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Le syndrome de DiGeorge un déficit immunitaire congénital (par absence ou sous-développement du thymus) 3560

Le syndrome de DiGeorge est un déficit immunitaire congénital dans lequel le thymus est absent ou sous-développé à la naissance, ce qui entraîne des problèmes avec les lymphocytes T, les globules blancs qui aident à identifier et détruire les cellules étrangères ou anormales. D’autres malformations congénitales cardiaques sont également présentes. Les enfants atteints du syndrome de DiGeorge naissent avec plusieurs anomalies, y compris des anomalies cardiaques, des glandes parathyroïdes peu développées ou absentes, un thymus peu développé ou absent et des traits faciaux caractéristiques. Les problèmes liés aux lymphocytes T provoquent des infections récurrentes. Ce trouble est appelé aussi syndrome de microdélétion 22q11 .2 . 1 /Quelles sont les causes de cette maladie La maladie résulte d'une perte (délétion) d'un petit fragment sur le chromosome 22 qui porte des gènes intervenant dans le développement du cœur, du cerveau, du thymus, et de la glande parathyroïde qui régule le taux de calcium dans le sang. Les conséquences de cette anomalie génétique sont très variables. Certaines personnes n'ont quasiment aucun symptôme et le diagnostic est parfois porté à posteriori , suite à la survenue d'un autre cas plus typique dans la famille. 2/ Quels sont les symptômes ? Il existe des déformations au niveau du visage avec une fente palatine à l’origine d’une occlusion incomplète de la cavité buccale lors de l'émission vocale. Les yeux sont généralement très écartés et la mâchoire est petite et en retrait. Sont observés un retard dans l’acquisition de la marche, de la parole et du langage avec une lenteur ou une maladresse dans l'exécution des gestes. Une susceptibilité particulière aux infections est fréquente avec notamment des otites à répétition et des infections pulmonaires. Une malformation cardiaque est présente dans 75 % des cas affectant essentiellement l’aorte. Un abaissement de la concentration de calcium dans le sang peut provoquer des crises de tétanie. 3/ Y a-t-il un traitement À l'heure actuelle, il n’est pas encore possible de remplacer le fragment chromosomique manquant. La prise en charge des troubles du langage repose essentiellement sur l'orthophonie. Une opération chirurgicale est parfois nécessaire. Un traitement antibiotique prolongé est parfois prescrit à visée préventive pour les enfants qui présentent des infections répétées. Une greffe de tissu thymique ou de cellules souches est effectuée en cas d’absence du thymus. DR MOUSSAYER KHADIJA, spécialiste en médecine interne et en gériatrienالدكتورة خديجة موسيار Présidente de l’Alliance Maladies Rares Maroc (AMRM) et de l’association marocaine des maladies auto-immunes et systémiques (AMMAIS), Vice-présidente du Groupe de l’Auto-Immunité Marocain (GEAIM) POUR EN SAVOIR PLUS : SYMPTÖMES CARACTERISTIQUES (SOURCE MSD MANUEL), SYMPTÖMES CARACTERISTIQUES (SOURCE MSD MANUEL) Le fœtus ne se développe pas normalement et des anomalies affectent souvent les organes suivants : Cœur : Les enfants naissent souvent présentant un trouble cardiaque congénital Glande parathyroïde : L’enfant naît généralement avec des glandes parathyroïdes (qui contribuent à réguler les taux de calcium dans le sang) insuffisamment développées ou inexistantes. Visage : L’enfant présente en général des traits faciaux caractéristiques, tels que des oreilles basses, une petite mâchoire en retrait ou