I naute pian hnu lawkah emaw, kar tlemte hnuah emaw vunah hian a sen, a hring emaw, a rawng mak tak tak emaw i hmu ngai em? A châng chuan nu angin hengte hi hmuh hi a hlauhawm thei hle. Vawiin hian kan sawi dawn a, chutiang bawka symptoms nei thei dinhmun kan sawi dawn a, chu chu a tlem deuh a, mahse hriat a pawimawh hle. Hei hi Incontinentia Pigmenti, a tawi zawngin IP an ti a.
Incontinentia Pigmenti (IP) hi eng nge ni tak tak?
A awlsam zawngin, hei hi genetic condition a ni. X-linked dominant a nih avangin mipa aiin hmeichhia a nghawng nasa zawk.
IP hi natna chi hrang hrang ``neurocutaneous disorders'' an tih zinga mi a ni. Ngaihtuah teh, hei hian he natna hian kan nervous system (chu chu thluak leh nerve) leh vun hi a rualin a nghawng thei tihna a ni. Chu mai ni lovin, a chang chuan mit, ha, leh ruhro pawh a nghawng thei bawk.
He natna hi pian tirh lai emaw, pian hnu kar hmasa berah emaw hmuh tur a awm ber a, vunah hian hnah leh vun chi hrang hrang a lo lang thin. Hei hi kan cell hnathawh leh thanlenna controltu NEMO (NF-kappaB essential modulator) tih gene-a mutation vang a ni. Tun thlengin hei hi tihdamna famkim a la awm lo a, mahse a natna lan chhuah dan thunun theihna tur enkawlna tha tak tak a awm.
Vun a inthlak danglamna te chu engte nge ni? Stage 4 a awm!
IP-a vun inthlak danglamna hi stage lian pali-ah hriat theih a ni. Mahse, heng stage te hi a inthlak danglam a ngai lo tih hre reng ang che. A châng chuan an inthlak danglam thei a, stage pahnih emaw pathum emaw chu a rualin hmuh theih a ni.
| Dawhsan | A lan dan & A sawifiahna |
|---|---|
| Stage 1: Vesicular tih a ni | Hei hi taksaah hian vun sen, tuiin a khat a lo lang thin. Hengte hi kut leh keah hmuh tur a tam ber. |
| Stage 2-na: Verrucal tih a ni | Blister awmna hmunah chuan hliam thar, wart ang chi a lo lang thin. |
| Stage 3: Hriselna (Hyperpigmentation) a awm | Hei hi he natna danglamna ber a ni. A vun chu gray, blue, emaw brown, marbled emaw wavy line angin a lang. Chungte chu taksa leh ke ruhah a lang tam ber. |
| Stage 4: Hriselna (Hypopigmentation) a ni | Hun a kal zel a, chu hmun dumte chu a bo vek a, vun rawng aiin vun rawng aiin vun rawng dum, hliam ang maia hring hring a hnutchhiah a ni. |
Skin spots chauh a ni em? Ni lo, nghawng dang pawh a awm thei bawk
Hetiang dinhmun hi vunah chauh a ni lo. Naupang thenkhat chuan harsatna dang an nei thei bawk. Mahse, heng symptoms zawng zawng hi naupang zawng zawngah a awm vek dawn lo tih i hrethiam tur a ni.
Neurological lama harsatna awm thei
IP vei naupang 20% vel chuan thluak lam harsatna an nei thei a ni.
- Motor hmasawnna tihkhawtlai: Entirnan, naute dang angin an kawr an hawi thei lo a, an inher thei lo va, an kal chak lo thei bawk.
- Intellectual disability: Zirna lama harsatna ang chi thil.
- Muscle chak lohna: Chak lo anga inhriatna.
- Seizures: Fit ang chi natna lo awm.
Hei hi thluak tissue tihtlem (cerebral atrophy) leh thluaka thil dum (white matter)-a khur te tak te lo awm vang te a ni thei.
Mit leh ha a nghawng dan
- Mit lama harsatna: Naupang thenkhat chuan strabismus, mitdelna, mitdelna nasa tak ang chi natna an nei thei.
- Ha lama harsatna: Ha bo leh peg ang maia ha te hmuh theih a ni.
A pawimawh ber chu hetiang symptom i hmuh veleh hlauthawng lo la, naupang enkawltu doctor hnenah thurawn dil a ni.
Hei hi engtin nge enkawl a nih?
IP hi naupang natna lan chhuah dan a zirin enkawl a ni. Mimal tinte tana enkawl dan tur dik tak chu a inang lo thei.
| Buaina (Symptom) 1.1. | Enkawl dan tur |
|---|---|
| Skin spot leh blisters te a awm | Thu lawmawm tak chu heng vun hrui te hian enkawl bik a ngai lo fo thin. Naupang a lo puitlin emaw, a puitlin emaw chuan anmahni chauhin an bo vek ang. |
| Nervous system lama harsatna (seizures, taksa ruh chak lohna) . | Hetiang thil atan hian neurologist i hmu tur a ni . Tichuan, heng dinhmunte hi damdawi emaw, a tul chuan damdawi hmanrua dang emaw hmangin control theih a ni. |
| Mitdelna a awm | Mit lama harsatna nei tan chuan mit lam doctor i hmu thei a, glasses, damdawi emaw, a nasat chuan operation emaw i la thei bawk. |
| Ha lama harsatna a awm | Ha pianzia emaw, a tam leh tam lohah harsatna a awm chuan ha enkawltu hnenah a tul anga enkawlna i zawng thei a ni. |
Chuti a nih chuan, hmalam hun hi kan hlau tur em ni?
Hei hi nu leh pa tam takin zawhna an neih a ni. Dik tak chuan, a tam zâwk chuan, nausen laia harsatna lian tham a awm loh chuan, IP vei naupangte chuan dam rei tak an hmang thei a, hrisel tak, nun pangngai tak an nei thei a ni Hun a kal zel a, chu vunah chuan a dum hringte chu a bo thei a, a bo vek thei bawk.
Mahse, a hmaa kan sawi tawh ang khan naupang thluak lam emaw, taksa lama harsatna dang emaw nei an awm. Chuvangin, i fate dinhmun chungchang hi Doctor nen sawipui hi a tha ber a ni. Tichuan i fate tana hriselna atana ruahmanna tha ber chu eng nge ni tih leh a hnua i tih tur chu chiang takin i hre thei ang.
Take-Home Thuchah a ni
- Incontinentia Pigmenti (IP) hi genetic condition hmuh tur awm lo tak a ni a, hmeichhe naupang a vei ber a ni.
- A lan chhuahna ber chu vun inthlak danglamna (blisters, rough patches, dark wrinkled patches, leh pale patches) a awm thin.
- Vun lama harsatna bakah hian naupang thenkhat chuan nervous system, mit leh ha nena inzawm harsatna an nei thei bawk.
- Skin spot te hi enkawl lohvin hun kal zelah a bo fo thin.
- Naupang tam zawk hi harsatna lian tham awm lovin nun pangngai, hrisel takin an khawsa thei a ni.
- I fa hian heng symptoms te hi a nei a nih chuan hlauthawng lo tur leh naupang doctor pan a, thurawn dik tak dil a pawimawh hle.
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