{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T14:17:55Z","timestamp":1784557075205,"version":"3.55.0"},"reference-count":6,"publisher":"Bioscientifica","issue":"1","license":[{"start":{"date-parts":[[2025,1,1]],"date-time":"2025-01-01T00:00:00Z","timestamp":1735689600000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["edm.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2025,1,1]]},"abstract":"\n Summary<\/jats:title>\n A 17-year-old girl presented with recurrent attacks of acute pancreatitis, associated with severe hyperglycemia and hypertriglyceridemia, despite being on intensive insulin therapy for the last 10 years. She had severe acanthosis nigricans, generalized loss of subcutaneous fat and prominent veins over extremities. The serum levels of glucose and triglyceride did not reduce significantly, even with maximally tolerated doses of metformin (2 g), pioglitazone (45 mg) and fenofibrate (160 mg), not uncommonly seen in poor rural families in West Bengal, India. A detailed dietary recall revealed a very high carbohydrate intake (70% of total calorie) with very low protein and fat intake. A switch to a very low carbohydrate (30% of total calorie) diet led to a remarkable improvement in glucose and lipid profiles (the daily insulin requirement came down by 50% and triglyceride level came down to 600 mg\/dL from 950 mg\/dL). A whole-exome sequencing study confirmed congenital generalized lipodystrophy type 4. A carbohydrate restriction strategy may improve difficult-to-control glycometabolic profile in lipodystrophic subjects on high-carbohydrate diet.<\/jats:p>\n <\/jats:sec>\n \n Learning points<\/jats:title>\n \n \n Lipodystrophy should be suspected in patient presenting with hyperglycemia, hypertriglyceridemia and low BMI.<\/jats:p>\n <\/jats:list-item>\n \n A very low carbohydrate diet (30% of total daily calorie intake) may significantly improve glucose and lipid profiles in patients with lipoatrophic diabetes.<\/jats:p>\n <\/jats:list-item>\n \n Blood glucose may be the most important initial step to control hypertriglyceridemia and risk of pancreatitis in this group of patients.<\/jats:p>\n <\/jats:list-item>\n <\/jats:list>\n <\/jats:sec>","DOI":"10.1530\/edm-24-0063","type":"journal-article","created":{"date-parts":[[2025,1,27]],"date-time":"2025-01-27T06:43:23Z","timestamp":1737960203000},"update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-2","source":"Crossref","is-referenced-by-count":2,"title":["A very low carbohydrate diet improved metabolic profile in congenital generalized lipodystrophy type 4"],"prefix":"10.1530","volume":"2025","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8511-6138","authenticated-orcid":true,"given":"Sayantan","family":"Chakraborty","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Subhankar","family":"Roy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Debaditya","family":"Das","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sayantani","family":"Chatterjee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pranab Kumar","family":"Sahana","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8033-5748","authenticated-orcid":true,"given":"Satinath","family":"Mukhopadhyay","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"416","reference":[{"key":"bib1","series-title":"Cell Metab","first-page":"310","article-title":"Deletion of Cavin\/PTRF causes global loss of caveolae, dyslipidemia, and glucose intolerance","volume":"8","author":"Liu L","year":"2008","unstructured":"Liu L\n, \nBrown D\n, \nMcKee M\n, et al.\n Deletion of Cavin\/PTRF causes global loss of caveolae, dyslipidemia, and glucose intolerance. Cell Metab 2008 8 310\u2013317. (https:\/\/doi.org\/10.1016\/j.cmet.2008.07.008)"},{"key":"bib2","series-title":"Am J Physiol Cell Physiol","first-page":"C222","article-title":"Caveolin-1-deficient mice show insulin resistance and defective insulin receptor protein expression in adipose tissue","volume":"285","author":"Cohen AW","year":"2003","unstructured":"Cohen AW\n, \nRazani B\n, \nWang XB\n, et al.\n Caveolin-1-deficient mice show insulin resistance and defective insulin receptor protein expression in adipose tissue. Am J Physiol Cell Physiol 2003 285 C222\u2013C235. (https:\/\/doi.org\/10.1152\/ajpcell.00006.2003)"},{"key":"bib3","series-title":"J Rare Dis Res and Treat","first-page":"1","article-title":"Congenital generalized lipodystrophy","volume":"3","author":"Lima JG","year":"2018","unstructured":"Lima JG\n, \ndos Santos MC\n & \nde Melo Campos JT\n\n. Congenital generalized lipodystrophy. J Rare Dis Res and Treat 2018 3 1\u20136. (https:\/\/doi.org\/10.29245\/2572-9411\/2018\/2.1147)"},{"key":"bib4","series-title":"Am J Med Genet A","first-page":"2245","article-title":"Congenital generalized lipodystrophy, type 4 (CGL4) associated with myopathy due to novel PTRF mutations","volume":"152A","author":"Shastry S","year":"2010","unstructured":"Shastry S\n, \nDelgado MR\n, \nDirik E\n, et al.\n Congenital generalized lipodystrophy, type 4 (CGL4) associated with myopathy due to novel PTRF mutations. Am J Med Genet A 2010 152A 2245\u20132253. (https:\/\/doi.org\/10.1002\/ajmg.a.33578)"},{"key":"bib5","series-title":"J Clin Res Pediatr Endocrinol","first-page":"58","article-title":"Syndrome of extreme insulin resistance (Rabson-Mendenhall phenotype) with atrial septal defect: clinical presentation and treatment outcomes","volume":"5","author":"Deep D","year":"2013","unstructured":"Deep D\n, \nIndira M\n, \nSujoy G\n, et al.\n Syndrome of extreme insulin resistance (Rabson-Mendenhall phenotype) with atrial septal defect: clinical presentation and treatment outcomes. J Clin Res Pediatr Endocrinol 2013 5 58\u201361. (https:\/\/doi.org\/10.4274\/jcrpe.857)"},{"key":"bib6","series-title":"BMJ open","first-page":"e005138","article-title":"Results from a dietary survey in an Indian T2DM population: a STARCH study","volume":"4","author":"Joshi SR","year":"2014","unstructured":"Joshi SR\n, \nBhansali A\n, \nBajaj S\n, et al\n. Results from a dietary survey in an Indian T2DM population: a STARCH study. BMJ open 2014 4 e005138. (https:\/\/doi.org\/10.1136\/bmjopen-2014-005138)"}],"container-title":["Endocrinology, Diabetes & Metabolism Case Reports"],"original-title":[],"link":[{"URL":"https:\/\/edm.bioscientifica.com\/view\/journals\/edm\/2025\/1\/EDM-24-0063.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/edm.bioscientifica.com\/downloadpdf\/journals\/edm\/2025\/1\/EDM-24-0063.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/edm.bioscientifica.com\/downloadpdf\/journals\/edm\/2025\/1\/EDM-24-0063.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T13:48:27Z","timestamp":1784555307000},"score":1,"resource":{"primary":{"URL":"http:\/\/journals.bioscientifica.com\/edm\/article\/3755"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,1,1]]},"references-count":6,"journal-issue":{"issue":"1"},"URL":"https:\/\/doi.org\/10.1530\/edm-24-0063","relation":{},"ISSN":["2052-0573"],"issn-type":[{"value":"2052-0573","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,1,1]]}}}