Thalassemia

Thalassemia ke lefu la mali le fetisoang ke malapa (a futsitsoeng) moo 'mele o etsang sebopeho se sa tloaelehang kapa hemoglobin e sa lekaneng. Hemoglobin ke protheine e lisele tse khubelu tsa mali tse tsamaisang oksijene. Boloetse bona bo baka hore lisele tse khubelu tsa mali li senyehe, e leng se lebisang khaello ea mali.

Hemoglobin e entsoe ka liprotheine tse peli:

• Alpha globin
• Globin ea Beta

Thalassemia e hlaha ha ho na le sekoli liphatseng tsa lefutso se thusang ho laola tlhahiso ea e 'ngoe ea liprotheine tsena.

Ho na le mefuta e 'meli e meholo ea thalassemia:

• Alpha thalassemia e etsahala ha liphatsa tsa lefutso kapa liphatsa tsa lefutso tse amanang le protheine ea alpha globin li haella kapa li fetoloa (li fetotsoe).
• Beta thalassemia e hlaha ha liphoso tse tšoanang tsa lefutso li ama tlhahiso ea protheine ea beta globin.

Alpha thalassemias e etsahala hangata ho batho ba tsoang Asia Boroa-bochabela, Middle East, China le ho ba litloholo tsa Afrika.

Beta thalassemias e etsahala hangata ho batho ba tsoang Mediterranean. Hanyane ka hanyane, Machaena, Maasia a mang le Maafrika a Amerika a ka ameha.

Ho na le mefuta e mengata ea thalassemia. Mofuta o mong le o mong o na le likaroloana tse fapaneng tse fapaneng. Alpha le beta thalassemia li kenyelletsa mefuta e 'meli e latelang:

• Thalassemia e kholo
• Thalassemia e nyane

U tlameha ho rua bofokoli ba lefutso ho batsoali ka bobeli ho nts'etsapele thalassemia e kholo.

Thalassemia e nyane e hlaha haeba o amohela mofuta o fosahetseng ho motsoali a le mong feela. Batho ba nang le mofuta ona oa lefu lena ke bajari ba lefu lena. Boholo ba nako, ha ba na matšoao.

Beta thalassemia e kholo e boetse e bitsoa Cooley anemia.

Lisosa tse kotsi tsa thalassemia li kenyelletsa:

• Morabe oa Asia, China, Mediterranean kapa Afrika Boroa
• Nalane ea lelapa ea lefu lena

Mofuta o matla ka ho fetesisa oa alpha thalassemia o baka tsoalo ea lefu (lefu la lesea le e-s'o hlahe nakong ea peleho kapa nakong ea bokhachane).

Bana ba hlahileng ba e-na le beta thalassemia e kholo (Cooley anemia) ba tloaelehile nakong ea tsoalo, empa ba ba le phokolo ea mali e matla selemong sa pele sa bophelo.

Matšoao a mang a kenyeletsa:

• Ho holofala ha masapo sefahlehong
• Mokhathala
• Ho hloleha ho hola
• Phefumoloho e khutšoanyane
• Letlalo le mosehla (jaundice)

Batho ba nang le mofuta o monyane oa alpha le beta thalassemia ba na le lisele tse khubelu tsa mali empa ha ba na matšoao.

Mofani oa hao oa tlhokomelo ea bophelo o tla etsa tlhahlobo ea 'mele ho batla spleen e atolositsoeng.

Sampole ea mali e tla romelloa laboratoring ho ea hlahlojoa.

• Lisele tse khubelu tsa mali li tla hlaha li le nyane li bile li bopehile ka mokhoa o sa tloaelehang ha li shejoa ka tlas'a microscope.
• Palo e felletseng ea mali (CBC) e senola phokolo ea mali.
• Teko e bitsoang hemoglobin electrophoresis e bonts'a boteng ba mofuta o sa tloaelehang oa hemoglobin.
• Teko e bitsoang tlhahlobo ea phetoho e ka thusa ho fumana alpha thalassemia.

Kalafo ea thalassemia e kholo hangata e kenyelletsa ho tšeloa mali khafetsa le litlatsetso tsa folate.

Haeba u amohela litšelo tsa mali, ha ua lokela ho noa lisebelisoa tsa tšepe. Ho etsa joalo ho ka baka tšepe e ngata haholo 'meleng, e ka bang kotsi.

Batho ba fumanang litšelo tse ngata tsa mali ba hloka kalafo e bitsoang chelation therapy. Sena se etsoa ho tlosa tšepe e fetelletseng 'meleng.

Ho kenyelletsa 'moko oa masapo ho ka thusa ho alafa lefu lena ho batho ba bang, haholo bana.

Thalassemia e matla e ka baka lefu la pele ho nako (lipakeng tsa lilemo tse 20 le 30) ka lebaka la ho hloleha ha pelo. Ho fumana litšelo tsa mali khafetsa le kalafo ea ho tlosa tšepe 'meleng ho thusa ho ntlafatsa sephetho.

Mefuta e fokolang haholo ea thalassemia hangata ha e khutsufatse nako ea bophelo.

U kanna ua batla ho fumana tlhabollo ea lefutso haeba u na le nalane ea lelapa ka boemo bona mme u nahana ho ba le bana.

Ha e sa alafatsoe, thalassemia e kholo e lebisa ho hloleha ha pelo le mathata a sebete. E boetse e etsa hore motho a be le monyetla oa ho ba le tšoaetso.

Litšelo tsa mali li ka thusa ho laola matšoao a mang, empa li na le kotsi ea litla-morao tse tsoang ho tšepe e ngata haholo.

Bitsa mofani oa hau haeba:

• Uena kapa ngoana oa hau le na le matšoao a thalassemia.
• U ntse u alafshoa lefu lena 'me matšoao a macha a hlaha.

Phokolo ea mali ea Mediterranean; Phokolo ea mali e pholileng; Beta thalassemia; Alpha Thalassemia

• Thalassemia e kholo
• Thalassemia e nyane

MD ea Cappellini. Thalassemias. Ka: Goldman L, Schafer AI, li-eds. Phekolo ea Goldman-Cecil. La 26th. Philadelphia, PA: Elsevier; 2020: khaolo ea 153.

Khaolo J, Giardina PJ. Li-syndromes tsa Thalassemia. 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 40.

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.