He kal natna hmuh tur awm lo tak hi i hria em? (Atypical Hemolytic Uremic Syndrome - a HUS) a ni a, a rilru a hah lutuk a, a rilru a buai em em bawk a.

I taksaa thisen kalna kawng te tak te chhungah thisen zam a lo awm tih han mitthla teh. A bik takin i kal chhunga thisen kalna kawngah. Heng thisen zamte hian i kal (kidney)-a thisen kal tur a tihkhawtlai hian kal (kidney failure) ang chi harsatna lian tak tak a thleng thei a ni. Chu chu Atypical Hemolytic Uremic Syndrome, aHUS-ah pawh a thleng thin. Hetiang natna hi damdawi awm lo mahse, enkawlna dik tak hmangin a enkawl tha thei a ni.

"Typical" leh "Atypical" HUS hi eng nge an danglamna?

He dinhmun, aHUS tia koh hi kan hmuh fo thin "Typical" HUS nen hian a danglam deuh hlek a ni. Typical HUS awm chhan ber chu bacteria chi hrang hrang, diarrhea thlentu a ni a, entirnan, bacteria infection `(E. coli)` leh `(Shigella)` te hi a ni. Mahse, aHUS awm chhan hi a buaithlak zawk.

He natna, aHUS an tih hi kum eng pawhah a awm thei. Naupang tê têah chuan mipa leh hmeichhia a nghawng vek a ni. Amaherawhchu, an kum a upat deuh deuh chuan hmeichhiate hian he natna hi an vei nasa zawk a ni. Hei hi a chhan chu naupai hi natna lo lang chhuaktu a nih theih vang a ni. Natna hmuh tur awm lo tak a ni. He natna hi mi engzat nge vei tih chiang taka hriat ni lo mah se, zirchianna thenkhatah chuan United States ang ramah chuan mi maktaduai khat zinga pahnih chauhvin he natna hi an vei niin an sawi.

Eng thilin nge aHUS a thlen?

aHUS case tam zawk hi genes inthlak danglamna avanga lo awm a ni a, chu chu gene mutation an ti a ni. Mahse, gene mutation nei zawng zawng hian he natna hi an nei vek dawn lo. Symptoms a lan theih nan trigger a ngai a ni.

A awlsam zawngin sawi ila, he natna hi genetically predisposed mi chuan a hnuaia thil pakhat a thlen chuan he natna hi a nei thei a ni:

• Naupai lai
• Hri kai theihna thenkhat
• Ngawt
• Damdawi thenkhat
• Organ transplant pakhat a ni
• Natna dang rei tak, entirnan ``systemic sclerosis`` emaw ``malignant hypertension`` emaw

Mahse a chang chuan aHUS vei zinga a chanve vel ah chuan doctor te hian an hmuh theih tur genetic mutation an nei lo. Heng case te hi ``idiopathic'' kan ti a, chu chu a chhan hmuh tur a awm lo tihna a ni. Researcher-te chuan he natna hi genetic mutation hriat chian loh avanga lo awm niin an ngai.

aHUS natna lan chhuahna chu engte nge ni?

aHUS symptoms hi trigger hnuah a lang nghal tlangpui. Heng flare-up te hi a na lo thei hle a, a na lo thei bawk. A nasat loh chuan kal (kidney) hi a nghawng vak lo thei bawk. A nasat chuan kal (kidney failure) an thlen thei a ni.

A tir lamah chuan heng ang symptoms hi i hmu thei ang:

• Chak lohna nasa tak leh nunna nei lo
• A tlangpuiin hrisel lohna neia inhriatna

Hei hi natna zual zel a ni. Chumi awmzia chu hun kal zelah symptoms a zual zel tihna a ni a, a bik takin natna hi a hmaa hriat chhuah a nih loh chuan a na zual zel tihna a ni. AHUS hian kal (kidney) hi a tum ber ni mahse, thisen kalna kawng a inkharkhip hian taksa peng dang thluak, thin, lung leh thinlung te pawh a nghawng thei bawk.

