家族性原発性副甲状腺機能亢進症の1家系 [in Japanese] FAMILIAL PRIMARY HYPERPARATHYROIDISM [in Japanese]
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他の内分泌腫瘍を伴わない家族性原発性副甲状腺機能亢進症の1家系を経験したので報告する.症例1は48歳女性,乳癌術後で外来通院中高Ca血症を指摘された.副甲状腺機能亢進症と診断され手術を施行,過形成の組織学的診断であった.症例2は63歳女性,症例1の叔母である.繰り返す腎結石を契機に副甲状腺機能亢進症と診断され手術を施行,組織学的診断は2腺の腺腫であった.本症例は術後12年を経過し,現在高Ca血症が再発している.症例3は38歳女性,症例1の妹である.家族性発生を疑い家系構成員の血清Ca値を測定したところ,本症例は正常値を超えており,続いて施行した頸部超音波検査にて副甲状腺と思われる腫瘤性病変を認めた.副甲状腺機能亢進症の初期と考えられた.<br> 家族性原発性副甲状腺機能亢進症はMultiple Endocrine Neoplasia (MEN) の不全表現型と考えられ,その組織型,手術方針について文献的考察を加えた.
We experienced a family consisting of three members with familial primary hyperparathyroidism without other endocrinal tumors.<br> Case-1: A 48-year-old woman, who had been followed at our outpatient clinic after an operation for a breast cancer, was accidentally pointed out hypercalcemia. She was diagnosed as hyperparathyroidism, and was operated on. The pathological diagnosis was hyperplasia. Case-2: A 63-year-old woman, the aunt of case-1 patient, had been suffering from attack of renal stone several times. She was diagnosed as hyperparathyroidism, and was also operated on. The pathological diagnosis was two adenomas of the parathyroid glands. The patient experienced a recurrence of hypercalcemia 12 years after the operation. Case-3: The patient was a 38-year-old woman, the sister of case-1 patient. A familial disease was suspected, and serum calcium levels of the family members were measured. The value of this patient was above the normal range. Ulrasonography of the neck revealed a tumor lesion, which appeared like a parathyroid gland. The patient was diagnosed as early stage of hyperparathyroidism.<br> We conclude that familial primary hyparparathyroidism is an incomplete type of multiple endocrine neoplasia. Its pathological type and the policy of operation are discussed.
- The journal of the Japanese Practical Surgeon Society
The journal of the Japanese Practical Surgeon Society 56(7), 1325-1329, 1995-07-25
Japan Surgical Association