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Journal Articles Diabetes, Obesity and Metabolism Year : 2023

Clinical features of generalized lipodystrophy in Turkey: A cohort analysis

Ilgin Yildirim Simsir
Beyhan Tuysuz
Mehmet Nuri Ozbek
Seher Tanrikulu
Merve Celik Guler
Asuman Nur Karhan
Yasemin Denkboy Ongen
Nilay Gunes
Utku Erdem Soyaltin
Canan Altay
Banu Nur
Servan Ozalkak
Ozlem Akgun Dogan
Fatma Dursun
Zafer Pekkolay
Mehmet Ali Eren
Yusuf Usta
Secil Ozisik
Basak Ozgen Saydam
Suleyman Cem Adiyaman
Mehmet Cagri Unal
Gokcen Gungor Semiz
Ihsan Turan
Erdal Eren
Hulya Kayserili
Isabelle Jeru
Tahir Atik
Huseyin Onay
Samim Ozen
Elif Arioglu Oral
Baris Akinci

Abstract

Abstract Aim To describe the Turkish generalized lipodystrophy (GL) cohort with the frequency of each complication and the death rate during the period of the follow‐up. Methods This study reports on 72 patients with GL (47 families) registered at different centres in Turkey that cover all regions of the country. The mean ± SD follow‐up was 86 ± 78 months. Results The Kaplan–Meier estimate of the median time to diagnosis of diabetes and/or prediabetes was 16 years. Hyperglycaemia was not controlled in 37 of 45 patients (82.2%) with diabetes. Hypertriglyceridaemia developed in 65 patients (90.3%). The Kaplan–Meier estimate of the median time to diagnosis of hypertriglyceridaemia was 14 years. Hypertriglyceridaemia was severe (≥ 500 mg/dl) in 38 patients (52.8%). Seven (9.7%) patients suffered from pancreatitis. The Kaplan–Meier estimate of the median time to diagnosis of hepatic steatosis was 15 years. Liver disease progressed to cirrhosis in nine patients (12.5%). Liver disease was more severe in congenital lipodystrophy type 2 (CGL2). Proteinuric chronic kidney disease (CKD) developed in 32 patients (44.4%) and cardiac disease in 23 patients (31.9%). Kaplan–Meier estimates of the median time to diagnosis of CKD and cardiac disease were 25 and 45 years, respectively. Females appeared to have a more severe metabolic disease, with an earlier onset of metabolic abnormalities. Ten patients died during the follow‐up period. Causes of death were end‐stage renal disease, sepsis (because of recurrent intestinal perforations, coronavirus disease, diabetic foot infection and following coronary artery bypass graft surgery), myocardial infarction, heart failure because of dilated cardiomyopathy, stroke, liver complications and angiosarcoma. Conclusions 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.

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Dates and versions

hal-04455978 , version 1 (13-02-2024)

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Ilgin Yildirim Simsir, Beyhan Tuysuz, Mehmet Nuri Ozbek, Seher Tanrikulu, Merve Celik Guler, et al.. Clinical features of generalized lipodystrophy in Turkey: A cohort analysis. Diabetes, Obesity and Metabolism, 2023, 25 (7), pp.1950-1963. ⟨10.1111/dom.15061⟩. ⟨hal-04455978⟩
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