Management of liver disease and portal hypertension in common variable immunodeficiency (CVID)

Baumert, Lukas S.; Shih, Angela; Chung, Raymond T.

doi:10.1016/j.jhepr.2023.100882

JHEP Reports

2023

DOI: 10.1016/j.jhepr.2023.100882

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Management of liver disease and portal hypertension in common variable immunodeficiency (CVID)

Lukas S. Baumert,

Angela Shih,

Raymond T. Chung

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2024

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Cited by 2 publications

References 120 publications

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Non-cirrhotic portal fibrosis/idiopathic portal hypertension: APASL recommendations for diagnosis and management

Shukla,

Rockey,

Kamath

et al. 2024

Hepatol Int

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Since the Asian Pacific Association for the Study of the Liver (APASL) published guidelines on non-cirrhotic portal fibrosis/ idiopathic portal hypertension in 2007, there has been a surge in new information, especially with the introduction of the term porto-sinusoidal vascular disorder (PSVD). Non-cirrhotic intra-hepatic causes of portal hypertension include disorders with a clearly identifiable etiology, such as schistosomiasis, as well as disorders with an unclear etiology such as non-cirrhotic portal fibrosis (NCPF), also termed idiopathic portal hypertension (IPH). This entity is being increasingly recognized as being associated with systemic disease and drug therapy, especially cancer therapy. An international working group with extensive expertise in portal hypertension was assigned with formulating consensus guidelines to clarify the definition, diagnosis, histological features, natural history, and management of NCPF/IPH, especially in the context of PSVD. The guidelines were prepared based on evidence from existing published literature. Whenever there was paucity of evidence, expert opinion was included after detailed deliberation. The goal of this manuscript, therefore, is to enhance the current understanding and help create global consensus on the issues surrounding NCPF/IPH.

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Non-cirrhotic portal fibrosis/idiopathic portal hypertension: APASL recommendations for diagnosis and management

Shukla,

Rockey,

Kamath

et al. 2024

Hepatol Int

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Pathogenic <i>TNFRSF13B</i> Variant in an Adult Japanese Patient with Common Variable Immunodeficiency

Nakamura,

Ikunari,

Tanaka

et al. 2024

Intern. Med.

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Common variable immunodeficiency (CVID) is a primary B cell immunodeficiency disorder. Symptoms do not develop immediately after birth, and patients are often diagnosed in childhood and adulthood. These patients often develop autoimmune diseases and malignant tumors. We herein report a 50-year-old woman with severe hypogammaglobulinemia and recurrent respiratory tract infections who was diagnosed with CVID. Target sequencing showed a TNFRSF13B heterozygous frameshift variant. The patient had many comorbidities, probably caused by a CVID-induced immune imbalance. Physicians who treat adult patients are often unaware of CVID. CVID should be recognized as a differential diagnosis in hypogammaglobulinemia and recurrent infections.

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Management of liver disease and portal hypertension in common variable immunodeficiency (CVID)

Cited by 2 publications

References 120 publications

Non-cirrhotic portal fibrosis/idiopathic portal hypertension: APASL recommendations for diagnosis and management

Non-cirrhotic portal fibrosis/idiopathic portal hypertension: APASL recommendations for diagnosis and management

Pathogenic <i>TNFRSF13B</i> Variant in an Adult Japanese Patient with Common Variable Immunodeficiency

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