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• W4283009970 abstract "IntroductionGeneralized congenital lipodystrophy [GCL] is a pathological condition of autosomal recessive transmission characterized by a severe reduction of adipose tissue, mainly caused by alterations in the AGPAT2 gene. It is associated with the development of severe insulin resistance, secondary to ectopic lipid deposition and the absence of adipokines, determining the development of hypertriglyceridemia, hepatic steatosis, and diabetes. In addition, this condition has been associated with the presence of proteinuria and renal pathologies, including focal segmental glomerulosclerosis and membranoproliferative glomerulonephritis.Kidney Disease [KD] is a very common complication in patients with GCL and usually initially corresponds to proteinuric nephropathy, often in the nephrotic range, in which the diabetic etiology is not common, and its causality is usually unknown. It has been described that in these patients both the presence of proteinuria and the decrease in glomerular filtration can be significantly reversed with leptin supplementation, suggesting a direct increase effect of this adipokine on renal function.MethodsClinical Case: Female patient, 38 years old, diagnosed with GCL, Diabetes mellitus [DM] since she was 12 years old, Hypertension [HT] 5 years ago, mixed dyslipidemia and anemia. In treatment with recombinant Leptin for 5 years and pharmacotherapy for the management of secondary pathologies.ResultsShe has presented proteinuria for the last 10 years, which initially improved with the administration of recombinant leptin, but has remained permanently high. In 2017, a renal biopsy was performed, observing findings consistent with diffuse and nodular diabetic glomerulosclerosis, presenting 75% renal function. Currently, she has proteinuria 3.84 g/24 hrs and GFR 32 ml/min/1.73m2 (Stage III Chronic Kidney Disease [CKD]) and has presented pictures of hyperkalemia secondary to type IV tubular acidosis.ConclusionsThe first case of CKD of glomerulosclerotic etiology, secondary to DM in this basal condition of CGL, is reported, where it is observed that treatment with leptin has not been sufficient to stop or reduce the progression of KD. Therefore, it is suggested to use combined strategies such as a low-protein or plant-based diet and a strict adjustment of hypotensive drug therapy to promote renoprotection.No conflict of interest IntroductionGeneralized congenital lipodystrophy [GCL] is a pathological condition of autosomal recessive transmission characterized by a severe reduction of adipose tissue, mainly caused by alterations in the AGPAT2 gene. It is associated with the development of severe insulin resistance, secondary to ectopic lipid deposition and the absence of adipokines, determining the development of hypertriglyceridemia, hepatic steatosis, and diabetes. In addition, this condition has been associated with the presence of proteinuria and renal pathologies, including focal segmental glomerulosclerosis and membranoproliferative glomerulonephritis.Kidney Disease [KD] is a very common complication in patients with GCL and usually initially corresponds to proteinuric nephropathy, often in the nephrotic range, in which the diabetic etiology is not common, and its causality is usually unknown. It has been described that in these patients both the presence of proteinuria and the decrease in glomerular filtration can be significantly reversed with leptin supplementation, suggesting a direct increase effect of this adipokine on renal function." @default.
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• W4283009970 date "2022-06-01" @default.
• W4283009970 modified "2023-09-30" @default.
• W4283009970 title "POS-043 DEVELOPMENT AND MANIFESTATION OF CHRONIC KIDNEY DISEASE IN GENERALIZED CONGENITAL LIPODYSTROPHY: REPORT OF A CHILEAN CASE AND REVIEW OF THE LITERATURE" @default.
• W4283009970 doi "https://doi.org/10.1016/j.ekir.2022.04.065" @default.
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