I chhungkuaa mi tu emaw hian kal natna a nei a ni mai thei. A nih loh leh kum 30 emaw 40 emaw vel a nih laiin thisen sang i nei tih hmuhchhuah a ni a, a chhan i zawng a ni mai thei. Vawiin hian natna danglam deuh, mahse tlanglawn tak, thlahtute atanga lo chhuak, kal (kidney) a nghawng dan kan sawi dawn a ni. Hei hi damdawi lam chuan Autosomal Dominant Polycystic Kidney Disease an ti a. Chu chu kan tan chuan a rei lutuk deuh avangin a tawi zawngin ADPKD kan ti ang.
A awlsam zawngin ADPKD chu eng nge ni?
A awlsam zawngin sawi ila, he natna hian i kal (kidney)-ah hian tui (fluid-filled cyst) tam tak a siam thin. Kan taksaah hian kal (kidney) hi filter angin ngaihtuah rawh. Heng cyst te hi an lo lian chhoh zel chuan kal (kidney) te hian hna an thawk tha thei tawh lo. Hei hian hriselna lama harsatna, thisen sang, zun kawng natna, kal (kidney stone) te a thlen thei a ni. Mi zawng zawng hian he natna hi vei lo mah se, mi thenkhat chuan kal (kidney failure) thlengin an kal thei a ni.
He natna danglam bik pakhat chu i nei a nih pawhin kum tam tak chhung chu symptoms i nei lo mai thei tih hi a ni. Kum 30 leh 40 inkar atang hian symptoms hi a lang tan fo thin a, chuvang chuan "adult PKD" an ti bawk.
Mahse, lungngai suh. Damdawi a la awm loh laiin, i natna lan chhuah dan i enkawl a, nun hrisel i hman a, i doctor nen i thawh dun chuan kum tam tak chhung chu nun active takin i nung thei ang.
ADPKD natna lan chhuah dan chu engte nge ni?
ADPKD vei zawng zawng hian symptoms an nei vek dawn lo. Ṭhenkhat tân chuan natna lan chhuah nân kum tam tak a ngai thei a ni. He natna kaihhnawih natna lan chhuah dan tlangpui thenkhat han thlir ila. He thu hi in hriatthiam theih nan hmun hnih ah ka then ang.
| Mihring ziarang chi khat | Thil hmuh tur awm |
|---|---|
| A lan chhuah hmasak ber (Thil hmasa ber hmasa ber) |
|
| Natna a zual zel angin symptoms lo lang thin (Kidneys failure hnaih tawh chuan) . |
|
Engvangin nge ADPKD hi a lo awm? Hrilhfiahna a ni em?
Ni e, hei hi thlahtu natna a ni . Hei hi a chhan ber chu kan DNA-a gene pahnih, `PKD1` leh `PKD2`-a chhiatna vang a ni. Heng genes te hi kidney cell te hnenah "engtikah nge an lo thanglian dawn" tih signal petu an ni. Heng gene pakhatah hian chhiatna a awm chuan kal cell te chu thunun theih lohvin an thang lian a, kan sawi tawh ang khan cyst te chu an siam ta a ni.
Ngaihtuah teh, thlahtu natna tam zawkah chuan i nu leh pa hnen atangin gene chhia chu i lak a ngai a ni. Mahse ADPKD-ah chuan nu leh pa pakhat hnen atanga gene chhia chu i hmuh chauh a ngai a ni . Chuvangin "autosomal dominant" an ti a ni.
Chumi awmzia chu i nu leh pa zinga pakhatin natna hi a neih chuan 50% in i vei theihna chance a awm tihna a ni . Mahse, a tlem hle a, mi pakhat gene-a random change avanga natna hi a lo awm thei bawk a, nu leh pa pahnihin natna hi an vei lo.
Engtin nge he natna hi i vei tih i hriat theih ang?
I doctor chuan i symptoms a zirin kidney problem a rinhlelh chuan kidney specialist (nephrologist) hnenah a refer thei che a ni. Doctor chuan zawhna a zawt ang che:
- I symptoms te chu engte nge ni a, engtikah nge a lo intan?
- I thisen sang i hria em?
- Khawiah mah hrehawmna a awm em? A nih chuan khawiah nge?
- Kidney stone i nei tawh em?
- Chhungkaw zingah kal natna nei an awm em?
Tichuan doctor chuan i kal (kidney) enfiah turin test eng emaw zat a ti ang.
