I naute kal (kidney) hian hna a thawk tha lo em ni? He Potter Syndrome hi i hrechiang ang u!

Nu ni tur i nih chuan i naute hriselna hi i ngaihtuah nasa hle mai thei, a dik em? I naute chu a pum chhungah a thang tha em tih leh engkim a kal tha em tih ngaihtuah hi thil pangngai tak a ni. Vawiin hian naute nghawng thei natna hlauhawm tak kan sawi dawn a ni. Potter Syndrome an ti a. He thu i hriat hian i lungngai viau mai thei, mahse fimkhur a pawimawh hle.

A awlsam zawngin Potter Syndrome hi eng nge ni?

Awle, i tichhia ang u. Potter syndrome, a then chuan Potter sequence tia koh a ni a, naute chu an pum chhunga an awm laiin an thanlenna tichhe thei thil awm lo tak a ni. Hei hi a chhan ber chu naute kal (kidney) a lo thang tha lo emaw, an hnathawh a tichhe emaw a ni . Naute chu a pum chhunga a awm lai hian an chhehvelah amniotic fluid a tam hle tih i hria em. He amniotic fluid awm zat tihtlem nan hian kidney hian hmun pawimawh tak a chang a ni. Chuvangin kal (kidney) hian hna a thawk tha lo a nih chuan he amniotic fluid hi a tlem phah thin. Hei hi doctor ten oligohydramnios an tih chu a ni.

Lungchhiatthlak takin, a chang chuan naute hi kal pahnih nei lovin a piang a, chu chu ``Bilateral Renal Agenesis`` an tih natna a ni a, chu chuan thihna a thlen thei a ni. Mahse, naute thenkhat chu an natna lan chhuah dan a nasat loh chuan emaw, tui tlakchhamna a nasat loh chuan an dam thei a ni. Mahse, heng naute hian an lo upat deuh deuh chuan lung leh kal (kidney) natna khirh tak tak an nei thei a ni.

Hetiang dinhmun hian tute nge a nghawng nasa ber?

Dik tak chuan he Potter syndrome an tih hian naute eng pawh a nghawng thei a ni. A chhan chu amniotic fluid tlem nen a inzawm tlat a ni. Mahse, zirchianna thenkhatah chuan naute mipa te hian he natna hi an vei tam zawk tih hmuhchhuah a ni .

Potter syndrome hi thlahtute atanga lo chhuak a ni em?

Hei hi a buaithlak deuh a ni. Potter syndrome hi genetic condition a ni lo. Mahse, a thlen theihna tur condition thenkhat a awm. Eng nge an nih hi han thlir ila:

• Polycystic kidney disease: Hei hi nu emaw pa emaw (autosomal dominant) emaw nu leh pa pahnih (autosomal recessive) atanga rochun theih a ni. Hei hian kal (kidney) ah cyst a siam thin.
• Renal agenesis: Hei hi kal (kidney) a lo thang chak loh vang a ni. A chang chuan hei hi genes FGF20 emaw GREB1L emaw a mutations vang a ni thei. Hetiang dinhmun hi autosomal dominant emaw autosomal recessive emaw hmanga inherit theih a ni. Chumi awmzia chu naute hian he genetic mutation hi nu leh pa pakhat emaw, nu leh pa pahnih emaw aṭangin a rochun a ngai tihna a ni.
• Sporadic genetic changes: A châng chuan, random genetic change hian Potter syndrome a thlen thei a, chu chu chhungkaw zingah tumahin he natna hi an la nei lo a nih pawhin.

Hetiang dinhmun hi engtiang chiahin nge a awm?

Potter syndrome hi a awm lo hle a ni . He natna hian naute piang 4,000 atanga 10,000 zinga pakhat chauh a vei nia chhut a ni. Chuvangin a chanchin hriat hian hlauthawng suh. Mahse fimkhur a pawimawh.

Potter syndrome hian engtin nge naute taksa a nghawng?

Hei hi a pawimawh ber a ni. Potter syndrome hian naute chhungril taksa peng hrang hrang, a bik takin kal (kidney) te lo thanglian dan leh hnathawh dan a nghawng a ni . I hriat angin kal hi kan taksa atanga bawlhhlawh leh tui tam zawk paih chhuaktu taksa peng pawimawh tak a ni. Nausen pum chhunga a awm chuan kal (kidney) hian zun a siam chhuak thin. He zun hi amniotic fluid angin recycle a ni.

Chuvangin, naute kal (kidney) hian hna a thawk tha lo a nih chuan a hual vel tur amniotic fluid a siam tling lovang. He amniotic fluid hi naute ti cushion tu a ni. He tui hi a bo chuan naute taksa peng leh taksa thanlen dan a nghawng a ni. Phên dangin sawi ila, taksa peng hrang hrangte chu an lo ṭhang lian kim lo . Tichuan chu taksa peng hrang hrangte chuan an hna an thawk tha thei lo. Hei hi nunna atana hlauhawm symptoms thlentu a ni.

