Distribution and protective role of hlab40 in Iranian patients with kawasaki disease; a report from southern Iran

Kawasaki disease [KD] clinically presents as a systemic vasculitis syndrome with significant cardiovascular involvement. With different incidence among different ethnic groups, the role of certain human leukocyte antigens and their products has been considered as a crucial predisposing factor in the immune responses in this disease. We determined the distribution of human leukocyte antigens type B for 90 Iranian patients with Kawasaki disease in order to evaluate a possible association between these antigens and this disease in our area. We used the polymerase chain reaction [PCR] sequence specific primers [PCR-SSP] technique for antigen typing. Distribution of these antigens for 89 healthy Iranians used as control. While 7 [3.9%] of our patients were positive for human leukocyte antigen type B 40, there were 18 [10.1%] subjects from the control group who had this antigen with statistically significant difference between patients and control group [CI= 95%, RR=1.15 and P= 0.02]. Data were analyzed by Pearson chi-square test and Fisher's exact test. SPSS version 15 was used for statistical analysis and a P value less than 0.05 considered statistically significant. The presence of higher frequency of allele type-B40 in the control group may represent a protective role for this antigen with resultant decreased susceptibility to KD in our area

Giant aneurysm of thoracic and proximal abdominal aorta in a patient with common variable immunodeficiency

Common variable immunodeficiency [CVID] is the most common symptomatic primary immunodeficiency disease, predisposing the patients to various tissue involvement and organ damage. Here a 16-year-old boy is presented who was referred to our center with cough, dyspnea, cyanosis, and history of recurrent pneumonia. The diagnosis of CVID was made according to reduction all serum immunoglobulin levels, normal numbers of T, B and NK lymphocyte subpopulations, poor antibodies responses. Considering abnormality in heart examination and chest X-ray, echocardiography and computed tomography angiography were performed which showed large thoraco-abdominal; aortic aneurysm in this patient. Although there are some reports of cardiovascular disease associated with primary antibody deficiencies, this is the first time that such large thoraco-abdominal aortic aneurysm is reported in CVID. This may be secondary to recurrent pulmonary infections or an unknown mutation process. Cardiovascular abnormalities are an entity that should be kept in mind in patients with primary immunodeficiency diseases

Efficacy of carvedilol in patients with dilated cardiomyopathy due to beta-thalassemia major; a double-blind randomized controlled trial

Dilated cardiomyopathy is the end result of chronic iron overload in patients with beta thalassemia major. The of the present study was to evaluate the safety and efficacy of Carvedilol in patients with beta thalassemia major and dilated cardiomyopathy. During a six-month period, fourteen patients with beta-thalassemia major and heart failure without diabetes mellitus referred to pediatric cardiology clinic enrolled in this double blind, randomly assigned study. All patients were on anti failure therapy with Digoxin, Captopril and Furosemide. Carvedilol was started at a dosage of 3.12 mg bid and for patients who had a systolic blood pressure >100 mmHg, heart rate >60/min and no signs of low cardiac output the dosage was increased every two weeks to a maximum of 25 mg bid. Clinical signs and symptoms, systolic and diastolic echocardiographic indexes and Tissue Doppler Imaging [TDI] data were collected from each patient. Eight patients received Carvedilol [Group 1] and six received placebo [Group 2]. The mean age of patients in Group1 and 2 were 16 +/- 0.7 years and 17 +/- 3 years respectively. Only one patent in Group 1 tolerated increasing Carvedilol dosage to more than 6.25 mg bid. Changes in New York Heart Association [NYHA] classification, Ejection fraction, End diastolic dimension changes, TDI systolic[S], early [Ea] and late [Aa] diastolic waves were not statistically significant in these two Groups [P>0.05]. Pulse Doppler E/A wave ratio of mitral valve in Group1 and Group 2 changed from 1.1 +/- 0.37 m/s to 1.8 +/- 0.40 m/s and from 1.34 +/- 0.30 m/s to 2.6 +/- 0.23m/s respectively [P=0.04]. Patients with thalassemia and dilated cardiomyopathy have poor tolerance to increasing Carvedilol dosage and develop decreased systolic blood pressure during advancement of the drug dosage. Carvedilol can be effective in prevention of progression of diastolic dysfunction in these patients