Tus Sau: William Ramirez
Hnub Kev Tsim: 22 Lub Cuaj Hli Ntuj 2021
Hloov Hnub: 11 Tau 2024
Anonim
Hereditary Spherocytosis (HS)
Daim Duab: Hereditary Spherocytosis (HS)

Cov kab mob ntshav liab (Heheitary spherocytic anemia) yog ib qho kev tsis sib haum xeeb ntawm cov txheej txheem saum npoo av (cov qog) ntawm cov ntshav liab. Nws ua rau cov qe ntshav liab uas zoo li tus kheej, thiab ua ntej qog ntawm cov ntshav liab (hemolytic anemia).

Qhov kev tsis zoo no yog tshwm sim los ntawm ib qho kev tsis xws luag muaj. Qhov tsis xws tshwm sim ua rau cov qe ntshav liab tsis txawv txav. Cuam tshuam cov hlwb muaj thaj tsam me dua rau lawv lub ntim ntau dua li cov ntshav liab, thiab tuaj yeem tawg qhib yooj yim.

Qhov mob ntshav liab tuaj yeem hloov ntawm qhov mob sib khuav mus rau mob hnyav. Nyob rau hauv cov mob loj heev cov kev tsis sib haum xeeb muaj peev xwm pom nyob rau ntawm thaum yau. Muaj qee tus mob nws yuav ploj mus txog thaum tsis tiav.

Qhov kev tsis zoo no feem ntau yog cov neeg ntawm qaum teb chaws Europe, tab sis nws tau pom muaj nyob hauv txhua haiv neeg.

Cov menyuam mos yuav muaj tawv nqaij daj thiab tawv muag (daj daj) thiab xim daj ntseg (pallor).

Lwm cov tsos mob tuaj yeem suav nrog:

  • Nkees
  • Vauv
  • Ua tsis taus pa
  • Neeg tsis muaj zog

Feem ntau, qhov hnoos qeev tau nthuav dav.

Kev kuaj hauv tsev kho mob tuaj yeem pab txheeb xyuas tus mob no. Kev ntsuam xyuas yuav muaj xws li:


  • Ntshav pleev qhia kom pom lub hlwb tsis zoo li lub cev
  • Bilirubin qib
  • Ua tiav ntshav suav los kuaj xyuas tus ua haujlwm tsis txaus
  • Coombs kuaj
  • LDH qib
  • Osmotic tsis muaj zog lossis kuaj tshwj xeeb los ntsuas rau cov ntshav liab lub cev tsis xws luag
  • Reticulocyte suav

Kev phais mob kom tshem tau tus po (splenectomy) kho cov ntshav liab tab sis tsis kho cov cell txawv txav.

Cov tsev neeg uas muaj keeb kwm mob spherocytosis yuav tsum coj lawv cov menyuam mus kuaj tus mob no.

Cov menyuam yaus yuav tsum tos kom txog hnub nyoog 5 xyoos kom muaj pob txha caj qaum vim muaj kev pheej hmoo kis mob. Hauv cov mob me me tau tshawb pom hauv cov neeg laus, nws tsis tsim nyog los tshem tus po.

Cov menyuam yaus thiab cov neeg laus yuav tsum tau txhaj tshuaj tiv thaiv kab mob ntsws ua ntej kev phais mob hnoos qeev. Lawv kuj yuav tsum tau txais cov tshuaj folic acid. Cov tshuaj tiv thaiv ntxiv yuav xav tau raws tus neeg li keeb kwm.

Cov khoom siv hauv qab no tuaj yeem muab cov ntaub ntawv qhia ntxiv ntawm cov kab mob pob txha caj qaum:

  • Chaw qhia txog kab mob thiab caj ces tsis raws cai - rarediseases.info.nih.gov/diseases/6639/hereditary-spherocytosis
  • Koom Haum Koom Tes rau Cov Kab Mob Tsis Txaus - rarediseases.org/rare-diseases/anemia-hereditary-spherocytic-hemolytic

Qhov tshwm sim feem ntau zoo nrog kev kho mob. Tom qab tus po raug tshem tawm, txoj sia ntawm cov qe ntshav liab rov qab coj li qub.


Teeb meem yuav suav nrog:

  • Gallstones
  • Ntau cov qe ntshav liab qis dua (ua rau muaj teebmeem txaus) tshwm sim los ntawm cov kab mob khaub thuas, uas tuaj yeem ua rau mob ntshav qis dua

Hu koj tus kws kho mob yog hais tias:

  • Koj cov tsos mob mob heev dua.
  • Koj cov tsos mob tsis zoo nrog kev kho tshiab.
  • Koj tsim cov tsos mob tshiab.

Qhov no yog ib qho qub txeeg qub teg tsis zoo thiab tej zaum yuav tsis tiv thaiv. Paub txog koj qhov kev pheej hmoo, xws li tsev neeg keeb kwm ntawm kev puas tsuaj, tuaj yeem pab koj txheeb thiab kho ntxov.

Congenital spherocytic hemolytic anemia; Spherocytosis; Hemolytic anemia - spherocytic

  • Cov qe ntshav liab - ib txwm
  • Cov ntshav liab - spherocytosis
  • Cov qe ntshav

Gallagher PG. Cov kab mob ntshav liab rau lub cev. Hauv: Hoffman R, Benz EJ, Silberstein LE, li al, eds. Hematology: Qhov Ntsiab Cai thiab Kev Coj UaCov. 7 tus ed. Philadelphia, PA: Elsevier; 2018: chap 45.


Merguerian MD, Gallagher PG. Cov kab mob txha caj qaum. Hauv: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Phau Ntawv ntawm PediatricsCov. 21st ed. Philadelphia, PA: Elsevier; 2020: cha 485.

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