Potter syndrome natna lan chhuah dan chu engte nge ni?

Potter syndrome natna lan chhuah dan hi nausen hrang hrangah a inang lo thei a, a nasat dan pawh a inang lo thei bawk. Heng chhinchhiahnate hian i naupai lai pawh a nghawng thei a, chu chuan nau piang hmasa a thlen thei a ni .

Amniotic fluid awm lohna

Naupai lai hian naute vel chu tui thianghlim, sen hring a awm a. Hei hi amniotic fluid a ni. Hei hian naute chu a venghim a, a thanlenna tur hmun a pe bawk. Uterine wall leh naute inkarah barrier angin a thawk a ni. Potter syndrome vei naute hian he amniotic fluid hi an nei tlem hle a, chuvangin uterine wall atanga pressure lo chhuak hian naute than dan a nghawng a ni.

Hmai leh taksa lama thil danglam bik

Amniotic fluid tlakchham vanga pressure awm hian naute taksa thanlen dan a nghawng a ni. Hei hian hmel lan dan bik a siam thei a ni. Hei hi "Potter facies" an ti a. Heng features te hi:

• Chin hi a lo thang tha lo (turn in angin a lang).
• A hmui hnuai lam hnuaiah wrinkle nei.
• Mit chu hla takah an dah a.
• Naha-a bridge chu a flatten ta a.
• Beng set hniam leh beng chhunga cartilage tlahniam.
• Mit kil khatah vun a inzawm khawm.

He pressure hian naute taksa peng dangte hmasawnna a nghawng thei bawk. Entir nan:

• Kut leh ke tawi te.
• Joints te uluk taka tihzauh leh tihdik theih lohna te, leh stiffness (contractures) te.
• Naute hi naupai kum nen khaikhin chuan a lian lo hle.

Organ hmasawn lo emaw, piansual lo emaw

Organ tichhe thei symptoms te hi nunna atana hlauhawm ber a ni.. Potter syndrome-ah chuan naute thanlenna a nghawng a, chuvangin chhungril taksa peng hrang hrangte hian an thanlenna tur atana an mamawh tur thupek emaw hun emaw an dawng lo. Chumi avanga symptom lo awm thei te chu:

• Thinlung natna (congenital heart conditions) a awm thin.
• Mit natna (e.g. mitdel, lens luxation).
• Kal natna (kidney failure rei tak, kal agenesis, polycystic kidney disease) te.
• Lung natna (lung natna rei tak, thawk lama harsatna).

Kidney lo thanglian dan pangngai lo hian nausen piang tharin zun a siam chhuah theih zat a nghawng bawk. Hei hi doctor-te’n Potter syndrome an hmuhchhuah huna an zawn thin symptom a ni bawk.

Potter syndrome hi engte nge ni?

Potter syndrome thlentu hi thil engemaw zat a awm a. Anni chu:

• Kidney a lo thang tha lo emaw, kidney nei lo emaw.
• Polycystic kidney natna a ni.
• Prune belly syndrome (Prune pum natna / Eagle-Barrett natna) tih hi a tha hle.
• Zun kawng (urinary tract) a block (blockage) thin.
• Membrane rupture avanga amniotic fluid leak.
• Nu-a damdawi lam dinhmun thunun loh, entirnan, type 1 diabetes.

Heng natna lan chhuahna, a bik takin kal (kidney) tichhe thei te hi naute hual vel tur khawpa nausen pum chhungah amniotic fluid a awm loh vang a ni .

Engvangin nge he amniotic fluid hi a tlahniam?

Hei hi chipchiar deuhin han thlir ila. A chhan ber chu kal (kidney) a lo thang dan pangngai lo vang a ni .

Naupai lai hian i naute chu tui thianghlim, sen hring, amniotic fluid an tihah a inphah thin tih i hria em. He tui hian i naute chu a venghim a, i naupai chhung zawngin a thang lian thei em? I naupai tirh lamah hian he amniotic fluid hi i taksa atanga tui leh nutrients atanga siam a ni. I naute hian he amniotic fluid hi a in thin. Kar 16 leh 20 inkarah hian i naute hian he amniotic fluid hi a thawh ve tan thin. Engtin nge i hriat? Zun chhuah hmangin! I naute hian he tui hi a in a, chutah chuan zun angin a pass chhuak leh thin. Hei hi cycle khatah a thleng thin.

Chuvangin, i naute hian Potter syndrome a neih chuan a zun siamtu taksa peng, kal (kidney) te chu a lo thang tha lo emaw, a bo emaw, a thawk lo emaw a ni. Naute chu a zun theih loh avangin amah venhimtu amniotic fluid a thawhpui thei lo. Chuvang chuan nausen pum chhungah amniotic fluid a tlem zawk a ni.

