Early-onset, progressive, frequent, extensive, and severe bone mineral and renal complications in multiple endocrine neoplasia type 1–associated primary hyperparathyroidism

• DOI: 10.1002/jbmr.125
• Publication Date: 2010-05-01T14:44:02Z
• Authors: Delmar M Lourenço; Flavia L Coutinho; Rodrigo A Toledo; Fabio LM Montenegro; Joya EM Correia-Deur; Sergio PA Toledo
• Keywords: MEN1; Osteopenia

Abstract Differences in bone mineral density (BMD) patterns have been recently reported between multiple endocrine neoplasia type 1–related primary hyperparathyroidism (HPT/MEN1) and sporadic HPT. However, studies on the early later outcomes of bone/renal complications HPT/MEN1 are lacking. In this cross-sectional study, performed a tertiary academic hospital, 36 patients cases with uncontrolled HPT from 8 unrelated MEN1 families underwent dual-energy X-ray absorptiometry (DXA) scanning proximal one-third distal radius (1/3DR), femoral neck, total hip, lumbar spine (LS). The mean age was 38.9 ± 14.5 years. Parathyroid hormone (PTH)/calcium values were mildly elevated despite an overall high percentage demineralization (77.8%). younger group (<50 years age), 1/3DR more frequent, severe, occurred earlier (40%; Z-score −1.81 0.26). older (>50 age) had higher frequency at all sites (p < .005) larger number affected .0001), BMD severely compromised = .007) LS .002). lower symptomatic (88.9%) than asymptomatic .006). Patients long-standing (>10 years) gastrinoma/HPT presented significantly values. Urolithiasis (<30 frequently (75%) associated related renal comorbidities (50%) insufficiency (33%). Bone mineral– urolithiasis-related early-onset, extensive, progressive. These data should be considered individualized clinical/surgical management MEN1-associated © 2010 American Society for Mineral Research.