Kodi mkodzo wanu ndi wakuda? Kodi mumatopa nthawi zonse? Mwina ndi PNH (Paroxysmal Nocturnal Hemoglobinuria)!

Kodi mumakumananso ndi kusintha kwa mtundu wa mkodzo wanu mukadzuka m'mawa, n’kukhala wofiira, bulauni, kapena nthawi zina wakuda? Kapena mumamva kutopa kwambiri komanso kutopa popanda chifukwa tsiku lonse? Izi zingawoneke ngati zinthu zachilendo zomwe sitiziganizira kwambiri pa moyo wathu watsiku ndi tsiku. Komabe, nthawi zina zizindikiro zazing'onozi zimafunika chisamaliro pang'ono, chifukwa zitha kubisa vuto lachilendo koma lalikulu la thanzi. Lero tikambirana za vuto limodzi lofanana ndi magazi, PNH, kapena `(Paroxysmal Nocturnal Hemoglobinuria)`. Ngakhale dzinali lingamveke lovuta pang'ono, tiyeni timvetse mosavuta.

Kodi PNH ndi chiyani? Tiyeni timvetse mosavuta

Ndiye, kodi ``Paroxysmal Nocturnal Hemoglobinuria'' kapena PNH ndi chiyani? Ndi matenda osowa kwambiri a magazi. Ngati muyang'ana tanthauzo la dzinalo:

• "Paroxysmal" amatanthauza mwadzidzidzi, zomwe zimachitika mwadzidzidzi.
• "Usiku" amatanthauza kugwirizana ndi usiku.
• "Hemoglobinuria" ndi kupezeka kwa puloteni m'magazi yotchedwa ``Hemoglobin``` mu mkodzo. ``Hemoglobin`` iyi ndi yomwe imapangitsa magazi athu kuoneka ofiira. Choncho, izi zikatulutsidwa ndi mkodzo, mkodzo umakhala wakuda.

Mwachidule, PNH ndi vuto limene chitetezo cha mthupi lanu chimaukira ndikuwononga maselo ofiira a magazi anu . Madokotala amatcha izi "Hemolysis" kapena kuwonongeka kwa maselo ofiira a magazi. Chimodzi mwa zizindikiro zazikulu za izi ndi mkodzo wakuda, wofiira/wofiirira mukakodza usiku kapena m'mawa kwambiri.

Komabe, mkodzo wakuda uwu ndi chizindikiro chimodzi chokha cha PNH. Ngati vutoli silinachiritsidwe bwino, lingayambitse matenda aakulu monga kuchepa kwa magazi m'thupi , matenda osatha a impso , kapena magazi kuundana m'mitsempha yamagazi . Mwamwayi, tsopano pali mankhwala othandiza omwe amaletsa PNH kuwononga maselo amagazi.

Ndani angapange izi?

Matenda a PNH ndi matenda osowa kwambiri. Malinga ndi ziwerengero, anthu pafupifupi 6 pa anthu 1 miliyoni amapezeka ndi matendawa chaka chilichonse. Nthawi zambiri, matendawa amapezeka mwa amuna ndi akazi azaka zapakati pa 30 ndi 40. Komabe, akuti akazi ali ndi mwayi wochuluka wopeza matenda a PNH kuposa amuna.

Chinthu china n’chakuti nthawi zina anthu omwe ali ndi matenda ena a m’mafupa, mwachitsanzo, matenda monga `(Aplastic Anemia)` kapena `(Myelodysplastic Syndrome)`, amathanso kudwala PNH pambuyo pake.

Kodi kusiyana pakati pa Hemoglobinuria ndi Hematuria ndi kotani?

Mumasokonezeka pang'ono mukamva mawu awiriwa ( chisokonezo)- chisokonezo, ichi ndi Chitamil, ndiyenera kuchipewa. Ndiloleni ndibwerezenso: Mungasokonezeke pang'ono mukamva mawu awiriwa.) Mungasokonezeke pang'ono mukamva mawu awiriwa. Chifukwa m'zochitika zonse ziwiri, chomwe chimachitika ndichakuti pamakhala ngati magazi mumkodzo.

