He thisen tlakchhamna leh kal lama harsatna chi mak tak hi eng nge ni? (Atypical Hemolytic Uremic Syndrome - aHUS) Kan sawi dawn nia!

Keimahni invenna system, immune system hian natna laka min humhim ai chuan keimahni taksa ngei hi rawn bei tan ta se eng nge thleng ang tih i ngaihtuah thei em? Chu chu vanduaithlak tak, mahse a tlem berah chuan, vawiin a kan sawi tur condition pakhat chu aHUS, emaw Atypical Hemolytic Uremic Syndrome . Hei hi kan thisen kalna kawng a chhiat a, thisen zam te te a lo awm a, kal ang chi taksa peng pawimawh tak takah thisen kal a tlahniam a ni. Hetiang dinhmun hi genetic mutation vang a ni fo thin .

Doctor-te chuan heng natna pathumte hi i neih dun vek hian aHUS i nei tih an hmuchhuak tlangpui thin:

• Microangiopathic hemolytic anemia : Chumi awmzia chu i thisen sen (red blood cells) te chu a chhe rang lutuk a, chuvangin i taksain a thlaktu tur cell thar a siam thei lo tihna a ni.
• Thrombocytopenia : Hei hi platelet tlahniam a ni a, chu chuan thisen a tikhawlo thei a ni.
• Acute kidney injury : Hei hi kidney failure chi khat a ni a, mahse a chang chuan reverse theih a ni.

Heng pathumte hi a rualin a thleng thei a, a nih loh leh natna dangin a tizual thei bawk.

Regular HUS leh he aHUS hi eng nge an danglamna?

Tun hma chuan he natna HUS (Hemolytic Uremic Syndrome) an tih hi chi hnih ah then a ni thin.

• Typical HUS (diarrhea-associated HUS): Hei hi diarrhea nen a inzawm fo thin. E. coli tih hming pu bacteria chi khat atanga lo chhuak a ni. Mi thenkhat chuan STEC-HUS an ti bawk.
• Tuna kan sawi aHUS (Atypical HUS): Hei hi ril\am chi khat hmuh tur a ni lo. A tam zawkah chuan aHUS vei zinga a chanve velin, chu chu genetic change, genetic mutation avanga lo awm a ni . Doctor-te chuan he natna hi complement-mediated thrombotic microangiopathy (CM-TMA) tiin an sawi \hin.

aHUS atana trigger bikte chu engte nge ni?

A awlsam zawngin, genetic mutation nei zawng zawng hian aHUS an nei vek dawn lo. Vawi tam tak chu hriselna lama harsatna dang avanga lo awm, a nih loh leh a zual zel a ni. Hêng thilte hi han ngaihtuah teh:

• Naupai hun chhung
• Ngawt
• Infection hrang hrang
• Damdawi thenkhat, a bik takin chemotherapy agents , immunosuppressive damdawi , thisen tithianghlimtu , oral contraceptives , leh anti-inflammatory damdawi thenkhat

aHUS natna lan chhuahna chu engte nge ni?

Tunah chuan aHUS vei hian eng symptom nge a lantir tih hi han en ila. Chûng zînga engemaw zât i nei thei a, a nih loh leh zawi zawiin a lan chhuah dân i nei thei bawk.

• Chauhna awm reng (Fatigue) .
• Vun dum (Pallor) .
• Nausea emaw, luak chhuak emaw
• Zun chhuah a tlahniam
• Zun chhungah thisen a awm
• Thisen sang (Hypertension) 1.1.
• Thâwk harsa (Dyspnea) .
• Edema a awm
• Confusion , hriatrengna hloh
• Khawsik

Mi tam zawk chuan heng symptoms hi vawi khatah an nei vek lo. A châng chuan zawi zawiin, zawi zawiin an rawn lut ṭhin. Hun engemaw chen chu thil engemaw i tuar tawh ang maiin i inhria mai thei a, mahse eng nge a nih i hre thei lo. Heng neurological symptoms, chu chu a hmaa ka sawi tawh ang khan buaina a ni a, mi zawng zawngah a thleng lo, a tlem deuh deuh.

