Paper Information

Journal:   JUNDISHAPUR SCIENTIFIC MEDICAL JOURNAL   SUMMER 2007 , Volume 6 , Number 2 (53); Page(s) 235 To 240.
 
Paper: 

A REPORT ON THREE CHILDREN WITH HYPOPHOSPHATEMIC RICKETS

 
 
Author(s):  AHMADZADEH A.*, AASAR SH.A.
 
* NEPHROLOGY UNIT, ABUZAR CHILDRE'S HOSPITAL
 
Abstract: 

A lack of adequate mineralization of growing bones results in rickets. Rickets may result from the deficiency of either calcium or phosphorus. The term vitamin D-resistant or refractory rickets excludes patients in whom rickets rapidly responds to moderate amount of vitamin D. Hypophosphatemic rickets (HPR) is characterized by renal phosphate wasting, hypophosphatemia and defective bone mineralization. Familial HPR is the most common form of refractory rickets. In recent years, though the overall incidence of rickets registered has a declined trend; the incidence of non-nutritional rickets has surpassed nutritional rickets. In this report three cases of vitamin D resistant rickets with advanced skeletal changes that were left undiagnosed for many years are presented. The first case was a 10.5 year-old girl, admitted owing to respiratory failure secondary to severe chest deformity. The second and third cases were 8 and 12 year-old girls with HPR who had been referred with advanced rachitic changes including severe bowlegs unresponsive to multiple doses of vitamin D. The final diagnosis was made on the clinical basis, X-ray and laboratory findings including renal phosphate wasting. These cases were confirmed by a dramatic clinical and biochemical response to therapeutic intervention which included oral phosphate, active form of vitamin D and polycitrate to correct metabolic acidosis and hypokalemia in the first case. Presently, all the patients returned to school with minimal problems. The finding of this study suggests that HPR should be considered in any child with rachitic changes particularly in those who do not respond to usual doses of vitamin D and in any older child with severe skeletal deformities.

 
Keyword(s): RICKTES, HYPOPHOSPHATEMIA, FANCONI SYNDROME
 
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