Common variable immunodeficiency. Epidemiology and clinical manifestations in 69 patients
Common variable immunodeficiency (CVID) is characterized by an impaired antibody production and an increased susceptibility to recurrent infections of the respiratory tract, mainly by extracellular encapsulated bacteria. We analyzed the clinical characteristics of 69 patients evaluated over a period...
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Veröffentlicht in: | Medicina (Buenos Aires) 2013, Vol.73 (4), p.315-323 |
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description | Common variable immunodeficiency (CVID) is characterized by an impaired antibody production and an increased susceptibility to recurrent infections of the respiratory tract, mainly by extracellular encapsulated bacteria. We analyzed the clinical characteristics of 69 patients evaluated over a period of 10 years at three centers in the city of Buenos Aires. At the onset of the study 14 patients were on follow up, and at its end the number of patients reached to 60. Most of them consulted for infection or hypogammaglobulinemia and nearly half had an established diagnosis of immunodeficiency. Sixty-five (94.2%) patients had infections by encapsulated bacteria, four (6.1%) sepsis and two tuberculosis. The average age of onset of infectious symptoms was 18.1 years; the average age at diagnosis was 29.6 years and the delay to diagnosis 11.9 years. Forty one (59.4%) patients reported a history of recurrent or chronic diarrhea. In 22 (31.9%) 13 autoimmune diseases were diagnosed, being the most frequent the hematological disorders and hypothyroidism. Eight patients had histological polyclonal lymphoproliferation, four (5.8%) with granulomatous disease affecting the liver, the larynx and/or the skin; and four as lymphoid interstitial pneumonitis (LIP). Nineteen (27.5%) patients had splenomegaly and 23/57 (40.3%) images suggestive of lymphocytic or granulomatous processes (including the 4 with LIP) in the chest CT. Three (4.3%) patients developed B cell lymphoma, four (5.8%) stomach adenocarcinoma and one breast cancer. The study had a median follow-up of 54 months, range 1-353 and four patients (5.8%) died during the follow up. |
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Epidemiology and clinical manifestations in 69 patients</title><source>MEDLINE</source><source>EZB-FREE-00999 freely available EZB journals</source><creator>Fernández Romero, Diego S ; Juri, María C ; Paolini, María V ; Malbrán, Alejandro</creator><creatorcontrib>Fernández Romero, Diego S ; Juri, María C ; Paolini, María V ; Malbrán, Alejandro</creatorcontrib><description>Common variable immunodeficiency (CVID) is characterized by an impaired antibody production and an increased susceptibility to recurrent infections of the respiratory tract, mainly by extracellular encapsulated bacteria. We analyzed the clinical characteristics of 69 patients evaluated over a period of 10 years at three centers in the city of Buenos Aires. At the onset of the study 14 patients were on follow up, and at its end the number of patients reached to 60. Most of them consulted for infection or hypogammaglobulinemia and nearly half had an established diagnosis of immunodeficiency. Sixty-five (94.2%) patients had infections by encapsulated bacteria, four (6.1%) sepsis and two tuberculosis. The average age of onset of infectious symptoms was 18.1 years; the average age at diagnosis was 29.6 years and the delay to diagnosis 11.9 years. Forty one (59.4%) patients reported a history of recurrent or chronic diarrhea. In 22 (31.9%) 13 autoimmune diseases were diagnosed, being the most frequent the hematological disorders and hypothyroidism. Eight patients had histological polyclonal lymphoproliferation, four (5.8%) with granulomatous disease affecting the liver, the larynx and/or the skin; and four as lymphoid interstitial pneumonitis (LIP). Nineteen (27.5%) patients had splenomegaly and 23/57 (40.3%) images suggestive of lymphocytic or granulomatous processes (including the 4 with LIP) in the chest CT. Three (4.3%) patients developed B cell lymphoma, four (5.8%) stomach adenocarcinoma and one breast cancer. 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Epidemiology and clinical manifestations in 69 patients</title><title>Medicina (Buenos Aires)</title><addtitle>Medicina (B Aires)</addtitle><description>Common variable immunodeficiency (CVID) is characterized by an impaired antibody production and an increased susceptibility to recurrent infections of the respiratory tract, mainly by extracellular encapsulated bacteria. We analyzed the clinical characteristics of 69 patients evaluated over a period of 10 years at three centers in the city of Buenos Aires. At the onset of the study 14 patients were on follow up, and at its end the number of patients reached to 60. Most of them consulted for infection or hypogammaglobulinemia and nearly half had an established diagnosis of immunodeficiency. Sixty-five (94.2%) patients had infections by encapsulated bacteria, four (6.1%) sepsis and two tuberculosis. The average age of onset of infectious symptoms was 18.1 years; the average age at diagnosis was 29.6 years and the delay to diagnosis 