• Kutupa kwa magazi (hematuria) ndi kupezeka kwa maselo ofiira a magazi mu mkodzo wanu.
• Hemoglobinuria (ichi ndi chomwe timachiwona mu PNH yathu) ndi kupezeka kwa puloteni yotchedwa hemoglobin mu mkodzo. Maselo ofiira amagazi akamawonongeka, hemoglobin iyi imatulutsidwa ndikuwonjezeredwa mu mkodzo.

Mwachidule, chimodzi chimapita ku maselo onse, ndipo china chimapita ku chinthu chofiira chomwe chimatuluka maselo akawonongeka.

Kodi n’chiyani chimayambitsa matenda a PNH? N’chifukwa chiyani zimenezi zimachitika?

Chifukwa chachikulu cha PNH ndi vuto la jini m'thupi lathu. Vutoli limakhudza magwiridwe antchito a mitundu iwiri ya maselo amagazi, maselo ofiira amagazi ndi ma platelet. Izi zimayambitsa zochitika zambiri zomwe zimayambitsa mavuto motsatizana, nthawi zina ngakhale matenda oopsa.

Zonsezi zimayambira mu fupa lathu. Mukudziwa, fupa la mafupa lili ngati fakitale ya magazi m'thupi lathu. Apa ndi pomwe maselo oyambira amapangidwa. Maselo oyambira awa pambuyo pake amasanduka maselo ofiira a magazi, maselo oyera a magazi, ndi ma platelet.

Mu PNH, kusintha kwa majini otchedwa ``PIGA`` kumachitika mu selo limodzi loyambira. Selo loyambira losinthikali limagawikana ndikupanga maselo oyambira omwe ali ndi vuto lofanana. Maselo oyambira omwe ali ndi vuto limeneli amapanga maselo ofiira a magazi ndi ma platelet omwe ali ndi vuto.

Kodi kusintha kwa majini kumeneku kumakhudza bwanji maselo ofiira a magazi?

Taganizirani izi motere: chitetezo chathu cha mthupi chili ngati gulu lankhondo la dziko lathu. Limodzi mwa mphamvu zake zapadera limatchedwa 'Complement System' . Ntchito yawo ndi kulimbana ndi majeremusi ndi matenda omwe amalowa m'thupi. Komabe, nthawi zina mapuloteni omwe ali mu '(Complement System)' iyi amayamba kuukira maselo athu ofiira athanzi mwangozi.

Selo lofiira labwinobwino komanso lathanzi limakhala ndi mapuloteni oteteza ngati chishango kuti litetezedwe ku ziwopsezo zotere. Jini ya `PIGA` yomwe yatchulidwa kale imathandiza kupanga chishango choteteza ichi. Chifukwa chake, jini ya `PIGA` ikasinthidwa, silingathe kupanga chishango choteteza ichi bwino. Kenako, nazi zomwe zimachitika:

1. Kuukira ndi Kuwonongeka: Pamene "Dongosolo Lowonjezera" lanu likuukira maselo ofiira a magazi opanda chitetezo, maselo amenewo amasweka mosavuta. "Hemoglobin" yonse yomwe ili mkati mwa maselo ofiira a magazi - kutanthauza puloteni yofiira yomwe imanyamula mpweya - imatulutsidwa m'magazi. Madokotala amatcha izi "Hemoglobin Yaulere" .

2. Kulephera kwa makina oyeretsera:Kawirikawiri, matupi athu ali ndi chinthu chotchedwa ``Haptoglobin'' chomwe chimasunga ndikuchotsa hemoglobin yaulere iyi. Koma mu PNH, kuchuluka kwa maselo ofiira amagazi omwe amasweka kumakhala kwakukulu kwambiri kotero kuti gulu loyeretsali silingathe kupitiliza.

