Drug-induced bile duct injury: progress and challenges / 中华肝脏病杂志

Drug-induced bile duct injury is a specific kind of drug-induced liver injury that has two main pathological types, namely ductopenia, or vanishing bile duct syndrome, and secondary sclerosing cholangitis. However, in recent years, the reports of new drugs that cause bile duct injury have been constantly increasing, and these drugs have different clinicopathological features and a novel pathogenesis. Therefore, this paper summarizes and analyzes the progress and challenges in the etiology, pathogenesis, diagnosis and treatment, and other aspects of drug-induced bile duct injury.

A Case of Stevens-Johnson Syndrome with Vanishing Bile Duct Syndrome / 대한피부과학회지

Stevens-Johnson syndrome (SJS) is a serious and potentially life-threatening disease. Vanishing bile duct syndrome (VBDS) is a rare cause of progressive cholestasis. Both syndromes are usually associated with the use of specific drugs. VBDS in patients with SJS is most commonly associated with drug hypersensitivity. To our knowledge, to date, the literature contains only a single report of toxic epidermal necrolysis associated with VBDS in Korea. We report a case of drug-induced SJS with acute onset of VBDS and present a review of the literature in this context. This case highlights the importance of awareness among clinicians regarding drug reactions, their severity, as well as diagnostic modalities and optimal management strategies in these cases.

Clinicopathologic features of drug-induced vanishing bile duct syndrome / 中华肝脏病杂志

Vanishing bile duct syndrome (VBDS) manifests as progressive destruction and disappearance of the intrahepatic bile duct caused by various factors and cholestasis. VBDS associated with drug-induced liver injury (D-VBDS) is an important etiology of VBDS, and immune disorder or immune imbalance may be the main pathogenesis. According to its clinical symptoms, serological markers, and course of the disease, D-VBDS is classified into major form and minor form, and its clinical features are based on various pathomorphological findings. Its prognosis is associated various factors including regeneration of bile duct cells, number of bile duct injuries, level and range of bile duct injury, bile duct proliferation, and compensatory shunt of bile duct branches. This disease has various clinical outcomes; most patients have good prognosis after drug withdrawal, and some patients may experience cholestatic cirrhosis, liver failure, and even death. Due to the clinical manifestation and biochemical changes are similar to the primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC), it need to identify by clinical physician.

Ibuprofen Associated Acute Vanishing Bile Duct Syndrome and Toxic Epidermal Necrolysis in an Infant

Acute vanishing bile duct syndrome, a rare but rapidly progressive destruction of the intrahepatic bile ducts with unknown pathogenesis, is most often a drug- or toxin-related. Toxic epidermal necrolysis is a serious dermatologic condition and a potentially life threatening disease, which is drug or infection induced. Ibuprofen associated acute vanishing bile duct syndrome and toxic epidermal necrolysis have not been reported previously in infants. We report a 7-month-old infant with ibuprofen associated toxic epidermal necrolysis, followed by severe and rapidly progressive vanishing bile duct syndrome. She recovered totally with supportive care.

A Case of Vanishing Bile Duct Syndrome Associated with Ibuprofen Overuse / 대한소아소화기영양학회지

Ibuprofen is currently widely prescribed in the pediatric population and has potentially very severe adverse events, even in the absence of an overdose. We treated a four year-old male who presented with severe jaundice, skin rash, xanthoma, eosinophilia and decreased hepatic function after overuse of ibuprofen for fever control. The liver biopsy revealed the vanishing bile duct syndrome. We report a case of vanishing bile duct syndrome associated with Ibuprofen overuse for the first case in Korea.

Spontaneous Resolution of Vanishing Bile Duct Syndrome in Hodgkin's Lymphoma / 대한간학회지

Cholestasis in a patient with Hodgkin's disease is uncommon, and the causes of cholestasis are mainly direct tumor involvement of the liver, hepatotoxic effects of drugs, viral hepatitis, sepsis and opportunistic infections. Vanishing bile duct syndrome (VBDS) represents a very rare cause for cholestasis in this disease. We report here on a case of a 45-year-old man who developed VBDS during the complete remission stage of Hodgkin's lymphoma. There was no history of hepatitis or intravenous drug abuse, and the patient had negative results for hepatitis A virus, hepatitis B virus, hepatitis C virus, cytomegalovirus, and human immunodeficiency virus. The serological studies for antinuclear antibodies, anti-mitochondrial antibodies and anti-smooth muscle antibodies were also negative. Liver biopsy disclosed the absence of interlobular bile ducts in 9 of 10 portal tracts without any active lymphocyte infiltration and there were no Reed-Sternberg cell in the liver. The patient's cholestasis was in remission and the serum bililrubin level was normalized after two months without treatment, but tumor recurrence was noted at multiple sites of the abdominal lymph nodes on follow-up abdomino-pelvic computed tomogram.

A Case of Vanishing Bile Duct Syndrome Associated with Hypersensitivity to Allopurinol / 대한간학회지

Allopurinol is frequently used for the treatment of hyperuricemia and gout. Sometimes, a life-threatening reaction develops, as is illustrated by the following case report. We describe a 60-year-old male patient who was treated with allopurinol because of asymptomatic hyperuricemia, and he was presented with fever, skin rash, eosinophilia, worsening renal function and vanishing bile duct syndrome. In this report, we discussed vanishing bile duct syndrome as a serious side effect of allopurinol, and we briefly reviewed the etiology, prevention, and treatment modalities for vanishing bile duct syndrome.