Immunogenicity of 23-Valent Pneumococcal Vaccine in Children with Systemic Lupus Erythematosus

ABSTRACT Background: Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disease which is characterized by B-cell abnormality and auto-antibody generation. Since bacterial infections are the most important causes of mortality in these patients, pneumococcal vaccination is recommended for children with SLE. Objective: To investigate humoral immunity and specific-antibody formation in response to a 23-valent polysaccharide pneumococcal vaccination in SLE children and asthmatic control group. Method: The case and control groups consisted of 30 children with the mean age of 13 years who were matched by sex and age. Anti-pneumococcal antibody titers were determined using Enzyme-Linked Immunosorbent Assay (ELISA) before the vaccination with the 23-valent pneumococcal vaccine and 3 weeks later in both groups. Also the correlation between anti-pneumococcal antibody titer and different factors including age, sex, lupus activity, disease duration, medications, history of recurrent infections, and laboratory data were investigated. Results: Both groups showed significant increases in anti-pneumococcal antibody level after vaccination (p≤0.001). The increase in antibody level were almost the same in both groups (p≥0.05) such that 77.7% of SLE children and 86.2% of control children showed at least 2-fold increase in anti-pneumococcal antibody titer following immunization. Significant correlations were seen between the level of post-immunization anti-pneumococcal antibody with the age of children with SLE (p=0.02) and their age of disease onset (p=0.02). Conclusion: It is concluded that pneumococcal vaccination is generally immunogenic in children with SLE. However, a small group of patients show impaired response to the vaccine

Survival of a Severe Combined Immunodeficiency Patient from Transfusion-associated Graft-versus-host Disease: A Case Report

Patients with Severe Combined Immunodeficiency are at high risk of Transfusion Associated Graft Versus Host Disease (TAGVHD) if they receive a non-radiated blood product that has a high mortality rate. This Case study reports on the case of a premature baby who had anemia of prematurity with a normal level of WBC. He received packed red blood cells, but after a while, he developed severe nausea and skin lesions. Endoscopy was done and the biopsy from the gastrointestinal lesions as well as the biopsy from the skin lesions showed graft versus host disease when he was 6 months old. However, he has received Hematopoietic Stem Cell Transplantation and is well now after about 4 months from his transplant

The Relationship between Tuberculin Response, BCG Vaccine Scar and Asthma

Recent studies have proposed that a decline in bacterial infections such as tuberculosis is a factor underlying the rising prevalence and severity of atopic disorder in developed countries. There are conflicting ideas about the inverse relationship between BCG (Bacillus Calmette-Guerin) vaccination and asthma. Stronger response to tuberculin test as an indicator of more potent TH1 response is supposed to influence TH2 modulated allergic reactions. BCG scar considered as an indicator of TH1 - immunoresponse has been proposed to be smaller in asthmatic children in some studies. In a case-control study, 97 asthmatic and 97 control children younger than 5 years of age and BCG vaccinated at birth were tested with 5 units of tuberculin intradermaly. After 48-72 hours, the indurated area was measured in two diameters. Mean while, the scar of BCG in both groups was measured. Severity of asthma in the case group was recorded and categorized into mild, moderate and severe groups. The case group consisted of 63% boys and 37% girls and their tuberculin response was significantly smaller than that of the control (p=0.000), but no data supported the inverse relationship between the tuberculin response and severity of asthma (p=0.113). The scar of BCG was not significantly different in the asthmatic children with variable severity of asthma and control group (p=0.864). Children with definite asthma had a significant weaker response to tuberculin. This might be an indication of less potent TH1-reponse in allergic patients, but it was not associated with severity of asthma. No significant relationship between the size of BCG scar and asthma or its severity was found so perhaps BCG scar is not a sensitive indicator for development of asthma in future

Clinical and Serological Findings in Juvenile Patients with Idiopathic Arthritis in Southwestern of Iran

Introduction: The purpose of this study was to describe clinical features and serological findings of children with idiopathic arthritis in south-western Iran.Methods: This descriptive study included 60 patients with juvenile idiopathic arthritis who were referred to a pediatric rheumatology clinic at a university hospital during 6-month period. Initial manifestations, first laboratory tests and clinical course of patients were reviewed.Results: Sixty children (32 boys and 28 girls) with idiopathic arthritis ranged in age from 1.5 to 16 years. The mean age at the first presentation was 4.92 years (SD= 3.68). Oligoarthritis was the most common subtype in 27 (45%), followed by systemic- onset in 17 (28.3%) and polyarthritis in 16 (26.7%) of patients. The most commonly involved joints were knee 53(88.3%), ankle 28(46.6%) and wrist 27(45%). Uveitis was detected in two patients, and positivity for ANA titer was revealed in one patient. Conclusions: In this study, the pattern of most clinical features in different subtypes of juvenile idiopathic arthritis resembles to other studies. Positive ANA was less; however, the low numbers of Iranian patients with uveitis was noteworthy

