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This study is the beginning of the research that focuses on unconventional ionic liquids (ILs) hydrodistillation (HD) extraction of the essential oil of Heliotropium europaeum L. using ILs as additives. Two ILs based on 1- butyl-3-methylimidazolium were used by switching the anions (Cl- and PF6 -). The effect of mass percent of the added ILs on its yield and composition was evaluated. Compared to the conventional HD, ILs-HD gives a higher yield of essential oils (0.10–0.36 %). Particularly, with [C4mim][PF6], the observation of morphological changes using scanning electron microscopy (SEM) confirmed the effectiveness of the ionic liquid in this distillation process. The GC– –MS analysis of essential oils (EOs) revealed the presence of sixty-six compounds in HD, ILs-HD methods. Gas chromatography–mass spectrometry analysis of the EOs revealed the predominance of eugenol (1.70–72.35 %), butylated hydroxytoluene (8.95–65.39 %) and phytol (18.20 %). The new distillation methods of H. europaeum with ILs identifies more compounds (50 compounds in ILs-HD [C4mim][PF6]; 22 compounds in ILs-HD ([C4mim][Cl]) than conventional hydrodistillation (25 compounds in HD). Therefore, the ILs- -based hydrodistillation approach is superior in improving the production of EOs. It is important to emphasize that the data presented in this study are not yet available for any of this Algerian Heliotropium species of genus and present the great potential of this medicinal plant as a source of novel bioactive extracts with possible therapeutic uses.

Introduction: Inborn errors of immunity (IEI) represent a heterogeneous large group of genetic disorders characterized by susceptibility of affected individuals to recurrent infections, autoimmune/inflammatory diseases, allergy, and malignancy. We aimed to report for the first time the Algerian registry for IEI in children., Methods: We described the characteristics of IEI in Algerian children from the data collected in the Algerian registry for IEI between 1985 and 2021., Results: Over a period of 37 years, we included 887 children (530 male, 59.6%) with a mean age at diagnosis of 3.23 years and a mean diagnosis delay of 2 years. The prevalence rate was estimated at 1.97/100,000 inhabitants or 5.91/100,000 children. The parental consanguinity was found in 52.6%. The most prevalent category was combined immunodeficiencies (CID) (35.5%), followed by predominantly antibody deficiencies (24.5%) and CID with syndromic features (18.3%). The most predominant diseases were severe CID (134 cases), MHC II deficiency (99 cases), agammaglobulinemia (82 cases), common variable immunodeficiency (78 cases), hyper IgE syndromes (61 patients), ataxia-telangiectasia (46 patients), Wiskott-Aldrich syndrome (40 patients) and chronic granulomatous disease (39 cases). The clinical presentation was dominated by lower respiratory tract infections (69%), failure to thrive (38.3%), and chronic diarrhea (35.2%). Genetic analysis was performed in 156 patients (17.6%). The global mortality rate was 28.4% mainly caused by CID., Conclusion: This is the first report of the Algerian registry for IEI in children. Data is globally similar to that of the Middle East and North African (MENA) registries with high consanguinity, predominance of CID, and significant mortality. This registry highlights the weak points that should be improved in order to provide better patient care., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: It has been demonstrated that alkylating agents such as cyclophosphamide (CYP) are effective in reducing the risk of relapse in frequently relapsing (FRNS) and steroid-dependent nephrotic syndrome (SDNS). Little is known about prognostic factors in SDNS and FRNS treated by CYP. The objectives of this study are to determine long-term outcomes and factors associated with sustained remission in these patients., Methods: We retrospectively studied the data from 143 children (104 boys) with SDNS and FRNS treated with CYP in six centres over 15 years. Relapse-free survival was estimated by Kaplan-Meier method. The determinants of long-term remission were assessed by univariate and multivariate analyses using Cox proportional hazard models., Results: Median age at diagnosis was 3.7 years (interquartile range: IQR 2.3-5.9), and median follow-up was 7.8 years (IQR 4.0-11.8). CYP treatment was introduced after a median time of 1.7 years (IQR 0.7-5.9) after diagnosis. Patients received a median cumulative dose of 168 mg/kg (IQR 157-197) body weight. Relapse-free survival was 65%, 44%, 27% and 13% after 6 months, 1 year, 2 years and 5 years, respectively. In multivariate analysis, sustained remission >2 years was associated with age at treatment >5 years (P = 0.02) and cumulative dose of CYP >170 mg/kg (P = 0.02). Frequently relapsing versus steroid-dependent status and female gender were predictors of borderline significance. Height and body mass index standard deviation score were significantly influenced by CYP treatment., Conclusion: In our study, long-term efficacy of cyclophosphamide in steroid-responsive nephrotic syndrome is disappointing. Further well-designed trials are required to evaluate the efficacy of other steroid-sparing agents.