11.9 years. Forty one (59.4%) patients reported a history of recurrent or chronic diarrhea. In 22 (31.9%) 13 autoimmune diseases were diagnosed, being the most frequent the hematological disorders and hypothyroidism. Eight patients had histological polyclonal lymphoproliferation, four (5.8%) with granulomatous disease affecting the liver, the larynx and/or the skin; and four as lymphoid interstitial pneumonitis (LIP). Nineteen (27.5%) patients had splenomegaly and 23/57 (40.3%) images suggestive of lymphocytic or granulomatous processes (including the 4 with LIP) in the chest CT. Three (4.3%) patients developed B cell lymphoma, four (5.8%) stomach adenocarcinoma and one breast cancer. The study had a median follow-up of 54 months, range 1-353 and four patients (5.8%) died during the follow up.</description><subject>Adult</subject><subject>Age Factors</subject><subject>Age of Onset</subject><subject>Aged</subject><subject>Aged, 80 and over</subject><subject>Argentina - epidemiology</subject><subject>Common Variable Immunodeficiency - complications</subject><subject>Common Variable Immunodeficiency - epidemiology</subject><subject>Common Variable Immunodeficiency - immunology</subject><subject>Disease Progression</subject><subject>Female</subject><subject>Granulomatous Disease, Chronic - immunology</subject><subject>Humans</subject><subject>Lung Diseases, Interstitial - immunology</subject><subject>Lymphoma, B-Cell - immunology</subject><subject>Male</subject><subject>Middle Aged</subject><subject>Sex Factors</subject><subject>Stomach Neoplasms - immunology</subject><subject>Time Factors</subject><subject>Young Adult</subject><issn>0025-7680</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2013</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNo1UE1LxDAUzEFx19W_IDl6qTRp0yZHKesHLHjRg6fy2r7Ik3zUphX231tw9zLDwDAzzAXb5rlUWV3pfMOuU_rO88LUprpiG1kYWSpptuyzid7HwH9hIugccvJ-CXFASz1h6I8PfD_SgJ6ii19HDmHgvaNAPTjuIZDFNMNMMSROgVeGj6vCMKcbdmnBJbw98Y59PO3fm5fs8Pb82jweslGUYs6UUKCEQFUDdtJKrLSoobaqBJ13RWfXmdqWKHS_ojaDBhhkZ4XQEgWaYsfu_3PHKf4s65rWU-rROQgYl9SuLaYohVDlar07WZfO49COE3mYju35juIPtMRb-Q</recordid><startdate>2013</startdate><enddate>2013</enddate><creator>Fernández Romero, Diego S</creator><creator>Juri, María C</creator><creator>Paolini, María V</creator><creator>Malbrán, Alejandro</creator><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>7X8</scope></search><sort><creationdate>2013</creationdate><title>Common variable immunodeficiency. 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Epidemiology and clinical manifestations in 69 patients</atitle><jtitle>Medicina (Buenos Aires)</jtitle><addtitle>Medicina (B Aires)</addtitle><date>2013</date><risdate>2013</risdate><volume>73</volume><issue>4</issue><spage>315</spage><epage>323</epage><pages>315-323</pages><issn>0025-7680</issn><abstract>Common variable immunodeficiency (CVID) is characterized by an impaired antibody production and an increased susceptibility to recurrent infections of the respiratory tract, mainly by extracellular encapsulated bacteria. We analyzed the clinical characteristics of 69 patients evaluated over a period of 10 years at three centers in the city of Buenos Aires. At the onset of the study 14 patients were on follow up, and at its end the number of patients reached to 60. Most of them consulted for infection or hypogammaglobulinemia and nearly half had an established diagnosis of immunodeficiency. Sixty-five (94.2%) patients had infections by encapsulated bacteria, four (6.1%) sepsis and two tuberculosis. The average age of onset of infectious symptoms was 18.1 years; the average age at diagnosis was 29.6 years and the delay to diagnosis 11.9 years. Forty one (59.4%) patients reported a history of recurrent or chronic diarrhea. In 22 (31.9%) 13 autoimmune diseases were diagnosed, being the most frequent the hematological disorders and hypothyroidism. Eight patients had histological polyclonal lymphoproliferation, four (5.8%) with granulomatous disease affecting the liver, the larynx and/or the skin; and four as lymphoid interstitial pneumonitis (LIP). Nineteen (27.5%) patients had splenomegaly and 23/57 (40.3%) images suggestive of lymphocytic or granulomatous processes (including the 4 with LIP) in the chest CT. Three (4.3%) patients developed B cell lymphoma, four (5.8%) stomach adenocarcinoma and one breast cancer. The study had a median follow-up of 54 months, range 1-353 and four patients (5.8%) died during the follow up.</abstract><cop>Argentina</cop><pmid>23924529</pmid><tpages>9</tpages></addata></record> |
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subjects | Adult Age Factors Age of Onset Aged Aged, 80 and over Argentina - epidemiology Common Variable Immunodeficiency - complications Common Variable Immunodeficiency - epidemiology Common Variable Immunodeficiency - immunology Disease Progression Female Granulomatous Disease, Chronic - immunology Humans Lung Diseases, Interstitial - immunology Lymphoma, B-Cell - immunology Male Middle Aged Sex Factors Stomach Neoplasms - immunology Time Factors Young Adult |
title | Common variable immunodeficiency. Epidemiology and clinical manifestations in 69 patients |
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