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胆汁管型肾病:一种在患有酒精性脂肪性肝炎的成人和患有威尔逊病的儿童中出现的、具有多种病因的病理表现。

Bile Cast Nephropathy: A Pathologic Finding with Manifold Causes Displayed in an Adult with Alcoholic Steatohepatitis and in a Child with Wilson's Disease.

作者信息

Torrealba Jose, Sweed Nathan T, Burguete Daniel, Hendricks Allen R

机构信息

Department of Pathology, University of Texas Southwestern Medical Center, Dallas, Texas, USA.

University of Texas Southwestern Medical School, Dallas, Texas, USA.

出版信息

Case Rep Nephrol Dial. 2018 Sep 27;8(3):207-215. doi: 10.1159/000493231. eCollection 2018 Sep-Dec.

Abstract

Bile cast nephropathy (BCN) is seen in patients who have acute kidney injury and severe hyperbilirubinemia due to a wide range of hepatobiliary system diseases. Findings seen by renal biopsy include acute tubular injury with necrotic and sloughed epithelial cells, yellow-green pigment within tubular epithelial cells, and pigmented granular casts. Hall's special stain for bile turns these casts green. In recent years, BCN has been described in a small number of case reports and clinical studies primarily in the setting of severe liver dysfunction. We present 2 diverse cases of BCN. The first involves an adult with hepatorenal syndrome secondary to alcoholic steatohepatitis and early cirrhosis. Second, we describe the first reported case of BCN in a child with fulminant hepatic failure due to Wilson's disease. Our cases expand the spectrum of causative diseases, and they provide further evidence that BCN is a distinct pathologic entity which may be found in both adult and pediatric patients with a variety of severe liver diseases.

摘要

胆汁管型肾病(BCN)见于因多种肝胆系统疾病导致急性肾损伤和严重高胆红素血症的患者。肾活检所见包括急性肾小管损伤,伴有坏死和脱落的上皮细胞、肾小管上皮细胞内的黄绿色素以及色素颗粒管型。Hall氏胆汁特殊染色可使这些管型变为绿色。近年来,主要在严重肝功能不全的情况下,有少数病例报告和临床研究描述了BCN。我们展示了2例不同的BCN病例。第一例涉及一名继发于酒精性脂肪性肝炎和早期肝硬化的肝肾综合征成人患者。第二例,我们描述了首例因威尔逊病导致暴发性肝衰竭的儿童BCN病例。我们的病例拓宽了致病疾病谱,并进一步证明BCN是一种独特的病理实体,可在患有各种严重肝病的成人和儿童患者中发现。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f1de/6206956/0ee97671803a/cnd-0008-0207-g01.jpg

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