Endoscopic nasobiliary drainage improves jaundice attack symptoms in benign recurrent intrahepatic cholestasis: A case report

Abstract

A 66-year-old male with unbearable pruritus and jaundice was admitted for detailed examination. Blood tests on admission showed increased bilirubin with a dominant direct fraction. Ultrasonography and computed tomography performed subsequent to admission showed no narrowing or distension of the bile ducts. As the jaundice symptoms were not improved by the oral administration of ursodeoxycholic acid (300 mg/day) that had been started immediately after admission, endoscopic retrograde cholangiopancreatography (ERCP) was performed on hospital day 14. This also showed no abnormalities of the bile ducts. After considerating its potential effects for improving jaundice, endoscopic nasobiliary drainage (ENBD) was performed on the same day and was followed by immediate improvements in pruritus and jaundice. Detailed examinations were performed to identify the cause of the jaundice, which was suspected to be viral hepatitis, autoimmune hepatitis or drug-induced liver injury, however, there were no findings suggestive of any of these conditions. Following a further increase in bilirubin levels, confirmed by additional blood tests, a liver biopsy was performed. Histological findings were consistent with the histological features of benign recurrent intrahepatic cholestasis (BRIC). Although ursodeoxycholic acid is used as a first-line treatment in most cases of BRIC, ENBD should also be considered for patients not responding to this treatment.

Treatment of pruritus with Prometheus dialysis and absorption system in a patient with benign recurrent intrahepatic cholestasis

“…In one patient, the effect was long‐lasting, but in the remaining two, cholestasis relapsed within a year after ENBD treatment . In a recent report, another patient with BRIC benefited from ENBD; however, in this case the diagnosis was not confirmed by the genetic studies and there was also a history of chronic alcohol intake …”

“…8 In a recent report, another patient with BRIC benefited from ENBD; however, in this case the diagnosis was not confirmed by the genetic studies and there was also a history of chronic alcohol intake. 9 Although the pathogenesis of cholestatic pruritus remains enigmatic, a recent discovery of lysophosphatidic acid (LPA) as the main pruritogen in cholestasis, shed new light on its molecular mechanism. 22,23 Circulating LPA is formed by autotaxin (ATX), previously described as an autocrine cell motility factor.…”

“…In severe cases, intermittent nasobiliary drainage, partial external biliary diversion or even liver transplantation have been reported. [7][8][9][10] Extracorporeal removal of pruritogens may be obtained by charcoal hemoperfusion, plasmapheresis or liver dialysis, namely, single pass albumin dialysis, the molecular adsorbent recirculating system (MARS) and the Prometheus system. In BRIC patients, clinical and biochemical improvement have been demonstrated after use of MARS.…”

“…There are still conflicting results regarding therapeutic efficacy of corticosteroids, cholestyramine, phenobarbitone and more recently ursodeoxycholic acid (UDCA), rifampicin or simvastatin. In severe cases, intermittent nasobiliary drainage, partial external biliary diversion or even liver transplantation have been reported …”

Arrival time parametric imaging of the hemodynamic balance changes between the hepatic artery and the portal vein during deep inspiration, using Sonazoid-enhanced ultrasonography: A case of Budd-Chiari syndrome

“…We have previously used At-PI to investigate the influence of the hepatic portal vein and the hepatic artery on hepatic parenchymal enhancement, and have reported its use in the clinical assessment of type C chronic liver disease (7) and alcohol-induced liver disease (8). We have also utilized At-PI to investigate the effects of portal vein thrombosis on hepatic parenchymal enhancement (9), demonstrating its efficacy in the differential diagnosis of benign recurrent intrahepatic and familial intrahepatic cholestasis (10). …”