Primary Hyperparathyroidism in Patients with Multiple Endocrine Neoplasia Type 1
Primary hyperparathyroidism may occur as a part of an inherited syndrome in a combination with pancreatic endocrine tumours and/or pituitary adenoma, which is classified as Multiple Endocrine Neoplasia type 1 (MEN-1). This syndrome is caused by a germline mutation in MEN-1 gene encoding a tumour-sup...
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| Format: | Article |
| Language: | English |
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Wiley
2010-01-01
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| Series: | International Journal of Endocrinology |
| Online Access: | http://dx.doi.org/10.1155/2010/928383 |
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| author | Grzegorz Piecha Jerzy Chudek Andrzej Więcek |
| author_facet | Grzegorz Piecha Jerzy Chudek Andrzej Więcek |
| author_sort | Grzegorz Piecha |
| collection | DOAJ |
| description | Primary hyperparathyroidism may occur as a part of an inherited syndrome in a combination with pancreatic endocrine tumours and/or pituitary adenoma, which is classified as Multiple Endocrine Neoplasia type 1 (MEN-1). This syndrome is caused by a germline mutation in MEN-1 gene encoding a tumour-suppressor protein, menin. Primary hyperparathyroidism is the most frequent clinical presentation of MEN-1, which usually appears in the second decade of life as an asymptomatic hypercalcemia and progresses through the next decades. The most frequent clinical presentation of MEN-1-associated primary hyperparathyroidism is bone demineralisation and recurrent kidney stones rarely followed by chronic kidney disease. The aim of this paper is to present the pathomechanism, screening procedures, diagnosis, and management of primary hyperparathyroidism in the MEN-1 syndrome. It also summarises the recent advances in the pharmacological therapy with a new group of drugs—calcimimetics. |
| format | Article |
| id | doaj-art-1e317416a2d84755a14b7bfa5837b3c4 |
| institution | Kabale University |
| issn | 1687-8337 1687-8345 |
| language | English |
| publishDate | 2010-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | International Journal of Endocrinology |
| spelling | doaj-art-1e317416a2d84755a14b7bfa5837b3c42025-02-03T05:48:17ZengWileyInternational Journal of Endocrinology1687-83371687-83452010-01-01201010.1155/2010/928383928383Primary Hyperparathyroidism in Patients with Multiple Endocrine Neoplasia Type 1Grzegorz Piecha0Jerzy Chudek1Andrzej Więcek2Department of Nephrology, Endocrinology and Metabolic Diseases, Medical University of Silesia, ul. Francuska 20/24, 40-027 Katowice, PolandDepartment of Nephrology, Endocrinology and Metabolic Diseases, Medical University of Silesia, ul. Francuska 20/24, 40-027 Katowice, PolandDepartment of Nephrology, Endocrinology and Metabolic Diseases, Medical University of Silesia, ul. Francuska 20/24, 40-027 Katowice, PolandPrimary hyperparathyroidism may occur as a part of an inherited syndrome in a combination with pancreatic endocrine tumours and/or pituitary adenoma, which is classified as Multiple Endocrine Neoplasia type 1 (MEN-1). This syndrome is caused by a germline mutation in MEN-1 gene encoding a tumour-suppressor protein, menin. Primary hyperparathyroidism is the most frequent clinical presentation of MEN-1, which usually appears in the second decade of life as an asymptomatic hypercalcemia and progresses through the next decades. The most frequent clinical presentation of MEN-1-associated primary hyperparathyroidism is bone demineralisation and recurrent kidney stones rarely followed by chronic kidney disease. The aim of this paper is to present the pathomechanism, screening procedures, diagnosis, and management of primary hyperparathyroidism in the MEN-1 syndrome. It also summarises the recent advances in the pharmacological therapy with a new group of drugs—calcimimetics.http://dx.doi.org/10.1155/2010/928383 |
| spellingShingle | Grzegorz Piecha Jerzy Chudek Andrzej Więcek Primary Hyperparathyroidism in Patients with Multiple Endocrine Neoplasia Type 1 International Journal of Endocrinology |
| title | Primary Hyperparathyroidism in Patients with Multiple Endocrine Neoplasia Type 1 |
| title_full | Primary Hyperparathyroidism in Patients with Multiple Endocrine Neoplasia Type 1 |
| title_fullStr | Primary Hyperparathyroidism in Patients with Multiple Endocrine Neoplasia Type 1 |
| title_full_unstemmed | Primary Hyperparathyroidism in Patients with Multiple Endocrine Neoplasia Type 1 |
| title_short | Primary Hyperparathyroidism in Patients with Multiple Endocrine Neoplasia Type 1 |
| title_sort | primary hyperparathyroidism in patients with multiple endocrine neoplasia type 1 |
| url | http://dx.doi.org/10.1155/2010/928383 |
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