Bile Cast Nephropathy Research Articles

Kidney involvement in Wilson's disease: a review of the literature.

Wilson's disease (WD) is a rare inherited disease due to the mutation of the ATP7B gene, resulting in impaired hepatic copper excretion and its pathological accumulation in various organs such as the liver, the nervous system, or the kidneys. Whereas liver failure and neuropsychiatric disorders are the most common features, less is known about the renal complications. We conducted a review of the literature to define the characteristics and pathophysiology of kidney involvement during WD. This review shed light on strong evidence for direct copper toxicity to renal tubular cells. Excessive tubular copper accumulation might present with various degrees of tubular dysfunction, ranging from mild hydroelectrolytic and acid-base disorders to complete Fanconi syndrome. Proximal and distal renal tubular acidosis also favors development of nephrolithiasis, nephrocalcinosis, and bone metabolism abnormalities. Indirect complications might involve renal hypoperfusion as occurs in hepatorenal or cardiorenal syndrome, but also tubular casts' formation during acute hemolysis, rhabdomyolysis, or bile cast nephropathy. Acute kidney failure is not uncommon in severe WD patients, and independently increases mortality. Finally, specific and long-term therapy by D-penicillamin, one of the most efficient drugs in WD, can cause glomerular injuries, such as membranous nephropathy, minimal-change disease, and, rarely, severe glomerulonephritis. Altogether, our study supports the need for interdisciplinary evaluation of WD patients involving nephrologists, with regular monitoring of tubular and glomerular functions, to provide adequate prevention of renal and bone involvement.

A Case Series of Bile Cast Nephropathy-induced Rapidly Progressive Renal Failure

Abstract A 45-year-old male presented to the hospital with symptoms of left inguinal swelling, which showed granulomatous adenitis on fine-needle aspiration cytology. He was diagnosed with tuberculous adenitis, for which he was started on antitubercular treatment. After 15 days of antitubercular treatment, the patient developed deranged renal parameters, with serum creatinine of 8.8 mg/dL while serum bilirubin rose to 7.4 mg/dL. The patient was oliguric and underwent four sessions of hemodialysis. Following this, a renal biopsy was done, which showed normal glomeruli with tubular casts that were pigmented, consistent with acute tubular injury secondary to bile cast nephropathy. The patient’s condition improved with one more session of hemodialysis and conservative management. Serum creatinine declined to 1.2 mg/dL, at the time of discharge, with normal urine output. This case illustrates features of bile cast nephropathy presenting as rapidly progressive renal failure secondary to antitubercular treatment.

A Case of Bile Cast Nephropathy Treated with Plasma Exchange Therapy for AKI Associated with Acute Hepatitis A

A Case of Bile Cast Nephropathy Treated with Plasma Exchange Therapy for AKI Associated with Acute Hepatitis A

Bile cast nephropathy; a neglected entity

This study investigates the role of bile cast nephropathy (BCN) in acute kidney injury associated with cholestatic liver disease. Bile cast nephropathy is characterized by kidney injury due to, bile-related factors, distinct from hepatorenal syndrome (HRS) linked to hemodynamic changes in liver disease. The mechanisms of BCN include oxidative damage, tubular toxicity, and obstructive physiology. Diagnosis is typically through biopsy, although alternatives like trans-jugular biopsy are considered due to bleeding risks. Treatment targets underlying causes of hyperbilirubinemia, and extracorporeal therapies like plasmapheresis and molecular adsorbent recycling system show potential efficacy. Awareness and further research on noninvasive diagnostic methods for BCN are recommended.

69 Prolonged Bile Cast Nephropathy Following Orthotopic Liver Transplant, a Diagnostic and Management Challenge

69 Prolonged Bile Cast Nephropathy Following Orthotopic Liver Transplant, a Diagnostic and Management Challenge

Bile Cast Nephropathy, An Often-Missed Diagnosis.

Bile Cast Nephropathy, An Often-Missed Diagnosis.

