Incazelo
I-Glucose 6 Ukuntuleka kwe-Phosphate Dehydrogenase (G6PD) yilapho kutholakala khona inzyme ejwayelekile kunazo zonke emhlabeni. Abantu abangaba ngu-400 million bathinteka emhlabeni jikelele. Kukhona ukuhlukahluka okukhulu kokuqina okususelwe ekuguqulweni okuzuzwa njengefa.
I-G6PD iyi-enzyme etholakala esitokisini segazi elibomvu elidingekayo ukunikeza amandla esitokisini. Ngaphandle kwaleli gesi, i-red cell selibhujiswa ngumzimba (i-hemolysis) eholela ekudleni kwegazi kanye ne-jaundice (ukuphuzila kwesikhumba).
Ubani Ongengozini Yokuntuleka Kwe-G6PD?
Isakhi se-G6PD sitholakala kwi-X chromosome eyenza abesilisa abatholakale kakhulu ekulahlekeni kwe-G6PD (i-X-linked disorder). Ukuntuleka kwe-G6PD kuvimbela abantu ekubeni bangenwe yi-malaria ngakho-ke kuvame ukubonakala ezindaweni eziphezulu zokutheleleka kwe-malaria njenge-Afrika, esifundeni saseMedithera nase-Asia. E-United States, ama-10% ase-Afrika-aseMelika abesilisa banesifo se-G6PD.
Ziyini Izimpawu?
Izimpawu zithembele ukuthi yikuphi ukuguqulwa okuzuzayo. Abanye abantu bangase bangabonwa ukuthi akukho zimpawu ezikhona. Ezinye iziguli ezinokuntuleka kwe-G6PD zinempawu kuphela lapho zivezwe emithi ethile noma ukudla (bheka uhlu ngezansi). Abanye abantu bangase babhekwe njengezingane ezisanda kuzalwa ngemva kokuzwa i-jaundice enzima (ebizwa ngokuthi i-hyperbilirubinemia). Kulezo ziguli nalabo abanezinhlobo ezimbi kakhulu zokuntuleka kwe-G6PD ene-hemolysis engapheli, izimpawu zifaka:
- Umbala ogqamile noma obala osesithombeni
- Ukushaya kwenhliziyo okusheshayo
- Ukukhathala noma ukukhathala
- Ukuzwa ukhululekile noma udizizi
- I-Yellowing yesikhumba (i-jaundice) noma amehlo (i-icterus yesikhumba)
- Umchamo omnyama
Ukuntula kwe-G6PD kutholakala kanjani?
Ukubona ukulahlekelwa kwe-G6PD kungaba yinkinga. Okokuqala, udokotela kufanele asole ukuthi une-anemia ye-hemolytic (ukwehla kwamaseli abomvu wegazi). Lokhu kuvame ukuqinisekiswa ngokubala okuphelele kwegazi kanye nokubala kwe-reticulocyte.
Ama-reticulocyte amamasosha egazi abomvu abomvu athunyelwe kusuka emthonjeni wethambo ngokuphendula i-anemia. I-anemia enezinombolo eziphakeme ze-reticulocyte ihambisana ne-anemia ye-hemolytic. Amanye ama-lab angase afake inani le-bilirubin elizophakanyiswa. I-Bilirubiin ikhishwa esitokisini segazi elibomvu uma idilikile futhi ibangela i-jaundice ngesikhathi sezinkinga ze-hemolytic.
Inqubo yokunquma ukuxilongwa, udokotela wakho uzodinga ukulawula i- autoimmune i-hemolytic anemia (AIHA). Ukuhlolwa okuqondile kwe-anti globulin (okubizwa ngokuthi i-Coombs 'yokuhlola okuqondile) kuhlola ukuthi ngabe kunezifo ezithathelwanayo kumangqamuzana egazi abomvu okubenza bahlaselwe yisistimu yakho yomzimba. Njengamanye amacala we-anemia ye-hemolytic, i-peripheral blood smear (i-microscope slide yegazi) iyasiza kakhulu. Ekulweni kwe-G6PD, amangqamuzana aluma nama cell blister avamile. Lezi zibangelwa izinguquko ezenzeka esitokisini segazi elibomvu njengoba libhujiswa.
Uma ukulahlekelwa kwe-G6PD kucatshangwa, izinga le-G6PD lingasuswa. Izinga eliphansi le-G6PD lihambisana nokuntuleka kwe-G6PD. Ngeshwa, phakathi nenkinga enkulu ye-hemolytic izinga elijwayelekile le-G6PD alinakuqeda ukungabi nalutho. I-reticulocytes eminingi ekhona phakathi nenkinga ye-hemolytic iqukethe amazinga avamile we-G6PD okwenza kube nomthelela wamanga.
Uma ukusola kakhulu, ukuhlolwa kufanele kuphindwe lapho isiguli sisesimweni sokuqala.
I-G6PD iphathwa kanjani?
Gwema imithi noma ukudla okubangela i-hemolytic (i-red red cell breakdown). Ezinye zazo zibalwe ngezansi.
- Ama-fava ubhontshisi (okubizwa nangokuthi ubhontshisi obanzi)
- Amabhola amabhola (noma ezinye imikhiqizo equkethe i-naphthalene)
- Ama-antibiotic e-Sulfa afana neBactrim / Septra, i-sulfadiazine
- Ama-antibiotic e-Quinolone afana ne-ciprofloxacin, i-levofloxacin
- Nitrofurantoin (i-antibiotic)
- Imithi yokulwa ne-malaria njenge-primaquine
- I-Methylene blue
- Imithi ye-TB efana ne-dapsone ne-sulfoxone
- Imithi yokwelapha umdlavuza njenge doxorubicin noma i-rasburicase
- I-Phenazopyridine
Ukumpontshelwa igazi kusetshenziswa uma i-anemia inzima futhi isiguli sinesifo. Ngenhlanhla, iziguli eziningi azidingi ukumpontshelwa igazi.