des yeux très écartés. Il peut présenter une fente dans le palais (fente palatine). Thymus : Le thymus est nécessaire pour le développement normal des lymphocytes T. Comme cette glande est absente ou insuffisamment développée, le nombre de lymphocytes T est faible, ce qui limite leur capacité de combattre de nombreuses infections.Le pronostic est en général lié à la gravité de l’atteinte cardiaque. DIAGNOSTIC DU SYNDROME Analyses de sang Parfois, examens d’imagerie (tels que radiographies du thorax et échocardiographie) Les médecins suspectent un syndrome de DiGeorge en se basant sur les symptômes et des analyses de sang. TRAITEMENT Il se fonde sur : Suppléments calciques et de vitamine D Parfois une greffe de tissu thymique ou de cellules souches Chez les enfants qui possèdent tout de même quelques lymphocytes T, le système immunitaire peut fonctionner correctement, sans traitement. Les infections qui se développent doivent être rapidement traitées.. Chez les enfants qui n’ont pas de lymphocytes T, ce trouble est mortel à moins de recourir à une greffe de tissu thymiqu BIBLIOGRAPHIE -DiGeorge or 22q11.2 deletion syndrome, https://primaryimmune.org/understanding-primary-immunodeficiency/types-of-pi/digeorge-or-22q112-deletion-syndrome -James Fernandez , MD, PhD, Cleveland Clinic Lerner College of Medicine at Case Western Reserve University, Traitement du syndrome de Digeorge, Le Manuel MSD, Vérifié/Révisé janv. 2023 https://www.msdmanuals.com/fr/accueil/troubles-immunitaires/d%C3%A9ficits-immunitaires/syndrome-de-digeorge#:~:text=Les%20enfants%20atteints%20du%20syndrome,T%20provoquent%20des%20infections%20r%C3%A9currentes L'Alliance des Maladies Rares aumar L’Alliance a pour missions de faire connaître et reconnaître les maladies rares auprès du public, des professionnels de santé et des pouvoirs publics en informant sur leurs enjeux scientifiques, sanitaires et sociaux, par tous les moyens : presse écrite, télévision, radio, sites internet et réseaux sociaux. Elle a également pour objectifs de contribuer à l’information des médecins en organisant des colloques thématiques sur les maladies rares. Elle œuvre à aider à la création d’associations de malades dédiées à chaque maladie rare et à améliorer l’accès au diagnostic et aux soins des maladies rares par la mise en place de centre de référence et de compétence et par une prise en charge réellement efficaces en inscrivant ces troubles comme des affections de longues durées (ALD) L’Alliance des Maladies Rares (AMRM) a été créée en 2017. Elle s’est inspirée des modèles des pays plus développés, où des associations de malades atteints de maladies rares et des malades dépourvus d’association se sont unis depuis plusieurs années en « Alliance », telles la France avec l’Alliance des Maladies Rares ou la Suisse avec Proraris. L’Alliance se donne par ailleurs pour but de contribuer à la création par les malades eux-mêmes d’associations spécifiques comme elle l’a fait par exemple pour : l’association marocaine des malades d’angioœdèmes (AMMAO) ou l’association pour les personnes atteintes de rachitisme vitamino résistant hypophosphatémique (RVRH-XLH). L’Alliance au Maroc collabore déjà étroitement avec beaucoup d’autres associations œuvrant sur une pathologie particulière comme l’Association de solidarité avec les enfants de la lune Maroc, l’Association Marocaine de Mucoviscidose, l’association des amis des myasthéniques (AAMM), l’association marocaine du syndrome de Rett (AMSR), l’association SOS Pku …
Dr Moussayer khadija Dr Moussayer khadija

