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Orthotopic liver transplantation (OLTx) in non-cirrhotic portal hypertension secondary to ADAMTS13 deficiency.

Raszeja-Wyszomirska J, Wasilewicz M, Andrzejewska A, Dudek K, Figiel W, Urasińska E, Ziarkiewicz-Wróblewska B, Milkiewicz P, Krawczyk M - Prz Gastroenterol (2015)

View Article: PubMed Central - PubMed

Affiliation: Liver and Internal Medicine Unit, Department of General, Transplant, and Liver Surgery, Medical University of Warsaw, Warsaw, Poland.

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Non-cirrhotic intrahepatic portal hypertension (NCIPH), also called idiopathic or benign, may lead to life-threatening complications... A 20-year-old, previously healthy male student presented with upper gastrointestinal bleeding... Gastroduodenoscopy revealed active bleeding from oesophageal varices, which was successfully treated with endoscopic banding ligation... However, the tissue sample size was rather slight and we were not able to measure hepatic venous pressure gradient (HVPG)... Percutaneous liver biopsy performed later confirmed these findings... Non-cirrhotic intrahepatic portal hypertension is also called idiopathic or benign portal hypertension... The physiological role of VWF, secreted from endothelium, is to facilitate platelet adhesion at sites of endothelial damage... Mutations of ADAMTS13 are seen in congenital TTP, while antibodies to ADAMTS13 are found in the majority of adult acquired cases... The imbalance in VWF and ADAMTS13 levels in portal microcirculation, where hepatic arterial blood pressures are superimposed, with upstream relation to hepatic stellate cells, would provide a mechanism for obliteration of terminal portal venules, which is characteristic of NCIPH... Chronic NCIPH leads to hepatic atrophy, although the progression of the disease is usually slow... The disorder can be associated with celiac disease and ulcerative colitis, and sustained deficiency of ADAMTS13 appears characteristic of NCIPH despite preserved liver function... Older age at first presentation, hepatic encephalopathy, and portal vein thrombosis were found to be significant predictors of reduced transplant-free survival with, as already mentioned, progression to decompensated liver disease and the need for liver transplantation in a significant proportion of patients.

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Computed tomography images of the liver hypotrophy and spleen enlargement
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Figure 0002: Computed tomography images of the liver hypotrophy and spleen enlargement

Mentions: A 20-year-old, previously healthy male student presented with upper gastrointestinal bleeding. Gastroduodenoscopy revealed active bleeding from oesophageal varices, which was successfully treated with endoscopic banding ligation. Physical examination showed splenomegaly, but was otherwise normal with no signs suggesting chronic liver disease. Laboratory investigations were all normal except thrombocytopenia of 40,000/ml. Viral, metabolic, and autoimmunological markers of liver disease as well as bone marrow examination were normal. Transjugular liver biopsy was performed and was essentially normal. However, the tissue sample size was rather slight and we were not able to measure hepatic venous pressure gradient (HVPG). Percutaneous liver biopsy performed later confirmed these findings. Contrast enhanced magnetic resonance imaging scan excluded portal vein thrombosis and Budd-Chiari syndrome. Laboratory investigations showed undetectable levels of ADAMTS13. The patient has been followed up over a period of 5 years, showing constant progression of his portal hypertension with continuous enlargement of his spleen (Figures 1 and 2) and frequent oesophageal/gastric variceal treatments. Follow-up liver biopsy showed progression to F2 fibrosis. He underwent OLTx with rapid recovery and remains extremely well 6 months after surgery. The histological changes in the explanted liver are shown in Figures 3 A–C.


Orthotopic liver transplantation (OLTx) in non-cirrhotic portal hypertension secondary to ADAMTS13 deficiency.

Raszeja-Wyszomirska J, Wasilewicz M, Andrzejewska A, Dudek K, Figiel W, Urasińska E, Ziarkiewicz-Wróblewska B, Milkiewicz P, Krawczyk M - Prz Gastroenterol (2015)

Computed tomography images of the liver hypotrophy and spleen enlargement
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4814532&req=5

Figure 0002: Computed tomography images of the liver hypotrophy and spleen enlargement
Mentions: A 20-year-old, previously healthy male student presented with upper gastrointestinal bleeding. Gastroduodenoscopy revealed active bleeding from oesophageal varices, which was successfully treated with endoscopic banding ligation. Physical examination showed splenomegaly, but was otherwise normal with no signs suggesting chronic liver disease. Laboratory investigations were all normal except thrombocytopenia of 40,000/ml. Viral, metabolic, and autoimmunological markers of liver disease as well as bone marrow examination were normal. Transjugular liver biopsy was performed and was essentially normal. However, the tissue sample size was rather slight and we were not able to measure hepatic venous pressure gradient (HVPG). Percutaneous liver biopsy performed later confirmed these findings. Contrast enhanced magnetic resonance imaging scan excluded portal vein thrombosis and Budd-Chiari syndrome. Laboratory investigations showed undetectable levels of ADAMTS13. The patient has been followed up over a period of 5 years, showing constant progression of his portal hypertension with continuous enlargement of his spleen (Figures 1 and 2) and frequent oesophageal/gastric variceal treatments. Follow-up liver biopsy showed progression to F2 fibrosis. He underwent OLTx with rapid recovery and remains extremely well 6 months after surgery. The histological changes in the explanted liver are shown in Figures 3 A–C.

View Article: PubMed Central - PubMed

Affiliation: Liver and Internal Medicine Unit, Department of General, Transplant, and Liver Surgery, Medical University of Warsaw, Warsaw, Poland.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Non-cirrhotic intrahepatic portal hypertension (NCIPH), also called idiopathic or benign, may lead to life-threatening complications... A 20-year-old, previously healthy male student presented with upper gastrointestinal bleeding... Gastroduodenoscopy revealed active bleeding from oesophageal varices, which was successfully treated with endoscopic banding ligation... However, the tissue sample size was rather slight and we were not able to measure hepatic venous pressure gradient (HVPG)... Percutaneous liver biopsy performed later confirmed these findings... Non-cirrhotic intrahepatic portal hypertension is also called idiopathic or benign portal hypertension... The physiological role of VWF, secreted from endothelium, is to facilitate platelet adhesion at sites of endothelial damage... Mutations of ADAMTS13 are seen in congenital TTP, while antibodies to ADAMTS13 are found in the majority of adult acquired cases... The imbalance in VWF and ADAMTS13 levels in portal microcirculation, where hepatic arterial blood pressures are superimposed, with upstream relation to hepatic stellate cells, would provide a mechanism for obliteration of terminal portal venules, which is characteristic of NCIPH... Chronic NCIPH leads to hepatic atrophy, although the progression of the disease is usually slow... The disorder can be associated with celiac disease and ulcerative colitis, and sustained deficiency of ADAMTS13 appears characteristic of NCIPH despite preserved liver function... Older age at first presentation, hepatic encephalopathy, and portal vein thrombosis were found to be significant predictors of reduced transplant-free survival with, as already mentioned, progression to decompensated liver disease and the need for liver transplantation in a significant proportion of patients.

No MeSH data available.


Related in: MedlinePlus