"Anemia" emaw "anemia" emaw tih hi i hre tawh ngei ang a dik em? A awlsam zawngin kan taksain thisen zam (red blood cells) a siam that tawk loh emaw, thisen sen (red blood cells) kan neihte hian hna a thawk tha lo emaw a ni. Kan taksa pum puia oxygen phurtu ber chu thisen sen (red blood cells) a ni. Chuvangin an hniam lai hian kan chau thei a, kan chau thei reng bawk. Heng thisen sen (red blood cells) te hi kan ruh chhunga tissue nem tak, spongy ang maia awm, ruhro (bone marrow) ah a lo piang chhuak thin a ni. Diamond-Blackfan Anemia (DBA) hi thisen tlakchhamna chi khat bik a ni a, ruhro (bone marrow) hian thisen sen (red blood cells) a siam chhuah tlem chuan a awm thin.
Eng thilin nge Diamond-Blackfan Anemia (DBA) hi a thlen?
Diamond-Blackfan Anemia, a then chuan ``acquired pure red cell aplasia'' an tih hi naupang kum khat a tlin hmain doctor-te chuan an hmuchhuak tlangpui. Hei hi a chhan ber chu kan taksa siamtu genes inthlak danglamna, a nih loh leh mutations a ni.
A châng chuan he genetic defect hi nu emaw, pa emaw aṭangin a rochun thei tih hre reng ang che. Mahse chutiang chu a ni reng reng lo. Naupang thenkhat genes pawh hi chhan awm lovin a takin a inthlak thei bawk. DBA vei naupang tam zawk chuan he natna hi an nei a, a chhan chu genetic mutation thar a awm vang a ni. Chuvangin nu leh pa kan nih angin he thil hi tul lova lungngai a ngai lo. DBA vei zinga a chanve vel chu genetic cause bik hmuhchhuah ni mah se, mi thenkhat tan chuan he natna hi a chhan bik hmuhchhuah a la ni lo.
DBA natna lan chhuah dan chu engte nge ni?
DBA vei naupangte pawhin thisen tlakchhamna chi dang chhinchhiahna tlanglawn tam tak an nei thei bawk. Chu chu,
- Chauhna awm reng
- Vun dum (pale skin) a ni
- Chak lo anga inhriatna
Heng thil tlangpui bakah hian DBA vei naupang thenkhat chuan an hmai leh taksaah pawn lam thil chiang tak tak an nei thei bawk. Eng nge an nih i han thlir teh ang.
| Taksa peng pakhat | Hmuh theih thil chi hrang hrang |
|---|---|
| Lu | Lu pangngai aia te zawk neih. |
| Hmai | Mit inkar hlat zawng tihpun leh hnar flat tak. |
| Beng | Beng te tak te pahnih, a tlangpui aia hniam zawka set. |
| Khabe | A hnuai lam jaw te tak te nei. |
| Nghawng | A kawr tawi, webbed tak a ni. |
| A kekawrte | Shoulder blade te te te. |
| Kut a ni | Thumbs pianzia danglam tak. |
| Ka | Palate emaw hmui emaw cleft nei. |
Chu bakah, DBA hian kal lama harsatna , thinlung chhiatna , leh mit lama harsatna, mitdel leh glaucoma te a thlen thei bawk. Mipa naupangte pawhin piansualna hypospadias an tih chu an nei thei a, chu chu zun kawng hawnna chu penis tip-ah a awm loh lai a ni.
He natna hi engtin nge dik taka kan hriat theih ang?
I doctor hian i fain DBA a nei em tih hriat nan test eng emaw zat a ti thei a ni. DBA vei naupangte hian red blood cell count an nei tlem a, mahse white blood cell leh platelet count pangngai an nei thung.
A pawimawh ber chu heng symptoms te hi i hmuh hian hlauthawng lo la, a rang thei ang bera doctor thiam tak pan hi a ni. Test tulte a ti ang a, thu dik ber ber a pe ang che.
