Clinical features of generalized lipodystrophy in Turkey: a cohort analysis
Yükleniyor...
Tarih
2023
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Wiley Online Library
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
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.
Açıklama
Anahtar Kelimeler
generalized lipodystrophy; metabolic disease; mortality; organ complications.
Kaynak
Diabetes, Obesity and Metabolism
WoS Q Değeri
N/A
Scopus Q Değeri
N/A
Cilt
25
Sayı
7
Künye
Yildirim Simsir, I., Tuysuz, B., Ozbek, M. N., Tanrikulu, S., Celik Guler, M., Karhan, A. N., ... & Akinci, B. (2023). Clinical features of generalized lipodystrophy in Turkey: A cohort analysis. Diabetes, Obesity and Metabolism, 25(7), 1950-1963.
Bağlantı
https://doi.org/10.1111/dom.15061
https://www.scopus.com/record/display.uri?eid=2-s2.0-85152786989&origin=resultslist&sort=plf-f&src=s&sid=e6ffd6956e9981255ba20ae83e3a4e9a&sot=b&sdt=b&s=DOI%2810.1111%2Fdom.15061%29&sl=22&sessionSearchId=e6ffd6956e9981255ba20ae83e3a4e9a
https://pubmed.ncbi.nlm.nih.gov/36946378/#full-view-affiliation-1
https://hdl.handle.net/20.500.12514/3546
https://www.scopus.com/record/display.uri?eid=2-s2.0-85152786989&origin=resultslist&sort=plf-f&src=s&sid=e6ffd6956e9981255ba20ae83e3a4e9a&sot=b&sdt=b&s=DOI%2810.1111%2Fdom.15061%29&sl=22&sessionSearchId=e6ffd6956e9981255ba20ae83e3a4e9a
https://pubmed.ncbi.nlm.nih.gov/36946378/#full-view-affiliation-1
https://hdl.handle.net/20.500.12514/3546
