Vol 11, No 1 (2012)

Articles

  • XML | PDF | downloads: 538 | views: 757 | pages: 1-13

    CD40 is recognized as a member of tumor necrosis factor receptor super family. It is expressed by the immune and non-immune cells. Its interaction with CD40 ligand (CD154) brings about  a regulatory effect on  the cellular and humoral immunity. The pathway of CD40-CD154 is influential in various diseases. Investigations on such diseases have revealed dimensional mechanisms whereby this route intensifies host protection. Moreover, through these mechanisms, pathogens subvert the signaling of the CD40, conditions in which the CD40–CD154  pathway promotes  disease and  also through  the  relevant modulation  for immunotherapy.
    This review focuses on the role of CD40–CD40L (CD154) interactions in dendritic cells (DCs) regulation, tolerogenic dendritic cells, role of CD40 in autoimmune disease, allograft rejection and induction of tolerance by down regulation of CD40. According to these roles, it is assumed that  CD40  is a functional molecule in the  pathologies of  conditions  like autoimmune diseases and allograft rejection caused by activated T and B cells.

  • XML | PDF | downloads: 319 | views: 542 | pages: 15-21

    The  non-classical MHC  class-I mainly involves in the  regulation of  innate  immune responses where HLA-E  plays a significant role in the cell identification by natural killer cells. HLA-E is a main regulatory ligand for natural killer cells and given the importance of these effector cells in hematopoietic stem cell transplantation, we investigated the effect of HLA-E polymorphisms on post-hematopoietic stem cell transplantation outcomes.
    The study group included 56 donor-patient pairs with underlying malignant hematological disorders undergoing HLA-E  matched allogeneic hematopoietic stem cell transplantation. They were genotyped for HLA-E locus using a sequence specific primer-polymerase chain reaction. The  median follow-up was 20.6 months  (range 0.2-114.8) and  the  parameters assessed were acute and chronic graft-versus-host disease and overall survival.
    We showed a lower frequency of acute graft-versus-host disease (grade II or more; p=0.02)and chronic graft-versus-host disease (extensive; p=0.04) in the patients with HLA- E*0103/0103 genotype compared to other genotypes of HLA-E. There was also an association between HLA-E*0103/0103 and improved overall survival (p=0.001).
    Conclusively, our  results  suggest a  protective  role  for  HLA-E*0103/0103  genotypeagainst acute graft-versus-host disease (grade II or more) and chronic graft-versus-host disease (extensive) as well as an association between this genotype and a better overall survival after HLA-E matched allogeneic hematopoietic stem cell transplantation.

  • XML | PDF | downloads: 355 | views: 585 | pages: 23-27

    Asthma is one of the most common chronic inflammatory disorders in children. Nonadherence to medical therapy is a major cause of poor clinical outcome the objective of this study was evaluating factors, which are resulted in nonadherence to medical therapy in children with asthma.
    In  this  descriptive study, 150 children with asthma  and  nonadherent  to  medication therapy were enrolled. General  information  and  probable  causes of  nonadherence  were recorded in self-report questionnaire and data were analyzed.
    In our study, 57.3% of children were male. Approximately 43%of children belonged to age group 6-9 years old. Prevalence of probable causes of nonadherence to treatment were concern about treatment  expenses(34.7%) ,fear of cardiac complications(34.7%), concern about drug dependency(38.7%), belief to growth inhibition(30.7%) and fear of osteopenia (32%). There was statistically significant reverse association between treatment with   multi- drug regimens and concern about bone  mineral abnormalities, cardiac complications and drug  dependency  (p=0.0001,  0.014  and  0.012  respectively). In  addition,  there  was  a significant association between mild asthma and fear about drug dependency (p=0.001).
    According to  our  results, factors  such  as  prolonged  duration  of  treatment,  various therapeutic regimens, and receiving multiple drugs before  diagnosis of  asthma pose  the highest frequencies for nonadherence.

