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

2018 ◽  
Vol 8 (3) ◽  
pp. 207-215 ◽  
Author(s):  
Jose Torrealba ◽  
Nathan T. Sweed ◽  
Daniel Burguete ◽  
Allen R. Hendricks
Keyword(s):  
Epithelial Cells ◽  
Wilson’S Disease ◽  
Case Reports ◽  
Kidney Injury ◽  
Wilson's Disease ◽  
Pathologic Finding ◽  
Severe Liver ◽  
Alcoholic Steatohepatitis ◽  
Cast Nephropathy ◽  
Wide Range

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.

scholarly journals Bile cast nephropathy is a common pathologic finding for kidney injury associated with severe liver dysfunction

2013 ◽  
Vol 84 (1) ◽  
pp. 192-197 ◽  
Author(s):  
Charles M. van Slambrouck ◽  
Fadi Salem ◽  
Shane M. Meehan ◽  
Anthony Chang
Keyword(s):  
Liver Dysfunction ◽  
Kidney Injury ◽  
Pathologic Finding ◽  
Severe Liver ◽  
Cast Nephropathy ◽  
Severe Liver Dysfunction

scholarly journals Parkinson’s Disease and Metal Storage Disorders: A Systematic Review

2018 ◽  
Vol 8 (11) ◽  
pp. 194 ◽  
Author(s):  
Edward Botsford ◽  
Jayan George ◽  
Ellen Buckley
Keyword(s):  
Systematic Review ◽  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Wilson’S Disease ◽  
Case Reports ◽  
Hereditary Hemochromatosis ◽  
Wilson's Disease ◽  
Neurological Dysfunction ◽  
Cross Sectional ◽  
Storage Disorders

Metal storage disorders (MSDs) are a set of rare inherited conditions with variable clinical pictures including neurological dysfunction. The objective of this study was, through a systematic review, to identify the prevalence of Parkinsonism in patients with MSDs in order to uncover novel pathways implemented in Parkinson’s disease. Human studies describing patients of any age with an MSD diagnosis were analysed. Foreign language publications as well as animal and cellular studies were excluded. Searches were conducted through PubMed and Ovid between April and September 2018. A total of 53 publications were identified including 43 case reports, nine cross-sectional studies, and one cohort study. The publication year ranged from 1981 to 2018. The most frequently identified MSDs were Pantothenate kinase-associated neurodegeneration (PKAN) with 11 papers describing Parkinsonism, Hereditary hemochromatosis (HH) (7 papers), and Wilson’s disease (6 papers). The mean ages of onset of Parkinsonism for these MSDs were 33, 53, and 48 years old, respectively. The Parkinsonian features described in the PKAN and HH patients were invariably atypical while the majority (4/6) of the Wilson’s disease papers had a typical picture. This paper has highlighted a relationship between MSDs and Parkinsonism. However, due to the low-level evidence identified, further research is required to better define what the relationship is.

scholarly journals THE DEPOSITION OF EXOGENOUS COPPER UNDER EXPERIMENTAL CONDITIONS WITH OBSERVATIONS ON ITS NEUROTOXIC AND NEPHROTOXIC PROPERTIES IN RELATION TO WILSON'S DISEASE

1959 ◽  
Vol 110 (5) ◽  
pp. 801-810 ◽  
Author(s):  
F. Stephen Vogel
Keyword(s):  
Epithelial Cells ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Renal Tubules ◽  
Human Beings ◽  
Experimental Conditions ◽  
Renal Epithelium ◽  
Central Nervous Systems ◽  
Parenchymal Cells ◽  
Large Neurons

Goldfish kept in water containing ionized copper and a detergent added with the aim of decreasing coagulation of the mucus on the gills, took in and retained this metal in their brains, livers, and kidneys, in concentrations comparable to those that occur naturally in Wilson's disease, as chemical assays disclosed. Histochemical studies made it clear that much copper had accumulated within the large neurons, principally in those of the telencephalon and anterior horn region of the spinal cord and in the tubular epithelial cells of the kidneys, the nuclei of the parenchymal cells of the liver, the sarcoplasm of the skeletal muscle, and in the epithelial covering of the gills. The intraneuronal deposition of copper was regularly associated after a time with conspicuous cytologic changes, notably contraction and hyperchromaticity of the nerve cells with tortuosity and fragmentation of the axis cylinders and lysis and loss of neurons. The accumulation of metal in the renal epithelium was frequently accompanied by necrosis and was regularly associated with hyperplasia and calcification of the epithelial cells of the larger renal tubules in all goldfish kept for prolonged periods in copper-rich water. The deposition of copper in the liver was not accompanied by consistent cytologic changes. The similarity of the cytologic alterations induced in the central nervous systems by copper and those that occur naturally in hepatolenticular degeneration in human beings provides evidence that copper itself plays an important role in the pathologic alterations of the brain in Wilson's disease.

