title: Acquired partial lipodystrophy is associated with increased risk for developing metabolic abnormalities.
abstract: OBJECTIVE
  Acquired partial lipodystrophy (APL) is a rare disorder characterized by progressive selective fat loss. In previous studies, metabolic abnormalities were reported to be relatively rare in APL, whilst they were quite common in other types of lipodystrophy syndromes.
  
  
  METHODS
  In this nationwide cohort study, we evaluated 21 Turkish patients with APL who were enrolled in a prospective follow-up protocol. Subjects were investigated for metabolic abnormalities. Fat distribution was assessed by whole body MRI. Hepatic steatosis was evaluated by ultrasound, MRI and MR spectroscopy. Patients with diabetes underwent a mix meal stimulated C-peptide/insulin test to investigate pancreatic beta cell functions. Leptin and adiponectin levels were measured.
  
  
  RESULTS
  Fifteen individuals (71.4%) had at least one metabolic abnormality. Six patients (28.6%) had diabetes, 12 (57.1%) hypertrigylceridemia, 10 (47.6%) low HDL cholesterol, and 11 (52.4%) hepatic steatosis. Steatohepatitis was further confirmed in 2 patients with liver biopsy. Anti-GAD was negative in all APL patients with diabetes. APL patients with diabetes had lower leptin and adiponectin levels compared to patients with type 2 diabetes and healthy controls. However, contrary to what we observed in patients with congenital generalized lipodystrophy (CGL), we did not detect consistently very low leptin levels in APL patients. The mix meal test suggested that APL patients with diabetes had a significant amount of functional pancreatic beta cells, and their diabetes was apparently associated with insulin resistance.
  
  
  CONCLUSIONS
  Our results show that APL is associated with increased risk for developing metabolic abnormalities. We suggest that close long-term follow-up is required to identify and manage metabolic abnormalities in APL.
authors.affiliation: Dokuz Eylul University, Division of Endocrinology, Izmir, Turkey. Electronic address: barisakincimd@gmail.com.
authors.forename: Baris
authors.initials: B
authors.lastname: Akinci
authors.affiliation: Tepecik Training Hospital, Department of Internal Medicine, Izmir, Turkey.
authors.forename: Fatos Dilan
authors.initials: FD
authors.lastname: Koseoglu
authors.affiliation: Ege University, Department of Medical Genetics, Izmir, Turkey.
authors.forename: Huseyin
authors.initials: H
authors.lastname: Onay
authors.affiliation: Kanuni Sultan Suleyman Training Hospital, Department of Dermatology, Istanbul, Turkey.
authors.forename: Sevgi
authors.initials: S
authors.lastname: Yavuz
authors.affiliation: Dokuz Eylul University, Department of Radiology, Izmir, Turkey.
authors.forename: Canan
authors.initials: C
authors.lastname: Altay
authors.affiliation: Ege University, Division of Endocrinology, Izmir, Turkey.
authors.forename: Ilgin Yildirim
authors.initials: IY
authors.lastname: Simsir
authors.affiliation: Dokuz Eylul University, Division of Endocrinology, Izmir, Turkey.
authors.forename: Secil
authors.initials: S
authors.lastname: Ozisik
authors.affiliation: Ataturk Training Hospital, Department of Biochemistry, Izmir, Turkey.
authors.forename: Leyla
authors.initials: L
authors.lastname: Demir
authors.affiliation: Yeditepe University, Division of Pediatric Gastroenterology, Istanbul, Turkey.
authors.forename: Meltem
authors.initials: M
authors.lastname: Korkut
authors.affiliation: Akdeniz University, Division of Endocrinology, Antalya, Turkey.
authors.forename: Nusret
authors.initials: N
authors.lastname: Yilmaz
authors.affiliation: Ege University, Department of Medical Genetics, Izmir, Turkey; Ege University, Division of Pediatric Endocrinology, Izmir, Turkey.
authors.forename: Samim
authors.initials: S
authors.lastname: Ozen
authors.affiliation: Dr.Behcet Uz Children's Hospital, Division of Pediatric Neurology, Izmir, Turkey.
authors.forename: Gulcin
authors.initials: G
authors.lastname: Akinci
authors.affiliation: Ege University, Department of Medical Genetics, Izmir, Turkey.
authors.forename: Tahir
authors.initials: T
authors.lastname: Atik
authors.affiliation: Dokuz Eylul University, Division of Endocrinology, Izmir, Turkey.
authors.forename: Mehmet
authors.initials: M
authors.lastname: Calan
authors.affiliation: Dokuz Eylul University, Department of Radiology, Izmir, Turkey.
authors.forename: Mustafa
authors.initials: M
authors.lastname: Secil
authors.affiliation: Dokuz Eylul University, Division of Endocrinology, Izmir, Turkey.
authors.forename: Abdurrahman
authors.initials: A
authors.lastname: Comlekci
authors.affiliation: Dokuz Eylul University, Division of Endocrinology, Izmir, Turkey.
authors.forename: Tevfik
authors.initials: T
authors.lastname: Demir
mesh_terms: D052242:Adiponectin / Q000097:blood; D000293:Adolescent; D000328:Adult; D000368:Aged; D015331:Cohort Studies; D048909:Diabetes Complications / Q000453:epidemiology / Q000209:etiology; D005234:Fatty Liver / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D020738:Leptin / Q000097:blood; D008060:Lipodystrophy / Q000150:complications* / Q000453:epidemiology; D008279:Magnetic Resonance Imaging; D008297:Male; D008659:Metabolic Diseases / Q000453:epidemiology / Q000209:etiology*; D008875:Middle Aged; D011446:Prospective Studies; D012042:Registries; D012306:Risk; D014421:Turkey / Q000453:epidemiology; D055815:Young Adult
chemical_list: D052242:Adiponectin; D020738:Leptin
keywords: APL; Diabetes; Hepatic steatosis; Hypertriglyceridemia; Insulin resistance