抄録
A 8-year-old girl with typical pseudohypoparathyroidism and a 38-year-old mother with pseudopseudohypoparathyroidism are presented. The girl was treated with 1α-hydroxyvitamin D3 which normalized her serum calcium levels within six days. However, the renal response observed by urinary excretion of phosphate and cyclic AMP to administration of parathyroid hormone could not be restored during the treatment.
Quantitative histological study of the iliac bone was achieved in these two patients. The iliac bone of this patient with pseudopseudohypoparathyroidism showed low turnover osteoporosis. Specimens of ectopic bone from her hand histologically indicated to have active bone formation and resorption.
The iliac bone of the case with pseudohypoparathyroidism revealed the increasing of active bone resorption and the widened osteoid seams. This data suggested that the bone of this patient seemed to have ability to respond to a high level of serum parathyroid hormone.
