A Review of Pyruvate Kinase: Chimwe Chimwe Chikonzero cheAnemia
Kudzidza iwe kana mudiwa wako ane kupererwa kwePK kunogona kutyisa. Iwe unganetseka, ndakawana sei izvi? Kana kuti nzira dzekurapa ndeipi? Tariro, ichi chidzidzo chekutanga chechiitiko chichapindura mamwe emibvunzo iyi.
Pyruvate kinase (PK) kushayikwa isati isati yawana nhaka yeanemia. Pyruvate kinase is enzyme, kana kuti chikemikari, inowanikwa mukati mako masero matsvuku eropa anodiwa kuita simba.
Pasina iyi enzyme inokosha, serofu yeropa inoputsika zvakanyanya nokukurumidza. Iyi kuregererwa kwemasero yeropa inonzi hemolysis uye kuperevedza kwePK inotsanangurwa sehemolytic anemia.
Vanhu vane urombo hwePK vanoberekwa nemamiriro aya. Izvo zvakatora nhaka yezvinyorwa zvekuzvidzivirira zvinoreva kuti vabereki vose vanofanirwa kunge vari vatakuri vehutano. Kana mubereki mumwe nomumwe ari mutakuri wekushaya PK, vane 1 mu4 mukana wekuva nemwana ane urombo hwePK.
Zviratidzo
Zviratidzo zvekushaya kwePK zvinogona kutanga muhupenyu. Hazvisi zvisingazivikanwi kuti vana vachangobva kuberekwa nePK vakashaya kukura jaundice (yellowing yeganda) pazuva rekutanga rehupenyu. Iyi jaundice inowanzova yakaoma kudarika physiologic jaundice kuti mitsva inowanzokura pakati pezuva 2 ne5 yehupenyu. Izwi rechiremba re jaundice ndiro hyperbilirubinemia. Bilirubin inonzi pigment inowanikwa mumasero matsvuku eropa. Kuparadzwa kwakakurumidza kwemasero matsvuku eropa mukushaiwa kwePK kunowedzera kuwanda kwebilirubin inopararira.
Nenzira yakanaka, iyi jaundice inogona kurapwa ne phototherapy (chiedza chebhuruu chinoshandiswa kuputsa pigment) uye / kana kuwedzerwa ropa.
Kana zvisina kudaro, vanhu vane kupererwa kwePK vane zviratidzo zveanemia : ganda rinenge riripo, kuneta kana kuti kuneta, kukurumidza kwemwoyo, kana kufema kwepfufu. Vamwe vanhu vane urombo hwePK vachakudziridza spleen yakakura, inonzi splenomegaly.
Vamwe vanhu vane maitiro akaoma ekushayikwa kwePK vanogona kuonekwa kuti mushure mehupenyu sezvo vangave vashoma zviratidzo.
Kuziva
Zvakafanana nedzimwe mhando dzeanemia, iyo yakazara ropa kuverenga (CBC) inopa ruzivo rwekutanga. Iyo hoemia inobatanidzwa nehutu hwePK inogona kuva yakakonzera kukonzerwa neawa yakanyanya hemoglobin uye / kana hematocrit. Kungofanana nedzimwe hemolytic anemias, reticulocyte count (yakangobva kuitwa masero matsvuku eropa) ichasimudzirwa, zvichiratidza kuwedzera kwemasero matsvuku ekugadzirwa kweropa.
Kushaikwa kwePK kunogona kusimbiswa nokutumira basa reropa kumakwikwi emakwenzi kuti aone hutsika hwePK mukati memasero matsvuku eropa. Zvimwewo, mazamu anoongorora maitiro ari kutarisa kuongorora kuongorora kwechiitiko ichi.
Matambudziko
Sezvakakurukurwa kare, kuparara kwemasero matsvuku eropa kunokonzera kuwedzera kwema bilirubin. Vacheche vane urombo hwePK vangada kurapwa ne phototherapy. Gare gare muupenyu, yakawedzera bilirubin inogona kuvaka sludge mu gallbladder uye pakupedzisira inotungamirira kukurudzirwa kwematombo .
