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For people with haemophilia (PwH) who live in developing countries, haemophilia continues to be a condition with serious medical and social consequences. In Morocco, the efforts of patient associations and medical teams have led to the creation of a national programme for haemophilia care since the end of 2012, and the country is no longer solely reliant on World Federation of Hemophilia (WFH) donations for access to factor products. There is growing recognition of the impact of the pain experienced by PwH. To continue to improve treatment for PwH in Morocco, it is important to ensure that they are also able to manage haemophilia-related pain.

To evaluate the rooting ability and growth performance in semihardwood cuttings of Argania spinosa under nonmist greenhouse conditions, our experimentation was conducted with three cutting diameters (0.10.3, 0.30.6 and 0.60.9 cm), four leaf retention treatments (leafless, 2, 4 and 8 leaves) and three different rooting substrates (fine sand, peat moss, a 1:1 mixture of fine sand/peat moss). Significant effects of cuttings diameter, leaf retention and rooting substrate on sprouting, rooting and survival ability from A. spinosa semihardwood cuttings were observed. Among all diameters tested, a diameter of (0.30.6 cm) showed maximum rooting and survival capacity, while cuttings with a diameter of 0.60.9 cm resulted in the greatest sprouting ability. Successful vegetative propagation was restricted to leafy stem cuttings. Moreover, it was observed that the highest rooting ability was reached in cuttings planted in finesand substrate. However, the highest sprouting ratio (85.0%) and survival rate (92.5%) were achieved in a mixture of sand and peat moss. Thus, argan trees vegetative propagation could be most effectively achieved using semihardwood cuttings with a 0.4 cm diameter and 4 leaves, planted in a fine sand substrate during the root initiation period and grown in a mixture of fine sand and peat moss for hardening. [ABSTRACT FROM AUTHOR]

The AguelmamAzegza lake is located in the Middle Atlas mountain range, this region consists of carbonate rocks of Lias. It is the best-watered and richest region in Morocco in terms of wetlands, especially natural lakes, rivers and fresh springs. The lake’s water supply is ensured by the inflows of water in the form of underwater sources fed by precipitation (rain and snow) following tectonics and the genesis of karstic formations by the dissolution of carbonate rocks. The declining trend in lake levels in recent years results from the decrease in precipitation which decreases during these years.

Investigating habitat selection and ecological factors trade-offs is a major avian ecology topic which is closely implicated for conservation purposes. Studies dealing with the impacts of ecological factors on wintering Spoonbills are overall scarce. Here, we used Principal Component Analyses (PCA) and Generalized Linear Mixed Models (GLMM) to test the relevance of geographical, environmental and anthropogenic factors in predicting the occupancy and abundance of the Eurasian spoonbills (Platalea leucorodia) during 2008-2011 within 28 Moroccan wetlands. The Eurasian Spoonbill mean annual occurrence was 59.2% (± 1.5% SE; 95%CI: 56.3%-62.1%). Among the occupied wetlands, 15 (83.3%) were regularly occupied. We found that the Eurasian spoonbill occurrence was negatively related to distance to coastline, altitude and human presence, whereas its abundance increased significantly with increasing mudflat areas. These findings highlight the significant effect of human presence in selecting wintering sites, but not in the prediction of abundance. Management strategies should therefore address specific attributes of coastal wetlands and should carefully consider the effects of habitat use especially those related to mudflats. We further suggest guidelines for future studies to understand the dynamic of Eurasian spoonbills wintering in the region.

Tourism merits on natural spaces are not exhausted, namely economic dynamics, development of the local population’s life conditions, species conservation and awareness of environmental protection. However, the damage caused by Coronavirus to natural and rural spaces has impacted such merits negatively, for example, suspension of tourism and recreation activities. Further, the rebirth of ecotourism will be slower and necessitates profound changes in travel design and service delivery. Hence, sustainability, sanitary and regulatory measures will probably help manage ecotourism systems. Nevertheless, these will be affected by the increase in exploring relations between nature and wildlife regarding zoonotic and vector-borne infections and the fragility of biodiversity. Because of his mobility, a tourist appears to be a potential pathogen transmission factor of infections to natural areas, local communities, ecotourism destinations, his country and the crossed ones. New procedures would reduce travel and control traveler’s behavior, and interactions between travelers and wild animals. The slowdown in demand may thus hinder the development of ecotourism. Sensitivity to epidemiological risk and vector-borne diseases will be greater and the need for health-friendly tourism will be further strengthened.

