Interventional pulmonary procedures and their outcomes in patients with STAT3 hyper IgE syndrome.

BACKGROUND: STAT3 hyperimmunoglobulin E syndrome (STAT3-HIES) also referred to as autosomal dominant HIES (AD-HIES) is an inborn error of immunity characterized by the classic triad of eczema, frequent opportunistic infections, and elevated serum IgE levels. As a consequence of lung sequels due to repeated infections and impaired tissue healing, patients may require interventional pulmonary procedures. METHOD: Four patients with dominant-negative STAT3 mutations who had received interventional pulmonary procedures were enrolled. The demographic, clinical, and molecular characteristics were gathered through a medical record search. All reported STAT3-HIES patients in the literature requiring pulmonary procedures as part of their treatment were reviewed. RESULT: Recurrent episodes of pneumonia and lung abscess were the most prevalent symptoms. The most common non-immunological features were scoliosis, failure to thrive, and dental problems such as primary teeth retention and disseminated decays. Bronchiectasis, lung abscess, pneumatocele, and cavitary lesion were the most prevalent finding on high-resolution computed tomography at the earliest recording. All patients underwent pulmonary surgery and two of them experienced complications. CONCLUSION: Patients with STAT3-HIES have marked pulmonary infection susceptibility which may necessitate thoracic surgeries. Since surgical procedures involve a high risk of complication, surgical options are recommended to be utilized only in cases of drug resistance or emergencies.

Investigation of the Relationship between Aspirin-Sensitivity and Poor Response to Medical Management in NSAIDs-exacerbated Respiratory Disease Patients with Sinonasal Polyposis.

Background: NSAID-exacerbated respiratory disease (N-ERD) is a highly heterogeneous disorder with various clinical symptoms. The aspirin challenge test is a gold standard method for its diagnosis, and there are still no reliable in vitro diagnostic biomarkers yet. Oral challenge tests are time-consuming and may be associated with a risk of severe systemic reactions. This study aimed to evaluate whether patients with poor responses to medical management are more susceptible to being aspirin-sensitive. Methods: In this cohort study, after CT scanning of all patients and subject selection, conventional medical treatment was started as follows and continued for three consecutive months: at first, saline nose wash twice per day, intranasal beclomethasone spray one puff in each nostril twice per day, montelukast 10 mg tablet once daily, a ten-day course of oral prednisolone starting with the dose of 25 mg per day and taper and discontinued thereafter. Sinonasal outcome test 22 (SNOT22) was used for the evaluation of symptom severity. Statistical analyses were performed with SPSS version 23, and data were analyzed using an independent samples T-test, paired T-test, and Receiver operating curve analysis. Results: 25 males and 53 females were enrolled in this study, with an average age of 41.56 ± 11.74 years old (18-36). Aspirin challenge test results were positive in 29 (37.2%) patients. The average SNOT22 scores before the treatment were 52.97 ± 17.73 and 47.04 ± 18.30 in aspirin-sensitive and aspirin-tolerant patients, respectively, and decreased to 27.41 ± 16.61 and 24.88 ± 16.72 in aspirin-sensitive and aspirin-tolerant patients after the treatment, respectively. There was no significant difference in SNOT22 scores between the groups. Conclusion: The severity of symptoms before treatment and clinical improvement after treatment are not good predictors of N-ERD.

Protective role of vitamin D status against COVID-19: a mini-review.

An outbreak of pneumonia caused by a severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection is called COVID-19 and has led to a pandemic worldwide. It is reasonable to investigate and control factors affecting disease severity and mortality. The relation between vitamin D and viral pneumonia has been previously reported. Vitamin D deficiency is common and may increase hospital admission and mortality rate in patients with COVID-19. This mini-review examines the pathways that show the association between vitamin D and COVID-19. On the other hand, it deals with the available evidence related to the relationship between vitamin D deficiency and the effect of vitamin D supplementation on the prevalence, severity, and mortality of COVID-19. Also, we described the pathophysiology of the organs' involvement in COVID-19 and the effect of vitamin D on these outcomes. Vitamin D strengthens the innate and adaptive immune system, modulates immune responses, prevents lung and cardiovascular system damage, and reduces thrombotic events. Vitamin D exerts these effects in several pathways. Vitamin D prevents virus entry and replication by maintaining the integrity of the body's physical barrier. Vitamin D reduces the damage to vital organs and thrombotic events by increasing the level of Angiotensin-converting enzyme 2 (ACE2), nitric oxide, and antioxidants or by reducing inflammatory cytokines and free radicals. Sufficient vitamin D may be reduced morbidity and mortality due to COVID-19. However, this issue should be investigated and confirmed by further research in the future.

