Bone Density in Patients With Berardinelli-Seip Congenital Lipodystrophy Is Higher in Trabecular Sites and in Type 2 Patients.

Autor: Lima JG; Departamento de Medicina Clínica, Hospital Universitário Onofre Lopes (HUOL), Natal, RN, Brazil; Health Graduate Program, Centro de Ciencias da Saúde, UFRN, Natal, RN, Brazil. Electronic address: josivanlima@gmail.com., Nobrega LHC; Departamento de Medicina Clínica, Hospital Universitário Onofre Lopes (HUOL), Natal, RN, Brazil., Lima NN; Departamento de Medicina Clínica, Hospital Universitário Onofre Lopes (HUOL), Natal, RN, Brazil., Dos Santos MCF; Departamento de Medicina Clínica, Hospital Universitário Onofre Lopes (HUOL), Natal, RN, Brazil., Baracho MFP; Departamento de Medicina Clínica, Hospital Universitário Onofre Lopes (HUOL), Natal, RN, Brazil., Bandeira F; Division of Endocrinology and Diabetes, University of Pernambuco Medical School, Recife, Brazil., Capistrano L; Instituto de Medicina Tropical do Rio Grande do Norte, Natal, RN, Brazil., Freire Neto FP; Instituto de Medicina Tropical do Rio Grande do Norte, Natal, RN, Brazil., Jeronimo SMB; Instituto de Medicina Tropical do Rio Grande do Norte, Natal, RN, Brazil; Departamento de Bioquímica, Centro de Biociências, Universidade Federal do Rio Grande do Norte, Natal, RN, Brazil; Institute of Science and Technology of Tropical Diseases (INCT-DT), Natal, RN, Brazil.
Jazyk: angličtina
Zdroj: Journal of clinical densitometry : the official journal of the International Society for Clinical Densitometry [J Clin Densitom] 2018 Jan - Mar; Vol. 21 (1), pp. 61-67. Date of Electronic Publication: 2016 Nov 25.
DOI: 10.1016/j.jocd.2016.10.002
Abstrakt: Berardinelli-Seip congenital lipodystrophy (BSCL) is a rare autosomal recessive syndrome characterized by a difficulty storing lipid in adipocytes, low body fat, hypoleptinemia, and hyperinsulinemia. We report here laboratory, bone mineral density (BMD), and bone mineral content findings of 21 patients (24.1 ± 8.4 yr old, 14 females, 18 diabetics, 5.3% total body fat) with BSCL. The mean leptin was very low (0.91 ± 0.42 ng/mL), and the mean values of the Z-scores for all studied sites were positive, except for the 33% radius (Z-score -0.5 standard deviation [SD]). Twelve patients (57.1%) had a BMD Z-score higher than +2.5 SD in at least 1 site. There was no significant difference in the Z-scores between males and females. None of type 1 (AGPAT2) patients had Z-scores higher than +2.5 SD, and these patients had a smaller Z-score of BMD total body (0.26 SD vs 1.90 SD, p = 0.022) and of bone mineral content (1.59 SD vs 3.3 SD, p = 0.032) than type 2 (seipin) patients. Insulin, as well as HOMA IR (homeostasis model assessment), correlated positively with the BMD of all sites, except for the 33% radius. Z-Scores on this site (33% radius) were the smallest of all. More than half of our patients with BSCL have BMD Z-scores higher than +2.5 SD on at least 1 site, and this increase is more pronounced in the trabecular sites and in type 2 patients.
(Copyright © 2016. Published by Elsevier Inc.)
Databáze: MEDLINE
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