Chhan awm lovin khawsik pawh i nei fo em? Anmahni rual hian harsatna dang i nei ve em? Hei hi Chhungkaw Mediterranean Fever (FMF) a ni thei!

Vaukhânna tel lovin khawsik i nei thut mai em? I pum na, i rilru a tight tih hriatna, emaw, ruh natna emaw i nei bawk em? A châng chuan heng symptoms te hi ni engemaw zat hnuah a bo a, rei vak lo hnuah a rawn lang leh mai thin em? Hetiang thil a thlen hian hlauhna nasa tak nei hi thil pangngai a ni. Vawiin hian heng symptoms thlentu natna pakhat kan sawi dawn a, chu chu a mak deuh a, mahse hriat a pawimawh hle.

`Chhungkaw Mediterranean Fever (FMF)` tih hi eng nge ni?

A awlsam zawngin, Familial Mediterranean Fever, a tawi zawngin FMF hi kan genes atanga lo chhuak dam chhung zawnga natna a ni. Hemiah hian thil thleng chu, chhan hriat lohvin, khawsik kan nei thut a, chutih rual chuan taksa peng hrang hrangah inflammation a awm bawk. A dik tak chuan hei hi kan taksa hriselna (immune system)-a harsatna tenau tak avanga thleng a ni. Chuvangin autoinflammatory disease an ti bawk .

Mediterranean ram a chengte a nghawng ber a ni. `serous membranes` an tih (hengte hian rilru chhung leh pum chhunga hmun hrang hrang a khuh) te, `serositis` an tih te chu a ti na a ni. Kan ruh khuh tu `synovial membranes` te pawh a ti na thei bawk a, chu chu `synovitis` an ti a ni. Symptoms hi ni engemaw zat a awm tlangpui a, chutah chuan a reh leh thin.

FMF natna lan chhuah dante chu engte nge ni?

FMF symptoms hi naupan lai atanga tan a ni tlangpui a, kum 20 i tlin hmain heng symptoms te hi a tir lamah chuan na tak a ni thei a, mahse i upat deuh deuh chuan a lo tha chho zauh zauh thei. Vawikhat natna i neih tawh chuan kar tam tak, thla tam tak, kum tam tak chhung pawh i nei lo mai thei. Mahse , a hmaa hriatchhuah leh enkawl hian symptoms a tihziaawm thei a, hun rei tak chhunga harsatna awm thei amyloidosis ang chi te pawh a veng thei bawk.

Tunah chuan heng symptoms te hi eng nge ni tih i lo en ang u:

• Khawsik sang tak chu 40.6 degree Celsius (105 Fahrenheit) thleng a ni a, a chang chuan khawsik leh khur pawh a awm bawk.
• Lu na leh kawr khauh tak nen a lo thleng thei bawk.
• Pum natna leh pum na (bloating ) (bloating).
• Pum ruh a tight a, constipation a awm thei bawk.
• Chest pain , thawk chhuah hian a pung thin.
• Joint na leh swelling (a tam zawk chu joint pakhatah).
• Mipa testicles ah hian a hring leh a na .
• Muscle aches , a bik takin taksa hnuai lam ah.
• Keah vun sen, sang tak a lo awm thei .

Heng symptoms te hi darkar tlemte chhungin a lo lang nghal thei a ni. Mahse, mi zawng zawng hian heng symptoms zawng zawng hi an nei vek dawn lo.. Khawsik hi a lan chhuah tam ber a ni. Naupan lai chuan khawsik hi a lan chhuahna awmchhun a ni thei. Natna hi ni engemaw zat a daih tlangpui.

FMF natna lan chhuah dan (Prodromal Symptoms) te

Mi thenkhat chuan he khawsik hi a lo chhuah hma ni engemaw zat atang hian symptom te te an nei tan thin. Mi thenkhatin ``migraine`` hmaa an hriat ang bawkin emaw hmeichhiain an ``menstruation`` hmaa an hriat ang bawkin. Hengte hi ``prodromal symptoms`` kan ti a ni. Hengte hi i hriat chuan ''Ah... tunah chuan chu buaina chu a lo thleng dawn ta'' tih hriatna i hmu thin.

Heng telltale signs te hi engte nge ni?

• Lungkhamna, rilru hahdam lohna
• Thinrimna (Iritability) a awm
• Lu na
• Luhai
• Taksa natna
• Malaise ( thahnemngaihna tlakchham, nunna nei lo ) .

Engvangin nge he `FMF` hi a lo awm?

Hei hi kan taksaa `MEFV` gene inthlak danglam vang a ni. He `MEFV` gene hian protein pawimawh tak siam turin a pui a, chu chu protein pakhat `pyrin` an tih hi a ni. Kan pianpui immune system – chu chu immune system a\anga natna hrik kai nghal ang chi hlauhawm laka hnathawktu a ni – chu he `pyrin` protein-ah hian a innghat a ni. Chuvangin, `MEFV` gene hian `pyrin` a siam tha thei lo a nih chuan, chu protein chuan immune system chhungah hna a thawk tha thei lo. Chutah chuan he harsatna hi a lo awm ta a ni.