COVID-19 pandemic has had huge impacts on multiple industries and sectors, not just ecotourism and wildlife protection in Morocco. Ecological health and wildlife are a critical resource for the country’s tourism sector recovery. Conservation is considered as one of the industries that are hit hard by the COVID-19 pandemic. Many of the threats facing biodiversity and protected areas have been exacerbated during and following, the outbreak with practices such as poaching, wildlife trafficking, and forest logging activities resumed due to the country lockdown and ecotourism collapse. Conservationists should take emergency actions in the short-term to help rural communities and grassroots organizations and review waysto achieve conservation and sustainability goals in the post-pandemic era.

The population of the African continent is significantly increasing which has given rise to societal, economic and environmental changes. This will generate an increase in demand for food, water and energy. Thus, strategies for sustainable management of natural resources need to be adopted urgently in order to match the future needs and to ensure sustainable development on the continent. The Water Energy Food (WEF) nexus is one the avenues for studying the current and future interactions and interdependencies of the WEF sectors under different scenarios and challenges in the effort towards establishing sustainable use of natural resources. Policies around the nexus approach are few or absent at the continental and countrylevels. This paper is a first review on the adoption of the Nexus in two model African countries, Morocco and South Africa. The review compares the water, energy and food sectors and the related national policies, plans and frameworks. The data related to the WEF Nexus revealed key difficulties of availability in both countries. A comparative analysis of the situation in both countries demonstrated differences in policies relating to the WEF Nexus across the sectors: water, energy, and agriculture. Additionally, the paper explores the relevance of technology-assisted frameworks for the WEF nexus and also analyzed risks to agriculture and food security in the context of the reviewed countries.

Le carcinome à cellules de Merkel (CCM) est une tumeur cutanée neuroendocrinerare d'évolution imprévisible et à grand potentiel métastatique. Ce néoplasme survient habituellement chez le sujet âgé au niveau des zones photo exposées. L'avidité constante du CCM au 18 fluorodésoxyglucose (FDG) justifie l'intérêt de la tomographie par émission de positon (TEP) au cours de cette pathologie. Toutefois, aucun consensus n'est établi à ce jour. Cette étude rapporte le cas d'une patiente de 25 ans suivie pour CCM métastatique, afin d'attirer l'attention sur cette tumeur particulière et d'illustrer l'intérêt de la TEP au 18 FDG dans la prise en charge de cette entité rare.

Muscle stem cells (MuSCs) enable muscle growth and regeneration after exercise or injury, but how metabolism controls their regenerative potential is poorly understood. We describe that primary metabolic changes can determine murine MuSC fate decisions. We found that glutamine anaplerosis into the tricarboxylic acid (TCA) cycle decreases during MuSC differentiation and coincides with decreased expression of the mitochondrial glutamate deaminase GLUD1. Deletion of Glud1 in proliferating MuSCs resulted in precocious differentiation and fusion, combined with loss of self-renewal in vitro and in vivo. Mechanistically, deleting Glud1 caused mitochondrial glutamate accumulation and inhibited the malate-aspartate shuttle (MAS). The resulting defect in transporting NADH-reducing equivalents into the mitochondria induced compartment-specific NAD + /NADH ratio shifts. MAS activity restoration or directly altering NAD + /NADH ratios normalized myogenesis. In conclusion, GLUD1 prevents deleterious mitochondrial glutamate accumulation and inactivation of the MAS in proliferating MuSCs. It thereby acts as a compartment-specific metabolic brake on MuSC differentiation., Competing Interests: Declaration of interests The authors declare no competing interests., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Introduction and Importance: Spontaneous coronary artery dissection is a rare cause of acute coronary syndromes not related to atherosclerosis. It involves the sudden tearing of the coronary artery wall, separating the inner intimal lining from the outer vessel wall, typically affecting a single coronary vessel. In 20% of cases, the cause of spontaneous coronary artery dissection is unknown. The other cases often occur in pregnant or postpartum women or in individuals with conditions such as connective tissue disorders or vasculitis., Case Presentation: Here, we describe a case of a 69-year-old African female presenting with non-ST-segment elevation myocardial infarction. Coronary angiography revealed an unusual triple-vessel spontaneous coronary artery dissection affecting peripheral segments, with further investigations suggesting polyarteritis nodosa., Conclusion: While triple-vessel spontaneous coronary artery dissection and polyarteritis nodosa (PAN) are individually rare, their coexistence is exceptionally uncommon and presents diagnostic and therapeutic challenges. Clinicians should be alert to vasculitic causes in patients with spontaneous coronary artery dissection, especially with atypical clinical features., (© 2024. The Author(s).)