Clinically Diagnosed Bile Cast Nephropathy in a Patient With Severe Alcoholic Hepatitis and COVID-19 Pneumonia

Clinically Diagnosed Bile Cast Nephropathy in a Patient With Severe Alcoholic Hepatitis and COVID-19 Pneumonia

Bile Cast Nephropathy in a Child With Acute Viral Hepatitis A Infection: A Case Report

Bile Cast Nephropathy in a Child With Acute Viral Hepatitis A Infection: A Case Report

Renal Dysfunction in Patients with Liver Cirrhosis

AbstractLiver cirrhosis is a major health problem that can affect people of different ages. It induces pivotal hemodynamic and metabolic systemic disturbances along with other organs dysfunction. Renal dysfunction in cirrhotic patients is not uncommon, and subtle renal impairment is an early and very frequent finding. Liver cirrhosis can afflict kidney functions through different mechanisms. Renal vasoconstriction is usually the initial response of splanchnic vasodilation and decreased effective renal plasma flow. This induces a reduction of intraglomerular pressure leading to stimulation of renin-angiotensin system to maintain the glomerular filtration rate. Other causes of renal dysfunction include electrolytes and acid-base disturbances, systemic inflammation, bile cast nephropathy, and intra-abdominal hypertension. Loss of renal reserve is usually the earliest manifestation of kidney dysfunction in cirrhotic patients. This makes the kidney supersensitive to any subsequent hemodynamic or metabolic abnormalities. Proper assessment of kidney function is one of the major challenges in cirrhotic patients. The use of serum creatinine and creatinine-based equations is inaccurate and can overestimate kidney function. Hepato-renal syndrome (HRS) is a life-threatening disorder. In the last decade, there was significant progress in understanding the mechanism of this mysterious disorder. In this article, we are focusing on different mechanisms of kidney dysfunction in cirrhotic patients and the major diagnostic and therapeutic challenges.

Bile Cast Nephropathy: A Comprehensive Review.

Bile cast nephropathy (BCN) or cholemic nephropathy (CN) is an acute renal dysfunction, including acute kidney injury (AKI) in the setting of liver injury. It is a common phenomenon in patients with liver disease and is associated with significant morbidity and mortality. CN is characterized by hemodynamic changes in the liver, kidney, systemic circulation, intratubular cast formation, and tubular epithelial cell injury. CN has been overlooked as a differential diagnosis in chronic liver disease patients due to more importance to hepatic injury. However, frequent and considerable reporting of case reports recently has further investigated this topic in the last two decades. This review determines the evidence behind the potential role of bile acids and bilirubin in acute renal dysfunction in liver injury, summarizing the implied pathophysiology risk factors, and incorporating the therapeutic mechanisms and outcomes.

Bile cast nephropathy

Bile cast nephropathy

A Donor With Green Kidneys—To Transplant or Not to Transplant: A Case Report

A Donor With Green Kidneys—To Transplant or Not to Transplant: A Case Report

Cholemic Nephrosis: An Autopsy Study of a Forgotten Entity.

The aim of the study is to do a clinicopathologic study of post mortem kidney biopsies with significant deposition of bilirubin pigment within tubular epithelial cells and in the lumen of distal tubules as a bile cast. All post mortem specimens with acute tubular necrosis, with the presence of bile casts in tubules or bile pigment deposition in the tubular epithelium during the period 2015-2018 were examined for gross and histopathology along with biochemical parameters and viral markers. Bile casts with sloughed renal tubular epithelial cells and occasional macrophages were present in the distal convoluted tubule in 78.6% of biopsies (11/14). The plugging of distal convoluted tubule with casts was similar to that seen in myeloma and myoglobin cast nephropathies. Bilirubin pigment deposition was present in 35.7% (5/14) of cases. The frequency of bile casts in each biopsy was variable and it did not have any association with serum bilirubin levels or etiology of liver dysfunction. A striking difference from earlier studies is the high number of toxin-induced liver damage including six cases of paraquat and 2 cases of yellow phosphorus poisoning. This study proves importance of the bile cast nephropathy as a reason for kidney injury, especially with varied hepatotoxic etiologies, especially paraquat and yellow phosphorus.

