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Chronic Granulomatous Disease (CGD) represents a group of inherited disorders of phagocytic system, manifesting recurrent infections at different sites. The present study was accomplished in order to determine the gastrointestinal manifestations of CGD patients. Fifty-seven patients (38 males and 19 females) with CGD, who had been referred to three immunodeficiency referral centers in Iran, were studied during a 24-year period (1980-2004). The median age at the time of study was 14.5 years old (1-56 years). The median onset age of symptoms was 5 months (1 month- 13.75 years), and that of diagnostic age was 5 years (2 months- 54.1 years), with a diagnostic delay of 33 months, on average. Seven patients were presented with acute diarrhea, 3 with oral candidiasis, and 2 with liver abscesses as the first chief complaints. Twenty-four cases (42.1%) had been complicated by gastrointestinal manifestations during their course of the disease. Of those, 12 cases (21.1%) had diarrhea, 7 (12.3%) oral candidiasis, 5 (8.8%) hepatitis, 4 (7.0%) hepatic abscess, and 2 cases (3.5%) gastric outlet obstruction. Also, failure to thrive was detected in 6 patients (10.5%). Four patients died (7%). CGD should be excluded in any patient with gastrointestinal manifestations especially chronic diarrhea, hepatic abscess, and gastric outlet obstruction.

CD8 deficiency is a rare primary immunodeficiency with low or absent peripheral CD8 cells which results from TAP deficiency, Zap 70 deficiency and CD8 alpha gene mutation.We report a 14 year old female who presented with a history of recurrent pneumonia, bronchiectasis, otitis, severe varicella, herpetic lesions of mouth, bilateral uveitis, and cataract formation since the age of 8 years.She had growth failure, a huge spleen and moderate clubbing. In immunologic workup, humoral and phagocytic systems were normal. DTH response to candida, PPD and DT were negative but LTT response to PHA mitogen was normal. HLA typing showed normal class I expression. Flowcytometry of peripheral blood showed CD8: 0 to 2% (absolute count, 0-60 cells/mm3) with increased CD4/CD8 ratio on several occasions.Diagnosis of this patient cannot be HLA class I deficiency (TAP1 or TAP2), because class I expression had been normal. It is possible to be Zap -70 deficiency or CD8 alpha gene mutation. Bilateral uveitis in our patient was a unique presentation which might have resulted because of immune dysregulation in CID.

Neutropenia is characterized by decrease in the absolute number of circulating neutrophils and an increase susceptibility to infections. The current study was performed in order to explain the clinical and laboratory findings of patients with antibody deficiency disorders associated neutropenia. The patients' records of 19 neutropenic cases out of 207 patients with antibody deficiencies, who had been referred to Children's Medical Center and enrolled in Iranian primary immunodeficiency registry, were reviewed. Nineteen cases (14 male and 5 female), with a mean age of 10.7+/-5.7 years, were associated with neutropenia (9.2%). The disorders with associated neutropenia were Hyper IgM syndromes (3 of 8), Common variable immunodeficiency (13 of 109), and X-linked agammaglobulinemia (3 of 45). The median age for the onset of disease and diagnosis age were 15 months (1-134) and 3.8 years (6 months-13 years), respectively. The most common infections during the course of illness were pneumonia (13 cases), diarrhea (12 cases), oral candidiasis (9 cases), otitis media (6 cases), sinusitis (6 cases), cutaneous infections (5 cases), and abscess (5 cases). Other less frequent infections were: conjunctivitis, oral ulcers, meningitis, and osteomyelitis. Three neutropenic patients died because of recurrent infections. Neutropenia may occur in any of the primary immunodeficiency disorders. Persistent or severe infections always pose a supposition, which deserves further evaluation for detecting an underlying immune deficiency syndrome and neutropenia, since a delay in diagnosis may result in a serious organ damage or even death of the patient.

