'Adulte Form' der kongenitalen Leberfibrose
| العنوان: | 'Adulte Form' der kongenitalen Leberfibrose |
|---|---|
| المؤلفون: | D Schacherer, J. Schölmerich, P Rümmele |
| المصدر: | DMW - Deutsche Medizinische Wochenschrift. 129:1500-1503 |
| بيانات النشر: | Georg Thieme Verlag KG, 2004. |
| سنة النشر: | 2004 |
| مصطلحات موضوعية: | Enlarged liver, medicine.medical_specialty, Pathology, business.industry, Ultrasound, General Medicine, medicine.disease, Ductal Plate Malformation, Esophageal varices, Biliary tract, Dysplasia, medicine, Congenital hepatic fibrosis, Histopathology, medicine.symptom, business |
| الوصف: | HISTORY AND ADMISSION FINDINGS During a routine check-up, a 37-year-old woman was found to have elevated levels of serum gamma-glutamyl transferase (gamma GTP) and IgA- and IgM-antibodies. One of the patient's brothers had died at the age of six from acute liver failure. We found a palpably enlarged liver with normal consistency and no particular helpful laboratory results. INVESTIGATIONS The abdominal ultrasound and computed tomography (CT) showed segmental and saccular dilatations of the biliary tract, hepatofugal flow in the portal vein, multiple collateral vessels as well as a mild splenomegaly. Histopathology revealed fibrotic liver parenchyma, a dilatated and branched biliary tract lined by cubical epithelium. COURSE Gastroscopy showed lowgrade esophageal varices. Seventeen months after the initial presentation there were no significant changes of the laboratory tests or the ultrasound. CONCLUSIONS The defective remodelling of the ductal plate ("ductal plate malformation") is associated with dysplasia of the biliary tract. Depending on the localisation of the lesions within the biliary tract and whether it is a more cystic or more fibrotic component, there are different malformations caused by ductal plate malformation. We diagnosed congenital hepatic fibrosis, because of the atypical age of presentation, it could be the "adult form" of congenital liver fibrosis. |
| تدمد: | 1439-4413 0012-0472 |
| DOI: | 10.1055/s-2004-826892 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_________::6f6a2fcf775087bf235a5e77def3601b https://doi.org/10.1055/s-2004-826892 |
| رقم الانضمام: | edsair.doi...........6f6a2fcf775087bf235a5e77def3601b |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 14394413 00120472 |
|---|---|
| DOI: | 10.1055/s-2004-826892 |