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Query: KEGG:D04296 (Asthma)
 25,733 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Bronchiectasis is a chronic debilitating condition characterized by abnormal dilated thick-walled bronchi. To investigate humoral immune function in bronchiectatic patients, this study was performed. Forty patients with established diagnosis of bronchiectasis, who were referred from two tertiary care pulmonology centers in Tehran, were investigated in this study. Immunoglobulin isotypes concentrations and IgG-subclasses were measured by nephelometry and enzyme-linked immunosorbent assay (ELISA) methods, respectively. All patients received unconjugated pneumococcal vaccine, and blood samples were taken before and 21 days after vaccination. Specific antibodies against whole pneumococcal antigens were measured using the ELISA method. Fifteen (37.5%) out of 40 patients were diagnosed to have defects in antibody mediated immunity including 5 (12.5%) patients with immunoglobulin class deficiency (2 with common variable immunodeficiency and 3 with IgA deficiency), 3 (7.5%) with IgG subclass deficiency and 7 (17.5%) patients had Specific antibody deficiency (SAD) against polysaccharide antigen despite normal levels of serum immunoglobulins and IgG subclasses. Our study along with several other studies confirmed that all patients with bronchiectasis should undergo thorough immunological evaluation in order to identify the presence of the underlying immunologic defect. This evaluation should include serum immunoglobulins, IgG subclasses concentrations and also determination of serum antibodies against pneumococcal antigens. Early diagnosis and appropriate treatment will prevent the subsequent complications and improve quality of life of affected individuals.
Iran J Allergy Asthma Immunol 2008 Jun
PMID:Evaluation of humoral immune function in patients with bronchiectasis. 1855 8

Hyper-immunoglobulin E syndrome is a rare primary immunodeficiency disease characterized by recurrent abscess formation, respiratory tract infections and very high titers of serum IgE associated with peculiar face and skeletal features. We report a seven-year old girl presenting with persistent productive cough and history of chronic eczematoid facial lesions since infancy and two episodes of hospitalizations due to pneumonia and perianal abscess. Additionally, in physical examination finger tip clubbing, laxity of joints and crackles in both lungs were detected. Immunologic work up revealed markedly raised IgE level and eosinophilia. The patient was diagnosed as hyper IgE syndrome based on his clinical and laboratory findings. Chest X-ray revealed multiple large cystic lesions in left lung which were confirmed by spiral CT-scan. Pneumonectomy specimen examination showed cystic adenomatoid malformation, characterized by the presence of various cysts lined by epithelium in different sizes. There are few reports of cystic adenomatoid malformation in children. To our best known, this is the first report of cystic adenomatoid malformation in a child with hyper IgE syndrome. Early diagnosis and surgical therapy are helpful in prevention of repeated infections in these patients.
Iran J Allergy Asthma Immunol 2008 Jun
PMID:Histopathologic Findings of Pneumatocele in a Patient with Hyper-IgE syndrome, compatible with cystic adenomatoid malformation. 1855 13

Hyper-IgE syndrome (HIES) is a complex primary immunodeficiency characterized by high serum IgE, chronic eczematoid dermatitis, and recurrent extracellular bacterial infections. Two types of HIES have been reported: type 1 and type 2. Type 1 HIES displays abnormalities in multiple systems, including the skeletal, dental, and immune systems, whereas type 2 shows abnormalities confined to the immune system. We recently identified hypomorphic mutations in the signal transducer and activator of transcription 3 (STAT3) gene in type 1 HIES and a null mutation in the tyrosine kinase 2 (Tyk2) gene, accompanied by susceptibility to intracellular bacteria in type 2 HIES. Analyses of cytokine responses in both types of HIES revealed that severe defects in the signal transduction for multiple cytokines, including interleukin-6 and interleukin-23, are leading to impaired T-helper type 17 function. These findings suggest that HIES is caused by the defects in multiple cytokine signals and that the susceptibility to various infections in HIES is associated with the T-helper type 17 defect.
Curr Allergy Asthma Rep 2008 Sep
PMID:Genetic origins of hyper-IgE syndrome. 1868 2