Nurses' Knowledge Regarding Oxygen Therapy; a Cross-Sectional Study.

Introduction: Oxygen therapy, if done correctly, can save patients' life promptly. However, improper use will be just as dangerous. The present study aimed to investigate the level of nurses' knowledge on properly using oxygen. Method: This was a cross-sectional study with a minimum sample size of 72 nurses who were randomly selected from various wards of Masih Daneshvari Hospital, Tehran, Iran. To determine the level of knowledge about oxygen therapy, a questionnaire was used to collect data. This questionnaire consists of seven items, each of which is designed to determine the level of the individual's knowledge about the various details of oxygen therapy. Results: Seventy-eight nurses with the mean age of 35.80±7.42 years participated in the study (87% female). The mean knowledge score of nurses regarding oxygen therapy was 8.89 ± 2.79 out of 16 points. 84.6% of the nurses were able to differentiate various types of oxygen masks. Accordingly, 94.9% of nurses had good knowledge on oxygen humidification. Also, 50% of the nurses had sufficient knowledge about the amount of oxygen flow produced by different masks. 10.3% of the nurses could choose the most appropriate mask for different clinical conditions. 6.4% of the nurses had knowledge of working with flowmeters, and 15.4% of the nurses had sufficient information about the maximum level of oxygen required for the patient. 17.9% of the nurses were familiar with measuring the appropriate amount of oxygen for patients. There was no statistically significant relationship between age (p = 0.57), gender (p = 0.09), employment status (p = 0.38), workplace (p = 0.86), current position (p = 0.11), degree (p = 0.27), and graduation time (p = 0.58) of nurses with good knowledge of using oxygen. However, a statistically significant relationship was reported between nurses' related work experience and their knowledge of the proper use of oxygen (p = 0.03). Conclusion: In general, the nurses' knowledge at Masih Daneshvari Hospital on how to properly use oxygen is at a moderate level. Nurses' knowledge in some areas, such as working with the flowmeter, choosing the suitable mask for specific clinical conditions, and the maximum oxygen required for patients, is meager and requires training intervention.

Expression of miRNA1, miRNA133, miRNA191, and miRNA24, as Good Biomarkers, in Non-Small Cell Lung Cancer Using Real-Time PCR Method.

BACKGROUND: Lung cancer has recently shown the highest incidence among all cancers. microRNAs (miRNAs) are the molecules playing a role in regulating gene expression and contributing to many pathogenic mechanisms. Therefore, these molecules could be used as biomarkers for the detection, anticipation, and treatment of cancer. With this in mind, we decided to investigate and compare the expression of miR-1, miR-133, miR-191, and miR-24 and also the expression differences in these four RNA molecules between lung cancer patients and the controls. METHODS: A total of 50 patients with lung cancer participated in this study. In addition, 50 healthy blood samples were selected as the control group. Real-time PCR determined the expression levels of miRNA. The RNAs extracted from the patients' white blood cells were initially synthesized, and then cDNA was extracted. Finally, the synthesized cDNA was amplified using real-time PCR, and its expression was compared with the control group. RESULTS: The result indicated a low expression level of miR-1 and miR-133, and a high expression level of miR-191 and miR-24 in the blood of patients with lung cancer compared to the healthy subjects. CONCLUSION: Our findings revealed that miR-1, miR-133, miR-191, and miR-24 are oncogenes, and their expression could result in cancer. It appears that a therapy to overexpress miR-1 and miR-133 and downexpress miR-191 and miR-24 could contribute to the treatment of lung cancer.

Allergic shiners in a patient with cough-variant asthma: a case report.

