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Congenital neutropenia and primary immunodeficiency diseases
Critical Reviews in Oncology/Hematology, 2019Neutropenia is a dangerous and potentially fatal condition that renders patients vulnerable to recurrent infections. Its severity is commensurate with the absolute count of neutrophil granulocytes in the circulation. In paediatric patients, neutropenia can have many different aetiologies.
Hamid Farajifard+3 more
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Immunoglobulin A and microbiota in primary immunodeficiency diseases
Current Opinion in Allergy & Clinical Immunology, 2019Purpose of review With the emergence of the microbiota as a potential driver of host inflammation, the role of iIgA is becoming increasingly important. This review discusses the current evidence regarding the effects of clinical IgA deficiency on the microbiota, and the possible role of microbial dysbiosis in driving ...
Berbers, Roos Marijn+2 more
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Liver disease in children with primary immunodeficiencies
Journal of Hepatology, 2001To investigate clinical features and to establish optimal management in children with primary immunodeficiency (PID) and liver disease. Study design A retrospective analysis of medical records of 147 children with PID who presented with abnormal liver tests to a tertiary center.Clinical evidence of liver disease was documented in 35 (23.8%) patients ...
Rodrigues, F+9 more
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Introduction on Primary Immunodeficiency Diseases
2016Primary immunodeficiency diseases (PIDs) are a heterogeneous group of disorders with defects in one or more components of the immune system. Primary immunodeficiency diseases could suffer from a variety of clinical manifestations, including recurrent, severe or unusual infections, autoimmunity, and lymphoproliferation/malignancies.
Jordan S. Orange+8 more
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Autoimmunity in human primary immunodeficiency diseases
Blood, 2002AbstractHuman primary immunodeficiency diseases are experiments of nature characterized by an increased susceptibility to infection. In many cases, they are also associated with troublesome and sometimes life-threatening autoimmune complications.
Arkwright, Peter D.+2 more
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Immunotherapy for Primary Immunodeficiency Diseases
Medical Clinics of North America, 2012The 2 most commonly encountered primary immunodeficiency syndromes in adult practice are antibody deficiency disorders and hereditary angioedema.Immunologic therapy for these disorders has significantly improved patient management. Therapy with immunoglobulin leads to improvement in overall quality of life. With increasing survival rates and decreasing
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The Primary Immunodeficiency Diseases
2016Patients with primary immunodeficiency diseases most often are recognized because of their increased susceptibility to infection (chronic or recurrent infections without other explanation, infection with an organism of low virulence, or infection of unusual severity). The type of pathogen and the location of the infection may give valuable insight into
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Neurological Manifestations of Primary Immunodeficiency Diseases
Clinical Pediatrics, 2017WOS: 000432052200001 PubMed: 29117743 …
Aydin, Omer Faruk, Anlar, Banu
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Primary immunodeficiency diseases in Norway.
Journal of clinical immunology, 2000This study represents the first national epidemiological survey of primary immunodeficiency diseases in Norway. Uniform questionnaires were sent out in April 1998 to all hospital departments considered relevant. As of February 1999, a total of 372 patients have been registered, of whom 69 patients are deceased. With a population of 4.45 million people,
Tore G. Abrahamsen+2 more
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Primary Immunodeficiencies and Immune Diseases
2020Immunodeficiency is a heterogeneous group of diseases caused by one or more alterations in the immune system. These diseases may be primary, associated with genetic disorders, or acquired during life. Their most common clinical manifestation tends to be predisposition to infections.
Eduardo Talesnik Guendelman+1 more
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