- Ultrasound Scan: Hei hian sound wave hmangin taksa chhung lam thlalak a la thin. Hei hi test hmasa ber a ni fo thin.
- MRI scan: scan chak tak, magnet leh radio wave hmanga tumor te tak te, ultrasound hmanga hmuh theih loh te hriatchhuahna.
- CT Scan: Scanning hmanraw chak tak dang, X-ray hmanga taksa chipchiar taka thlalak hmuh theihna.
Tin, i DNA pawh test theih a ni a, gene dik lo `PKD1` emaw `PKD2` emaw i nei em tih pawh i enfiah thei bawk. Mahse, he genetic test hian gene chhia i neih leh neih loh a hriat theih laiin, natna chu engtikah nge a lo awm dawn tih leh engtiang chiahin nge a nasat dawn tih erawh a sawi thei lo .
Enkawlna leh in lama tih theih thilte
ADPKD hi damdawi a la awm lo. Mahse he natna avanga hriselna lama harsatna awmte enkawl dan tur leh kal (kidney failure) venna tur hian kan tih theih tam tak a awm.
Damdawi lam enkawlna
- Kidney failure tihziaawmna tur damdawi: Puitling, natna zual chakna hlauhawm tak tan chuan kal hnathawh tlahniam tihziaawm nan `Tolvaptan (Jynarque)` ang chi damdawi pek thin a ni.
- Thisen sang control thei damdawi.
- Urinary tract infection tan antibiotics hman tur a ni.
- Natna tihdamna damdawi.
I kal (kidney) a chhiat chuan dialysis tih damdawi i neih a ngai mai thei. Hei hian i thisen filter nan machine hman a, i taksa atanga bawlhhlawh, chi leh tui tam lutuk paih chhuah a huam a ni. Thil dang chu kidney transplant a ni. Hemi chungchang hi i doctor nen sawiho la, a tam zawk hriat duh chuan.
In lama i tih theih thilte
I kal venhim tur leh a rei thei ang bera hnathawh that theih nan a hrisel thei ang bera awm a pawimawh.
- Ei leh in dik: Chaw (balanced diet) chi tlem ei thin ang che. Chi (salt) hian thisen sang a tisang thei a ni.
- Active takin awm rawh: Exercise hian i taksa rihna leh thisen sang a control thei a, mahse contact sport hi pumpelh la, chu chuan i kal a tichhe thei a ni.
- Meizial zuk loh: Meizial hian kal (kidney)-a thisen kalna kawng a tichhe a, cyst siam theihna a tipung thei bawk.
- Tui in tam: Dehydration hian cyst a siam tam thei bawk.
ADPKD avanga harsatna dang lo awm thei te
ADPKD hian hriselna lama harsatna dang a thlen theihna a tipung thei bawk a, hengte pawh hi hriat a pawimawh hle.
| Hriselna lama harsatna awm thei dangte (Possible Complications) . | |
|---|---|
| Aneurysm (thluaka thisen kalna kawng bang chak lo leh bulh) . | Liver leh pancreas-a cyst siam |
| Diverticulosis (intestine lian chhunga pouch te tak te) . | Hernias a awm thin |
| Lung kawng natna (e.g. Mitral valve prolapse, Aortic regurgitation) . | Kidney hnathawh a hloh |
| Naupai laiin thisen sang (Preeclampsia) . | Natna kal zel |
Natna a zual zel dan hi `PKD1` emaw `PKD2` gene-a chhiatna i neih leh neih lohah a innghat a ni. A tlangpuiin `PKD1` gene-a chhiatna nei te hi `PKD2` defect nei te aiin kal (kidney failure) nei hma zawk an ni. Chuvangin i dinhmun dik tak hriat tumin i doctor nen inbiakna neih a tha ber.
Take-Home Thuchah a ni
- ADPKD hi inherited disease a ni a, chu chuan kidney-ah fluid-filled cyst a siam thin.
- Kum 30 leh 40 chho vela lang thin thisen sang hi he natna lan chhuahna hmasa ber a ni thei.
- Nu leh pa pakhatin he natna hi an neih chuan naupangin a vei theihna chance 50% a nei a ni.
- Hei hi damdawi tluantling awm lo mah se, i thisen sang control-na leh nunphung hrisel tak zawm chungin i kal (kidney) i humhim thei a ni .
- Hemi chungchangah hian rinhlelhna emaw, symptom emaw i neih chuan hlauthawng suh la , doctor rawn la, thurawn la rawh.











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