Leucémie à plasmocytes : A propos de 5 observations cliniques: Plasma cell leukemia: report of 5 cases report

Plasma cell leukemia is a malignant plasma cell proliferation in bone marrow and peripheral blood. It is the most agressive form of plasma cell malignancies. It occurs in two forms: primary and secondary plasma cell leukemia. Plasma cell leukemia is defined by the presence of more than 2Giga/l peripheral blood plasma cells or plasmocytosis more than 20% of leukocyte count. In this context, we report 5 observations of plasma cell leukemia (1 case of primary plasma cell leukemia and 4 cases of secondary plasma cell leukemia) encountered during 9 years in Farhat Hached Hospital in Sousse-Tunisia. Through these observations, we describe clinical, paraclinical, prognostic and therapeutic characteristics of this pathology. La leucémie à plasmocytes est une prolifération maligne de cellules plasmocytaires dans la moelle osseuse et le sang périphérique. Elle représente la forme la plus agressive des néoplasies plasmocytaires. Elle se présente sous deux formes : une forme primitive et une forme secondaire compliquant un myélome multiple. Elle est définie par une plasmocytose sanguine supérieure à 2 Giga/l ou un taux de plasmocytes supérieur à 20% des leucocytes. Dans ce contexte, nous rapportons 5 observations de leucémie à plasmocytes (1 cas de leucémie à plasmocytes primitive et 4 cas de leucémie à plasmocytes secondaire) survenus en 9 ans à l’hôpital Farhat Hached de Sousse (Tunisie). A travers ces observations, nous décrivons les caractéristiques cliniques, paracliniques, pronostiques et thérapeutiques de cette pathologie. &nbsp

Mortality from gastrointestinal congenital anomalies at 264 hospitals in 74 low-income, middle-income, and high-income countries: a multicentre, international, prospective cohort study

Summary Background Congenital anomalies are the fifth leading cause of mortality in children younger than 5 years globally. Many gastrointestinal congenital anomalies are fatal without timely access to neonatal surgical care, but few studies have been done on these conditions in low-income and middle-income countries (LMICs). We compared outcomes of the seven most common gastrointestinal congenital anomalies in low-income, middle-income, and high-income countries globally, and identified factors associated with mortality. Methods We did a multicentre, international prospective cohort study of patients younger than 16 years, presenting to hospital for the first time with oesophageal atresia, congenital diaphragmatic hernia, intestinal atresia, gastroschisis, exomphalos, anorectal malformation, and Hirschsprung’s disease. Recruitment was of consecutive patients for a minimum of 1 month between October, 2018, and April, 2019. We collected data on patient demographics, clinical status, interventions, and outcomes using the REDCap platform. Patients were followed up for 30 days after primary intervention, or 30 days after admission if they did not receive an intervention. The primary outcome was all-cause, in-hospital mortality for all conditions combined and each condition individually, stratified by country income status. We did a complete case analysis. Findings We included 3849 patients with 3975 study conditions (560 with oesophageal atresia, 448 with congenital diaphragmatic hernia, 681 with intestinal atresia, 453 with gastroschisis, 325 with exomphalos, 991 with anorectal malformation, and 517 with Hirschsprung’s disease) from 264 hospitals (89 in high-income countries, 166 in middleincome countries, and nine in low-income countries) in 74 countries. Of the 3849 patients, 2231 (58·0%) were male. Median gestational age at birth was 38 weeks (IQR 36–39) and median bodyweight at presentation was 2·8 kg (2·3–3·3). Mortality among all patients was 37 (39·8%) of 93 in low-income countries, 583 (20·4%) of 2860 in middle-income countries, and 50 (5·6%) of 896 in high-income countries (p<0·0001 between all country income groups). Gastroschisis had the greatest difference in mortality between country income strata (nine [90·0%] of ten in lowincome countries, 97 [31·9%] of 304 in middle-income countries, and two [1·4%] of 139 in high-income countries; p≤0·0001 between all country income groups). Factors significantly associated with higher mortality for all patients combined included country income status (low-income vs high-income countries, risk ratio 2·78 [95% CI 1·88–4·11], p<0·0001; middle-income vs high-income countries, 2·11 [1·59–2·79], p<0·0001), sepsis at presentation (1·20 [1·04–1·40], p=0·016), higher American Society of Anesthesiologists (ASA) score at primary intervention (ASA 4–5 vs ASA 1–2, 1·82 [1·40–2·35], p<0·0001; ASA 3 vs ASA 1–2, 1·58, [1·30–1·92], p<0·0001]), surgical safety checklist not used (1·39 [1·02–1·90], p=0·035), and ventilation or parenteral nutrition unavailable when needed (ventilation 1·96, [1·41–2·71], p=0·0001; parenteral nutrition 1·35, [1·05–1·74], p=0·018). Administration of parenteral nutrition (0·61, [0·47–0·79], p=0·0002) and use of a peripherally inserted central catheter (0·65 [0·50–0·86], p=0·0024) or percutaneous central line (0·69 [0·48–1·00], p=0·049) were associated with lower mortality. Interpretation Unacceptable differences in mortality exist for gastrointestinal congenital anomalies between lowincome, middle-income, and high-income countries. Improving access to quality neonatal surgical care in LMICs will be vital to achieve Sustainable Development Goal 3.2 of ending preventable deaths in neonates and children younger than 5 years by 2030

