Ingabe ikhona inkinga nge-oxygen egazini? Ake sifunde nge-methemoglobinemia!

Wake wabona isikhumba nezindebe zomuntu ziphenduka umbala oluhlaza okwesibhakabhaka ngokuzumayo? Noma wake wezwa ukuthi ezinye izingane zizalwa ziluhlaza okwesibhakabhaka kancane? Esinye sezizathu zalokhu kungaba ukuphazamiseka kwegazi okungavamile kakhulu, kodwa okungenzeka kube kubi kakhulu. Namuhla sizokhuluma ngesinye salezi zimo , i-methemoglobinemia . Abanye abantu basibiza nangokuthi "i-blue baby syndrome."

Iyini ngempela i-methemoglobinemia?

Kalula nje, i-methemoglobinemia yisimo lapho amangqamuzana abomvu egazi lethu engakwazi ukuthwala umoya-mpilo kwamanye amangqamuzana nezicubu emzimbeni. Cabanga ngakho, amangqamuzana abomvu egazi yiwo asiza ukuthwala umoya-mpilo emizimbeni yethu, akunjalo? Lawa mangqamuzana abomvu egazi aqukethe iphrotheni ekhethekile ebizwa ngokuthi i-hemoglobin . Kufana nebhasi, futhi le hemoglobin ithwala umoya-mpilo emzimbeni wonke.

Kodwa-ke, kulesi simo esibizwa ngokuthi ``Methemoglobinemia``, okwenzekayo ukuthi ``Hemoglobin`` yethu ethwala umoya-mpilo iyashintsha futhi ibe uhlobo oluhlukile lwento ebizwa ngokuthi ``Methemoglobin``. Inkinga ukuthi, le ``Methemoglobin`` ayikwazi ukuthwala umoya-mpilo. Kufana nebhasi elithwala umoya-mpilo liyaphuka futhi alikwazi ukuthwala abagibeli.

Lesi simo singazuzwa njengefa (ngokwezakhi zofuzo) kwabanye abantu. Kodwa ngokuvamile sibangelwa imithi ethile esiyisebenzisayo, izidakamizwa zokuzijabulisa, noma ukuchayeka kumakhemikhali athile . Ngezinye izikhathi kungaba yingozi empilweni, ikakhulukazi ezinganeni ezizalwa zinesimo esibi salesi simo nakubantu abasebenzisa izidakamizwa. Kodwa-ke, ezimweni eziningi, odokotela bangakunikeza imithi yokwehlisa izinga le-"Methemoglobin" nokuqeda izimpawu.

Lokhu kuthinta kanjani umzimba wami?

Abantu abaningi abane-methemoglobinemia babhekana nesimo esibizwa ngokuthi i-cyanosis . I-Cyanosis yilapho izinzipho zakho, ulimi, izindebe, kanye nesikhumba sakho kuba nombala oluhlaza okwesibhakabhaka noma onsomi. Lokhu kwenzeka lapho igazi lakho lingenawo umoya-mpilo owanele. Njengoba ngishilo ngaphambili, amangqamuzana egazi abomvu yiwo avame ukuthwala umoya-mpilo kuwo wonke umzimba. Iphrotheni ebizwa ngokuthi i-hemoglobin iyasiza kulokhu.

Ku-methemoglobinemia, i-``Methemoglobinemia`` yenzeka lapho i-``Hemoglobin``` iba yi-``Methemoglobin``, kungaba ngenxa yokuguquka kwezakhi zofuzo noma esinye isizathu sangaphandle. Njengoba i-``Methemoglobin`` ingakwazi ukuthwala umoya-mpilo, inani lomoya-mpilo egazini liyancipha bese uba ne-``Cyanosis```.

Ziyini izimpawu ze-Methemoglobinemia?

Izimpawu zingahluka kumuntu nomuntu, futhi zincike ohlotsheni lwesifo onaso. Ake sibheke ukuthi lezi zimpawu zivame ukubonakala kanjani.

