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کاربرد نوع شرط:
- جایگاه : پژوهشی
- مجله: Iranian Journal of Immunology
- نوع مقاله: Journal Article
- کلمات کلیدی: Hereditary Angioedema,C1 Inhibitor,Complement System
- چکیده:
- چکیده انگلیسی: Background: Hereditary angioedema (HAE) is a rare autosomal dominant primary immunodeficiency with complement system defect characterized by recurrent episodes of angioedema involving the skin or mucosa of the upper respiratory and gastrointestinal tracts.
Objective: To characterize the clinical and laboratory data of hereditary angioedema in Iran.
Methods: Patients with probable diagnosis of angioedema were enrolled in this study. Demographic and clinical data were documented in the designed questionnaire including history of attacks, triggering factors and laboratory data such as C4, C1 esterase inhibitor level and function.
Results: Among 63 patients who were clinically suspicious for angioedema (23 males and 40 females), 8 cases (12.7%) were diagnosed with HAE. Among these 8 HAE patients, 3 were diagnosed with HAE type 1 and five patients were diagnosed with HAE type 2. The mean ages of HAE type 1 and type 2 patients were 25.6 ± 13.5 and 22.4 ± 12.32 years. The mean age of onset in HAE type 1 group was 8 ± 5 years and in HAE type 2 group was 18.8 ± 11.84 years. The mean diagnosis delay was 17.6 years in HAE type 1 patients and 2.6 years in HAE type 2. The most common clinical manifestation was facial swelling presented in all HAE patients followed by swelling of extremities which was present in 7 patients with HAE.
Conclusion: The clinical criteria of the Iranian patients with HAE were consistent with the known clinical patterns of the disease.- انتشار مقاله: 14-05-1395
- نویسندگان: Shervin Shahinpour,Marzieh Tavakol,Hassan Abolhass Ani,Payam Mohammadinejad,Masoud Movahedi,Saba Arshi,Asghar Aghamohammadi
- مشاهده
- جایگاه : پژوهشی
- مجله: Iranian Journal of Immunology
- نوع مقاله: Journal Article
- کلمات کلیدی: Diagnosis,Prevalence,Primary Immunodeficiency Disorders
- چکیده:
- چکیده انگلیسی: Background: Primary immunodeficiency disorders (PID) are a group of hereditary disorders characterized by an increased susceptibility to severe and recurrent infections, autoimmunity, lymphoproliferative disorders, and malignancy.
Objective: To evaluate the demographic and clinical data of PID patients diagnosed in a referral pediatric hospital.
Method: All PID cases with a confirmed diagnosis, according to the criteria of International Union of Immunological Societies, who were referred to the Children’s Medical Center in Tehran, Iran, between March 2006 and March 2013 were enrolled in this retrospective cohort study.
Results: Three-hundred and seven PID patients were investigated. Predominantly antibody deficiencies were the most common group of PID observed in 118 cases (38.4%), followed by the well-defined syndromes with immunodeficiency in 52 (16.9%), congenital defects of phagocyte in 45 (14.7%), combined immunodeficiencies in 36 (11.7%), autoinflammatory disorders in 34 (11.4%), immune dysregulation in 11 (3.6%), complement deficiencies in 7 (2.3%), and defects in innate immunity in 3 (1%). Selective IgA deficiency was the most prevalent disorder which affected 46 individuals (14.9%). The median diagnostic delay was 15 months.
Conclusion: Increased awareness and availability of diagnostic tests could result in the better recognition of more undiagnosed PID cases and a decrease in diagnostic delay.- انتشار مقاله: 15-05-1395
- نویسندگان: Payam Mohammadinejad,Babak Mirminachi,Bamdad Sadeghi,Masoud Movahedi,Mohammad Gharagozlou,Javad Mohammadi,Hassan Abolhassani,Nima Rezaei,Asghar Aghamohammadi
- مشاهده
- جایگاه : پژوهشی
- مجله: International Journal of Pediatrics
- نوع مقاله: Journal Article
- کلمات کلیدی: Pneumonia,bronchiectasis,Children, Cystic fibrosis,Primary ciliary dyskinesia
- چکیده:
- چکیده انگلیسی: Background
Bronchiectasis is defined by permanent and abnormal widening of the bronchi. Although this process occurs in the context of chronic airway infection and inflammation, since there is no accurate estimation of the etiology of the disease. This study aimed to determine the most important cause of bronchiectasis in Tehran, Iran.
Materials and Methods
In this retrospective cohort study we used the information of 91 patients admitted to two subspecialty lung hospitals in Tehran-Iran, where a wide range of bronchiectasis patients from around the country referred during 2013to 2014 period. Patients referring with the manifestation of chronic productive cough who had not responded to conventional treatment with the evidences of bronchiectasis on high resolution computed tomography were included. Data were analyzed using SPSS-16.0.
