Evaluation of humoral immune function in patients with bronchiectasis

Bronchiectasis is a chronic debilitating condition characterized by abnormal dilated thickwalled bronchi. To investigate humoral immune function in bronchiectatic patients, this study was performed. Forty patients with established diagnosis of bronchiectasis, who were referred from two tertiary care pulmonology centers in Tehran, were investigated in this study. Immunoglobulin isotypes concentrations and IgG-subclasses were measured by nephelometry and enzymelinked immunosorbent assay [ELISA] methods, respectively. All patients received unconjugated pneumococcal vaccine, and blood samples were taken before and 21 days after vaccination. Specific antibodies against whole pneumococcal antigens were measured using the ELISA method. Fifteen [37.5%] out of 40 patients were diagnosed to have defects in antibody mediated immunity including 5 [12.5%] patients with immunoglobulin class deficiency [2 with common variable immunodeficiency and 3 with IgA deficiency], 3 [7.5%] with IgG subclass deficiency and 7 [17.5%] patients had Specific antibody deficiency [SAD] against polysaccharide antigen despite normal levels of serum immunoglobulins and IgG subclasses. Our study along with several other studies confirmed that all patients with bronchiectasis should undergo thorough immunological evaluation in order to identify the presence of the underlying immunologic defect. This evaluation should include serum immunoglobulins, IgG subclasses concentrations and also determination of serum antibodies against pneumococcal antigens. Early diagnosis and appropriate treatment will prevent the subsequent complications and improve quality of life of affected individuals

Leukocyte adhesion deficiency type 1 presenting as leukemoid reaction.

Leukocyte Adhesion Deficiency (LAD) is characterized by the inability of leukocytes, in particular neutrophilic granulocytes, to emigrate from the blood stream towards sites of inflammation. Infectious foci are nonpurulent and may eventually become necrotic because of abnormal wound healing. LAD-I is characterized by the absence of the beta-2 integrins CD II and CD 18 on leukocytes. We present a male neonate referred with continuation of leukemoid reaction in spite of antibiotic therapy of simultaneous omphalitis. The diagnosis of LAD-I was confirmed by flow cytometry, showing a deficiency of beta-2 integrins on the surface of the leukocytes. After management, the patient was discharged with prophylactic antibiotics.

Sweat test in asthmatic children; a single center study

Asthma and Cystic Fibrosis [CF] have some identical manifestations. In the present study, the frequency of positive sweat test was assessed in asthmatic children. This cross-sectional study was performed in asthmatic children, who were referred to Bahrami Children Hospital, Tehran, during July 2003 to July 2005. Sweat test was performed for all children. One hundred and thirty five [95 males and 40 females] asthmatic were studied. Sweat test was positive in 35 [26%] patients. Major signs and symptoms among these 35 patients were: Cough [35/35], Dyspnea [7/35], Chronic Diarrhea [6/35], and steatorrhea [1/35]. Failure to thrive was found in 31 of 35 patients. The mean duration of asthma in patients with positive test was 49.3 months, which was significantly higher than 18.1 months in the group with negative test [P=0.001]. Significant relations between recurrent respiratory tract infections [P=0.029], chronic diarrhea [P=0.001], failure to thrive [P=0.0001], and positive sweat test were found. Sweat test should be recommended in asthmatic children with recurrent upper respiratory tract infections, long duration of asthma, chronic diarrhea, and failure to thrive

Clinical and laboratory findings in Iranian children with cyclic neutropenia

Cyclic neutropenia is a rare immunodeficiency syndrome, characterized by regular periodic oscillations in the circulating neutrophil count from normal to neutropenic levels through 3 weeks period, and lasting for 3-6 days. In order to determine the clinical features of cyclic neutropenia, this study was performed. Seven patients with cyclic neutropenia [3 males and 4 females], who experienced neutropenic periods every 3 weeks [5 with severe and 2 with moderate neutropenia], were investigated in this study. They had been referred to Iranian Primary Immunodeficiency Registry during 23 years [1980-2003]. The range of patients' ages was from 7 to 13 years [median 11 years]. The median age at the onset of the disease was 12 months [1 month- 2 years] and the median age of diagnosis was 2 [1.5-5] years, with a median diagnosis delay of 1 year [2 months- 5 years]. Neutropenia was associated with leukopenia [3 patients], anemia [3 patients], and thrombocytopenia [1 patient]. Patients were asymptomatic in healthy phase, but during the episode of neutropenia suffered from aphthous ulcers, abscesses and overwhelming infections. The most commonly occurred manifestations were: otitis media [6 cases], oral ulcers [5 cases], abscesses [4 cases], pneumonia [3 cases], diarrhea [3 cases], oral candidiasis [3 cases], cutaneous infections [2 cases], and periodontitis [2 cases]. One of these patients subsequently died because of recurrent infections. Unusual, persistent or severe infections should be the initiating factors to search for an immune deficiency syndrome such as cyclic neutropenia, because a delay in diagnosis may result in chronic infection, irretrievable end-organ damage or even death of the patient

Neutropenia in patients with primary antibody deficiency disorders

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

Gastrointestinal manifestations of patients with Chronic Granulomatous Disease

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

prevalence of asthma among the students [7-18 years old] in Tehran during 2002-2003

Asthma is one of the most common problems of childhood, responsible for a significant proportion of abstinence from school because of chronic illness. This study was carried out among the school-aged children [7-18 years] in Tehran schools during 2002-2003, in order to determine the frequency of asthma. According to the recommendation of WHO [World Health Organization], we designed a questionnaire, containing 8 standard questions, and the students were given necessary information to complete the questionnaires. The pre-high and high schools students completed the questionnaires but the parents of primary school students completed them on their behalf. The prevalence of asthma was 35.4% in Tehran; this prevalence was higher in the boys [37.1%], as compared to the girls [33.5%]. The prevalence of this disease has been estimated about 39.5% in pre-high schools, 35.4% in high schools and 31.6% in primary students. Based on this survey, the most common clinical manifestations of asthma were: prolonged cough lasting more than 10 days [22.4%], and exercise-induced wheezing or dyspnea [16.9%], followed by repeated dyspnea or wheezing [6.4%].The prevalence of asthma is high among the students of Tehran schools and it needs more careful screening programs along with additional information to the patients and parents about the disease