Publication:
Clinical features of generalized lipodystrophy in Turkey: A cohort analysis

dc.contributor.authorYıldırım Şimsir, Ilgin
dc.contributor.authorTuysuz, Beyhan
dc.contributor.authorÖzbek, Mehmet Nuri
dc.contributor.authorTanrıkulu, Seher
dc.contributor.authorÇelik Güler, Merve
dc.contributor.authorKarhan, Asuman Nur
dc.contributor.authorGüneş, Nilay
dc.contributor.authorSoyaltin, Utku Erdem
dc.contributor.authorAltay, Canan
dc.contributor.authorNur, Banu
dc.contributor.authorÖzalkak, Servan
dc.contributor.authorAkgün Doğan, Özlem
dc.contributor.authorDursun, Fatma
dc.contributor.authorPekkolay, Zafer
dc.contributor.authorEren, Mehmet Ali
dc.contributor.authorUsta, Yusuf
dc.contributor.authorÖzışık, Seçil
dc.contributor.authorÖzgen Saydam, Basak
dc.contributor.authorAdıyaman, Süleyman Cem
dc.contributor.authorÜnal, Mehmet Cağrı
dc.contributor.authorGüngor Semiz, Gökçen
dc.contributor.authorTuran, Ihsan
dc.contributor.authorKayserili, Hülya
dc.contributor.authorJeru, Isabelle
dc.contributor.authorVigouroux, Corinne
dc.contributor.authorAtik, Tahir
dc.contributor.authorOnay, Hüseyin
dc.contributor.authorÖzen, Samim
dc.contributor.authorArioğlu Oral, Elif
dc.contributor.authorAkıncı, Barış
dc.contributor.buuauthorDENKBOY ÖNGEN, YASEMİN
dc.contributor.buuauthorDenkboy Ongen, Yasemin
dc.contributor.buuauthorEREN, ERDAL
dc.contributor.buuauthorEren, Erdal
dc.contributor.departmentTıp Fakültesi
dc.contributor.departmentÇocuk sağlığı ve Hastalıkları Ana Bilim Dalı
dc.contributor.orcid0000-0002-5657-4260
dc.contributor.researcheridKHZ-1491-2024
dc.contributor.researcheridJPK-3909-2023
dc.date.accessioned2024-12-04T05:42:54Z
dc.date.available2024-12-04T05:42:54Z
dc.date.issued2023-04-11
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.
dc.identifier.doi10.1111/dom.15061
dc.identifier.endpage1963
dc.identifier.issn1462-8902
dc.identifier.issue7
dc.identifier.scopus2-s2.0-85152786989
dc.identifier.startpage1950
dc.identifier.urihttps://doi.org/10.1111/dom.15061
dc.identifier.urihttps://hdl.handle.net/11452/48850
dc.identifier.volume25
dc.identifier.wos000969368300001
dc.indexed.wosWOS.SCI
dc.language.isoen
dc.publisherWiley
dc.relation.journalDiabetes Obesity & Metabolism
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi
dc.rightsinfo:eu-repo/semantics/openAccess
dc.subjectLeptin
dc.subjectManifestations
dc.subjectPrevalence
dc.subjectGeneralized lipodystrophy
dc.subjectMetabolic disease
dc.subjectMortality
dc.subjectOrgan complications
dc.subjectScience & technology
dc.subjectLife sciences & biomedicine
dc.subjectEndocrinology & metabolism
dc.titleClinical features of generalized lipodystrophy in Turkey: A cohort analysis
dc.typeArticle
dspace.entity.typePublication
local.contributor.departmentTıp Fakültesi/Çocuk sağlığı ve Hastalıkları Ana Bilim Dalı
local.indexed.atWOS
local.indexed.atScopus
relation.isAuthorOfPublicationac939042-fc3d-410c-85ac-ec38841d5cad
relation.isAuthorOfPublication2d1c6521-88a9-4270-9918-92f16f98006c
relation.isAuthorOfPublication.latestForDiscovery2d1c6521-88a9-4270-9918-92f16f98006c

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