Uvavanyo lwegazi lwePorphyrins

Iiporphyrins zinceda ukwenza izinto ezininzi ezibalulekileyo emzimbeni. Enye yazo yihemoglobin. Le yiprotein ekwiiseli ezibomvu zegazi ezithwala ioksijini egazini.

Iiporphyrins zinokulinganiswa egazini okanye kumchamo. Eli nqaku lixoxa ngovavanyo lwegazi.

Isampulu yegazi iyafuneka.

Isampulu ke ibekwa emkhenkceni isiwe kwangoko elabhoratri. I-porphyrins ezintathu zihlala zilinganiswa ngesixa esincinci egazini lomntu. Bazi:

• ICoproporphyrin
• Iprotoporphyrin (PROTO)
• IUroporphyrin

Ipropoporphyrin ihlala ifumaneka kwelona nani liphezulu. Olunye uvavanyo luyafuneka ukubonisa amanqanaba eeforfini ezithile.

Akufanele utye iiyure ezili-12 ukuya kwezi-14 ngaphambi kolu vavanyo. Ungasela amanzi kanye phambi kovavanyo. Iziphumo zakho zovavanyo zinokuchaphazeleka ukuba awuyilandeli le miyalelo.

Xa kufakwa inaliti ukutsala igazi, abanye abantu baziva iintlungu eziphakathi. Abanye baziva behlaba okanye behlaba kuphela. Emva koko, kunokubakho ukubetha okanye ukutyumza kancinci. Oku kuya kuhamba kungekudala.

Olu vavanyo lusetyenziselwa ukufumanisa i-porphyrias. Eli liqela leengxaki ezinqabileyo ezidla ngokudlula kumalungu osapho.

Ingasetyenziselwa kunye nolunye uvavanyo ukufumanisa ityhefu ekhokelayo kunye nenkqubo ethile yeemvakalelo kunye nokuphazamiseka kolusu.

Olu vavanyo ngokuthe ngqo luthatha onke amanqanaba e-porphyrin. Kodwa, amaxabiso esalathiso (uluhlu lwamaxabiso abonwe kwiqela labantu abasempilweni) ngamacandelo ngamanye nawo afakiwe:

• Amanqanaba e-porphyrin ewonke: 0 ukuya kwi-1.0 mcg / dL (0 ukuya kwi-15 nmol / L)
• Inqanaba leCoproporphyrin: 2 mcg / dL (30 nmol / L)
• Inqanaba leprotoporphyrin: 16 ukuya kwi-60 mcg / dL (0.28 ukuya kwi-1.07 µmol / L)
• Inqanaba le-Uroporphyrin: 2 mcg / dL (2.4 nmol / L)

Amaxabiso esiqhelo ahluka kancinci phakathi kweelebhu ezahlukeneyo. Ezinye iilebhu zisebenzisa imilinganiselo eyahlukeneyo okanye ukuvavanya iisampulu ezahlukeneyo. Thetha nogqirha wakho malunga nentsingiselo yeziphumo zakho zovavanyo.

Ukunyuswa kwamanqanaba ee-coproporphyrins kunokuba ngumqondiso we:

• Isizalo se-erythropoietic porphyria
• I-Hepatic coproporphyria
• I-Sideroblastic anemia
• I-variegate porphyria

Inqanaba lepropoporphyrin elonyukayo linokuba luphawu lwe:

• I-Anemia yesifo esinganyangekiyo
• I-protoporphyria yokuzalwa erythropoietic
• Ukwanda kwe-erythropoiesis
• Usulelo
• Ukunqongophala kwe-anemia
• Ityhefu yelothe
• I-Sideroblastic anemia
• Thalassemia
• I-variegate porphyria

Inqanaba le-uroporphyrin elonyukayo linokuba luphawu lwe:

• Isizalo se-erythropoietic porphyria
• I-Porphyria cutanea tarda

Akukho mngcipheko ubandakanyekileyo ekuthatheni igazi lakho. Imithambo kunye nemithambo iyahluka ngobukhulu ukusuka komnye umntu ukuya komnye, kwaye ukusuka kwelinye icala lomzimba ukuya kwelinye. Ukufumana isampulu yegazi kwabanye abantu kunokuba nzima ngakumbi kunabanye.

Eminye imingcipheko enxulumene nokutsalwa kwegazi incinci, kodwa inokubandakanya:

• Ukopha kakhulu
• Ukufa isiqaqa okanye ukuziva ungenantloko
• Ukugqobhoza okuninzi ukufumana imithambo
• I-Hematoma (igazi eliqokelela phantsi kwesikhumba)
• Usulelo (umngcipheko omncinci nangaliphi na ixesha ulusu lwaphukile)

Amanqanaba eprotoporphyrin; Porphyrins - iyonke; Amanqanaba eCoproporphyrin; Uvavanyo lwePROTO

• Uvavanyo lwegazi

IChernecky CC, iBerger BJ. Porphyrins, ubungakanani - igazi. Ku: IChernecky CC, iBerger BJ, ii-eds. Iimvavanyo zaselebhu kunye neenkqubo zokuqonda isifo. Umhla wesi-6. ISt Louis, MO: Elsevier Saunders; Ngo-2013: 891-892.

I-SJ epheleleyo, iWiley JS. I-Heme biosynthesis kunye nokuphazamiseka kwayo: i-porphyrias kunye ne-sideroblastic anemias. 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 38.