Clinical and immunological characteristics of 69 leukocyte adhesion deficiency-I patients.

BACKGROUND: In order to support the comprehensive classification of Leukocyte Adhesion Deficiency-I (LAD-I) severity by simultaneous screening of CD11a/CD18, this study assessed clinical, laboratory, and genetic findings along with outcomes of 69 LAD-I patients during the last 15 years. METHODS: Sixty-nine patients (40 females and 29 males) with a clinical phenotype suspected of LAD-I were referred to Immunology, Asthma, and Allergy research institute, Tehran, Iran between 2007 and 2022 for further advanced immunological screening and genetic evaluations as well as treatment, were enrolled in this study. RESULTS: The diagnosis median age of the patients was 6 months. Delayed umbilical cord separation was found in 25 patients (36.2%). The median diagnostic delay time was 4 months (min-max: 0-82 months). Forty-six patients (66.7%) were categorized as severe (CD18 and/or CD11a: below 2%); while 23 children (33.3%) were in moderate category (CD18 and/or CD11a: 2%-30%). During the follow-ups, 55.1% of children were alive with a mortality rate of 44.9%. Skin ulcers (75.4%), omphalitis (65.2%), and gingivitis (37.7%) were the most frequent complaints. Genetic analysis of the patients revealed 14 previously reported and three novel pathogenic mutations in the ITGB2 gene. The overall survival of patients with and without hematopoietic stem cell transplantation was 79.3% and 55.6%, respectively. CONCLUSION: Physicians' awareness of LAD-I considering delayed separation of umbilical cord marked neutrophilic leukocytosis, and variability in CD11 and CD18 expression levels, and genetic analysis leads to early diagnosis and defining disease severity. Moreover, the prenatal diagnosis would benefit families with a history of LAD-I.

Interpretation of Hematological, Biochemical, and Immunological Findings of COVID-19 Disease: Biomarkers Associated with Severity and Mortality.

The severe acute respiratory syndrome-coronavirus 2 (SARS-CoV-2) spread rapidly all over the world in late 2019 and caused critical illness and death in some infected patients. This study aimed at examining several laboratory factors, especially inflammatory and immunological mediators, to identify severity and mortality associated biomarkers. Ninety-three hospitalized patients with confirmed coronavirus disease 2019 (COVID-19) were classified based on disease severity. The levels of biochemical, hematological, immunological, and inflammatory mediators were assessed, and their association with severity and mortality were evaluated. Hospitalized patients were mostly men (77.4%) with an average (standard deviation) age of 59.14 (14.81) years. The mortality rate was significantly higher in critical patients (85.7%). Increased serum levels of blood sugar, urea, creatinine, uric acid, phosphorus, total bilirubin, serum glutamic-oxaloacetic transaminase, serum glutamic-oxaloacetic transaminase, lactic dehydrogenase, C-reactive protein, ferritin, and procalcitonin were significantly prevalent (p=0.002, p<0.001, p<0.001, p=0.014, p=0.047, p=0.003, p<0.001, p<0.001, p<0.001, p<0.001, P<0.001, and p<0.001, respectively) in COVID-19 patients. Decreased red blood cell, hemoglobin, and hematocrit were significantly prevalent among COVID-19 patients than healthy control subjects (p<0.001 for all). Troponin-I, interleukin-6, neutrophil/lymphocyte ratio (NLR), procalcitonin, and D-dimer showed a significant association with the mortality of patients with specificity and sensitivity more than 60%. Age, sex, underlying diseases, blood oxygen pressure, complete blood count along with C-reactive protein, lactic dehydrogenase, procalcitonin, D-dimer, and interleukin-6 evaluation help to predict the severity and required management for COVID-19 patients. Further investigations are highly recommended in a larger cohort study for validation of the present findings.

The most common allergens according to skin prick test: The role of wheal diameter in clinical relevancy.

