Boloetse ba sele

Boloetse ba Sickle cell ke bokuli bo fetisoang ke malapa. Lisele tse khubelu tsa mali tse atisang ho bōptjoa joaloka disk li nka sekele kapa sebopeho sa seoli. Lisele tse khubelu tsa mali li tsamaisa oksijene 'meleng oohle.

Boloetse ba sele bo kulang bo bakoa ke mofuta o sa tloaelehang oa hemoglobin o bitsoang hemoglobin S. Hemoglobin ke protheine e kahare ho lisele tse khubelu tsa mali tse tsamaisang oksijene.

• Hemoglobin S e fetola lisele tse khubelu tsa mali. Lisele tse khubelu tsa mali lia fokola 'me li bōpehile joaloka li-crescent kapa lisekele.
• Lisele tse sa tloaelehang li isa oksijene e fokolang liseleng tsa 'mele.
• Li ka ts'oaroa habonolo methapong e menyenyane ea mali ebe li robeha likotoana. Sena se ka sitisa phallo ea mali e phetseng hantle 'me sa fokotsa le ho feta palo ea oksijene e phallelang lisele tsa' mele.

Boloetse ba lisele tse kulang bo futsitsoe ho batsoali ka bobeli. Haeba u fumana lefutso la sele ea sekele ho motsoali a le mong feela, u tla ba le tšobotsi ea sele ea sekele. Batho ba nang le tšobotsi ea sele ea sekele ha ba na matšoao a lefu la sele ea sekele.

Boloetse ba lisele tsa boloetse bo atile haholo bathong ba litloholo tsa Afrika le Mediterranean. E boetse e bonoa ho batho ba tsoang Amerika Boroa le Bohareng, Caribbean le Middle East.

Matšoao hangata ha a hlahe ho fihlela kamora likhoeli tsa 4.

Hoo e batlang e le batho bohle ba nang le lefu la sele ea sekele ba na le linako tse bohloko tse bitsoang mathata. Tsena li ka nka lihora ho isa matsatsing. Mathata a ka baka bohloko mokokotlong o ka tlase, leoto, manonyeletso le sefubeng.

Batho ba bang ba ba le ketsahalo e le 'ngoe ka mor'a lilemo tse' maloa. Ba bang ba na le mananeo a mangata selemo se seng le se seng. Mathata a ka ba matla hoo a ka hlokang ho lula sepetlele.

Ha phokolo ea mali e ba matla le ho feta, matšoao a ka kenyelletsa:

• Mokhathala
• Paletso
• Ho otla ha pelo ka potlako
• Phefumoloho e khutšoanyane
• Bosehla ba mahlo le letlalo (jaundice)

Bana ba banyenyane ba nang le lefu la sele ea sekele ba na le litlhaselo tsa bohloko ba mpeng.

Matšoao a latelang a ka hlaha hobane methapo e menyenyane ea mali e koaloa ke lisele tse sa tloaelehang:

• Ho tsoha ho bohloko le halelele (priapism)
• Ho se bone hantle kapa bofofu
• Mathata a ho nahana kapa pherekano e bakoang ke liropo tse nyane
• Lisosa maotong a ka tlase (ho bacha le ho batho ba baholo)

Ha nako e ntse e ea, spleen e emisa ho sebetsa. Ka lebaka leo, batho ba nang le maloetse a likokoana-hloko ba ka ba le matšoao a tšoaetso e kang:

• Tšoaetso ea masapo (osteomyelitis)
• Tšoaetso ea gallbladder (cholecystitis)
• Tšoaetso ea matšoafo (pneumonia)
• Tšoaetso ea pampitšana ea moroto

Matšoao a mang a kenyelletsa:

• Kholo e liehang le bohlankana
• Manonyeletso a bohloko a bakoang ke ramatiki
• Ho hloleha ha pelo kapa sebete ka lebaka la tšepe e ngata haholo (ho tsoa tšelo ea mali)

Liteko tse atisang ho etsoa ho fumana le ho beha leihlo batho ba nang le lefu la sele ea kelete li kenyelletsa:

• Bilirubin
• Ho khotsofatsa mali ha oksijene ea mali
• Palo e felletseng ea mali (CBC)
• Hemoglobin electrophoresis
• Serum creatinine
• Potassium ea seramu
• Teko ea lisele tse kulang

Morero oa kalafo ke ho sebetsana le ho laola matšoao, le ho fokotsa palo ea mathata. Batho ba nang le lefu la sele ea sekele ba hloka kalafo e tsoelang pele, leha ba se na mathata.

Batho ba nang le boemo bona ba lokela ho noa li-folic acid. Folic acid e thusa ho etsa lisele tse khubelu tsa mali.

