{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T14:17:43Z","timestamp":1784557063427,"version":"3.55.0"},"reference-count":0,"publisher":"Bioscientifica","license":[{"start":{"date-parts":[[2021,4,1]],"date-time":"2021-04-01T00:00:00Z","timestamp":1617235200000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["edm.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2021,4,1]]},"abstract":"<jats:sec>\n                    <jats:title>Summary<\/jats:title>\n                    <jats:p>\n                      Berardinelli\u2013Seip congenital lipodystrophy (BSCL) is a rare autosomal recessive disease, characterized by the absence of subcutaneous adipose tissue, leptin deficiency and severe metabolic complications, such as insulin resistance, diabetes mellitus\n                      <jats:italic>,<\/jats:italic>\n                      and dyslipidemia. The most common mutation occurs in\n                      <jats:italic>BCSL2<\/jats:italic>\n                      which encodes seipin, a protein involved in adipogenesis. We report a patient with BSCL who was diagnosed with diabetes at 11 years old. He was started on metformin 1000 mg twice daily, which lowered glycated hemoglobin (HbA1c) to less than 7%. Four months later, HbA1c raised above 7.5%, indicating secondary failure to metformin. Therefore, we added the peroxisome proliferator-activated receptor-gamma (PPARG) agonist, pioglitazone. Since then and for the last 5 years his HbA1c has been within the normal range. These findings indicate that pioglitazone should be considered as a valid alternative in the treatment of diabetes in BSCL patients. To the best of our knowledge, this is the first specific report of successful long-term treatment with pioglitazone in a patient with BSCL.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Learning points<\/jats:title>\n                    <jats:list list-type=\"bullet\">\n                      <jats:list-item>\n                        <jats:p>Berardinelli\u2013Seip congenital lipodystrophy (BSCL) is a recessive genetic disorder associated with severe insulin resistance and early onset diabetes, usually around puberty. Failure of oral antidiabetic medication occurs within the first years of treatment in BSCL patients.<\/jats:p>\n                      <\/jats:list-item>\n                      <jats:list-item>\n                        <jats:p>When failure to achieve metabolic control with metformin occurs, pioglitazone may be a safe option, lowering insulin resistance and improving both the metabolic control and lipodystrophic phenotype.<\/jats:p>\n                      <\/jats:list-item>\n                      <jats:list-item>\n                        <jats:p>Herein we show that pioglitazone can be a safe and efficient alternative in the long-term treatment of BSCL patients with diabetes.<\/jats:p>\n                      <\/jats:list-item>\n                    <\/jats:list>\n                  <\/jats:sec>","DOI":"10.1530\/edm-20-0183","type":"journal-article","created":{"date-parts":[[2021,4,21]],"date-time":"2021-04-21T06:58:05Z","timestamp":1618988285000},"update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-2","source":"Crossref","is-referenced-by-count":4,"title":["Successful long-term use of pioglitazone in Berardinelli\u2013Seip lipodystrophy-associated diabetes"],"prefix":"10.1530","volume":"2021","author":[{"given":"Carolina","family":"Chaves","sequence":"first","affiliation":[{"name":"1Servi\u00e7o de Endocrinologia e Nutri\u00e7\u00e3oHospital Divino Esp\u00edrito Santo de Ponta Delgada, Ponta Delgada, A\u00e7ores, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariana","family":"Chaves","sequence":"additional","affiliation":[{"name":"2Servi\u00e7o de RadiologiaHospital Divino Esp\u00edrito Santo de Ponta Delgada, Ponta Delgada, A\u00e7ores, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jo\u00e3o","family":"Anselmo","sequence":"additional","affiliation":[{"name":"1Servi\u00e7o de Endocrinologia e Nutri\u00e7\u00e3oHospital Divino Esp\u00edrito Santo de Ponta Delgada, Ponta Delgada, A\u00e7ores, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rui","family":"C\u00e9sar","sequence":"additional","affiliation":[{"name":"1Servi\u00e7o de Endocrinologia e Nutri\u00e7\u00e3oHospital Divino Esp\u00edrito Santo de Ponta Delgada, Ponta Delgada, A\u00e7ores, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"416","container-title":["Endocrinology, Diabetes &amp; Metabolism Case Reports"],"original-title":[],"link":[{"URL":"https:\/\/edm.bioscientifica.com\/view\/journals\/edm\/2021\/1\/EDM20-0183.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/edm.bioscientifica.com\/downloadpdf\/journals\/edm\/2021\/1\/EDM20-0183.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T13:47:31Z","timestamp":1784555251000},"score":1,"resource":{"primary":{"URL":"http:\/\/journals.bioscientifica.com\/edm\/article\/3396"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,4,1]]},"references-count":0,"URL":"https:\/\/doi.org\/10.1530\/edm-20-0183","relation":{},"ISSN":["2052-0573"],"issn-type":[{"value":"2052-0573","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,4,1]]}}}