S2580 Plasmapharesis for Hyperbilirubinemia: A Bridge to Transplant

INTRODUCTION: Plasma exchange (PE) has been used to successfully treat hyperbilirubinemia in conditions such as primary biliary cholangitis. However, it is not routinely used to prevent worsening renal function due to bile cast nephropathy, as a bridge to liver transplant. We present a case of using PE to treat severe hyperbilirubinemia in a patient with acute on chronic liver failure prior to orthotopic liver transplantation (OLT). CASE DESCRIPTION/METHODS: This is a 62 year old woman with a past medical of NASH cirrhosis, esophageal varices, hepatic encephalopathy, and factor V leiden deficiency who presented to our institution for management of worsening liver function. On admission, her total bilirubin was 29.3mg/dl (baseline was around 5mg/dl), direct bilirubin 18mg/dl and MELD was 21. Hemolysis workup was negative. She had completed a course of azithromycin for pneumonia, raising concern for drug induced liver injury. MRI abdomen revealed portal vein thrombosis, splenomegaly, portal enteropathy, ascites and no biliary ductal dilation. Her course was complicated by worsening bilirubin up to 70mg/dl and subsequent worsening kidney function with a creatinine of 3.90 mg/dl concerning for cholestatic nephrosis. She was transferred to the intensive care unit where she received two sessions of PE. After PE her bilirubin improved to 40 mg/dl, her creatinine improved to 1.39 mg/dl and urine output improved. Patient was re-MELDed at 40. She successfully underwent OLT ten days after her last PE session. Post-OLT, her bilirubin and creatinine normalized. DISCUSSION: While we are transplanting at higher MELD scores, we see patients with severe hyperbilirubinemia, which can cause significant complications, including bile cast nephropathy. Cholemic nephrosis can cause renal injury from proximal tubulopathy to intrarenal bile cast formation. PE has been used to successfully treat hyperbilirubinemia in few cases of fulminant hepatic failure not amendable to OLT in an attempt to allow hepatocyte recovery and regeneration. However, it is not routinely used to treat hyperbilirubinemia in liver failure as a bridge to transplant. Preserving renal function is important for a successful post-transplant course. Our case is one of the few cases in the literature where PE was used as a bridge prior to OLT in patients with hyperbilirubinemia in the setting of acute on chronic liver failure. Hence we conclude that PE can be considered to treat hyperbilirubinemia to prevent renal failure as a bridge to transplant.

The Successful Treatment of Bile Cast Nephropathy with Plasma Exchange

The Successful Treatment of Bile Cast Nephropathy with Plasma Exchange

Role of inflammation and inflammasome activation in human bile cast nephropathy

Bile cast nephropathy (BCN) is an underdiagnosed cause of acute kidney injury (AKI). The precise pathogenesis of bilirubin tubular toxicity remains unknown. The aim of this study is to explore the cellular and molecular pathophysiology of human BCN. Paraffin-embedded sections of renal biopsy tissue from a BCN patient were stained by immunohistochemistry (IHC) for oxidative stress (4-hydroxynonenal), immune cell subpopulations, including dendritic cells (CD1c), macrophages (CD68) and T cells (CD3), and inflammasome activation by staining for active-caspase-1 and the inflammasome adaptor protein, ASC (apoptosis-associated speck-like protein containing a caspase activation and recruitment domain). Quantitative analyses of IHC staining were compared to healthy renal cortical tissue. We identified yellow to brown granular casts within the BCN case, consistent with the presence of bile pigment. The presence of bile pigment was associated with strong tubular 4-hydroxynonenal staining intensity, a marker of oxidative stress. Diffuse tubulointerstitial inflammatory cell infiltrate was detected, with elevated CD1c, CD68 and CD3 staining. Foci of inflammasome activity were co-localized with this intense immune cell infiltration, with increased active-caspase-1 and ASC staining. Our findings are the first to suggest that bile casts may lead to oxidative stress and trigger the inflammasome signalling cascade, leading to interstitial inflammation and driving AKI pathobiology. SUMMARY AT A GLANCE The report suggests that bile casts may lead to oxidative stress and trigger the inflammasome signalling cascade, leading to interstitial inflammation and driving bile cast nephropathy pathobiology.

