PMID- 10522858 OWN - NLM STAT- MEDLINE DCOM- 19991110 LR - 20190704 IS - 0004-0010 (Print) IS - 0004-0010 (Linking) VI - 134 IP - 10 DP - 1999 Oct TI - Osteoporosis in multiple endocrine neoplasia type 1: severity, clinical significance, relationship to primary hyperparathyroidism, and response to parathyroidectomy. PG - 1119-23 AB - 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. HYPOTHESIS: Parathyroidectomy improves bone mineral density for patients with primary hyperparathyroidism in the setting of MEN 1. DESIGN: Case series. SETTING: Tertiary referral center. PATIENTS: Twenty-nine women with MEN 1 belonging to a single family with a history of MEN 1. INTERVENTIONS: Parathyroidectomy. MAIN OUTCOME MEASURES: 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. FAU - Burgess, J R AU - Burgess JR AD - Department of Diabetes, Royal Hobart Hospital, Australia. jburges@postoffice.utas.edu.au FAU - David, R AU - David R FAU - Greenaway, T M AU - Greenaway TM FAU - Parameswaran, V AU - Parameswaran V FAU - Shepherd, J J AU - Shepherd JJ LA - eng PT - Clinical Trial PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Arch Surg JT - Archives of surgery (Chicago, Ill. : 1960) JID - 9716528 SB - IM MH - Adult MH - Aged MH - Female MH - Humans MH - Hyperparathyroidism/*etiology/*surgery MH - Middle Aged MH - Multiple Endocrine Neoplasia Type 1/*complications MH - Osteoporosis/*etiology/*prevention & control MH - *Parathyroidectomy MH - Severity of Illness Index EDAT- 1999/10/16 00:00 MHDA- 1999/10/16 00:01 CRDT- 1999/10/16 00:00 PHST- 1999/10/16 00:00 [pubmed] PHST- 1999/10/16 00:01 [medline] PHST- 1999/10/16 00:00 [entrez] AID - 10.1001/archsurg.134.10.1119 [doi] PST - ppublish SO - Arch Surg. 1999 Oct;134(10):1119-23. doi: 10.1001/archsurg.134.10.1119.