Background: Acute lymphoblastic leukemia is the most common childhood cancer, with an 80% frequency in children between 1 and 10 years old. The outcome and prognosis of acute lymphoblastic leukemia in children depends on various factors, such as age, clinical and biological features, and cytogenetic factors., Case Presentation: We report the case of a pediatric patient, a 4-year-old Moroccan female who was referred to the Hematology and Oncology Department of 20 August 1953 Hospital in Casablanca and diagnosed with B-cell acute lymphoblastic leukemia associated with a rare genetic chromosomal abnormality., Conclusion: Translocation (1;4)(p21;p15) is a relatively rare chromosomal abnormality found in human leukemia and was never described isolated in pediatric B-cell acute lymphoblastic leukemia patients. It showed a good evolution by complete remission and recovery of this patient after receiving all chemotherapy and after 8 years of follow-up., (© 2023. The Author(s).)

Purpose: Peripheral neuroblastic tumors are the most common extracranial cancers found in children, and they are characterized by a diverse spectrum of clinical manifestations and heterogeneous behaviors. This study aimed to investigate the epidemiological and clinical characteristics of children with peripheral neuroblastic tumors admitted to the Department of Pediatric Hematology and Oncology of the Hospital August 20 in Casablanca., Methods: The medical files of 48 children with peripheral neuroblastic tumors addressed to our department between February 2018 and February 2023 were reviewed. The clinical and demographic characteristics of patients were analyzed by the Statistical Package for the Social Sciences (SPSS), survival curves were obtained by Kaplan-Meier technique, and we assigned the tumor stage to patients based on the International Neuroblastoma Risk Group Staging System (INRGSS)., Results: The median age of diagnosis was 30 months (1-174), with a ratio F/M of 1.28. 93.75% of patients had neuroblastoma, and the rest had ganglioneuroma. About 64.6% of patients had at their initial presentations stage M of peripheral neuroblastic tumors. The adrenal region made up 71% of the primary tumor site. The bone was one of the most prevalent metastatic sites (54.2%). The five-year overall survival rate was 35.4%., Conclusion: Overall, this study revealed a high stage of peripheral neuroblastic tumors in the majority of the diagnosed patients in our Department of Pediatric Hematology and Oncology. Moreover, the heterogeneity of peripheral neuroblastic tumors makes clinical recognition difficult and, in general, too late., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Metabolite genome-wide association studies (mGWAS) have advanced our understanding of the genetic control of metabolite levels. However, interpreting these associations remains challenging due to a lack of tools to annotate gene-metabolite pairs beyond the use of conservative statistical significance threshold. Here, we introduce the shortest reactional distance (SRD) metric, drawing from the comprehensive KEGG database, to enhance the biological interpretation of mGWAS results. We applied this approach to three independent mGWAS, including a case study on sickle cell disease patients. Our analysis reveals an enrichment of small SRD values in reported mGWAS pairs, with SRD values significantly correlating with mGWAS p values, even beyond the standard conservative thresholds. We demonstrate the utility of SRD annotation in identifying potential false negatives and inaccuracies within current metabolic pathway databases. Our findings highlight the SRD metric as an objective, quantitative and easy-to-compute annotation for gene-metabolite pairs, suitable to integrate statistical evidence to biological networks., Competing Interests: J.G.H. received speaker honoraria from Dalcor and District 3 Innovation Centre. C.D.R. received research support from Agilent Technologies and is a scientific consultant for Larimar Therapeutics. P.B. is consultant for ADDMEDICA, NOVARTIS, ROCHE, GBT, Bluebird, EMMAUS, HEMANEXT, AGIOS, VERTEX, received lecture fees for NOVARTIS, ADDMEDICA, AGIOS, JAZZPHARMA, VERTEX, is on steering committee for NOVARTIS and ADDMEDICA, receives research support from ADDMEDICA, foundation Fabre, NOVARTIS, Bluebird, EMMAUS, GBT, and is cofounder of INNOVHEM., (© 2023 The Authors.)

