Bile cast nephropathy after liver transplantation

Bile cast nephropathy is a rare condition of renal dysfunction related to hyperbilirubinemia. The kidney injury is generally reversible if bilirubin levels are decreased early. Pathohistological findings are a result of bile casts formation and direct bile acids toxicity on renal tissue. Herein, we present a case of bile cast nephropathy after liver transplantation. A 60-year old male underwent liver transplantation (LT) in 2016 due to cryptogenic cirrhosis. Seven months after LT his liver function unexpectedly deteriorated and he developed severe jaundice (bilirubin=415 μmol/L) accompanied by acute renal failure (creatinine= 190μmol/L). A liver biopsy revealed an acute injury of unidentified aetiology, while the renal findings indicated bile cast nephropathy with nephroangiosclerosis and fibrosis. The treatment included plasmapheresis which resulted in partial recovery of renal and liver function. However, in proceeding weeks patient’s liver function deteriorated (bilirubin=501 μmol/L; AST=267IU/L; ALT=503IU/L), and due to unidentified liver failure, the patient was retransplanted. His renal function partially improved with resolving hyperbilirubinemia following LT (creatinine=145μmol/L). He was discharged from the hospital with normally functioning graft and improved but still impaired kidney function. There are only a few reported cases of this condition described in the literature and aetiology of this condition is still not completely understood. The presented case report demonstrates the particular background of bile cast nephropathy and the subsequent treatment. Though, we emphasize the need for further investigations of this condition, as well as the established treatment guidelines

Bile cast nephropathy after liver transplantation

Bile cast nephropathy is a rare condition of renal dysfunction related to hyperbilirubinemia. The kidney injury is generally reversible if bilirubin levels are decreased early. Pathohistological findings are a result of bile casts formation and direct bile acids toxicity on renal tissue. Herein, we present a case of bile cast nephropathy after liver transplantation. A 60-year old male underwent liver transplantation (LT) in 2016 due to cryptogenic cirrhosis. Seven months after LT his liver function unexpectedly deteriorated and he developed severe jaundice (bilirubin=415 μmol/L) accompanied by acute renal failure (creatinine= 190μmol/L). A liver biopsy revealed an acute injury of unidentified aetiology, while the renal findings indicated bile cast nephropathy with nephroangiosclerosis and fibrosis. The treatment included plasmapheresis which resulted in partial recovery of renal and liver function. However, in proceeding weeks patient’s liver function deteriorated (bilirubin=501 μmol/L; AST=267IU/L; ALT=503IU/L), and due to unidentified liver failure, the patient was retransplanted. His renal function partially improved with resolving hyperbilirubinemia following LT (creatinine=145μmol/L). He was discharged from the hospital with normally functioning graft and improved but still impaired kidney function. There are only a few reported cases of this condition described in the literature and aetiology of this condition is still not completely understood. The presented case report demonstrates the particular background of bile cast nephropathy and the subsequent treatment. Though, we emphasize the need for further investigations of this condition, as well as the established treatment guidelines

Digital Clubbing in Hereditary Hemorrhagic Telangiectasia/Juvenile Polyposis Syndrome

Hereditary hemorrhagic telangiectasia (HHT) (Osler- Weber-Rendu Syndrome) is a rare autosomal dominant vascular disorder characterized by the presence of multiple arteriovenous malformations (AVMs) and recurrent bleeding episodes. The diagnosis is based on the Curacao criteria: (i) spontaneous recurrent epistaxis, (ii) mucocutaneous telangiectasia, (iii) AVMs of visceral organs, and (iv) first degree relatives with a similar condition (1). Due to a common genetic pathway and SMAD4 gene mutation, juvenile polyposis syndrome (JPS) may coexist with HHT (2). The disease burden is high in overlapping HHT/JPS, but digital clubbing may be the only physical finding. Continuous meticulous management may improve the quality of life and reduce the risk of complication

Neuroendocrine tumor – recipient or donor derived?