Izimpawu ze-Acquired Methemoglobinemia

Abantu abaningi baba nalesi simo ngemva kokuthatha imithi ethile yokunciphisa ubuhlungu, imithi, noma ukuchayeka ezintweni ezinobuthi . Lokhu kubizwa ngokuthi i-acquired methemoglobinemia. Izimpawu ezingase zivele zifaka:

• Isikhumba esikhanyayo (ubumthubi)
• Ukuzizwa ukhathele kakhulu (ukukhathala)
• Ubuthakathaka
• Ikhanda elibuhlungu

Ngezinye izikhathi, abantu abane-accomplied methemoglobinemia bangase babe nezimpawu ezinzima ezidinga usizo lwezokwelapha ngokushesha . Lezi zimpawu zifaka:

• Isicanucanu nokuhlanza.
• Ukulala ngokweqile, ukukhuluma okungacacile, kanye nokuphendula kancane: Lokhu kungaba izimpawu zokucindezeleka kwesimiso sezinzwa esiyinhloko .
• Ukulahlekelwa ukwazi noma ukunyakaza komzimba okungalawuleki (njengokuxhuzula): Lezi izimpawu zokuxhuzula okubangelwa yisifo sokuxhuzula .
• Ukuphefumula okusheshayo, ukushaya kwenhliziyo okukhuphukile, kanye nokudideka: Lezi yizimpawu zesimo esibizwa ngokuthi i-metabolic acidosis .

Okubalulekile: Uma othile onawe ekhombisa noma yiziphi zalezi zimpawu zesifo sokuwa, `(Metabolic Acidosis)` noma `(Central Nervous System Depression)`, kufanele ushayele u-1990 ngokushesha bese ushayela i-ambulensi .

Izimpawu ze-Congenital Methemoglobinemia

I-methemoglobinemia yokuzalwa iyisimo esingavamile kakhulu. Zimbalwa kakhulu izimo ezibikiwe emhlabeni wonke. Ngokusekelwe kulolo lwazi, odokotela bayihlukanise ngezinhlobo ezintathu eziyinhloko: Uhlobo 1, Uhlobo 2, kanye nesifo se-Hemoglobin M (HbM) .

• Abantu abanesifo se-hemoglobin M (HbM) bavame ukuba ne-cyanosis (isikhumba esiluhlaza okwesibhakabhaka), kodwa ngokuvamile baphilile.
• Abantu abane -Type 1 Methemoglobinemia (Type 1 MetHb) nabo bane-cyanosis, kodwa ezinye izinkinga ezinkulu zempilo azivamile.
• Kodwa-ke, izingane ezizalwa zine- Type 2 methemoglobinemia (Type 2 MetHb) zingaba nezinkinga ezinkulu zezinzwa ngesikhathi sezinezinyanga ezingaba ngu-9 ubudala. Ngokudabukisayo, lezi zingane aziphili isikhathi eside.

Kungani kwenzeka i-methemoglobinemia?

Lesi simo singazuzwa njengefa, noma, njengoba ngishilo ekuqaleni, singabangelwa kamuva yimithi ethile, izidakamizwa, noma amakhemikhali anobuthi.

Izimbangela ze-Acquired Methemoglobinemia

• Iziqeda-buhlungu ezifana ne -benzocaine ne-lidocaine (ikakhulukazi lezo ezitholakala kuma-gel angaphandle kanye nezifutho) kanye ne- dapsone yama-antibiotic kungabangela lokhu.
• Ama-nitrate asetshenziswa kweminye imithi, Ama-Nitrite, angase abe khona ezilondolozini zokudla kanye namanzi athile omthombo, kanye nokuchayeka kwezinye izibulali-zinambuzane ezisetshenziswa kwezolimo nakho kuyimbangela.
• Abantu abasebenzisa izidakamizwa zokuzijabulisa njenge -amyl nitrite (eyaziwa nangokuthi i-poppers), i-nitrous oxide (igesi ehlekayo), kanye neminye imithi yokubulala izinzwa exutshwe nezidakamizwa ezifana ne-cocaine basengozini enkulu yezinkinga zezokwelapha ezisongela ukuphila ngenxa yamazinga aphezulu kakhulu e-methemoglobin.

Izimbangela ze-Congenital Methemoglobinemia

I-Congenital methemoglobinemia (Congenital MetHb) ibangelwa izinguquko ezimbili ezahlukene zofuzo.

• Ezinhlotsheni 1 no-2 , ukuguquka kwezakhi zofuzo i- CYB5R kuphazamisa ibhalansi phakathi kwe-hemoglobin ne-methemoglobin kumaseli abomvu egazi.
• Isifo se-Hemoglobin M (HbM) sibangelwa izinguquko ezakhiweni eziningana ze-Hemoglobin M.

I-Congenital methemoglobinemia iyisifo esibangelwa yi-autosomal recessive disorder . Kalula nje, ukuze ingane ibe nalesi sifo, bobabili abazali kumele babe ngabathwali bokuguqulwa kwezakhi zofuzo futhi ingane kumele izuze ukuguquka kwezakhi zofuzo kubo bobabili.

