Ukusilela kweGlucose-6-phosphate dehydrogenase

Ukusilela kwe-Glucose-6-phosphate dehydrogenase (G6PD) yimeko apho iiseli ezibomvu zegazi ziyehla xa umzimba usiva iziyobisi ezithile okanye uxinzelelo losulelo. Ilifa, oko kuthetha ukuba lidlulisiwe kwiintsapho.

Ukusilela kwe-G6PD kwenzeka xa umntu engekho okanye engenayo ngokwaneleyo i-enzyme ebizwa ngokuba yi-glucose-6-phosphate dehydrogenase. Le enzyme inceda iiseli ezibomvu zegazi zisebenze ngokufanelekileyo.

I-G6PD encinci ikhokelela ekutshatyalalisweni kweeseli ezibomvu zegazi. Le nkqubo ibizwa ngokuba yi-hemolysis. Xa le nkqubo isenzeka, ibizwa ngokuba sisiqwengana sehemolytic. Iziqendu zihlala zifutshane. Kungenxa yokuba umzimba uyaqhubeka nokuvelisa iiseli ezibomvu zegazi, ezinomsebenzi oqhelekileyo.

Ukutshatyalaliswa kweseli ebomvu kungabangelwa zizifo, ukutya okuthile (njengeembotyi ze-fava), kunye namayeza athile, kubandakanya:

• Amayeza Antimalarial ezifana quinine
• I-Aspirin (iidosi eziphezulu)
• Izidakamizwa ezichasayo ezichasayo (i-NSAID)
• Iquinidine
• Iziyobisi zeSulfa
• Amayeza okubulala iintsholongwane afana nequinolones, nitrofurantoin
  

Ezinye iikhemikhali, ezinjengezo zemothballs, nazo zinokubangela isiqephu.

E-United States, ukusilela kwe-G6PD kuxhaphake kakhulu phakathi kwabantsundu kunabamhlophe. Amadoda kunokwenzeka ukuba abe nale ngxaki kunabafazi.

Kungenzeka ukuba uphuhlise le meko ukuba:

• Ngaba baseMelika baseMelika
• Ngaba banesidima kuMbindi Mpuma, ikakhulu amaKurdish okanye amaSephardic angamaJuda
• Yindoda
• Unembali yosapho yokusilela

Uhlobo lwesi sifo luqhelekile kubelungu bemvelaphi yeMeditera. Le fomu inxulunyaniswa neziqendu ze-hemolysis. Iziqendu zinde kwaye zinzima kunezinye iintlobo zesifo.

Abantu abakule meko ababonakalisi naziphi na iimpawu zesi sifo de iiseli ezibomvu zegazi zibonakale kwiikhemikhali ezithile ekutyeni okanye kumayeza.

Iimpawu zixhaphake kakhulu emadodeni kwaye zinokubandakanya:

• Umchamo omnyama
• Ifiva
• Intlungu esiswini
• I-spleen eyandisiweyo kunye nesibindi
• Ukudinwa
• Umtsalane
• Inqanaba lentliziyo elikhawulezayo
• Ukuqhawukelwa ngumphefumlo
• Umbala wolusu omthubi (jaundice)

Uvavanyo lwegazi lunokwenziwa ukujonga inqanaba le-G6PD.

Olunye uvavanyo olunokwenziwa lunokubandakanya:

• Inqanaba leBilirubin
• Gcwalisa ubalo lwegazi
• Ihemoglobin - umchamo
• Inqanaba leHaptoglobin
• Uvavanyo lwe-LDH
• Uvavanyo lokunciphisa iMethemoglobin
• Ukubala kweReticulocyte

Unyango lunokubandakanya:

• Amayeza anyanga usulelo, ukuba akhona
• Ukuyeka nawaphi na amayeza abangela ukutshabalalisa iiseli ezibomvu
• Utofelo-gazi, ngamanye amaxesha

Kwiimeko ezininzi, iziqendu ze-hemolytic ziyahamba zodwa.

Kwiimeko ezinqabileyo, ukusilela kwezintso okanye ukufa kunokubakho kulandela isiganeko esibuhlungu sehemolytic.

Fowunela umboneleli wakho wezempilo ukuba uneempawu zale meko.

Tsalela umnikezeli wakho ukuba ufumene ukuba une-G6PD defence kwaye iimpawu azipheli emva konyango.

Abantu abanesiphene se-G6PD kufuneka baziphephe ngokungqongqo izinto ezinokubangela isiqephu. Thetha nomboneleli wakho malunga namayeza akho.

Ingcebiso ngemfuzo okanye ukuvavanywa kunokufumaneka kwabo banembali yosapho ngale meko.

Ukusilela kwe-G6PD; I-hemolytic anemia ngenxa yokusilela kwe-G6PD; I-Anemia-hemolytic ngenxa yokusilela kwe-G6PD

• Iiseli zegazi

UGregg XT, uPrchal JT. I-enzymopathies yegazi ebomvu. Ku: Hoffman R, Benz EJ, Silberstein LE, et al, ii-eds. I-Hematology: Imigaqo esisiseko kunye nokuziqhelanisa. Umhla wesi-7. IPhiladelphia, PA: Elsevier; I-2018: isahluko 44.

ULissauer T, uCarroll W. Haematological ukuphazamiseka. Ku: Lissauer T, Carroll W, ii-eds. Incwadi ebhaliweyo yemifanekiso yePediatrics. Umhla we-5. IPhiladelphia, PA: Elsevier; I-2018: isahluko 23.

UMichel M. Ukuzenzekelayo kunye ne-intravascular hemolytic anemias. Ku: IGoldman L, iSchafer AI, ii-eds. Amayeza eGoldman-Cecil. Ngomhla wama-26. IPhiladelphia, PA: Elsevier; 2020: isahl. 151.