Ingaba kukho ingxaki ngeoksijini egazini? Masifunde nge-methemoglobinemia!

Ngaba wakha wabona ulusu nemilebe yomntu zijika ngequbuliso zibe luhlaza okwesibhakabhaka? Okanye wakha weva ukuba ezinye iintsana zizalwa ziluhlaza okwesibhakabhaka? Esinye sezizathu zoku kusenokuba sisifo segazi esingaqhelekanga, kodwa esinokuba sibi kakhulu. Namhlanje siza kuthetha ngesinye sezi meko , i-methemoglobinemia . Abanye abantu babiza ngokuba yi-"blue baby syndrome."

Yintoni kanye kanye i-methemoglobinemia?

Ngamafutshane, i-methemoglobinemia yimeko apho iiseli ezibomvu zegazi egazini lethu zingakwazi ukuthwala ioksijini ukuya kwezinye iiseli kunye nezicubu emzimbeni. Cinga ngayo, iiseli ezibomvu zegazi zezona zinceda ukuthwala ioksijini emizimbeni yethu, akunjalo? Ezi seli zibomvu zegazi ziqulethe iproteni ekhethekileyo ebizwa ngokuba yi-hemoglobin . Ifana nebhasi, kwaye le hemoglobin ithwala ioksijini emzimbeni wonke.

Nangona kunjalo, kule meko ibizwa ngokuba yi-"Methemoglobinemia", okwenzekayo kukuba i-"Hemoglobin" yethu ethwala ioksijini iyatshintsha ize ibe yinto eyahlukileyo ebizwa ngokuba yi-"Methemoglobin." Ingxaki kukuba, le "Methemoglobin" ayinakuthwala ioksijini. Kufana nebhasi ethwala ioksijini ewohlokayo kwaye ayikwazi ukuthwala abakhweli.

Le meko inokuthi ifuzwe (ngokwemfuza) kwabanye abantu. Kodwa idla ngokubangelwa ngamayeza athile esiwasebenzisayo, iziyobisi zokuzonwabisa, okanye ukuchanabeka kwiikhemikhali ezithile . Ngamanye amaxesha inokuba yingozi ebomini, ingakumbi kwiintsana ezizelwe zinesimo esibi sale meko nakubantu abasebenzisa iziyobisi. Nangona kunjalo, kwiimeko ezininzi, oogqirha banokunika amayeza okunciphisa inqanaba le-"Methemoglobin" kunye nokususa iimpawu.

Oku kuyichaphazela njani umzimba wam?

Abantu abaninzi abane-methemoglobinemia bafumana imeko ebizwa ngokuba yi-cyanosis . I-Cyanosis kuxa iinzipho zakho, ulwimi, imilebe, kunye nolusu zijika zibe nombala oluhlaza okwesibhakabhaka okanye omfusa. Oku kwenzeka xa igazi lakho lingenawo umoya-mpilo owaneleyo. Njengoko benditshilo ngaphambili, iiseli ezibomvu zegazi zezona zithwala umoya-mpilo emzimbeni wonke. Iproteni ebizwa ngokuba yi-hemoglobin iyanceda koku.

Kwi-methemoglobinemia, i-``Methemoglobinemia`` yenzeka xa i-``Hemoglobin``` iba yi-``Methemoglobin``, nokuba kungenxa yotshintsho lwemfuza okanye esinye isizathu sangaphandle. Ekubeni i-``Methemoglobin`` ingenakuthwala ioksijini, ubungakanani beoksijini egazini buyancipha kwaye uphuhlisa i-``Cyanosis```.

Zithini iimpawu zeMethemoglobinemia?

Iimpawu zingahluka kumntu nomntu, kwaye zixhomekeke kuhlobo lwesifo onaso. Makhe sijonge indlela ezi mpawu ezibonakala ngayo rhoqo.

Iimpawu zeMethemoglobinemia efunyenweyo

Abantu abaninzi baba nale meko emva kokusebenzisa amayeza athile okudambisa iintlungu, okanye ukudibana nezinto ezinobungozi . Oku kubizwa ngokuba yi-acquired methemoglobinemia. Iimpawu ezinokubakho ziquka:

• Ulusu oluphaphathekileyo (olumnyama)
• Ndiziva ndidiniwe kakhulu (ndidinwe kakhulu)
• Ubuthathaka
• Intloko ebuhlungu

Ngamanye amaxesha, abantu abane-acquired methemoglobinemia banokuba neempawu ezinzima ezifuna unyango olukhawulezileyo . Ezi ziquka:

• Isicaphucaphu nokuhlanza.
• Ukulala kakhulu, ukuthetha okungacacanga, kunye nokucotha kokuphendula: Ezi zisenokuba ziimpawu zokudakumba kwenkqubo yemithambo-luvo ephakathi .
• Ukulahlekelwa zingqondo okanye iintshukumo zomzimba ezingalawulekiyo (njengokuxhuzula): Ezi ziimpawu zokuxhuzula okubangelwa kukuxhuzula .
• Ukuphefumla ngokukhawuleza, ukunyuka kwesantya sentliziyo, kunye nokudideka: Ezi ziimpawu zesifo esibizwa ngokuba yi-metabolic acidosis .