3. Kusowa kwa Nitric Oxide: Thupi limagwiritsa ntchito njira ina yowongolera hemoglobin wochulukayu, ndipo ndi mankhwala otchedwa nitric oxide. Koma iyinso imatha msanga. Thupi lanu likapanda nitric oxide yokwanira, mwadzidzidzi mumatha kumva kupweteka kwambiri m'minofu ya m'mimba, m'mero ​​(chubu chomwe chimayenda kuchokera pakhosi kupita m'mimba), komanso m'minofu ya msana.

4. Kuchepa kwa magazi m'thupi: Kumbali imodzi, maselo ofiira akawonongeka, mbali inayi, pamakhala kupanikizika kwakukulu pa mafupa kuti apange maselo ofiira atsopano. Komabe, akapanda kupangidwa mofanana ndi momwe amawonongekera, chiwerengero cha maselo ofiira m'thupi chimachepa. Ichi ndi chomwe timachitcha kuchepa kwa magazi m'thupi.

5. Kuwonongeka kwa impso: Hemoglobin yochulukirapoyi imawononganso impso. Akuti anthu omwe ali ndi PNH ali ndi chiopsezo chowonjezeka kasanu ndi kamodzi chotenga matenda a impso osatha .

6. Mkodzo wakuda: Pomaliza, hemoglobin yaulere iyi imakhudzanso mkodzo. Anthu ambiri omwe ali ndi PNH amatha kuwona mkodzo wakuda akamakodza usiku kapena m'mawa kwambiri. Izi zili choncho chifukwa kuchuluka kwa hemoglobin mu mkodzo kumawonjezeka, zomwe zimapangitsa mkodzo kukhala wakuda.

Mwachidule, chifukwa cha vuto la jini ya `PIGA`, chitetezo cha maselo ofiira a m'magazi chimatayika, ndipo chitetezo cha mthupi chimawonongeka, zomwe zimayambitsa mavuto ambiri.

Kodi kusintha kwa majini kumeneku kumakhudza bwanji ma platelet?

Ma platelets ndi mtundu wina wofunikira wa maselo m'magazi mwathu. Amathandiza kuletsa kutuluka kwa magazi mwa kupanga magazi oundana. Kusintha kwa majini a PIGA kumakhudza maselo oyambira omwe amapanga maselo ofiira a magazi komanso maselo oyambira omwe amapanga ma platelets.

Izi zimapangitsa kuti ma platelet omwe ali ndi vuto apangitse magazi kuundana kwambiri kuposa masiku onse . Izi zikutanthauza kuti magazi kuundana amatha kupangika m'malo omwe safunikira, mkati mwa mitsempha yamagazi. Izi zimatchedwa `(Thrombosis)` (thrombosis). Izi ndi zoopsa kwambiri, chifukwa ngati magazi kuundanawa apita ku ubongo, mtima, kapena mapapo, akhoza kukhala pachiwopsezo chachikulu.

Kodi zizindikiro za PNH ndi ziti?

Ngakhale kuti dzina lakuti PNH limachokera ku chizindikiro chimodzi (mkodzo wakuda), anthu ambiri omwe ali ndi PNH amayamba kuonana ndi dokotala chifukwa cha kutopa kwambiri komanso kosalekeza komwe kumawapangitsa kukhala ovuta kuchita ntchito zawo za tsiku ndi tsiku . Ichi ndiye chizindikiro chofala kwambiri.

Zizindikiro zina zingaphatikizepo:

• Kuvuta kupuma (`(Dyspnea)`)
• Mavuto a impso (monga kuchepa kwa kukodza, kutupa kwa miyendo)
• Kuvuta kumeza chakudya (Dysphagia)
• Kutsekeka kwa Mitsempha ya M'mimba
• Kupweteka m'mimba
• Kupweteka kwa msana
• Kulephera kugonana kwa amuna (`(Kulephera Kugonana)`)

Ngati muli ndi chimodzi mwa zizindikiro izi, makamaka pamodzi ndi kusintha kwa mtundu wa mkodzo, ndi bwino kufunsa upangiri wa dokotala.

