Thalassemia

ʻO Thalassemia kahi maʻi koko i iho ʻia i waena o nā ʻohana (hoʻoilina) kahi e hana ai ke kino i kahi ʻano kūpono ʻole a lawa ʻole paha o ka hemoglobin. ʻO Hemoglobin ka protein i loko o nā hunaola ʻulaʻula e lawe i ka oxygen. Hoʻopau ka maʻi i nā nui o nā hunaola ʻulaʻula e luku ʻia, a hiki i ka anemia.

Hana ʻia ʻo Hemoglobin i nā protein ʻelua:

• ʻAlima globin
• Beta globin

Hana ʻia ʻo Thalassemia ke loaʻa kahi hemahema i kahi ʻāpana e kōkua i ka mālama ʻana i ka hana ʻana i kekahi o kēia mau protein.

ʻElua ʻano nui o thalassemia:

• Kū ʻia ʻo Alpha thalassemia ke nalowale a loli ʻia paha kahi ʻāpana a i ʻole nā ​​ʻāpana e pili ana i ka protein alpha globin (mutated).
• Hana ʻia ka beta thalassemia ke hoʻopili nā hemahema ʻē aʻe i ka hana o ka protein beta globin.

Kū pinepine ʻo Alpha thalassemias i ka poʻe mai Hikina Hema ʻo ʻAsia, ka Hikina Waena, Kina, a me nā poʻe o nā kūpuna ʻApelika.

Kū pinepine nā thalassemias beta i ka poʻe o ke kumu Mediterranean. I kahi liʻiliʻi, hiki i Kina, nā ʻAsia ʻē aʻe, a me ʻAmelika ʻApelika ke hoʻopili ʻia.

Nui a hewahewa ke ʻano o ka thalassemia. Nui nā ʻano subtypes like ʻole o kēlā me kēia ʻano. ʻO nā alpha a me ka beta thalassemia e hoʻopili i nā ʻano ʻelua aʻe:

• Thalassemia nui
• ʻO Thalassemia ʻōpio

Pono ʻoe e hoʻoilina i ka ʻāpana kīnā mai nā mākua ʻelua e hoʻomohala i ka thalassemia nui.

Hana ʻia ʻo Thalassemia ʻōpio inā loaʻa iā ʻoe ka ʻaoʻao hewa mai hoʻokahi makua wale nō. ʻO nā poʻe me kēia ʻano o ka maʻi he hāpai i ka maʻi. ʻO ka hapa nui o ka manawa, ʻaʻohe o lākou hōʻailona.

Kapa ʻia ka beta thalassemia nui ʻo Cooley anemia.

ʻO nā kumu pilikia no ka thalassemia me:

• ʻO ʻAsia, Kina, Kaiwaenahonua, a i ʻole ʻAmelika ʻAmelika
• ʻO ka moʻolelo ʻohana o ka maʻi

ʻO ke ʻano koʻikoʻi o ka alpha thalassemia nui ke kumu o ka hānau hānau ʻana (make ʻana o ka pēpē i hānau ʻole ʻia a i ʻole nā ​​hopena hope o ka hāpai ʻana).

ʻO nā keiki i hānau ʻia me ka beta thalassemia nui (Cooley anemia) maʻamau i ka wā hānau, akā hoʻomohala i ka anemia koʻikoʻi i ka makahiki mua o ke ola.

Hiki i nā ʻōuli ʻē aʻe ke hoʻopili pū me:

• ʻO nā deformities o ka iwi i ka maka
• Ka luhi
• Hewa ulu
• Pokole o ka hanu
• ʻIli melemele (jaundice)

ʻO ka poʻe me ka liʻiliʻi o ke ʻano alpha a me beta thalassemia he mau puʻuwai koko ʻulaʻula akā ʻaʻohe ʻōuli.

E hana i kāu mea mālama ola i kahi hoʻokolohua kino e nānā i kahi āpau hoʻonui.