Kubalulekile: Ukuba umntu ohamba nawe ubonisa naziphi na ezi mpawu zesifo sokuwa, `(Metabolic Acidosis)` okanye `(Central Nervous System Depression)`, kufuneka utsalele umnxeba ku-1990 ngoko nangoko kwaye utsalele i-ambulensi .

Iimpawu zeCongenital Methemoglobinemia

I-methemoglobinemia yokuzalwa yimeko engaqhelekanga kakhulu. Zimbalwa kakhulu iimeko eziye zaxelwa kwihlabathi liphela. Ngokusekelwe kolo lwazi, oogqirha baye bahlulahlula zibe ziintlobo ezintathu eziphambili: Uhlobo 1, Uhlobo 2, kunye nesifo seHemoglobin M (HbM) .

• Abantu abanesifo se-hemoglobin M (HbM) badla ngokuba ne-cyanosis (ulusu oluluhlaza okwesibhakabhaka), kodwa ngokubanzi basempilweni.
• Nangona abantu abane -Type 1 methemoglobinemia (Type 1 MetHb) nabo benayo i-cyanosis, ezinye iingxaki ezinkulu zempilo azixhaphakanga.
• Nangona kunjalo, iintsana ezizelwe zine- Type 2 methemoglobinemia (Type 2 MetHb) zinokuba neengxaki ezinkulu zemithambo-luvo xa zifikelela kwiinyanga ezili-9 ubudala. Okubuhlungu kukuba, ezi ntsana aziphili ixesha elide.

Kutheni i-methemoglobinemia isenzeka?

Le meko isenokufunyanwa njengelifa, okanye, njengoko benditshilo ngaphambili, inokubangelwa kamva ngamayeza athile, amayeza, okanye iikhemikhali ezinobuthi.

Izizathu zeMethemoglobinemia efunyenweyo

• Izinto ezibulala iintlungu ezifana ne -benzocaine kunye ne-lidocaine (ingakumbi ezo zifumaneka kwiijeli ezisetyenziswa kwiindawo zokuthambisa kunye nezitshizi) kunye ne-antibiotic dapsone zinokubangela oku.
• Ii-nitrate ezisetyenziswa kwamanye amayeza, iiNitrites, ezinokubakho kwizigcini zokutya kunye namanzi athile, kunye nokuchatshazelwa kwezinye izibulali-zinambuzane ezisetyenziswa kwezolimo nazo zizizathu.
• Abantu abasebenzisa iziyobisi zokuzonwabisa ezifana ne -amyl nitrite (ekwaziwa ngokuba yi-poppers), i-nitrous oxide (igesi ehlekayo), kunye nezinye izinto ezibangela iintlungu ezixutywe neziyobisi ezifana ne-cocaine basengozini enkulu yeengxaki zempilo ezisongela ubomi ngenxa yamanqanaba aphezulu kakhulu e-methemoglobin.

Izizathu zeCongenital Methemoglobinemia

I-Congenital methemoglobinemia (Congenital MetHb) ibangelwa ziinguqu ezimbini ezahlukeneyo zemfuza.

• Kwiintlobo 1 kunye no-2 , utshintsho kwi-gene CYB5R luphazamisa ibhalansi phakathi kwe-hemoglobin kunye ne-methemoglobin kwiiseli ezibomvu zegazi.
• Isifo se-Hemoglobin M (HbM) sibangelwa kukuguquka kwezakhi zofuzo ezininzi ze-Hemoglobin M.

I-Congenital methemoglobinemia sisifo esibangelwa yi-autosomal recessive disorder . Ngamafutshane, ukuze umntwana abe nesi sifo, bobabini abazali kufuneka babe ngabathwali be-gene mutation kwaye umntwana kufuneka azuze loo nguqu kubo bobabini.