Potter syndrome hi chi hrang hrang a awm em?

Ni e, doctor-te chuan Potter syndrome chi hrang hrang an thliar hrang a, chu chu kal (kidney) tichhe thei symptoms a zirin a ni.

• Classic Potter syndrome: Hei hi a tam ber a ni. Nausen kal (kidney) pahnih nei lo a pian hian a thleng thin.
• Potter syndrome chi khat chu:Hetiang dinhmun hi polycystic kidney disease an tih vang a ni a, chu chu nu leh pa pahnih atanga rochun a ni a, autosomal recessive condition a ni. Hetiang dinhmunah hian kal (kidney)-ah hian cyst a lo awm thin.
• Potter syndrome type II: Hetiang syndrome hi naupai laiin nausen pum chhunga kal (kidney) hmasawnna dik lo avanga lo awm a ni.
• Potter syndrome type III: Hei hi polycystic kidney disease vang pawh a ni a, type I ang bawkin a ni a, mahse nu leh pa pakhat chauh (Autosomal dominant) atanga rochun a ni.
• Potter syndrome type IV: Hetiang chi hi nausen pum chhunga naute than dan pangngai lo vanga zun kawng (obstructive uropathy) a tihkhawtlai vang a ni.

Engtin nge Potter syndrome hi an hriat theih?

Potter syndrome hi naupai laiin prenatal exam hmangin hriat theih a ni . Naupai laiin he natna hi i nei thei tih chhinchhiahna pakhat chu naute chhehvela amniotic fluid awm loh vang a ni. I doctor chuan ultrasound scan laiin hei hi a zawng ang. Tin, taksa lama natna lan chhuah dan, ruh (joint stiffness) (contractures) te pawh an zawng dawn a ni.

Hei hi naute pian hmaa hriatchhuah a nih loh chuan nausen pian hnuah doctor chuan taksa enfiahna a nei ang a, a natna lan chhuah dan a enfiah ang. Heng symptoms te hi:

• Zun chhuah a tlem hle.
• Hmai lam hawi bik nei.
• Thâwk harsa.

Hei hi finfiah nan eng test nge tih thin?

Doctor chuan a natna hriat chian nan test eng emaw zat a ti thei a:

• Genetic blood test hmanga natna lanchhuahna atana mawhphurtu gene hriat theih a ni.
• I fate lung, kal, leh zun kawngte chu X-ray, MRI, a nih loh leh ultrasound ang chi imaging test hmangin enfiah rawh .
• Thisen emaw zun emaw test a, electrolyte leh enzyme level enfiah thin.
• Lung natna chhinchhiahna awm leh awm loh enfiah nan echocardiogram test neih a ni.

Hei hi eng enkawlna nge ni?

Potter syndrome enkawlna chu i fate lung leh thinlung lama harsatna (pulmonary hypoplasia) nasat danah a innghat a, chubakah i fate kal (kidney) hnathawhna tur atana duhthlan tur awm thei te pawh a innghat bawk.

Ngaihtuah teh, i naute lo thang lian chu a lungphu a lo thanglian tha theih nan naupai chhung zawngin amniotic fluid-in a hual vel a ngai a ni. Naute rilru chu rei tak a engorged chuan a pian hnua dam khawchhuah theihna tur lung tissue a hloh thei a ni.

Nau piang thar, kal (kidney failure) nei tawh enkawl chu thil harsa tak a ni thei. A then phei chuan intensive care intervention a tlem a, naute leh nu leh pate inzawmkhawmna tur leh naute thlamuanna pe turin neonatal palliative care hman a ni .Methodological treatment hi duhthlan tur a ni thei.

I naute chu a pian hnua a damchhuah chuan enkawlnaah hian nunna atana hlauhawm symptoms venna lam a ngaih pawimawh ber ang. Heng enkawlnate hi a hnuaia mi ang hian a ni thei:

• Thâwk chhuahna hmanrua (e.g. ventilator ) hman.
• Lung hnathawhna tura puitu damdawi.
• Urinary tract-a blockage awmte siamthat emaw tihbo emaw tura operation tih.
• IV nutrition therapy, nasogastric tube emaw, feeding tube emaw hmanga chaw pek dan tihchangtlunna tur operation.
• Dialysis hi kal (kidney) dik lo vanga thisenah toxin awm khawmte paih chhuah nan a ni. Kum engemaw zat hnuah dialysis enkawlna a hlawhtling lo a nih chuan doctor chuan kal thlak (kidney transplant) a rawt thei a ni .

I naute chu engtikah nge a natna hmuhchhuah a nih dan azirin naupai laiin enkawl tan theih a ni. Investigational treatment pawh i dawng thei bawk a, chu chu amnioinfusion a ni a, chu chuan i naute chhehvela tui awm chu thlak turin i amniotic cavity-ah tui a dah belh a ni. He enkawlna hian naupai kar 22 hmain a thawk tha ber.

