I-Proteus syndrome: iyini, ungayikhomba kanjani futhi uyiphathe kanjani

-Delile

• Izici eziyinhloko
• Yini ebangela lesi sifo
• Yelashwa kanjani
• Indima yesazi sokusebenza kwengqondo ku-Proteus syndrome

I-Proteus syndrome yisifo esingajwayelekile sezakhi zofuzo esibonakala ngokukhula ngokweqile nangokulinganayo kwamathambo, isikhumba nezinye izicubu, okuholela ekugigiteni kwezitho nezitho eziningana, ikakhulu izingalo, imilenze, ugebhezi nomgogodla.

Izimpawu zeProteus Syndrome zivame ukuvela phakathi kwezinyanga eziyisithupha kuya kwezingu-18 ubudala futhi ukukhula okweqile nokungalingani kuvame ukuma ebusheni. Kubalulekile ukuthi i-syndrome ikhonjwe ngokushesha ukuze kuthathwe izinyathelo ezisheshayo zokulungisa ukukhubazeka nokwenza ngcono isimo somzimba seziguli zesifo, ukugwema izinkinga zengqondo, njengokuhlukaniswa nabantu kanye nokudangala, ngokwesibonelo.

Izici eziyinhloko

I-Proteus syndrome ivame ukudala ukubonakala kwezinye izici, njenge:

• Ukukhubazeka ezingalweni, emilenzeni, kugebhezi nakumgogodla;
• I-asymmetry yomzimba;
• Amafolda esikhumba ngokweqile;
• Izinkinga zomqolo;
• Ubuso obude;
• Izinkinga zenhliziyo;
• Izinsumpa namabala akhanyayo emzimbeni;
• Ubende obukhulisiwe;
• Khulisa ububanzi beminwe, obubizwa nge-digital hypertrophy;
• Ukukhubazeka kwengqondo.

Yize kunezinguquko eziningana zomzimba, ezimweni eziningi, iziguli ezine-syndrome zithuthukisa amakhono azo obuhlakani ngokujwayelekile, futhi zingaba nokuphila okujwayelekile.

Kubalulekile ukuthi i-syndrome ikhonjwe kusenesikhathi, ngoba uma ukuqapha kwenziwa kusukela kwavela izinguquko zokuqala, kungasiza hhayi kuphela ukugwema ukuphazamiseka kwengqondo, kepha futhi nokugwema ezinye izinkinga ezijwayelekile zalesi sifo, njengokuvela kwezimila ezingavamile noma ukuvela kwe-thrombosis ejulile ye-venous.

Yini ebangela lesi sifo

Imbangela yeProteus syndrome ayikakasungulwa kahle, kepha kukholakala ukuthi kungaba yisifo sofuzo esivela ekuguqulweni okuzenzekelayo kuhlobo lwe-ATK1 olwenzeka ngesikhathi sokukhula kombungu.

Yize iyisakhi zofuzo, iProteus syndrome ayithathwa njengelifa, okusho ukuthi abukho ubungozi bokudlulisa ukuguquka kusuka kubazali kuye ezinganeni. Kodwa-ke, uma kunamacala e-Proteus syndrome emndenini, kunconywa ukuthi ukwelulekwa ngezofuzo kwenziwe, ngoba kungaba nokuhleleka okukhulu kokuvela kwalokhu kuguquka.

Yelashwa kanjani

Akukho ukwelashwa okuqondile kweProteus syndrome, futhi imvamisa kunconywa udokotela ukusebenzisa amakhambi athile ukulawula ezinye izimpawu, ngaphezu kokuhlinzwa ukulungisa izicubu, ukususa izicubu nokwenza ngcono ubuhle bomzimba.

Lapho sitholwa kusaqalwa, i-syndrome ingalawulwa ngokusebenzisa umuthi obizwa ngeRapamycin, okuwumuthi wokuvikela umzimba okhonjiswe ngenhloso yokuvimbela ukukhula kwezicubu okungavamile nokuvimbela ukwakheka kwezimila.

Ngaphezu kwalokho, kubaluleke kakhulu ukuthi ukwelashwa kwenziwe yithimba lezinhlobonhlobo zochwepheshe bezempilo, okufanele lifake odokotela bezingane, odokotela bamathambo, odokotela abahlinzayo bepulasitiki, abesikhumba, odokotela bamazinyo, ama-neurosurgeons kanye nezazi zokusebenza kwengqondo, ngokwesibonelo. Ngaleyo ndlela, umuntu uzoba nakho konke ukwesekwa okudingekayo ukuze abe nempilo enhle.

Indima yesazi sokusebenza kwengqondo ku-Proteus syndrome

Ukuqapha ngokwengqondo kubaluleke kakhulu hhayi kuphela esigulini esinalesi sifo kepha nakumalungu omndeni waso, ngoba ngale ndlela kungenzeka ukuqonda isifo nokusebenzisa izindlela ezithuthukisa izinga lomuntu lokuphila nokuzethemba. Ngaphezu kwalokho, isazi sokusebenza kwengqondo kubalulekile ukuthuthukisa ubunzima bokufunda, ukwelapha amacala okudangala, ukunciphisa ukungaphatheki kahle komuntu nokuvumela ukuxhumana nabantu.