All issues > Volume 33(4); 1990

Original Article

J Korean Pediatr Soc. 1990;33(4):437-447. Published online April 30, 1990.

Hypophosphatemic Rickets.

Kyung Mo Kim¹, Seong Hoon Ha¹, Dong Kyu Jin¹, Kwang Wook Ko¹

¹Department of Pediatrics, Seoul National University, Seoul, Korea

Received: September 29, 1989;  Accepted: September 29, 1989.

Abstract

Seventeen patients who were diagnosed as primary hypophosphatemic rickets at Department of Pediatrics, Seoul National University Children’s Hospital were analyzed to assess its clinical aspect and effect of treatment, especially on height and growth velosity. The average age of onset was 2 years, and their chief complaints were bowleg and short stature. Sex ratio was l.l:l(male: female). Familial hypophoshatemic rickets was known as X-linked domi- nant inheritance, however, majority of patients (7.4〜5.2%) in this study were sporadic cases. Their height was near or below 3rd percentile. With treatment their growth pattern was found to be pararrel to normal growth curve, and catch up growth (height gain) was not observed in majority, but early treatmnet in earlier age facilitated growth velosity and height and catch up was observed in a few patients. On an appropriate therapy with Vitamin D (8427 IU/kg) for 11 months, though phosphate level was not changed, serum alkaline phosphatase was notably decreased below 300IU/L of therapeutic level. We observed two patient whose serum alkaline phosphatase rebounded back during growth spurt at puberty when additional further increment of vitamin D was required. In all patients on appropriate treatment, x-ray findings of rickets revealed marked improvement.

Keywords :Longitudinal growth, Vitamin D effect of treatment, Hypophosphatemic rickets