Neuroendocrine tumors (NET) are a heterogeneous group of neoplasms with a predominant localization in the gastrointestinal tract. NET metastases within the liver represent a rare indication for liver transplantation (LT). We report a case of NET within a graft detected after liver transplantation. 49-year old male was transplanted in 10/2013 due to alcoholic liver cirrhosis with no other comorbidities. His early postoperative period was uneventful and he was discharged on triple immunosuppressive therapy. 11 days after LT, an oval hypoechogenic 15mm lesion was detected by ultrasound, localized in the left lobe of the liver. During follow-up the lesion was stable until 11 months later when abdominal MRI identified 2 hypovascular lesions (20mm and 11mm), also confirmed by MSCT and characterised as hemangiomas. 2.5 years after LT, MRI showed multiple lesions throughout the liver parenchyma which biopsy identified as NET grade II. In 9/2018. the patient was re-transplanted. Histology report showed that 60% of liver was infiltrated by NET. 5 months after reLT the patient is unremarkable with no signs of NET recurrence/dissemination. Tumors after solid organ transplantation develop as a) a recurrence/dissemination of a primary tumor, b) de novo formation or c) as donor/organ derived tumors. This case demonstrates that transplanted organs can be the primary source of tumors. Although rare, these cases have been previously described. This case also emphasizes the importance of long term follow-up after LT

Anemia after Solid Organ Transplantation

Anemija nakon transplantacije solidnih organa česta je komplikacija. Uzroci anemije jednaki su onima u općoj populaciji poput nedostatka željeza ili specifični kao posljedica odbacivanja, disfunkcije presatka, nedostatka eritropoetina, virusne infekcije, imunosupresivne ili antiviralne terapije. Većina je anemija nakon transplantacije reverzibilna, a osnovu liječenja čine nadoknada željeza i/ili eritropoetina, koncentrata eritrocita uz korekciju imunosupresivne i potporne terapije. Inhibitori hipoksija-inducibilnih faktora predstavljaju nadolazeće mogućnosti liječenja. Pravovremeno prepoznavanje i liječenje ove komplikacije značajno utječe na ishode pacijenata nakon transplantacije solidnih organa. U ovome preglednom radu donosimo dosadašnja saznanja o anemijama nakon transplantacije solidnih organa poput bubrega, jetre, srca i pluća s naglaskom na etiologiju i liječenje.Anemia after solid organ transplantation is a common complication. The causes of anemia can be the same as those in the general population such as iron deficiency, or specific due to rejection or graft dysfunction, erythropoietin deficiency, viral infection, immunosuppressive or antiviral therapy. Most anemia after transplantation is reversible, and the basis of treatment is iron and/or erythropoietin supplementation, erythrocyte concentrate transfusion, with correction of immunosuppressive and supportive therapy. Inhibitors of hypoxia-inducible factors represent emerging treatment options. Timely recognition and treatment of this complication significantly affects patient outcomes after solid organ transplantation. In this review, we present the current knowledge about anemia after transplantation of solid organs such as kidneys, liver, heart and lungs, with an emphasis on etiology and treatment

Hepatitis E virus infection after kidney transplantation

HEV usually presents as acute self-limiting hepatitis. However, in immunocompromised populations it may lead to chronic hepatitis and liver cirrhosis. We present a case of an HEV infection in a kidney transplant recipient

A case of overlapping primary sclerosing cholangitis and autoimmune hepatitis presented as acute on chronic liver failure

7-14% of patients suffering from primary sclerosing cholangitis (PSC) may present with clinical and serological features, indicating an overlap syndrome with autoimmune hepatitis (AIH). In an otherwise indolent course of a chronic liver disease, an exacerbation can result in acute liver failure (ALF). A 22-year-old man with a history of thrombotic events and verified thrombophilia (PAI-1 mutation) presented with jaundice (bilirubin=211,3 μmol/L) and abdominal discomfort. His synthetic liver function rapidly deteriorated (AST=3084 U/L, ALT=2928 U/L, ALP=154 U/L, GGT=63 U/L). Initial workup excluded viral causes and he was referred to the University Hospital Merkur with the diagnosis of ALF of unknown etiology. Whilst pursuing the underlying cause of the ALF and concomitant anaemia, the diagnosis of Crohn’s disease was established via lower endoscopy. In the following days, the patient’s state severely worsened, including the development of stage 3 hepatic encephalopathy. 7 days after admission, the patient was successfully transplanted. Histological assessment confirmed the diagnosis of overlapping AIH and PSC. The patient was uneventful for 2.5 years, when after the rise of liver enzymes (AST=296 U/L, ALT=682 U/L, GGT=93 U/L, ALP=165 U/L), a liver biopsy confirmed the relapse of AIH. Remission was attained with the administration of corticosteroids. In the 3-year follow-up, the patient was relapse-free without thromboembolic events. This complex case emphasizes the importance of prompt management of ALF and the difficulty of distinguishing acute on chronic liver failure from ALF. Overlapping liver diseases can be accompanied by various conditions, most commonly inflammatory bowel diseases, which makes the treatment particularly challenging