Kalafo ea koluoa ​​ea sele ea sekele e kenyelletsa:

• Litšelo tsa mali (li kanna tsa fuoa khafetsa ho thibela stroke)
• Meriana ea bohloko
• Metsi a mangata

Mefuta e meng ea kalafo ea lefu la sekele e ka kenyelletsa:

• Hydroxyurea (Hydrea), e thusang ho fokotsa palo ea linako tsa bohloko (ho kenyelletsa bohloko ba sefuba le mathata a ho hema) ho batho ba bang
• Lithibela-mafu, tse thusang ho thibela tšoaetso ea baktheria e atileng ho bana ba nang le lefu la sele ea sekele
• Meriana e fokotsang tšepe ka bongata 'meleng
• Mefuta e mecha ea phekolo e fokotsang maqhubu le ho tiea ha mathata a bohloko e amohetsoe

Kalafo e ka 'nang ea hlokahala ho sebetsana le mathata a lefu la sickle cell e kenyelletsa:

• Dialysis kapa phetoho ea liphio bakeng sa lefu la liphio
• Ho fana ka tlhabollo bakeng sa mathata a kelello
• Ho tlosoa ha gallbladder ho batho ba nang le lefu la gallstone
• Hip e nkela sebaka sa avascular necrosis ea letheka
• Ho buoa ka mathata a mahlo
• Kalafo ea tšebeliso e feteletseng kapa tšebeliso e mpe ea meriana ea bohloko ba narcotic
• Tlhokomelo ea maqeba a liso tsa maoto

Motsoako oa masapo kapa methapo ea methapo ea methapo e ka phekola lefu la seleng, empa kalafo ena ha se khetho ho batho ba bangata. Batho ba nang le lefu la sele ea sekele hangata ba sitoa ho fumana bafani ba li-cell ba tšoanang hantle.

Batho ba nang le maloetse a likokoana-hloko ba lokela ho ba le liente tse latelang ho fokotsa kotsi ea tšoaetso:

• Ente ea Haemophilus influenzae (Hib)
• Ente ea Pneumococcal conjugate (PCV)
• Ente ea pneumococcal polysaccharide (PPV)

Ho kenela sehlopha sa ts'ehetso moo litho li arolelanang litaba tse tloaelehileng ho ka imolla khatello ea maikutlo ea lefu le sa foleng.

Nakong e fetileng, batho ba nang le maloetse a likokoana-hloko hangata ba ne ba hlokahala ba le pakeng tsa lilemo tse 20 le 40. Ka lebaka la tlhokomelo ea sejoale-joale, batho ba se ba ka phela lilemo tse 50 le ho feta.

Lisosa tsa lefu li kenyelletsa ho hloleha ha setho le tšoaetso.

Bitsa mofani oa tlhokomelo ea bophelo haeba u na le:

• Matšoao a tšoaetso (feberu, bohloko ba 'mele, hlooho, mokhathala)
• Mathata a bohloko
• Erection e bohloko le ea nako e telele (ka banna)

Phokolo ea mali - sele ea sekele; Lefu la Hemoglobin SS (Hb SS); Phokolo ea mali ea sele

• Lisele tse khubelu tsa mali, sele ea sekele
• Lisele tse khubelu tsa mali - li tloaelehile
• Lisele tse khubelu tsa mali - lisele tse ngata tsa sekele
• Lisele tse khubelu tsa mali - lisele tsa sekele
• Lisele tse khubelu tsa mali - sekele le Pappenheimer
• Likarolo tse entsoeng tsa mali
• Lisele tsa mali

Howard J. Sickle lefu la sele le li-hemoglobinopathies tse ling. Ka: Goldman L, Schafer AI, li-eds. Phekolo ea Goldman-Cecil. La 26th. Philadelphia, PA: Elsevier; 2020: khaolo ea 154.

Meier ER. Khetho ea kalafo bakeng sa lefu la sekele. Kliniki ea bana North Am. 2018; 65 (3) 427-443. PMID 29803275 e fetotsoe.ncbi.nlm.nih.gov/29803275/.

Webosaete ea National Heart Lung le Blood Institute. Tsamaiso e thehiloeng bopaking ba lefu la sele ea sekele: tlaleho ea sehlopha sa litsebi, 2014. www.nhlbi.nih.gov/health-topics/evidence-based-management-sickle-cell-disease. E ntlafalitsoe ka Loetse 2014. E fihlile ka la 19 Pherekhong 2018.

Saunthararajah Y, Vichinsky EP. Boloetse ba Sickle cell: likarolo tsa bongaka le tsamaiso. Ka: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Melao-motheo le Tloaelo ea Motheo. La 7th. Philadelphia, PA: Elsevier; 2018: khaolo ea 42.

Smith-Whitley K, Kwiatkowski JL. Hemoglobinopathies. Ka: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, bahlophisi. Buka ea Nelson ea Pediatrics. La 21 Ed. Philadelphia, PA: Elsevier; 2020: khaolo ea 489.