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Prolactinomas and somatotropinomas are reported to be the pituitary lesions most frequently associated with multiple endocrine neoplasia type 1 (MEN 1). However, few reports have documented the full spectrum of pituitary disease in this condition. We report herein the clinical, biochemical (PRL, alpha-subunit, insulin-like growth factor-I, cortisol, and(More)
Multiple endocrine neoplasia type 1 (MEN-1) is inherited as an autosomal dominant disease characterised by hyperplasia or neoplasia of the parathyroids, anterior pituitary and the endocrine pancreas (Wermer, 1954). Recently, both para-thyroid and pancreatic lesions in MEN-1 have been reported to show allelic loss of heterozygosity on chromosome 11 In the(More)
OBJECTIVE Little data are available on the natural history of untreated multiple endocrine neoplasia type 1 (MEN-1). These data are essential in deciding treatment that may carry significant morbidity. We determined the causes of death in a large MEN-1 kindred with data available over a period of 130 years. Most cases were unrecognized as MEN-1 at the time(More)
In 1983 a large family with MEN-1 (designated Tasman 1) was identified in Tasmania. Kindred screening and case follow-up over the subsequent 15 years has yielded data on over 160 MEN-1-affected patients. Hyperparathyroidism is present in over 60% of gene carriers by age 20 years and 95% by age 30 years. Hyperplasia is the characteristic pathological(More)
BACKGROUND The majority of reports describing the natural history and prognosis of multiple endocrine neoplasia type 1 (MEN 1) utilize phenotypic rather than molecular genetic criteria to establish a diagnosis of MEN 1. OBJECTIVES AND PATIENTS We sought to determine the spectrum of endocrine abnormality amongst 152 members (64 gene carriers and 88(More)
BACKGROUND Sporadic primary hyperparathyroidism (PHPT) occurs most frequently in postmenopausal women. Multiple endocrine neoplasia type 1 (MEN 1) is an autosomal-dominant disease in which mild to moderate PHPT develops in most gene carriers by 20 years of age. Primary hyperparathyroidism associated with MEN 1 is typically recurrent, despite initially(More)
OBJECTIVE To review the prevalence and natural history of adrenal lesions occurring in patients from a single kindred with multiple endocrine neoplasia type 1 (MEN-1). DESIGN Case series. SETTING Tertiary referral center. PATIENTS Medical records of 33 patients from the Tasman 1 MEN-1 kindred who had undergone abdominal computed tomographic (CT)(More)