I doctor-in nangmah emaw, i fate emaw chuan kal lama harsatna i neih thu a hrilh che a, chutih rual chuan hming sei tak, buaithlak tak `Complement 3 Glomerulopathy` tih ang chi a hrilh che hi thil pangngai a ni. Hei hi a tawi zawngin `C3G` an ti a. Hei hi kal natna hmuh tur awm lo deuh a ni. He natna hian naupang leh puitling tleirawlte a nghawng tam ber nachungin, a takah chuan kum eng pawhah a lo awm thei a ni. Chuvangin vawiinah hian he natna C3G an tih hi, kan thianpa nen kan inbia ang maiin, awlsam takin kan sawi dawn nia.
He C3G hi eng nge ni?
A awlsam zawngin sawi ila, C3G hi kal natna a ni a, chu chu "complement system" avanga lo awm a ni a, chu chu kan taksa immune system-a tel a ni a, hna a thawk tha lo. He complement system hi kan taksaa lut natna hrik leh bacteria te dotu police force ang a ni. Mahse, C3G vei tan chuan he system hnathawh hi a tibuai a, anmahni kal (kidney) ngei pawh a tichhe tan ta a ni.
C3G natna hi chi hnih a awm a, chungte chu:
1. Dense Deposit natna (DDD) 1. A rilru a hah lutuk chuan a rilru a buai em em a.
2. C3 Glomerulonephritis (C3 Glomerulonephritis - C3GN) natna hi a tha ber pawl a ni.
Heng chi hnih hi genetic cause (hereditary pawh ni thei) emaw, dam chhunga hriselna lama harsatna dang emaw avanga lo awm thei a ni.
Eng thilin nge hei hi a thlen? Kidney-ah hian eng nge thleng?
Kan kal (kidney) hi tea filter angin han ngaihtuah teh. Heng filter te hi glomeruli an ti a . Hengte hian kan thisen a filter a, a tifai a, zun hmanga bawlhhlawh duh loh zawng a paih chhuak thin.
C3G natnaah chuan a chunga kan sawi tak complement system-a malfunction hian heng delicate filters glomeruli an tih te hi a tichhia a ni. Hetiang chhiatna a thlen hian filter chhunga pores te chu a lian a, thisen chhunga awm tur protein bakah thisen cell sen leh var te chu zun chhungah a luang lut tan thin. Hun a kal zel a, he chhiatna hi a pung thei a, kal (kidney failure) pawh a thlen thei bawk. Zirna thenkhatah chuan DDD hian C3GN aia naupang zawkte a nghawng tih an hmuchhuak.
C3G natna lanchhuahna chu engte nge ni?
C3G vei zawng zawng hian symptom inang an nei vek dawn lo. Amaherawhchu, hei hi hriat chian a pawimawh. A lan chhuah dan bulpui te han thlir ila.
| Symptom a ni | A sawifiahna awlsam tak |
|---|---|
| Zun chhunga protein awm (Proteinuria) . | Zun chhunga protein tam lutuk. Zun chu foamy, cloudy, thim emaw a ni thei. Hetiang dinhmun hi a nasat (nephrotic syndrome) a nih chuan symptom dang, swelling te pawh a awm thei bawk. |
| Zun chhunga thisen (Hematuria) . | Kidney filter a chhiat chuan zun chhungah thisen sen (red blood cells) a awm khawm thin. Zun chu a sen, pink emaw, brown emaw a ni thei. |
| Edema a awm | Kidney te hian hna a thawk tha lo a, taksaah tui a pungkhawm a, chu chuan ke, kut, ke ruh leh mit vel ah a hring chhuak thin. |
| Thisen sang lutuk | Kidney atanga tui lak chhuah dik loh avanga thisen kalna kawnga pressure sang. |
| Chauhna leh mutthilhna | Kidney hian bawlhhlawh a filter tha lo a, thisenah a pung khawm thin. Hei hian thluak a nghawng thei a, hahna, mutthilh leh ngaihtuahna fim harsa tak a thlen thei a ni. Kidney natna hian thisen tlakchhamna a thlen thei bawk a, chu chuan hahna a tizual thei bawk. |
| Hri kai fo thin | Complement system chak lo chuan taksain natna hrik a do theihna a ti tlem a, chu chuan natna hrik a kai fo thei a ni. |
| Mit Harsatna a awm | Mitdelna hi retina-a protein leh thau (drusen) awm vang a ni thei. |
| Vunah thau awm dan mumal lo (Acquired partial lipodystrophy) . | Complement system-in thau cell a tihchhiat hian taksa hmun thenkhat chu a ti hring thei a, hmun thenkhat chu a lianin thau tam zawk a awm thei bawk. |
Chu bâkah, natna lan chhuahna, rilru hahna, rilṭâm, ei duh lohna, mut theih lohna, vun hring leh hnim hring, ka chhunga metallic taste, leh zan lama taksa ruh hring (muscle twitching) te hi kidney failure-ah a awm thei bawk
Engtin nge hei hi i hmuh theih, Doctor?
C3G natna hi chiang taka hriat theihna awmchhun chu kidney biopsy neih hi a ni . Hei hian kidney tissue te tak te chu light anesthetic hmanga lak a, microscope hmanga enfiah a huam a ni. Hei chauh hi kal a chhiat nasat zia hriat theihna a ni.
