Proteus syndrome: ke eng, mokhoa oa ho e tseba le ho e phekola
Litaba
- Lintlha tse ka sehloohong
- Lefu le bakoa ke eng
- Kalafo e etsoa joang
- Karolo ea setsebi sa kelello ho Proteus syndrome
Proteus syndrome ke lefu le sa tloaelehang la liphatsa tsa lefutso le khetholloang ke kholo e feteletseng le e sa lekanang ea masapo, letlalo le lisele tse ling, tse hlahisang gigantism ea maoto le matsoho a 'maloa, haholo-holo matsoho, maoto, lehata le lesapo la mokokotlo.
Matšoao a Proteus Syndrome hangata a hlaha lipakeng tsa likhoeli tse 6 ho isa ho tse 18 mme kholo e fetelletseng le e sa lekanyetsoang e atisa ho emisa bocheng. Ho bohlokoa hore lefu lena le tsejoe ka potlako e le hore ho nkuoe mehato e potlakileng ho lokisa bofokoli le ho ntlafatsa sebopeho sa bakuli ba lefu lena, ho qoba mathata a kelello, joalo ka ho itšehla thajana sechabeng le khatello ea maikutlo.
Proteus syndrome e letsohongLintlha tse ka sehloohong
Proteus syndrome hangata e baka ponahalo ea likarolo tse ling, joalo ka:
- Ho holofala matsohong, maotong, lehata le mokokotlong;
- Asymmetry ea 'mele;
- Letlalo le feteletseng;
- Mathata a mokokotlo;
- Sefahleho se selelele;
- Mathata a pelo;
- Li-warts le mabala a bobebe 'meleng;
- Spleen e atolositsoeng;
- Eketsa bophara ba menoana, e bitsoang digital hypertrophy;
- Ho holofala kelellong.
Leha ho na le liphetoho tse 'maloa mmeleng, maemong a mangata, bakuli ba nang le lefu lena ba holisa bokhoni ba bona ba kelello ka mokhoa o tloaelehileng,' me ba ka phela bophelo bo tloaelehileng.
Ho bohlokoa hore lefu lena le tsejoe kapele kamoo ho ka khonehang, hobane haeba tlhahlobo e etsoa ho tloha ha ho hlaha liphetoho tsa pele, e ka thusa eseng feela ho qoba mathata a kelello, empa hape le ho qoba mathata a mang a tloaelehileng a lefu lena, joalo ka joalo ka ponahalo ea lihlahala tse sa tloaelehang kapa ketsahalo ea thrombosis e tebileng ea venous.
Lefu le bakoa ke eng
Sesosa sa lefu la Proteus ha se so tsejoe hantle, leha ho le joalo ho lumeloa hore e ka ba lefu la lefutso le bakoang ke phetoho e itlelang feela ea lefutso la ATK1 le hlahang nakong ea kholo ea lesea.
Leha e le lefutso, Proteus syndrome ha e nkoe e le lefutso, ho bolelang hore ha ho na kotsi ea ho fetisetsa phetoho ho tloha ho batsoali ho ea ho bana. Leha ho le joalo, haeba ho na le linyeoe tsa Proteus syndrome ka lapeng, ho kgothaletswa hore tlhabollo ea lefutso e etsoe, kaha ho ka ba le monyetla o moholo oa ho hlaha ha phetoho ena.
Kalafo e etsoa joang
Ha ho na kalafo e ikhethang bakeng sa Proteus syndrome, mme hangata ho khothaletsoa ke ngaka ho sebelisa litlhare tse ikhethileng ho laola matšoao a mang, ntle le ts'ebetso ea ho buoa ho lokisa lisele, ho tlosa lihlahala le ho ntlafatsa botle ba 'mele.
Ha lefu lena le fumanoa maemong a pele, le ka laoloa ka ts'ebeliso ea moriana o bitsoang Rapamycin, e leng moriana o thibelang khatello ea mmele o bontšitsoeng ka sepheo sa ho thibela kholo e sa tloaelehang ea lisele le ho thibela ho theoa ha lihlahala.
Ntle le moo, ho bohlokoa haholo hore kalafo e etsoe ke sehlopha sa litsebi tse ngata tsa bophelo bo botle, se lokelang ho kenyelletsa lingaka tsa bana, lingaka tsa meno, lingaka tsa polasetiki, lingaka tsa meno, lingaka tsa meno, lingaka tsa methapo le lingaka tsa kelello, mohlala. Ka tsela eo, motho o tla ba le ts'ehetso eohle e hlokahalang hore a be le bophelo bo botle.
Karolo ea setsebi sa kelello ho Proteus syndrome
Tlhokomelo ea kelello e bohlokoa haholo eseng feela ho mokuli ea nang le lefu lena empa le ho litho tsa malapa a bona, kaha ka tsela ena ho ka khoneha ho utloisisa lefu lena le ho nka mehato e ntlafatsang boleng ba bophelo ba motho le boitšepo ba hae. Ntle le moo, setsebi sa kelello se bohlokoa ho ntlafatsa mathata a ho ithuta, ho sebetsana le maemo a khatello ea maikutlo, ho fokotsa ho se phutholohe ha motho le ho lumella ho sebelisana le sechaba.