Osteoporosis in multiple endocrine neoplasia type 1: severity, clinical significance, relationship to primary hyperparathyroidism, and response to parathyroidectomy.
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.
Burgess
J R
JR
Department of Diabetes, Royal Hobart Hospital, Australia. jburges@postoffice.utas.edu.au
David
R
R
Greenaway
T M
TM
Parameswaran
V
V
Shepherd
J J
JJ
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