Differences in Pulmonary Complications in Common Variable Immunodeficiency and X-Linked Agammaglobulinemia

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A 20-YEAR SURVEY OF INFECTIOUS COMPLICATIONS IN 64 PATIENTS WITH COMMON VARIABLE IMMUNODEFICIENCY

Common variable immunodeficiency (CV ID) is a heterogeneous primary immunodeficiency disorder, characterized by hypogammaglobulinemia and increased susceptibility to recurrent bacterial infections. To determine the spectrum of infectious complications in patients with common variable immunodeficiency (CV ID), we reviewed the hospital records of 64 CVID patients, who were diagnosed in Child...

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Common variable immunodeficiency (CVID) is a primary immunodeficiency disease characterized by hypogammaglobulinemia and recurrent bacterial infections especially in respiratory and gastrointestinal systems. We present here 2 cases of CVID with gastrointestinal complications.Case 1 is a 25-year-old man with a history of chronic diarrhoea from childhood. Ultrasonography revealed ascites, with li...

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Genotype/phenotype correlations in X-linked agammaglobulinemia.

No clear genotype/phenotype correlations have been established in patients with X-linked agammaglobulinemia (XLA). To determine if the specific mutation in Btk might be one of the factors that influences the severity of disease or if polymorphic variants in Tec, a cytoplasmic tyrosine kinase that might substitute for Btk, could contribute to the clinical phenotype, we examined the age at diagno...

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Alterations of the X-linked lymphoproliferative disease gene SH2D1A in common variable immunodeficiency syndrome.

X-linked lymphoproliferative (XLP) disease is a primary immunodeficiency caused by a defect in the SH2D1A gene. At least 3 major manifestations characterize its clinical presentation: fatal infectious mononucleosis (FIM), lymphomas, and immunoglobulin deficiencies. Common variable immunodeficiency (CVID) is a syndrome characterized by immunoglobulin deficiency leading to susceptibility to infec...

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ژورنال

عنوان ژورنال: Journal of Allergy and Clinical Immunology

سال: 2017

ISSN: 0091-6749

DOI: 10.1016/j.jaci.2016.12.357