Pediatric patients with recurrent and refractory cancers are in most need for new treatments. This study developed patient-derived-xenograft (PDX) models within the European MAPPYACTS cancer precision medicine trial (NCT02613962). To date, 131 PDX models were established following heterotopical and/or orthotopical implantation in immunocompromised mice: 76 sarcomas, 25 other solid tumors, 12 central nervous system tumors, 15 acute leukemias, and 3 lymphomas. PDX establishment rate was 43%. Histology, whole exome and RNA sequencing revealed a high concordance with the primary patient's tumor profile, human leukocyte-antigen characteristics and specific metabolic pathway signatures. A detailed patient molecular characterization, including specific mutations prioritized in the clinical molecular tumor boards are provided. Ninety models were shared with the IMI2 ITCC Pediatric Preclinical Proof-of-concept Platform (IMI2 ITCC-P4) for further exploitation. This PDX biobank of unique recurrent childhood cancers provides an essential support for basic and translational research and treatments development in advanced pediatric malignancies., (© 2023. Springer Nature Limited.)

Providing sufficient energy for autonomous systems at the nanoscale is one of the major challenges of the Internet of Nano Things (IoNT). Existing battery technologies and conventional integrated circuits cannot be used in such small dimensions. Even if they are small enough to be used at the nano level, they still cannot be used in medical applications due to biocompatibility issues. M13 is a very promising virus with piezoelectric properties, which has attracted much interest in the scientific community as a bioenergy harvester. However, M13 studies presented so far in the literature are designed only for macroscale systems. In this paper, we simulate two designs of a bio-inspired nanogenerator based on the properties of M13 for nanosystems. We derive the stiffness matrix of M13, its dielectric and piezoelectric matrices and its density. We verify our calculated values by comparing our simulations with the results of experimental studies presented in the literature. We also evaluate the system's performance in terms of frequency response and loading characteristics. The results presented in this study show that a single M13 is a very promising nano-generator that can be used for medical applications.

Cancer cells reprogram their metabolism to support growth and invasion. While previous work has highlighted how single altered reactions and pathways can drive tumorigenesis, it remains unclear how individual changes propagate at the network level and eventually determine global metabolic activity. To characterize the metabolic lifestyle of cancer cells across pathways and genotypes, we profiled the intracellular metabolome of 180 pan-cancer cell lines grown in identical conditions. For each cell line, we estimated activity for 49 pathways spanning the entirety of the metabolic network. Upon clustering, we discovered a convergence into only two major metabolic types. These were functionally confirmed by 13 C-flux analysis, lipidomics, and analysis of sensitivity to perturbations. They revealed that the major differences in cancers are associated with lipid, TCA cycle, and carbohydrate metabolism. Thorough integration of these types with multiomics highlighted little association with genetic alterations but a strong association with markers of epithelial-mesenchymal transition. Our analysis indicates that in absence of variations imposed by the microenvironment, cancer cells adopt distinct metabolic programs which serve as vulnerabilities for therapy., (© 2022 The Authors. Published under the terms of the CC BY 4.0 license.)

Breeding hybrids with maximum heterosis requires efficient cross-pollination and an improved male sterility system. Renewed efforts have been made to dissect the phenotypic variation and genetic basis of hybrid floral traits, although the potential of tailoring the appropriate flower design on seed setting is less known. To this end, elite wheat genotypes were crossed using a chemical hybridizing agent at different doses. A total of 23 hybrids were developed from a partial diallel design; and planted in an alpha lattice design with their parents at two locations in Morocco, for two years, to evaluate for yield components, heterosis and combining abilities. The 13.5 L ha -1 dose induced a maximum level of sterility (95%) and seed set showed large phenotypic variation and high heritability. In parallel, seed set showed tight correlation with pollen mass (0.97), visual anther extrusion (0.94) and pollen shedding (0.91) ( p < 0.001), allowing direct selection of the associated traits. Using the combined data, mid-parent heterosis ranges were -7.64-14.55% for biomass (BM), -8.34-12.51% for thousand kernel weight (TKW) and -5.29-26.65% for grain yield (YLD); while best-parent heterosis showed ranges of -11.18-7.20%, -11.35-11.26% and -8.27-24.04% for BM, TKW and YLD, respectively. The magnitude of general combining ability (GCA) variance was greater than the specific combining ability (SCA) variance suggesting a greater additive gene action for BM, TKW and YLD. The favorable GCA estimates showed a simple method to predict additive effects contributing to high heterosis and thus could be an effective approach for the selection of promising parents in early generations.

