Polycystic kidney disease (PCK) pawh i nei em? Hemi chungchang hi han sawi ila!

Kidney chhungah hian bawm te tak te, tuiin a khat a lo awm thin tih natna i hre tawh em? I chhungkuaa tu emaw chuan a nei a ni mai thei, a nih loh leh khawi emaw laiah i lo hre tawh a ni mai thei. Hei hi Polycystic Kidney Disease (PKD) kan tih chu a ni. A hming hi a hlauhawm deuhin a lang thei a, mahse a chungchang hriat belh chuan awmzia a nei thei hle. Chuvangin, vawiinah hian PKD chungchang hi chipchiar zawkin han sawi ila.

PKD chu eng nge ni? A awlsam zawngin...

A awlsam zawngin, Polycystic Kidney Disease (PKD) hi i kal chhungah tui-a khat cyst tam tak, blister te tak te ang maia awm i neihna a ni Genetic condition a ni. Chumi awmzia chu i taksaah mutated gene i neih a ngai tihna a ni. Hei hi kidney cyst nen a danglam hle a, i kal chauhah a lo awm tlangpui a, a tlangpuiin a hlauhawm lo.

Heng PKD avanga cyst lo awm hian zawi zawiin kal pahnih hi a ti lian thei a, a ti lian thei bawk. Ngaihtuah teh, a chang chuan heng cyst te hian kal pakhat rit zawng chu pound 30 (kilogram 13.5 vel) thlengin a tisang thei a ni! Tichuan, kan kal (kidney) te hian thisen atanga bawlhhlawh paih chhuah hna ber chu an thawk thei tawh lo. Hun a kal zel a, he natna hi Chronic Kidney Disease ah a lo chang thei a, a tawpah chuan kidney failure a thlen thei a ni. Dik tak chuan PKD hi United States-ah hian kal (kidney failure) 2% vel awm chhan a ni. PKD vei tam tak chuan an dam chhungin engtik lai pawhin dialysis emaw kidney transplant emaw an mamawh dawn a ni.

Chuvangin, doctor-in PKD a vei tih a hriat chuan a pawimawh ber chu hlauthawng ni lovin, he natna avanga harsatna lo awm thei te enkawl dan tur enkawlna ruahmanna tha tak siam turin a thawhpui a ni.

PKD chi hrang hrangte chu engte nge ni?

PKD hi chi hnih a awm a. An chanchin tlem han zir ila.

1. Autosomal Dominant Polycystic Kidney Disease (ADPKD) a ni a, a chhuahna tur hmun leh a hmanna tur a awm lo.

Hei hi PKD chi khat a ni ber. Puitling lai, kum 30 leh 50 inkar vel a ni tih hmuhchhuah a ni ber a, mahse, a chang chuan naupan lai emaw, tleirawl lai emaw a lo lang thei. Hetiang PKD chi hi nei tur chuan i nu leh pa pakhatin gene mutation a neih a ngai a ni. ADPKD vei te hian he mutation hi `PKD1` emaw `PKD2` emaw an tih genes ah hian an nei thin.

2. Autosomal Recessive Polycystic Kidney Disease (ARPKD) te hi a tha ber pawl a ni.

Hei hi a rare type a ni. Infantile PKD an ti bawk. Naute chu nu pum chhunga a la awm laiin, chu chu nausen piang chhoh lai a ni., kal (kidney) te hi a thang tha lo hle. Doctor-te chuan naupai lai emaw, naute pian hnu emawa prenatal ultrasound scan-ah an hmu fo thin. Naupangin he ARPKD chi hi a neih theih nan chuan nu leh pa pahnih hian `PKHD1` an tih gene mutation an neih a ngai a, naupang chuan an pahnih hnen atangin gene hi a rochun a ngai a ni.

PKD hi engtiang chiahin nge a hluar?

Statistics atanga a lan dan chuan United States-ah ringawt pawh hian PKD vei hi 500,000 (500,000) vel an awm a ni.

A hmaa kan sawi tawh chi, ADPKD hi ARPKD aiin a tam zawk hle. PKD vei zinga 90% velin ADPKD an nei. ARPKD hi a tlem hle a ni. Mi 25,000 zinga pakhat velin a nghawng a ni.

PKD natna lan chhuah dan tlangpui chu engte nge ni?

PKD i neih chi hrang hrang a zirin symptoms a inang lo thei.

