neutropenia is characterized by decrease in the absolute number of circulating neutrophils and an increase susceptibility to infections. the current study was performed in order to explain the clinical and laboratory findings of patients with antibody deficiency disorders associated neutropenia. the patients' records of 19 neutropenic cases out of 207 patients with antibody deficiencies, who ha...

Abstract Mycobacterium abscessus appears to be increasing cause of pulmonary infection in children with underlying risk factors including cystic fibrosis, chronic lung disease and immunodeficiency syndromes. We present a case M. pediatric patient primary ciliary dyskinesia he was successfully treated parenteral amikacin, linezolid oral clarithromycin combined inhaled amikacin. Clinical improvem...

Primary Immune Deficiencies are a group of heterogeneous disorders that involve the innate or acquired immune system, or a combination of them. The underlying disorder may be related to decreased levels or function, or a complete lack of one or more components of the immune system in general. These diseases can occur with a prevalence of about 1 in 10000 live births. According to the fourth upd...

chronic granulomatous disease (cgd) is a rare primary immunodeficiency disorder due to a genetic defect in one of the components of nicotinamide adenine dinucleotide phosphate (nadph) oxidase complex. this complex is composed of membrane-bound gp91- phox and p22- phox subunits, and cytosolic subunits consisting of p47- phox , p67- phox , and p40- phox . a mutation in cybb gene encoding gp91- ph...

The primary immunodeficiency diseases are a heterogeneous group of over 50 disorders which affect the cells, tissues and proteins of the immune system. These disorders were originally felt to be rare, to occur only in infants and young children and to be associated with severe clinical symptoms. However, as clinical experience with the primary immunodeficiency diseases has grown, it has become ...

in this case report we will describe a rare association between anhyrotic ectodermal dysplasia (aed) and immunodeficiency and autoimmunity [in our case: idiopathic thrombocytopenic purpura (itp) and crohn disease]. aed is a rare congenital disorder characterized by sparse hair, abnormal teeth and anhidrosis due to lack of eccrine glands. the survey of 87 cases with (aed) revealed only one irrit...