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Common variable immunodeficiency (CVID) associated liver disease is an underrecognized and poorly studied non-infectious complication that lacks an established treatment. We describe a CVID patient with severe multiorgan complications, including non-cirrhotic portal hypertension secondary to nodular regenerative hyperplasia leading to diuretic-refractory ascites. Remarkably, treatment with rituximab, administered for concomitant immune thrombocytopenia, resulted in the complete and sustained resolution of portal hypertension and ascites. Our case, complemented with a literature review, suggests a beneficial effect of rituximab that warrants further research.
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http://dx.doi.org/10.3389/fimmu.2023.1264482 | DOI Listing |
World J Gastroenterol
August 2025
Liver Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD 20892, United States.
Background: Cardiopulmonary changes in noncirrhotic portal hypertension (NCPH) are poorly understood.
Aim: To investigate cardiopulmonary changes using transthoracic echocardiography (TTE) in NCPH and their correlation with clinical features.
Methods: Prospective cohort including 10 preclinical NCPH [without portal hypertension (PH)] and 32 NCPH subjects who underwent TTE with agitated saline injection and comprehensive clinical evaluation were assessed.
Am J Clin Pathol
September 2025
Department of Pathology, Duke University Health System, Durham, NC, United States.
Objective: The number of orthotopic heart transplants (OHTs) performed each year continues to increase, as does the post-transplant survival rate. Little is known, however, about the morphologic changes in the liver after the patient has undergone an OHT. In this study, we retrospectively reviewed liver pathology in patients following OHT to comprehensively describe the histopathologic changes, particularly fibrosis.
View Article and Find Full Text PDFAbdom Radiol (NY)
August 2025
Department of Radiology, Hamamatsu University School of Medicine, 1-20-1, Handayama, Chuo-ku, Hamamatsu City, Shizuoka, 431-3192, Japan.
Hepatobiliary (HB)-specific magnetic resonance imaging (MRI) contrast agents, particularly gadoxetic acid, are being increasingly utilized for liver imaging, with well-established clinical utility. The most distinctive feature of gadoxetic acid is HB phase imaging performed 20 min after administration. In clinical practice, most focal liver lesions lack gadoxetic acid uptake and appear hypointense against the background of normally enhancing liver parenchyma owing to the absence of hepatocyte function within the tumor tissue.
View Article and Find Full Text PDFRadiographics
September 2025
From the Department of Radiology and Surgery, Division of Body Imaging (A.A.B., M.H., R.C., F.H.M.), Department of Medicine, Division of Gastroenterology and Hepatology (J.R.B.), and Department of Radiology, Division of Interventional Radiology (B.T.), Northwestern University Feinberg School of Medi
Noncirrhotic portal hypertension (NCPH) is an uncommon but important entity caused by impaired flow dynamics in the portal venous system in the absence of advanced fibrosis. The exact prevalence of this disease is unknown since many patients with NCPH are labeled as having cryptogenic cirrhosis. Numerous disease processes performed with different mechanisms can result in this entity.
View Article and Find Full Text PDFSkeletal Radiol
August 2025
Department of Radiology, Mass General Brigham, Boston, MA, USA.
Objectives: Patients with extremity sarcomas may require amputation when limb salvage surgery is not possible. Targeted muscle reinnervation (TMR) and regenerative peripheral nerve interface (RPNI) are procedures that can reduce neuroma formation, though neuromas can still occur. This study aims to describe the MRI characteristics of TMR/RPNI, examine changes in neuromas over time, and differentiate these from sarcoma recurrence on MRI.
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