Isifo se-Hemoglobin M (HbM) yisifo esivame ukwenzeka ngenxa yokukhubazeka kwe-autosomal . Lokhu kusho ukuthi ingane ingasithola lesi sifo ngisho noma izuza ukuguquka kwezakhi zofuzo kumzali oyedwa kuphela.

Odokotela bakuthola kanjani lokhu?

Odokotela baxilonga lesi simo ngokuthatha umlando wakho wezokwelapha ophelele bese benza ukuhlolwa ngokomzimba . Uma umuntu enezimpawu ze-accomplied methemoglobinemia, angabuza nganoma yimiphi imithi ayisebenzisayo noma amakhemikhali anobuthi okungenzeka ukuthi abhekane nawo.

Yiziphi izivivinyo ezenziwayo ukuze kutholakale lokhu?

Lezi zivivinyo zivame ukwenziwa:

• Ukuhlolwa Kwegazi: Igazi elithathwe emithanjeni yegazi yabantu abanalesi simo livame ukuba nombala onsundu omnyama. Lo mbala onsundu omnyama usho ukuthi imithambo yegazi ayithwali umoya-mpilo kahle egazini.
• Ukuhlolwa kwezinga le-Methemoglobin (MetHb): Lokhu kulinganisa ukuthi ingakanani i-methemoglobin egazini lakho.
• Umsebenzi we-enzyme ye-CYB5R: Uma umsebenzi wale enzyme uphansi kunojwayelekile, kuwuphawu lwe-methemoglobinemia yokuzalwa.
• Ukuhlola izakhi zofuzo: Uma usola ukuthi une-methemoglobinemia yokuzalwa, i-DNA yakho izohlolwa ukuze kutholakale izinguquko zofuzo ukuqinisekisa ukuxilongwa. Lokhu kungasiza futhi ekuboneni uhlobo lwesifo.
• I-Hemoglobin Electrophoresis:Lena indlela yokuhlola i-"Hemoglobin" emangqamuzaneni abomvu egazi. Lokhu kuhlola kungasetshenziswa ukuthola isifo se-hemoglobin M ``(HbM).``

Kuphathwa kanjani lokhu?

Izinketho zokwelapha ziyahlukahluka kuye ngohlobo lwe-methemoglobinemia onayo. Isibonelo, ukwelashwa kosana olusanda kuzalwa olunesifo sohlobo 2 kuhluke kakhulu ekwelashweni komuntu othole lesi simo ngokuchayeka ekusetshenzisweni kwamakhemikhali anobuthi noma izidakamizwa.

• Abantu abanesifo se-Type 1 Methemoglobinemia noma i-Hemoglobin M bangase bangadingi ukwelashwa. Uma kudingeka, odokotela bangasebenzisa le mithi ukwehlisa amazinga e-Methemoglobin:
• I-Methylene Blue: Lesi yisisombululo esiyinhloko se-methemoglobinemia.
• I-Vitamin C kanye ne-Vitamin B2.

Ukwelashwa kwe-Acquired Methemoglobinemia

Kuye ngesimo, i-acquired methemoglobinemia ingaba yisimo esiphuthumayo sezokwelapha. Ezimweni ezinjalo, kungadingeka uketshezi olufakwa emthanjeni kanye nomoya-mpilo . Ngokuvamile, abantu abane-acquired methemoglobinemia banikezwa i-methylene blue .

Yiziphi izinkinga zokwelashwa?

Uma umuntu onesifo sokuntuleka kwe-G6PD (futhi esiwufuzo) enikezwa ukwelashwa kwe-methylene blue njalo, isimo esibizwa ngokuthi i-hemolysis singaba khona. I-Hemolysis ukuwohloka kwamangqamuzana akho abomvu egazi ngaphambi kwesikhathi noma ngokungadingekile.

Ngingayinciphisa yini ingozi yami yokuthola i-methemoglobinemia?

• Abantu abazuza uhlobo oluthile lwezakhi zofuzo lwalesi simo kufanele bagweme amakhemikhali anobuthi (isib. izibulala-zinambuzane), imithi ethile, kanye nemithi eminingi yokubulala izinzwa yendawo engandisa amazinga e-methemoglobin. Uma unalesi simo, buza udokotela wakho ngalokho ongase ube nakho ukungezwani komzimba.
• Abantu abasebenzisa izidakamizwa ezifana ne -amyl nitrite noma i-cocaine bazibeka engcupheni enkulu yokuthola i-methemoglobinemia. Uma usebenzisa le mithi futhi udinga usizo lokuyeka, khuluma nodokotela mayelana nezindlela zokwelapha ezitholakalayo.

