Clinical features of generalized lipodystrophy in Turkey: A cohort analysis
An erratum to this article was published (Diabetes Obes Metab, Feb 2024 ↗)
Authors' conclusion
Standard treatment approaches have only a limited impact and do not prevent the development of severe metabolic abnormalities and early onset of organ complications in GL.
Study context
- Population
- Patients with generalized lipodystrophy (GL) registered at centres covering all regions of Turkey; 47 families47 families
- Participants
- 72 patients
- Outcome
- Frequency of each complication and death rate during follow-up
- Follow-up
- Mean ± SD 86 ± 78 months
Methodological detailsmulticentre, national registry cohort
- Multicentre
- National registry cohort
Main finding
As reported in the abstract
- Median time to diagnosis of diabetes and/or prediabetes (Kaplan-Meier)
- 16 years
- Hyperglycaemia not controlled in patients with diabetes
- 37 of 45 (82.2%)
- Hypertriglyceridaemia
- 65 (90.3%)
- Median time to diagnosis of hypertriglyceridaemia (Kaplan-Meier)
- 14 years
- Severe hypertriglyceridaemia (≥ 500 mg/dl)
- 38 (52.8%)
- Pancreatitis
- 7 (9.7%)
- Median time to diagnosis of hepatic steatosis (Kaplan-Meier)
- 15 years
- Progression of liver disease to cirrhosis
- 9 (12.5%)
- Proteinuric chronic kidney disease (CKD)
- 32 (44.4%)
- Cardiac disease
- 23 (31.9%)
- Median time to diagnosis of CKD (Kaplan-Meier)
- 25 years
- Median time to diagnosis of cardiac disease (Kaplan-Meier)
- 45 years
- Deaths during follow-up
- 10
Observational data: associations, not effects.
Further findings
Liver disease by subtype
- Liver disease more severe in congenital lipodystrophy type 2 (CGL2)
Metabolic disease by sex
- Females appeared to have more severe metabolic disease, with earlier onset of metabolic abnormalities
Original publication
Clinical features of generalized lipodystrophy in Turkey: A cohort analysis. Diabetes Obes Metab. Published 11 April 2023. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →