Clinical features of generalized lipodystrophy in Turkey: a cohort analysis

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dc.contributor.authorÖzbek, Mehmet Nuri
dc.contributor.authorYildirim Simsir, Ilgin
dc.contributor.authorTuysuz, Beyhan
dc.contributor.authorTanrikulu, Seher
dc.contributor.authorCelik Guler, Merve
dc.contributor.authorAkinci, Baris
dc.date.accessioned2023-07-27T07:59:51Z
dc.date.available2023-07-27T07:59:51Z
dc.date.issued2023en_US
dc.departmentMAÜ, Fakülteler, Tıp Fakültesi, Dahili Tıp Bilimleri Bölümü, Çocuk Sağlığı ve Hastalıkları Ana Bilim Dalıen_US
dc.description.abstractAim: 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.en_US
dc.identifier.citationYildirim 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.en_US
dc.identifier.doi10.1111/dom.15061en_US
dc.identifier.endpage1963en_US
dc.identifier.issue7en_US
dc.identifier.pmid36946378en_US
dc.identifier.scopus2-s2.0-85152786989en_US
dc.identifier.scopusqualityN/Aen_US
dc.identifier.startpage1950en_US
dc.identifier.urihttps://doi.org/10.1111/dom.15061
dc.identifier.urihttps://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
dc.identifier.urihttps://pubmed.ncbi.nlm.nih.gov/36946378/#full-view-affiliation-1
dc.identifier.urihttps://hdl.handle.net/20.500.12514/3546
dc.identifier.volume25en_US
dc.identifier.wosWOS:000969368300001en_US
dc.identifier.wosqualityN/Aen_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherWiley Online Libraryen_US
dc.relation.ispartofDiabetes, Obesity and Metabolismen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.subjectgeneralized lipodystrophy; metabolic disease; mortality; organ complications.en_US
dc.titleClinical features of generalized lipodystrophy in Turkey: a cohort analysisen_US
dc.typeArticleen_US

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