title: Liver status in congenital, untreated, isolated GH deficiency.
abstract: Nonalcoholic fatty liver disease (NAFLD) is known to be associated with insulin resistance, atherosclerosis, and low serum IGF1 levels. We have described a large cohort of patients with isolated GH deficiency (IGHD) due to the c.57+1G→A mutation in the GHRH receptor gene. These subjects have increased insulin sensitivity (IS), delayed atherosclerosis, and normal longevity. We hypothesized that, despite visceral obesity, NAFLD would be absent or mild due to the increased IS. To assess the prevalence and severity of NAFLD in adult subjects with lifetime, congenital, untreated IGHD, we studied 22 IGHD adults and 25 controls (COs) matched for age and sex. NAFLD was assessed by a comprehensive liver function panel, and ultrasonographic pattern (hyperechogenic pattern, HP) coded as follows: 0, absent; 1, mild; 2, moderate; and 3, severe. Compared with COs, IGHD individual had lower serum IGF1 (P<0.0001), higher total cholesterol (P=0.027), lower prothrombin time (P=0.017), and similar activated partial thromboplastin time and fibrinogen values. Alanine transaminase (ALT) values were similar in the two groups, but aspartate transaminase was higher in IGHD (P=0.013). However, more IGHD subjects (7/22) than COs (3/23) had ALT above the upper limit of normal (P=0.044). The prevalence of NAFLD was higher in IGHD than COs (61 vs 29%, P=0.032), and the HP score was higher in IGHD than COs (P=0.041), but severe NAFLD was not observed in any IGHD (or CO) individual. Liver HP score is increased in lifetime, untreated, congenital IGHD, but the increase in transaminases is mild, suggesting a lack of advanced forms of NAFLD.
authors.affiliation: Division of EndocrinologyDiabetes and Metabolism, Federal University of Sergipe, Aracaju, BrazilDivision of EndocrinologyDiabetes and Metabolism, the Johns Hopkins University School of Medicine, 1830 East Monument Street Suite #333, Baltimore, Maryland 21287, USADivision of HepatologyDivision of RadiologyFederal University of Sergipe, Aracaju, Brazil.
authors.forename: Rachel D C A
authors.initials: RD
authors.lastname: Diniz
authors.affiliation: Division of EndocrinologyDiabetes and Metabolism, Federal University of Sergipe, Aracaju, BrazilDivision of EndocrinologyDiabetes and Metabolism, the Johns Hopkins University School of Medicine, 1830 East Monument Street Suite #333, Baltimore, Maryland 21287, USADivision of HepatologyDivision of RadiologyFederal University of Sergipe, Aracaju, Brazil.
authors.forename: Renata M
authors.initials: RM
authors.lastname: Souza
authors.affiliation: Division of EndocrinologyDiabetes and Metabolism, Federal University of Sergipe, Aracaju, BrazilDivision of EndocrinologyDiabetes and Metabolism, the Johns Hopkins University School of Medicine, 1830 East Monument Street Suite #333, Baltimore, Maryland 21287, USADivision of HepatologyDivision of RadiologyFederal University of Sergipe, Aracaju, Brazil salvator@jhmi.edu.
authors.forename: Roberto
authors.initials: R
authors.lastname: Salvatori
authors.affiliation: Division of EndocrinologyDiabetes and Metabolism, Federal University of Sergipe, Aracaju, BrazilDivision of EndocrinologyDiabetes and Metabolism, the Johns Hopkins University School of Medicine, 1830 East Monument Street Suite #333, Baltimore, Maryland 21287, USADivision of HepatologyDivision of RadiologyFederal University of Sergipe, Aracaju, Brazil.
authors.forename: Alex
authors.initials: A
authors.lastname: Franca
authors.affiliation: Division of EndocrinologyDiabetes and Metabolism, Federal University of Sergipe, Aracaju, BrazilDivision of EndocrinologyDiabetes and Metabolism, the Johns Hopkins University School of Medicine, 1830 East Monument Street Suite #333, Baltimore, Maryland 21287, USADivision of HepatologyDivision of RadiologyFederal University of Sergipe, Aracaju, Brazil.
authors.forename: Elenilde
authors.initials: E
authors.lastname: Gomes-Santos
authors.affiliation: Division of EndocrinologyDiabetes and Metabolism, Federal University of Sergipe, Aracaju, BrazilDivision of EndocrinologyDiabetes and Metabolism, the Johns Hopkins University School of Medicine, 1830 East Monument Street Suite #333, Baltimore, Maryland 21287, USADivision of HepatologyDivision of RadiologyFederal University of Sergipe, Aracaju, Brazil.
authors.forename: Thiago O
authors.initials: TO
authors.lastname: Ferrão
authors.affiliation: Division of EndocrinologyDiabetes and Metabolism, Federal University of Sergipe, Aracaju, BrazilDivision of EndocrinologyDiabetes and Metabolism, the Johns Hopkins University School of Medicine, 1830 East Monument Street Suite #333, Baltimore, Maryland 21287, USADivision of HepatologyDivision of RadiologyFederal University of Sergipe, Aracaju, Brazil.
authors.forename: Carla R P
authors.initials: CR
authors.lastname: Oliveira
authors.affiliation: Division of EndocrinologyDiabetes and Metabolism, Federal University of Sergipe, Aracaju, BrazilDivision of EndocrinologyDiabetes and Metabolism, the Johns Hopkins University School of Medicine, 1830 East Monument Street Suite #333, Baltimore, Maryland 21287, USADivision of HepatologyDivision of RadiologyFederal University of Sergipe, Aracaju, Brazil.
