Isifo seMaroteaux-Lamy

I-Maroteaux-Lamy Syndrome noma i-Mucopolysaccharidosis VI isifo esingajwayelekile sokuzalwa, lapho iziguli zinezici ezilandelayo:

• Mfushane,
• ukukhubazeka ebusweni,
• intamo emfushane,
• i-otitis ephindaphindayo,
• izifo zokuphefumula,
• ukukhubazeka kwamathambo kanye
• ukuqina kwemisipha.

Lesi sifo sidalwa yizinguquko ku-enzyme i-Arylsulfatase B, esivimbela ukuthi senze umsebenzi waso, okwehlisa isithunzi ama-polysaccharides, nawo aqoqelwe kumaseli, athuthukise izimpawu zesifo.

Abantu abane-syndrome banobuhlakani obujwayelekile, ngakho-ke izingane azidingi isikole esikhethekile, kuphela izinto eziguquliwe ezisebenzisa ukuxhumana nothisha nabantu ofunda nabo.

Ukuxilongwa kwenziwa ngofuzo olususelwa ekuhlolweni kwemitholampilo nasekuhlaziyweni kwelabhoratri yamakhemikhali. Ukuxilongwa eminyakeni yokuqala yokuphila kubaluleke kakhulu ekwandisweni kohlelo lokungenelela kusenesikhathi, oluzosiza ekukhuleni kwengane nasekudluliseleni kwabazali ekululekeni ngokwengqondo, ngoba basengozini yokudlulisela lesi sifo ku izingane zabo zakamuva.

Alikho ikhambi leMaroteaux-Lamy Syndrome, kepha okunye ukwelashwa okufana nokufakelwa komnkantsha wethambo kanye nokwelashwa okufaka esikhundleni se-enzyme kuyasebenza ekwehliseni izimpawu. I-Physiotherapy isetshenziselwa ukunciphisa ukuqina kwemisipha futhi yenyuse ukunyakaza komzimba komuntu. Akuzona zonke izithwali ezinezimpawu zesifo, ubucayi buyehluka kuye ngomuntu ngamunye, abanye bayakwazi ukuphila impilo ejwayelekile.