Isifo se-Hemoglobin M (HbM) sisifo esilawulayo esibangelwa yi-autosomal dominant disorder . Oku kuthetha ukuba umntwana unokufumana esi sifo nokuba uzuze utshintsho lwezakhi zofuzo kumzali omnye kuphela.

Oogqirha bayixilonga njani le nto?

Oogqirha bafumanisa le meko ngokuthatha imbali yakho epheleleyo yezonyango baze benze uvavanyo lomzimba . Ukuba umntu uneempawu ze-acquired methemoglobinemia, unokubuza malunga namayeza awasebenzisayo okanye iikhemikhali ezinobuthi asenokuba ebechaphazeleke kuzo.

Zeziphi iimvavanyo ezenziwayo ukufumanisa oku?

Olu vavanyo ludla ngokwenziwa:

• Uvavanyo lweGazi: Igazi elithathwe kwimithambo yegazi yabantu abanale meko lidla ngokuba mdaka omnyama. Lo mbala umdaka omnyama uthetha ukuba imithambo yegazi ayithwali ioksijini kakuhle egazini.
• Uvavanyo lwenqanaba leMethemoglobin (MetHb): Olu vavanyo lulinganisa ukuba ingakanani i-methemoglobin egazini lakho.
• Umsebenzi we-enzyme ye-CYB5R: Ukuba umsebenzi wale enzyme uphantsi kunesiqhelo, luphawu lwe-methemoglobinemia yokuzalwa.
• Ukuchonga iijini: Ukuba ukrokrela ukuba une-methemoglobinemia yokuzalwa, i-DNA yakho iya kuhlolwa ukuze kuqinisekiswe ukuba ifunyenwe. Oku kunokunceda ekuchongeni uhlobo lwesifo.
• I-Hemoglobin Electrophoresis:Le yindlela yokuvavanya i-"Hemoglobin" kwiiseli ezibomvu zegazi. Olu vavanyo lungasetyenziselwa ukufumanisa isifo se-hemoglobin M ``(HbM).``

Iphathwa njani le nto?

Iindlela zonyango ziyahluka ngokuxhomekeke kuhlobo lwe-methemoglobinemia onayo. Umzekelo, unyango losana olusandul’ ukuzalwa olunesifo sohlobo lwesibini lwahlukile kakhulu kunyango lomntu oye waba nale meko ngokuchanabeka kwikhemikhali enobuthi okanye ekusebenziseni iziyobisi.

• Abantu abane -Type 1 Methemoglobinemia okanye isifo se-Hemoglobin M basenokungadingi unyango. Ukuba kuyimfuneko, oogqirha banokusebenzisa la mayeza ukunciphisa amanqanaba e-Methemoglobin:
• I-Methylene Blue: Le yeyona ndlela iphambili yokuthintela i-methemoglobinemia.
• I-Vitamin C kunye ne-Vitamin B2.

Unyango lweMethemoglobinemia efunyenweyo

Ngokuxhomekeke kwimeko, i-acquired methemoglobinemia inokuba yingxamiseko yezonyango. Kwiimeko ezinjalo, ukufakelwa i-intravenous hydration kunye ne-oxygen kunokufuneka . Rhoqo, abantu abane-acquired methemoglobinemia banikwa i-methylene blue .

Zithini iingxaki zonyango?

Ukuba umntu one- G6PD Deficiency (kwakhona yimeko yemfuza) unikwa unyango lwe-methylene blue rhoqo, imeko ebizwa ngokuba yi -hemolysis inokwenzeka. I-Hemolysis kukonakala kweeseli zakho ezibomvu zegazi ngaphambi kwexesha okanye ngokungeyomfuneko.

Ndingawunciphisa na umngcipheko wam wokuba ne-methemoglobinemia?

• Abantu abafumana ilifa lohlobo lwemfuza lwale meko kufuneka baphephe iikhemikhali ezinobungozi (umz. izibulali-zinambuzane), amayeza athile, kunye nezinye izibulali-zintlungu ezininzi zasekuhlaleni ezinokunyusa amanqanaba e-methemoglobin. Ukuba unale meko, buza ugqirha wakho malunga nento onokuba nayo kwi-allergy.
• Abantu abasebenzisa iziyobisi ezifana ne -amyl nitrite okanye i-cocaine bazibeka emngciphekweni omkhulu wokufumana i-methemoglobinemia. Ukuba usebenzisa la mayeza kwaye ufuna uncedo lokuyeka, thetha nogqirha malunga nonyango olufumanekayo.

Kuza kwenzeka ntoni ukuba ndinesi sifo?