Vanhu vane urombo hwePK vanogonawo kukudziridza simbi kuwandisa . Izvi zvinowanzoitika kune vanhu vanoda kuwedzerwa ropa nguva dzose kuti vabate chirwere chavo chakanyanya kuora ropa. Zvisinei, izvi zvinogona kuitika kune vanhu vasina mari inokosha yekuwedzerwa ropa.
Kune mishonga inogona kubatsira muviri kuti ubvise humwe hwehuni hunonzi chelators.
Kufanana nevanhu vose vane hemolytic anemias, vanhu vane kupererwa kwePK vari pangozi yekutapukira kweplastiki . Izvi zvinokonzerwa nechirwere chinonzi parvovirus B19 (chinokonzera Firi Chirwere muvana). Iyo parvovirus inodzivirira marora emapfupa kubva pakuita masero matsvuku eropa kwemazuva manomwe kusvika ku10. Pasina kukwanisa kubvisa masero matsvuku eropa akaparadzwa nokukurumidza, chirwere chakanyanya kuora ropa chinogona kukura uye kuwedzerwa kunogona kudiwa kusvikira zvigadzirwa zvadzokera kune zvakajairika.
Iyo chirwere chakakomba chinokonzerwa nekushayikwa kwePK chinogona kuitika kuvana kunyange vasati vaberekwa.
Izvi zvinonzi hydrous fetalis. Hydrops fetalis ishoko rinowanzoshandiswa kurondedzera kuwanikwa kune ultrasound. Hemolytic anemias yakadai sePK inoshaya, inogona kukonzera ichi chinetso. Zvichienderana nekuoma, izvi zvinogona kurapwa ne intrauterine (kuburikidza nechibereko) kuwedzerwa ku fetus.
Kurapa
Vamwe vanachiremba vangakukurudzira kuti utore folic acid zuva nezuva. Folic acid ndiyo vitamini inofanirwa kuita masero matsvuku eropa. Nenzira yakanaka, muUnited States, zvakawanda zvezvokudya zvedu zvakasimbiswa ne folic acid zvakare.
Kazhinji, chirwere chinokonzera chirwere chakakomba chinorapwa nekuwedzerwa ropa. Vamwe vanhu vane urombo hwePK vanoda kuwedzerwa ropa rimwe nerimwe kwemavhiki matatu kusvika kuchina. Vamwe vanhu vangangoda kuwedzerwa ropa pane imwe nguva panguva dzekurwara kana kushungurudzika, asi vamwe vangave vasingadi kuwedzerwa ropa.
Kana zviyero zvebilirubin zvichisvika pamatambudziko anokosha mumucheche, kuwedzerwa kuwedzerwa kunogona kudiwa. Muchiitiko ichi ropa rinobviswa kubva mucheche (uyewo kubvisa bilirubin) uye ropa iri rinotsiviwa neropa reropa. Izvi zvinoshanda zvikuru pakubvisa zvakanyanya bilirubin kubva muropa.
Zvipfeko zvinokonzerwa nokushayikwa kwePK zvingada kubviswa necholecystectomy, kubviswa kwegorovladder. Splenectomy , kubviswa kushandiswa kwepleen, zvinogona kuitwa mukuedza kuderedza kuoma kweropa, kunyanya kune vanhu vanoda kuwedzerwa ropa. Zvinosuruvarisa, splenectomy haishandi kune munhu wose ane PK inoshaya.
Shoko Rinobva
Kudzidza iwe kana mwana wako ane urombo hwePK hunogona kukumanikidza. Nenzira yakanaka kune nzira dzokurapa uye vanhu vakawanda vane kupererwa kwePK vanoita zvakanaka. Kune hutsva hunoenderera mberi mukushayikwa kwePK uye tarisiro kune huchava nemamwe maitiro ekurapa munguva yemberi.