Our results indicate that different mutations underlie the development of LAD I. Definitive molecular diagnosis is valuable for genetic counseling and prenatal diagnosis. Regarding clinical presentations, it seems that omphalitis is the most consistent finding seen in LAD I infants.

Background Common variable immunodeficiency (CVID) is the most prevalent symptomatic primary immunodeficiency disorder characterized by infectious and noninfectious complications. Bronchiectasis continues to be a common respiratory problem and therapeutic challenge in CVID. The aim of this study is to estimate the overall prevalence of bronchiectasis and its associated phenotype in patients with CVID. Methods A systematic literature search was performed in Web of Science, PubMed, and Scopus from the earliest available date to February 2019 with standard keywords. All pooled analyses of bronchiectasis prevalence and the corresponding 95% confidence intervals (CIs) were based on random‐effects models. Results Fifty‐five studies comprising 8535 patients with CVID were included in the meta‐analysis. Overall prevalence of bronchiectasis was 34% (95% CI: 30‐38; I2 = 90.19%). CVID patients with bronchiectasis had significantly lower serum immunoglobulin A (IgA) and IgM levels at the time of diagnosis compared with those without bronchiectasis. Among the clinical features, the frequencies of splenomegaly, pneumonia, otitis media, and lymphocytic interstitial pneumonia were significantly higher in CVID patients with bronchiectasis compared with those without bronchiectasis, respectively. Conclusion A higher prevalence of bronchiectasis in patients with CVID should be managed by controlling recurrent and severe pneumonia episodes which are immune dysregulation since this complication is associated with poor prognosis in these patients.

Energetic materials containing aluminum powder are hazardous compounds, which have wide applications as propellants, explosives, and pyrotechnics. This work introduces a new method on the basis of the laser-induced breakdown spectroscopy technique in air and argon atmospheres to investigate determination of aluminum content and detonation performance of 1,3,5-trinitro-1,3,5-triazine (RDX)-based aluminized explosives. Plasma emission of aluminized RDX explosives are recorded where atomic lines of Al, C, H, N, and O, as well as molecular bands of AlO and CN are identified. The formation mechanism of AlO and CN molecular bands is affected by the aluminum percentage and oxygen content present in the composition and plasma. Relative intensity of the Al/O is used to determine detonation velocity and pressure of the RDX/Al samples. The released energy in the laser-induced plasma of aluminized RDX composition is related to the heat of explosion and percentage of aluminum.

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