Kodi mumatuluka magazi m'mphuno nthawi zambiri? Kungakhale HHT (Hereditary Hemorrhagic Telangiectasia)!

Kodi nthawi zambiri mumayamba kutuluka magazi m'mphuno? Kapena mwawona madontho ofiira ang'onoang'ono m'thupi lanu, mwachitsanzo, pankhope panu, pamilomo, kapena m'zala? Nthawi zina sitimaganiza kuti izi ndi zinthu zachilendo, koma kumbuyo kwawo pakhoza kukhala vuto lachipatala lomwe likufunika chisamaliro. Ndicho chimene HHT ili, koma anthu ambiri sadziwa za izo. Lero tikulankhula za HHT iyi, kapena m'mawu azachipatala, `(Hereditary Hemorrhagic Telangiectasia)`, matenda obadwa nawo. Ena amatchanso `(Osler-Weber-Rendu syndrome)`.

Kodi kwenikweni HHT ndi chiyani?

Mwachidule, HHT ndi matenda a majini omwe amakhudza mitsempha yamagazi m'matupi athu. Mukudziwa, tili ndi machubu ang'onoang'ono awa omwe amanyamula magazi m'matupi athu onse, omwe timawatcha kuti mitsempha yamagazi. Pa vutoli, chomwe chimachitika ndichakuti mitsempha yamagazi iyi, makamaka yaying'ono kwambiri yotchedwa capillaries, sikukula bwino. Timatcha ma capillaries opangidwa molakwika awa kuti ``telangiectasias''.

Taganizirani izi, tili ndi mitsempha ikuluikulu yomwe imanyamula magazi kuchokera mumtima mwathu kupita ku thupi lathu lonse, ndi mitsempha yomwe imanyamula magazi amenewo kubwerera kumtima. Kulumikizana pakati pa ziwirizi kumapangidwa ndi zomwe zimatchedwa capillaries. Mu HHT, nthawi zina mitsempha iyi ndi mitsempha imatha kukhala ndi kulumikizana kosazolowereka mwachindunji, popanda kudutsa m'mitsempha. Timatcha izi arteriovenous malformations, kapena AVM mwachidule.

Mitsempha yamagazi yosazolowereka iyi ndi yofooka kwambiri komanso yofooka. Imatha kuphulika kapena kusweka mosavuta, zomwe zimapangitsa kuti magazi atuluke (kutuluka magazi). Zizindikiro ndi zovuta zake zimadalira komwe magazi akuchokera.

Ndani angapange HHT?

Matendawa otchedwa HHT angakhudze akazi, amuna, komanso ana aang'ono . Fuko, chipembedzo, ndi mtundu wa munthu sizili zofunika pa izi. Makamaka chifukwa chakuti amapatsiridwa kuchokera ku mibadwomibadwo kudzera m'majini, ngati wina m'banjamo ali nawo, ena ali ndi mwayi woti nawonso adwale matendawa.

Izi zimaonedwa kuti ndi vuto losowa kwambiri. Komabe, nthawi zambiri silipezeka mokwanira. Izi zikutanthauza kuti, ngakhale kuti akuti munthu m'modzi mwa anthu 5,000 padziko lonse lapansi ali ndi matendawa, ambiri mwa iwo sadziwa kuti ali nawo.

Kodi n’chiyani chimayambitsa HHT?

Monga ndanenera kale, HHT ndi matenda obadwa nawo m'majini . Izi zikutanthauza kuti amatengera kwa makolo kupita kwa ana. Amaonedwa kuti ndi "vuto lalikulu". Mwachidule, mwana akhoza kudwala matendawa ngakhale kholo limodzi lokha, mayi kapena bambo, ndi omwe alandira jini losazolowereka .

Asayansi apeza mazanamazana a masinthidwe osiyanasiyana m'majini asanu ndi limodzi omwe amalumikizidwa ndi HHT. Komabe, pakadali pano, masinthidwe ofala kwambiri ali m'majini awiri otchedwa ENG ndi ACVRL1. Asayansi akufufuzabe izi.