Primary immunodeficiencies (PID) are a group of disorders, characterized by an unusual susceptibility to infections. Delay in diagnosis results in increased morbidity and mortality in affected patients. The purpose of this study was to determine the mortality rate of Iranian immunodeficient patients referred to Children Medical Center Hospital affiliated to Tehran University of Medical Sciences over a period of 20 years. In this study, records of 235 (146 males, 89 females) patients with immunodeficiency who were diagnosed and followed in our center, during 22 years period (1979-2001) were reviewed. The diagnosis of immunodeficiency was based on the standard criteria. The cause of death was determined by review of death certificates. Antibody deficiency was the most common diagnosis made in our patients. The overall five-year survival rate was 22.7% in our studied patient group; this was greatest in antibody deficiency. During the 22 year period of study, 32 patients died. As some of the patients could not be located, the true mortality rate ranged between 13.6% and 17.5%. The main leading cause of death were lower respiratory tract involvement in 14 cases (44%). The most common pathogenic microorganisms causing fatal infections were psudomonas and staphylococcus in 9 cases (28.1%) followed by E. coli in 7 (21.9%), tuberculosis in 13 (40.6%) and salmonella in 1 (3.1%). Based on our study, delay in diagnosis in patients with PID results in tissue and organ damage and several complications. Mortality and morbidity are increased in undiagnosed patients.

In the management and evaluation of asthma, simple instruments for measurements of peak expiratory flow rate (PEFR) are necessary. The aim of this study was to determine normal PEFR of the healthy children in Babol, Iran. This study was randomly done on 1050 students (primary and secondary schools) in Babol. Mini-Wright peak flow meter was used for measurement of PEFR. The range of age, weight and height were defined. Information was statistically analyzed by SPSS, T- Test and R2 and due to formula with regression. Mean age of 1050 students (525 male and 525 female) who participated in this study was 10.26 years. The mean of PEFR was 262.35±71.97 L/Min. Obtained PEFR with all anthropometrics variants indicate a high correlation. Correlation between PEFR and height was more significant and between PEFR and weight was lower, and according to importance of height and age, formulas suggested for prediction of PEFR in females {(age×4/8)+ (height×0/6)-25)} and in males ({(age×1/7)+ (height×2/1)-208)}. The results of this study can determine normal PEFR and it can be useful for treatment and monitoring of children with asthma who live in this region.

Primary antibody deficiencies are the most frequent primary immunodeficiency disorders. Bronchiectasis as a feature of these disorders may be developed due to some factors such alpha-1- antitrypsin deficiency. In order to determine the prevalence of two common alpha-1-antitrypsin deficiency alleles (PI*Z and PI*S) in Iranian patients with antibody deficiency, this study was performed. The prevalence of PI*M, PI*S, and PI*Z allele combinations was determined in 40 patients with primary antibody deficiency (with and without bronchiectasis) and compared with 60 healthy control subjects. Phenotyping was performed by isoelectric focusing. The phenotype frequencies among patients were as follow: M in 92.5%, S in 2.5% and Z in 5%. There was not any significant difference in distribution of alleles or phenotypes between patients and control subjects. Moreover, no significant difference was found between patients with and without bronchiectasis. We did not find evidence to support an association between alpha-1-antitrypsin phenotypes and primary antibody deficiencies in a small, controlled study. Larger studies will be required to clarify the relationship between alpha-1-antitrypsin genotype and susceptibility to bronchiectasis in patients with antibody deficiency.

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 (p0.001 and p0.001 respectively) compared with children without a chronic health condition. Patients had lower PEDQOL scores in all age groups compared with normal sample (p0.001). 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.

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.

Ataxia-telangiectasia is a multisystem disorder characterized by progressive neurologic impairment, variable immunodeficiency, impaired organ maturation, x-ray hypersensitivity, oculocutaneous telangiectasia, and a predisposition to malignancy. To evaluate clinical and immunologic features of Iranian patients with ataxia-telangiectasia, the records of 104 patients with ataxia-telangiectasia (54 male, 50 female) with the age range of 1.6-23.5 years were reviewed. The Iranian Primary Immunodeficiency Registry was used as the data source. Progressive ataxia was seen in all the patients. Other symptoms were eye movement disorders (n = 84), slurred speech (n = 70), mental retardation (n = 10), and ocular (n = 87) and cutaneous (n = 73) telangiectasia. Three patients developed leukemia and lymphoma, and 17 patients had family history of malignancy. Positive correlation was seen between clinical immunologic symptoms and immunoglobulin deficiencies (P = 0.004). The predominant infections were sinopulmonary and acute and recurrent infections (78 cases). Infections included pneumonia (56 patients), otitis media (34 patients), and sinusitis (50 patients). Average serum alpha-fetoprotein level was 149 +/- 137 ng/dL. The incidence of ataxia-telangiectasia in Iran is high, possibly due to familial marriages. Treatment should be focused on supportive management to prolong survival.

