5. Rett-szindróma a fiúk esetében

Dr. Jill Clayton Smith cikke nyomán
    Sokak számára meglepő lehet a kijelentés, mely szerint a RSZ nem csak lányok, hanem fiúk esetében is elő fordulhat. Már számos esetben leírtak fiúknál is „rettes” tüneteket, de nem bizonyították ezeket genetikai tesztekkel.
Jól tudjuk, hogy a RSZ-át az X kromoszómán található hibás gén okozza (MECP2). Az X kromoszóma a kromoszómapár egyik tagja, amely a gyermek nemét meghatározza. A lányoknak két X, a fiúknak egy X és egy Y kromoszómájuk van.
Ha a lány hordozza az egyik X kromoszómáján a hibás gént, a másik egészséges X kromoszómája jól tudja kompenzálni az egyes hibákat, s habár ennek a működése nem lesz tökéletes, és RSZ-ás lesz a gyermek, de legalább életképes marad. Azonban ha egy fiú X kromoszómáján sérül a gén, nincs háttér, ami kompenzálná a hibákat. A RSZ génje olyan meghatározó szerepű az embrionális fejlődésben, hogy ha ez nem működik legalább részlegesen, a terhesség nem lesz képes megmaradni. Ez az oka annak, hogy oly kevés fiúgyermek születik meg Rett-szindrómával. A kutatások szerint másra is fény derült az elmúlt időszakban. Ha a fiúgyermeknek két X és egy Y kromoszómája van, akkor a második X képes a kompenzációra, s a gyermek életben marad fiú létére.  Egy másik esetben úgy tűnt, hogy egy fiú nem minden testi sejtjében hordozta a hibás gént, illetve néhány sejtje a MECP2 jelenléte ellenére normálisan működött, s ez tette lehetővé a túlélést számára. Ezt az esetet hívják mozaicizmusnak, és valószínű, hogy a legtöbb fiú, aki a RSZ ellenére életben marad, a mozaicizmusnak köszönheti létét.
Felmerülhet a kérdés, hogy vajon milyen gyakori a RSZ a fiúknál. A válasz szerint ez valószínűleg nagyon ritka. Előfordulhat, hogy a tünetek sem olyan klasszikusak, mint a lányoknál, de még sok RSZ-ás fiút kell megvizsgálni ahhoz, hogy erről többet megtudjunk.
Néhányukban felmerülhet a kérdés, vajon érintett lehet-e a család, főleg ahol a RSZ-ás lánynak fiútestvére is van. Ez valószínűleg elég ritka, hiszen a hatalmas kutatási mintát alapul véve az állapítható meg, hogy a „rettes” gén egyszer jelenik meg, magában az érintett személyben, a szülei nem hordozói annak. Nagyon kevés az esély arra, hogy egy családban még egy RSZ-ás gyermek szülessen, akár lány, akár fiú. A Rett-szindróma valószínűleg csupán egyszeri, spontán mutáció alapján fordul elő, a familiáris ismétlődés nem jellemző. Egypetéjű ikrek esetében láttunk példát az ismétlődésre, de az okok ebben az esetben érthetőek (Nemzetközi Rett Szindróma Alapítvány, 2005).

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