Chu bâkah, i doctor chuan thisen leh zun test leh genetic test pawh a rawt thei bawk.
Doctor hnenah eng nge ka zawh ang?
Hetiang hunah hian i rilruah zawhna tam tak i nei mai thei. Heng thilte hi i doctor nen chiang takin sawiho rawh. Entir nan:
- Engtin nge C3G ka hmuh theih?
- Eng ang C3G nge ka neih? (DDD nge C3GN?)
- He natna karah hian ka hriselna enkawl dan tha ber berte chu engte nge ni?
- Eng enkawlna kawng nge ka neih?
- He natna nen hian nakin lawkah eng nge ka beisei ang?
Hei hi eng enkawlna nge ni?
Dik tak chuan C3G natna tidam kim thei tur damdawi pakhat mah a la awm lo. Amaherawhchu, he natna hi thunun thei, a natna lanchhuahna tiziaawm thei, kal (kidney) chhiatna tiziaawm thei tur enkawlna chi hrang hrang a awm.
- Thisen sang leh protein chhuahna control: Thisen sang damdawi (Angiotensin-converting enzyme (ACE) inhibitor leh Angiotensin II type-1 receptor blockers (ARBs)) te hian zun atanga protein paih chhuah a ti tlem thei a, natna kal zel tur a tikhawlo thei bawk.
- Cholesterol control: Cholesterol sang (hyperlipidemia) i neih chuan zirchianna atanga a lan dan chuan cholesterol tihhniamna damdawi hian C3G natna zual zel chu a thunun thei a ni.
- Immunosuppressive drugs: Immune system titawp thei damdawi, glucocorticoids leh mycophenolate mofetil (MMF) te inzawmkhawm hian damlo thenkhatah chuan natna lo lang leh tur a veng thei a ni.
- Tunlai enkawlna: Anti-complement damdawi `eculizumab` te hian complement system hnathawh a control thei a, natna kal zel dan a tikhawlo thei bawk. Hei bakah hian damdawi thar `iptacopan` tih chu tunah hian zirchian mek a ni. Results beisei awm tak tak a lantir a, chuvangin a chungchanga zirchianna chak zawka kalpui turin pawm a ni.
Engtin nge ka in enkawl ang?
Damdawi ang bawkin he natna thunun nan hian i in enkawl dan pawh a pawimawh hle.
- Ei leh in hrisel: Thei, thlai, legumes, leh whole grains tam tak ei thin ang che. Saturated fat, chi leh sugar te tihtlem. I doctor chuan potassium, sodium (salt), a nih loh leh phosphate i ei zat tihtlem turin a hrilh thei che a ni. Doctor rawn hmasa lovin i ei leh inah inthlak danglamna lian tham siam ngai suh.
- I hriselna enkawl rawh: I dam tha a nih pawhin i damdawi ei tur chu i doctor-in a tih ang chiah chiah kha ei la. Damdawi dang, vitamin emaw, supplement emaw i ei lai chu i doctor hnenah hrilh rawh.
- Exercise: Taksa tihchak hian mut a titha thei a, chakna a tipung thei a, thinlung natna vei theihna a ti tlem thei bawk. I doctor nen i tana tha tur exercise plan chungchang sawipui rawh.
- Meizial zuk loh tur: Hei hi i hriselna zawng zawng atan a tha hle.
- Zu khap: I doctor nen zu in chungchang sawipui la, thurawn la rawh.
Nakin hun atan eng nge kan beisei theih ang?
C3G natna i vei tih hriat a nih veleh i medical team chuan enkawlna tur tha ber zawng turin an pui ang che.
A then phei chuan natna hi a na thei hle. C3G vei zinga 50% vel chuan an hmuhchhuah atanga kum 10 chhungin end-stage renal disease (ESRD) an nei thin. Hetih lai hian kal (kidney) hian bawlhhlawh paih chhuah theihna chu a bo vek tawh a ni. Dialysis emaw, kal thlak emaw a ngai mai thei.
Mahse hriat reng tur chu kal thlak hnuah pawh kal thara C3G natna a lo awm leh theihna chance a sang hle. Chuvangin i doctor nen i tana duhthlan tur tha ber chungchang chu rei tak sawipui rawh.
I medical team-ah hian nephrologist, hematologist, leh pathologist te pawh an tel thei. Anmahni nena inlaichinna tha neih reng a pawimawh.
Take-Home Thuchah a ni
- C3G hi kal natna hmuh tur awm lo mahse na tak a ni a, chu chu taksa immune system-a tel complement system-a chhiatna avanga lo awm a ni.
- A lan chhuah dan ber chu zun chhunga protein emaw thisen emaw, a hring, thisen sang, leh hahna te a ni thei.
- He natna hi kidney biopsy hmanga hriat chian a ni.
- Hemi atana damdawi bik awm lo mah se, thisen sang control-na te, immunity control tu damdawi te, nun hrisel tak te hmangin he natna hi control theih a ni a, kal (kidney) te pawh venhim theih a ni.
- I medical team te nena inzawmna nei reng tur leh an thupek dik taka zawm hi a pawimawh hle.
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