A very low carbohydrate diet improved metabolic profile in congenital generalized lipodystrophy type 4
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 ov...
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Bioscientifica
2025-01-01
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| Series: | Endocrinology, Diabetes & Metabolism Case Reports |
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| Online Access: | https://edm.bioscientifica.com/view/journals/edm/2025/1/EDM-24-0063.xml |
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| author | Sayantan Chakraborty Subhankar Roy Debaditya Das Sayantani Chatterjee Pranab Kumar Sahana Satinath Mukhopadhyay |
| author_facet | Sayantan Chakraborty Subhankar Roy Debaditya Das Sayantani Chatterjee Pranab Kumar Sahana Satinath Mukhopadhyay |
| author_sort | Sayantan Chakraborty |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-d783b5f9732641568c767c42b013c07f |
| institution | Kabale University |
| issn | 2052-0573 |
| language | English |
| publishDate | 2025-01-01 |
| publisher | Bioscientifica |
| record_format | Article |
| series | Endocrinology, Diabetes & Metabolism Case Reports |
| spelling | doaj-art-d783b5f9732641568c767c42b013c07f2025-02-05T10:16:51ZengBioscientificaEndocrinology, Diabetes & Metabolism Case Reports2052-05732025-01-012025110.1530/EDM-24-00631A very low carbohydrate diet improved metabolic profile in congenital generalized lipodystrophy type 4Sayantan Chakraborty0Subhankar Roy1Debaditya Das2Sayantani Chatterjee3Pranab Kumar Sahana4Satinath Mukhopadhyay5Department of Endocrinology, IPGME&R, Kolkata, IndiaDepartment of Endocrinology, IPGME&R, Kolkata, IndiaDepartment of Endocrinology, IPGME&R, Kolkata, IndiaDepartment of Endocrinology, IPGME&R, Kolkata, IndiaDepartment of Endocrinology, IPGME&R, Kolkata, IndiaDepartment of Endocrinology, IPGME&R, Kolkata, IndiaA 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.https://edm.bioscientifica.com/view/journals/edm/2025/1/EDM-24-0063.xmlcongenital generalized lipodystrophyhypertriglyceridemialow carbohydrate dietinsulin resistance |
| spellingShingle | Sayantan Chakraborty Subhankar Roy Debaditya Das Sayantani Chatterjee Pranab Kumar Sahana Satinath Mukhopadhyay A very low carbohydrate diet improved metabolic profile in congenital generalized lipodystrophy type 4 Endocrinology, Diabetes & Metabolism Case Reports congenital generalized lipodystrophy hypertriglyceridemia low carbohydrate diet insulin resistance |
| title | A very low carbohydrate diet improved metabolic profile in congenital generalized lipodystrophy type 4 |
| title_full | A very low carbohydrate diet improved metabolic profile in congenital generalized lipodystrophy type 4 |
| title_fullStr | A very low carbohydrate diet improved metabolic profile in congenital generalized lipodystrophy type 4 |
| title_full_unstemmed | A very low carbohydrate diet improved metabolic profile in congenital generalized lipodystrophy type 4 |
| title_short | A very low carbohydrate diet improved metabolic profile in congenital generalized lipodystrophy type 4 |
| title_sort | very low carbohydrate diet improved metabolic profile in congenital generalized lipodystrophy type 4 |
| topic | congenital generalized lipodystrophy hypertriglyceridemia low carbohydrate diet insulin resistance |
| url | https://edm.bioscientifica.com/view/journals/edm/2025/1/EDM-24-0063.xml |
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