Potter syndrome hi ven theih a ni em?

Vanduaithlak takin Potter syndrome hi ven theih a ni lo .

Ka fa hian Potter syndrome a neih chuan eng nge ka beisei ang?

Potter syndrome hi damdawi a awm lo . A tam zawkah chuan naupai laiin he natna hi hriat hmasak a nih chuan i doctor chuan him taka nau neih dan tur ruahmanna a siam thei a, i naute chu a pian hnua a dam khawchhuah theih nan enkawlna a pe thei bawk.

Potter syndrome symptoms hi nunna atana hlauhawm tak a ni. Mahse, i naute lung leh kal a natna lanchhuahnain nasa taka a nghawng loh chuan i naute hian a hmalam hun tur a nei tha deuh thei a ni.

Nau piang hnu lawka enkawl tan a nih avangin enkawlna zawng zawng hi a hlawhtling vek lo. An naupan laiin operation vawi tam tak an neih a ngai mai thei.

Potter syndrome vei naute dam rei zawng chu engzat nge ni ang?

Naute Potter syndrome vei nia hriatte hi an dam rei thei hle a ni . Hei hi mi zawng zawng tan a danglam a, a natna lan chhuah danah a innghat bawk. A symptoms a nasat chuan, leh taksa peng lian tak tak, thinlung, lung, leh kal (kidney) te hmasawnna leh hnathawh dan a nghawng chuan prognosis a tha lo. Naute tam zawk hi an dam chhung ni hmasa berah an dam lo . Mild case, symptoms nasat lutuk lo leh taksa peng hrang hrang a nghawng nasa lo hmunah chuan dam rei deuh pawh a rei thei.

I doctor chuan i naute natna hmuhchhuah a nih theih dan tur a sawipui ang che a, a dam rei theihna tur enkawlna tur a rawt bawk ang che. I naute natna hmuhchhuah a pawi hle a nih chuan, palliative care hi i hmangaih tak boral avanga lungngaihna i tuar theihna tur kawng khat a ni thei.Lungngaihna emaw, lungngaihna emaw counselling neih pawh a rawt thei bawk.

Engtikah nge Doctor ka hmuh ang?

I naupai laiin inthlak danglamna i hmuh chuan, a bik takin i naute chu a che tha hnua a che a chawlhsan nghal a nih chuan , doctor hmu nghal rawh. I naute chu beisei aia hmaa piang a ni thei. Chuvangin naute piang tur atana inbuatsaih nan doctor nen prenatal exam neih fo a pawimawh hle.

Doctor hnenah eng zawhna nge ka zawh ang?

Hetiang hun lai hian i rilruah zawhna tam tak awm hi thil pangngai tak a ni. I doctor hnenah heng zawhnate hi zawt tum ang che:

• Ka naute natna hmuhchhuah chhan bulpui ber chu eng nge ni?
• Ka naute a pian hnuah operation ka mamawh dawn em?
• I enkawl dan tur i rawt te hian eng side effect nge a neih?
• Potter syndrome nei ka naute hi eng nge him ber?
• Ka naute dam khawchhuah theih nan eng nge ka tih theih ang?

I naute chu nunna atana hlauhawm natna hmuhchhuah a nih chuan a damchhuak lo mai thei tih thuthang hmachhawn chu thil lungchhiatthlak tak leh harsa tak a ni thei. I doctor chuan i naute natna hriat chian nan hna a thawkpui ang che. I naute chu a him tih an enfiah ang a, a pian hnua a natna lan chhuah dan tihziaawmna turin enkawlna rang tak an dawng bawk ang.

A tawp berah chuan thil hriat reng tur

Potter Syndrome hi thinlung ti na tak a ni. I hriat chuan i lungngai hle mai thei a, i hlauthawng hle mai thei. Chu chu thil pangngai a ni.

• Hei hi thil awm lo tak a ni tih hre reng ang che .
• Hei hi a chhan ber chu naute kal (kidney) a lo thang tha lo a, chuvang chuan amniotic fluid a tlahniam a ni .
• Naupai laiin hriat hmasak a pawimawh .
• A natna lan chhuah dan a nasat chuan naute tam tak chu an dam thei lo vang . He thu hi hriat a har hle a, mahse rinawm taka sawi a pawimawh.
• Nangmah chauh i ni lo. He hun harsa tak karah hian i medical team, chhungte, leh i thiante chuan an thlawp ang che . A tul chuan counselling zawng hreh suh.

He thu hian he natna hlauhawm tak hi hriatthiamna engemaw chen i neih theih nan a puih ngei kan beisei. Zawhna dang i neih chuan doctor nen inbiakna neih hreh suh.

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