Sickle-cell intrahepatic cholestasis (SCIC) is an uncommon complication of sickle-cell disease (SCD), which can be life-threating if left untreated. We present the case of a 28-year-old man with SCD, who presented wit...Sickle-cell intrahepatic cholestasis (SCIC) is an uncommon complication of sickle-cell disease (SCD), which can be life-threating if left untreated. We present the case of a 28-year-old man with SCD, who presented with jaundice and abdominal pain, one month after hydroxyurea discontinuation. Laboratory investigation revealed solely increased serum bilirubin, mainly conjugated, and imaging studies revealed choledocholithiasis. The patient unterwent an endoscopic sphincterectomy, but his jaundice deteriorated. Sickle-cell intrahepatic cholestasis was suspected and he received aggressive exchange transfusion therapy in combination with hydroxyurea. The jaundice had no signs of improvement;in fact total bilirubin raised up to 1053.7 μmol/L (61.62 mg/dl). Subsequently, the patient was treated with single-pass albumin dialysis (SPAD), in order to remove the excess bilirubin and protect mainly the brain and liver cells from its toxic effects. His laboratory values started to improve after one-and-a-half months of treatment. For the next two months, exchange transfusions were continued and bilirubin gradually returned to baseline values. The successful response appeared after the combined use of exchange transfusion and SPAD, which is being reported for the first time.展开更多
Acute kidney injury (AKI) is a common complication of liver cirrhosis and is of the utmost clinical and prognostic relevance. Patients with cirrhosis, especially decompensated cirrhosis, are more prone to develop AKI ...Acute kidney injury (AKI) is a common complication of liver cirrhosis and is of the utmost clinical and prognostic relevance. Patients with cirrhosis, especially decompensated cirrhosis, are more prone to develop AKI than those without cirrhosis. The hepatorenal syndrome type of AKI (HRS–AKI), a spectrum of disorders in prerenal chronic liver disease, and acute tubular necrosis (ATN) are the two most common causes of AKI in patients with chronic liver disease and cirrhosis. Differentiating these conditions is essential due to the differences in treatment. Prerenal AKI, a more benign disorder, responds well to plasma volume expansion, while ATN requires more specific renal support and is associated with substantial mortality. HRS–AKI is a facet of these two conditions, which are characterized by a dysregulation of the immune response. Recently, there has been progress in better defining this clinical entity, and studies have begun to address optimal care. The present review synopsizes the current diagnostic criteria, pathophysiology, and treatment modalities of HRS–AKI and as well as AKI in other chronic liver diseases (non-HRS–AKI) so that early recognition of HRS–AKI and the appropriate management can be established.展开更多
文摘Sickle-cell intrahepatic cholestasis (SCIC) is an uncommon complication of sickle-cell disease (SCD), which can be life-threating if left untreated. We present the case of a 28-year-old man with SCD, who presented with jaundice and abdominal pain, one month after hydroxyurea discontinuation. Laboratory investigation revealed solely increased serum bilirubin, mainly conjugated, and imaging studies revealed choledocholithiasis. The patient unterwent an endoscopic sphincterectomy, but his jaundice deteriorated. Sickle-cell intrahepatic cholestasis was suspected and he received aggressive exchange transfusion therapy in combination with hydroxyurea. The jaundice had no signs of improvement;in fact total bilirubin raised up to 1053.7 μmol/L (61.62 mg/dl). Subsequently, the patient was treated with single-pass albumin dialysis (SPAD), in order to remove the excess bilirubin and protect mainly the brain and liver cells from its toxic effects. His laboratory values started to improve after one-and-a-half months of treatment. For the next two months, exchange transfusions were continued and bilirubin gradually returned to baseline values. The successful response appeared after the combined use of exchange transfusion and SPAD, which is being reported for the first time.
文摘Acute kidney injury (AKI) is a common complication of liver cirrhosis and is of the utmost clinical and prognostic relevance. Patients with cirrhosis, especially decompensated cirrhosis, are more prone to develop AKI than those without cirrhosis. The hepatorenal syndrome type of AKI (HRS–AKI), a spectrum of disorders in prerenal chronic liver disease, and acute tubular necrosis (ATN) are the two most common causes of AKI in patients with chronic liver disease and cirrhosis. Differentiating these conditions is essential due to the differences in treatment. Prerenal AKI, a more benign disorder, responds well to plasma volume expansion, while ATN requires more specific renal support and is associated with substantial mortality. HRS–AKI is a facet of these two conditions, which are characterized by a dysregulation of the immune response. Recently, there has been progress in better defining this clinical entity, and studies have begun to address optimal care. The present review synopsizes the current diagnostic criteria, pathophysiology, and treatment modalities of HRS–AKI and as well as AKI in other chronic liver diseases (non-HRS–AKI) so that early recognition of HRS–AKI and the appropriate management can be established.