  • XML | PDF | downloads: 334 | views: 709 | pages: 29-36

    Cow’s  milk  anaphylaxis is  the  most  common  food-induced  anaphylaxis in  Iranian children. The clinical and laboratory findings of cow’s milk anaphylaxis are evaluated in this study. All children who had experienced cow’s  milk anaphylaxis and had been referred to Immunology, asthma and allergy research center during a 5-year period were considered. After fulfilling a questionnaire, patients underwent measurement of total IgE and cow’s milk- specific IgE by Immunocap test and Skin prick test (SPT) with cow’s milk extract. Patients with a convincing history and one positive cow’s milk-specific IgE test (SPT or Immunocap test) and patients with both positive tests were enrolled, in this study.
    Out of 49 patients, 59.2% were male. Patients’ mean age was 5 years old and their mean age at the  time of  first attack was 5.7 months  (SD =  4.3). Most  of  the  patients  have experienced more than one episode of anaphylaxis (79.5%) and in 85.7% of all cases, first attack occurred during the first year of life. Severity grading 1-5 were 2%, 6.1%, 18.4%,69.4%, 4.1% respectively. Most common manifestations were cutaneous 98%, Respiratory91.8%, Gastrointestinal  55.1%, Cardiovascular 46.9% and  neurologic  46.9%  signs  and symptoms  respectively. Twenty four  patients showed positive SPT. Mean total IgE  was 239.6±3.3  (IU/mL)  and  mean  cow’s  milk-specific IgE  was 19.28±27.2  (IU/mL).  Most patients showed reactions only after ingestion of cow’s milk or after dairy foods (81.6%).
    It  is concluded  that  cow’s milk anaphylaxis may happen  early in life. Regarding the severity of attacks and remarkable number of patients with several attacks, poor knowledge about this disorder is evident.

  • XML | PDF | downloads: 397 | views: 494 | pages: 37-50

    Atopic dermatitis (AD) is a common chronically relapsing skin disease associated with abnormal cytokine production, and activation of T-helper 2 cells. The aim if this study was to determine whether cytokine gene polymorphisms might influence the development of AD. Single nucleotide polymorphisms in the genes for I-L1alpha, IL-1beta, IL-1R, IL-2, IL-4, IL-6, IL-10, IL-12, TGF beta, TNF and IFNgamma were investigated by PCR and sequence specific primers in Macedonian patients with AD (67 children, age of 6 months to 5 years) and  301 normal  unrelated  individuals. Susceptible cytokine polymorphisms  for  AD  for eleven genotypes (IL-4 -33/T:T  IL-4 -1098/G:G, TGFbeta cdn25C:G,    IL-4 -1098/T:T,  IL-1alpha -889/C:T, IL-2 +166/T:T, IL-1beta -511/C:T, IL-12 -1188/C:T, IL-10 -1082/A:G, IL-1beta +3962/C:T, IFNgamma +874/A:T), five diplotypes, six haplotypes, and for alleles were found. Protective cytokine polymorphisms for AD for seven cytokine genotypes (IL-4 -1098/G:T,  TGFbeta  cdn25/G:G,  IL-4  -33/C:C,  IL-1alpha -889/C:C,  IFNgamma  +874/A:A, IL-10 -1082/A:A,   IL-1beta -511/C:C), one cytokine diplotypes, two cytokine haplotypes, and four cytokine alleles were also found. We concluded that several cytokine polymorphisms are protective, or susceptible associated with AD in population of Macedonians.

  • XML | PDF | downloads: 351 | views: 621 | pages: 51-56

    Severe Congenital Neutropenia is one of primary immunodeficiency disorders that characterized by severe neutropenia and is associated with severe systemic bacterial infections from  early  infancy.  Granulocyte  Colony  Stimulating  Factor  (GCSF)  is  clinically  used  as  a treatment for congenital and acquired neutropenia. The aim of this study was evaluation of GCSF (PD- Grastim) in treatment of these patients.
    Patients with severe congenital neutropenia referred to Immunology, Asthma and Allergy Research Institute between Jan 2007 and Dec 2010 enrolled the study. Other causes of neutropenia were excluded by serial CBC and bone marrow studies, medical and drug histories and immunological tests. Patients were visited and examined monthly to evaluate their CBC and ANC  (Absolute  Neutrophil  Count),  GCSF  side  effects  and  dosage  adjustment.  Cytogenetic studies were being done for all the patients for early detection of progression to AML/MDS.
    From twenty two patients who enrolled this study, 16 patients regularly evaluated. They were ten males and six females, range in age from 2 to 18 years old. Two patients failed to continue our follow up unfortunately and four patients died due to disease complications. Patients were followed for 24 to 48 months. In a period of 12-24 months before treatment, the mean of hospitalization frequency was 3.1 times and duration was 10 days; while during receiving treatment, they decreased to 0.2 times and 3 days, respectively (p<0.01). Also significant increase in mean ANC was observed during follow up (315/µl before treatment versus 1749/µl after 12 month regular treatment). Bone pain was the most common side effect.
    There have been no evidences of developing AML/MDS up to present time. Treatment with GCSF  significantly  reduced  the  duration  and  the  frequency  of  hospitalization.  Because  of plausible progression to AML/MDS, regular follow-up of patients should be continued.