ADPKD natna lan chhuah dan

ADPKD vei, a tam ber chi chu puitling an nih hma chuan symptoms an nei lo thei. A châng chuan, kal (kidney)-a cyst lo awmte hi a lian lutuk hma loh chuan hriat theih a ni lo thei. Symptoms a lo lang tak tak a nih chuan:

• Hnunglam emaw, a sir emaw a natna (``flank pain'' an ti bawk) .
• Thisen sang a sang
• Lu na fo thin
• Zun chhunga thisen (Hematuria) .
• Urinary tract infection (UTI) awm fo thin .
• Kidney-ah lung a awm

ARPKD natna lan chhuah dan

Naute ARPKD, a awm ve lo tak takte hian an pian tirh emaw, an naupan lai emaw pawhin an natna lan chhuahna an nei thei a ni. A châng chuan, nausen kal hmaa ultrasound an tih laiin doctor chuan nausen kal (kidney)-ah cyst a hmu thei bawk.

Nausen piang tharin ARPKD a vei chuan a natna lan chhuahna te chu:

• Nau piang rit lo
• Pum a lo hring chhuak
• Pian tirh atanga thisen sang neih
• Thâwk harsa

Naupan laia ARPKD i vei chuan hetiang hian symptoms i nei thei a:

• Growth failure (hei hi ``Growth failure'' an ti) .
• Urinary tract infection (UTI) awm fo thin .
• Thisen sang a sang
• Pum emaw hnungzang hnuai lam emaw a na

Nausen chhungah ARPKD a awm chuan scan-ah hian a hnuaia mi ang hian a lang thei ang:

• Kidney pangngai aia lian zawk
• Amniotic fluid level hniam tak a ni

PKD symptoms hi a lang rang fo em?

Ni lo, a ni lo. He natna hi clinically silent a ni fo va, symptoms a awm lo . Chumi awmzia chu pawn lam chhinchhiahna engmah awm lovin taksa chhungah natna a lo awm thei tihna a ni. Ngaihtuah teh, he natna vei zinga a chanve vel chuan an dam chhungin an vei tih pawh an hre lo mai thei. Chutiang tui rimtui (cyst) te chu a lo lian hnuah symptoms a lo lang fo thin.

PKD hi eng thilin nge a thlen?

Kan sawi tawh ang khan PKD thlentu ber chu genetic mutations a ni . Genes hi kan taksa siamtu bulpui ber ang a ni tih i hria a. Heng genes te hi kan nu leh pa hnen atangin kan dawng thin. Heng genes te hian kan taksa a than dan tur leh a hnathawh dan tur a hril a ni. A châng chuan embryonic stage-ah hian he gene hi a inthlak danglam a, a copy tha lo. Chutiang genetic mutation i neih chuan i fate hnenah pawh a kai thei bawk. PKD hi hetiang hian a lo thang lian fo thin.

Mahse, a tlem hle a, nu leh pa pahnih hian he gene hi an nei lo a nih pawhin, gene-a random mutation avang hian natna hi a lo awm thei a ni.

Hetiang dinhmun hi engte nge ni?

PKD hi genetic condition a nih avangin, i vei theihna tur risk factor ber chu chhungkaw history a ni. Chu chu i nu leh pa zinga pakhatin natna a nei (a bik takin ADPKD a nih chuan) emaw, nu leh pa pahnih hi carrier an ni emaw (ARPKD a nih chuan) tihna a ni.

PKD avanga harsatna awm thei te chu engte nge ni?

PKD hian puitling leh naute tan hriselna lama harsatna lian tak a thlen thei a ni. A châng chuan PKD symptom anga kan hmuh hi a takah chuan complication a ni thei. Entir nan:

• Kidney-ah lung a awm
• Thisen sang a sang
• Zun kawng natna (UTIs) .

Tin, PKD hian harsatna lian tham dang a thlen thei bawk a, chungte chu:

• Thluak chhunga thisen kalna kawng puak (``Brain aneurysms'' an tih) .
• Colon problems, entirnan, diverticulitis, colon chhungah pouch te te a lo awm a, a kai thin
• Heart valve lama harsatna nei
• Kidney tha lo a awm
• Liver cyst leh pancreatic cyst te a awm bawk
• Naupai laiin thisen sang (``Preeclampsia'' an tih) .

Hei hi naute ARPKD na tak nei tan chuan thihna thlen thei a ni . ARPKD vei nausen piangte tan chuan an dam thla hmasa ber hi a pawimawh hle a, chutih chhung chuan thawk lama harsatna nasa tak leh kal (kidney failure) an nei thei a ni.

Engtin nge PKD hi hmuhchhuah a nih?

