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揭示肾脂质沉积:一例罕见的肝移植后类似卵磷脂胆固醇酰基转移酶(LCAT)缺乏症的肝肾小球硬化病例。

Unveiling Renal Lipid Deposition: A Rare Case of Hepatic Glomerulosclerosis Resembling Lecithin-Cholesterol Acyltransferase (LCAT) Deficiency Post Liver Transplantation.

作者信息

Zghayer Aseel, Borys Ewa, Picken Maria M, Vellanki Kavitha

机构信息

Department of Nephrology, Loyola University Medical Center, Maywood, USA.

Department of Pathology, Loyola University Medical Center, Maywood, USA.

出版信息

Cureus. 2024 Jul 7;16(7):e64004. doi: 10.7759/cureus.64004. eCollection 2024 Jul.

Abstract

Hepatic glomerulosclerosis, a renal complication of liver cirrhosis, presents challenges in diagnosis and management. This case report discusses the rarity of kidney biopsy findings resembling lecithin-cholesterol acyltransferase (LCAT) deficiency post liver transplantation. We present the case of a patient with end-stage liver disease (ESLD) from alcohol-related cirrhosis, who underwent orthotopic liver transplantation (OLT) with persistent proteinuria after transplantation. Kidney biopsy revealed features of hepatic glomerulopathy resembling both IgA nephropathy (IgAN) and LCAT deficiency. The histopathological similarities between hepatic glomerulosclerosis and LCAT deficiency suggest a potential link between liver disease and lipid deposition in the kidneys. The clinical course and outcomes of such renal alterations post liver transplantation remain uncertain, highlighting the need for further research in glomerular pathology in the context of liver transplantation. This case underscores the importance of kidney biopsy in ESLD patients and the necessity for more attention to glomerular pathology post liver transplantation, especially in the current era of increasing liver transplantation rates.

摘要

肝性肾小球硬化症是肝硬化的一种肾脏并发症,在诊断和管理方面存在挑战。本病例报告讨论了肝移植后肾活检结果类似于卵磷脂胆固醇酰基转移酶(LCAT)缺乏症的罕见情况。我们报告了一例因酒精性肝硬化导致终末期肝病(ESLD)的患者,该患者接受了原位肝移植(OLT),移植后持续存在蛋白尿。肾活检显示出类似于IgA肾病(IgAN)和LCAT缺乏症的肝性肾小球病特征。肝性肾小球硬化症与LCAT缺乏症之间的组织病理学相似性表明肝脏疾病与肾脏脂质沉积之间可能存在联系。肝移植后这种肾脏改变的临床过程和结果仍不确定,这凸显了在肝移植背景下对肾小球病理学进行进一步研究的必要性。本病例强调了肾活检在ESLD患者中的重要性,以及肝移植后更多关注肾小球病理学的必要性,尤其是在当前肝移植率不断上升的时代。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e6b8/11304406/c6d769b10ea8/cureus-0016-00000064004-i01.jpg

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