Engtin nge aHUS hi hmuhchhuah a nih?

I chhungte zingah tumahin he natna hi an la vei lo a nih chuan aHUS hriatchhuah a harsa deuh thei. Doctor, kal natna (nephrologist) emaw, thisen natna lama tui (hematologist) emaw chu a hriat theih nan a tha ber. Heng chhinchhiahna pathumte hi an zawng ber ang:

1. Hemolytic thisen tlakchhamna (Hemolytic anemia).

2. Platelet count a tlahniam

3. Kidney function lama harsatna nei

I doctor chuan he natna hi finfiah turin test hrang hrang a ti thei ang.

Enkawlna kawng hrang hrangte chu engte nge ni?

aHUS enkawlna atana FDA-in a pawmpui damdawi chi hnih a awm a.

• `Eculizumab (Soliris) tih a ni
• `Ravulizumab-cwcz (Ultomiris)` tih a ni

Heng damdawi pahnih hi ``monoclonal antibodies'' tih pawla mi an ni. Heng damdawi te hian thisenah platelet leh thisen sen (red blood cells) a tipung thei a ni. He enkawlna hi natna bul tan tirh a nih chuan kal chhiatna pawh a tidanglam thei a ni.

Heng damdawi te hi injection angin pek thin a ni. Mahse, side effects, lu na, khawsik, luak chhuak, leh taksa na te an thlen thei a ni. Hengte hi i doctor chuan a hriattir ang che.

Hei bakah hian plasma therapy hmangin symptoms enkawl nan an hmang bawk. Hemiah hian damlo chu mi hrisel atanga lak plasma pek emaw, damlo thisen atanga plasma lakchhuah a, plasma hrisel hmanga thlak emaw a ni.

Heng enkawlnate hi kal (kidney)-in a chhan loh chuan kal (kidney dialysis) emaw, kal (kidney transplant) emaw tih a ngai mai thei.

Damdawi ina kal nghal vat a ngaih huna thil awm dan

A hnuaia chhinchhiahna na tak tak te hi a lo lang a nih chuan damdawi in hnai ber Emergency Department (ETU)-ah kal nghal tur a ni.

• Zun chhuah a tlem hle.
• Buaina emaw, rilru fiah lohna emaw.
• Taksa a hring nasa hle.
• Thâwk harsa.

Natna nena kan nunpui lai hian engtin nge rilru hriselna kan vawn that theih ang?

aHUS ang natna khirh tak nena kan nun hian stress leh lungkhamna a awm fo thin. Natna a nasat poh leh symptom i neih tam poh leh i rilru hriselnaah nghawng a nei nasa zawk thin. Mahse hei hi nangmah chauhin i hmachhawn a ngai lo.

• I chhûngte leh i ṭhiante nêna inbiak rawh: I rintlak mi hnêna i ngaihtuahna leh rilru put hmang i hrilh chuan thlamuanna nasa tak a pe thei a ni.
• Doctor leh counselor te nen inbia: Hemi chungchang hi i doctor nen inbia ang che. A tul chuan mental health counselor hnenah an refer thei che a ni.
• I hriselna zawng zawng kha han ngaihtuah teh: Mut tam rawh. Stress tihziaawmna tur kawng zawng rawh. I kal tan a tha tur diet ei rawh. I theih chuan exercise engemaw zat la rawh. Taksa hrisel nih hian rilru hrisel taka awm reng turin a pui bawk.

Hei hi dam chhung zawnga awm thin ni mahse, a hmaa hriatchhuah te, enkawl dan dik te , leh damdawi lam thurawn zawm te chuan he natna hian a nghawng dan hi i enkawl thei a, nun pangngaiin i nung thei a ni. Chuvangin, i doctor nen inzawmna nei reng la, i enkawlna tur ruahmanna chu zawm rawh.