Ukuhlaziywa kwe-Pyruvate Kinase: Isizathu esiqhelekileyo se-Anemia
Ukufunda wena okanye wakho umthandayo unako ukukhubazeka kwe-PK kunokusongela. Umele uzibuze, ndifumene njani le nto? Okanye ziziphi iindlela zokonyango? Ndiyathemba, oku kuhlaziywa okusisiseko kwimeko kuya kuphendula le mibuzo.
Inkunkuma yePyruvate kinase (PK) yintlobo engavumelekanga yokuzuza i-anemia. I-pyruvate kinase iyinzyzy, okanye ikhemikhali, efunyenwe kwiiseli zakho ezibomvu zegazi ezifunekayo ukwenza amandla.
Ngaphandle kwale nzymo ebalulekileyo, iseli elibomvu legazi lihla ngokukhawuleza. Ukutshatyalaliswa kweesibomvu segazi kuthiwa yi-hemolysis kunye nokulahleka kwe-PK kubalwa njenge-anemia ye-hemolytic.
Abantu abanokukhubazeka kwePK bazalwe nale mqathango. Ifa eliyifumene le-autosomal pattern excessive (ie autosomal pattern pattern) ebonisa ukuba bobabini abazali kufuneka babe ngabathwali be-disorder. Ukuba ngamnye umzali ungumthwali we-PK ukusilela, bane-1 kwithuba eli-4 lokuba nomntwana onokukhubazeka kwe-PK.
Iimpawu
Iimpawu zokunqongophala kwe-PK zingaqalisa ekuqaleni kwexesha. Akuqhelekanga kwiintsana ezinokukhubazeka kwe-PK ukuphuhlisa i-jaundice (ukuphuka okwephuzi) ngosuku lokuqala lokuphila. Le jaundice idla ngokugqithiseleyo kune-jaologic ye-physiologic ukuba iintsana zikhula ngokukhawuleza phakathi komhla wesi-2 no-5 wobomi. Ixesha lonyango le-jaundice li-hyperbilirubinemia. I-Bilirubin i-pigment etholakala kwiiseli ezibomvu zegazi. Ukutshatyalaliswa ngokukhawuleza kweeseli ezibomvu zegazi kwi-PK ukulahleka kwandisa inani le-bilirubin.
Ngethamsanqa, le ntlupheko ingaphathwa nge-phototherapy (ukukhanya okwesibhakabhaka esetyenziselwa ukuphula i-pigment) kunye / okanye ukumpontshelwa igazi.
Ngaphandle koko, abantu abane-PK ukusilela baneempawu ze- anemia : isikhumba esiluhlaza, ukukhathala okanye ukukhathala, izinga lentliziyo ngokukhawuleza, okanye ukuphefumula okufutshane. Abanye abantu abanokukhubazeka kwe-PK baya kuphuhlisa ipeni ekhulisiweyo, ebizwa ngokuba yi-splenomegaly.
Abanye abantu abaneentlobo ezintle ze-PK ukusilela banokufunyanwa kamva ebomini njengoko banokuba neempawu ezimbalwa.
Ukuxilongwa
Ngokufanayo nezinye iifom ye-anemia, inani elipheleleyo legazi (CBC) linika inkcazelo yokuqala. Ukukhulelwa kwegazi okunxulumene nokusilela kwe-PK kunokuba nzima kakhulu kubangelwa i-hemoglobin ephantsi kakhulu kunye / okanye i-hematocrit. Njengamanye ama-hemolytic anemias, inani le-reticulocyte (esandul 'ukwenziwa ngamaseli obomvu asanda kufakwa) luya kuphakanyiswa, lubonise ukunyuka kweefom zegazi ebomvu.
Ukusilela kwe-PK kungaqinisekiswa ngokuthumela umsebenzi wegazi kumabhanki okhethekileyo ukufumana izinga eliphantsi le-PK ngaphakathi kweeseli ezibomvu zegazi. Ngaphandle koko, iimvavanyo zofuzo ezijonge ukuguqulwa komzimba ziyaba vavanyo oluqhelekileyo lokuxilongwa kule mqathango.
