Sawifiah theih loh khawpin i pum na nasa tak, luak chhuak leh zun dum i nei tawh em? A châng chuan hei hi a chhan chu kan zînga tumahin kan hriat ngai loh thil awm lo tak a ni thei bawk. A tlem em avangin mi tam takin an hre lo. Vawiin hian chutiang natna pakhat, Acute Hepatic Porphyria (AHP) chungchang kan sawi dawn a ni. Ngaihtuah buai suh, he thu hi a awlsam zawngin, mi zawng zawngin an hriatthiam theih tur angin kan sawi dawn a ni.
A awlsam zawngin Acute Hepatic Porphyria (AHP) hi eng nge ni?
Acute Hepatic Porphyria, a tawi zawngin AHP tih hi kan taksaa chemical chi khat, thin leh taksa peng dangah porphyrins a tam lutuk vang a ni. Hei hi a takah chuan natna pakhat chauh a ni lo va, natna pawl khat a ni. Chutianga porphyrins a punkhawm chuan nervous system a nghawng thei a, symptom dang nuam lo tak tak a thlen thei bawk.
He natna hi damdawi la awm lo mah se, enkawl dan dik chuan a natna lan chhuah dan a thunun thei a, nun tha zawk i neih theih nan a pui thei bawk.
Engvangin nge he natna hi a lo awm? Ngun zawkin han thlir ila.
Hei hi dik taka hrethiam tur chuan kan taksaa thil pawimawh tak tak kan hriat hmasak a ngai a ni. Chu chu ' Heme ' an ti a .
Heme hi kan thisen sen (red blood cells)-a protein hemoglobin- a thil pawimawh tak a ni. I hriat angin hemoglobin hi lung atanga oxygen la chhuakin taksa pum puiah a sem darh thin a ni. Chuvangin he heme hi liver, muscles, leh bone marrow ang hmunte hnathawhna atan a pawimawh hle.
Tunah en teh, kan taksa hian he ‘heme’ hi a siam hian enzyme chi 8 hian chu process chu a dik taka a thlen theih nan a pui thin. Dish siam nan hian thil rimtui chi 8 a ngai ang mai a ni. Eng emaw chhan vanga heng enzyme 8 zinga pakhatah hian tlakchhamna emaw, chak lohna emaw a awm chuan ‘heme’ siam dan chu a chanve velin a tawp ta a ni. Chutih laia thil thleng chu a hmaa kan sawi tawh ‘porphyrins’, ‘heme’ siamna atana hman tur thilte chu taksaah a pung khawm tan ta a ni
AHP ah chuan he chemical ' porphyrin ' an tih hi liver ah a awm khawm ber a ni . He accumulation-in nghawng a neih hi symptom anga lang chhuak a ni.
| A lan chhuah dan tlangpui | Hemi chungchang hi tlem tal sawi belh leh ila |
|---|---|
| Pum natna nasa tak | Hei hi a symptom ber leh na ber a ni. Chhan hriat lohvin, a chhan hriat lohvin i pum na nasa tak i nei thei a ni. |
| Nausea leh luak chhuak | Hetiang dinhmun hi pum natna nasa tak nen a awm thei. |
| Zun dum | Zun chu a dum emaw, a sen emaw a ni thei. |
| Vun sensitivity a awm | Porphyria chi thenkhatah chuan vun hi ni êng lakah a sensitive hle thin. |
| Nervous system lama harsatna a awm | Thil na tak tak, rilru hahna, natna, taksa ruh chak lohna, leh seizures te pawh a awm thei. |
AHP "attack" hi pum na tak atanga tan a ni tlangpui. Hei hi ni tam tak chhung a daih thei. Chutiang hunah chuan damdawi ina dah ngei ngei tur a ni . Hei hian Emergency Department (ETU) pan tihna a ni.
He natna hi tuin nge vei? Tute nge hlauhawma awm?
Vawi tam tak chu, he natna hi thlahtute aṭanga lo chhuak a ni . Chu chu he natna thlentu genes hi i nu leh pate hnen atangin i rochun tihna a ni.
Mahse hetah hian thil pawimawh tak chu natna thlentu gene nei zawng zawng hian symptoms an nei vek dawn lo . AHP gene nei tam zawk hi an dam chhungin hrisel takin an awm thei a, symptom engmah an nei lo.
Hei hi sakeibaknei muhil ang a ni. Gene chu awm mah se, a tiharh thei tur, emaw, "a tiharh" theihna tur trigger a awm loh chuan harsatna awm lovin a awm thei a ni.
Chuti a nih chuan he natna thlentu hi engte nge ni?
Hei hi a pawimawh hle a ni. Nangmah emaw, i hriat emaw hian AHP a nei a nih chuan heng thilte hi hriat chian a pawimawh hle.
| Ti per | Point pawimawh tak tak |
|---|---|
| Damdawi thenkhat | Painkiller, sedative, barbiturates, tranquilizer, leh birth control pill thenkhat chu a thlentu lian ber a ni thei. AHP i rinhlelh emaw, i vei tih hmuhchhuah a nih chuan doctor rawn hmasa lovin damdawi engmah ei loh tur a ni. |
| Zu in leh meizuk | Hengte hian awlsam takin natna a thlen thei a, a chhan chu liver-ah pressure nasa tak a siam avangin. |
| Dieting khauh tak emaw, chaw nghei emaw | Chaw ei khap emaw, chaw ei loh emaw, taksa rihna tihtlem nghal tumna hi heng damlote tan hian a pawi hle. Taksa mamawh calorie a hmuh loh hi trigger chak tak a ni. |
| Rilru leh taksa lama harsatna | Stress nasa tak, operation, leh infection ang chi thilte pawhin natna hi a thlen thei bawk. |
Engtin nge ka tih ang?
He thuziak-a tarlan symptoms eng emaw zat , a bik takin na tak, sawifiah theih loh pum na, i tawn fo chuan , khawngaihin a pangngaiah ngai suh. Doctor thiam tak hmu nghal rawh.
Doctor chuan a enfiah ang che a, a tul chuan zun, thisen leh genetic test a order ang a, natna i vei em tih a hriat theih nan.
AHP vei tan chuan ei leh in inthlau tak hi a pawimawh hle. A tul chuan i doctor chuan nutritionist hnenah a refer thei che a ni.
Take-Home Thuchah a ni
- Acute Hepatic Porphyria (AHP) hi natna hmuh tur awm lo, rochun atanga lo chhuak a ni a, chu chu liver-a chemical porphyrin an tih a lo awm khawm vang a ni.
- Hei hi a chhan ber chu ‘heme’ siamna atana enzyme mamawh a tlakchham vang a ni.
- A lan chhuahna ber chu tuar theih loh pum na a ni. Tin, nervous system lama harsatna a thlen thei bawk.
- Natna thlentu gene i nei a nih pawhin natna thlentu trigger i pumpelh chuan symptoms i nei lo mai thei.
- Damdawi thenkhat, zu, meizuk, ei leh in khauh tak, leh stress hi a thlentu lian ber a ni.
- Heng symptoms te hi i neih emaw, AHP i vei nia i rinhlelh emaw chuan doctor nen inbia ngei ang che. Mahni chauha chhuakin damdawi la ngai suh.
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