Evaluation of liver diseases in Iranian patients with primary antibody deficiencies

Motamed, Farzaneh; Aghamohammadi, Asghar; Soltani, Mitra Ahmad; Mansouri, Mahboubeh; Rezaei, Nima; Teimourian, Shahram; Pouladi, Nima; Abdollahzade, Sina; Parvaneh, Nima

doi:10.1016/s1665-2681(19)31765-x

Cited by 5 publications

(5 citation statements)

References 38 publications

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“…Hepatobiliary infection, sclerosing cholangitis, nodular regenerative hyperplasia, and portal hypertension are frequent conditions in CD40L deficiency [48, 49]. …”

Section: Methodsmentioning

confidence: 99%

“…Results from a study focusing mainly on pediatric patients with PIDD indicated clinical evidence of liver disease, including hepatomegaly, in 35.5 %; 8 patients (13 %) had clinical and/or laboratory evidence of chronic liver disease. Hepatobiliary infection, sclerosing cholangitis, nodular regenerative hyperplasia, and portal hypertension are frequent conditions in CD40L deficiency [ 48 , 49 ].…”

Section: Methodsmentioning

confidence: 99%

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Attending to Warning Signs of Primary Immunodeficiency Diseases Across the Range of Clinical Practice

et al. 2013

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PurposePatients with primary immunodeficiency diseases (PIDD) may present with recurrent infections affecting different organs, organ-specific inflammation/autoimmunity, and also increased cancer risk, particularly hematopoietic malignancies. The diversity of PIDD and the wide age range over which these clinical occurrences become apparent often make the identification of patients difficult for physicians other than immunologists. The aim of this report is to develop a tool for educative programs targeted to specialists and applied by clinical immunologists.MethodsConsidering the data from national surveys and clinical reports of experiences with specific PIDD patients, an evidence-based list of symptoms, signs, and corresponding laboratory tests were elaborated to help physicians other than immunologists look for PIDD.ResultsTables including main clinical manifestations, restricted immunological evaluation, and possible related diagnosis were organized for general practitioners and 5 specialties. Tables include information on specific warning signs of PIDD for pulmonologists, gastroenterologists, dermatologists, hematologists, and infectious disease specialists.ConclusionsThis report provides clinical immunologists with an instrument they can use to introduce specialists in other areas of medicine to the warning signs of PIDD and increase early diagnosis. Educational programs should be developed attending the needs of each specialty.

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“…Hepatobiliary infection, sclerosing cholangitis, nodular regenerative hyperplasia, and portal hypertension are frequent conditions in CD40L deficiency [48, 49]. …”

Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Attending to Warning Signs of Primary Immunodeficiency Diseases Across the Range of Clinical Practice

et al. 2013

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“…[ 88,89 ] Hepatomegaly with histopathology showing chronic active hepatitis with negative viral hepatitis serology has been mentioned. [ 10 ] NRH was seen in 6 out of 21 patients in one patient cohort, with a median age of diagnosis of 20 years. [ 90 ] Noncirrhotic portal hypertension (NCPH) secondary from NRH with clinical course complicated by esophageal varices & Spontaneous Bacterial Peritonitis (SBP) was seen in one XLA patient who died later from bleeding varices.…”

Section: Clinical Approach To Liver Disease In Ieismentioning

confidence: 99%

“…[12,[77][78][79] Few cases of SC and progressive hepatic insufficiency are noted with no mention of coexisting Cryptosporidium infection. [10,13] Primary liver malignancy such as HCC are uncommon and are associated with HCV-related liver failure and described in the absence of liver disease. [72,80,81] B cell lymphomas and T cell lymphomas involving the liver are described only in case reports.…”

Section: Common Variable Immunodeficiencymentioning

confidence: 99%

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Tip of the iceberg: A comprehensive review of liver disease in Inborn errors of immunity

et al. 2022

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Inborn errors of immunity (IEIs) consist of numerous rare, inherited defects of the immune system that affect about 500,000 people in the United States.As advancements in diagnosis through genetic testing and treatment with targeted immunotherapy and bone marrow transplant emerge, increasing numbers of patients survive into adulthood posing fresh clinical challenges.A large spectrum of hepatobiliary diseases now present in those with immunodeficiency diseases, leading to morbidity and mortality in this population. Awareness of these hepatobiliary diseases has lagged the improved management of the underlying disorders, leading to missed opportunities to improve clinical outcomes. This review article provides a detailed description of specific liver diseases occurring in various inborn errors of immunity.A generalized approach to diagnosis and management of hepatic complications is provided, and collaboration with hepatologists, immunologists, and pathologists is emphasized as a requirement for optimizing management and outcomes.

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Predominantly Antibody Deficiency

Aghamohammadi

Abolhassani

Eibl³

et al. 2012

Clinical Cases in Primary Immunodeficiency Diseases

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Evaluation of liver diseases in Iranian patients with primary antibody deficiencies

Cited by 5 publications

References 38 publications

Attending to Warning Signs of Primary Immunodeficiency Diseases Across the Range of Clinical Practice

Attending to Warning Signs of Primary Immunodeficiency Diseases Across the Range of Clinical Practice

Tip of the iceberg: A comprehensive review of liver disease in Inborn errors of immunity

Predominantly Antibody Deficiency

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