Familial Hyperparathyroidism

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Familial hyperparathyroidism.

Familial hyperparathyroidism (HPT) is a hereditary disease in which HPT is transmitted in an autosomal dominant fashion. It includes a variety of diseases: multiple endocrine neoplasia (MEN) type 1 and type 2, and familial isolated hyperparathyroidism (FIHPT). We screened for MEN 1 mutations by direct nucleotide sequencing of all protein-coding regions and identified the germline mutations of t...

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The investigation of familial isolated hyperparathyroidism (FIHP) has been greatly facilitated in recent years by the identification of the genes responsible for most cases of syndromic familial hyperparathyroidism (HPT). Kindreds with apparently isolated hyperparathyroidism have been evaluated with clinical, biochemical, imaging and gene mutational tests designed to recognize multiple endocrin...

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Parathyroid carcinoma in familial hyperparathyroidism.

Two families with hereditary hyperparathyroidism are described. One member of each family developed a parathyroid carcinoma. In one case this recurred locally and metastasised. This patient showed hyperplasia of one of the three other parathyroid glands. It is possible that the different parathyroid lesions found in familial hyperparathyroidism may be the result of a progression from hyperplasi...

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Endocrine neoplasms in familial syndromes of hyperparathyroidism.

Familial syndromes of hyperparathyroidism, including multiple endocrine neoplasia type 1 (MEN1), multiple endocrine neoplasia type 2A (MEN2A), and the hyperparathyroidism-jaw tumor (HPT-JT), comprise 2-5% of primary hyperparathyroidism cases. Familial syndromes of hyperparathyroidism are also associated with a range of endocrine and nonendocrine tumors, including potential malignancies. Complic...

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Clinical, genetic, and histopathologic investigation of CDC73-related familial hyperparathyroidism.

CDC73 (HRPT2) germline mutations are responsible for more than half of cases of hyperparathyroidism-jaw tumor syndrome (HPT-JT) and for a subset of familial isolated HPT (FIHP). We performed a clinical, genetic, and histopathologic study in three unrelated Italian kindreds with HPT-JT and FIHP. We identified three germline inactivating mutations of the CDC73 gene in the probands and affected pa...

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ژورنال

عنوان ژورنال: Proceedings of the Royal Society of Medicine

سال: 1971

ISSN: 0035-9157

DOI: 10.1177/003591577106401028