Iranian Journal of Allergy, Asthma and Immunology
Volume:5 Issue: 1, Mar 2006

Abolhassan Farhoudi, professor of pediatrics and clinical immunology in Tehran University of Medical Sciences died on April 19, 2006, at the age of 82. We were terribly shocked when we heard his sudden death. He had been working continuously until last week before his death.

Cystic fibrosis (CF) is the most common inherited disorder in Caucasian populations, with over 1400 cystic fibrosis transmembrane conductance regulator (CFTR) mutations. The type of mutations and their distributions varies widely between different countries and/or ethnic groups. Seventy Iranian cystic fibrosis patients were screened for the CFTR gene mutation using ARMS/PCR (amplification refractory mutation system) for the following mutations: ∆F508, N1303K, G542X, 1717-1G>A, R553X, W1282X, G551D, 621+1G>T, ∆I507 and R560T. Single strand conformation polymorphism (SSCP) analysis of exons 3, 7, 10, 11 and 17b, including both the exon/intron junctions, of the CFTR gene was performed in patients in whom no mutation could be identified on one or both CFTR genes. As a result of this screening, only three mutations were found: ∆F508 mutation was found in 25 (17.8%) alleles, N1303K in six (4.3%) alleles and G542X in five (3.6%) alleles. Thus, a total of 3 mutations cover 25.7% of CF alleles. These finding will be used for planning future screening and appropriate genetic counseling programs in Iranian CF patients.

It has been recognized that acute and chronic stress has an impact on the immune system. Acute stress may have a stimulating effect on the immune system, while in the case of chronic stress specially depression, the immune system could be down-regulated. However, an association between depression and a higher number of circulating white blood cells with increased activity has been reported. Elevation in immune cell numbers and alteration in cytokine profiles are documented for women suffering sporadic spontaneous abortion with a high stress score. In spite of these contradictory results and to make a new approach in immunological (NK activity) as well as psychological parameters (stress/depression) in women suffering from recurrent spontaneous abortion (RSA) the present study was planned. Forty-five women with a history of RSA and a matched control group were participated in this study. A questionnaire for life events known as life change units (LCU) and the Beck Depression Inventory (BDI) outlines were used and the socio-psychological events were recorded after visiting and interview. Fresh peripheral blood lymphocytes were taken as a source of NK activity and K562 cell line were used as NK sensitive target. The experiments were performed and the cells were analyzed with a flow-cytometer. The stress and the depression scores were determined 245±83.6 and 27.6±8.8 for women with RSA and 224±79.6 and 19.4±7.1 for non-RSA group respectively. There was an association between life stress scores and depression scores with r=0.65 and P=0.000 for RSA women. A correlation with r = -0.34 and P = 0.02 was found between depression scores and NK cytotoxicity. The Pearson correlation test showed a lack of relationship between high stress score and NK activity with the r=0.011 and P=0.95, but r= -0.30 and P=0.072 was obtained for high depression scores and NK cytotoxicity. Therefore, it could be suggested that in the case of women with a history of recurrent spontaneous abortion, modulation for immunological parameters (i.e immunotherapy) concurrently with managing psychological aspects (stress/depression) could be modified for the benefit of the patients.

Corticosteroids are recommended for emergency management of an asthmatic attack. This study was designed to compare the effectiveness of oral and intramuscular steroid on spirometric results in acute asthma.We performed a randomized trial involving 88 adults, aged 15-70 years, with acute exacerbation of asthma requiring treatment with steroids. All had been treated with standard bronchodilator regimens and then received oral prednisone, 40 mg/day for 7 days, or 40 mg/day intramuscular triamcinolone long acting (LA) for 3 days. Spirometric variable and percentage of change to baseline forced vital capacity (FVC) and forced expiratory volume in 1 second (FEV1) after treatment were calculated.Baseline characteristic were comparable in the oral prednisone group (n=44) and in the intramuscular triamcinolone LA groups (n=44). After 7 days of treatment, the mean (SD) FEV1 and FVC in both groups improved statistically over baseline values (P<0.001). The median percentage change improvement of FEV1 between two treatment groups was statistically significant: 68±45.3% vs. 53.4±46.5%, P=0.04) respectively, but for FVC although improvement with prednisone was better than intramuscular triamcinolone LA groups, it was not statistically significant (52.6±40.1% vs. 45.8±39.9%, P=0.43) respectively.We conclude that in adults with acute asthma, oral prednisone is more effective than intramuscular triamcinolone LA in improvement of FEV1, but although efficacy of oral prednisone in improvement of FVC is more than intramuscular triamcinolone LA group, this effect is not significant.