BACKGROUND: Chronic cough, with a duration of coughing of more than 8 weeks in adults, affects 5-10% of the general population. One of the most common causes of chronic cough is cough-variant asthma, which accounts for approximately one-third of cases. This phenotype of asthma is characterized by extreme sensitivity of the neuronal pathways mediating cough to environmental irritants, which results in an urge to cough. This case is an example of cough-variant asthma presenting with allergic shiners due to her severe cough. CASE PRESENTATION: A 38-year-old Iranian woman, who was well before the start of the coronavirus disease 2019 pandemic, presented with a nonproductive hacking cough that had begun after excessive use of antiseptic solutions. The only positive finding on physical examination was a reddish-purple rash on and around the eyelids mimicking a heliotrope rash, which had probably evolved due to the severity of the cough. The results of the pulmonary function test were within normal limits. Methacholine challenge test and chest x-ray were both normal. Chest high-resolution computed tomography revealed hyperinflation and tree-in-bud opacities. All other laboratory tests were normal. Because of the reversibility in her pulmonary function test, despite normal baseline parameters, asthma treatment was initiated, resulting in disappearance of the cough and the eye discoloration, being indicative of the correct diagnosis and proper treatment. CONCLUSION: Patients with cough-variant asthma may often have no other classic symptoms of asthma other than cough.

Evaluating Possible Mechanisms Linking Obesity to COVID-19: a Narrative Review.

Currently, pneumonia caused by the coronavirus disease 2019 (COVID-19) is a pandemic. To date, there is no specific antiviral treatment for the disease, and universal access to the vaccine is a serious challenge. Some observational studies have shown that COVID-19 is more common in countries with a high prevalence of obesity and that people with COVID-19 have a higher body mass index. In these studies, obesity increased the risk of disease, as well as its severity and mortality. This study aimed to review the mechanisms that link obesity to COVID-19.

Respiratory pattern complexity in newly-diagnosed asthmatic patients.

BACKGROUND: The intensity of respiratory symptoms and expiratory airflow limitations in asthma fluctuate over time. Some studies have reported variable complexity of the respiratory patterns in asthmatic patients. Thus, we conducted a novel study to assess the correlation between asthma severity and breathing pattern dynamics in newly-diagnosed asthmatic patients. METHODS: A total of 20 newly-diagnosed asthmatic patients (7 male, 13 female) and 20 healthy cases (11 male, 9 female) were included. The respiratory patterns of all participants and the asthma severity for asthmatic patients were measured using a spirometer (before and after a bronchodilator exposure) and airflow recorder, respectively. The peak-to-peak intervals and the amplitude of peaks were considered as the inter-breath interval (IBI) and lung volume (LV) series. The Detrended Fluctuation Analysis (DFA), Sample Entropy (SampEn), Multi-scale Entropy (MSE), short-term (SD1) and long-term (SD2) variability, and IBI and LV Cross-Sample Entropy of the respiratory pattern dynamics were calculated using MATLAB (Mathwork, USA). RESULTS: Asthma patients showed notable increase in the average of sample entropy in both IBI and LV parameters (p = 0.025 and p = 0.018, respectively) and also decreased synchronization between IBI and LV (p = 0.042). The multi-scale sample entropy of both IBI and LV was significantly higher in asthmatic patients (p < 0.05). Furthermore, SD1 and SD2 were higher in the patients with asthma (p < 0.05). Significant correlations were detected between spirometric (forced expiratory flow (FEF) change, pre FEF, pre forced expiratory volume in one second (FEV1) / forced vital capacity (FVC), FVC change) and respiratory pattern (mean-IBI, mean-LV, mean-respiratory rate (RR), coefficient of variation (CV)-IBI, CV-LV, cross-sample entropy) parameters (p < 0.05). Furthermore, we identified a negative correlation between CV of IBI and asthma severity (r = -0.52, p = 0.021). CONCLUSION: Here, we took a novel approach and observed increased irregularity (more complexity) in the breathing pattern of patients newly-diagnosed with asthma. Remarkable correlations were detected between breathing complexity markers and spirometric indices along with disease severity in asthmatic patients. Thus, our data suggests respiratory pattern indices could be utilized as an indicator of asthma and its severity. However, more clinical data are required to support this conclusion.

Leukocytoclastic vasculitis in patients with IL12B or IL12RB1 deficiency: case report and review of the literature.