Kwiimeko ezininzi, unyango lunokunciphisa iimpawu. Uninzi lwabantu abane-Type 1 Congenital Methemoglobinemia, okanye isifo se-Hemoglobin M, baneempawu ezimbalwa. Bahlala bephila ixesha elide njengomntu ongenaso esi sifo.

Ndingazinyamekela njani?

• Ngokubanzi, abantu abazelwe benesifo "esizalwa naso" kufuneka balumkele amayeza kunye neekhemikhali ezinokubangela ukuba esi sifo sibe sibi ngakumbi. Ekubeni imeko yomntu wonke yahlukile kancinci, kungcono ukubonana nogqirha wakho.
• Abantu abane-acquired methemoglobinemia (Acquired MetHb) kufuneka baziphephe ngokupheleleyo izinto ezibangele loo nto - ezinje ngamayeza, iintlungu zokudambisa iintlungu, okanye iikhemikhali ezinobungozi.

Ndifanele ndimbone nini ugqirha?

• Ukuba une-methemoglobinemia ezuzwe njengelifa, bonana nogqirha ukuba ubona utshintsho emzimbeni wakho, njengokudinwa okanye ukudinwa. Ezi zisenokuba ziimpawu zokuba iiseli zakho ezibomvu zegazi azisakwazi ukuthwala ioksijini emzimbeni wakho wonke.
• Ukuba uneempawu ezinzima ezifana ne-cyanosis (ulusu oluluhlaza okwesibhakabhaka) kunye nokuxhuzula okanye ukozela kakhulu, fowunela u-911 ngoko nangoko okanye uye kwicandelo elikufutshane lesibhedlele.

Ndingayibuza yiphi imibuzo ugqirha?

I-Methemoglobinemia sisifo segazi esingaqhelekanga kakhulu. Abanye abantu bayifumana njengelifa (Congenital MetHb), kodwa uninzi lwabantu luyifumana kamva (Acquired MetHb). Ngokuxhomekeke kwimeko yakho, ungathanda ukubuza ugqirha wakho imibuzo efana nale:

Malunga ne-Acquired Methemoglobinemia (i-Acquired MetHb):

• Kutheni oku kwenzeke kum?
• Ngaba unyango luza kuziphelisa iimpawu zam?
• Ngaba iimpawu zam zingabuya?
• Ndingenza ntoni ukuthintela into efana nale ukuba ingaphindi yenzeke?

Malunga ne-Type 1 Congenital MetHb:

• Ingaba i-cyanosis (ulusu oluluhlaza okwesibhakabhaka) yingxaki enkulu yezonyango?
• Ngaba kuya kufuneka ndihlale ndithatha unyango lwe-methemoglobinemia (MetHb)?
• Ngaba ndingaba nezinye iimpawu?
• Ngaba abantwana bam banokufumana esi simo njengelifa?

Uhlobo lwesibini lweCongenital MetHb (ukuba lusemntwana):

• Le meko iza kumchaphazela njani umntwana wam?
• Ngaba kukho unyango lokunciphisa iimpawu zomntwana wam?
• Ndingenza ntoni ukunceda umntwana wam?
• Ukuba ndinabantwana abaninzi, ngaba oku kuya kwenzeka nakubo?

Ngamafutshane (Umyalezo Wokuya Ekhaya)

I-Methemoglobinemia sisifo esingaqhelekanga segazi esichaphazela amandla eeseli zethu ezibomvu zegazi ukuthwala ioksijini emzimbeni. Kwabanye, sifunyanwa njengelifa, kodwa kubantu abaninzi sibangelwa ngamayeza athile, ukuchatshazelwa ziikhemikhali ezinobungozi, okanye ukusetyenziswa kweziyobisi zokuzonwabisa. I-Congenital methemoglobinemia (ingakumbi uhlobo lwesi-2) ngamanye amaxesha inokubangela iingxaki ezinkulu zemithambo-luvo, kodwa amaxesha amaninzi ayibangeli iingxaki ezinkulu zempilo. Nangona kunjalo, i-acquired methemoglobinemia (acquired methemoglobinemia) ngamanye amaxesha inokuba yingozi ebomini.

Eyona nto ibalulekileyo kukuba ukuba ucinga ukuba usemngciphekweni wokufumana esi sifo, ungoyiki ukuthetha nogqirha ngaso. Uya kukuvuyela ukukunceda.

I- Methemoglobinemia, i-Methemoglobinemia, i-Blue baby syndrome, i-Cyanosis, ukunqongophala kweoksijini egazini, i-Hemoglobin, izifo zegazi, izifo zemfuza