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BACKGROUND Primary antibody deficiency, the most common primary immunodeficiency disorder, represents a heterogeneous spectrum of conditions caused by a defect in any critical stage of B cell development and is characterized by impaired production of normal amounts of antigen-specific antibodies. OBJECTIVE This retrospective study aimed at description and analysis of demographic, clinical, im...

primary immunodeficiency diseases (pids) consist of a group of genetic disorders that predispose the patients to immune-mediated complications. the aim of this study was to assess the knowledge of iranian general practitioners and pediatricians about pids. a questionnaire consisting 52 closed questions on clinical symptoms, laboratory data,associated syndromes and management of pids patients wa...

hyper ige syndrome (hies) is a rare primary immunodeficiency disease. most of hies cases are sporadic. hies type ad is caused by mutation in signal transducer and activator of transcription-3 (stat-3). a number of mosaicism hies has been reported that is associated with intermediate phenotype. autosomal recessive hies (ar-hies) is due to mutation in dock-8 or cytokine sis 8 and tyk2 or tyrosine...

The International Union of Immunological Societies now recognizes over 430 single gene inborn errors immunity, making the investigation suspected immunodeficiency in children increasingly complex. use immunomodulatory therapies, including biologics, may also cause both transient and permanent secondary immunodeficiency. This review outlines a practical approach to guide paediatric doctors allie...

Background Microcephalic osteodysplastic primordial dwarfism type I (MOPD I, also known as Taybi-Linder syndrome) is a rare genetic disorder associated with severe intrauterine growth retardation, short stature, microcephaly, brain anomalies, stunted limbs, and early mortality. RNU4ATAC, the gene responsible for this disorder, does not encode protein but instead U4atac small nuclear RNA (snRNA)...

Background: Scleroderma is a chronic systemic disorder that affects the connective tissues. It is characterized by several immune manifestations, inflammation, vascular damage, and fibrosis. Some of the viral infections with complex mechanisms are involved in the development and progression of many autoimmune diseases, such as scleroderma. The present study aimed to investigate the serological ...

AIDS (Acquired Immunodeficiency Syndrome) is a condition caused by the Human Virus (HIV). HIV virus that attacks immune system and weakens body's ability to fight off infections diseases. Approximately 37.6 million people (about twice population of New York) worldwide are infected with human immunodeficiency (HIV) according data from Joint United Nations Program on HIV-AIDS.

objective: based on pittsburg sleep quality index, it has been reported that most human immunodeficiency virus (hiv) positive patients suffer from various degrees of sleep problems. sleep disorders can affect quality of life, physical and social functioning and can also cause chronic fatigue. some psychological and physiological factors are related to sleep quality. the purpose of the present s...