Long-term intracaval calcium infusion therapy in end-organ resistance to 1,25-dihydroxyvitamin D

摘要: Two boys aged six and four with the syndrome of hereditary resistance to 1,25-dihydroxyvitamin D3 rickets alopecia growth retardation are presented. After unsuccessful therapeutic trials pharmacologic doses vitamin D or its active metabolites, patients were treated by long-term intracaval infusions calcium through an implantable catheter. A total 0.5 0.9 g elemental was infused daily for 18 months serum concentration maintained at 9 10 mg/dl. Bone pain subsided within one week treatment. Serum phosphorus, immunoreactive parathyroid hormone, concentrations alkaline phosphatase activity normalized nine months. Radiographs knees hands revealed progressive healing complete resolution after year The gained 12 cm 8 per in height as compared 3 2 cm, respectively, previous year. transilial bone biopsy obtained from patient prior treatment severe osteomalacia associated osteitis fibrosa. follow-up examined therapy showed almost normal mineralization. These observations indicate following: (1) Long-term effective mode these patients, (2) When adequate phosphorus maintained, rate could be achieved even absence a receptor-effector system.