ArticleCureus2022
Transjugular Intrahepatic Portosystemic Shunt for Refractory Ascites in Gaucher Disease.
Article in Cureus, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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0 citing papers in PubMed, 0 citations in OpenAlex.
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Authors and funding
4 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Gaucher disease is rare, inherited lysosomal storage disorder that leads to the excessive accumulation of certain lipids, especially within the bone marrow, liver, and spleen. We present a case of a 30-year-old man with Gaucher disease who underwent a splenectomy at the age of eight for severe cytopenia. His subsequent history was notable for recurrent avascular osteonecrosis and his liver disease progressed to portal hypertension, variceal bleeding, and refractory ascites. Upon evaluation of his candidacy for liver transplantation, he was sarcopenic, with tense, high serum-ascites albumin gradient (SAAG) ascites and florid venous collaterals on his anterior abdominal wall. His hepatic venous pressure gradient (HVPG) was 22 mmHg. He underwent a transjugular intrahepatic portosystemic shunt (TIPS) procedure, following which his HVPG was reduced to 2 mmHg and striking reversal of ascites as well as improvement of his nutritional state. TIPS was not complicated by hepatic encephalopathy. The successful outcome of TIPS in Gaucher disease with advanced hepatic disease underscores its utility as a bridge to liver transplantation with continuing enzyme replacement therapy.
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