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与高降钙素血症相关的假性甲状旁腺功能减退症。

Pseudohypoparathyroidism associated with hypercalcitoninaemia.

作者信息

Wägar G, Lehtivuori J, Salvén I, Backman R, Sivula A

出版信息

Acta Endocrinol (Copenh). 1980 Jan;93(1):43-8. doi: 10.1530/acta.0.0930043.

Abstract

A case of a 33 year old man with type I of pseudohypoparathyroidism associated with hypercalcitoninaemia and deficiency of prolactin and growth hormone is reported. The diagnosis of pseudohypoparathyroidism was made by recognition of the classic skeletal signs of Albright's osteodystrophy and confirmed by an infusion test with parathyroid hormone, which revealed a lack of both a cyclic AMP and phospaturic response. By investigation of the endocrine status the function of the pituitary/thyroid and pituitary/testis axes as well as the adrenal cortex was observed to be normal. The basal prolactin concentration was however, lower than normal and a lack of response in the growth hormone level in two glucagon tests was observed. Furthermore the basal calcitonin level was higher than normal and by provacation with pentagastrine the calcitonin secretion was very high despite an only slightly decreased serum calcium level. By operation the thyroid gland was normal and by light microscopy of a biopsy no signs of medullary carcinoma was observed. No good explanation for the high calcitonin basal level and secretion was found.

摘要

报告了一例33岁男性,患有I型假性甲状旁腺功能减退症,伴有高降钙素血症以及催乳素和生长激素缺乏。假性甲状旁腺功能减退症的诊断是通过识别出Albright骨营养不良的典型骨骼体征,并通过甲状旁腺激素输注试验得以证实,该试验显示缺乏环磷酸腺苷和磷尿反应。通过对内分泌状态的调查,观察到垂体/甲状腺轴、垂体/睾丸轴以及肾上腺皮质的功能正常。然而,基础催乳素浓度低于正常水平,并且在两次胰高血糖素试验中观察到生长激素水平缺乏反应。此外,基础降钙素水平高于正常,尽管血清钙水平仅略有下降,但通过五肽胃泌素激发后降钙素分泌非常高。手术显示甲状腺正常,活检的光镜检查未发现髓样癌迹象。对于高降钙素基础水平和分泌,未找到合理的解释。

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