PKD hmuhchhuah leh enkawlna kawngah hian nephrologist (kidney natna lama tui tak doctor) hian hna a thawk ber tlangpui. I natna lan chhuah dan leh chhungkaw chanchin ngun takin a enfiah ang a, i kal enfiah turin imaging exam a ti thei bawk ang, chu chu:

• Kidney ultrasound: Hei hi hman tlanglawn ber, natna nei lo a ni.
• Prenatal ultrasound: Hei hian nausen pum chhunga nausen hian PKD a nei em tih hriat nan a pui a ni.
• CT scan (computed tomography scan): Hei hian kal leh zunthlum (bladder) te chiang zawkin a entir thei a ni.
• MRI (magnetic resonance imaging): Hei hian kal (kidney) thlalak chipchiar tak tak hmuh theihna a pui bawk.

Hei bakah hian doctorGenetic testing pawh tih a tha thei bawk. He test hian thisen emaw, hmui emaw sample-ah PKD thlentu mutated gene a awm leh awm loh a enfiah thei a ni. Hei hian eng PKD chi nge i neih tih pawh a nemnghet thei bawk.

PKD enkawlna tur engte nge ni?

Dik tak chuan PKD hi damdawi a la awm lo. Enkawlna hi natna zual zel tur tihkhawtlai te, a lan chhuah dan control te, leh harsatna awm thei venna te a ni ber. PKD enkawlna atana hmanraw lian ber berte chu:

• Thisen sang enkawl dan: Thisen sang hi PKD hmelma lian tak a ni. Chuvangin i doctor chuan damdawi hmangin i thisen sang control nan a pui ang che, chi tlem ei leh in hrisel tak leh exercise hmangin i thisen sang control turin a pui ang che. I thisen sang chu him taka dah hian lung natna leh stroke ang chi natna khirh tak tak i neih theihna a ti tlem thei a ni.
• Thâwk chhuahna lama ṭanpuina: ARPKD vei naute, an lungphu a ṭhang kim lo, thawk harsa tak takte chu mechanical ventilator hmanga ṭanpui an ngai mai thei
• Dialysis: I kal (kidney) a tha lo a, a hna a thawk thei tawh lo a nih chuan ``Dialysis'' (i thisen artificial-a tihthianghlimna) tih a ngai mai thei. Hetiang a nih avang hian ``Hemodialysis'' tih machine hian i thisen chu i taksa pawnah a filter thin. ``Peritoneal dialysis'' ah chuan i thisen chu tui bik leh i pum line siamtu peritoneum an tih membrane hmangin a filter thin.
• Growth therapy: ARPKD vei naute thau lo leh thang chak lote chu an thanlen theih nan tanpuina an mamawh thei. Doctor chuan special nutritional therapy emaw human growth hormone emaw a rawt thei ang .
• Kidney transplant: ADPKD chu kidney failure thlenga a kal chuan kidney transplant i mamawh mai thei . Transplant chu surgical procedure a ni a, a kal chak lo chu lakchhuah a, donor atanga kal hrisel tak hmanga thlak a ni.
• Pain management: Damdawi hmang hian infection, kidney stone, emaw PKD vanga cyst puak avanga natna lo awm chu control theih a ni. Mahse, natna tidamtu i ei apiang chu i doctor-in a pawmpui tur a ni . Painkiller thenkhat (a bik takin NSAID) hian kal chhiatna a tipung thei a ni.

PKD vei dam rei zawng chu engzat nge ni ang?

He thu i hriat hian i hlauthawng deuh mai thei, mahse thudik hriat a pawimawh.

ADPKD vei te hian enkawlna mumal tak an dawng a, natna hi uluk taka enkawl a, nunphung hrisel an zawm chuan,Nun rei tak, famkim i nung thei a ni. ADPKD vei zinga a chanve vel chuan kum 70 an tlin chuan kidney failure an nei ang a , dialysis emaw kidney transplant emaw an mamawh ang .

Mahse naupang ARPKD vei tan chuan prognosis hi a tha vak lo. ARPKD vei nausen piang hmun thuma ṭhena hmun khat vel, a bik takin thawk lama harsatna nasa tak nei te chu nausen laiin an thi thin. Nausen damchhuakte hian an dam chhung zawngin damdawi lam enkawlna leh enkawlna bik an mamawh fo thin. Naupang nausen piah lama dam chhuak zinga a chanve vel chuan kum 15 leh 20 inkar an kal (kidney failure) an nei thin.

PKD hi ven theih a ni em?

Vanduaithlak takin PKD hi genetic disease a nih avangin a pumpelh kim thei lo . Mahse, nunphung hrisel tak zawm te, thisen sang thunun te, damdawi lam thurawn te uluk taka zawm te hian natna zual zel tur chu i tikhawlo thei a, a nih loh leh kal chak lohna ang chi harsatna lo awm tur chu i tikhawtlai thei a ni.

