title: Osteoporosis in multiple endocrine neoplasia type 1: severity, clinical significance, relationship to primary hyperparathyroidism, and response to parathyroidectomy.
abstract: 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 successful subtotal parathyroidectomy. Osteoporosis is considered a complication of sporadic PHPT and an indication for parathyroidectomy. In the setting of MEN 1, however, the relationship of bone mass to PHPT, fracture risk, and parathyroidectomy is unknown.
  
  
  OBJECTIVE
  Parathyroidectomy improves bone mineral density for patients with primary hyperparathyroidism in the setting of MEN 1.
  
  
  METHODS
  Case series.
  
  
  METHODS
  Tertiary referral center.
  
  
  METHODS
  Twenty-nine women with MEN 1 belonging to a single family with a history of MEN 1.
  
  
  METHODS
  Parathyroidectomy.
  
  
  METHODS
  Bone mineral density (BMD) and history of skeletal fracture.
  
  
  RESULTS
  Osteopenia and osteoporosis were diagnosed in 41% and 45% of patients, respectively. Forty-four percent of patients with uncontrolled PHPT had severe osteopenia (T score, <-2.0) by 35 years of age. Reduction in BMD was greatest at the femoral neck. Reduced BMD was associated with an increased likelihood of skeletal fracture (P = .05). Patients with uncontrolled PHPT had lower femoral neck and lumbar spine BMDs than those in whom PHPT was controlled by parathyroidectomy (P = .005 and .02, respectively). Successful parathyroidectomy improved femoral neck and lumbar spine BMDs by a mean +/- SEM of 5.2% +/- 2.5% and 3.2% +/- 2.9%, respectively.
  
  
  CONCLUSIONS
  Osteoporosis is a frequent and early complication of PHPT in MEN 1. Despite difficulty in achieving a cure of PHPT in MEN 1, parathyroidectomy has an important role in the optimization of BMD for patients with MEN 1.
authors.affiliation: Department of Diabetes, Royal Hobart Hospital, Australia. jburges@postoffice.utas.edu.au
authors.forename: J R
authors.initials: JR
authors.lastname: Burgess
authors.forename: R
authors.initials: R
authors.lastname: David
authors.forename: T M
authors.initials: TM
authors.lastname: Greenaway
authors.forename: V
authors.initials: V
authors.lastname: Parameswaran
authors.forename: J J
authors.initials: JJ
authors.lastname: Shepherd
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D006961:Hyperparathyroidism / Q000209:etiology* / Q000601:surgery*; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000150:complications*; D010024:Osteoporosis / Q000209:etiology* / Q000517:prevention & control*; D016105:Parathyroidectomy; D012720:Severity of Illness Index