A Rare Case of an Acquired Isolated Factor VII Deficiency was Discovered in a 23-Year-Old Female Patient

Introduction: Factor VII (FVII) deficiency, a rare bleeding disorder, can manifest as an autosomal recessive congenital or an acquired coagulopathy. Acquired FVII deficiency, although infrequently reported, presents unique challenges in understanding its mechanisms and identifying underlying causes.&nbsp;Case presentation: We present a case of acquired FVII deficiency discovered in a 23-year-old female patient with no apparent underlying disease. The patient exhibited spontaneous ecchymosis and gingival hemorrhage, along with low FVII activity and isolated prolongation of prothrombin time. Extensive laboratory investigations excluded liver dysfunction, familial deficiency, vitamin K deficiency, and inhibitory antibodies. Prompt treatment with Fresh Frozen Plasma (FFP) and bypassing agents resulted in a favorable response and resolution of hematomas.&nbsp;Conclusion: Acquired FVII deficiency was identified with bleeding symptoms in association with prolonged prothrombin time and a low level of FVII activity. In literature, this deficiency has been associated with various conditions such as sepsis, aplastic anemia, stem cell transplantation, and neoplasms, although approximately 14% of cases remain idiopathic. Clinical outcomes remain generally poor, with limited complete remissions reported

Allergic manifestations of inborn errors of immunity and their impact on the diagnosis: A worldwide study

International audienceBackground: Allergies have long been observed in Inborn Errors of Immunity (IEI) and might even be the first presentation resulting in delayed diagnosis or misdiagnosis in some cases. However, data on the prevalence of allergic diseases among IEI patients are limited and contradictory. Objective: To provide a worldwide view of allergic diseases, across a broad spectrum of IEI, and their impact on the timely diagnosis of IEI. Methods: This is a worldwide study, conceived by the World Allergy Organization (WAO) Inborn Errors of Immunity Committee. A questionnaire was developed and pilot-tested and was sent via email to collect data from 61 immunology centers known to treat pediatric and/or adult IEI patients in 41 countries. In addition, a query was submitted to The United States Immunodeficiency Network (USIDNET) at its website. Results: Thirty centers in 23 countries caring for a total of 8450 IEI patients responded. The USIDNET dataset included 2332 patients. Data from responders showed that a median (IQR) of 16.3% (10-28.8%) of patients experienced allergic diseases during the course of their IEI as follows: 3.6% (1.3-11.3%) had bronchial asthma, 3.6% (1.9-9.1%) atopic dermatitis, 3.0% (1.0-7.8%

Influence of COVID-19 on lifestyle behaviors in the Middle East and North Africa Region: a survey of 5896 individuals

Background: Coronavirus disease (COVID-19) pandemic has affected health and lifestyle behaviors of people globally. This project aims to identify the impact of COVID-19 on lifestyle behavior of individuals in the Middle East and North Africa (MENA) region during confinement. Methods: We conducted an online survey in 17 countries (Egypt, Jordan, United Arab Emirates, Kuwait, Bahrain, Saudi Arabia, Oman, Qatar, Yemen, Syria, Palestine, Algeria, Morocco, Libya, Tunisia, Iraq, and Sudan) from the MENA region on August and September 2020. The questionnaire included self-reported information on lifestyle behaviors, including physical activity, eating habits, smoking, watching television, social media use and sleep before and during the pandemic. Logistic regression was performed to analyze the impact of COVID-19 on lifestyle behaviors. Results: A total of 5896 participants were included in the final analysis and 62.8% were females. The BMI of the participants was 25.4 &plusmn; 5.8&nbsp;kg/m2. Around 38.4% of the participants stopped practicing any physical activities during the confinement (P &lt; 0.001), and 57.1% reported spending more than 2&nbsp;h on social media (P &lt; 0.001). There were no significant changes in smoking habits. Also, 30.9% reported an improvement in their eating habits compared with 24.8% reported worsening of their eating habits. Fast-food consumption decreased significantly in 48.8% of the study population. This direct/indirect exposure to COVID-19 was associated with an increased consumption of carbohydrates (OR = 1.09; 95% CI = 1.02&ndash;1.17; P = 0.01), egg (OR = 1.08; 95% CI = 1.02&ndash;1.16; P = 0.01), sugar (OR = 1.09; 95% CI = 1.02&ndash;1.16; P = 0.02), meat, and poultry (OR = 1.13; 95% CI = 1.06&ndash;1.20; P &lt; 0.01). There was also associated increase in hours spent on watching television (OR = 1.07; 95% CI = 1.02&ndash;1.12; P &lt; 0.01) and social media (OR = 1.09; 95% CI = 1.01&ndash;1.18; P = 0.03). However, our results showed a reduction in sleeping hours among those exposed to COVID-19 infection (OR = 0.85; 95% CI = 0.77&ndash;0.94; P &lt; 0.01). Conclusions: The COVID-19 pandemic was associated with an increase in food consumption and sedentary life. Being exposed to COVID-19 by direct infection or through an infected household is a significant predictor of amplifying these changes. Public health interventions are needed to address healthy lifestyle behaviors during and after the COVID-19 pandemic