نتایج جستجو برای: antibody deficiency syndrome
تعداد نتایج: 889912 فیلتر نتایج به سال:
The Bernard-Soulier syndrome is an inherited bleeding disorder that is due to a deficiency in platelet glycoprotein Ib. Bernard-Soulier platelets fail to agglutinate in response to ristocetin despite normal levels of factor VIII:von Willebrand factor. We report a patient who developed severe refractory thrombocytopenia postsurgically while receiving procainamide therapy. Thrombocytopenia was im...
The Bernard-Soulier syndrome is an inherited bleeding disorder that is due to a deficiency in platelet glycoprotein lb. Bernard-Soulier platelets fail to agglutinate in response to ristocetin despite normal levels of factor Vlll:von Willebrand factor. We report a patient who developed severe refractory thrombocytopenia postsurgically while receiving procainamide therapy. Thrombocytopenia was im...
The clinical and laboratory combination of recurrent infections due to antibody deficiency, spondyloepiphyseal dysplasia, growth retardation and retinal dystrophy is novel. Four patients with strikingly similar phenotypes from three different families of diverse genetic backgrounds are described, suggesting a similar underlying genotype. Increased awareness of this syndrome will hopefully lead ...
Antibody deficiencies constitute the largest group of symptomatic primary immunodeficiency diseases. In several patients, mutations in CD19 have been found to underlie disease, demonstrating the critical role for the protein encoded by this gene in antibody responses; CD19 functions in a complex with CD21, CD81, and CD225 to signal with the B cell receptor upon antigen recognition. We report he...
and the rest of the family were still in the Marquesas Islands and could not be examined. Heparin treatment (Calciparine, Du Pont) associated with AT-III was administered on April 27. Despite anticoagulant therapy, pulmonary embolism occurred, resulting in an abortion and inferior vena cava occlusion. Currently, the patient is taking oral anticoagulants and has speech and gait defects. One of t...
Abstract Background and Objective: The patients with Post-Infectious Irritable bowel syndrome (PI-IBS), a subtype of irritable bowel syndrome, suffer from bacterial gastroenteritis. Since campylobacter Jejuni (CJ) is one of the most common agents in this syndrome, we aimed to evaluate the prevalence of Campylobacter Jejuni in patients with Irritable Bowel Disease. Material and Methods: Th...
Coincidence of autoimmune diseases such as immune thrombocytopenic purpura (ITP) with immunodeficiencies has been reported previously in patients who suffered from primary antibody deficiency (PAD). But there is no original study on immunological profiles of ITP patients to find out their probable immune deficiency. In this case-control study, ITP patients' humoral immunity was investigated for...
Several studies have reported a possible link between antiaquaporin- 4 antibody and vitamin B12 deficiency in neuromyelitis optica spectrum disorder (NMOSD). Bilaterally symmetric hyperintense signals on magnetic resonance imaging (MRI) of the posterior columns, called inverted V sign, are characteristic feature subacute combined degeneration associated with deficiency. We report patient anti-a...
BACKGROUND Antibody deficiency comprises a heterogeneous group of disorders characterised by the body's inability to mount an effective antibody response to pathogens. Although it has been reported that asthma and allergic disease are frequent in antibody deficiencies, there are no data that evaluate and compare bronchial hyperreactivity (BHR) in all groups of antibody deficiencies. In this stu...
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