E hoʻouna ʻia kahi laʻana koko i kahi keʻena hoʻokolohua e hoʻāʻo ai.

• E ʻike ʻia nā hunaola ʻulaʻula a liʻiliʻi a ʻano hewa hoʻi ke nānā ʻia ma lalo o kahi microscope.
• Hōʻike kahi helu piha koko (CBC) i ka anemia.
• Hōʻike kahi hōʻike i kapa ʻia he hemoglobin electrophoresis i ke alo o kahi ʻano hemoglobin.
• Hiki i kahi hoʻokolohua i kapa ʻia ʻo ka mutational analysis ke kōkua i ka ʻike ʻana i ka alpha thalassemia.

ʻO ka mālama ʻana no ka thalassemia nui e pili pinepine ana i nā transfusions o ke koko maʻamau a me nā mea kōkua folate.

Inā loaʻa ʻoe i ke koko, ʻaʻole pono ʻoe e lawe i nā mea hoʻohui hao. ʻO ka hana ʻana pēlā ke kumu o ka nui o ka hao e kūkulu i loko o ke kino, kahi e hōʻino ai.

ʻO ka poʻe i loaʻa ka nui o nā kahe o ke koko e pono i kahi lāʻau i kapa ʻia ʻo chelation therapy. Hana ʻia kēia e wehe i ka hao nui mai ke kino.

Hiki paha i kahi transplant pūpū iwi ke kōkua i ka mālama ʻana i ka maʻi i kekahi poʻe, keu hoʻi i nā keiki.

Hiki i ka thalassemia koʻikoʻi ke kumu o ka make mua (ma waena o 20 a 30 mau makahiki) ma muli o ka maikaʻi ʻole o ka puʻuwai. ʻO ka loaʻa pinepine ʻana o ke kahe o ke koko a me ka hoʻōla e hoʻoneʻe i ka hao mai ke kino e kōkua ai i ka hoʻomaikaʻi ʻana i ka hopena.

ʻO nā ʻano ʻoi loa o ka thalassemia ʻaʻole e hoʻopōkole i ke ola.

Makemake paha ʻoe e ʻimi i nā ʻōlelo aʻoaʻo kūpuna inā he moʻolelo ʻohana kāu o ke ʻano a ke manaʻo nei ʻoe he keiki kāu.

ʻAʻole mālama ʻia, ka thalassemia nui e alakaʻi i ka puʻuwai puʻuwai a me nā pilikia o ke ake. Hoʻolālā paha ia i kahi maʻi i nā maʻi.

Hiki i ke koko ke kōkua i ke kaohi ʻana i kekahi mau hōʻailona, ​​akā lawe i kahi hopena o nā hopena ʻaoʻao mai ka nui o ka hao.

Kāhea i kāu mea hāʻawi inā:

• ʻO ʻoe a ʻo kāu keiki paha he mau ʻōuli o ka thalassemia.
• Mālama ʻia ʻoe no ka maʻi a ulu nā ʻōuli hou.

Anemia Kaiwaenahonua; Cooley anemia; Beta thalassemia; ʻO thalassemia Alpha

• Thalassemia nui
• ʻO Thalassemia ʻōpio

ʻO Cappellini MD. ʻO nā thalassemias. I: Goldman L, Schafer AI, eds. ʻO ka lāʻau lapaʻau Goldman-Cecil. 26th ed. Piladelapia, PA: Elsevier; 2020: chap 153.

Chapin J, Giardina PJ. Nā syndrome Thalassemia. I: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Nā Kumumanaʻo a me ka Hoʻomeheu. ʻEpekema 7 Piladelapia, PA: Elsevier; 2018: chap 40.

Smith-Whitley K, Kwiatkowski JL. Hemoglobinopathies. I: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. ʻO Puka Textbook of Pediatrics ʻo Nelson. 21st ed. Piladelapia, PA: Elsevier; 2020: chap 489.