Kwenzekani uma nginalesi simo?

Ezimweni eziningi, ukwelashwa kunganciphisa izimpawu. Iningi labantu abane-Type 1 Congenital Methemoglobinemia, noma isifo se-Hemoglobin M, banezimpawu ezimbalwa. Ngokuvamile baphila isikhathi eside njengomuntu ongenaso lesi simo.

Ngizinakekela kanjani?

• Ngokuvamile, abantu abazalwa benesifo "sokuzalwa" kufanele baqaphele imithi namakhemikhali angasenza sibe sibi kakhulu lesi sifo. Njengoba isimo sabo bonke sihlukile kancane, kungcono ukubonisana nodokotela wakho.
• Abantu abane-acquired methemoglobinemia (Acquired MetHb) kufanele bagweme ngokuphelele izinto ezibangele lokhu - njengemithi, izinhlungu zobuhlungu ezisetshenziswa endaweni yokwelapha, noma amakhemikhali anobuthi.

Kufanele ngimbone nini udokotela?

• Uma une-methemoglobinemia ezuzwe njengefa, bona udokotela uma ubona izinguquko emzimbeni wakho, njengokukhathala noma ukudinwa. Lokhu kungaba izimpawu zokuthi amangqamuzana akho abomvu egazi awasakwazi ukuthwala umoya-mpilo emzimbeni wakho wonke.
• Uma unezimpawu ezinzima njenge-cyanosis (isikhumba esiluhlaza okwesibhakabhaka) kanye nokuquleka noma ukozela ngokweqile, shayela u-911 ngokushesha noma uye emnyangweni wezimo eziphuthumayo esibhedlela esiseduze.

Yimiphi imibuzo okufanele ngiyibuze udokotela?

I-Methemoglobinemia iyisifo segazi esingavamile kakhulu. Abanye abantu banesifo esidluliselwa ngofuzo (Congenital MetHb), kodwa abantu abaningi basithola kamuva (Acquired MetHb). Kuye ngesimo sakho, ungase ufune ukubuza udokotela wakho imibuzo efana nale:

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

• Kungani lokhu kwenzeke kimi?
• Ingabe ukwelashwa kuzoqeda izimpawu zami?
• Ingabe izimpawu zami zingabuya?
• Yini okufanele ngiyenze ukuze ngivimbele into efana nale ukuthi ingaphinde yenzeke?

Mayelana ne-Type 1 Congenital MetHb:

• Ingabe i-cyanosis (isikhumba esiluhlaza okwesibhakabhaka) iyinkinga enkulu yezokwelapha?
• Ingabe kuzomele ngihlale ngithatha ukwelashwa kwe-methemoglobinemia (MetHb)?
• Ngingaba nezinye izimpawu?
• Ingabe izingane zami zingazuza lesi simo njengefa?

Uhlobo 2 lwe-Congenital MetHb (uma isenganeni):

• Lesi simo sizomthinta kanjani umntwana wami?
• Ingabe kukhona ukwelashwa kokunciphisa izimpawu zomntwana wami?
• Yini engingayenza ukuze ngisize ingane yami?
• Uma nginezingane eziningi, ingabe lokhu kuzokwenzeka nakubo?

Ngamafuphi (Umyalezo Wokuya Ekhaya)

I-Methemoglobinemia iyisifo esingavamile segazi esithinta ikhono lamaseli ethu abomvu okuthwala umoya-mpilo emzimbeni. Kwabanye, sizuzwa njengefa, kodwa kubantu abaningi sibangelwa imithi ethile, ukuchayeka kumakhemikhali anobuthi, noma ukusetshenziswa kwezidakamizwa zokuzijabulisa. I-Congenital methemoglobinemia (ikakhulukazi uhlobo 2) ngezinye izikhathi ingabangela izinkinga ezinkulu zezinzwa, kodwa ngokuvamile ayibangeli izinkinga ezinkulu zempilo. Kodwa-ke, i-acquired methemoglobinemia (acquired methemoglobinemia) ngezinye izikhathi ingaba yingozi empilweni.

Into ebaluleke kakhulu ukuthi uma ucabanga ukuthi usengozini yokuthola lesi sifo, ungesabi ukukhuluma nodokotela ngaso. Uzokujabulela ukukusiza.

I- Methemoglobinemia, i-Methemoglobinemia, i-Blue baby syndrome, i-Cyanosis, Ukuntuleka komoya-mpilo egazini, i-Hemoglobin, Izifo zegazi, Izifo zofuzo