Lefa la hemorrhagic telangiectasia

Lefa la hemorrhagic telangiectasia (HHT) ke lefu le futsitsoeng la methapo ea mali le ka bakang ho tsoa mali haholo.

HHT e fetisoa ka malapa ka mokhoa o ikhethileng oa autosomal. Sena se bolela hore liphatsa tsa lefutso tse sa tloaelehang li hlokahala ho motsoali a le mong feela molemong oa ho rua lefu lena.

Bo-rasaense ba supile liphatsa tsa lefutso tse 'ne tse amehang boemong bona. Liphatsa tsena tsohle tsa lefutso li bonahala li le bohlokoa hore methapo ea mali e hōle hantle. Phetoho efe kapa efe ea liphatsa tsena tsa lefutso e ikarabella ho HHT.

Batho ba nang le HHT ba ka hlahisa methapo ea mali e sa tloaelehang libakeng tse 'maloa tsa' mele. Likepe tsena li bitsoa arteriovenous malformations (AVMs).

Haeba li le letlalong, li bitsoa telangiectasias. Libaka tse atileng haholo li kenyelletsa molomo, leleme, litsebe le menoana. Methapo e sa tloaelehang ea mali le eona e ka hlaha bokong, matšoafong, sebeteng, mala, kapa libakeng tse ling.

Matšoao a lefu lena a kenyelletsa:

• Ho tsoa mali ka makhetlo a mangata baneng
• Ho tsoa mali ka mpeng (GI), ho kenyelletsa tahlehelo ea mali ka setuloaneng, kapa litulo tse lefifi kapa tse ntšo
• Ho oa kapa ho hlaseloa, likoti tse nyane (ho tsoa mali ho tsoa bokong)
• Phefumoloho e khutšoanyane
• Sebete se atolositsoeng
• Ho hloleha ha pelo
• Phokolo ea mali e bakoang ke tšepe e tlase

Mofani oa tlhokomelo ea bophelo o tla etsa tlhahlobo ea 'mele' me a botse ka matšoao a hau. Mofani ea nang le boiphihlelo a ka fumana li-telangiectases nakong ea tlhahlobo ea 'mele. Hangata ho na le nalane ea lelapa ea boemo bona.

Liteko li kenyelletsa:

• Liteko tsa khase ea mali
• Liteko tsa mali
• Ho nahana ka teko ea pelo e bitsoang echocardiogram
• Endoscopy, e sebelisang kh'amera e nyane e hokahantsoeng le tube e tšesaane ho sheba kahare ho mmele oa hau
• MRI ho fumana li-AVM bokong
• Lisebelisoa tsa CT kapa tsa ultrasound ho fumana li-AVM sebeteng

Ho hlahlojoa ha liphatsa tsa lefutso hoa fumaneha ho batla liphetoho liphatseng tsa lefutso tse amanang le lefu lena.

Kalafo e ka kenyelletsa:

• Ho buoa ho phekola ho tsoa mali libakeng tse ling
• Electrocautery (ho futhumatsa lisele ka motlakase) kapa ho buuoa ka laser ho phekola mali a tsoang hangata kapa a boima
• Endovascular embolization (ho kenya ntho ka har'a tube e tšesaane) ho phekola methapo ea mali e sa tloaelehang bokong le likarolong tse ling tsa 'mele

Batho ba bang ba arabela kalafo ea estrogen, e ka fokotsang linako tsa ho tsoa mali. Iron le eona e ka fuoa haeba ho na le tahlehelo e ngata ea mali, e lebisang ho phokolo ea mali. Qoba ho noa meriana e fokotsang mali. Meriana e meng e amang nts'etsopele ea methapo ea mali e ntse e ithutoa ka mekhoa ea kalafo ea nako e tlang.

Batho ba bang ba kanna ba hloka ho noa lithibela-mafu pele ba etsa mosebetsi oa meno kapa opereishene. Batho ba nang le li-AVM tsa matšoafo ba lokela ho qoba ho qoela ka scuba ho thibela mafu a sithabetsang (ho koba). Botsa mofani oa hau hore na u lokela ho nka mehato efe e meng ea ho itšireletsa.

Lisebelisoa tsena li ka fana ka tlhaiso-leseling e batsi ka HHT:

• Litsi tsa Tlhokomelo le Thibelo ea Maloetse - www.cdc.gov/ncbddd/hht
• Phekola HHT - curehht.org
• Mokhatlo oa Naha oa Mathata a Rare - rarediseases.org/rare-diseases/hereditary-hemorrhagic-telangiectasia

Batho ba nang le lefu lena ba ka phela bophelo bo tloaelehileng ka botlalo, ho latela hore na li-AVM li hokae 'meleng.

Mathata ana a ka hlaha:

• Ho hloleha ha pelo
• Khatello ea mali e phahameng matšoafong (khatello ea methapo ea mali)
• Ho tsoa mali ka hare
• Phefumoloho e khutšoanyane
• Leqeba

Bitsa mofani oa hau haeba uena kapa ngoana oa hau a tsoa mali khafetsa kapa matšoao a mang a lefu lena.

Tlhabollo ea lefutso e khothalletsoa banyalani ba batlang ho ba le bana le ba nang le nalane ea lelapa ea HHT. Haeba u na le boemo bona, kalafo ea meriana e ka thibela mefuta e itseng ea lipolao le ho hloleha ha pelo.

HHT; Lefu la Osler-Weber-Rendu; Lefu la Osler-Weber-Rendu; Lefu la Rendu-Osler-Weber

• Tsamaiso ea potoloho ea mali
• Metsing ea boko

Brandt LJ, Aroniadis OC. Mathata a vascular ea pampiri ea meno. Ka: Feldman M, Friedman LS, Brandt LJ, bahlophisi. Sleisenger le Fordtran's Mpeng le Mafu a Sebete. 10th ed. Philadelphia, PA: Elsevier Saunders; 2016: khaolo ea 37.

Cappell MS, Lebwohl O. Lefu la hemorrhagic telangiectasia Ho: Lebwohl MG, Heymann WR, Berth-Jones J, Coulson IH, eds. Kalafo ea Mafu a Letlalo: Mekhoa e Feletseng ea Phekolo. La 4 ed. Philadelphia, PA: Elsevier Saunders; 2014: khaolo ea 98.

McDonald J, Pyeritz RE. Lefa la hemorrhagic telangiectasia. Ka: Adam MP, Ardinger HH, Pagon RA, et al, eds. GeneReviews [Marang-rang]. Seattle, WA: Univesithi ea Washington, Seattle; 1993-2019. E ntlafalitsoe ka la 2 Hlakola 2017. E fihlile ka la 6 Mots'eanong, 2019.