Baris Akinci1, Sadiye Mehtat Unlu2, Ali Celik3, Ilgin Yildirim Simsir4, Sait Sen5, Banu Nur6, Fatma Ela Keskin7, Basak Ozgen Saydam1, Nilufer Kutbay Ozdemir8, Banu Sarer Yurekli4, Bekir Ugur Ergur9, Melda Sonmez10, Tahir Atik11, Atakan Arslan12, Tevfik Demir1, Canan Altay12, Ulku Aybuke Tunc13, Tugba Arkan14, Ramazan Gen15, Erdal Eren16, Gulcin Akinci17, Aslihan Arasli Yilmaz18, Habib Bilen19, Samim Ozen20, Aygul Celtik21, Senay Savas Erdeve18, Semra Cetinkaya18, Huseyin Onay11, Sulen Sarioglu2, Elif Arioglu Oral22. 1. Division of Endocrinology, Department of Internal Medicine, Dokuz Eylul University, Izmir, Turkey. 2. Department of Pathology, Dokuz Eylul University, Izmir, Turkey. 3. Division of Nephrology, Department of Internal Medicine, Dokuz Eylul University, Izmir, Turkey. 4. Division of Endocrinology, Department of Internal Medicine, Ege University, Izmir, Turkey. 5. Department of Pathology, Ege University, Izmir, Turkey. 6. Division of Pediatric Genetics, Department of Pediatrics, Akdeniz University, Antalya, Turkey. 7. Division of Endocrinology, Department of Internal Medicine, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey. 8. Division of Endocrinology, Diyarbakir Training Hospital, Diyarbakir, Turkey. 9. Department of Histology, Dokuz Eylul University, Izmir, Turkey. 10. Koc University School of Medicine, Istanbul, Turkey. 11. Division of Pediatric Genetics, Department of Pediatrics, Ege University, Izmir, Turkey. 12. Department of Radiology, Dokuz Eylul University, Izmir, Turkey. 13. Division of Endocrinology, Karabuk State Hospital, Karabuk, Turkey. 14. Division of Endocrinology, Kocaeli Training Hospital, Kocaeli, Turkey. 15. Division of Endocrinology, Department of Internal Medicine, Mersin University, Mersin, Turkey. 16. Division of Pediatric Endocrinology, Department of Pediatrics, Uludag University, Bursa, Turkey. 17. Division of Pediatric Neurology, Dr. Behcet Uz Children's Hospital, Izmir, Turkey. 18. Division of Pediatric Endocrinology, Dr. Sami Ulus Obstetrics and Gynecology, Children's Health and Disease Training and Research Hospital, Ankara, Turkey. 19. Division of Endocrinology, Department of Internal Medicine, Ataturk University, Erzurum, Turkey. 20. Division of Pediatric Endocrinology, Department of Pediatrics, Ege University, Izmir, Turkey. 21. Division of Nephrology, Department of Internal Medicine, Ege University, Izmir, Turkey. 22. Division of Endocrinology and Metabolism, Brehm Center for Diabetes Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA.
Abstract
OBJECTIVES: Lipodystrophy syndromes are a group of heterogeneous disorders characterized by adipose tissue loss. Proteinuria is a remarkable finding in previous reports. STUDY DESIGN: In this multicentre study, prospective follow-up data were collected from 103 subjects with non-HIV-associated lipodystrophy registered in the Turkish Lipodystrophy Study Group database to study renal complications in treatment naïve patients with lipodystrophy. METHODS: Main outcome measures included ascertainment of chronic kidney disease (CKD) by studying the level of proteinuria and the estimated glomerular filtration rate (eGFR). Kidney volume was measured. Percutaneous renal biopsies were performed in 9 patients. RESULTS: Seventeen of 37 patients with generalized and 29 of 66 patients with partial lipodystrophy had CKD characterized by proteinuria, of those 12 progressed to renal failure subsequently. The onset of renal complications was significantly earlier in patients with generalized lipodystrophy. Patients with CKD were older and more insulin resistant and had worse metabolic control. Increased kidney volume was associated with poor metabolic control and suppressed leptin levels. Renal biopsies revealed thickening of glomerular basal membranes, mesangial matrix abnormalities, podocyte injury, focal segmental sclerosis, ischaemic changes and tubular abnormalities at various levels. Lipid vacuoles were visualized in electron microscopy images. CONCLUSIONS: CKD is conspicuously frequent in patients with lipodystrophy which has an early onset. Renal involvement appears multifactorial. While poorly controlled diabetes caused by severe insulin resistance may drive the disease in some cases, inherent underlying genetic defects may also lead to cell autonomous mechanisms contributory to the pathogenesis of kidney disease.
OBJECTIVES:Lipodystrophy syndromes are a group of heterogeneous disorders characterized by adipose tissue loss. Proteinuria is a remarkable finding in previous reports. STUDY DESIGN: In this multicentre study, prospective follow-up data were collected from 103 subjects with non-HIV-associated lipodystrophy registered in the Turkish Lipodystrophy Study Group database to study renal complications in treatment naïve patients with lipodystrophy. METHODS: Main outcome measures included ascertainment of chronic kidney disease (CKD) by studying the level of proteinuria and the estimated glomerular filtration rate (eGFR). Kidney volume was measured. Percutaneous renal biopsies were performed in 9 patients. RESULTS: Seventeen of 37 patients with generalized and 29 of 66 patients with partial lipodystrophy had CKD characterized by proteinuria, of those 12 progressed to renal failure subsequently. The onset of renal complications was significantly earlier in patients with generalized lipodystrophy. Patients with CKD were older and more insulin resistant and had worse metabolic control. Increased kidney volume was associated with poor metabolic control and suppressed leptin levels. Renal biopsies revealed thickening of glomerular basal membranes, mesangial matrix abnormalities, podocyte injury, focal segmental sclerosis, ischaemic changes and tubular abnormalities at various levels. Lipid vacuoles were visualized in electron microscopy images. CONCLUSIONS: CKD is conspicuously frequent in patients with lipodystrophy which has an early onset. Renal involvement appears multifactorial. While poorly controlled diabetes caused by severe insulin resistance may drive the disease in some cases, inherent underlying genetic defects may also lead to cell autonomous mechanisms contributory to the pathogenesis of kidney disease.
Authors: Tomasz Klupa; Magdalena Szopa; Jan Skupien; Katarzyna Wojtyczek; Katarzyna Cyganek; Irina Kowalska; Maciej T Malecki Journal: Endocrine Date: 2009-10-27 Impact factor: 3.633
Authors: Katharine R Owen; Mollie Donohoe; Sian Ellard; Tom J Clarke; Anthony J Nicholls; Andrew T Hattersley; Coralie Bingham Journal: Nephron Clin Pract Date: 2004
Authors: Ho Lim Lee; Meryl A Waldman; Sungyoung Auh; James E Balow; Elaine K Cochran; Phillip Gorden; Rebecca J Brown Journal: J Clin Endocrinol Metab Date: 2019-04-16 Impact factor: 5.958