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一名患有舌甲状腺和催乳素瘤样病变的成年患者出现颅内钙化和短指畸形,酷似奥尔布赖特遗传性骨营养不良。

Intracranial calcification and brachydactyly mimicking Albright's hereditary osteodystrophy in an adult patient with lingual thyroid and prolactinoma-like lesion.

作者信息

Tsai K S, Wu D J, Lai S M, Gong S T

机构信息

Department of Clinical Pathology, National Taiwan University Hospital, Taipei, R.O.C.

出版信息

J Formos Med Assoc. 1991 Feb;90(2):189-94.

PMID:1678415
Abstract

Hyperprolactinemia and prolactinoma in patients with long-term primary hypothyroidism have been recognized for decades. We report a case of 57-year-old female patient with lingual thyroid and cretinism who had a high serum prolactin level (greater than 200 ng/ml) and a pituitary tumor with suprasellar and parasellar extension. The tumor regressed to a size undetectable by CT scan after 2 years of thyroid hormone replacement therapy, but complete normalization of the hyperprolactinemia required additional bromocriptine therapy. This patient showed generalized short metacarpal and phalangeal bones, calcification of the basal ganglia and dentate nuclei bilaterally, and subcutaneous calcification at both gluteal regions, while serum calcium, phosphorus and c-PTH levels were all normal. Thus in addition to short stature, brachydactyly, a round face, and obesity, which are related to hypothyroidism, she also presented features uniquely mimicking the Albright's hereditary osteodystrophy seen in patients with pseudohypoparathyroidism and pseudopseudohypoparathyroidism. Since she had no family history of pseudohypoparathyroidism and had a normal level of Gs alpha protein on the membrane of the red blood cells, there is no evidence of pseudopseudohypoparathyroidism. The cause of the ectopic calcification remains unknown.

摘要

长期原发性甲状腺功能减退患者的高催乳素血症和催乳素瘤已被认识数十年。我们报告一例57岁患有舌甲状腺和克汀病的女性患者,其血清催乳素水平较高(大于200 ng/ml),并患有垂体瘤,肿瘤向鞍上和鞍旁延伸。经过2年的甲状腺激素替代治疗后,肿瘤缩小至CT扫描无法检测到的大小,但高催乳素血症的完全正常化还需要额外的溴隐亭治疗。该患者显示掌骨和指骨普遍短小,双侧基底节和齿状核钙化,双侧臀区皮下钙化,而血清钙、磷和c-PTH水平均正常。因此,除了与甲状腺功能减退相关的身材矮小、短指、圆脸和肥胖外,她还表现出独特的特征,类似于假性甲状旁腺功能减退和假假性甲状旁腺功能减退患者中所见的奥尔布赖特遗传性骨营养不良。由于她没有假性甲状旁腺功能减退的家族史,且红细胞膜上的Gsα蛋白水平正常,因此没有假假性甲状旁腺功能减退的证据。异位钙化的原因尚不清楚。

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Intracranial calcification and brachydactyly mimicking Albright's hereditary osteodystrophy in an adult patient with lingual thyroid and prolactinoma-like lesion.一名患有舌甲状腺和催乳素瘤样病变的成年患者出现颅内钙化和短指畸形,酷似奥尔布赖特遗传性骨营养不良。
J Formos Med Assoc. 1991 Feb;90(2):189-94.
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