Isifo seBirt-Hogg-Dubé

Umxholo

• Imifanekiso yeBirt-Hogg-Dubé Syndrome
• Iimpawu zeBirt-Hogg-Dubé Syndrome
• Unyango lwe-Birt-Hogg-Dubé Syndrome
• Amakhonkco aluncedo:

I-Birt-Hogg-Dubé Syndrome sisifo esinqabileyo semfuza esidala ulusu, izilonda zezintso kunye nee-cysts emiphungeni.

Kwi oonobangela be-Birt-Hogg-Dubé Syndrome zizinto eziguqukayo kwimfuza kwi-chromosome 17, ebizwa ngokuba yi-FLCN, ephulukana nomsebenzi wayo njenge-suppressor ye-tumor kwaye ikhokelele ekubonakaleni kwezidumbu ebantwini.

THE Isifo seBirt-Hogg-Dubé asinyangi kwaye unyango lwayo lubandakanya ukususa amathumba kunye nokuthintela ukubonakala kwawo.

Imifanekiso yeBirt-Hogg-Dubé Syndrome

Kwiifoto unokuchonga izilonda zesikhumba ezivela kwi-Birt-Hogg-Dubé Syndrome, ekhokelela kwizidumbu ezincinci ezinobungozi ezijikeleza iinwele.

Iimpawu zeBirt-Hogg-Dubé Syndrome

Iimpawu zeBirt-Hogg-Dubé Syndrome inokuba:

• Amathumba ebenign kulusu, ikakhulu ebusweni, entanyeni nasesifubeni;
• Cysts kwezintso;
• Izibilini zezintso okanye umhlaza wezintso;
• Cysts pulmonary;
• Ukuqokelelwa komoya phakathi kwemiphunga kunye ne-pleura, ekhokelela ekubonakaleni kwe-pneumothorax;
• Amaqhuqhuva e-thyroid.

Abantu abane-Birt-Hogg-Dubé Syndrome kunokwenzeka ukuba baphuhlise umhlaza kwamanye amalungu omzimba afana nebele, i-amygdala, umphunga okanye amathumbu.

Izilonda ezibonakala eluswini zibizwa ngokuba yi-fibrofolliculomas kwaye ziqulathe amaqhakuva amancinci akhokelela ekuqokeleleni i-collagen kunye nemicu ejikeleze iinwele. Ngokubanzi, olu phawu kulusu lweBirt-Hogg-Dubé Syndrome luvela phakathi kweminyaka engama-30 ukuya kwengama-40 ubudala.

O Ukufunyaniswa kweBirt-Hogg-Dubé Syndrome kufezekiswa ngokuchonga iimpawu zesifo kunye novavanyo lwemfuza ukufumanisa utshintsho kwi-FLNC gene.

Unyango lwe-Birt-Hogg-Dubé Syndrome

Unyango lwe-Birt-Hogg-Dubé Syndrome ayisinyangi isifo, kodwa iyanceda ukunciphisa iimpawu kunye neziphumo kubomi babantu.

Izidumbu zeBenign ezivela kulusu zinokususwa ngotyando, i-dermo-abrasion, ilaser okanye ukunxiba ulusu.

Iiculmonary cysts okanye izilonda zezintso kufuneka zithintelwe kusetyenziswa ikhompyuter ye-tomography, i-magnetic resonance okanye iimviwo ze-ultrasound. Ukuba ubukho bama-cysts okanye tumors bufunyenwe kwiimviwo, kufuneka zisuswe ngotyando.

Kwiimeko apho umhlaza wezintso ukhula khona, unyango kufuneka lube notyando, ichemotherapy okanye unyango ngemitha.

Amakhonkco aluncedo:

• Izintso zezintso
• Pneumothorax