authors.forename: João A M
authors.initials: JA
authors.lastname: Santana
authors.affiliation: Division of EndocrinologyDiabetes and Metabolism, Federal University of Sergipe, Aracaju, BrazilDivision of EndocrinologyDiabetes and Metabolism, the Johns Hopkins University School of Medicine, 1830 East Monument Street Suite #333, Baltimore, Maryland 21287, USADivision of HepatologyDivision of RadiologyFederal University of Sergipe, Aracaju, Brazil.
authors.forename: Francisco A
authors.initials: FA
authors.lastname: Pereira
authors.affiliation: Division of EndocrinologyDiabetes and Metabolism, Federal University of Sergipe, Aracaju, BrazilDivision of EndocrinologyDiabetes and Metabolism, the Johns Hopkins University School of Medicine, 1830 East Monument Street Suite #333, Baltimore, Maryland 21287, USADivision of HepatologyDivision of RadiologyFederal University of Sergipe, Aracaju, Brazil.
authors.forename: Rita A A
authors.initials: RA
authors.lastname: Barbosa
authors.affiliation: Division of EndocrinologyDiabetes and Metabolism, Federal University of Sergipe, Aracaju, BrazilDivision of EndocrinologyDiabetes and Metabolism, the Johns Hopkins University School of Medicine, 1830 East Monument Street Suite #333, Baltimore, Maryland 21287, USADivision of HepatologyDivision of RadiologyFederal University of Sergipe, Aracaju, Brazil.
authors.forename: Anita H O
authors.initials: AH
authors.lastname: Souza
authors.affiliation: Division of EndocrinologyDiabetes and Metabolism, Federal University of Sergipe, Aracaju, BrazilDivision of EndocrinologyDiabetes and Metabolism, the Johns Hopkins University School of Medicine, 1830 East Monument Street Suite #333, Baltimore, Maryland 21287, USADivision of HepatologyDivision of RadiologyFederal University of Sergipe, Aracaju, Brazil.
authors.forename: Rossana M C
authors.initials: RM
authors.lastname: Pereira
authors.affiliation: Division of EndocrinologyDiabetes and Metabolism, Federal University of Sergipe, Aracaju, BrazilDivision of EndocrinologyDiabetes and Metabolism, the Johns Hopkins University School of Medicine, 1830 East Monument Street Suite #333, Baltimore, Maryland 21287, USADivision of HepatologyDivision of RadiologyFederal University of Sergipe, Aracaju, Brazil.
authors.forename: Alécia A
authors.initials: AA
authors.lastname: Oliveira-Santos
authors.affiliation: Division of EndocrinologyDiabetes and Metabolism, Federal University of Sergipe, Aracaju, BrazilDivision of EndocrinologyDiabetes and Metabolism, the Johns Hopkins University School of Medicine, 1830 East Monument Street Suite #333, Baltimore, Maryland 21287, USADivision of HepatologyDivision of RadiologyFederal University of Sergipe, Aracaju, Brazil.
authors.forename: Allysson M P
authors.initials: AM
authors.lastname: Silva
authors.affiliation: Division of EndocrinologyDiabetes and Metabolism, Federal University of Sergipe, Aracaju, BrazilDivision of EndocrinologyDiabetes and Metabolism, the Johns Hopkins University School of Medicine, 1830 East Monument Street Suite #333, Baltimore, Maryland 21287, USADivision of HepatologyDivision of RadiologyFederal University of Sergipe, Aracaju, Brazil.
authors.forename: Francisco J
authors.initials: FJ
authors.lastname: Santana-Júnior
authors.affiliation: Division of EndocrinologyDiabetes and Metabolism, Federal University of Sergipe, Aracaju, BrazilDivision of EndocrinologyDiabetes and Metabolism, the Johns Hopkins University School of Medicine, 1830 East Monument Street Suite #333, Baltimore, Maryland 21287, USADivision of HepatologyDivision of RadiologyFederal University of Sergipe, Aracaju, Brazil.
authors.forename: Eugênia H O
authors.initials: EH
authors.lastname: Valença
authors.affiliation: Division of EndocrinologyDiabetes and Metabolism, Federal University of Sergipe, Aracaju, BrazilDivision of EndocrinologyDiabetes and Metabolism, the Johns Hopkins University School of Medicine, 1830 East Monument Street Suite #333, Baltimore, Maryland 21287, USADivision of HepatologyDivision of RadiologyFederal University of Sergipe, Aracaju, Brazil.
authors.forename: Viviane C
authors.initials: VC
authors.lastname: Campos
authors.affiliation: Division of EndocrinologyDiabetes and Metabolism, Federal University of Sergipe, Aracaju, BrazilDivision of EndocrinologyDiabetes and Metabolism, the Johns Hopkins University School of Medicine, 1830 East Monument Street Suite #333, Baltimore, Maryland 21287, USADivision of HepatologyDivision of RadiologyFederal University of Sergipe, Aracaju, Brazil.
authors.forename: Manuel H
authors.initials: MH
authors.lastname: Aguiar-Oliveira
keywords: GH; IGF1; NAFLD; isolated GH deficiency