Leukocyte adhesion deficiency type I (LAD I) is a rare, inherited, autosomal recessive, immunodeficiency disease caused by the combined loss of expression on the surface of leukocytes of the leukocyte integrins. We describe the clinical and laboratory findings for 15 patients with LAD I. The range of patients' ages was from 10 month to 14 years (median 4 years) and 93.3% of their parents had consanguineous marriages. The most commonly occurred manifestations were: recurrent infections (93.3%), poor wound healing (86%), oral ulcers (86%), and skin abscesses (80%). The most specific laboratory findings were defect in CD18 in all of 15 patients. The most common symptoms in these patients are poor wound healing and oral ulcer, so, the clinical physicians should pay special attention to these symptoms. Furthermore, because of considerable rate of consanguineous marriages in parents of LAD patients, we suggested more genetic studies on this disease and genetic consultation for these families.

In order to determine the clinical and laboratory findings of Iranian patients with presumed hyper-immunoglobulin E syndrome (HIES), the medical records of 22 patients from 21 unrelated families, who had been registered in the Iranian Primary Immunodeficiency Registry, were observed. The median age of patients at the time of first symptom and at the time of diagnosis was 1 month and 52.5 months, respectively, with a median diagnosis delay of 70 months. 13 families had consanguineous marriages. IgE level was higher than 2000 IU/ml in all patients, ranging from >2000 to 80,000 IU/ml. The most commonly occurring manifestations were: eczema and dermatitis, pneumonia, upper respiratory tract infections, cutaneous abscesses, diarrhoea, deep abscesses, and otitis media. Other less frequent manifestations were: mucocutaneous candidiasis, sinusitis, cutaneous ulcers, Molluscum contagiosum, herpetic keratitis, onychomycosis, conjunctivitis, septic arthritis, and meningitis. Five patients were complicated by bronchiectasis due to recurrent pneumonia and 5 patients died because of severe infections and malignancy. The HIES is a multisystem disorder that affects especially cutaneous, respiratory, skeletal and the immune system. Although HIES is a rare condition, the recurrent infections should always raise a suspicion, which deserves further evaluation for detecting the syndrome.

Primary immunodeficiency disorders (PID) are a heterogeneous group of diseases, characterized by an increased susceptibility to infections. A total of 930 patients (573 males and 357 females) are registered in Iranian PID Registry (IPIDR) during three decades. Predominantly antibody deficiencies were the most common (38.4%), followed by congenital defects of phagocyte number and/or function (28.3%), other well-defined immunodeficiency syndromes (17.7%), combined T- and B-cell immunodeficiencies (11.0%), complement deficiencies (2.4%), and diseases of immune dysregulation (2.3%). Common variable immunodeficiency was the most frequent disorder (20.8%), followed by chronic granulomatous disease, ataxia-telangiectasia, btk deficiency, selective IgA deficiency, and T-B-severe combined immunodeficiency. The frequency of other PID disorders was less than 50 in number (

Common variable immunodeficiency (CVID) is a primary immunodeficiency disorder, which presents with hypogammaglobulinemia and recurrent bacterial infections. Patients with CVID have frequent and severe episodes of pneumonia. The standard intravenous immunoglobulins (IVIG) therapy has led to the reduction of pulmonary infections in these patients. The aim of this study was to evaluate the effectiveness of IVIG treatment in reducing the incidence of pneumonia in patients with CVID.Twenty six Iranian patients with CVID whose diseases had been diagnosed at the Children Medical Center and had received regular IVIG for at least 9 months were selected. The numbers of episodes of pneumonia and hospital admissions were documented before and during treatment with IVIG.Of 26 patients with CVID, 80.5% had experienced pneumonia at least once before receiving immunoglobulin and 88.5% required hospital admission. After starting treatment with IVIG (mean treatment period, 41.5 +/- 35.4 months), the annual incidence of pneumonia significantly decreased from 80.5% to 34.6% (p=0.0017), and the rate of hospitalization from 88.5% to 46% (p=0.0025) . The incidence of pneumonia requiring treatment or hospitalization fell from 3.4 to 0.7 per year (p0.0005).Regular IVIG therapy can significantly reduce the incidence of pneumonia and hospital admission due to infections in patients with CVID.

