Biochemical, bone and renal patterns in hyperparathyroidism associated with multiple endocrine neoplasia type 1

Abstract

Primary hyperparathyroidism associated with multiple endocrine neoplasia type I (hyperparathyroidism/multiple endocrine neoplasia type 1) differs in many aspects from sporadic hyperparathyroidism, which is the most frequently occurring form of hyperparathyroidism. Bone mineral density has frequently been studied in sporadic hyperparathyroidism but it has very rarely been examined in cases of hyperparathyroidism/multiple endocrine neoplasia type 1. Cortical bone mineral density in hyperparathyroidism/multiple endocrine neoplasia type 1 cases has only recently been examined, and early, severe and frequent bone mineral losses have been documented at this site. Early bone mineral losses are highly prevalent in the trabecular bone of patients with hyperparathyroidism/multiple endocrine neoplasia type 1. In summary, bone mineral disease in multiple endocrine neoplasia type 1-related hyperparathyroidism is an early, frequent and severe disturbance, occurring in both the cortical and trabecular bones. In addition, renal complications secondary to sporadic hyperparathyroidism are often studied, but very little work has been done on this issue in hyperparathyroidism/multiple endocrine neoplasia type 1. It has been recently verified that early, frequent, and severe renal lesions occur in patients with hyperparathyroidism/multiple endocrine neoplasia type 1, which may lead to increased morbidity and mortality. In this article we review the few available studies on bone mineral and renal disturbances in the setting of hyperparathyroidism/multiple endocrine neoplasia type 1. We performed a meta-analysis of the available data on bone mineral and renal disease in cases of multiple endocrine neoplasia type 1-related hyperparathyroidism.

Clinical and Genetic Analysis of Multiple Endocrine Neoplasia Type 1-Related Primary Hyperparathyroidism in Chinese

“…The clinical manifestations of MEN1-related PHPT (MHPT) differ from SHPT in many aspects, including an earlier age of onset, more common multiglandular lesions, and milder biochemical presentation in MHPT patients [3, 4]. Although change of bone mineral density (BMD) has been widely evaluated in SHPT patients, information on BMD in MHPT patients are limited.…”

Metastatic Pancreatic Gastrinoma, Severe Primary Hyperparathyroidism, And Adrenal Cushing Syndrome In A Patient With Multiple Endocrine Neoplasia Type 1: An Unusual Presentation And Review Of The Literature

“…Parathyroid tumors affect approximately 95% of all MEN1 patients (1,3,(7)(8)(9)11,12) and the fraction of PHPT patients with MEN1 is estimated to be 1 to 5%. These tumors are usually the first clinical expression of MEN1 and are often diagnosed in the second decade of life (4,13).…”

“…The prevalence of pituitary tumors in patients with MEN1 varies from 10 to 65% in different studies (3,4,8,9,12). Treatment varies according to the type of pituitary tumor (4,5) and is similar to that in patients with sporadic disease (7).…”

“…a Dual-energy X-ray Absorptiometry (DXA, LUNAR Prodigy, GE). Pancreatic islet cell tumors occur in 30 to 80% of MEN1 patients in different series (1)(2)(3)(4)6,9,12). Tumors are often multifocal (4) and benign, although some gastrinomas are malignant (1,4,12).…”

A differential diagnosis of inherited endocrine tumors and their tumor counterparts

“…5 – Multicentric tumors - A typical observation in IETs is the occurrence of multicentric tumors, as observed in MEN2-related tumors (MTC, HPT, PHEO), MEN1-related tumors (PETs, HPT), VHL-related PHEO, and CS (5,15,16). Conversely, sporadic MTC, PHEO, HPT, PETs, and PITs are usually represented by a single tumor (31,32).…”