Dr Moussayer khadija

Dr MOUSSAYER KHADIJA الدكتورة خديجة موسيار Spécialiste en médecine interne et en Gériatrie en libéral à Casablanca. Présidente de l’Alliance Maladies Rares Maroc (AMRM) et de l’association marocaine des maladies auto-immunes et systémiques (AMMAIS), Vice-présidente du Groupe de l’Auto-Immunité Marocain (GEAIM)


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Morocco and the Trust Economy: The Invisible Capital of Development... 406

In the economic history of nations, some assets are visible, such as natural resources, geographical position, infrastructure, or market size. Others, however, are invisible but often decisive. Among them, trust holds a central place and constitutes the true cement of sustainable economies. An economy can survive with few natural resources, but it cannot prosper sustainably without trust. Morocco today has many assets: remarkable political stability, a strategic position, world-class infrastructure, and active economic diplomacy. Yet, the decisive step in development now consists of building a true trust economy, capable of sustainably reassuring citizens, entrepreneurs, and investors. This is not a slogan. Trust is an institutional and cultural architecture that is built over time. It is the primary capital of a modern economy and a determining factor. It reduces transaction costs, encourages investment, facilitates innovation, and stimulates individual initiative. When an entrepreneur knows that the rules of the game are stable, that contracts will be respected, and that justice is swift and independent, he invests more easily. When a citizen trusts the tax administration and institutions, he more willingly accepts taxes and participates in the formal economy. Conversely, a lack of trust generates precautionary behaviors: capital flight, informality, low long-term investment. The economy then becomes cautious, fragmented, and inefficient. For Morocco, the central question is therefore not only to attract investments, but to create an environment where trust becomes a collective reflex. It would be unfair not to recognize the considerable progress made over the past decades. The foundations are solid. The country has massively invested in infrastructure: Tanger Med is today one of the world's most important logistics hubs. Nador and Dakhla are coming soon. Industrial zones have enabled the emergence of high-performing sectors, in the automotive industry with Renault Group and Stellantis, and in aeronautics with Boeing, Airbus, and Safran. The country's ambition in energy transition is exemplary. This shows that it is capable of carrying out structuring projects and offering a stable macroeconomic environment. However, the next step in development requires a qualitative leap: moving from an opportunity economy to a trust economy with a determining role for the rule of law. Trust first rests on the solidity of institutions. For investors as for entrepreneurs, the predictability of rules is a decisive element. Laws must be stable, readable, and applied equally, with three particularly crucial dimensions: **The independence and efficiency of justice** A swift, accessible, and credible justice system is the keystone of any trust economy. Commercial disputes must be resolved within reasonable timeframes. Judicial decisions must be enforced without ambiguity. Legal security is often the primary factor of attractiveness. **Fiscal stability** Investors do not necessarily expect very low tax rates; they primarily seek stability and readability. Predictable taxation allows companies to plan investments over the long term. Morocco has already undertaken several major tax reforms, but the challenge now is to go further and consolidate a clear and durable fiscal pact. **The fight against rents and privileges** Trust disappears when the rules of the game seem unequal. A dynamic economy relies on fair competition and equal opportunities. Transparency in public markets, competition regulation, and limiting rent situations are essential levers. A trust economy is also an economy of freedom, capable of unleashing entrepreneurial energy. The freedom to enterprise, innovate, and experiment is one of the fundamental engines of growth. Morocco has a talented youth, competent engineers, and an influential diaspora. However, several obstacles remain: administrative complexity, access to financing for SMEs, slowness of certain procedures. The challenge is to create an environment where individual initiative becomes the norm rather than the exception. Moroccan startups in fintech, artificial intelligence, or agricultural technologies already demonstrate the country's potential. With a more fluid ecosystem, they could become tomorrow's economic champions. In a world marked by geopolitical uncertainty and economic recompositions, trust also becomes a comparative advantage. If Morocco manages to position itself as a country where rules are stable, justice reliable, and administration predictable, it could become one of the main investment platforms between Europe and Africa. This ambition aligns with the Kingdom's African strategies and its growing international openness. Trust could thus become Morocco's true economic hallmark. Several strategic orientations deserve to be prioritized: - Accelerate the modernization of the judicial system, particularly in handling commercial disputes and enforcing judicial decisions. - Radically simplify administrative procedures for businesses through complete digitalization of public services. - Establish multi-year fiscal stability to enhance visibility. - Promote transparency and fair competition in all economic sectors. - Strengthen training and valorization of human capital, particularly in technological and scientific fields. - Develop a culture of trust between the State, businesses, and citizens. This dimension is often overlooked, yet it constitutes the invisible foundation of development. Morocco finds itself today at a pivotal moment in its economic history. The infrastructure is in place, strategic ambitions are affirmed, and the international environment offers new opportunities. The next step therefore consists of building a sustainable trust ecosystem. If Morocco succeeds in this gamble, and it must, it could not only accelerate its development but also become one of the most credible and attractive economies in the emerging world. In the 21st-century global economy, trust is undoubtedly the rarest and most powerful capital.

Football: When Passion Kills the Game in Impunity and Tolerance.. 1099

Football (Soccer for Americans) is first and foremost a matter of emotions. By its very essence, it is an open-air theater where human passions play out in their rawest, most primal form. It generates joy, anger, pride, humiliation, and a sense of belonging. From the stands of Camp Nou to those of the Diego Armando Maradona Stadium, through the fervor of the Mohamed V sport Complex in Casablanca, the vibrant enclosures of Stade Léopold Sédar Senghor in Dakar, or even the Parc des Princes in Paris, the Vélodrome In Marseille, and the Bernabeu In Madrid, football transcends the mere framework of the game to become a total social phenomenon. But this emotional intensity, which makes football's beauty, also constitutes its danger. For without rigorous regulation, it quickly tips into excess, then into violence. Today, it must be acknowledged that the rules exist, but they are too often circumvented, stripped of their substance, or applied with disconcerting leniency. On the pitches as in the stands, excesses are multiplying: insults toward referees, provocations between players, systematic challenges, physical violence, projectile throwing, pitch invasions, xenophobic remarks, racist offenses. What was once the exception is tending to become a tolerated norm. Astonishingly, we are starting to get used to it. Recent examples are telling. In Spain, in stadiums renowned for their football culture, racist chants continue to be belted out without shame, targeting players like Vinícius Júnior. Most recently, it was the Muslim community that was insulted. And yet, Spain's current football prodigy is Muslim. An overheated crowd that has doubtless forgotten it wasn't so long ago that it was Muslim itself. Among those chanting these remarks, and without a doubt, some still carry the genes of that recent past... In Dakar, just a few days ago, clashes escalated, turning a sports celebration into a scene of chaos. In Italy, incidents involving supporters who invaded the pitch, during a friendly match, no less, endangered players and officials, recalling the dark hours of European hooliganism in the 1980s. These episodes are not isolated; they reflect a worrying normalization of violence in and around stadiums. Even at the highest level of African football, behavioral excesses are becoming problematic. The 2025 Africa Cup of Nations final left a bitter taste. What should have been a moment of celebration for continental football was marred by behaviors contrary to sporting ethics. Pressures on refereeing, excessive challenges, and game interruptions have become commonplace. When a coach manipulates a match's rhythm to influence a refereeing decision, it is no longer strategy but a challenge to the very foundations of the sport. Despite international outrage, the sanctions imposed on teams, clubs, or players involved remain often symbolic, insufficient to eradicate these behaviors. A very surprising phenomenon: rarely have clubs or federations clearly distanced themselves from such crowds. They accommodate them, and when they condemn them, it is half-heartedly, in a muffled, timid tone with no effect. The problem is twofold. On one hand, disciplinary regulations exist but lack firmness. On the other, their application suffers from a lack of consistency and political courage. Bodies like FIFA, continental confederations, and national federations hesitate to impose truly dissuasive sanctions such as point deductions, prolonged closed-door matches, competition exclusions, or even administrative relegations. Yet without fear of sanction, the rule loses all effectiveness. It suffices to compare with other sports to measure the gap. In rugby, for example, respect for the referee is a cardinal value. The slightest challenge is immediately sanctioned. In athletics, a false start leads to immediate disqualification, no discussion. Football, meanwhile, still tolerates too many behaviors that should be unacceptable. This permissiveness has a cost. It undermines football's image, discourages some families from attending stadiums, and endangers the safety of the game's actors. More gravely, it paves the way for future tragedies. History has already taught us, through catastrophes like the Heysel Stadium disaster, that violence in stadiums can have tragic consequences. It is therefore urgent to react. Regulating football does not mean killing its soul, but rather preserving it. It is not about extinguishing passions, but channeling them. This requires strong measures, exemplary sanctions against offending clubs and players, accountability for national federations, increased use of technology to identify troublemakers, and above all, a clear political will from national and international governing bodies. Football cannot continue to be this "market of emotion" left to its own devices. For by tolerating the intolerable, it risks losing what makes its greatness and its ability to unite rather than divide. If FIFA does not decide to act firmly, the danger is real: that of seeing football sink into a spiral where violence triumphs over the game, and where, one day, tragedies exceed the mere framework of sport. The long-awaited decision of the Court of Arbitration for Sport (CAS) in the 2025 AFCON final case should confirm rigor and integrity in the application of rules, at least at this level, thereby strengthening the credibility of the pan-African competition and football in general.