Kodi zizindikiro za HHT ndi ziti?

Zizindikiro za HHT zimatha kusiyana kwambiri kuchokera kwa munthu ndi munthu. Zimatengera komwe mitsempha yamagazi yolakwika yomwe ndatchulayi ili m'thupi. Anthu ena sangakhale ndi zizindikiro zazikulu. Koma ena akhoza kukhala ndi zizindikiro zoopsa kwambiri.

Komabe, chizindikiro chofala kwambiri pakati pa anthu omwe ali ndi HHT ndi kutuluka magazi pafupipafupi (epistaxis) . Anthu ena amatha kutuluka magazi kangapo patsiku. Vutoli likhoza kukhalapo kuyambira ali ana.

Kuphatikiza apo, anthu ena omwe ali ndi HHT amatha kuona madontho ofiira ang'onoang'ono (telangiectasias) m'thupi lawo. Izi zingawoneke ngati zasintha pang'ono kwa inu. Izi zimawonekera kwambiri pa:

• Pankhope
• Mu zala kapena zala
• M'manja
• Mkati mwa pakamwa (pa nembanemba ya mucous)
• Pamilomo
• Mozungulira mphuno

Kuwonjezera pa izi, anthu ena omwe ali ndi HHT angakumanenso ndi izi:

• Kuchepa kwa magazi m'thupi : Izi zikutanthauza kusowa kwa maselo ofiira m'thupi. Izi zitha kuchitika chifukwa cha kutuluka magazi pafupipafupi.
• Kutuluka magazi m'mimba kapena m'matumbo : Izi sizingawonekere, koma pakhoza kukhala magazi m'chimbudzi kapena chimbudzicho chingakhale chakuda.

Malformations a Arteriovenous (AVMs) ndi zotsatira zake

Anthu omwe ali ndi HHT nthawi zina amatha kukhala ndi aneurysms (AVMs) m'mitsempha ikuluikulu yamagazi. Ma AVM amenewa amatha kufalikira makamaka m'ziwalo monga mapapo, ubongo, msana, ndi chiwindi. Kenako amatha kuyambitsa zizindikiro zosiyanasiyana zokhudzana ndi chiwalo chilichonse.

• Ngati muli ndi ma AVM m'mapapo mwanu:
• Kusanduka kwa mtundu wa buluu pakhungu (makamaka milomo, misomali)
• Kutsokomola magazi (hemoptysis)
• Kumva kutopa mwachangu
• Kuvuta kupuma (kupuma movutikira)
• Ngati muli ndi `(AVM)` muubongo: Izi ndi zomwe zimakhala zoopsa pang'ono. Chifukwa ngati imodzi mwa `(AVM)` izi yaphulika mkati mwa ubongo, zimakhala zoopsa kwambiri.
• Chizungulire
• Masomphenya awiri
• Kugwidwa ndi khunyu
• Matenda ofanana ndi sitiroko amatha kuchitika.
• Ngati muli ndi matenda a chiwindi (AVM):
• Mtima ukhoza kukhala ndi vuto la "kulephera kwa mtima" chifukwa magazi akamadutsa mu "(AVM)" m'chiwindi, mtima umayenera kugwira ntchito molimbika kuti upereke magazi ku thupi lonse.
• Ngati muli ndi ma AVM a msana:
• Kupweteka kwa msana
• Kusamva bwino kapena kutaya kumva m'manja kapena m'mapazi kungachitike.

Chofunika: Ngati ``(AVM)`` izi zaphulika ndi kutuluka magazi, ndi vuto lalikulu kwambiri. Chifukwa chake, ngati muli ndi zizindikiro zilizonse zomwe zatchulidwa pamwambapa, ndikofunikira kwambiri kufunafuna upangiri wa dokotala nthawi yomweyo.

Kodi ana anga nawonso angathe kukhala ndi HHT?

