05). According to univariate analysis results, parental consanguinity was a risk factor for developing chronic kidney disease (HR = 4.56, 95% CI: 1.06 to 19.47; P < .05).

Patients with FSGS plus parental consanguinity presented less renal survival time with more resistance to cures being more predisposed to the development of CKD.