Inflammatory bowel disease (IBD) is a lifelong inflammatory disorder with relapsing-remission cycles, which is currently diagnosed by clinical symptoms and signs, along with laboratory and imaging findings. However, such clinical findings are not parallel to the disease activity of IBD and are difficult to use in treatment monitoring. Therefore, non-invasive quantitative imaging tools are required for the multiple follow-up exams of IBD patients in order to monitor the disease activity and determine treatment regimens. In this study, we evaluated a dual P- and E-selectin-targeted microbubble (MB Selectin ) in an interleukin-2 receptor α deficient (IL-2Rα -/- ) spontaneous chronic IBD mouse model for assessing long-term anti-inflammatory effects with ultrasound molecular imaging (USMI). We used IL-2Rα -/- (male and female on a C57BL/6 genetic background; n = 39) and C57BL/6 wild-type (negative control; n = 6) mice for the study. USMI of the proximal, middle, and distal colon was performed with MB Selectin using a small animal scanner (Vevo 2100) up to six times in each IL-2Rα -/- mouse between 6-30 weeks of age. USMI signals were compared between IL-2Rα -/- vs. wild-type mice, and sexes in three colonic locations. Imaged colon segments were analyzed ex vivo for inflammatory changes on H&E-stained sections and for selectin expression by immunofluorescence staining. We successfully detected spontaneous chronic colitis in IL-2Rα -/- mice between 6-30 weeks (onset at 6-14 weeks) compared to wild-type mice. Both male and female IL-2Rα -/- mice were equally ( p = 0.996) affected with the disease, and there was no significant ( p > 0.05) difference in USMI signals of colitis between the proximal, middle, and distal colon. We observed the fluctuating USMI signals in IL-2Rα -/- mice between 6-30 weeks, which might suggest a resemblance of the remission-flare pattern of human IBD. The ex vivo H&E and immunostaining further confirmed the inflammatory changes, and the high expression of P- and E-selectin in the colon. The results of this study highlight the IL-2Rα -/- mice as a chronic colitis model and are suitable for the long-term assessment of treatment response using a dual P- and E-selectin-targeted USMI.

Hybrid wheat breeding is one of the most promising technologies for further sustainable yield increases. However, the cleistogamous nature of wheat displays a major bottleneck for a successful hybrid breeding program. Thus, an optimized breeding strategy by developing appropriate parental lines with favorable floral trait combinations is the best way to enhance the outcrossing ability. This study, therefore, aimed to dissect the genetic basis of various floral traits using genome-wide association study (GWAS) and to assess the potential of genome-wide prediction (GP) for anther extrusion (AE), visual anther extrusion (VAE), pollen mass (PM), pollen shedding (PSH), pollen viability (PV), anther length (AL), openness of the flower (OPF), duration of floret opening (DFO) and stigma length. To this end, we employed 196 ICARDA spring bread wheat lines evaluated for three years and genotyped with 10,477 polymorphic SNP. In total, 70 significant markers were identified associated to the various assessed traits at FDR ≤ 0.05 contributing a minor to large proportion of the phenotypic variance (8-26.9%), affecting the traits either positively or negatively. GWAS revealed multi-marker-based associations among AE, VAE, PM, OPF and DFO, most likely linked markers, suggesting a potential genomic region controlling the genetic association of these complex traits. Of these markers, Kukri&#95;rep&#95;c103359&#95;233 and wsnp&#95;Ex&#95;rep&#95;c107911&#95;91350930 deserve particular attention. The consistently significant markers with large effect could be useful for marker-assisted selection. Genomic selection revealed medium to high prediction accuracy ranging between 52% and 92% for the assessed traits with the least and maximum value observed for stigma length and visual anther extrusion, respectively. This indicates the feasibility to implement genomic selection to predict the performance of hybrid floral traits with high reliability.