Inde, ngati muli ndi HHT, mwana wanu aliyense ali ndi mwayi wa 50% wolandira matendawa.Inde. Izi zikutanthauza kuti nthawi iliyonse mwana akabadwa, pali mwayi wa 50% kuti mwanayo adzakhale ndi HHT ndipo mwayi wa 50% kuti sadzakhalapo. Zili ngati kuponya ndalama ndikupeza mitu kapena michira.

Kodi HHT imapezedwa bwanji?

Kuyezetsa majini kungachitike kuti kutsimikizire HHT. Koma nthawi zambiri, madokotala amazindikira matendawa kutengera zomwe zimachitika kuchipatala, monga zizindikiro ndi mbiri ya banja. Mukapita kwa dokotala, nthawi zambiri amachita izi:

• Adzakufunsani mafunso atsatanetsatane okhudza mbiri yanu yachipatala (monga nthawi yomwe mwakhala mukutuluka magazi m'mphuno, kangati, komanso mavuto ena omwe mwakhala nawo).
• Funsani za mbiri yachipatala ya banja lanu (makolo, abale, ana) chifukwa izi zimachitika m'mabanja.
• Thupi lanu lidzafufuzidwa (kuti lione ngati pali madontho ofiira kapena ena otero).
• Ngati kuli kofunikira, pitani ku mayeso (monga mitundu ya `(scan)`, `(Endoscopy)`) kuti muwone momwe ziwalo zamkati zilili .

Dokotala angakayikire kapena kudziwa kuti muli ndi HHT ngati muli ndi zizindikiro zitatu zotsatirazi:

• Kutuluka magazi m'mphuno pafupipafupi.
• Kupezeka kwa ma telangiectasias angapo m'malo omwe amapezeka kwambiri pakhungu (monga nkhope, milomo, zala).
• Kupezeka kwa ``telangiectasias'' kapena ``AVM'' m'ziwalo zamkati (monga mapapo, ubongo, m'mimba, m'matumbo) (izi zimapezeka pokhapokha poyesa).
• Kukhala ndi wachibale wapafupi ndi HHT.

Kodi mankhwala a HHT ndi ati?

Mwatsoka, palibe mankhwala a HHT. Komabe, pali mankhwala omwe angathandize kuwongolera zizindikiro, kuchepetsa zizindikiro, komanso kuchepetsa chiopsezo cha mavuto aakulu. Asayansi akugwirabe ntchito kuti apeze mankhwala a HHT, makamaka chifukwa cha chitukuko cha mankhwala oletsa angiogenic.

Ngakhale dokotala wanu amachiza zizindikiro zomwe zilipo kale, iyenso adzayang'ana mavuto aliwonse okhudzana ndi HHT omwe mwina alibe zizindikiro.

Chithandizo chingaphatikizepo izi:

• Chithandizo cha laser (`(Ablation)`): Njira yaying'ono yochitira opaleshoni yomwe imagwiritsa ntchito kuwala kwa laser kuti iletse kutuluka kwa magazi m'malo omwe amatuluka magazi nthawi zambiri (telangiectasias).
• Kuchotsa magazi m'thupi: Njira yaying'ono yochitira opaleshoni yomwe imatseka mtsempha wamagazi womwe umatsogolera kumalo otuluka magazi kapena AVM yomwe ili pachiwopsezo chotuluka magazi.
• Chithandizo cha kuchepa kwa magazi m'thupi: Kupereka mapiritsi achitsulo, ndi kupereka magazi (kuika magazi) ngati pakufunika kutero.
• Kuletsa kutuluka magazi m'mphuno: Gwiritsani ntchito mankhwala monga mafuta odzola ndi saline spray kuti mkati mwa mphuno mukhale ndi chinyezi.
• Kuchotsa ma AVM: Ma AVM ena amatha kuchotsedwa pogwiritsa ntchito radiation therapy kapena opaleshoni.
• Mankhwala oletsa angiogenic: Awa angaperekedwe ngati ma infusions kapena mapiritsi.

Mukhozanso kuonana ndi akatswiri kuti azitha kuchiza machitidwe enaake a thupi, monga chiwindi, mapapo, m'mimba, ndi ubongo.