Results
The etiology of bronchiectasis was diagnosed in 73 of 91 patients (80.2%), the most important of which included cystic fibrosis, post infectious, and primary ciliary dyskinesia (PCD). The most common causes of bronchiectasis in the children group (Age ≤ 18 years), were cystic fibrosis (57.1%), allergic bronchopulmonary aspergillusis (14.3%) and PCD (9.5%), respectively. In the adults group (Age >18 years), the most common causes were post infectious (22.6%), PCD (15.7%) and cystic fibrosis (14.3%), respectively.
Conclusion
Main causes of bronchiectasis in this study were not significantly different from other studies. Special attention should be paid to the probable causes of bronchiectasis in order to effectively execute on-time diagnosis, proper treatment and management of complications.- انتشار مقاله: 23-08-1395
- نویسندگان: Seyed Javad Sayedi,Payam Mohammadinejad,Mohammadreza Modaresi,Gholamreza Azizi,Seyed Alireza Mahdaviani,Asghar Aghamohammadi
- مشاهده
- جایگاه : پژوهشی
- مجله: International Journal of Hospital Research
- نوع مقاله: Journal Article
- کلمات کلیدی: Coronary Artery Disease,patient safety,Patient outcome,CABG,Therapeutic Angiogenesis,Fibroblast Growth Factor,Patient management
- چکیده:
- چکیده انگلیسی: Background and Objectives: Complete revascularization is not possible in up to 37% of patients with coronary artery disease (CAD). Therapeutic angiogenesis may be considered as an option in the management of these patients. The aim of the present study was to evaluate the effectiveness and safety of therapeutic angiogenesis using basic fibroblast growth factor in patients with CAD.
Methods: In this double-blind, placebo-controlled study, eighteen patients with a severe diffuse atherosclerotic disease along the left anterior descending (LAD) artery who were a CABG candidate with at least one graftable coronary artery and the presence of ischemia and viable areas along the LAD were enrolled. The patients were randomized into two groups to either undergo CABG and simultaneous FGF-2 therapy (bFGF group) or CABG without FGF-2 therapy (control group). During the CABG procedure in bFGF group, FGF-2/alginate-heparin-sepharose microcapsules, each contains 100 mcg FGF-2, were implanted in the subepicardial layer of the diffusely defective LAD territory via 2-3 mm stab incisions. Seven patients in each group were followed up for a period of 24 months.
Findings: The result of left ventricular evaluation with echocardiography and perfusion scans showed significant improvement in FGF-2-receiving group with no significant change in controls, 3 and 6 months after the intervention. New Yor; Heart Association (NYHA) class was significantly lower in the intervention group (1.43±0.535 vs. 2.57±.535, P = 0.002), In addition, intervention group remained free of angina 24 months after the intervention while three patients in the control group were hospitalized due to the acute chest pain.
Conclusions: Our study revealed that FGF-2 can improve the outcomes of patients with CAD undergoing CABG, without serious adverse effects. Considering other advantages associated with protein therapy our finding may help open novel avenues to safe and cost-effective therapy of the target patients.- انتشار مقاله: 19-05-1395
- نویسندگان: Hossein Ahmadi,Armita Mahdavi,Morteza Daliri,Mohammad Imani,Abbasali Karimi Karimi,Abbas Salehi Omran,Mehdi Najafi,Mohsen Ahmadi,Farhad Fathi,Pegah Bikdeli,Payam Mohammadinejad
- مشاهده
- جایگاه : پژوهشی
- مجله: Immunology and Genetics Journal
- نوع مقاله: Journal Article
- کلمات کلیدی: Chronic Granulomatous Disease,Unilateral Flaccid Paraplegia,Associated
- چکیده:
- چکیده انگلیسی: Chronic Granulomatous Disease (CGD) can be associated with several neurological complications. Abscess in the brain in patients diagnosed with CGD has been reported in several publications. Different pathogens have been linked with brain abscess development including fungal or bacterial infections. Other neurologic complications may include white matter disease and formation of a granulomatous lesion in the central nervous system. In addition to these common reports, observation of leptomeningeal, along with focal brain infiltration by pigmented, lipid-laden macrophages, fungal brain infection, Aspergillus abscess resembling a brain tumor, spinal cord infection by Aspergillus, and fungal granuloma of the brain have also been described. Physicians should be aware of Streptococcus- and Candida-induced meningitis in a selected group of CGD patients as well. Herein, we report a case of the attenuated ill-defined lesion in the right cerebellar hemisphere in a CGD patient without involvement of the sinuses or lungs.
- انتشار مقاله: 30-01-1398
- نویسندگان: Shabnam Pourhamdi,Payam Mohammadinejad
- مشاهده