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

Neutropenia may occur in some of the primary immunodeficiency disorders. We reviewed the records of 56 neutropenic patients. The most common disorders were Shwachman-Diamond syndrome, cyclic neutropenia, Kostmann disease, Chediak-Higashi syndrome, hyper IgM syndromes, severe combined immunodeficiency, hyper IgE syndrome, and common variable immunodeficiency.

Another group of authors from Iran attempted experimentally to determine the required time for the appropriate enzymatic treatment of the ideal segment to complete de-epithelialization, thus reducing its absorptive function. They found that 25 min of enzymatic treatment of the ideal segment was adequate for this, and that it was recommended from their experimental study for cystoplasty. OBJECTIVES To determine the time required for the appropriate enzymatic treatment of an ileal segment to de-epithelialize its mucosa and to reduce its absorptive function for cystoplasty in dogs. MATERIALS AND METHODS Twenty-one adult female Persian mixed-breed dogs were divided into seven equal groups: group 1 (negative control group) had no ileocystoplasty; group 2 (positive control group) had a routine ileocystoplasty with no enzymatic treatment of the ileal segment; and groups 3–7 had an ileocystoplasty with 5, 10, 15, 20 or 25 min, respectively, of enzymatic treatment of the ileal segment with collagenase and trypsin. The seven groups were then compared for haematological, biochemical and histological changes, and glucose reabsorption assessed using a glucose-absorption test. RESULTS No dogs showed any signs of metabolic disturbances, biochemical and haematological changes. There were significant differences in blood glucose level (BGL) for the groups at different times after the glucose-absorption test, but a pair-wise comparison showed significant differences in BGL between group 1 and the other groups, except group 7. The degree of histopathological change was associated with the duration of enzymatic treatment, in that changes were more prominent in group 7. There was no shrinkage or collagen deposition. CONCLUSIONS In these conditions, 25 min of enzymatic treatment of the ileal segment is sufficient to remove the absorptive function of the augmented bladder, and is recommended for cystoplasty in dogs.

Neutropenia is characterized by decrease in the absolute number of circulating neutrophils and an increase susceptibility to infections. The current study was performed in order to explain the clinical and laboratory findings of patients with antibody deficiency disorders associated neutropenia. The patients' records of 19 neutropenic cases out of 207 patients with antibody deficiencies, who had been referred to Children's Medical Center and enrolled in Iranian primary immunodeficiency registry, were reviewed. Nineteen cases (14 male and 5 female), with a mean age of 10.7±5.7 years, were associated with neutropenia (9.2%). The disorders with associated neutropenia were Hyper IgM syndromes (3 of 8), Common variable immunodeficiency (13 of 109), and X-linked agammaglobulinemia (3 of 45). The median age for the onset of disease and diagnosis age were 15 months (1-134) and 3.8 years (6 months-13 years), respectively. The most common infections during the course of illness were pneumonia (13 cases), diarrhea (12 cases), oral candidiasis (9 cases), otitis media (6 cases), sinusitis (6 cases), cutaneous infections (5 cases), and abscess (5 cases). Other less frequent infections were: conjunctivitis, oral ulcers, meningitis, and osteomyelitis. Three neutropenic patients died because of recurrent infections. Neutropenia may occur in any of the primary immunodeficiency disorders. Persistent or severe infections always pose a supposition, which deserves further evaluation for detecting an underlying immune deficiency syndrome and neutropenia, since a delay in diagnosis may result in a serious organ damage or even death of the patient.