Allergic bronchopulmonary aspergillosis in patients with cystic fibrosis and non-cystic fibrosis bronchiectasis

Background: Aspergillus sensitization (AS) and allergic bronchopulmonary aspergillosis (ABPA) can occur as a cause of permanent lung damage in patients with cystic fibrosis (CF) and non-CF bronchiectasis. Objective: The aim of this study was to determine the frequency of AS and ABPA in patients with CF and non- CF bronchiectasis in southwestern Iran. Methods: This cross-sectional study was conducted on 33 patients with CF and 27 patients with non-CF bronchiectasis from southwestern Iran who were referred to Namazi Hospital affiliated to Shiraz University of Medical Sciences from July 2015 to February 2016. Skin prick test to Aspergillus fumigatus, peripheral blood eosinophil count, total serum IgE, specific IgE and IgG against Aspergillus fumigatus as well as radiologic chest studies were done for each patient. Statistical analysis was done by Mann-Whitney U test, Fisher Exact test, and Kappa weighted in SPSS software version 18. Level of significance was set at p<0.05. Results: Nine patients with CF (27.3%) and one patient with non-CF bronchiectasis (3.7%) had positive skin tests to Aspergillus. There was 81.2% agreement between positive skin test and specific IgE to Aspergillus fumigatus (p<0.001). Three patients with CF (9%) met the diagnostic criteria for ABPA, whereas ABPA was not seen in patients with non-CF bronchiectasis. Conclusion: ABPA was low in this study, considering more frequency of AS in patients with cystic fibrosis, clinicians should keep in mind the diagnosis of ABPA for those CF patients that do not respond to usual medical therapy and have positive skin tests to Aspergillus allergens

Clinical and Genetic Features in Patients with Cystic Fibrosis in Southwestern Iran

Objective: Cystic fibrosis (CF) is a common autosomal recessive genetic disease caused by a mutation in the CF transmembrane conductance regulatory (CFTR ) gene. This study attempted to identify the most common CFTR mutations and any correlations between certain mutations and the clinical presentation of the disease in CF patients in southwestern Iran. Methods: Twenty nine common CFTR gene mutations were examined in 45 CF patients. Findings: Chronic cough, intestinal obstruction, dehydration, heat exhaustion and steatorrhea were the most common early clinical symptoms among our patients. The most common mutation was ΔF508, with an allele frequency of 21%. The homozygous ΔF508 mutation was observed in eight patients (18%), and three patients (7%) were ΔF508 carriers. The 2183AA>G mutation was observed in four patients, one of whom was also a ΔF508 carrier. The R1162X mutation was detected in two patients. The G542X, R334W and N1303K mutations were detected each in one patient, the first of whom was also a ΔF508 carrier. Conclusion: Out of 45 patients, 27 (60%) had none of the CFTR gene mutations we tested for. The most frequent mutations in southwestern Iranian patients with CF should be identified by sequencing the entire CFTR gene in order to optimize the design of a diagnostic kit for common regional mutations

Severe Allergic Bronchopulmonary Mycosis and Long-Term Follow-Up

Allergic bronchopulmonary aspergillosis (ABPA) is the most common immunologic reaction following fungal allergen exposure in asthmatic patients. A less frequent syndrome in response to other fungal species like candida is allergic bronchopulmonary mycosis (ABPM). This reaction is mostly associated with asthma exacerbation, changes in Immunoglobulin E levels, and nonspecific findings in high resolution computed tomography (HRCT). This study presents a 9-year-old girl, a known case of childhood asthma, resolved 4 years ago as a novel case of ABPM due to Candida albicans manifested by severe emphysema, bronchiectasis, and pneumothorax which consequently required long-term treatment to get relieved

An unusual case of cystic fibrosis with pancytopenia due to copper deficiency and blindness caused by vitamin A deficiency: A case-report

Cystic fibrosis (CF) is a multi-systemic autosomal recessive disease which mostly involves the respiratory, digestive, and reproductive systems, but it can present with various clinical presentations, especially in adulthood. We describe a 19-year-old boy, a known case of CF who presented with less known clinical presentations of CF, blindness, liver cirrhosis, vitamin A deficiency, and pancytopenia