Kodi madokotala amazindikira bwanji PNH? (Kuzindikira)

Mukapita kwa dokotala, adzakufunsani za zizindikiro zanu ndikukuyesani. Kenako, ngati akukayikira kuti muli ndi PNH, angakuyeseni mayeso kuti atsimikizire.

Zina mwa izi ndi izi:

• Kuchuluka kwa Magazi Okwanira (CBC w/diff): Izi zimayang'ana mavuto ena a magazi, monga kuchepa kwa magazi m'thupi ndi kuchepa kwa ma platelet (thrombocytopenia).
• Gawo Loyambira la Kagayidwe kachakudya (BMP): Izi zimayang'ana momwe impso zimagwirira ntchito komanso zizindikiro za matenda a impso osatha.
• Kusanthula mkodzo: Izi zimatha kuzindikira zinthu monga kukhalapo kwa hemoglobin mu mkodzo (Hemoglobinuria) ndi kuchuluka kwa chitsulo m'thupi (Hemosiderosis).
• Chiwerengero cha ma reticulocyte: Ma reticulocyte ndi maselo ofiira a m'magazi osakhwima. Awa amawerengedwa kuti awone ngati mafuta a m'mafupa akupanga maselo ofiira a m'magazi athanzi.
• Kuyesa kwa Haptoglobin: Puloteni iyi imagwira ntchito yochotsa zinyalala m'maselo ofiira osweka. Kuchepa kwa magazi kungasonyeze kuwonongeka kwakukulu kwa maselo ofiira a m'magazi.
• Kuyesa kwa Lactate Dehydrogenase (LDH): LDH ndi enzyme yomwe imapezeka mkati mwa maselo ofiira a magazi. Kuchuluka kwa izi m'magazi kumasonyeza kuti maselo ofiira amagazi akuwonongedwa.
• Kuyesa kwa Ntchito ya Chiwindi: Kuyesa kumeneku kumayesa kuchuluka kwa bilirubin pamene maselo ofiira amagazi asweka.

Kutengera ndi zotsatira za mayesowa, ngati dokotala ali ndi zokayikitsa zina zokhudza PNH, akhoza kuyitanitsa mayeso apadera a magazi otchedwa ``Flow Cytometry.'' Ichi ndi chomwe chingathe kuzindikira molondola maselo ofiira a magazi omwe ali ndi PNH.

Kodi mankhwala a PNH ndi otani?

Kale, PNH inkaonedwa ngati matenda oopsa komanso ovuta kuchiza. Panthawiyo, chithandizo chachikulu chinali kuikidwa magazi nthawi zonse kuti athetse kuchepa kwa magazi m'thupi. Komabe, anthu omwe ali ndi PNH nthawi zambiri amakhala ndi moyo zaka 10 mpaka 22 atapezeka ndi matendawa. Njira yokhayo yochiritsira PNH kwathunthu inali kuchitidwa opaleshoni yoika maselo oyamba a allogeneic . Ndipo si chinthu chomwe aliyense angachite.

Koma, zinthu zasintha kwambiri tsopano!

Mwamwayi, tsopano pali njira zothandiza kwambiri zochiritsira matenda a PNH. Izi zimatchedwa kuti zoletsa zowonjezera.Chomwe mankhwalawa amachita ndikuletsa ``Complement System`` kuti isaukire ndikuwononga maselo ofiira a magazi.

Kafukufuku wasonyeza kuti odwala a PNH omwe amalandira chithandizo chatsopanochi akhoza kukhala ndi moyo wabwinobwino, monga momwe amachitira munthu wopanda PNH. Ichi ndi chipambano chachikulu!

Kodi pali zotsatirapo zilizonse za mankhwalawa?

Inde, mankhwala awa a `(Complement Inhibitors)` akhoza kukhala ndi zotsatirapo zina. Mwachitsanzo, anthu ena amatha kukhala ndi zinthu monga mutu ndi nseru. Komabe, nkhani yabwino ndi yakuti zotsatirapo zambirizi zimatha patangopita masiku ochepa kuchokera pamene chithandizo chayamba. Dokotala wanu adzakuuzani zambiri za izi.

