Nhaka yekubuda ropa telangiectasia

Hereditary hemorrhagic telangiectasia (HHT) chirwere chakagarwa nhaka yemidzi yeropa iyo inogona kukonzera kubuda ropa zvakanyanya.

HHT inopfuudzwa kuburikidza nemhuri mune ye autosomal yakanyanya pateni. Izvi zvinoreva kuti geni risina kujairika rinodiwa kubva kumubereki mumwe chete kuti ugare nhaka chirwere.

Masayendisiti akawana majini mana akabatanidzwa mune ino mamiriro. Ese majini aya anoita kunge akakosha kuti tsinga dzeropa dzigone kukura zvakanaka. Shanduko mune chero yeaya magene inokonzera HHT.

Vanhu vane HHT vanogona kukura zvisina kujairika mitsipa yeropa munzvimbo dzinoverengeka dzemuviri. Iyi midziyo inonzi arteriovenous malformations (AVMs).

Kana ivo vari paganda, vanonzi telangiectasias. Nzvimbo dzinowanzozivikanwa dzinosanganisira miromo, rurimi, nzeve, neminwe. Tsinga dzeropa dzisina kujairika dzinogona zvakare kukura muuropi, mapapu, chiropa, ura, kana dzimwe nzvimbo.

Zviratidzo zvechirwere ichi zvinosanganisira:

• Kazhinji kubuda mhino muvana
• Kubuda ropa mudumbu (GI), kusanganisira kurasikirwa neropa muzvigaro, kana rima kana zvitema zvituru.
• Kugwinha kana kusatsanangurwa, kurohwa kudiki (kubva pakubuda ropa kupinda muuropi)
• Kufema kwekufema
• Yakawedzera chiropa
• Kutadza kwemoyo
• Anemia inokonzerwa nesimbi yakaderera

Anopa hutano anozoongorora muviri uye obvunza nezve zviratidzo zvako. Anopa ruzivo anogona kuona telangiectases panguva yekuongorora kwepanyama. Iko kazhinji kune nhoroondo yemhuri yeichi chiitiko.

Miedzo inosanganisira:

• Ropa gasi bvunzo
• Kuongororwa kweropa
• Kufungidzira kuyedza kwemoyo kunonzi echocardiogram
• Endoscopy, iyo inoshandisa kamera diki yakasungirirwa pombi yakatetepa kutarisa mukati memuviri wako
• MRI yekuona maAVM muuropi
• CT kana ultrasound inoongorora kuona maAVM muchiropa

Kuongorora kweGenetic kunowanikwa kutsvaga shanduko mumageneti ane hukama neiyi syndrome.

Mishonga inogona kusanganisira:

• Kuvhiya kwekurapa kubuda ropa mune dzimwe nzvimbo
• Electrocautery (kudziyisa tishu nemagetsi) kana laser kuvhiya kurapa kakawanda kana rinorema nosebleeds
• Endovascular embolization (jekiseni chinhu kuburikidza netete chubhu) kurapa zvisina kujairika mitsipa yeropa muuropi uye zvimwe zvikamu zvemuviri

Vamwe vanhu vanopindura kune estrogen kurapwa, iyo inogona kudzikisa kubuda kweropa zvikamu. Iron inogona zvakare kupihwa kana paine yakawanda kurasikirwa kweropa, zvichikonzera kushomeka. Dzivisa kutora mishonga yekuchera ropa. Mimwe mishonga inokanganisa kukura kwemidziyo yeropa iri kuongororwa senzira yekurapa yeramangwana.

Vamwe vanhu vangangoda kutora maantibayoti vasati vaita mazino kana kuvhiyiwa. Vanhu vane AVM yemapapu vanofanirwa kudzivirira scuba diving kudzivirira decompression chirwere (iyo inokombama). Kumbira mupiki wako kuti ndezvipi zvimwe zvekuchengetedza zvaunofanira kutora.

Izvi zviwanikwa zvinogona kupa rumwe ruzivo nezveHHT:

• Nzvimbo dzekudzivirira nekudzivirira hosha - www.cdc.gov/ncbddd/hht
• Poresa HHT - curehht.org
• National Sangano reRare Dambudziko - rarediseases.org/rare-diseases/hereditary-hemorrhagic-telangiectasia

Vanhu vane chirwere ichi vanogona kurarama hupenyu hwakajairika, zvinoenderana nekuti mumuviri maAVM aripi.

Aya matambudziko anogona kuitika:

• Kutadza kwemoyo
• Kuwedzera kweropa mumapapu (pulmonary hypertension)
• Kubuda ropa mukati
• Kufema kwekufema
• Kurohwa

Fonera waunopa kana iwe kana mwana wako muchigara muchibuda mhino kana zvimwe zviratidzo zvechirwere ichi.

Genetic counselling inokurudzirwa kune vakaroorana vanoda kuve nevana uye vane nhoroondo yemhuri yeHHT. Kana iwe uine ichi chiitiko, kurapwa kwekurapa kunogona kudzivirira mamwe marudzi eshamhu uye moyo kutadza.

HHT; Osler-Weber-Rendu chirwere; Osler-Weber-Rendu chirwere; Rendu-Osler-Weber chirwere

• Kutenderera system
• Tsinga dzehuropi

Brandt LJ, Aroniadis OC. Vascular kusagadzikana kwepamuviri wedumbu. Muna: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger uye Fordtran's Gastrointestinal uye Chirwere Chiropa. 10th ed. Philadelphia, PA: Elsevier Saunders; 2016: chits. 37.

Cappell MS, Lebwohl O. Hereditary hemorrhagic telangiectasia Mu: Lebwohl MG, Heymann WR, Berth-Jones J, Coulson IH, eds. Kurapa kweGanda Rezvirwere: Dzakakwana Therapeutic Strategies. 4th ed. Philadelphia, PA: Elsevier Saunders; 2014: chits. 98.

McDonald J, Pyeritz RE. Nhaka yekubuda ropa telangiectasia. Mune: Adam MP, Ardinger HH, Pagon RA, et al, eds. GeneReviews [Indaneti]. Seattle, WA: Yunivhesiti yeWashington, Seattle; 1993-2019. Yakagadziridzwa Kukadzi 2, 2017. Yakasvika muna Chivabvu 6, 2019.