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Hypophosphatemic Rickets

Journal of the Korean Pediatric Society 1994;37(3):376-382.
Published online March 15, 1994.
Hypophosphatemic Rickets
Jae un, June Huh, Il Soo Ha, Hae Il Cheong, Yong Choi
Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea
저인산혈성 구루병
고재성, 허준, 하일수, 정해일, 최용
서울대학교 의과대학 소아과학교실
Abstract
The clinical data of 27 patients with hypophosphatemic rickets treated with phosphate and 1 α-hydroxyvitamin D were analysed retrospectively. The median age at diagnosis was 4 years, and the main clinical manifestations were bowleg and short stature. Among total 24 families, 5 families(21%) had X-linked dominant mode of inheritance, 1 family (4%) had autosomal dominant mode and 17 families (71%) had no family history. The serum phosphorus concentration rose from initial value of 2.7±0.13mg/dl to 3.5±0.19mg/dl. The serum alkaline phosphatase was reduced from 871±63IU/L to 393±41IU/L. Healing of rickets was demonstrated by rdiography. Patients treated for at least two years before the onset of puberty had an increase in the mean height SD score from -1.58 to - 0.79. Orthopedic surgeries for severe lower extremity deformity were performed in 11 patients, of whom 8(73%) were dignosed over 5 years of age. Complications of therapy were as follows; 12 patients (44%) developed more than one episode of hypercalciuria, 5 patients (19%) developed more than one episode of hypercalcemia, and nephrocalcinosis was noted in 5(33%) out of 15 patients by renal ultrasound. The group with nephrocalcinosis had a higher incidence of hypercalcemic episodes than the group without nephrocalcinosis. In conclusion, treatment of hypophosphatemic rickets results in healing of rickets and acceleration of growth, and we must evaluate complications of therapy such as hypercalcemia and nephrocalcinosis.
Key Words: Hypophosphatemic rickets


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