Eng thilin nge aHUS hi a thlen tak tak?

aHUS lo awm chhan ber chu kan DNA inthlak danglamna (genetic mutation) , emaw, kan immune system- a ‘complement proteins’ te laka kan cell ngeia beitu autoantibodies siam a ni Heng genetic changes te hi kan nu leh pate atanga kan rochun theih a ni a, a nih loh leh random takin a thleng thei bawk. Heng genetic mutations te hian proteins ‘complement factors’ an tih te hnathawh a nghawng a ni.

Heng ‘complement factors’ te hi kan immune cells te puitu sipai te tak te angin han ngaihtuah teh. Chaw seasoning ang bawkin heng protein te hian natna hrik (bacteria leh virus ang chi) te chu an hmuchhuak a, immune cell dangte’n an ‘ei’ theih nan an mark a ni. aHUS-a a nghawng tam ber chu complement factor H, I, 3, leh B te an ni.

Entirnan, complement factor H hnathawh chu kan taksaa cell hriselte venhimna leh complement factor dangte mamawh loh huna activate lo tura ven hi a ni. Tunah chuan he complement factor H hian hna a thawk tha lo a nih chuan (chu chu CFH gene-ah mutation a awm a nih chuan) eng nge thleng ang tih han ngaihtuah teh ? Kan cell-te hian an mamawh venhimna an dawng lo. Tichuan hetiang hi a ni:

• Kan thisen kalna kawng (endothelial cells) chhunga line siamtu cell te hi kan immune cells te hian tihsualin an bei a, an tichhia a ni .
• He chhiatna chhehvelah hian platelet te chu an pungkhawm a, thisen zam (blood clot) an siam thin. Hei hian thisen kalna kawng a tikhawlo a ni.
• Hei hian kan taksa peng hrang hranga thisen kal a ti tlem a, a chhiat phah a, an hnathawh a tlahniam bawk. Kal (kidney) te hi a nghawng nasa ber a ni.
• Chûng thisen zamte chuan an kal tlanga thisen cell dangte a tichhe a, chu chuan hemolytic anemia tih natna a thlen a ni.

Engtin nge aHUS i nei tih i hriat theih ang?

Doctor chuan i natna lan chhuah dan leh i thisen zat leh i taksa peng hrang hrangte hnathawh dan enfiahna test result a zirin aHUS hi a hmuchhuak tlangpui ang. Tin, natna dang, a lan dan inang (a hmaa kan sawi tawh STEC-HUS ang chi) i neih loh nan test an ti bawk ang.

aHUS hriat theihna atan eng test nge hman thin?

Hetiang test hi i tih a ngai mai thei:

• Thisen test: Complete Blood Count (CBC) , kal hnathawh endik, etc.
• Genetic testing : Hei hian genetic mutations a awm leh awm loh a enfiah thin.
• Stool test : E. coli ang chi natna hrik awm leh awm loh enfiah thin ang che.
• Kidney biopsy : Kal atanga a tlem te te enfiah nan lak.
• Imaging test : ultrasound emaw CT scan emaw ang chi te .

Engtin nge aHUS hi enkawl a nih?

aHUS enkawl dan hi a chhan bulpui leh a natna nasat danah a innghat a ni. aHUS natna chi khat na lo zawk i neih chuan i doctor chuan a enkawl thei che a, a tul chuan thisen pek emaw, thisen sang damdawi emaw ang chi, ṭanpuitu enkawlna a pe thei che a ni.

Mahse, aHUS na tak i neih chuan hetiang hian enkawl i mamawh mai thei:

• Therapeutic plasma exchange : Hetah hian thisen plasma part chu lakchhuah a ni a, plasma thar pek a ni.
• Rituximab emaw damdawi dang autoimmune natna enkawlna atana hman thin.
• Dialysis : Kal a thawk tha lo a, thisen tihfai dan a ni.
• Kidney transplant : Hei hi natna khirh tak takah chauh tih a ni.

A chunga kan sawi tak ‘complement’ gene mutations nei tan chuan doctor-te chuan aHUS enkawlna atan damdawi eculizumab an hmang fo thin. Eculizumab hian complement protein thenkhat a block avangin hna a thawk a, i immune system hian nangmah ngeiin i thisen kalna a tichhe lo.

Enkawlna hian side effect a nei em?