Kodi HHT ingapewedwe?

Popeza ndi matenda obadwa nawo, palibe njira yopewera HHT kapena kuchepetsa chiopsezo chanu chotenga matendawa. Komabe, ngati makolo anu, abale anu, kapena ana anu ali ndi HHT, ndikofunikira kuuza dokotala wanu. Izi zingakuthandizeni kuzindikira ngati muli ndi vutoli msanga, kuyamba chithandizo msanga, ndikupewa mavuto.

Kodi chiyembekezo cha anthu omwe ali ndi HHT ndi chotani?

Anthu omwe ali ndi HHT amatha kukhala ndi moyo wautali. Komabe, matenda a AVM ndi kutuluka magazi kosalekeza m'mapapo ndi muubongo ndi oopsa ndipo amafunika chithandizo. Ndi chisamaliro choyenera, anthu ambiri amatha kukhala ndi moyo wabwinobwino.

Kodi mungachite chiyani ngati mukutuluka magazi m'mphuno chifukwa cha HHT?

Kutuluka magazi m'mphuno n'kofala kwambiri mu HHT, kotero pali zinthu zingapo zomwe mungachite kuti muwapewe:

• Pewani kugwiritsa ntchito mankhwala ena, makamaka ochepetsa ululu monga aspirin ndi NSAIDs (monga ibuprofen, diclofenac). Izi zimachepetsa magazi kuundana ndikuwonjezera kutuluka kwa magazi. Funsani dokotala musanamwe mankhwala aliwonse.
• Lembani tsiku ndi tsiku. Lembani zakudya zomwe mumadya komanso zochita zomwe mumachita zomwe zimayambitsa kutuluka magazi m'mphuno mwanu. Kenako mutha kupewa zinthu zimenezo.
• Sungani mkati mwa mphuno yanu muli wonyowa komanso wopaka mafuta. Izi zitha kuchitika pogwiritsa ntchito chotenthetsera, kugwiritsa ntchito mafuta odzola omwe dokotala wanu wakupatsani, kapena kugwiritsa ntchito mankhwala opopera m'mphuno. Mphuno youma imayamba kutuluka magazi.

Ndi chiyani chinanso chomwe ndingafunse dokotala wanga chokhudza HHT?

Ngati mwapezeka ndi HHT, ndi bwino kufunsa dokotala mafunso monga awa:

• Kodi banja langa lonse liyenera kuyezedwa izi?
• Kodi ndingathe kusewera masewera? Ndi masewera ati omwe ndi abwino ndi omwe si abwino?
• Kodi pali zinthu zinazake zomwe ndiyenera kupewa?
• Kodi pali mowa, zakudya zapadera, kapena mankhwala ena omwe ndiyenera kupewa?
• Kodi ndingachite chiyani kuti ndipewe kutuluka magazi m'mphuno kapena kuimitsa msanga?
• Kodi ndi bwino kutenga mimba? Kodi pali zinthu zina zapadera zomwe ndiyenera kudziwa?
• Ngati ndituluka magazi, ndi liti pamene ndiyenera kupita kuchipatala nthawi yomweyo?

Pomaliza, zomwe muyenera kukumbukira

HHT ndi matenda a majini omwe nthawi zambiri sapezeka. Zizindikiro zake zimatha kuyambira kutuluka magazi m'mphuno pafupipafupi mpaka mavuto akuluakulu omwe amakhudza ziwalo zosiyanasiyana za thupi. Ngati mukukayikira kuti inuyo kapena wina m'banja mwanu ali ndi HHT, chonde lankhulani ndi dokotala.Kulandira chithandizo msanga kungathandize kupewa mavuto. Kuyezetsa majini kungathandizenso kudziwa ngati achibale ena akhudzidwa ndi vutoli. Kudziwa zambiri ndikofunikira kwambiri pazochitika ngati izi.

HHT , Hereditary Hemorrhagic Telangiectasia, Osler-Weber-Rendu syndrome, mphuno yotuluka magazi, mitsempha yamagazi, matenda a majini, AVM, telangiectasia