Reference intervals for serum immunoglobulins IgG, IgA, IgM and complements C3 and C4 in Iranian healthy children

Determination of reference ranges of each serum protein in normal population of each country is required for studies and clinical interpretation. The aim of this study was defining reference range values of immunoglobulins and complement components in Iranian healthy children. This study was conducted from June 2003 to June 2006 in Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences. Serum levels of IgG, IgM, IgA, C3 and C4 in 800 Iranian healthy children from newborn to 18 years of age in four population were measured by nephelometry. Kolmogrov-Smirnov tests and Pearson correlation tests were used for analysis. Our results mainly agree with previous reports, except for some discrepancy that might be due to the ethnic and geographic variety. There was a significant difference between two sexes only with IgA in the group of 1-3 months old, which was higher in male group and IgM in groups of 3-5, 6-8 and 9-11 years old that were higher in female groups. Mean of other serum immunoglobulins and complements was not significantly different between male and female groups. These results can be considered as a local reference for use in laboratories, clinical interpretations, and research for Iranian children

Strategic planning in a health leadership sector: a report from UNESCO chair in health education, Iran

Strategic planning defines the formal decision of a company for its future. Like all organizations, health care sectors need to prepare their strategic planning and act according to it. UNESCO chair in health education as a leader health sector, describes the course and steps for preparing its strategic planning based on SWOT analysis technique

[Correlation of null Btk expression and gene noncoding mutations in XLA patients]

X-linked agammaglobulinemia [XLA] is a primary immunodeficiency disorder characterized by recurrent bacterial infections, profound lack of serum antibodies and reduced circulating B lymphocytes. Mutations in Bruton's tyrosine kinase gene [BTK] result in XLA. It is shown that absence of Btk protein expression may be accompanied by no mutations in coding regions in some cases, instead alterations in conserved regulatory domains of promoter and the first intron of BTK gene maybe occurred The aim of this study was evaluation of Btk expression and mutation analysis in coding and regulatory regions of the gene. In this study, eleven XLA patients were enrolled. Btk expression was analyzed by western immunoblotting method. Mutation analysis was carried out in eight patients. In three cases, PCR of the regulatory regions was performed with designed primers, followed by sequencing. According to western blot, normal Btk expression in three patients and null expression in eight others was observed. Mutation analysis showed two novel BTK mutations in two patients [1038-1040 delAGG and IVS8-2delA]. No coding or regulatory region mutations were found in three cases with null Btk expression. Based on these results, three cases with null expression and had no coding or regulatory region mutations are interesting. It is possible that some rare regulatory defects may have been occurred, other than conventional sites. This must be taken into account for future investgations

Point mutations on mitochondrial DNA in Iranian patients with Friedreich's ataxia

Mitochondrial DNA [mt DNA] is considered a candidate modifier factor for neuro-degenerative disorders. The most common type of ataxia is Friedreich's ataxia [FA]. The aim of this study was to investigate different parts of mt DNA in 20 Iranian FA patients and 80 age-matched controls by polymerase chain reaction [PCR] and automated DNA sequencing methods to find any probable point mutations involved in the pathogenesis of FA. We identified 13 nucleotide substitutions including A3505G, T3335C, G3421A, G8251A, A8563G, A8563G, G8584A, T8614C, T8598C, C8684T, A8701G, G8994A and A9024G. Twelve of 13 nucleotide substitutions had already been reported as polymorphism. One of the nucleotide substitutions [A9024G] had not been reported before. The A9024G nucleotide substitution does not change its amino acid. The controls were also investigated for this polymorphism which was found in two of them [2.5%].None of the mutations found in this study can affect the clinical manifestations of FA. This survey also provides evidence that the mtDNA A9024G allele is a new nonpathogenic polymorphism. We suggest follow-up studies for this polymorphism in different populations

[Immunological evaluation of children with recurrent ear, nose, and throat [ENT] infections]

