Isifo seMaroteaux-Lamy

IMaroteaux-Lamy Syndrome okanye iMucopolysaccharidosis VI sisifo esinqabileyo selifa, apho izigulana zinezi mpawu zilandelayo:

• Mfutshane,
• ukukhubazeka ebusweni,
• intamo emfutshane,
• otitis ephindaphindayo,
• izifo zokuphefumula,
• ukungasebenzi kakuhle kwamathambo kunye
• ukuqina kwemisipha.

Esi sifo sibangelwa lutshintsho kwi-enzyme i-Arylsulfatase B, ethintela ukuba yenze umsebenzi waso, ukuthoba iipolysaccharides, eziye zaqokelelwa kwiiseli, zikhulisa iimpawu zesifo.

Abantu abanesifo sengqondo banengqondo eqhelekileyo, ngoko abantwana abayidingi isikolo esikhethekileyo, kuphela izinto ezilungelelanisiweyo eziququzelela ukusebenzisana nootitshala kunye nabo bafunda nabo.

Uxilongo lwenziwa sisazinzulu ngokusekwe kuvavanyo lweklinikhi kunye nohlalutyo lwebhayiloji ye-biochemical. Ukuxilongwa kwiminyaka yokuqala yobomi kubaluleke kakhulu ekwandisweni kwesicwangciso sokungenelela kwangethuba, esiya kuthi sincede ekuphuhliseni umntwana nasekudluliseleni abazali kwingcebiso ngemfuzo, kuba basemngciphekweni wokudlulisela esi sifo abantwana babo bamva.

Akukho lunyango lweMaroteaux-Lamy Syndrome, kodwa ezinye zonyango ezinje ngokufakelwa komongo wethambo kunye nokunyanga endaweni ye-enzyme ziyasebenza ukunciphisa iimpawu. I-Physiotherapy isetyenziselwa ukunciphisa ukuqina kwemisipha kunye nokwandisa ukunyakaza komzimba. Ayizizo zonke izinto ezithwala abanazo zonke iimpawu zesi sifo, ubuzaza buyahluka ngokwabantu ngabanye, abanye bayakwazi ukuphila ubomi obuqhelekileyo.