Vitamin D-dependent rickets (VDDR) type 1: case series of two siblings with a CYP27B1 mutation and review of the literature.

Rachita Singh Dhull, Reena Jain, Bobbity Deepthi, Hae Ii Cheong, Abhijeet Saha, Mohit Mehndiratta, Srikanta Basu

Journal: Jornal brasileiro de nefrologia 2021;42(4):494-497

PMID: 32926064

Abstract

Two siblings presented with clinical and biochemical features of rickets, initially suspected as hypophosphatemic rickets. There was no improvement initially, hence the siblings were reinvestigated and later diagnosed as having vitamin D-dependent rickets (VDDR) type 1 due to a rare mutation in the CYP27B1 gene encoding the 1α-hydroxylase enzyme. Both siblings improved with calcitriol supplementation. The initial presentation of VDDR is often confusing and algorithmic evaluation helps in diagnosis. We also present a brief review of the literature, including genetics.

Address: Lady Hardinge Medical College and associated Kalawati Saran Children Hospital, Department of Pediatrics, Division of Pediatric Nephrology, New Delhi, India.; Seoul National University Children's Hospital, Department of Pediatrics, Seoul, Korea.; Seoul National University College of Medicine, Kidney Research Institute, Medical Research Centre, Seoul, Korea.; University College of Medical Sciences, Department of Biochemistry, New Delhi, India.
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