Methemoglobinemia

Methemoglobinemia (MetHb) ke lefu la mali leo ho lona ho hlahisoang methemoglobin e sa tloaelehang. Hemoglobin ke protheine e liseleng tse khubelu tsa mali (RBCs) e tsamaisang le ho aba 'mele' meleng. Methemoglobin ke mofuta oa hemoglobin.

Ka methemoglobinemia, hemoglobin e ka tsamaisa oksijene, empa ha e khone ho e lokolla ka nepo ho lisele tsa 'mele.

Boemo ba MetHb e ka ba:

• Ho fetisoa ke malapa (a futsitsoeng kapa a tsoalo)
• E bakoa ke ho pepesehela meriana e itseng, lik'hemik'hale kapa lijo (tse fumanoeng)

Ho na le mefuta e 'meli ea lefa la MetHb. Foromo ea pele e fetisoa ke batsoali ka bobeli. Hangata batsoali ha ba na boemo bona ka bobona. Ba jere liphatsa tsa lefutso tse bakang boemo boo. E etsahala ha ho na le bothata ba enzyme e bitsoang cytochrome b5 reductase.

Ho na le mefuta e 'meli ea MetHb e futsitsoeng:

• Mofuta oa 1 (o bitsoang hape ho haella ha erythrocyte reductase) o etsahala ha li-RBC li se na enzyme.
• Mofuta oa 2 (o bitsoang hape khaello ea reductase e akaretsang) o etsahala ha enzyme e sa sebetse 'meleng.

Mofuta oa bobeli oa MetHb e futsitsoeng o bitsoa lefu la hemoglobin M. E bakoa ke bokooa ba protheine ea hemoglobin ka boeona. Ke motsoali a le mong feela ea hlokang ho fetisetsa lefutso le sa tloaelehang hore ngoana a tle a rue lefu lena.

MetHb e fumanoeng e atile haholo ho feta mefuta eo re e futsitseng. E etsahala ho batho ba bang kamora hore ba pepesetsoe lik'hemik'hale tse itseng le meriana, ho kenyeletsoa:

• Li-anesthetics tse kang benzocaine
• Nitrobenzene
• Meriana e meng ea lithibela-mafu (ho kenyeletsoa dapsone le chloroquine)
• Li-nitrite (tse sebelisoang e le li-additives ho thibela nama hore e se ke ea senyeha)

Matšoao a mofuta oa 1 MetHb a kenyelletsa:

• Mmala o moputsoa oa letlalo

Matšoao a mofuta oa 2 MetHb a kenyelletsa:

• Ho lieha ho ntlafatsa
• Ho hloleha ho atleha
• Bokooa ba kelello
• Ho oa

Matšoao a lefu la hemoglobin M a kenyelletsa:

• Mmala o moputsoa oa letlalo

Matšoao a MetHb e fumanoeng a kenyelletsa:

• Mmala o moputsoa oa letlalo
• Ho opeloa ke hlooho
• Lerata
• Boemo bo fetohileng ba kelello
• Mokhathala
• Phefumoloho e khutšoanyane
• Ho hloka matla

Lesea le nang le boemo bona le tla ba le 'mala o moputsoa oa letlalo (cyanosis) ha le hlaha kapa nakoana kamora moo. Mofani oa tlhokomelo ea bophelo o tla etsa liteko tsa mali ho fumana boemo. Liteko li ka kenyelletsa:

• Ho hlahloba boemo ba oksijene maling (pulse oximetry)
• Teko ea mali ho lekola likhase maling (tlhahlobo ea khase ea mali ea mali)

Batho ba nang le lefu la hemoglobin M ha ba na matšoao. Kahoo, ba kanna ba se hloke kalafo.

Moriana o bitsoang methylene blue o sebelisetsoa ho phekola MetHb e matla. Methylene blue e kanna ea se sireletsehe ho batho ba nang le kapa ba ka ba kotsing ea lefu la mali le bitsoang khaello ea G6PD. Ha baa lokela ho noa moriana ona. Haeba uena kapa ngoana oa hau o na le khaello ea G6PD, lula u bolella mofani oa hau pele u fumana kalafo.

Ascorbic acid e ka sebelisoa ho fokotsa boemo ba methemoglobin.

Mefuta e meng ea kalafo e kenyelletsa kalafo ea oksijene ea hyperbaric, ho tšeloa lisele tse khubelu tsa mali le ho tšela phapanyetsano.

Maemong a mangata a MetHb a bonolo, ha ho hlokahale kalafo. Empa o lokela ho qoba moriana kapa khemikhale e bakileng bothata. Maemo a matla a ka hloka tšelo ea mali.

Batho ba nang le mofuta oa 1 MetHb le hemoglobin M lefu hangata ba etsa hantle. Mofuta oa 2 MetHb o tebile haholo. Hangata e baka lefu lilemong tse 'maloa tsa pele tsa bophelo.

Batho ba nang le MetHb e fumanoeng hangata ba etsa hantle haholo hang ha moriana, lijo kapa k'hemik'hale e bakileng bothata e tsejoa ebile e qojoa.

Mathata a MetHb a kenyelletsa:

• Ho tshoha
• Ho oa
• Lefu

Bitsa mofani oa hau haeba u:

• Eba le nalane ea lelapa ea MetHb
• Hlaolela matšoao a lefu lena

Bitsa mofani oa hau kapa litšebeletso tsa tšohanyetso (911) hang-hang haeba u na le phefumoloho e matla.

Ho fanoa ka tlhabollo ea lefutso bakeng sa banyalani ba nang le nalane ea lelapa ea MetHb mme ba nahana ho ba le bana.

Masea a likhoeli tse 6 kapa ho feta a na le monyetla oa ho ba le methemoglobinemia. Ka hona, lijana tse entsoeng ka maiketsetso tsa masea tse entsoeng ka meroho e nang le li-nitrate tsa tlhaho tse ngata joalo ka lihoete, beetroots kapa spinach li lokela ho qojoa.

Lefu la Hemoglobin M; Khaello ea erythrocyte reductase; Khaello ea kakaretso ea reductase; MetHb

• Lisele tsa mali

Benz EJ, Ebert BL. Mefuta e fapaneng ea hemoglobin e amanang le phokolo ea mali ea hemolytic, kamano e fetotsoeng ea oksijene le methemoglobinemias. Ka: Hoffman R, Benz EJ, Silberstein LE, bahlophisi. Hematology: Melao-motheo le Tloaelo ea Motheo. La 7th. Philadelphia, PA: Elsevier; 2018: khaolo ea 43.

Letterio J, Pateva I, Petrosiute A, Ahuja S.Hematologic le mathata a oncologic a lesea le ka popelong. Ka: Martin RJ, Fanaroff AA, Walsh MC, bangoli. Fanaroff le Phekolo ea Martin ea Neonatal-Perinatal. 11th ed. Philadelphia, PA: Elsevier; 2020: khaolo ea 79.

E bolela RT. Atamela li-anemias. Ka: Goldman L, Schafer AI, li-eds. Phekolo ea Goldman-Cecil. La 26th. Philadelphia, PA: Elsevier; 2020: khaolo ea 149.