Bilal Mohammad, Kazemi Ali, Babich Michael
Division of Gastroenterology, Hepatology and Nutrition, Allegheny General Hospital, Pittsburgh, Pa., USA.
Case Rep Gastroenterol. 2016 May 19;10(1):95-8. doi: 10.1159/000444443. eCollection 2016 Jan-Apr.
Idiopathic adulthood ductopenia (IAD) is a chronic cholestatic entity of unknown origin characterized by loss of inter-lobular bile ducts that was first described two decades ago. Although the diagnostic criteria have been described in detail, IAD continues to be a rare diagnosis. Our thorough literature search revealed less than a hundred cases of IAD reported. Here we present a 34-year-old female with no significant past history who was evaluated for persistent elevation of serum alkaline phosphate levels. Serology was negative for all viral hepatitides, and a chronic liver disease workup was unremarkable. Magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography did not reveal any abnormalities in the biliary tree. Finally, a liver biopsy demonstrated ductopenia involving greater than 50% of the portal triads, making a diagnosis of IAD. Since the disease can progress rapidly, close follow-up is warranted, so liver transplantation can be pursued if deemed necessary.
特发性成人胆管减少症（IAD）是一种病因不明的慢性胆汁淤积性疾病，其特征为小叶间胆管缺失，该病于二十年前首次被描述。尽管诊断标准已被详细描述，但IAD仍然是一种罕见的诊断。我们全面的文献检索发现报道的IAD病例不到100例。在此，我们报告一名34岁女性，既往无重大病史，因血清碱性磷酸酶水平持续升高接受评估。所有病毒性肝炎的血清学检查均为阴性，慢性肝病检查无异常。磁共振胰胆管造影和内镜逆行胰胆管造影未发现胆管树有任何异常。最后，肝脏活检显示胆管减少累及超过50%的门三联，从而诊断为IAD。由于该病可能迅速进展，因此有必要密切随访，如有必要可考虑肝移植。