In order to determine the clinical and laboratory features of X-linked agammaglobulinemia, the records of 33 male patients with XLA were reviewed during 22 years (1980-2002) in the Iranian referral center of primary immunodeficiency disorders. The patients' ages ranged from 20 to 360 months (median 113 months). The median age at the onset of the disease was 8 months and the median age of diagnosis was 48 months, with a median diagnosis delay of 33 months. Almost all of the patients presented common infectious diseases, which were: pneumonia, otitis, diarrhea, sinusitis, and arthritis. During the course of illness, infections in the respiratory tract, gastrointestinal tract, central nervous system, and musculoskeletal system were seen in 93.9%, 75.8%, 33.3%, and 21.2% of XLA patients, respectively. The most common complications of these patients were chronic infections in 75.8% of them, including: chronic otitis media, chronic sinusitis, chronic diarrhea, and bronchiectasis.

Background: Chronic granulomatous disease (CGD) represents a group of inherited disorders of the phagocytic system, involving recurrent infections at different sites, especially the respiratory system. The present study was accomplished in order to determine the clinical spectrum of Iranian patients with CGD. Methods: Forty-one patients (29 males and 12 females) with CGD, who had already been referred to two immunodeficiency referral centers in Iran, were reviewed during a 22-year period (1980–2002). Results: These patients belonged to 34 families, and 56% of them were consanguineous. The median age at the time of study was 12 years (3 months to 22 years). The median age at onset of symptoms was 4 months (1 month to 12 years), and the median diagnostic age was 5.5 years (2 months to 20 years), with a diagnostic delay of 3 years on average. The most common presenting complaint in our CGD patients was lymphadenopathy (seen in 11 patients, 26.8%). The most common manifestations of CGD (in descending order) were lymphadenopathy (75.6%), pulmonary infections (65.9%) and skin involvement (63.4%) during their illness, followed by gastrointestinal (56.1%), skeletal (29.3%), upper respiratory tract (26.8%) and central nervous system (2.4%) involvement. Conclusions: Early diagnosis of the disease is crucial. In view of the possibility of timely treatment, i.e. prophylactic treatment of infection, CGD should be excluded in any patient with unexplained infections or granulomas.

Agammaglobulinemia is characterized by failure of B-cell differentiation (hypogammaglobulinemia) and increased susceptibility to bacterial infections. The present study was set up in order to evaluate the effectiveness of intravenous immunoglobulin (IVIG) treatment on the incidence of pneumonia in patients with agammaglobulinemia. We carried out chart reviews of 23 patients with agammaglobulinemia (mean age 11.5+/-5.4 years), who had been observed in a 22-year period (July 1981-January 2003) in Iran's referral center for primary immunodeficiency disorders. Nineteen of these 23 (82.5%) had been infected with pneumonia at least once before receiving the immunoglobulin treatment and 11 of them had experienced multiple episodes. During treatment with gamma-globulin - over a mean period of 6.8+/-4.1 years (range: 0.8-15.3 years) - the incidence of pneumonia requiring treatment or hospitalization decreased from 0.82 to 0.12 per patient per year (P=0.006). During IVIG replacement, hospitalization due to pneumonia decreased from 0.58 to 0.05 per patient per year (P=0.08) and the immunoglobulin G level (mean+/-S.D.) changed from 66.2+/-63.9 (range: 0-210 mg dl(-1)) to 552.4+/-199.1 (range: 136-942 mg dl(-1)) (P

Methods ISMAR is an international, multicenter, non-interventional, cross-sectional study conducted in adults and children (≥ 6 years) with physician diagnosis-AR of at least 1 year of duration. Physicians recruited consecutive patients to whom the ISMAR questionnaire was administered. Data collection was performed during a single visit. Other 2 additional documents (the investigator’s questionnaire and Case Record Form) were also filled in. Statistical analysis was descriptive. For statistical inferences, Student tor Wilcoxon test were performed according to the variables normality. Herein we show the results on Patient Optimal Management. Patients fulfilling the following criteria were considered as optimally managed: Patient education, environmental control and allergen avoidance Optimal pharmacological treatment according to ARIA guidelines Results Two thousand three hundred patients (2298 were analyzed), mean age 28.8 ± 15.9 years, 54.4% of males were included in 5 new countries and in 4 countries (new centres) that already participated in ISMAR phase 1. The nasal symptoms frequency was 4 days per week (29.1%) and > 4 consecutive weeks (21.8%) [N=2207]. 85.7% of patients had never smoked. Current smokers suffered most commonly of persistent rhinitis (61.3%). Patients reported having within the past 12 months, most frequently: recurrent cough (57.1%), nocturnal cough (40.8%), recurrent dyspnea/breathlessness (36.5%), dyspnea/breathlessness after exercise (33.1%), recurrent wheezing (32.1%), cough after exercise (29.8%), nocturnal dyspnea/breathlessness (29.5%). AR symptoms impaired sleep (63.5%), mood (61.9%), physical activities (50.4%), and working performance (42.1%). 96.3% of patients received recommendations to avoid allergens and irritants. 96.6% were receiving AR treatment, mainly antihistamines and nasal corticosteroids but also anti-leukotrienes and nasal and oral decongestants. 55.8% of patients had an optimal treatment. Patients with a frequency of nasal symptoms > 4 days per week and > 4 consecutive weeks and those with recurrent dyspnea/breathlessness appeared as more frequently optimally managed (p

