{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T13:22:20Z","timestamp":1790688140213,"version":"4.1.0"},"reference-count":26,"publisher":"The Endocrine Society","issue":"11","funder":[{"name":"Aegerion Pharmaceuticals","award":["N\/A"],"award-info":[{"award-number":["N\/A"]}]},{"DOI":"10.13039\/100000062","name":"National Institute of Diabetes and Digestive and Kidney Diseases","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000062","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Lipodystrophy Fund at the University of Michigan","award":["N\/A"],"award-info":[{"award-number":["N\/A"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2019,11,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Context<\/jats:title>\n                  <jats:p>Limited natural history data are available in patients with non-HIV\u2013related lipodystrophy syndromes who never received disease-specific therapies, making interpretation of benefits of therapies in lipodystrophy syndromes challenging.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Objective<\/jats:title>\n                  <jats:p>We assessed the natural history of non-HIV\u2013related generalized lipodystrophy (GL) and partial lipodystrophy (PL) in patients who have never received leptin or other lipodystrophy-specific therapies.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Design\/Setting\/Patients<\/jats:title>\n                  <jats:p>We conducted an international chart review of 230 patients with confirmed GL or PL at five treatment centers who never received leptin or other lipodystrophy-specific therapies. Patients were observed from birth to loss to follow-up, death, or date of chart abstraction.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Outcome Measures<\/jats:title>\n                  <jats:p>Lifetime prevalence of diabetes\/insulin resistance and select organ abnormalities, time to diabetes\/insulin resistance, first organ abnormality, disease progression, and mortality were described.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>Diabetes\/insulin resistance was identified in 58.3% of patients. Liver abnormalities were the most common organ abnormality (71.7%), followed by kidney (40.4%), heart (30.4%), and pancreatitis (13.0%). Kaplan-Meier estimates of mean (SE) time to first organ abnormality were 7.7 years (0.9) in GL and 16.1 years (1.5) in PL (P &lt; 0.001). Mean time to diabetes\/insulin resistance was 12.7 years (1.2) in GL and 19.1 years (1.7) in PL (P = 0.131). Mean time to disease progression was 7.6 years (0.8) and comparable between GL and PL subgroups (P = 0.393). Mean time to death was 51.2 years (3.5) in GL and 66.6 years (1.0) in PL (P &lt; 0.001).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>This large-scale study provides comprehensive, long-term data across multiple countries on the natural history of non-HIV\u2013related lipodystrophy.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1210\/jc.2018-02730","type":"journal-article","created":{"date-parts":[[2019,7,17]],"date-time":"2019-07-17T09:06:10Z","timestamp":1563354370000},"page":"5120-5135","source":"Crossref","is-referenced-by-count":72,"title":["Comorbidities and Survival in Patients With Lipodystrophy: An International Chart Review Study"],"prefix":"10.1210","volume":"104","author":[{"given":"Baris","family":"Akinci","sequence":"first","affiliation":[{"name":"Dokuz Eyl\u00fcl University, Izmir, Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elif A","family":"Oral","sequence":"additional","affiliation":[{"name":"Division of Metabolism, Endocrine & Diabetes and Brehm Center for Diabetes Research, Department of Internal Medicine, Michigan Medicine, University of Michigan, Ann Arbor, Michigan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adam","family":"Neidert","sequence":"additional","affiliation":[{"name":"Division of Metabolism, Endocrine & Diabetes and Brehm Center for Diabetes Research, Department of Internal Medicine, Michigan Medicine, University of Michigan, Ann Arbor, Michigan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diana","family":"Rus","sequence":"additional","affiliation":[{"name":"Division of Metabolism, Endocrine & Diabetes and Brehm Center for Diabetes Research, Department of Internal Medicine, Michigan Medicine, University of Michigan, Ann Arbor, Michigan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wendy Y","family":"Cheng","sequence":"additional","affiliation":[{"name":"Analysis Group Inc., Boston, Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9047-3941","authenticated-orcid":false,"given":"Philippe","family":"Thompson-Leduc","sequence":"additional","affiliation":[{"name":"Analysis Group Inc., Boston, Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hoi Ching","family":"Cheung","sequence":"additional","affiliation":[{"name":"Analysis Group Inc., Boston, Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pamela","family":"Bradt","sequence":"additional","affiliation":[{"name":"Aegerion Pharmaceuticals Inc., Cambridge, Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Cristina","family":"Foss de Freitas","sequence":"additional","affiliation":[{"name":"University of S\u00e3o Paulo-Ribeirao Preto Medical School, S\u00e3o Paulo, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"suffix":"Jr.","given":"Renan Magalh\u00e3es","family":"Montenegro","sequence":"additional","affiliation":[{"name":"Federal University of Cear\u00e1, Cear\u00e1, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Virg\u00ednia Oliveira","family":"Fernandes","sequence":"additional","affiliation":[{"name":"Federal University of Cear\u00e1, Cear\u00e1, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elaine","family":"Cochran","sequence":"additional","affiliation":[{"name":"National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rebecca J","family":"Brown","sequence":"additional","affiliation":[{"name":"National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"80","published-online":{"date-parts":[[2019,7,17]]},"reference":[{"issue":"12","key":"2019092505513613500_bib1","doi-asserted-by":"crossref","first-page":"4500","DOI":"10.1210\/jc.2016-2466","article-title":"The diagnosis and management of lipodystrophy syndromes: a multi-society practice guideline","volume":"101","author":"Brown","year":"2016","journal-title":"J Clin Endocrinol Metab"},{"issue":"11","key":"2019092505513613500_bib2","doi-asserted-by":"crossref","first-page":"3313","DOI":"10.1210\/jc.2011-1159","article-title":"Clinical review#: Lipodystrophies: genetic and acquired body fat disorders","volume":"96","author":"Garg","year":"2011","journal-title":"J Clin Endocrinol Metab"},{"key":"2019092505513613500_bib3","doi-asserted-by":"crossref","first-page":"375","DOI":"10.2147\/DMSO.S130810","article-title":"Estimating the prevalence of generalized and partial lipodystrophy: findings and 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Acquired lipodystrophy. Available at: https:\/\/rarediseases.org\/rare-diseases\/acquired-lipodystrophy\/. Accessed 1 October 2018."},{"issue":"11","key":"2019092505513613500_bib5","doi-asserted-by":"crossref","first-page":"964","DOI":"10.1016\/S1473-3099(13)70271-8","article-title":"Metabolic disease in HIV infection","volume":"13","author":"Lake","year":"2013","journal-title":"Lancet Infect Dis"},{"key":"2019092505513613500_bib6","unstructured":"Garg\n              A\n            \n          . The physician\u2019s guide to lipodystrophy disorders. Available at: https:\/\/rarediseases.org\/physician-guide\/lipodystrophy-disorders\/. 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