The primary immunodeficiency (PI) disorders are abnormalities in development and maturation of the immune system. Individuals with PI disease may experience frequent infections, which limit their abilities to exhibit physical and psychological well-being secondary to their illness. In this survey we compared health-related quality of life of primary immune deficient patients with healthy children. The case-control study was designed for patients with PI disease who were referred to Children Medical Center in 2004-2005. Demographic information was taken and Pediatric Quality Of Life (PEDQOL) questionnaire were filled for 50 PI patients and 100 healthy children. The mean age in PI patients was 12.62± 3.65 (range from 8 to 18) years and in the control group was 11.04± 3.3 years. In PI patients 68% were male and 32% female. Most patients with PI disease had a diagnosis of common variable immunodeficiency (54%) or X-linked agammaglobulinemia (24%). Patients with PI disease had great limitations in physical functioning and psychological well-being (p<0.001 and p<0.001 respectively) compared with children without a chronic health condition. Patients had lower PEDQOL scores in all age groups compared with normal sample (p<0.001). Long duration of disease significantly correlated with low psychological score. (r = -3.23. P= 0.03) Children with PI disease experience poorer health related quality of life than healthy children, indicating more attention should be paid to early diagnosis and treatment of PI disease, as well as more attention to their social limitation. PI patients may need psychological consultation for better coping with their illness.

Allergen immunotherapy involves the administration of gradually increasing quantities of specific allergens to patients with IgE-mediated conditions until a dose is reached that is effective in reducing disease severity from natural exposure. In the present study we evaluated a period of six years immunotherapy allergic rhinitis and allergic asthma patients with positive skin prick test of common aeroallergen. The immunotherapy was performed on 156 patients. One hundred twenty of the cases were allergic rhinitis (80%), 29 cases had allergic asthma and 7 cases were mixed (4.5%). 70% in allergic rhinitis group, 75% in allergic asthma group and 42.8% in mixed group completely improved. Immunotherapy, an older therapeutic method, has now been updated, and with appropriate indications, precautions and methods, has been clearly shown to be effective in the treatment of allergic rhinitis and in some cases of asthma and insect hypersensitivity.

Many investigations have proved relations between ABO blood groups with some diseases and factor VIII and von willebrand level in plasma. In this study we investigated a relation between ABO blood groups and factor VIII and IX inhibitors in 102 patients with haemophilia A and 48 patients with haemophilia B. The assay of inhibitor was done by Bethesda method. There were no relation between ABO blood groups and factor VIII and IX inhibitors.

There are few reports about congenital indifference to pain or Hereditary and Sensory Autonomic Neuropathy (HSAN). Several investigations for pathophysiology of this syndrome have been performed and different classifications about it. In this report we present a case of HSAN type II with general absence of pain and self amputations and leprosy–like damage of extremities which was suspected to be phagocytic immunodeficiency due to past history of repeated ulcer and abscess formation.

Polyclonal anti-thymocyte globulin (ATG) is used as an immunosuppressive agent in the treatment of aplastic anemia (AA). Serum sickness is a recognized side effect of ATG. We observed abnormal skin manifestation in patient with aplastic anemia who had been treated with ATG. We conclude that abnormal immune function caused by aplastic anemia and ATG and corticosteroids may aggravate the signs of serum sickness.