Idiopathic Adulthood Ductopenia

Domínguez-Antonaya, Mercedes; Coba-Ceballos, Jose María; Gómez-Rubio, Mariano; Cuenca, B; Ortega-Muñoz, Pilar; Garcia, J

doi:10.1097/00004836-200003000-00018

Cited by 15 publications

(5 citation statements)

References 8 publications

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“…It is important for medical practitioners to be aware of IAD because it has a very benign presentation and can be easily missed or neglected. The progression of this entity also varies from a benign course to serious outcomes [ 4 , 5 ]. Ursodiol has been used in some case studies with symptomatic relief [ 5 , 6 ].…”

Section: Discussionmentioning

confidence: 99%

Idiopathic Adulthood Ductopenia: 'It Is Out There'

Bilal

Kazemi

Babich

2016

Case Rep Gastroenterol

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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.

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Section: Discussionmentioning

confidence: 99%

Idiopathic Adulthood Ductopenia: 'It Is Out There'

Bilal

Kazemi

Babich

2016

Case Rep Gastroenterol

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“…Nonetheless, IAD remains a diagnosis of exclusion in young-to-middle-aged adults without history of infantile cholangiopathy, and is characterized by biochemical evidence of cholestatic liver disease, as well as histological evidence of ductopenia, the loss of interlobular and septal bile ducts in at least 50% of the portal tracts [ 1 , 2 ]. Clinically, patients with IAD may present with episodic jaundice and pruritus or without symptoms [ 11 ]. The course of IAD can be variable and two types of IAD, associated with different prognoses, are currently recognized.…”

Section: Discussionmentioning

confidence: 99%

“…Since IAD is associated with variable etiologies, its treatment options may vary. In patients with non-progressive IAD, UDCA has been shown to improve liver enzymes [ 6 , 11 , 12 ]. On the other hand, patients with progressive diseases, and those with advanced cases, will ultimately require orthotopic liver transplantation [ 2–4 , 6 , 13 ].…”

Section: Discussionmentioning

confidence: 99%

A Case of mild idiopathic adulthood ductopenia and brief review of literature

Kaung

Sundaram

Dhall

et al. 2014

Gastroenterology Report

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Mild idiopathic adulthood ductopenia (IAD) is a rare cholestatic disease of unknown cause and characterized by interlobular bile duct loss in less than 50% of the portal tracts. We describe the case of a middLe-aged male who presented with persistent elevation of transaminases and alkaline phosphatase. He had a normal biliary tree on endoscopic retrograde cholangiopathy and negative anti-mitochondrial antibody. His liver biopsy specimen showed chronic biliary disease, duct loss in 4 out of 15 portal tracts and prominent cholestasis. Based on the work-up, he likely had mild IAD. Liver transplantation would be necessary if his disease becomes progressive.

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“…Currently, the diagnosis of IAD is considered with adult or adolescent onset, biochemical evidence of cholestatic liver disease, evidence of ductopenia by liver biopsy with normal cholangiography, and an unknown etiology. A diagnosis of IAD requires exclusion of other conditions with chronic cholestasis that are associated with ductopenia [1][2][3]. To date, less than 100 cases have been reported in the medical literature, with only 1 prior case in Korea [4].…”

Section: Introductionmentioning

confidence: 99%

A Case of Idiopathic Adulthood Ductopenia

Kang¹,

Jang²,

Lee³

et al. 2013

Korean J Pancreas Biliary Tract

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Idiopathic adulthood ductopenia (IAD) is a chronic cholestatic liver disease of unknown etiology characterized by adult onset, an absence of autoantibodies, inflammatory bowel disease, and a loss of interlobular bile ducts. In the present report, a case fulfilling the IAD criteria is described. A 19-year-old man was admitted to the hospital for persistent elevation of transaminases and alkaline phosphatase without clinical symptoms. Viral hepatitis markers and autoantibodies were absent. The patient had a normal extrahepatic biliary tree and had no evidence of inflammatory bowel disease. A liver biopsy specimen showed absence of interlobular bile ducts from 58% of the portal tracts. He was diagnosed with IAD and was treated with ursodeoxycholic acid.

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Idiopathic Adulthood Ductopenia

Cited by 15 publications

References 8 publications

Idiopathic Adulthood Ductopenia: 'It Is Out There'

Idiopathic Adulthood Ductopenia: 'It Is Out There'

A Case of mild idiopathic adulthood ductopenia and brief review of literature

A Case of Idiopathic Adulthood Ductopenia

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