The DOI matches its Crossref record; 38 of the 38 references it cites were found in a registry or an index.
Proteinuric Nephropathy in Acquired and Congenital Generalized Lipodystrophy: Baseline Characteristics and Course during Recombinant Leptin Therapy
Abstract
Generalized lipodystrophy is characterized by adipose tissue absence, hypoleptinemia, hypertriglyceridemia, insulin resistance, diabetes, hepatomegaly, and nonalcoholic steatohepatitis. In the course of recruiting patients for treatment with recombinant leptin, we were struck by the frequency and severity of proteinuria. We evaluated 25 patients with generalized lipodystrophy. Eighteen were treated with recombinant leptin, and we have followed 15 on leptin for 4-36 months. We followed renal parameters at baseline and during follow-up visits. Renal biopsies were performed as clinically indicated. At baseline, 22 of 25 patients (88%) had elevated urine albumin excretion (>30 mg/24 h), 15 (60%) had macroalbuminuria (>300 mg/24 h), and five (20%) had nephrotic-range proteinuria (>3500 mg/24 h). Twenty-three (92%) had elevated creatinine clearance (>125 ml/min.1.73 m(2)). Eleven of 15 patients (73%) treated with recombinant leptin exhibited reduction in proteinuria, associated with reduction of hyperfiltration. Four patients who did not improve are discussed individually. Renal biopsy findings were remarkable for focal segmental glomerulosclerosis in four patients, membranoproliferative glomerulonephritis in two patients, and diabetic nephropathy in one patient. In conclusion, generalized lipodystrophy is associated with proteinuria and unique renal pathologies, including focal segmental glomerulosclerosis and membranoproliferative glomerulonephritis. The majority treated with recombinant leptin demonstrated reduction in proteinuria and hyperfiltration.
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“A notable observation during this protocol was the prevalence of proteinuria and unique nephropathies of these patients (13).”
From Long-Term Efficacy of Leptin Replacement in Patients With Generalized Lipodystrophy (2005) · cites this paper as [13]
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Cites
Effects of Recombinant Leptin Therapy in a Child with Congenital Leptin Deficiency
Farooqi, I. S., Jebb, S. A., Langmack, G., Lawrence, E., Cheetham, C. H., Prentice, A. M., Hughes, I. A., McCamish, M. A., & O'Rahilly, S. (1999) · New England Journal of Medicine · doi:10.1056/nejm199909163411204 · Cited by 1,998
Self-citationHeymsfield, S. B., Greenberg, A. S., Fujioka, K., Dixon, R. M., Kushner, R., Hunt, T., Lubina, J. A., Patane, J., Self, B., Hunt, P., et al. (1999) · JAMA · doi:10.1001/jama.282.16.1568 · Cited by 1,422
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Self-citationLeptin-Replacement Therapy for Lipodystrophy
Oral, E. A., Simha, V., Ruiz, E., Andewelt, A., Premkumar, A., Snell, P., Wagner, A. J., DePaoli, A. M., Reitman, M. L., Taylor, S. I., et al. (2002) · New England Journal of Medicine · doi:10.1056/nejmoa012437 · Cited by 1,247
Self-citationLeptin reverses insulin resistance and hepatic steatosis in patients with severe lipodystrophy
Petersen, K. F., Oral, E. A., Dufour, S., Befroy, D., Ariyan, C., Yu, C., Cline, G. W., DePaoli, A. M., Taylor, S. I., Gorden, P., et al. (2002) · Journal of Clinical Investigation · doi:10.1172/jci0215001 · Cited by 800
Self-citationIdentification of the gene altered in Berardinelli–Seip congenital lipodystrophy on chromosome 11q13
Magré, J., Délepine, M., Khallouf, E., Gedde‐Dahl, T., Maldergem, L. V., Sobel, E. M., Papp, J. C., Meier, M., Mégarbané, A., BSCL Working Group, et al. (2001) · Nature Genetics · doi:10.1038/ng585 · Cited by 755