Hengte hi doctor-in test a tih ber thenkhatte an ni.
| Fiahna | Eng nge a zawn? |
|---|---|
| Thisen chhiar kim (CBC) 1.1. | Thisenah hian thil tam tak teh a ni a, chu chu thisen sen zat, thisen var, platelet, oxygen kengtu protein hemoglobin, leh thisen sen (hematocrit) volume te hi a ni. |
| Reticulocyte chhiar chhuah a ni | Hei hian thisen sen puitling lo, a nih loh leh siam thar zat a teh a ni. Hei hian ruhro hian red cell a siam tha em tih a hriat theih. |
| eADA level enfiah a ni | DBA vei zinga 80% vel chuan enzyme pakhat erythrocyte adenosine deaminase (eADA) an tih chu a sang hle a ni. Hei hi he test hian a zawn chu a ni. |
| Nausen chhunga hemoglobin level awm zat | Hei hi nausen pum chhunga hemoglobin chi khat a ni. Pian hnuah a tlahniam tur a ni. Mahse, DBA vei naupangte chu an lo puitlin chuan a sang chho thei tho. |
| Bone marrow aspiration leh biopsy hmanga tih a ni | Ruhro atang hian cell leh tui tlemte chu needle hmangin lakchhuah a ni a, test a ni. DBA vei naupangte hian an ruhroah thisen sen hrisel tak tak an nei tlem hle. |
| Genetic test neih a ni | Hei hian DBA emaw, nu leh pate hnen atanga rochun thisen tlakchhamna dang emaw nena inzawm genes a test thin. |
DBA vang hian eng harsatna dang nge awm thei?
DBA vei te hian natna leh dinhmun thenkhat an vei theihna chance a sang deuh hlek a ni. Chung zingah chuan thisen leh ruhro cancer (acute myeloid leukemia), ruh cancer (osteosarcoma), leh ruhroin thisen cell hrisel a siam theih loh natna dang (myelodysplastic syndrome) te a tel. Mahse hei hi ngaihtuah buai suh. Doctor ten hei hi an vil reng a, chuvangin a hun takah damdawi lam hmalakna tul tak tak i la thei a ni.
Hei hi eng enkawlna nge ni?
Hei hi a pawimawh ber leh thlamuanthlak ber a ni. Naupang DBA vei nia hriatte chu damdawi hmanga enkawl dam rei tak, hlawhtling takin an nung thei a ni. Naupang thenkhat chu complete remission-ah an kal a, chu chu hun engemaw chen chu symptoms a bo tihna a ni.
Enkawlna hman ber pahnih chu corticosteroid damdawi leh thisen pek hi a ni.
- Corticosteroid damdawi: `(Prednisone)` ang damdawi hian ruhro a tichak a, thisen sen tam zawk siam turin a pui bawk.
- Thisen pek: Hei hi steroid damdawi a hlawhtlin loh chuan, emaw, thisen tlakchhamna a nasat chuan duhthlan tur tha tak a ni. Hei hian naupang thisen emaw, thisen sen emaw petu hrisel tak hnen atanga pek a huam a ni.
- Bone marrow/stem cell transplant: Hei hi DBA damdawi awmchhun a ni. Mahse, a risky deuh. Tin, matching donor hmuh pawh a harsa thei bawk.
Heng enkawlna zawng zawng te, a thatna leh that lohna te hi chiang takin i doctor nen sawiho a, i fa tana tha ber tur thlan a pawimawh hle.
Take-Home Thuchah a ni
- Diamond-Blackfan Anemia (DBA) hi naupangte zingah chuan ruhro (bone marrow) hian thisen sen (red blood cells) a siam tling thei lo a, hei hi genetic condition a awm lo hle.
- He natna hian natna lan chhuah dan tlangpui, hah fo leh vun rawng hring (paleness) te bakah hian hmai leh taksaah pawn lam thil chiang tak tak a siam thei bawk.
- Doctor chuan thisen test leh ruhro examination hmangin he natna hi dik takin a hre thei a ni.
- Steroid damdawi leh thisen pek ang chi enkawlna hian naupangte chu dam rei tak, hrisel takin a pui thei a ni. Bone marrow transplant hi damdawi awmchhun a ni.
- I fa hian heng symptoms te hi a nei tih rinhlelhna tlemte pawh i neih chuan hlauthawng suh la , naupang doctor pan nghal rawh. Hriat chian thuai leh enkawl hi a pawimawh hle.
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