Ukubukezwa kwe-Pyruvate Kinase: Isizathu Esivamile Sokwelapha Kwegazi
Ukufunda wena noma othandekayo wakho kunenkinga ye-PK ingasabisa. Ungase uzibuze, ngithole kanjani lokhu? Noma yiziphi izinketho zokwelapha? Sethemba, lokhu kubuyekezwa okuyisisekelo kwalesi simo kuzophendula eminye yale mibuzo.
Ukuntuleka kwe-Pyruvate kinase (PK) kuyindlela engavamile yokuthola i-anemia. I-pyruvate kinase iyi-enzyme, noma amakhemikhali, atholakale ngaphakathi kwamaseli akho abomvu egazi afanele ukwenza amandla.
Ngaphandle kwalesi sivivinyo esibalulekile, iseli elibomvu legazi liyehla ngokushesha. Ukuchithwa kwe-cell cell ebomvu kubizwa ngokuthi i-hemolysis kanye nokuntuleka kwe-PK kuthiwa yi-anemia ye-hemolytic.
Abantu abanenkinga ye-PK bazalwa nalesi simo. Izuze ifa elilodwa le-autosomal pattern ethandanayo esho ukuthi bobabili abazali kufanele babe yizinkampani zesifo. Uma umzali ngamunye ethwala umthamo we-PK, bane-1 ku-4 amathuba okuba nengane ene-PK yokuntula.
Izimpawu
Izimpawu zokuntuleka kwe-PK zingaqala ekuqaleni kokuphila. Kuyinto engavamile ukuba abantwana abasha abane-PK bakwazi ukuthuthukisa i-jaundice (ukuphuzila kwesikhumba) ngosuku lokuqala lokuphila. Le-jaundice ivame kakhulu kune-jaundice ye-physiologic ukuthi izinsana zivame ukukhula phakathi kwelanga 2 no-5 lokuphila. Igama lezokwelapha nge-jaundice yi-hyperbilirubinemia. I-Bilirubin i-pigment etholakala kuma-red cells. Ukubhujiswa okusheshayo kwamangqamuzana abomvu e-red ekwenzeni i-PK kwandisa inani le-bilirubin ekusakazeni.
Ngenhlanhla, le-jaundice ingaphathwa nge-phototherapy (ukukhanya okwesibhakabhaka okusetshenziselwa ukudiliza i-pigment) kanye / noma ukumpontshelwa igazi.
Ngaphandle kwalokho, abantu abane-PK abangenayo izimpawu ze- anemia : isikhumba esilahlekile, ukukhathala noma ukhathala, izinga lokushaya kwenhliziyo ngokushesha, noma ukuphefumula okuncane. Abanye abantu abanokukhubazeka kwe-PK bazokwakha ipule ekhulisiwe, ebizwa ngokuthi i-splenomegaly.
Abanye abantu abanezinhlobo ezincane zokuntuleka kwe-PK bangase batholakale kamuva ekuphileni ngoba kungenzeka ukuthi bangabonakaliswa izimpawu.
Ukuxilongwa
Ngokufanayo nezinye izinhlobo ze-anemia, inani eliphelele legazi (CBC) linikeza inkomba yokuqala. I-anemia ehlobene nokuntuleka kwe-PK ingaba nzima kakhulu ngenxa ye-hemoglobin ephansi kakhulu kanye / noma i-hematocrit. Njengamanye ama-hemolytic anemias, isibalo se-reticulocyte (esanda kwenziwa amangqamuzana egazi abomvu) sizophakanyiswa, sibonise ukukhiqizwa kwamaseli obomvu okwandayo.
Ukuntuleka kwe-PK kungaqinisekiswa ngokuthumela umsebenzi wegazi kumalabhu akhethekile ukuthola izinga eliphansi le-PK ngaphakathi kwamaseli abomvu egazi. Ngaphandle kwalokho, izivivinyo zofuzo ezibheke ukuguqulwa kwezinto eziphilayo ziba ukuhlolwa kokujwayelekile kokuxilongwa kwalesi simo.
Izinkinga
Njengoba kubuyekezwe ekuqaleni, ukwehla kwamaseli obomvu kubangela amazinga akhulayo we-bilirubin. Izinsana ezinenkinga ye-PK zingadinga ukwelashwa nge-phototherapy. Kamuva empilweni, ukwanda kwe-bilirubin kungakha umsila endaweni ye-gallbladder futhi ekugcineni kuholele ekuthuthukiseni kwamatshe .