Epidemiological studies have shown wide geographical and racial variation in the prevalence and patterns of immunodeficiency disorders. To determine the frequency of primary immunodeficiencies (PID) in Iran, the Iranian Primary Immunodeficiency Registry (IPIDR) was organized in 1999. We extracted the patient's data, by using a uniform questionnaire from their hospital records. The diagnosis of patients was based on WHO criteria. By now, 440 patients with PID, who were observed during a period of 20 years, have been registered in our registry. Among these patients, the following frequencies were found: predominantly antibody deficiency in 45.9% of patients (n = 202), phagocytic disorders in 29.09% (n = 128), T-cell disorders in 24.31% (n = 107), and complement deficiencies in 0.68% (n = 3). Common variable immunodeficiency was the most frequent disorder (n = 98), followed by chronic granulomatous disease (n = 86), ataxia telangiectasia (n = 48), x-linked agammaglobulinemia (n = 45), selective IgA deficiency (n = 42), combined immunodeficiency (n = 15), and severe combined immunodeficiency (n = 14). This study revealed that antibody deficiencies is the most frequently diagnosed primary immunodeficiency disorder in our patients, which is similar to that observed in other registries. A comparative study shows some differences between our results and other registries.

RATIONALE - Interleukin-4 (IL-4) has pivotal role in promotion of T helper2 responses. ll-4 is also an important regulator of adaptive immune responses. This study is aimed at investigating of association of polymorphisms in IL-4 and IL-4-receptor genes with susceptibility to CIU. METHODS - A matched case-control study was conducted on 89 patients with CIU and 138 healthy controls. Autologous serum skin test (ASST) was performed according to international standards. Total IgE levels, thyroid peroxidase antibodies (TPO) and anti thyroglobulin antibodies (ATG) were investigated using spectrophotometry and enzyme-linked immunosorbant-assay, respectively. Single nucleotide polymorphisms at following positions were genotyped using polymerase chain reaction: IL-4-1098T>G (rs243248), -590C>T (rs2243250), -33C>T (rs2070874), and IL-4-receptor+1902A>G (rs1801275). Estimated frequencies were compared between patients and controls. RESULTS - ASSTwas positive in 39 (43.8%) and abnormal TPO and ATG were found in 12 (13.4%) and 6 (6.7%) of patients which were significantly higher than controls (pT (OR 2.39, 95%CI (1.41 to 4.05), p T (OR 4.5, 95%CI (1.9 to 10.82)) and -33C>T (OR 3.46, 95%CI (1.88 to 6.43)), were significantly higher in CIU patients (p

Background Spirometry is widely regarded as a clinically invaluable measurement method that is of genuine recommend for the diagnosis and management of asthmatic patients. FEV-1 and FEV1/FVC are vastly perceived as asthma severity and control assessment indices, according to the present clinical guidelines. Since FEV-1 index is chiefly within normal ranges even in the most severe cases, the certain criteria for asthma diagnosis is immensely base upon it’s significant alteration after bronchodilator challenge test . FEF25-75% represents a well-established indicator of small airway disease now for decades, however, and It has been demonstrated that asthmatic patients do have remarkably lower FEF25-75%. Additionally FEF 2575% meaningful response to the bronchodilator challenge test is seen in some asthmatics that are healthy in terms of other prognostic parameters.