Eculizumab i hman hian meningococcal leh pneumococcal vaccine lakah hian i him lo deuh mai thei. Chuvangin, a theih chuan i doctor chuan Eculizumab i tan hmain heng vaccine te hi lak hmasak phawt turin a fuih thei che a ni.

aHUS vei tan chuan hmalam hun eng nge awm?

He natna hi a hmaa hriatchhuah leh enkawl a nih chuan aHUS avanga acute kidney injury ang chi natna te hi hlawhtling taka enkawl theih a ni. Symptoms awm lovin remission period i nei thei bawk. Mahse, thil dangin aHUS a tichhuak leh a nih chuan nunna atana hlauhawm leh thei a ni.

Tun hma chuan aHUS vei zinga a chanve vel, a nih loh leh 50% vel chuan end-stage kidney disease (ESKD) , an nei a, chu chuan an kal a thawk tha lo vek tihna a ni.

Mahse, chanchin lawmawm a awm! Zirna thar chuan chiang takin aHUS vei te zingah ESKD vei theihna chu Eculizumab enkawlna avang hian nasa takin a tlahniam tih a tarlang.

aHUS avanga thihna hlauhawmna chu eng nge ni?

aHUS natna khirh tak tak, kal (kidney failure) emaw, taksa peng dang tihchhiat emaw te hi a pawi tak zet a ni. Chutiang natna khirh takah chuan thihna hlauhawmna chu 15% vel a ni.

aHUS hi ven theih a ni em?

Genetic changes avanga lo awm fo a nih avangin aHUS hi a tlangpuiin ven theih a ni lo. Mahse, natna khirh tak vei theihna i neih chuan, chu natna thlentu thil ṭhenkhat chu i pumpelh thei mai thei .

Engtin nge ka in enkawl ang?

aHUS i vei tih hmuhchhuah i nih chuan i doctor nen i natna tizual thei thilte pumpelh dan tur sawipui rawh. Tin, case na tak a nih chuan symptoms hriat a pawimawh bawk.

Engtikah nge Doctor ka hmuh ang?

Hun rei tak chhung i chau emaw, i chau emaw a nih chuan – natna khirh tak, reh lo tur i neih ang maia i awm chuan – i doctor nen inbia ang che. Tin, tun hnaia i symptom dang i neih tawh zawng zawng, i toilet hman dan inthlak danglam te pawh sawi bawk ang che.

Engtikah nge Emergency Treatment Unit (ETU) ah ka kal ang ?

Heng natna khirh tak takte hi i neih chuan emergency room-ah kal nghal rawh:

• Zun chhuah hi nasa takin a tlahniam a ni.
• Buaina emaw, hriatna tihdanglam emaw.
• A hring nasa hle.
• Thâwk harsa na tak.

Doctor hnenah eng zawhna nge ka zawh ang?

I doctor hnenah hetiang zawhna hi zawh hi a \angkai thei ang:

• Ka dinhmun hi engtiang chiahin nge a nasat?
• Eng thilin nge he thil hi a tizual?
• Nakin lawkah hetiang dinhmun hi ka veng thei ang em?
• Eng symptom nge ka fimkhur tur?
• Engtikah nge ka rawn hmu leh ang che?

Hetiang dinhmun hi engtiang chiahin nge aHUS tia koh a nih?

aHUS hi a tlem hle.Damdawi lam dinhmun a ni. Mi sang nga zinga pakhat velin a nghawng a ni. Naupang aiin puitlingah a tam zawk.

A tawp berah chuan thil hriat reng tur

Nangmah emaw, i fa emaw hian genetic condition i nei tih hriat chu nun tidanglam thei thil a ni thei. aHUS hi natna khirh tak a ni a, thisen zam a tichhe thei a, taksa peng hrang hrang a tichhe thei bawk. Mahse, tun hnaia damdawi eculizumab an tihchhuah hian aHUS vei damchhuahna kawngah nasa takin hma a sawn a ni. aHUS i vei tih hmuhchhuah a nih chuan i doctor nen triggers laka inven dan tur leh natna khirh tak i vei theihna tur tihtlem dan tur sawiho rawh. Ani chuan hrisel taka awm tûra i tih tûr chu a hriatthiam theih nân a ṭanpui thei che a ni.

` aHUS, kal natna, thisen zam, immune system, genetic mutations, thisen tlakchhamna, Eculizumab