Recurrent and chronic infections of ear, nose, and throat [ENT] such as sinusitis [rhinosinusitis] and otitis media are one of the most common health care problems worldwide and significantly impact quality of life in both children and adults. Antibody deficiencies are the most common type of primary immunodeficiency and also the most likely to present with recurrent ENT infections. A study was carried out to search for underlying immunodeficiencies in 103 patients with recurrent or chronic ear, nose and throat infections. Serum total IgG, IgA, and IgM levels were measured by kinetic nephelometry, and IgG subclasses by enzyme-linked immunosorbent assay [ELISA]. All patients were immunized intramuscularly with polyvalent pneumococcal vaccine [PENEUMO 23]. Blood samples were drawn immediately before and 21 days after vaccination and antibodies to pneumococcal antigens were measured using a modified ELISA technique. Of 103 patients twenty one [20%] were found to have an immunodeficiency. One had a common variable immunodeficiency [CVID], 5 had selective IgA deficiency [one of them was associated with IgG2 deficiency and one with specific antibody deficiency]. Eight patients had IgG-subclass deficiency including seven with an IgG2 deficiency and one patient with IgG3 deficiency. In 75 patients antibody titers of whole pneumococcal antigens were determined before and 21 days after immunization. Ten patients were found to have abnormally low antibody titers. The results of this study suggest that in a subpopulation of patients with a long standing history of ENT infections, a low serum immunoglobulin concentration or hypo responsiveness to pneumococcal antigen would be associated with susceptibility to recurrent infections

UNESCO chair in health education: a step toward public health improvement

The UNESCO Chair in Health Education in TUMS [TUMS], Iran, was established in Immunology, Asthma and Allergy Research Institute in April 2004. The purpose of this chair is to promote an integrated system of research, training, information and documentation activities in the field of health education. The target group includes the public, health care workers, students, trainees and researchers. During its one year existing, the Chair has supported financially eleven admitted proposals and supported technically eight submitted proposals in divers fields regarding health issues with the cooperation of other research institutes in TUMS. It has made connections with most of the national research institutes and universities to establish multi-centric collaborations. The activities of the Chair are being directed towards the health priorities of the country and region. The Chair has contributed to an important international congress on immunodeficiency disorders as well as another meeting on Asthma in near future. The Chair is to publish several educational books, booklets and CDs related to health education; it is also going to prepare a thorough proposal on HIV/AIDS prevention with collaboration of other institutions, which could be applicable regionally, and in neighboring countries and states

Unilateral proptosis: a local experience

This paper presents the experience with unilateral proptosis at Allama Iqbal Medical College [Jinnah Hospital] and King Edward Medical College [Mayo Hospital], Lahore from July 1995 to October 2001. There were 45 patients of unilateral proptosis managed over a period of six years and three months. There was a male predominance with a ratio of 2:1. The age range was 2 - 70 years. The average age was 33 years. The commonest cause was a disease in the ethmoids [27/45 - 60%]. Commonest pathology was chronic ethmoiditis and polyposis [10/45 - 22%]. External ethmoidectomy performed through the Lynch Howarth approach has been the commonest surgical procedure required [20/45 - 44%]. Unilateral proptosis is a multidisciplinary problem and requires collaboration of different specialties

Papillon-lefe'vre syndrome: neutrophil motility and killing defect in a child with recurrent severe infections

A case of palmoplantar hyperkeratosis with periodontosis and a history of recurrent severe pyoderma, pneumonia and multiple liver abscesses is described in a 12 year old girl. The patient demonstrated neutrophil dysfunction characterized by decreased random migration and chemotaxis and defective bactericidal activity. The exact immunopathological mechanism for susceptibility to infections in Papillon-Lefe'vre syndrome patients still remains to be determined. However, the mode of clinical presentation, laboratory findings and response to retinoid treatment, all support the speculation of Papillon-Lefe'vre syndrome as a primary immunologic disease with a variable defect in neutrophil motility and bactericidal activity. The pattern of clinical presentations as skin and periodontal lesions alone or with susceptibility to infection in other sites will change accordingly