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کاربرد نوع شرط:
- جایگاه : پژوهشی
- مجله: Immunology and Genetics Journal
- نوع مقاله: Journal Article
- کلمات کلیدی: Keywords: Agammaglobulinemia,autoimmunity,Primary Immunodeficiency Disorders
- چکیده:
- چکیده انگلیسی: Background: Agammaglobulinemia is a primary immunodeficiency disorders (PID) which is identified by increased susceptibility to the bacterial infections, significant low antibodies and isohemagglutins and decreased peripheral B cells counts. Different clinical manifestations could be observed in these patients. Some of agammaglobulinemia patients manifest autoimmune disorders, while the association of autoimmunity and agammaglobulinemia is not clarified yet. In this study, we evaluated the frequency of autoimmunity in agammaglobulinemia patients and we compared their demographic, clinical and laboratory data in two groups of the patients with and without autoimmunity.
Material and Methods: The present study included 147 patients diagnosed with agammaglobulinemia who were diagnosed at the Research Centre for Immunodeficiencies at the children’s medical Centre. A comprehensive history, demographic information, clinical manifestations, laboratory data were obtained from all patients to assess the autoimmune complications.
Results: Among 147 agammaglobulinemia patients, we identified 21 (14.2%) (18 males and 3 females) agammaglobulinemia patients who had autoimmune disorders. In a total of the patients, the most prominent clinical presentation was respiratory infections (72.4%). Among autoimmune disorders, Juvenile Rheumatoid Arthritis was the most important autoimmunity (38%) following Immune thrombocytopenia with the frequency of 14%.
Conclusion: Autoimmune diseases are not very common among agammaglobulinemia patients, however, these disorders should be considered as an important factor for management in patients. Early diagnosis and proper management of autoimmunity in agammaglobulinemia cases lead to enhancement of patient's quality of life.- انتشار مقاله: 28-04-1398
- نویسندگان: Paniz Shirmast,Kawthar Jasim Mohammad Rida Al-Hussieni
- مشاهده
- جایگاه : پژوهشی
- مجله: Immunology and Genetics Journal
- نوع مقاله: Journal Article
- کلمات کلیدی: Gastrointestinal,Diarrhea,Agammaglobulinemia,Vomiting,Gastroenteritis
- چکیده:
- چکیده انگلیسی: Background: The most common symptoms of agammaglobulinemia are recurrent respiratory and gastrointestinal problems. The purpose of this study was to define the prevalence and type of gastrointestinal (GI) manifestations in patients with agammaglobulinemia.
Methods: A total of 147 patients with agammaglobulinemia were entered into this study. For each patient clinical, immunological and laboratory data were documented.
Results: The GI manifestations as the first presentation of the immunodeficiency diseases were reported in 14.5% of patients mostly due to chronic and recurrent diarrhea. History of GI manifestations was evident in 67 patients (45.6%). Recurrent, chronic or bloody diarrhea (36.1%), vomiting (10.2%), and gastroenteritis (6.1%) were the most common GI manifestations in patients with agammaglobulinemia, respectively.
Conclusion: The bowel is exposed to multiple antigens including gut microbiota and pathogens from the environment. Mucosal antibodies play a major role in protection and homeostasis; hence, decreasing level of IgA can provide the opportunity for infectious diarrhea and more pathogen to attack.- انتشار مقاله: 28-04-1398
- نویسندگان: Kawthar Jasim Mohammad Rida Al-Hussieni,Erfan Rasouli,Gholamreza Azizi,Mehdi Mosavian,Rahman Matani,Marzieh Tavakol
- مشاهده