BACKGROUND: Mendelian susceptibility to mycobacterial disease (MSMD) is an inborn error of immunity, resulting in susceptibility to weakly virulent mycobacteria and other intramacrophagic pathogens. Rheumatologic manifestations and vasculitis are considered rare manifestations in MSMD patients. CASE PRESENTATION: In this study, we reported a 20-year-old female who was presented with recurrent lymphadenitis following bacillus Calmette-Guérin (BCG) vaccination and a history of recurrent disseminated rash diagnosed as leukocytoclastic vasculitis (LCV). A slight reduction in lymphocyte subsets including CD4+, CD19+, and CD 16 + 56 T-cell count, as well as an elevation in immunoglobulins level (IgG, IgA, IgM, IgE), were observed in the patient. Whole exome sequencing revealed a homozygous Indel-frameshift mutation, c.527_528delCT (p. S176Cfs*12), at the exon 5 of the IL12B gene. She experienced symptom resolution after treatment with anti-mycobacterial agents and subcutaneous IFN-γ. We conducted a manual literature search for MSMD patients reported with vasculitis in PubMed, Web of Science, and Scopus databases. A total of 18 MSMD patients were found to be affected by a variety of vasculitis phenotypes mainly including LCV and Henoch-Schönlein purpura (HSP) with often skin involvement. Patients were all involved with vasculitis at the median age of 6.8 (2.6-7.7) years, nearly 6.1 years after the initial presentations. Sixteen patients (88.9%) had IL12RB1 defects and concurrent Salmonella infection was reported in 15 (88.2%) patients. CONCLUSION: The lack of IL-12 and IL-23 signaling/activity/function and salmonella infection may be triggering factors for the development of leukocytoclastic vasculitis. IL12B or IL12RB1 deficiency and salmonellosis should be considered in MSMD patients with vasculitis.

The Effect of Aspirin on Moderate to Severe Asthmatic Patients with Aspirin Hypersensitivity, Chronic Rhinosinusitis, and Nasal Polyposis.

Asthmatic patients may have aspirin-exacerbated respiratory disease and experience acute dyspnea and nasal symptoms within 3 hours after the ingestion of aspirin. This study aimed to evaluate the effect and outcome of daily low-dose aspirin in the treatment of moderate to severe asthma in patients with concomitant aspirin hypersensitivity and chronic rhinosinusitis with nasal polyposis (CRSwNP). This clinical trial was conducted from February 2014 to February 2015 on 46 adult patients with moderate to severe asthma accompanied by CRSwNP. Patients with a positive aspirin challenge were blindly randomized in three groups receiving placebo/day (A); aspirin 100 mg/day (B); and aspirin 325mg/day (C), respectively. Clinical findings, FEV1 and ACT scores were recorded and compared before, during, and after treatment for 6 months. Of 46 participants at baseline, 30 patients completed this 6-month trial study. The level of asthma control was significant; based on Asthma Control Test (ACT) when comparing the results in groups A and C and also groups B and C, but it was not significant when comparing ACT scores between groups A and B. FEV1 before and after treatment was significant when comparing groups A and B, groups A and C, and groups B and C. To conclude, aspirin desensitization with a daily dose of 325 mg aspirin resulted in the improvement of long-term control of asthma. A daily aspirin dose of 100 mg was not associated with such an increase in ACT score.

The Prevalence of Atopic Manifestations in 313 Iranian Patients with Inborn Errors of Immunity.

INTRODUCTION: Inborn errors of immunity (IEIs) are rare inherited disorders with a broad spectrum of manifestations. Here, we aimed to delineate the atopy burden in a cohort of patients with IEIs. METHODS: 313 patients with IEIs were enrolled in the study within a 9-years period, and data were collected via a questionnaire. All statistical analyses were performed using SPSS software (v. 25.0, Chicago, IL, USA). The statistical significance level was set at p < 0.05. RESULTS: Overall, 51 out of 313 (16.3%) patients were identified to have atopic manifestations. Food allergy was detected in 34 (10.2%), atopic dermatitis in 21 (6.7%), as well as allergic asthma and allergic rhinitis each in 4 (1.3%) patients. The allergic disorders were reported as initial manifestations among 14 out of 35 (40.0%) atopic patients. Most of these 51 patients fell within the category of combined immunodeficiency (CID) (n = 38, 74.5%), followed by, severe CID (SCID) (n = 5, 9.8%), common variable immunodeficiency (n = 3, 5.9%), chronic granulomatous disease (n = 3, 5.9%), selective IgA deficiency (n = 1, 2.0%), and leukocyte adhesion defect (n = 1, 2.0%). No patient with Mendelian susceptibility to mycobacteria was found to have atopic manifestation. Atopic dermatitis (p = 0.001) and food allergy (p < 0.001) were both significantly higher in patients with CID than in other IEI groups. Among atopic patients with CID and SCID, food allergy and atopic dermatitis were the most prevalent comorbidities. DISCUSSION/CONCLUSION: Atopic diseases may contribute to the clinical picture of IEIs, particularly in patients with CID. Atopy in association with other warning signs of IEIs increases the possibility of an underlying IEI.