Abantu abanenkinga ye-PK nabo bangahlakulela inqwaba ye- iron . Lezi zinkinga zivame kakhulu kubantu abadinga ukumpontshelwa igazi njalo ukuze baphathe i-anemia yabo enamandla. Noma kunjalo, lokhu kungahle kwenzeke kubantu abangenawo isidingo esikhulu sokumpontshelwa igazi.
Kunezidakamizwa ezingasiza umzimba ukulahla insimbi eyengeziwe ebizwa ngokuthi i-chelators.
Njengabo bonke abantu abane-anemia ye-hemolytic, abantu abanokukhubazeka kwe-PK basengozini yokubhekana nesimo sezinsizakalo ezingapheli . Lokhu kubangelwa ukutheleleka kwe-parvovirus B19 (okubangela izifo eziyisihlanu ezinganeni). I-parvovirus ivimbela umthambo wethambo ekwenzeni amangqamuzana amasha wegazi obomvu izinsuku ezingu-7 kuya kwezingu-10. Ngaphandle kokukwazi ukushintsha amangqamuzana egazi obomvu asheshe abhubhise, i-anemia enamandla ingathuthuka futhi i-transfusion ingadingeka kuze kube yilapho ukukhiqizwa kubuyela ekujwayelekile.
I-anemia enamandla ehambisana nokuntuleka kwe-PK ingenzeka ezinganeni ngisho nangaphambi kokuba zizalwe.
Lesi simo sibizwa ngokuthi i-hydrous fetalis. Ama-Hydrops fetalis yigama elivamile elisetshenziselwa ukuchaza okufundwayo kwi-ultrasound. Ama-hemolytic anemias afana nokuntuleka kwe-PK, angabangela le nkinga. Ngokuya ngobukhulu, lokhu kungaphathwa nge-intrauterine (ngokusebenzisa isisu) ukumpontshelwa embuseni.
Ukwelapha
Abanye odokotela bangakutshela ukuba uthathe i-folic acid nsuku zonke. I-folic acid i-vitamin edingekayo yokwenza amangqamuzana amasha wegazi elibomvu. Ngenhlanhla, e-United States, ukudla kwethu okuningi kuqiniswe nge-folic acid.
Ngokuvamile, i-anemia enamandla iphathwa ngokumpompela igazi. Abanye abantu abanesifo se-PK badinga ukumpontshelwa njalo ngemva kwamasonto amathathu kuya kwangu-4. Abanye abantu bangase badinga kuphela ukumpontshelwa ngezikhathi ezithile ngezikhathi zokugula noma ukucindezeleka, kodwa abanye bangalokothi badinge ukumpontshelwa igazi.
Uma amazinga e-bilirubin afinyelela emazingeni aphansi ezinganeni ezisanda kuzalwa, kungadingeka ukuthi kusetshenziswe igazi lokushintshaniswa. Kule nqubo igazi likhishwa usana (futhi lisusa i-bilirubin) futhi legazi ithathelwa igazi elimpontshelwa igazi. Le nqubo iphumelela kakhulu ekususeni i-bilirubin ngokweqile egazini.
Ama-gallstones abangelwa ukulahlekelwa kwe-PK angadinga ukususwa nge-cholecystectomy, ukususwa kokuhlinzwa kwe-gallbladder. I-splenectomy , ukususwa kokuhlinzwa kwe-spleen, kungenzeka kwenziwe ngomzamo wokunciphisa ubukhulu be-anemia, ikakhulukazi kubantu abadinga ukumpontshelwa njalo. Ngeshwa, i-splenectomy ayisebenzi kuwo wonke umuntu onenkinga ye-PK.
Izwi elivela
Ukufunda wena noma ingane yakho inokukhubazeka kwe-PK kungakusiza. Ngenhlanhla kunezinketho zokwelashwa kanti abantu abaningi abane-PK abakwazi kahle. Kukhona ucwaningo oluqhubekayo lokuntuleka kwe-PK futhi ngethemba lokuthi kuzoba nokunye ukhetho lwezokwelapha esikhathini esizayo.