Medical renal diseases are frequent but often unrecognized in adult autopsies

Kidney diseases affect many hospitalized patients and contribute to morbidity and mortality. Therefore, kidney disease should be prevalent, but the frequency and spectrum of medical renal pathology in autopsy specimens has not been well documented. We sought to determine the spectrum of medical renal pathology in adult autopsy specimens and the frequency of overlooked diagnoses. We reviewed the hematoxylin- and eosin-stained kidney sections from 140 adult autopsies performed at a large teaching hospital over a 2-year period. Fifty-eight cases (41%) had findings warranting further analysis, including alterations in glomerular matrix and/or cellularity, atypical or pigmented casts, thrombi, tubulointerstitial or vascular inflammation, or deposition of amorphous material. After additional studies and clinical correlation, the pathologic changes in 43 cases (31%) were categorized as follows: diabetic nephropathy, bile cast nephropathy, thrombotic microangiopathy, infection-related glomerulonephritis, focal necrotizing/crescentic glomerulonephritis, oxalate nephropathy, light-chain cast nephropathy, amyloidosis, urate nephropathy, hemosiderosis, antineutrophil cytoplasmic antibody-associated vasculitis, polyoma virus nephropathy, atheroembolic disease, and nephrocalcinosis. These diagnoses were not reported in 26 (60%) cases during the initial autopsy evaluation. This study demonstrates that medical renal diseases are common in autopsy cases, but significant diagnoses can be easily overlooked. Autopsy kidney specimens are a rich source of renal pathology and their evaluation should be emphasized in anatomic pathology residency training. Ultimately, our understanding of how kidney disease contributes to morbidity and mortality will benefit from accurate recognition of renal pathology in autopsy specimens.

Bile Cast Nephropathy Caused by Obstructive Pancreatic Carcinoma and Failed ERCP

Bile Cast Nephropathy Caused by Obstructive Pancreatic Carcinoma and Failed ERCP

Bile Cast Nephropathy Caused by Obstructive Pancreatic Carcinoma and Failed ERCP

Bile Cast Nephropathy Caused by Obstructive Pancreatic Carcinoma and Failed ERCP

Bile Cast Nephropathy in Patients with Acute Kidney Injury Due to Hepatorenal Syndrome: A Postmortem Kidney Biopsy Study.

Background and Aims: The role of bile cast nephropathy (BCN) in pathogenesis of hepatorenal syndrome (HRS) in decompensated cirrhosis and acute on chronic liver failure (ACLF) is unknown. This study aimed to determine the frequency of BCN detected on postmortem renal biopsy among patients with decompensated cirrhosis and ACLF who had been admitted with acute kidney injury due to HRS (HRA-AKI) and expired during that hospitalization.Methods: One-hundred-twenty-seven postmortem renal biopsies with adequate size (>1 cm in length) were included for analysis. These were obtained from 84 patients with decompensated cirrhosis and 43 patients with ACLF.Results: BCN was detected in 57 of the total 127 (44.8%) renal biopsy specimens. Patients with BCN had significantly higher levels of serum total bilirubin, total leukocyte count and model for end-stage liver disease score, as compared to those without BCN. BCN was detected in 32/43 (74.4%) of the patients with ACLF, as compared to 25/84 (29.7%) of the patients with decompensated cirrhosis (p < 0.001). On multivariate analysis, direct bilirubin (OR (95% CI): 1.27 (1121–1.698); p < 0.001) and presence of ACLF (OR (95% CI): 2.603 (1.054–7.111); p = 0.041) were found to be significant predictors of BCN on postmortem renal biopsy.Conclusion: BCN was found in 72.1% of patients with ACLF and 27.4% patients with decompensated cirrhosis who had been hospitalized with an admitting diagnosis of HRS-AKI and who expired during that hospitalization and underwent postmortem renal biopsy. Direct serum bilirubin and presence of ACLF were found to be significant predictors of BCN on postmortem renal biopsy.