Evaluation of a new protocol for wheat desensitization in patients with wheat-induced anaphylaxis.

AIM: New approaches such as oral immunotherapy (OIT) may be useful in IgE-mediated anaphylaxis to wheat. PATIENTS & METHODS: 12 patients underwent OIT protocol that comprised of two phases: the first with semolina flour and the second with spaghetti. Total and specific wheat IgE were assayed by ELISA before and after OIT and 18 months later. Skin prick tests were also performed. RESULTS: Patients successfully tolerated 50 g of wheat. The median baseline total IgE was decreased after up-dosing phase and decreased after follow-up (p < 0.01). The median baseline wheat-specific IgE was increased after up-dosing and decreased after follow-up (p < 0.001). CONCLUSION: The efficiency and safety of our OIT protocol were shown on wheat allergic patients but further investigation is needed.

Cow's Milk Desensitization in Anaphylactic Patients: A New Personalized-dose Method.

Cow's milk allergy (CMA) is the most frequent food allergy in children and oral immunotherapy (OIT) is a promising approach for treatment of patients. The most challenging cases are anaphylactic with coexisting asthma and proposing safe protocols is crucial especially in high risk groups. Considering that CMA varies among patients, an individualized OIT protocol would be beneficial to achieve a safer and more efficient method of desensitization. 18 children more than 3 years of age with IgE-mediated CMA were enrolled. CMA was confirmed by positive skin prick test (SPT) and positive oral food challenge (OFC) and 60% of individuals had a convincing history of persistent asthma. SPT with milk extracts, whole fresh milk and serially diluted milk concentrations were performed.  The dilution of milk that induced 3-5 mm of wheal in each individual was selected as the starting dilution for OIT. Desensitization began by 1 drop of the defined dilution and continued increasingly. Overall, 16 out of 18 children (88.8%) achieved the daily intake of 120 mL of milk. Four out of these 16 children accomplished the protocol without any adverse allergic reactions. 12 patients experienced mild to severe reactions. Wheal and erythema in SPT (p≤0.001), and sIgE (p≤0.003) to most milk allergens were significantly decreased following desensitization. We successfully desensitized 16 of 18 children with IgE-mediated CMA by individualized desensitization protocol. Individualizing the OIT protocol would be helpful to save time and perhaps to relieve the allergic symptoms after ingesting cow's milk intake.

Characteristics, Etiology and Treatment of Pediatric and Adult Anaphylaxis in Iran.

Despite the increasing prevalence of anaphylaxis, there is little information about the characteristics and practice of healthcare providers in treating anaphylaxis, so this study was conducted to record the characteristics and therapeutic approaches of anaphylaxis from May 2012 until April 2015, the data of all patients diagnosed with anaphylaxis in the Allergy department of three referral university hospitals in Tehran, Iran were recorded. Thereafter, the demographics, clinical features, triggers and therapeutic approach were evaluated. This study investigated 136 individuals, 64 males (47%) between 6 months and 68 years old, as well as 72 others (52.94%) under 18 years of age (pediatric). The following were the most common organs involved: Skin 86.02% (pediatric 91.66% vs adult 79.68%), respiratory tract 51.47% (pediatric 43.05% vs adult 60.93%), cardiovascular 50.73% (pediatric 54.16% vs adult 46.87%), gastrointestinal 20.58% (pediatric 27.7% vs adult 12.5% ) and neurologic system 5.88% (only in adults). The following were the most identified causing foods 69 (50.37%)[42 pediatric (children) and 27 adults], drugs 34( 25%)[14 pediatric and 20 adults], idiopathic 16( 11.77%)[3 pediatric and 13 adults], insect sting 7( 5.15%)[3 pediatric and 4 adults] , exercise 6( 4.42%) [1 pediatric and 5 adults]. Milk, egg and wheat were the most common causative foods in pediatric cases but sesame, as well as egg and milk were the most common causes in adults. Epinephrine injection, auto injector epinephrine prescription as a discharging plan and referral to an allergist were: 10.78, 1.96 and 7.8 %, respectively. In this case series we found that, cutaneous, respiratory, cardiovascular and gastrointestinal complains were the most common manifestations and food, drug and idiopathic were the most common causes.In this study, the diagnosis of anaphylaxis, epinephrine subscription and referral to an allergist were significantly lower in comparison to other studies.