Iingxaki
Njengoko kuhlolwe ngaphambilana, ukuchithwa kweeseli ezibomvu zegazi kubangela amazinga okwanda kwe-bilirubin. Iintsana ezinokukhubazeka kwePK zingadinga unyango nge-phototherapy. Kamva ebomini, ukwanda kwe-bilirubin kunokwakha i-sludge kwi-gallbladder kwaye ekugqibeleni kukhokelela ekuphuhlisweni kwama- gallstones .
Abantu abanokukhubazeka kwe-PK nabo banokuphuhlisa i- iron overload . Le nkxalabo ibonakala kakhulu kubantu abafuna ukumpontshelwa igazi rhoqo ukuba baphathe i-anemia yabo. Nangona kunjalo, oku kuya kwenzeka kubantu abangenayo imfuneko enkulu yokumpontshelwa igazi.
Kukho imithi enokukunceda umzimba ulahle i-iron eyongezelelweyo ebizwa ngokuthi i-chelators.
Njengabo bonke abantu abane-anemia ye-hemolytic, abantu abane-PK abasweleko basengozini yokufumana ingxaki yexesha elide . Oku kubangelwa i-parvovirus B19 isifo (esibangela ukuba iSifo sesiFundisa kubantwana). I-parvovirus inqanda umongo wethambo ekwenzeni amatsha egazi obomvu iintsuku ezi-7 ukuya kwi-10. Ngaphandle kokukwazi ukutshintsha amangqamuzana egazi obomvu ngokukhawuleza, i-anemia ekhulayo ingahlakulela kwaye ukuxilongwa kuyadingeka de kube yimihla yokubuyela kwimveliso.
I-anemia echaphazelekayo ehambelana nobunzima be-PK inokuvela kubantwana nangaphambi kokuba bazalwe.
Le meko ibizwa ngokuba yi-hydrous fetalis. I-Hydrops fetalis yigama eliqhelekileyo elisetyenziswe ukuchaza iziphumo kwi-ultrasound. I-anemia ye-Hemolytic efana ne-PK yokuntula, ingabangela le ngxaki. Ngokuxhomekeka kubunzima, oku kungaphathwa nge-intrauterine (nge-uterus) ukumpontshelwa kumntwana.
Unyango
Abanye oogqirha banokululeka ukuba uthathe i-folic acid imihla ngemihla. I-aclic acid i-vitamin iyadingeka ukwenza izitsha zegazi ezibomvu. Ngethamsanqa, eUnited States, ezininzi ukutya kwethu ziqiniswe nge-folic acid ngokunjalo.
Ngokuqhelekileyo, i-anemia enkulu iphathwa ngegazi. Abanye abantu abanePK abasweleko badinga ukumpontshelwa rhoqo kwiiveki ezintathu ukuya kwezine. Abanye abantu banokufuna ukuxilongwa ngamanye amaxesha ngexesha lokugula okanye uxinzelelo, kodwa abanye abanakuze bafune ukudluliselwa.
Ukuba amazinga e-bilirubin afinyelela kumanqanaba asemantwini azalwayo, kusenokufuneka ukutshintshiselwa imali. Kwinkqubo yegazi isuswa kwintsana (kwakhona iyakususa i-bilirubin) kwaye legazi ithathelwa endaweni yegazi. Le nkqubo inempumelelo xa isusa i-bilirubin ngokweqile kwigazi.
Iigalstones ezibangelwa ukusilela kwe-PK zingadinga ukuba zisuswe nge-cholecystectomy, ukukhutshwa kwe-gallbladder. I-Splenectomy , ukususwa kokugqithwa kwenteni , inokwenziwa ngenzame yokunciphisa ubunzima be-anemia, ngakumbi kubantu abafuna ukumpontshelwa rhoqo. Ngelishwa, i-splenectomy ayisebenzi kuwo wonke umntu onokukhubazeka kwe-PK.
ILizwi
Ukufunda wena okanye umntwana wakho unokukhubazeka kwe-PK unokukumangalela. Ngethamsanqa kukho iindlela zokonyango kwaye abaninzi abantu abane-PK balala kakuhle. Kukho uphando oluqhubekayo kwi-PK ukusilela kwaye sinethemba lokuba kukho iindlela ezininzi zokonyango kwixesha elizayo.