The skin prick test (SPT) could be applied as a useful in vivo method for the detection of sensitization in epidemiological and diagnostic studies if the wheal size is ideally evaluated. We focused on SPT wheal size to identify sensitization pattern to common inhalant and food allergens. In this cross-sectional study, SPT results were obtained from a total of 972 allergic patients. Common allergen extracts for SPT were selected according to the type of allergic diseases, and the geographical pattern. SPT with food allergens was performed for patients with atopic dermatitis (AD) and chronic urticaria (CU). A total of 461 male (47.4%) and 511 female (52.6%) participated in this study (median age: 31 years). The majority of individuals were affected with allergic rhinitis (AR) (n = 624) and asthma (n = 224); while 129 and 67 patients suffered from AD and CU, respectively. The most common aeroallergens were Russian thistle (52.1%) and lamb's quarter (50.7%) with the largest wheal diameter. The wheal size of lamb's quarter was significantly different between patients with asthma and AR (P<.001). In addition, a significant difference was detected in wheal diameter in response to the Russian thistle between patients with AR and AD (P = .001). Shrimp (23.6%) and Peanut (22.5%) caused the most common food sensitization in patients with AD and CU. Having in mind the most common weed pollens including the Russian thistle and lamb's quarter, preventive strategies, such as, removing unwanted weeds or preventing them from growing, avoidance, and specific immunotherapy may be crucial for better disease control.

Paediatric asthma prevalence: The first national population-based survey in Iran.

BACKGROUND: The actual prevalence of paediatric asthma as a worldwide chronic disease has been surveyed in developed countries. However, no sufficient survey has been conducted in most of the eastern developing countries. Herein, we took measures to evaluate the prevalence of paediatric asthma in Iran. METHODS: In this national cross-sectional study, the prevalence of asthma symptoms was estimated throughout the country using a randomized multistage stratified cluster sampling method in 16 410 and 16 850 individuals aged 6-7 and 13-14 years, respectively. A validated questionnaire including core questions of the International Study of Asthma and Allergies in Childhood (ISAAC) was applied between November 2015 and February 2016. RESULTS: The total prevalence of asthma was 10.9% (n = 3624) (95% confidence interval [CI]: 10.6%-11.2%) which was significantly higher among 13- to 14-year olds compared to a younger age group (12.4% vs. 9.4%, P < 0.001), males versus females (12.1% vs. 9.8%, P < 0.001) and residents of urban compared to rural areas (P = 0.003). The prevalence of severe asthma was 3.9%, being significantly more prevalent in higher age groups and male individuals (P < 0.001). A significant relationship was found between asthma and passive smoking in both 6- to 7- and 13- to 14-year olds (P < 0.001). CONCLUSIONS: The prevalence of asthma and severe asthma in the paediatric population of Iran was similar to other developing countries. It is recommended to pay special attention to urban regions, male individuals and higher age groups for better controlling of asthma. Nevertheless, further national surveys are necessary to determine the trend of paediatric asthma in Iran.

The prevalence of asthma in Iranian adults: The first national survey and the most recent updates.

BACKGROUND: The worldwide increase in the prevalence of asthma has made it a major public-health concern. We aimed to identify the prevalence of asthma and asthma symptoms in adults living in urban and rural areas of Iran as a populated country with about 80 millions of residents. METHODS: This cross-sectional study was conducted to estimate the prevalence of asthma in adults between 20 and 44 years old in all provinces of Iran. Data were collected by personal interview via a standardized questionnaire [European Community Respiratory Health Survey (ECRHS)] between November 2015 and February 2016. RESULTS: A total of 24 344 individuals were enrolled. The prevalence of asthma was 8.9% [95% confidence interval (CI): 8.5-9.3]. The most common asthma symptoms were wheezing (14.2%, n = 3465), nocturnal cough (13.3%, n = 3234) and chest tightness (11.3%, n = 2760). Additionally, the prevalence of current asthma (taking asthma medications or asthma attack) was estimated to be 4.7% (n = 1155). Asthma was significantly more prevalent in males compared to females (P = .002), while no significant relationship was detected between gender and asthma after adjusted analysis with other variables. The prevalence of asthma was significantly higher in older participants (P < .001) and individuals with low educational level (P < .001). Interestingly, there was no significant relationship between asthma and area of residency (P = .8). CONCLUSIONS: The prevalence of asthma in Iran was similar to other Asian and European countries. However, repeated national surveys are required to determine the trend of asthma prevalence in Iran in comparison to other countries.