LPS-Responsive Beige-Like Anchor Gene Mutation Associated With Possible Bronchiolitis Obliterans Organizing Pneumonia Associated With Hypogammaglobulinemia and Normal IgM Phenotype and Low Number of B Cells.

LPS-Responsive Beige-like Anchor (LRBA) deficiency is a disease which has recently been described in a group of patients with common variable immunodeficiency (CVID) in association with autoimmunity and/or inflammatory bowel disease (IBD)-like phenotype. We here describe a 10-year-old boy who experienced recurrent infections, mainly in the respiratory system, associated with thrombocytopenia and anemia. Immunological workup showed low numbers of B cells and low IgG, but normal IgM levels. In spite of therapeutic doses of antibiotics, antivirals, and antifungal agents, in addition to immunoglobulin replacement therapy, he developed disseminated involvement of both lungs with peripheral nodules; transbronchial lung biopsy revealed possible bronchiolitis obliterans organizing pneumonia (BOOP). Combined homozygosity mapping and exome sequencing identified a homozygous LRBA mutation in this patient (p.Asp248Glufs*2). Such clinical and immunological findings have not been described to date and illustrate the broad and variable clinical phenotype of human LRBA deficiency.

Phenotyping and follow up of forty-seven Iranian patients with common variable immunodeficiency

BACKGROUND: Common variable immune deficiency (CVID) is a heterogeneous syndrome with a wide variety of signs and symptoms. This study describes the phenotyping and survival of the CVID patients in the allergy and clinical immunology department of Rasol-E-Akram Hospital of Iran University of Medical Sciences in Tehran. METHOD: We retrospectively reviewed hospital files of CVID patients in our department until January 2014. All patients were diagnosed with standard diagnostic criteria of CVID, treated and visited monthly, during the follow-up period. We divided the patients into four phenotypes; infection only, cytopenia, polyclonal lymphocytic infiltration and unexplained enteropathy. The immunologic, demographic and clinical findings in different phenotypes were analysed. RESULTS: The study included 47 CVID patients with mean age at onset of symptoms and diagnosis of 11.2 and 20.2 years, respectively. Phenotyping of our patients was: only infection (62%), cytopenia (26%) and PLI (19%) and 94% of cases had only one phenotype. We did not find a significant relation between the clinical phenotypes and immunologic or demographic data. Rate of parental consanguinity in our cases was 47%. Parental consanguinity was related to lower age at onset, lower age at diagnosis and higher baseline IgG levels. Patients with malignancy and autoimmunity had significantly higher age at onset. Our patients were followed-up for 6.9 years and the mortality rate during this time was 6%. CONCLUSIONS: Parental consanguinity and age at onset of CVID symptoms may have important roles in CVID manifestations

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Phenotyping and follow up of forty-seven Iranian patients with common variable immunodeficiency.

BACKGROUND: Common variable immune deficiency (CVID) is a heterogeneous syndrome with a wide variety of signs and symptoms. This study describes the phenotyping and survival of the CVID patients in the allergy and clinical immunology department of Rasol-E-Akram Hospital of Iran University of Medical Sciences in Tehran. METHOD: We retrospectively reviewed hospital files of CVID patients in our department until January 2014. All patients were diagnosed with standard diagnostic criteria of CVID, treated and visited monthly, during the follow-up period. We divided the patients into four phenotypes; infection only, cytopenia, polyclonal lymphocytic infiltration and unexplained enteropathy. The immunologic, demographic and clinical findings in different phenotypes were analysed. RESULTS: The study included 47 CVID patients with mean age at onset of symptoms and diagnosis of 11.2 and 20.2 years, respectively. Phenotyping of our patients was: only infection (62%), cytopenia (26%) and PLI (19%) and 94% of cases had only one phenotype. We did not find a significant relation between the clinical phenotypes and immunologic or demographic data. Rate of parental consanguinity in our cases was 47%. Parental consanguinity was related to lower age at onset, lower age at diagnosis and higher baseline IgG levels. Patients with malignancy and autoimmunity had significantly higher age at onset. Our patients were followed-up for 6.9 years and the mortality rate during this time was 6%. CONCLUSIONS: Parental consanguinity and age at onset of CVID symptoms may have important roles in CVID manifestations.

The effects of vitamin D supplementation on airway functions in mild to moderate persistent asthma.

