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Early‐onset, progressive, frequent, extensive, and severe bone mineral and renal complications in multiple endocrine neoplasia type 1–associated primary hyperparathyroidism
Author(s) -
Lourenço Delmar M,
Coutinho Flavia L,
Toledo Rodrigo A,
Montenegro Fabio LM,
CorreiaDeur Joya EM,
Toledo Sergio PA
Publication year - 2010
Publication title -
journal of bone and mineral research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.882
H-Index - 241
eISSN - 1523-4681
pISSN - 0884-0431
DOI - 10.1002/jbmr.125
Subject(s) - medicine , men1 , bone mineral , primary hyperparathyroidism , multiple endocrine neoplasia , urology , parathyroid hormone , hyperparathyroidism , osteoporosis , femoral neck , asymptomatic , tertiary hyperparathyroidism , osteopenia , gastroenterology , endocrine system , secondary hyperparathyroidism , hormone , calcium , biochemistry , chemistry , gene
Differences in bone mineral density (BMD) patterns have been recently reported between multiple endocrine neoplasia type 1–related primary hyperparathyroidism (HPT/MEN1) and sporadic primary HPT. However, studies on the early and later outcomes of bone/renal complications in HPT/MEN1 are lacking. In this cross‐sectional study, performed in a tertiary academic hospital, 36 patients cases with uncontrolled HPT from 8 unrelated MEN1 families underwent dual‐energy X‐ray absorptiometry (DXA) scanning of the proximal one‐third of the distal radius (1/3DR), femoral neck, total hip, and lumbar spine (LS). The mean age of the patients was 38.9 ± 14.5 years. Parathyroid hormone (PTH)/calcium values were mildly elevated despite an overall high percentage of bone demineralization (77.8%). In the younger group (<50 years of age), demineralization in the 1/3DR was more frequent, more severe, and occurred earlier (40%; Z ‐score −1.81 ± 0.26). The older group (>50 years of age) had a higher frequency of bone demineralization at all sites ( p < .005) and a larger number of affected bone sites ( p < .0001), and BMD was more severely compromised in the 1/3DR ( p = .007) and LS ( p = .002). BMD values were lower in symptomatic (88.9%) than in asymptomatic HPT patients ( p < .006). Patients with long‐standing HPT (>10 years) and gastrinoma/HPT presented significantly lower 1/3DR BMD values. Urolithiasis occurred earlier (<30 years) and more frequently (75%) and was associated with related renal comorbidities (50%) and renal insufficiency in the older group (33%). Bone mineral– and urolithiasis‐related renal complications in HPT/MEN1 are early‐onset, frequent, extensive, severe, and progressive. These data should be considered in the individualized clinical/surgical management of patients with MEN1‐associated HPT. © 2010 American Society for Bone and Mineral Research.