Forward genetic screens with genome-wide CRISPR libraries are powerful tools for resolving cellular circuits and signaling pathways. Applying this technology to organoids, however, has been hampered by technical limitations. Here we report improved accuracy and robustness for pooled-library CRISPR screens by capturing sgRNA integrations in single organoids, substantially reducing required cell numbers for genome-scale screening. We applied our approach to wild-type and APC mutant human intestinal organoids to identify genes involved in resistance to TGF-β-mediated growth restriction, a key process during colorectal cancer progression, and validated hits including multiple subunits of the tumor-suppressive SWI/SNF chromatin remodeling complex. Mutations within these genes require concurrent inactivation of APC to promote TGF-β resistance and attenuate TGF-β target gene transcription. Our approach can be applied to a variety of assays and organoid types to facilitate biological discovery in primary 3D tissue models., Competing Interests: Declaration of Interests H.C. is an inventor on applications/patents related to organoid technology. The other authors declare no competing interests., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objective : Acute mouse models of inflammatory bowel disease (IBD) fail to mirror the chronic nature of IBD in patients. We sought to develop a chronic mouse IBD model for assessing long-term anti-inflammatory effects with ultrasound molecular imaging (USMI) by using dual P- and E-selectin targeted microbubbles (MB Selectin ). Materials and Methods : Interleukin 10 deficient (IL-10 -/- on a C57BL/6 genetic background; n=55) and FVB (n=16) mice were used. In IL-10 -/- mice, various experimental regimens including piroxicam, 2,4,6-trinitrobenzenesulfonic acid (TNBS) or dextran sulfate sodium (DSS), respectively were used for promoting colitis; colitis was induced with DSS in FVB mice. Using clinical and small animal ultrasound scanners, evolution of inflammation in proximal, middle and distal colon, was monitored with USMI by using MB Selectin at multiple time points. Imaged colon segments were analyzed ex vivo for inflammatory changes on H&E staining and for P-selectin expression on immunofluorescence staining. Results : Sustained colitis was not detected with USMI in IL-10 -/- or FVB mice with various experimental regimens. USMI signals either gradually decreased after the colitis enhancing/inducing drug/agents were discontinued, or the mortality rate of mice was high. Inflammation was observed on H&E staining in IL-10 -/- mice with piroxicam promotion, while stable overexpression of P-selectin was not found on immunofluorescence staining in the same mice. Conclusion : Sustained colitis in IL-10 -/- mice induced with piroxicam, TNBS or DSS, and in FVB mice induced with DSS, was not detected with USMI using MB Selectin , and this was verified by immunofluorescence staining for inflammation marker P-selectin. Thus, these models may not be appropriate for long-term monitoring of chronic colitis and subsequent treatment response with dual-selectin targeted USMI., Competing Interests: Competing Interests: The authors have declared that no competing interest exists.

Aim: The goals of this paper are: to report the incidence of AML in elderly, to describe cytogenetic characteristics of this population, to observe rare and novel cytogenetic abnormalities and lastly, to compare our finding with that previously reported in the literature., Methods: We conducted a retrospective analysis of 283 patients with acute myeloid leukaemia (AML) treated in our unit, we will report the incidence of AML in elderly, describe cytogenetic characteristics of this population, observe rare and novel cytogenetic abnormalities and compare our finding with that previously reported in the literature., Results: Among the 283 patients, 157 (54.4%) patients performed the karyotype, the cytogenetic analysis failed in 17 cases (11%). Prognostic group distribution was found to be favorable in 8 patients (6%) with 6 cases of t (8; 21) and 2 cases of inv (16), intermediate group in 94 patients (67%), including 58 cases (41,5%) with a normal karyotype, and an unfavorable group in 38 patients (27%) including complex karyotype (15%), -5 or del 5q (3%), -7 or del 7q (3.5%), t (9; 22) (2%). Some rare anomalies were observed., Conclusion: However, the occurrence of a complex karyotype was more frequent than younger patients. In our unit, elderly benefit from supportive care, our study shows that it is a heterogeneous group and our treatment approach have to change especially for the patient from favourable risk group who can benefit from intensive chemotherapy. We have to improve our diagnosis with including molecular genetics that provides a documented substrate for a thoughtfully considered treatment plan.

