Baris Akinci1, Fatos Dilan Koseoglu2, Huseyin Onay3, Sevgi Yavuz4, Canan Altay5, Ilgin Yildirim Simsir6, Secil Ozisik7, Leyla Demir8, Meltem Korkut9, Nusret Yilmaz10, Samim Ozen11, Gulcin Akinci12, Tahir Atik3, Mehmet Calan7, Mustafa Secil5, Abdurrahman Comlekci7, Tevfik Demir7. 1. Dokuz Eylul University, Division of Endocrinology, Izmir, Turkey. Electronic address: barisakincimd@gmail.com. 2. Tepecik Training Hospital, Department of Internal Medicine, Izmir, Turkey. 3. Ege University, Department of Medical Genetics, Izmir, Turkey. 4. Kanuni Sultan Suleyman Training Hospital, Department of Dermatology, Istanbul, Turkey. 5. Dokuz Eylul University, Department of Radiology, Izmir, Turkey. 6. Ege University, Division of Endocrinology, Izmir, Turkey. 7. Dokuz Eylul University, Division of Endocrinology, Izmir, Turkey. 8. Ataturk Training Hospital, Department of Biochemistry, Izmir, Turkey. 9. Yeditepe University, Division of Pediatric Gastroenterology, Istanbul, Turkey. 10. Akdeniz University, Division of Endocrinology, Antalya, Turkey. 11. Ege University, Department of Medical Genetics, Izmir, Turkey; Ege University, Division of Pediatric Endocrinology, Izmir, Turkey. 12. Dr.Behcet Uz Children's Hospital, Division of Pediatric Neurology, Izmir, Turkey.
Abstract
OBJECTIVE: Acquired partial lipodystrophy (APL) is a rare disorder characterized by progressive selective fat loss. In previous studies, metabolic abnormalities were reported to be relatively rare in APL, whilst they were quite common in other types of lipodystrophy syndromes. METHODS: In this nationwide cohort study, we evaluated 21 Turkish patients with APL who were enrolled in a prospective follow-up protocol. Subjects were investigated for metabolic abnormalities. Fat distribution was assessed by whole body MRI. Hepatic steatosis was evaluated by ultrasound, MRI and MR spectroscopy. Patients with diabetes underwent a mix meal stimulated C-peptide/insulin test to investigate pancreatic beta cell functions. Leptin and adiponectin levels were measured. RESULTS: Fifteen individuals (71.4%) had at least one metabolic abnormality. Six patients (28.6%) had diabetes, 12 (57.1%) hypertrigylceridemia, 10 (47.6%) low HDL cholesterol, and 11 (52.4%) hepatic steatosis. Steatohepatitis was further confirmed in 2 patients with liver biopsy. Anti-GAD was negative in all APL patients with diabetes. APL patients with diabetes had lower leptin and adiponectin levels compared to patients with type 2 diabetes and healthy controls. However, contrary to what we observed in patients with congenital generalized lipodystrophy (CGL), we did not detect consistently very low leptin levels in APL patients. The mix meal test suggested that APL patients with diabetes had a significant amount of functional pancreatic beta cells, and their diabetes was apparently associated with insulin resistance. CONCLUSIONS: Our results show that APL is associated with increased risk for developing metabolic abnormalities. We suggest that close long-term follow-up is required to identify and manage metabolic abnormalities in APL.
OBJECTIVE: Acquired partial lipodystrophy (APL) is a rare disorder characterized by progressive selective fat loss. In previous studies, metabolic abnormalities were reported to be relatively rare in APL, whilst they were quite common in other types of lipodystrophy syndromes. METHODS: In this nationwide cohort study, we evaluated 21 Turkish patients with APL who were enrolled in a prospective follow-up protocol. Subjects were investigated for metabolic abnormalities. Fat distribution was assessed by whole body MRI. Hepatic steatosis was evaluated by ultrasound, MRI and MR spectroscopy. Patients with diabetes underwent a mix meal stimulated C-peptide/insulin test to investigate pancreatic beta cell functions. Leptin and adiponectin levels were measured. RESULTS: Fifteen individuals (71.4%) had at least one metabolic abnormality. Six patients (28.6%) had diabetes, 12 (57.1%) hypertrigylceridemia, 10 (47.6%) low HDL cholesterol, and 11 (52.4%) hepatic steatosis. Steatohepatitis was further confirmed in 2 patients with liver biopsy. Anti-GAD was negative in all APLpatients with diabetes. APLpatients with diabetes had lower leptin and adiponectin levels compared to patients with type 2 diabetes and healthy controls. However, contrary to what we observed in patients with congenital generalized lipodystrophy (CGL), we did not detect consistently very low leptin levels in APLpatients. The mix meal test suggested that APLpatients with diabetes had a significant amount of functional pancreatic beta cells, and their diabetes was apparently associated with insulin resistance. CONCLUSIONS: Our results show that APL is associated with increased risk for developing metabolic abnormalities. We suggest that close long-term follow-up is required to identify and manage metabolic abnormalities in APL.
Authors: Elif A Oral; Phillip Gorden; Elaine Cochran; David Araújo-Vilar; David B Savage; Alison Long; Gregory Fine; Taylor Salinardi; Rebecca J Brown Journal: Endocrine Date: 2019-02-25 Impact factor: 3.633