AGPAT2 is mutated in congenital generalized lipodystrophy linked to chromosome 9q34
Agarwal, A. K., Oral, E. A., de Almeida, S., Akkoç, N., Taylor, S. I., Bowcock, A. M., Barnes, R. I., & Garg, A. (2002) · Nature Genetics · doi:10.1038/ng880 · Cited by 527
Self-citationRadioimmunoassay of leptin in human plasma
Ma, Z., Gingerich, R. L., Santiago, J. V., Klein, S., Smith, C. H., & Landt, M. (1996) · Clinical Chemistry · doi:10.1093/clinchem/42.6.942 · Cited by 490
Wolf, G., Hamann, A., Han, D. C., Helmchen, U. M., Thaiss, F., Ziyadeh, F. N., & Stahl, R. A. K. (1999) · Kidney International · doi:10.1046/j.1523-1755.1999.00626.x · Cited by 391
Cited by
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Inflammation and the pathogenesis of diabetic nephropathy
Wada, J., & Makino, H. (2012) · Clinical Science · doi:10.1042/cs20120198 · Cited by 793
The Diagnosis and Management of Lipodystrophy Syndromes: A Multi-Society Practice Guideline
Brown, R. J., Araújo‐Vilar, D., Cheung, P. T., Dunger, D. B., Garg, A., Jack, M., Mungai, L., Oral, E. A., Patni, N., Rother, K. I., et al. (2016) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2016-2466 · Cited by 540
Self-citationWang, Z., Jiang, T., Li, J., Proctor, G., McManaman, J. L., Lucia, S. M., Chua, S. C., & Levi, M. (2005) · Diabetes · doi:10.2337/diabetes.54.8.2328 · Cited by 302
Proctor, G., Jiang, T., Iwahashi, M., Wang, Z., Li, J., & Levi, M. (2006) · Diabetes · doi:10.2337/db05-0603 · Cited by 303
Congenital generalized lipodystrophies—new insights into metabolic dysfunction
Patni, N., & Garg, A. (2015) · Nature Reviews Endocrinology · doi:10.1038/nrendo.2015.123 · Cited by 262
Metadata
- Authors
- Javor, E. D., Moran, S. A., Young, J. R., Cochran, E. K., DePaoli, A. M., Oral, E. A., Turman, M. A., Blackett, P. R., Savage, D. B., O’Rahilly, S., Balow, J. E., & Gorden, P.
- Affiliations
- National Institutes of Health (US)
- Amgen (United States) (US)
- University of Oklahoma Health Sciences Center (US)
- University of Cambridge (GB)
- Corresponding author
- Javor, Edward D.
- Venue
- The Journal of Clinical Endocrinology & Metabolism
- Publisher
- The Endocrine Society
- Published
- 2004-07
- Volume
- 89
- Issue
- 7
- Pages
- 3199-3207
- ISSN
- 0021-972X (ISSN-L)
- 1945-7197
- OpenAlex
- W2113142131
- Cited by
- 114
- References
- 38
- Citations by year
- 2026: 4 · 2025: 4 · 2024: 5 · 2023: 4 · 2022: 5 · 2021: 5 · 2020: 6 · 2019: 8 · 2018: 8 · 2017: 4 · 2016: 5 · 2015: 10 · 2014: 8 · 2013: 3 · 2012: 5
- FWCI
- 2.90
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- The Journal of Clinical Endocrinology & Metabolism (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Nephrology › Renal Diseases and Glomerulopathies
- Health Sciences › Medicine › Nephrology › Chronic Kidney Disease and Diabetes
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adipokines, Inflammation, and Metabolic Diseases
- Keywords
- generalized lipodystrophy, proteinuria, nephrotic-range proteinuria, macroalbuminuria, leptin deficiency, hyperleptinemia, focal segmental glomerulosclerosis, membranoproliferative glomerulonephritis, diabetic nephropathy, glomerular hyperfiltration, renal biopsy findings, congenital lipodystrophy, acquired lipodystrophy
- MeSH terms
- Adolescent, Adult, Aged, Biopsy, Child, Creatinine, Diabetic Nephropathies, Female, Glomerulosclerosis, Focal Segmental, Humans, Kidney, Kidney Diseases, Lipodystrophy, Male, Middle Aged, Proteinuria, Recombinant Proteins, Syndrome, Glomerulonephritis, Membranoproliferative, Leptin
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