Methods A survey was conducted of patients ≥ 6y rs. old with a diagnosis of asthma who were registered in Iranian Pediatric Asthma Registration program (IPAR) in Immunology,Asthma and Allergy Research Institute (IAARI), Tehran, Iran since 2008. Mangement practice and level of asthma control, according to GINA guidline, for 517 children were evaluated. Data were analyzed using the statistical package SPSS version 16. Univarite analysis were performed to identify association between influenza vaccination and asthma control. Results Among 172 well controlled patients, 59 one (34.3%) influenza vaccination was not current, 103 (59.54%) vaccination was current, and 10 patients didn’t receive vaccine. In group II (partly controlled+ uncontrolled) 301 patients were registered, in which 164 (34.55%) was current, 131 (43.52%) was not current, and 64 (21.26%) didn’ tr eceive vaccine. There was a significant relation between receiving vaccination and well-controlled asthma (p

Cochlosoma sp. infection was identified in a single case among 60 stunted diarrheic native turkey poults, Meleagris galopavo. A large number of the flagellated parasites was found free or within the intervillous spaces of the jejunum, ileum and cecum. Moderate enteritis was associated with the parasites. In TEM studies of the parasagittal sections of the parasite, a prominent ventral sucker like disc and flagella emerging from an opening on the ventrodorsal surface of the pyriform uninuclear parasite were found. The morphological characteristics of this protozoan match with those described for Cochlosoma anatis. The parasite could be considered as an intestinal pathogenic protozoan causing stunting and diarrhea in turkeys in Iran.

BACKGROUND: The prevalence of allergy and asthma among children is increasing in many countries. However, such inclination has not been completely cleared in North of Iran. This study was carried out to evaluate the prevalence of asthma and allergic diseases in school children in Gorgan and also to evaluate the association between allergies and sex, family history of atopic disorders, and personal symptomatic atopy. METHODS: This was a cross-sectional study and the study population included 2800 school children aged 12 to 13 (53% female and 47% male). All participants completed an ISAAC (International Study of Asthma and Allergies in Childhood) written questionnaire. RESULTS: Among the selected children, 7% were asthmatic, 19% had eczema during the last 6 months, and 35.3% had rhinitis. Family history of allergy included 8.4% asthma, 22.1% rhinitis and 12% eczema. CONCLUSIONS: The prevalence of asthma is higher in boys compared with girls. There was a strong relation between family history of atopy and allergic diseases and asthma in children.

Ovarian cystadenocarcinoma is characterized by marked heterogeneity and may be composed of an admixture of histologic growth patterns, including acinar, papillary and solid. In the present study, a case of isolated small intestine metastasis of ovarian papillary cystadenocarcinoma was reported. A 7-year-old female mixed-breed dog presented with a mass in the left upper quadrant with progressive enlargement of the abdomen, periodic bloody discharge from the vulva and incontinence. The tumor was histologically characterized by the presence of cysts and proliferation of papillae, both lined by single- or multi-layered pleomorphic epithelial cells. Furthermore, the mass was composed by intense cellular and nuclear pleomorphism and numerous mitotic figures. These findings indicate a tumor of high-grade malignancy with infiterative tumor cells resembling the papillary ovarian tumor in the serosal surface of the small intestine along with an intact serosa. Immunohistochemically, tumor was positive for CK7 and negative immunoreactivity for CK20. The histopathologic features coupled with the CK7 immunoreactivity led to a diagnosis of high grade ovarian papillary cystadenocarcinoma. To the best of our knowledge, this is the first case of small intestine serousal surface metastasis from ovarian papillary cystadenocarcinoma.