The 23-valent-polysaccharide pneumococcal vaccine (PPV23) is currently recommended for patients at high risk for invasive disease from Streptococcus pneumoniae. It is also frequently used in the evaluation of patients with suspected immunodeficiency. Reports of systemic adverse reactions are rare. Our objective is to describe a patient with an apparent systemic reaction to PPV23 and review our hospital's 2-year experience with pneumococcal vaccine. Chart review of 173 patients given PPV23 between January 1, 2004 and December 31, 2005 revealed five who had significant adverse reactions, including local cellulitis, fever, and vomiting. Variables considered included age at immunization, indication for PPV23, and prior pneumococcal vaccines. The mean age of all patients given PPV23 during the defined time period was 8.6 years, and the most common indication for vaccination was recurrent infection. Of those patients who had adverse reactions, the mean age was 6.5 years. The time from prior pneumococcal vaccination was a mean of 20 months in children who did not have an adverse reaction. None of the patients who had an adverse reaction had documentation of receiving PPV23 before. Local reactions to PPV23 occur in approximately 50% of recipients, and revaccination of immunocompetent individuals increases the risk for local reaction. Systemic reactions are less common and only occur in approximately 1% of recipients. Our patient had no identifiable risk factors for development of an adverse reaction. Additional studies are indicated to determine whether there are identifiable risk factors for the development of adverse reactions to PPV23.
Allergy Asthma Proc
PMID:Systemic reaction to pneumococcal vaccine: how common in pediatrics? 1870 88

Immunodeficiency and autoimmune disease may occur concomitantly in the same individual. Some of the immunodeficiency syndromes, especially humoral defects are associated with autoimmune disorders. Hematological manifestations such as thrombocytopenia and hemolytic anemia are the most common presentations. Persistent antigen stimulation due to an inherent defect in the ability of the immune system to eradicate pathogens is the primary cause leading to autoimmunity in patients with primary immunodeficiency states.We describe a 10 year old Iranian girl with chronic granulomatous disease -the autosomal recessive type with mutation of NCF1 gene P47- associated with selective IgA deficiency, refractory immune thrombocytopenia that showed an excellent response to Rituximab (Anti-CD20 monoclonal antibody).Patients with primary immunodeficiencies may have variable autoimmune manifestations. So for early detection and appropriate treatment, autoimmune diseases should always be suspected in such patients.
Iran J Allergy Asthma Immunol 2008 Sep
PMID:Autosomal recessive chronic granulomatous disease, IgA deficiency and refractory autoimmune thrombocytopenia responding to Anti-CD20 monoclonal antibody. 1878 Sep 54

Otitis media is one of the most common childhood infections and may result from a variety of underlying problems. Suspicion of immunodeficiency should increase when ear infections are frequent; suppurative; unresponsive to antibiotics; caused by unusual organisms; or seen in the context of other frequent infections, severe eczema, or failure to thrive. Humoral immune deficiencies, particularly with an inability to make antibody to encapsulated organisms, are the immunodeficiencies most likely to cause increased otitis media. Immune system evaluation should concentrate on humoral immunodeficiency disorders, but the presenting history and physical findings also should be considered when designing the work-up. Treating the underlying immune deficiency is usually necessary to adequately control the ear infections.
Curr Allergy Asthma Rep 2008 Nov
PMID:Otitis media as a presenting complaint in childhood immunodeficiency diseases. 1894 Jan 44

Selective IgA deficiency (IgAD) (serum IgA concentration of <0.07 g/l) is the most common primary immunodeficiency in Caucasians, with an estimated prevalence of 1/600. There are strong indications for involvement of genetic factors in development of the disease and the frequency of several extended major histocompatibility complex haplotypes (including HLA-A1, B8, DR3, DQ2) have previously been shown to be increased among Caucasian patients with IgAD.PCR was used to type HLA B, DR, and DQ alleles in 29 Iranian individuals with IgAD and 299 Swedish individuals with IgAD.The results indicate a strong association with the HLA B14, DR1 alleles in Iranian subjects and HLA B8, B12, B13, B14, B40, DR1, DR3, DR7, DQ2 and DQ5 alleles in Swedish subjects.Differences in HLA association of IgAD in Iran and Sweden confirm the notion of a genetic background of the disease and that multiple, potentially different genes within the MHC region might be involved in the pathogenesis of IgAD in different ethnic groups.
Iran J Allergy Asthma Immunol 2008 Dec
PMID:Human leukocyte antigens (HLA) associated with selective IgA deficiency in Iran and Sweden. 1905 50

