A subset of patients with acquired partial lipodystrophy developing severe metabolic abnormalities

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dc.contributor.authorSaydam, Basak Ozgen
dc.contributor.authorSonmez, Melda
dc.contributor.authorSimsir, Ilgin Yildirim
dc.contributor.authorErturk, Mehmet Sercan
dc.contributor.authorKulaksizoglu, Mustafa
dc.contributor.authorArkan, Tugba
dc.contributor.authorHekimsoy, Zeliha
dc.date.accessioned2024-02-23T14:20:15Z
dc.date.available2024-02-23T14:20:15Z
dc.date.issued2019
dc.departmentNEÜen_US
dc.description.abstractPurpose/Aim of the study: Acquired partial lipodystrophy (APL) is a rare disease characterized by selective loss of adipose tissue. In this study, we aimed to present a subset of patients with APL, who developed severe metabolic abnormalities, from our national lipodystrophy registry. Materials and Methods: Severe metabolic abnormalities were defined as: poorly controlled diabetes (HbA1c above 7% despite treatment with insulin more than 1 unit/kg/day combined with oral antidiabetics), severe hypertriglyceridemia (triglycerides above 500 mg/dL despite treatment with lipid-lowering drugs), episodes of acute pancreatitis, or severe hepatic involvement (biopsy-proven non-alcoholic steatohepatitis (NASH)). Results: Among 140 patients with all forms of lipodystrophy (28 with APL), we identified 6 APL patients with severe metabolic abnormalities. The geometric mean for age was 37 years (range: 27-50 years; 4 females and 2 males). Five patients had poorly controlled diabetes despite treatment with high-dose insulin combined with oral antidiabetics. Severe hypertriglyceridemia developed in five patients, of those three experienced episodes of acute pancreatitis. Although all six patients had hepatic steatosis at various levels on imaging studies, NASH was proven in two patients on liver biopsy. Our data suggested that APL patients with severe metabolic abnormalities had a more advanced fat loss and longer disease duration. Conclusions: We suggest that these patients represent a potential subgroup of APL who may benefit from metreleptin or investigational therapies as standard treatment strategies fail to achieve a good metabolic control.en_US
dc.identifier.doi10.1080/07435800.2018.1513029
dc.identifier.endpage54en_US
dc.identifier.issn0743-5800
dc.identifier.issn1532-4206
dc.identifier.issue1.Şuben_US
dc.identifier.pmid30182761en_US
dc.identifier.scopus2-s2.0-85053404701en_US
dc.identifier.scopusqualityQ2en_US
dc.identifier.startpage46en_US
dc.identifier.urihttps://doi.org/10.1080/07435800.2018.1513029
dc.identifier.urihttps://hdl.handle.net/20.500.12452/13083
dc.identifier.volume44en_US
dc.identifier.wosWOS:000465354000005en_US
dc.identifier.wosqualityQ4en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherTaylor & Francis Incen_US
dc.relation.ispartofEndocrine Researchen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectAcquired Partial Lipodystrophyen_US
dc.subjectDiabetesen_US
dc.subjectHepatic Steatosisen_US
dc.subjectHypertriglyceridemiaen_US
dc.subjectInsulin Resistanceen_US
dc.titleA subset of patients with acquired partial lipodystrophy developing severe metabolic abnormalitiesen_US
dc.typeArticleen_US

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