title: Ghrelin, leptin and adiponectin levels in Gaucher disease type I patients on enzyme replacement therapy.
abstract: BACKGROUND
  Gaucher disease type I (GD type I) is characterized by clinical heterogeneity and is associated with metabolic abnormalities such as increased basal metabolic rate.
  
  
  OBJECTIVE
  To evaluate ghrelin, leptin and adiponectin levels in patients with GD type I on enzyme replacement therapy (ERT).
  
  
  METHODS
  A cross-sectional study of patients with GD type I (n = 15), matched for sex, age and BMI with healthy controls. The levels of glucose, insulin, ghrelin, leptin and adiponectin were assessed in both groups. Insulin resistance was defined by the index HOMA-IR.
  
  
  RESULTS
  Eight patients had adequate weight, seven were overweight (4 preobese, 3 obese class I). Eight patients presented metabolic syndrome, five of whom with insulin resistance. The median levels of ghrelin, leptin and adiponectin of the patients did not differ from those of the controls. Ghrelin and adiponectin levels were correlated with each other; inversely correlated with BMI, waist circumference and triglyceride levels; and directly correlated with HDL-cholesterol. Leptin levels were inversely correlated with LDL-cholesterol and directly correlated with BMI, waist circumference, enzyme dosage, triglycerides, insulin, and HOMA-IR.
  
  
  CONCLUSIONS
  Metabolic syndrome and overweight appear to be common in patients with GD type I on ERT. As leptin was strongly associated with insulin and HOMA index, it could become a biomarker to assess early evidence of insulin resistance in patients with GD. Further studies are needed to investigate the associations found.
authors.affiliation: LTD Laboratory, Nutrition Program, Medical School, Universidade Federal do Rio Grande do Sul, Brazil.
authors.forename: Divair
authors.initials: D
authors.lastname: Doneda
authors.affiliation: School of Physical Education, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil; Physical Education Program, University Center UNIVATES, Lageado, RS, Brazil.
authors.forename: André L
authors.initials: AL
authors.lastname: Lopes
authors.affiliation: School of Physical Education, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil.
authors.forename: Bruno C
authors.initials: BC
authors.lastname: Teixeira
authors.affiliation: Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil.
authors.forename: Suzana D
authors.initials: SD
authors.lastname: Mittelstadt
authors.affiliation: Centre for Studies in Food and Nutrition (CESAN), HCPA/UFRGS, Brazil; Department of Internal Medicine, School of Medicine, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil.
authors.forename: Cileide C
authors.initials: CC
authors.lastname: Moulin
authors.affiliation: Post-Graduate Program in Medicine, Medical Sciences, Universidade Federal do Rio Grande do Sul, Brazil; Laboratory B.R.A.I.N, HCPA/UFRGS, Brazil; Genetics Department, Universidade Federal do Rio Grande do Sul, Brazil. Electronic address: idadschwartz@gmail.com.
authors.forename: Ida V D
authors.initials: IV
authors.lastname: Schwartz
mesh_terms: D052242:Adiponectin / Q000097:blood*; D000328:Adult; D015992:Body Mass Index; D008076:Cholesterol, HDL / Q000097:blood; D008078:Cholesterol, LDL / Q000097:blood; D003430:Cross-Sectional Studies; D056947:Enzyme Replacement Therapy / Q000379:methods*; D005260:Female; D005776:Gaucher Disease / Q000097:blood* / Q000150:complications / Q000628:therapy*; D054439:Ghrelin / Q000097:blood*; D006801:Humans; D007333:Insulin Resistance; D020738:Leptin / Q000097:blood*; D008297:Male; D024821:Metabolic Syndrome / Q000097:blood / Q000209:etiology; D008875:Middle Aged; D015596:Nutrition Assessment; D009752:Nutritional Status; D050177:Overweight / Q000097:blood / Q000209:etiology; D014280:Triglycerides / Q000097:blood; D055815:Young Adult
chemical_list: D052242:Adiponectin; D008076:Cholesterol, HDL; D008078:Cholesterol, LDL; D054439:Ghrelin; D020738:Leptin; D014280:Triglycerides
keywords: Adiponectin; Gaucher disease type I; Ghrelin; Insulin; Leptin