X-linked Agammaglobulinemia (XLA) is a hereditary immunodeficiency, characterized by an early onset of recurrent bacterial infections, hypogammaglobulinemia and markedly reduced B lymphocytes number. In order to determine the association of neutropenia among Iranian patients with XLA, hospital records of 30 patients with confirmed XLA in Children Medical Center Hospital, were reviewed. Eight out of 30 XLA patients (26.7%) developed neutropenia during the course of the disease. In two patients, episodes of neutropenia were identified before or at the time of diagnosis of XLA. Other six patients whom were not visited regularly and did not receive periodical immunoglobulin replacement therapy experienced neutropenia after diagnosis of XLA. Neutropenia in XLA is mainly associated with infection and is resolved with intravenous immunoglobulin replacement and antibiotics therapy.
Iran J Allergy Asthma Immunol 2009 Mar
PMID:Neutropenia associated with X-linked Agammaglobulinemia in an Iranian referral center. 1927 58

Elevated serum immunoglobulin E(IgE) can be caused by allergies, infections and immune conditions including hyper IgE syndrome (HIES). HIES is a rare primary immunodeficiency disease most commonly characterized by a triad of findings, including increased serum IgE levels, recurrent skin abscesses, and pneumonias leading to pneumatocele formation. The objective of this study was to characterize the clinical profile of patients presenting with increased IgE levels (>or=2000 IU/mL) focusing specifically on HIES. A database search identified 70 patients in the pediatric age range (<or=18 yrs.) between January 1997 and December 2006 who had an IgE level of >or=2000 IU/mL. Charts were abstracted for clinical diagnosis, comorbidities, and laboratory parameters. Data were analyzed using the students t-test, Wilcoxon signed rank test, and univariate/multivariate regression models. Clinical diagnosis in 70 patients with elevated IgE levels were: atopic diseases (n = 54; 77%), parasitic diseases (n = 1; 1.5%), malignancy (n = 2; 3%), and HIES (n = 6; 8%), among other causes. There was a statistically significant association between IgE levels and the severity of eczema (p = 0.009). Ninety percent of the subjects with IgE level >or=2000 IU/mL did not have HIES. There was no correlation between IgE levels and the diagnosis of HIES (p = 0.5). A variety of clinical situations result in an elevated IgE level, with atopy being the most common cause. In the absence of typical clinical features, elevated serum IgE levels are not predictive of HIES.
Allergy Asthma Proc
PMID:Elevated serum immunoglobulin E (IgE): when to suspect hyper-IgE syndrome-A 10-year pediatric tertiary care center experience. 1933 17

There is little data in the literature regarding outpatient consultation in allergy/immunology (A/I). The purpose of this study was to determine the relative frequency of different reasons for A/I outpatient consultation to help guide graduate medical education (GME) and assist with A/I practice management. We retrospectively reviewed the electronic medical records of all outpatient A/I consultations from January 1, 2006 to December 31, 2006. The study was performed at our tertiary care referral center which is a GME training site. There were 1412 A/I consults requested during the 1-year period. The consults per month ranged from a low of 69 to a high of 157. The referrals consisted of 35% pediatric and 65% adult patients. There were 52.8% female and 47.2% male patients. We received 74.3% of referrals from primary care, 19.8% from specialty care, and 5.9% from the emergency department. The most common reasons for consultation included 808 (57.2%) patients for chronic rhinitis, 288 (20.4%) for asthma, 196 (13.9%) for food allergy, 89 (6.3%) for venom allergy, 68 (4.8%) for atopic dermatitis, 66 (4.7%) for drug allergy, 62 (4.4%) for chronic urticaria, 45 (3.2%) for acute urticaria, 34 (2.4%) for immunodeficiency, 31 (2.2%) for anaphylaxis, and 162 (11.5%) for other reasons. More than one reason was given for 27.1% of consults, and there was an average of 1.3 reasons for consultation per patient. Although the allergist/immunologist is consulted for a variety of reasons, the top three reasons make up a majority of outpatient consults, and consults are often requested to address more than one diagnosis.
Allergy Asthma Proc
PMID:Reasons for outpatient consultation in allergy/immunology. 1933 22


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