Idiopathic Adulthood Ductopenia: 'It Is Out There'
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 liter...
| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
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Karger Publishers
2016-05-01
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| Series: | Case Reports in Gastroenterology |
| Subjects: | |
| Online Access: | http://www.karger.com/Article/FullText/444443 |
| _version_ | 1819207023860908032 |
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| author | Mohammad Bilal Ali Kazemi Michael Babich |
| author_facet | Mohammad Bilal Ali Kazemi Michael Babich |
| author_sort | Mohammad Bilal |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-23T05:16:54Z |
| format | Article |
| id | doaj.art-8a85e230fbf84db9a5314e97c16458b0 |
| institution | Directory Open Access Journal |
| issn | 1662-0631 |
| language | English |
| last_indexed | 2024-12-23T05:16:54Z |
| publishDate | 2016-05-01 |
| publisher | Karger Publishers |
| record_format | Article |
| series | Case Reports in Gastroenterology |
| spelling | doaj.art-8a85e230fbf84db9a5314e97c16458b02022-12-21T17:58:49ZengKarger PublishersCase Reports in Gastroenterology1662-06312016-05-01101959810.1159/000444443444443Idiopathic Adulthood Ductopenia: 'It Is Out There'Mohammad BilalAli KazemiMichael BabichIdiopathic 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.http://www.karger.com/Article/FullText/444443Elevated alkaline phosphataseCholestasisIdiopathic adulthood ductopenia |
| spellingShingle | Mohammad Bilal Ali Kazemi Michael Babich Idiopathic Adulthood Ductopenia: 'It Is Out There' Case Reports in Gastroenterology Elevated alkaline phosphatase Cholestasis Idiopathic adulthood ductopenia |
| title | Idiopathic Adulthood Ductopenia: 'It Is Out There' |
| title_full | Idiopathic Adulthood Ductopenia: 'It Is Out There' |
| title_fullStr | Idiopathic Adulthood Ductopenia: 'It Is Out There' |
| title_full_unstemmed | Idiopathic Adulthood Ductopenia: 'It Is Out There' |
| title_short | Idiopathic Adulthood Ductopenia: 'It Is Out There' |
| title_sort | idiopathic adulthood ductopenia it is out there |
| topic | Elevated alkaline phosphatase Cholestasis Idiopathic adulthood ductopenia |
| url | http://www.karger.com/Article/FullText/444443 |
| work_keys_str_mv | AT mohammadbilal idiopathicadulthoodductopeniaitisoutthere AT alikazemi idiopathicadulthoodductopeniaitisoutthere AT michaelbabich idiopathicadulthoodductopeniaitisoutthere |