Asthma is one of the most common acute and chronic conditions in children, and the pediatricians are expected to provide an important role for asthma care in this age group, however there is no published information describing the different aspects of their practices about children asthma in Iran. This study was done to characterize the knowledge of the Iranian pediatricians about the diagnosis, treatment and education of asthma in children. Validated questionnaires were completed by 193 pediatricians from different parts of Iran during the International Congress of Pediatrics in Tehran. A total of 193 returned questionnaires (96.5%) were eligible for the survey and analysis. About 49% of the respondents were male and 18% were sub-specialists. Wheezing was the most common mentioned symptoms in taking asthma into consideration. About 40% of these physicians had no plan for doing spirometry in eligible children and 35.2% of them did not have familiarity with peak flowmeter. Also about 17.6% of them paid no regular visits to their asthmatic patients. Only 29% of the respondents indicated that they would prescribe inhaled corticosteroids for a 6-year-old child with moderate persistent asthma and 73.3% of them would prescribe inhaled bronchodilator (Salbutamol) for an acute asthmatic attack as the first drug, while 17.1% of them used epinephrine injection for this purpose. About 42.2% of the respondents did not consider any education or action plan for their patients and only 60.6% of them had access to standard guidelines and educational programs. The results show that there are numerous aspects of children asthma management in Iran which are not consistent with standardized guidelines and recommendations. This survey and the attained information suggest areas for interventions to improve the pediatricians' knowledge about asthma and the disease management.

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.

Background Although long-term intravenous immunoglobulin infusion is an effective treatment for children with antibody deficiencies, it can be complicated by systemic adverse reactions. Objective To evaluate the adverse reactions of intravenous immunoglobulin therapy in patients with primary immunodeficiency. Methods Seventy-one immunodeficient patients receiving intravenous immunoglobulin were evaluated during a 7-year period (1995–2002) at Children's Medical Center in Tehran, Iran. Immunological diagnoses were as follows: common variable immunodeficiency (31 patients), X-linked agammaglobulinemia (25 patients), IgG subclass deficiency (5 patients), hyper-IgM syndrome (2 patients), and ataxia-telangiectasia (8 patients). Results One hundred fifty-two cases (12.35%) of adverse reactions occurred following 1,231 infusions in 35 patients. The most frequent immediate adverse reactions were mild reactions (131 infusions), including chills, fever, flushing, muscle pains, nausea, headache, and anxiety. Moderate reactions, such as vomiting, chest pain, and wheezing, occurred in 19 infusions. Two patients experienced severe adverse reactions. The highest proportion (23.06%) of reaction to injection was in patients with common variable immunodeficiency. Conclusions Intravenous immunoglobulin is a well tolerated medical agent for patients with antibody deficiency. However, to prevent occurrence of immediate adverse reactions during infusion in these patients, physicians should perform a detailed history and proper physical examination and check the titer of anti-IgA.

Background: Identification of liver flukes, Fasciola hepatica, and Fasciola gigantica by morphometric parameters is not always reliable due to the overlapping measurements. This study aimed to characterize the liver flukes of animals from different parts of Iran by the genetic markers, ITS1, and COXI. Methods: We collected flukes from infected livestock in six provinces of Iran from Sep to Nov 2016. The flukes were identified by amplification of a 680 bp sequence of ITS1 locus followed by a restriction fragment polymorphism (RFLP) assay. The genetic diversity among isolates was evaluated by amplification and sequencing of a 493 bp fragment of the COXI gene. Results: We obtained 38 specimens from Khuzestan, 22 from Tehran, 10 from Isfahan, 10 from Mazandaran, 4 from Kurdistan, and 3 from Ardabil provinces. PCR-RFLP analysis revealed two patterns, representing F. hepatica, and F. gigantica. Fifty specimens from cattle and sheep exhibited F. hepatica pattern and 37 from the cattle, sheep, buffalo, and goat that of F. gigantica. The phylogeny based on COXI revealed two distinct clades separating F. hepatica from F. gigantica. In our phylogeny, the Iranian F. gigantica isolates showed a distinct separation from the African flukes, while grouped with the East Asia specimens demonstrating a common ancestor. The F. hepatica isolates clustered with the flukes from different parts of the world, including East Asia, Europe, and South America. Conclusion: The present study revealed a substantial genetic difference between F. gigantica populations of Asia and Africa, while F. hepatica isolates from different parts of the world shared high similarities.

Cryptosporial and bacterial co-infection is reported in a budgerigar with clinical manifestations of septicemia and respiratory tract infection. Microscopically large number of round to oval 2-5μm cryptosporidial organisms were found to be lodged on the parabronchial epithelial cells of the respiratory tract. The bacterial colonies were seen around the parabronchial spaces of the lung tissue. It is suggested that the C. baileyi is the most likely cryptosporidium species which caused respiratory cryptosporidiosis in the budgerigar.