severe congenital neutropenia is one of primary immunodeficiency disorders that characterized by severe neutropenia and is associated with severe systemic bacterial infections from  early  infancy.  granulocyte  colony  stimulating  factor  (gcsf)  is  clinically  used  as  a treatment for congenital and acquired neutropenia. the aim of this study was evaluation of gcsf (pd- grastim) in treatme...

Tuberculosis is a high-incidence public health problem in Peru. In our context, seen commonly as pulmonary infection. Extrapulmonary tuberculosis gastric rare, both primary or secondary infection, with just few case reports published about these kinds of presentations. This report presents 44-year-old female patient weight loss, dyspepsia, and abdominal pain. Upper endoscopy reveals antral poly...

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Bacille Calmette-Guerin (BCG) vaccine is administered to all newborns in countries where tuberculosis is still endemic. It is a live attenuated vaccine and considered quite safe in immunocompetent children. Disseminated BCG disease is the most serious complication seen only in individuals with underlying primary or secondary immunodeficiencies. We report a case of disseminated BCG disease in an...

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...

primary immunodeficiency disorders are a heterogeneous group of genetic disorders, with different modes of inheritance, consisting of more than 100 different types. we constructed the dna banking of primary immunodeficiency disorders for the first time in iran. the dna of 31 immunodeficient patients and their families (total of 92 samples) were collected, as the first step for construction of d...

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 clinical features of a 7-year-old girl with enamel hypoplasia secondary to autoimmune hypoparathyroidism and chronic mucocutaneous candidiasis are detailed. The combination of features are typical of a rare, probably genetically determined immunodeficiency termed candidiasis endocrinopathy syndrome (CES). Affected individuals have chronic mucocutaneous candidiasis and a spectrum of autoimmu...

Dynamic interactions between B and T cells underpin the development of adaptive humoral immune responses to infections and vaccines. Recent advances in the molecular and spatiotemporal control of these interactions during primary responses have contributed greatly to elucidating the molecular pathogenesis of numerous immunodeficiency and autoimmune diseases. The next challenge is to determine h...