  • XML | PDF | downloads: 402 | views: 615 | pages: 57-64

    Primary immunodeficiency diseases (PIDs) consist of a group of genetic disorders that predispose the patients to immune-mediated complications. The aim of this study was to assess the knowledge of Iranian general practitioners and pediatricians about PIDs.
    A questionnaire consisting 52 closed questions on clinical symptoms, laboratory data,associated syndromes and management of PIDs patients was made valid and reliable by a pair pilot study. Then the questionnaire was filled by pediatricians, general practitioners and pediatric residents from different regions of Iran.
    Totally, 333 physicians (50 general practitioners, 52 pediatric residents, 182 pediatric specialists, and 49 pediatric sub specialists) participated in this study. The mean total score was 55.9±14.3 (i.e. about 29 correct answers out of 52 questions). One hundred and five participants (31.9%) answered correctly more than two third of all questions. In order to qualitatively compare the groups a ranking system was used. Total scores was significantly different between physicians groups (p<0.01). Pediatric subspecialties gained the  highest rank, which was significantly over the other participants (p<0.05).
    This study showed that there is a considerable lack of awareness on PIDs in physicians. This may be one of the major reasons in late diagnosis and the delay in adequate treatment deteriorating patients’ morbidity and mortality. Retraining classes and reconsidered educating schedules are needed as an efficient strategies and improving physicians' knowledge about PIDs.

  • XML | PDF | downloads: 785 | views: 1505 | pages: 65-71

    Chronic  obstructive  pulmonary  disease (COPD)  and  asthma  are  major  public  health problems, which seems to have close association with psychiatric disorders. The present study was conducted to compare the psychological status between asthmatic and COPD patients and clarify the relationship with severity of pulmonary obstruction.
    This cross-sectional study was planned to compare the psychological status in 67 stable obstructive  lung patients (17 asthma, 24 asthmatic bronchitis  and 26 COPD)  referred to respiratory clinic of Ghaem hospital and 33 healthy controls, in Mashhad city, north east of Iran. Severity of  pulmonary obstruction  was determined based on  GOLD  criteria. “Beck Depression Inventory”, “Hamilton Anxiety Rating Scale” and “SCL-90-R” questionnaires were used to determine the psychological status.
    Prevalence of general psychopathology in asthma, COPD, asthmatic bronchitis and control groups were 64.7%, 42.3%, 33.3% and 36.4% respectively. Psychological status was directly related to severity of pulmonary obstruction (p=0.048), Prevalence of depression in asthmatic, COPD and asthmatic bronchitis groups were 66.7%, 54.2% and 44.4% respectively. Depression score was related to severity of pulmonary obstruction  (p=0.000).Prevalence of anxiety in asthma, COPD and asthmatic bronchitis were 46.7%, 45.8% and 40.7% respectively. Anxiety score was related to marital status and satisfaction with incomeAsthmatic and COPD  patients are at equal risk of developing psychiatric disorders and both  require  psychological evaluations  in  respiratory  clinics. Therapists  must  pay  more attention to patients with severe pulmonary disease.

  • XML | PDF | downloads: 568 | views: 854 | pages: 73-77

    Many patients with atopic eczema (AE) would “march” to develop allergic rhinitis (AR) and asthma. Physicians, patients and their families often do not appreciate the significance of these diseases as co-morbidities of atopy.
    The aim of this study was to evaluate the prevalence and severity of airway atopies in patients with AE. AR and asthma severity were assessed in consecutive AE patients seen at a pediatric dermatology clinic by ARS (allergic rhinitis score) and ACT (asthma control test). Eczema severity (SCORAD and Nottingham Eczema Severity Score: NESS) were recorded.
    110 patients with AE and 42 patients without AE were recruited. Allergic rhinitis and asthma were significantly more prevalent in patients with AE [odds ratio for AR was 2.9 (CI:1.3 – 6.5) and for asthma 4.3 (CI: 1.3 – 16.10)]. 23 (45%) of the AE patients with AR reported that they were currently on oral antihistamine whereas none of the non-AE group reported such usage. Both groups reported relatively higher sneezing and nasal congestion scores and low “eye watering” score. Comparing mild with moderate-to-severe AE, there was essentially no  difference between the  prevalence of  allergic rhinitis and  asthma, or severity of symptoms by ARS and ACT, but females reported more severe symptoms of sneezing and itching nose.
    We conclude that  allergic disorders of airway are very common  among AE  patients independent of the eczema severity. Most of the patients have mild-to-moderate AR and asthma. There is a lot of room for parent/patient education, and childhood eczema may prompt early awareness of these airway co-morbidities of atopy.

  • XML | PDF | downloads: 192 | views: 448 | pages: 77-79

    Occupational asthma has been reported  to  be the most  common  chronic respiratory occupational disease in many developed countries, and  as with other  occupational lung diseases, occupational asthma is potentially preventable.
    We report the case of a 24-year-old baker who experienced pneumomediastinum as a consequence of workplace exposure.
    This is the first report of pneumomediastinum as an acute complication of occupational asthma, and it exemplarily shows that the lack of medical surveillance at the workplace may lead to an acute, although unusual, complication.