BACKGROUND: Vitamin D is hypothesized to have some roles in innate and adaptive immunity, inflammation reduction, and remodeling; therefore, it is supposed to affect the asthma phenotype, severity, and response to inhaled corticosteroid (ICS). OBJECTIVE: To explore the synergistic effects of vitamin D supplementation in addition to asthma controllers (ICS or ICS plus long-acting ß-agonist) on airway functions. METHODS: A randomized clinical trial was conducted in 130 individuals aged 10 to 50 years who lived in Tehran during a 24-week period. Data on age, sex, body mass index, stage of asthma, serum total IgE, history of allergic rhinitis, atopic dermatitis, food allergy, and urticaria were collected. Spirometric parameters (forced expiratory volume in 1 second [FEV1] and ratio of FEV1 to forced vital capacity) and serum vitamin D measurement were obtained before and 8 and 24 weeks after the intervention. Patients were divided in 2 groups randomly. Both groups received asthma controllers (budesonide or budesonide plus formoterol) according to their stage, but the intervention group received vitamin D supplementation (100,000-U bolus intramuscularly plus 50,000 U orally weekly) in addition to asthma controllers. RESULTS: FEV1 improved significantly in both groups after 8 weeks, but no significant difference was found between the 2 groups at baseline (P = .20) or after 8 weeks (P = .99); however, a significant improvement was seen in the intervention group in the last 16 weeks, and FEV1 was significantly better in the intervention group than the other group after 24 weeks (P < .001). CONCLUSION: Vitamin D supplementation associated with asthma controllers could significantly improve FEV1 in mild to moderate persistent asthma after 24 weeks. TRIAL REGISTRATION: irct.ir Identifier: IRCT201302079608N1.

The impact of preload alteration on the myocardial performance index through implementing positive end expiratory pressure.

Load independent methods should be used for the assessment of ventricular function. Debate still exists regarding whether tissue Doppler imaging (TDI) indices are influenced by preload. Here, we evaluated the effect of positive end expiratory pressure (PEEP) related preload reduction on both conventional pulsed Doppler (PD) and TDI myocardial performance index (MPI). Thirty-eight mechanically ventilated patients of 3 months to 12 years old (mean ± SD age of 30 ± 11 months) without overt heart disease were enrolled. Doppler mitral inflow velocities, isovolumetric contraction and relaxation times and aortic ejection time in addition to TDI peak systolic, early and late diastolic velocities from the basal segment of left ventricular lateral wall were determined for each patient before and after applying high PEEP (10 cmH(2) O).PD-MPI was load dependent (0.61 ± 0.22 vs. 0.78 ± 0.25, P = 0.002). However, TDI-MPI did not significantly change after the use of high PEEP declining the left ventricular volume loading (0.78 ± 0.21 vs. 0.84 ± 0.22, P = 0.23). Hence, regarding various interfering pathophysiologic factors particularly preload reduction, it seems that TDI-MPI would be a more reliable index for the assessment of ventricular function.

Assessment of left atrial ejection force in mildly asphyxiated newborns.

OBJECTIVE: Asphyxia-induced cardiac insult is one of the major causes of mortality and morbidity in the course of perinatal asphyxia. Nowadays, a remarkable trend of interest is sensed introducing a plausible modality for early detection of cardiac insults at the beginning stages of asphyxia. In this study we aimed to evaluate diagnostic utility of transmitral Doppler-derived parameters as well as left atrial ejection force index as a marker of left atrial contractile function in these patients. METHODS: In a prospective study selected cases of 26 asphyxiated newborns with preserved systolic function underwent conventional transmitral Doppler flow echocardiographic assessment. Left atrial ejection force index was further calculated for all patients. Data was compared with normal ranges of healthy newborns in order to clarify the diagnostic utility of these parameters for determining minor cardiac insults in this age group. FINDINGS: We found that mildly asphyxiated newborns showed an increase in the values of left atrial ejection force index (5.44±2.12 kilodyne vs. 6.66±2.17 kilodyne, P= 0.02) and left atrial filling fraction (39%±10% vs. 45%±8%, P= 0.01). Furthermore, the acceleration and deceleration rate of early filling flow peak velocity were decreased in this group of asphyxiated newborns. CONCLUSION: Assessment of left atrial ejection force in mildly asphyxiated newborns reveals that newborns with even mild asphyxia, although could not be categorized in conventional grading system, suffer to some extent from a ventricular filling abnormality. This type of latent ventricular filling abnormality could simply be unmasked by calculation of atrial ejection force index.