Early-Onset, Progressive, Frequent, Extensive, and Severe Bone Mineral and Renal Complications in Multiple Endocrine Neoplasia Type 1-Associated Primary Hyperparathyroidism

Autor(es)

Lourenco, Jr., Delmar M. ^[1] ; Coutinho, Flavia L. ^[1] ; Toledo, Rodrigo A. ^[1] ; Montenegro, Fabio L. M. ^[2] ; Correia-Deur, Joya E. M. ^[1] ; Toledo, Sergio P. A. ^[1]

Afiliação do(s) autor(es)

^[1] Univ Sao Paulo, Sch Med, Div Endocrinol, Endocrine Genet Unit LIM 25, BR-01246903 Sao Paulo - Brazil

^[2] Univ Sao Paulo, Sch Med, Hosp Clin, Div Head & Neck Surg, BR-01246903 Sao Paulo - Brazil

• Tipo de documento: Artigo Científico
• Fonte: Journal of Bone and Mineral Research; v. 25, n. 11, p. 2382-2391, NOV 2010.
• Citações Web of Science: 32

Resumo

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 389 +/- 145 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 gastnnoma/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 (C) 2010 American Society for Bone and Mineral Research (AU)

• Processo FAPESP: 09/15386-6 - Análise dos genes CDKN1A, CDKN1B, CDKN2B e CDKN2C, nas neoplasias endócrinas múltiplas tipo 1 e 2.
• Beneficiário: Rodrigo de Almeida Toledo
• Modalidade de apoio: Bolsas no Brasil - Pós-Doutorado
• Processo FAPESP: 08/58552-0 - Neoplasia endócrina múltipla tipo 1 (NEM1): análises de mutações germinativas nos genes supressores de tumor MEN1, CDKN1B/p27Kip1 e AIP; estudo de expressão gênica e proteica e; caracterização clínica
• Beneficiário: Delmar Muniz Lourenço Jr
• Modalidade de apoio: Bolsas no Brasil - Pós-Doutorado