2009
DOI: 10.1590/s1806-37132009001000010
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Abstract: Primary immunodeficiency diseases comprise a genetically heterogeneous group of disorders that affect distinct components of the innate and adaptive immune system, such as neutrophils, macrophages, dendritic cells, complement proteins and natural killer cells, as well as T and B lymphocytes. The study of these diseases has provided essential insights into the functioning of the immune system. Primary immunodeficiency diseases have been linked to over 120 different genes, abnormalities in which account for appr… Show more

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Cited by 10 publications
(18 citation statements)
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References 40 publications
(51 reference statements)
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“…The prevalence of chronic pulmonary complications at CVI diagnosis is high (27.0-34.2%). ( 9 ) The most common such complication is bronchiectasis. Extensive pneumonia and the chronicity of infectious pulmonary episodes are responsible for the poor prognosis in CVI patients.…”
Section: To the Editormentioning
confidence: 99%
“…The prevalence of chronic pulmonary complications at CVI diagnosis is high (27.0-34.2%). ( 9 ) The most common such complication is bronchiectasis. Extensive pneumonia and the chronicity of infectious pulmonary episodes are responsible for the poor prognosis in CVI patients.…”
Section: To the Editormentioning
confidence: 99%
“…Hematological dysfunction in CHS causes important changes in CBC, such as qualitative/morphological and quantitative abnormalities (5,12,18,24) . Neutropenia persisstende is observed by increasing apoptosis, throughout hospitalization, evolving to febrile neutropenia.…”
Section: Discussionmentioning
confidence: 99%
“…These granules are present during the process, and cease in stimulus absence. CHS lysosomal granules are not restricted to neutrophils (Figure 2B), they may be present in lymphocytes (Figure 2A) and monocytes ( Figure 2C), in addition, they are larger and rougher, and are present in all stages of disease, with or without symptomatology (12,13,16) . Other inclusions that may get confused with CHS are Alder Reilly inclusions, which are more common in individuals with Tay-Sachs disease and mucopolysaccharidosis, which present azurophils variable in size and shape from spherical to ellipsoid, that may hide nucleus cell (16,22) .…”
Section: Discussionmentioning
confidence: 99%
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“…Cada genótipo podendo desenvolver fenótipos diferentes. Estes defeitos gênicos podem afetar enzimas, proteínas estruturais, moléculas de transdução de sinais ou proteínas de reparo do DNA 10 . 18 .…”
Section: Imunodeficiências Primáriasunclassified