HGV and fulminant Wilson's disease. Case reports

1998 ◽  
Vol 28 ◽  
pp. 229
Author(s):  
W. Kryczka ◽  
J. Kubicka ◽  
E. Brojer ◽  
P. Grabarczyk ◽  
B. Walewska-Zielecka ◽  
...  
Keyword(s):  
Wilson’S Disease ◽  
Case Reports ◽  
Wilson's Disease ◽  
Disease Case

Renal tubular injury as an uncommon presentation of Wilson’s disease

2021 ◽  
Vol 2 (3) ◽  
Author(s):  
Hadar Mudrik-Zohar ◽  
◽  
Keren Cohen-Hagai ◽  
Danny Alon ◽  
◽  
...  
Keyword(s):  
Acute Kidney Injury ◽  
Wilson’S Disease ◽  
Renal Involvement ◽  
Clinical Manifestations ◽  
Kidney Injury ◽  
Wilson's Disease ◽  
Tubular Dysfunction ◽  
Tubular Injury ◽  
Uncommon Presentation ◽  
Renal Tubular

Wilson's disease is an autosomal recessive disorder caused by a mutant ATP7B gene on chromosome 13. This mutation causes a reduction in hepatic copper excretion, which accumulates in hepatocytes and deposits in other tissues and organs (brain, cornea, kidney, etc.) as the disease progresses. Wilson's disease was described worldwide, with estimated prevalence of one case per 30,000 live births. Age of presentation is usually 4-60 years; however, the disease may present at any age. Clinical manifestations are predominantly hepatic, neurologic and psychiatric. Renal involvement is less common and characterized by proximal tubular dysfunction, Glomerular Filtration Rate (GFR) decline, renal tubular acidosis, aminoaciduria and nephrolithiasis. Herein, we aimed to report a case which describes an uncommon path to the diagnosis of this rare, yet well-known disease. The diagnosis resulted from an investigation of acute kidney injury in a 48-year-old man. Keywords: Wilson’s disease; Acute kidney injury; Renal tubular injury.

scholarly journals Acute Interstitial Nephritis With Karyomegalic Epithelial Cells After Nivolumab Treatment—Two Case Reports

2019 ◽  
Vol 12 ◽  
pp. 117954761985364 ◽  
Author(s):  
Masaki Ryuzaki ◽  
Hirobumi Tokuyama ◽  
Kiyotaka Uchiyama ◽  
Hideaki Nakaya ◽  
Kazuhiro Hasegawa ◽  
...  
Keyword(s):  
Renal Function ◽  
Epithelial Cells ◽  
Interstitial Nephritis ◽  
Checkpoint Inhibitors ◽  
Case Reports ◽  
Kidney Injury ◽  
Corticosteroid Treatment ◽  
Acute Interstitial Nephritis ◽  
Tubular Epithelial Cells ◽  
Ki 67

Clinical application of immune checkpoint inhibitors (CPIs) including nivolumab is expanding in the field of oncology treatment. Nivolumab is an anti-programmed death 1 protein (PD-1) antibody designed to augment an immunologic reaction against cancer cells. On the contrary, CPIs are known to cause a unique variety of side effects termed as immune-related adverse events, which can affect any organ including kidney. However, the characteristics of renal disorders by nivolumab treatment are poorly described. We describe two cases of acute kidney injury that were treated with nivolumab. Two patients, one with renal-cell carcinoma and the other with lung cancer, exhibited progressive renal dysfunction after the initiation of nivolumab treatment. By kidney biopsy, each case was diagnosed as acute interstitial nephritis (AIN). Of note, tubular epithelial cells enlarged with hyperchromatic nuclei were focally observed, and this finding was consistent with karyomegalic tubular epithelial cells. In immunostaining, most of the enlarged tubular epithelial cells were positive for Ki-67, which suggested regeneration of tubular epithelial cells. Clinically, in one case, renal function was partially recovered with the discontinuation of nivolumab, while in another case renal function was fully recovered with additional corticosteroid treatment. We presented nivolumab-induced AIN with karyomegalic changes of tubular epithelia. We propose that immunosuppressive therapy may be necessary for the full recovery from renal impairment.

scholarly journals Emergent living related liver transplantation for a patient with severe liver failure due to Wilson's disease

Kanzo ◽  
2004 ◽  
Vol 45 (9) ◽  
pp. 473-478 ◽  
Author(s):  
Masato KATO ◽  
Keiichi KUBOTA ◽  
Junnji KITA ◽  
Mitsugi SIMODA ◽  
Takehiko NEMOTO ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Liver Failure ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Severe Liver ◽  
Living Related Liver Transplantation ◽  
Living Related
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