Background: Previous studies have reported that up to 75 % of milk allergic subjects tolerate heated milk products. However, the food used for heated milk challenge is often prepared in a non-standardized manner by the parents at home, which may prove inconvenient and even sometimes raise concerns with regards to test validity. Instant skim milk powder is made by a food process that involves heating skim milk to up to 250 °C (390 °F) for up to 30 min which ought to be sufficient to denature thermo-labile proteins., Objective: To appraise the use of instant skim milk for the purpose of heated milk food challenge., Methods: We reviewed all oral food challenges to instant skim milk powder performed at Sainte-Justine University Hospital Center in Montreal, Canada between November 2008 and January 2013 (cumulative dose of 4 g proteins)., Results: During the study period, 39 children underwent an open food challenge to instant skim milk powder. Thirty patients (76.9 %) passed the challenge without clinical reaction, of which 26 successfully introduced heated milk products at home. The remaining four children reported intermittent mild reactions to specific forms of heated milk goods while they tolerated others. Subjects' clinical and paraclinical characteristics were comparable to previous cohorts evaluating baked milk challenge, which reported similar rates of heated milk positive challenges, ranging from 17 to 28 %., Conclusion: Challenge with instant skim milk powder could be a safe, convenient and easily standardizable alternative to home baked food for heated milk challenge. Further controlled studies are needed before this can be implemented to practice.

Sickle cell anemia results from a single amino acid substitution in the gene encoding the β-globin subunit. Polymerization of deoxygenated sickle hemoglobin leads to decreased deformability of red blood cells. Hashimoto's thyroiditis is a common thyroid disease now recognized as an auto-immune thyroid disorder, it is usually thought to be haemolytic autoimmune anemia. We report the case of a 32 years old women admitted for chest pain and haemolysis anemia in which Hashimoto's thyroiditis and sickle cell anemia were found. In our observation the patient is a young woman whose examination did not show signs of goitre but the analysis of thyroid function tests performed before an auto-immune hemolytic anemia (confirmed by a high level of unconjugated bilirubin and a Coombs test positive for IgG) has found thyroid stimulating hormone (TSH) and positive thyroid antibody at rates in excess of 4.5 times their normal value. In the same period, as the hemolytic anemia, and before the atypical chest pain and anguish they generated in the patient, the search for hemoglobinopathies was made despite the absence of a family history of haematological disease or painful attacks in childhood. Patient electrophoresis's led to research similar cases in the family. The mother was the first to be analyzed with ultimately diagnosed with sickle cell trait have previously been ignored. This case would be a form with few symptoms because the patient does not describe painful crises in childhood or adolescence.

Background: We previously estimated that the annual rate of accidental exposure to peanut in 1411 children with peanut allergy, followed for 2227 patient-years, was 11.9% (95% CI, 10.6, 13.5). This cohort has increased to 1941 children, contributing 4589 patient-years, and we determined the annual incidence of accidental exposure, described the severity, management, location, and identified associated factors., Findings: Children with physician-confirmed peanut allergy were recruited from Canadian allergy clinics and allergy advocacy organizations from 2004 to May 2014. Parents completed questionnaires regarding accidental exposure to peanut over the preceding year. Five hundred and sixty-seven accidental exposures occurred in 429 children over 4589 patient-years, yielding an annual incidence rate of 12.4% (95% CI, 11.4, 13.4). Of 377 accidental exposures that were moderate or severe, only 109 (28.9%) sought medical attention and of these 109, only 40 (36.7%) received epinephrine. Of the 181 moderate/severe accidental exposures treated outside a health care facility, only 11.6% received epinephrine. Thirty-seven percent of accidental exposures occurred at home. In multivariate analyses, longer disease duration, recruitment through an allergy advocacy association, and having other food allergies decreased the likelihood of accidental exposures. Age ≥ 13 years at study entry and living with a single parent increased the risk., Conclusion: Despite increased awareness, accidental exposures continue to occur, mainly at home, and most are managed inappropriately by both health care professionals and caregivers. Consequently, more education is required on the importance of strict allergen avoidance and the need for prompt and correct management of anaphylaxis.