PKD vei, fa neih tumte tan chuan genetic counselor nena inbiakna neih a pawimawh hle a, chutiang chuan an fate hian he natna hi an rochun theih dan tur leh a venna atana hmalak theih dan tur te hriattir an ni ang.

PKD nena nun awlsam zawk nan eng nge ka tih theih ang?

PKD vei nun hi a harsa thei hle a, mahse nunphung hrisel zawm leh natna hriat chian chuan zinkawng chu i ti awlsam thei a ni. I tanpui theihna tur tips thenkhat chu hetiang hi a ni:

• Kidney-friendly diet ei rawh . Hei hian ei tur chi, potassium leh phosphorus tlakchhamna lam a ngaihtuah tur a ni. Hemi chungchangah hian i doctor emaw dietitian emaw hnen atangin thurawn chiang tak la rawh.
• I duh ang exercise la, ni khatah minute 30 tal, kar khat chhunga ni tam zawkah.
• Taksa rihna hrisel tak vawng reng rawh.
• I thisen sang kha enfiah fo la, i doctor thupek angin control rawh.
• Zuk leh hmuam i zuk emaw, zuk leh hmuam dang i hman emaw chuan titawp nghal rawh.
• A theih ang anga zu in loh tur.
• Stress tihziaawmna tur kawng zawng rawh. Meditation leh yoga ang chi thilte hian a pui thei a ni.
• Nitin tui leh caffeine tel lo (tea, coffee tlem) tam tak in thin ang che. Mahse, kal natna stage eng pawhah tui in khap a ngai a nih chuan doctor thurawn zawm tur a ni.
• Zan tin darkar sarih tal mut tha rawh.
• I doctor-in damdawi a pek zawng zawng chu dik takin, a hun takah leh a pek zat angin ei vek tur a ni. Damdawi engmah ei hi damdawi lam thurawn tel lo chuan chawlhsan suh.

PKD chungchangah engtikah nge doctor i hmuh ang?

PKD i neih chuan medical supervision hnuaiah i awm tawh tur a ni. Mahse, heng symptoms zinga pakhat tal i neih thut chuan doctor hmu la, damdawi inah kal nghal rawh :

• I ke, ke ruh emaw, i ke emaw a lo hring nghal a nih chuan (hei hi edema an ti) .
• I rilru natna nasa tak i neih emaw, i rilru a tight tih hriat emaw i neih chuan
• Thâwk harsa nghal i neih chuan
• I zun theih loh chuan
• Zun nen thisen chhuak tam lutuk a awm chuan
• Lu na nasa tak i neih emaw, mit a danglam emaw chuan

Doctor hnenah eng zawhna nge ka zawh ang?

He natna chungchanga zawhna tam tak neih hi thil pangngai a ni. I doctor hnenah zawhna zawh hreh suh ang che:

• Eng ang PKD nge ka neih? (ADPKD nge ARPKD?)
• Ka fate’n he natna hi ka hnen atanga an rochun hlauhawmna chu eng nge ni?
• Special test dang tih a ngai em?
• Eng enkawl dan nge ka tan a tha ber?
• Ka ei leh inah eng inthlak danglamna nge ka siam ang, a bik takin chi, potassium, leh phosphorus chungchangah?
• Ka nunphung (exercise, mut, etc.) ah hian tihdanglam ka ngai em?
• Nakin lawkah dialysis emaw kidney transplant emaw ka mamawh thei ang em?
• Eng side effect emaw complication nge ka hriat bik tur?
• Damdawi ka ei hian eng side effect nge a neih?

A tawp berah chuan hriat reng tur (Take-Home Message) .

Tunah chuan Polycystic Kidney Disease (PKD) hi genetic condition a ni a, chu chuan kidney-ah cyst a siam thin tih i hre tawh ang. Heng cyst te hian kal a ti lian thei a, an hnathawh a tibuai thei bawk. Puitlingte a nghawng tam ber a, mahse a tlem berah chuan PKD chi hlauhawm tak chu naute zingah a lo awm thei a ni.

PKD damdawi awm lo mah se, lungngai suh. I natna lan chhuah dan dik taka enkawl a, doctor thurawn zawm a, nun hrisel tak i neih chuan he natna hi i nunpui thei ang. A pawimawh ber chu i doctor nena thawhhona tha tak neih a, i mamawh test leh treatment te neih hi a ni. Nangmah chauh i ni lo va, he zinkawngah hian doctor, chhungte leh ṭhiante pawh an awm bawk.

` Polycystic Kidney Disease, PKD, kal natna, kal cyst, genetic natna, ADPKD, ARPKD