Ukushoda kwe-Glucose-6-phosphate dehydrogenase

Ukushoda kweGlucose-6-phosphate dehydrogenase (G6PD) kuyisimo lapho amaseli abomvu ehla khona lapho umzimba uvezwa yimithi ethile noma ingcindezi yokutheleleka. Kuyifa, okusho ukuthi kudluliselwa emindenini.

Ukushoda kwe-G6PD kwenzeka lapho umuntu elahlekile noma engenayo i-enzyme ebizwa nge-glucose-6-phosphate dehydrogenase. Le enzyme isiza amangqamuzana abomvu egazi ukuthi asebenze kahle.

I-G6PD encane kakhulu iholela ekubhujisweni kwamaseli abomvu egazi. Le nqubo ibizwa ngokuthi i-hemolysis. Lapho le nqubo yenzeka ngenkuthalo, ibizwa ngesiqephu se-hemolytic. Iziqephu zivame ukuba zifushane. Lokhu kungenxa yokuthi umzimba uyaqhubeka nokukhiqiza amangqamuzana egazi abomvu amasha, anomsebenzi ojwayelekile.

Ukubhujiswa kwamangqamuzana egazi abomvu kungadalwa izifo, ukudla okuthile (njengobhontshisi we-fava), nemithi ethile, kufaka phakathi:

• Imithi elwa namagciwane efana ne-quinine
• I-Aspirin (amanani aphezulu)
• Izidakamizwa ezingezona ukuvuvukala (ama-NSAID)
• I-Quinidine
• Izidakamizwa zeSulfa
• Ama-antibiotic afana ne-quinolones, i-nitrofurantoin
  

Amanye amakhemikhali, njengalawo asemabholeni enundu, nawo angadala isiqephu.

E-United States, ukushoda kwe-G6PD kuvame kakhulu kwabamnyama kunabamhlophe. Abesilisa kungenzeka kakhulu ukuthi babe nalesi sifo kunabesifazane.

Kungenzeka ukuthi uthuthukise lesi simo uma:

• Ingabe ama-African American
• Bangabantu abahloniphekile baseMpumalanga Ephakathi, ikakhulukazi amaKurdish noma amaSephardic Jewish
• Abesilisa
• Yiba nomlando womndeni wokushoda

Ifomu lalesi sifo livamile kwabamhlophe abavela eMedithera. Leli fomu lihlotshaniswa neziqephu ezinzima ze-hemolysis. Iziqephu zinde futhi zinzima kakhulu kunezinye izinhlobo zesifo.

Abantu abanalesi sifo abakhombisi zimpawu zesifo kuze kube yilapho amangqamuzana abo abomvu egazi etholakala kumakhemikhali athile ekudleni noma emithini.

Izimpawu zivame kakhulu emadodeni futhi zingafaka:

• Umchamo omnyama
• Imfiva
• Ubuhlungu esiswini
• Ubende obukhulisiwe nesibindi
• Ukukhathala
• Pallor
• Ukushaya kwenhliziyo okusheshayo
• Ukuphelelwa umoya
• Umbala wesikhumba ophuzi (i-jaundice)

Ukuhlolwa kwegazi kungenziwa ukuhlola izinga le-G6PD.

Olunye uvivinyo olungenziwa lubandakanya:

• Izinga leBilirubin
• Qedela ukubalwa kwegazi
• I-hemoglobin - umchamo
• Izinga le-Haptoglobin
• Ukuhlolwa kwe-LDH
• Isivivinyo sokunciphisa iMethemoglobin
• Ukubalwa kwe-Reticulocyte

Ukwelashwa kungafaka:

• Imithi yokwelapha ukutheleleka, uma ikhona
• Ukuyeka noma imiphi imithi edala ukubhujiswa kwamangqamuzana egazi abomvu
• Ukumpontshelwa, kwezinye izimo

Ezimweni eziningi, iziqephu ze-hemolytic ziyahamba zodwa.

Ezimweni ezingavamile, ukwehluleka kwezinso noma ukufa kungenzeka ngemuva kwesigameko esibi se-hemolytic.

Shayela umhlinzeki wakho wezokunakekelwa kwezempilo uma unezimpawu zalesi simo.

Shayela umhlinzeki wakho uma kutholakale ukuthi unesifo se-G6PD futhi izimpawu azipheli ngemuva kokwelashwa.

Abantu abanenkinga ye-G6PD kumele bagweme ngokuqinile izinto ezingadala isiqephu. Khuluma nomhlinzeki wakho ngemithi yakho.

Ukwelulekwa noma ukuhlolwa ngofuzo kungatholakala kulabo abanomlando womndeni waleso simo.

Ukushoda kwe-G6PD; I-hemolytic anemia ngenxa yokushoda kwe-G6PD; I-anemia - hemolytic ngenxa yokushoda kwe-G6PD

• Amaseli egazi

UGregg XT, uPrchal JT. Ama-enzyme egazi abomvu. Ku: Hoffman R, Benz EJ, Silberstein LE, et al, ama-eds. I-Hematology: Izimiso Eziyisisekelo Nokuzijwayeza. Umhla wesi-7. IPhiladelphia, PA: Elsevier; 2018: isahluko 44.

ULissauer T, uCarroll W. Haematological ukuphazamiseka. Ku: Lissauer T, Carroll W, ama-eds. I-Illustrated Textbook of Paediatrics. Umhlaka 5. IPhiladelphia, PA: Elsevier; 2018: isahluko 23.

UMichel M. Autoimmune kanye ne-intravascular hemolytic anemias. Ku: IGoldman L, iSchafer AI, ama-eds. Imithi yeGoldman-Cecil. Umhla ka-26. IPhiladelphia, PA: Elsevier; 2020: isahluko 151.