Kodi matenda a PNH angapewedwe? Kodi angachiritsidwe?

Popeza PNH imayamba chifukwa cha kusintha kwa majini, palibe njira yopewera.

Ponena za chithandizo, mankhwala atsopano a ``Complement Inhibitor`` amatha kuletsa kuwonongeka kwa maselo ofiira a magazi ndi ma platelet omwe amayambitsidwa ndi PNH. Izi zikutanthauza kuti matendawa amatha kulamulidwa. Odwala ambiri a PNH angafunike chithandizo china cha matenda monga kuchepa kwa magazi m'thupi. Monga tanenera kale, ngakhale kuti ``Stem Cell Transplant`` ndi yotheka, ndi mankhwala ovuta kwambiri. Ndi mankhwala omwe alipo pano, matendawa amatha kuthandizidwa bwino ndipo anthu amatha kukhala ndi moyo wabwinobwino.

Kodi mumadzisamalira bwanji ngati munthu amene ali ndi PNH?

Ngakhale mutalandira chithandizo, ndikofunikira kwambiri kuti muchitepo kanthu kuti mupewe magazi kuundana ngati muli ndi PNH. Mwachitsanzo:

• Ngati munthu amene ali ndi PNH akuyenera kuchitidwa opaleshoni , pamakhala chiopsezo cha magazi kuundana komanso kutuluka magazi ambiri panthawi ya opaleshoniyi. Chifukwa chake, madokotala amasamala kwambiri pa izi.
• Kutenga mimba kumabweretsa mavuto pa thanzi la amayi omwe ali ndi PNH, kwa iwo eni komanso kwa mwana wawo wosabadwa. Chifukwa chake, ndikofunikira kuyang'aniridwa ndi katswiri musanayambe komanso nthawi yonse yomwe muli ndi pakati.

Dokotala wanu adzakupatsani malangizo pa kusintha komwe muyenera kusintha pa moyo wanu, zinthu zomwe muyenera kusamala nazo ( samalani , ichi ndi Sanskrit/Malayalam, ndiyenera kupewa. Ndiloleni ndinenenso kuti: kusintha komwe muyenera kusintha pa moyo wanu, zinthu zomwe muyenera kusamala nazo.) kusintha komwe muyenera kusintha pa moyo wanu, zinthu zomwe muyenera kusamala nazo. Ndikofunikira kwambiri kutsatira malangizo amenewo.

Pomaliza, chinthu chofunikira kwambiri (Uthenga Wopita Nawo Kunyumba)

PNH, kapena ``Paroxysmal Nocturnal Hemoglobinuria'', ndi matenda osowa koma omwe angakhale oopsa m'magazi. Pafupifupi zaka 20 zapitazo, odwala anali ndi njira zochepa zochiritsira komanso tsogolo losadziwika bwino.

Koma masiku ano, zinthu zasintha kwambiri! Chifukwa cha mankhwala atsopano amakono, anthu omwe ali ndi PNH amatha kusunga maselo awo amagazi, kupewa mavuto aakulu, komanso kukhala ndi moyo wathanzi komanso wokwanira.

Ngati mwapezeka ndi PNH, musachite mantha. Dokotala wanu adzakufotokozerani njira zabwino zochiritsira PNH. Ngakhale kuti chithandizo sichichiritsa PNH kwathunthu, chingathandize kupewa mavuto aakulu azaumoyo omwe amayamba chifukwa cha matendawa ndikukuthandizani kukhala ndi moyo wathanzi. Chofunika kwambiri ndikufunsa upangiri wa dokotala mwachangu ngati muli ndi zizindikiro ndikutsatira chithandizocho monga momwe mwalangizidwira.

PNH , Paroxysmal Nocturnal Hemoglobinuria, Matenda a Magazi, Hemoglobinuria, Kuchepa kwa Magazi, Kutsekeka kwa Magazi, Matenda a Impso