経過中しばしば正常カルシウム血を呈した偽性副甲状腺機能低下症の一症例

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概要

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• A 38-year-old woman was diagnosed as having pseudohypoparathyroidism on the basis of hypocalcemia (7.1 mg/dl), hyperphosphatemia (4.9 mg/dl) and elevated circulating immunoreactive parathyroid hormone (0.44 ng/ml, carboxy-terminal assay). After her admission, her serum calcium level unexpectedly returned to the normal value (8.8 mg/dl), where-as the serum phosphate level was still elevated (5.3 mg/di). Although the circulating immunoreactive parathyroid hormone was elevated (0.46 ng/ml), nephrogenous cyclic AMP was rather reduced (1.02 nmol/100 mlGF), which suggested renal resistance to the endogenous parathyroid hormone. All of the vitamin D metabolites levels such as 25-hydroxyvitamin D, 24, 25-dihydroxyvitamin D and 1, 25-dihydroxyvitamin D were within the normal range.<BR>The oral administration of phosphate (3.0 g/day) easily produced a decrease in serum calcium level and tetany. Urinary phosphate and cyclic AMP excretions in response to the exogenous parathyroid hormone (Parathormone, Lilly) were higher than those in pseudohypoparathyroidism and lower than those in idiopathic hypoparathyroidism. The response of plasma cyclic AMP to Parathormone was higher than the response range of pseudohypoparathyroidisms and lower than that of idiopathic hypoparathyroidisms.<BR>Since the patient manifested hypocalcemic tetany in the outpatient clinic, oral administration of 1, 25-dihydroxyvitamin D3 (1.5 μg/day) was tried, and it resulted in the normalization of serum calcium, serum phosphate and circulating immunoreactive parathyroid hormone with no tetany.<BR>It is clear from these results that the present case had an endorgan resistance to the parathyroid hormone. However, her serum calcium level was within the normal range with the normal level of circulating 1, 25-dihydroxyvitamin D. This may be explained by the maximal stimulation of 25-hydroxyvitamin D-la-hydroxylation system with elevated endogenous parathyroid hormone. The present case may belong to the category of normocalcemic pseudohypoparathyroidism.

• 日本内分泌学会の論文

著者

• 古川 洋太郎

  東北大学医学部第二内科

• 弓田 滋

  東北大学医学部第二内科

• 海上 寛

  東北大学医学部第二内科

• 孫 孝義

  東北大学医学部第二内科

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