`FMF` thlentu genetic mutations (`variations`) 30 vel hmuhchhuah a ni tawh bawk. Chung zinga a tam ber chu `V726A`, `M680I`, `E148Q`, `M694V` leh `M694I` te an ni. Hengte hi Mediterranean leh Middle Eastern atanga lo piang population-ah a tam ber. Entir nan:

• Arab ho an ni
• Armenia mite an ni
• Grik mite an ni
• Iran mite an ni
• Italian ho an ni
• Juda mite
• Kurds te an ni
• Turk mite an ni

FMF atana triggers te

FMF hi a lo thleng nghal vat a, chhan hriat lohvin a lo thleng fo niin a lang fo nain, mi thenkhat tan chuan thil engemaw avanga lo awm a ni. Mi thenkhat tan chuan taksa emaw rilru emawa harsatna avanga lo awm thei a ni. Mite chuan FMF hi thil hrang hrang atanga lo chhuak thei niin an sawi:

• Khua a vawt avanga inhriatthiamna
• Exercise emaw, hah lutuk emaw
• Tun hnaia hri kai pakhat
• Tun hnaia hliam emaw, operation emaw a neih
• Stress na tak (nun harsatna lian tak ang chi) .
• Hmeichhiain thla tin an period emaw ovulation emaw an neih hun

FMF avanga harsatna awm thei

FMF khawsik hi ni engemaw zat hnuah reh mah se, taksaa inflammation chhunzawm zel hian hun kal zelah kan taksa a nghawng thei a ni. Chuvang chuan doctor-te chuan hei hi enkawl chhunzawm zel turin an rawt a ni. Hetianga inflammatory process kal zel a awm chuan kan immune system chu pressure nasa tak hnuaiah a awm a, natna dang pawh a awm thei bawk.

Hei hi a harsatna lian ber chu `secondary amyloidosis` tih hi a ni . Hei hian kal (kidney failure) pawh a thlen thei a ni. Chuvang chuan hemi chungchangah hian fimkhur a pawimawh hle.

Harsatna dangte pawh hi:

• Splenomegaly (splenomegaly) tih hi a awm lo.
• Nau pai theih lohna
• Autoimmune natna dang (`Secondary autoimmune disease`) te pawh a awm thei.

Engtin nge FMF hi hmuhchhuah a nih?

Engtin nge doctor hian FMF hi a hriat theih ang? Hetiang thil hi an ti thin:

• I natna lan chhuah dan te, a lo awm fo dan te, leh eng chen nge a awm tawh tih te ngun takin an ngaithla ang .
• I chhungkuaah hian he natna hi an vei tawh em tih leh i genetic heritage chu eng nge ni tih an zawt ang.
• I damloh chuan i taksaah inflammation emaw irritation emaw a awm em tih an enfiah ang che .
• Natna a awm lai hian thisen test a , inflammation chhinchhiahna a awm leh awm loh enfiah thin a ni .
• FMF thlentu ber MEFV gene-a mutation awm leh awm loh enfiah nan genetic testing neih a tha hle .
• FMF enkawlna atana i chhan dan pawh an en bawk ang.

`FMF` enkawl dan tur

FMF enkawlna ber chu anti-inflammatory drug colchicine a ni. Hei hian thisen var cell chi khat neutrophils an tih, inflammation-a inrawlh te hnathawh tihtlem hmangin hna a thawk a ni. Colchicine hian FMF flare awm tamna tur venna emaw tihtlem emaw a pui thei a, harsatna awm thei pawh a ti tlem thei bawk. FMF vei zinga 90% aia tam chu an dam tawh . Mi tam zawk chuan an dam chhung zawng nitin he damdawi hi an ei a ngai a ni.

Mahse, mi tlemte chauhvin colchicine atanga duhthusam result an hmu lo a, a nih loh leh hriselna lam thil dang avangin he damdawi hi an ei thei lo mai thei bawk. Chutiang mite tan chuan doctor-te chuan damdawi chi dang an rawt a. Chung zinga ṭhenkhat chu:

• `Anakinra` tih hi a ni.
• `Rilonacept` tih a ni
• `Canakinumab` tih a ni

`FMF` i neih chuan eng nge thleng ang?

FMF hi damdawi awm lo mah se, damdawi hmangin a enkawl tha thei a ni . Mi tam tak tan chuan damdawi an ei tan hian khawsik leh taksa na chu a bo vek tawh mai. Midang tan chuan flare-up awm tam leh tam loh a tlahniam. Amaherawhchu, heng damdawite hian hna a thawk tha tur chuan, natna lan chhuah loh lai pawhin ei fo a ngai a ni.. I damloh tawh laiin he damdawi hi ei hian natna chu a titawp nghal lovang. He damdawi hian natna a thlen loh nan chauh a veng a ni.

FMF hi ven theih a ni em?

Mi tam tak chuan FMF thlentu gene variant an nei tih pawh an hre lo. FMF dawng tur chuan i nu leh pa pahnih hnen atangin gene variant copy pahnih i lak a ngai a ni. Copy pakhat chauh i neih chuan FMF symptoms i nei lovang. Mahse chu gene variant chu i fate hnenah i pass thei a ni. I partner pawhin chu gene variant chu a neih chuan i fate pawhin FMF an nei thei. Genetic testing hian gene variant carrier i nih leh nih loh a hriat theih nan a pui thei che a ni.

Hmanlai atang tawhin hetiang khawpa khawsik hi a awm fo tih report a awm tawh a. He natna `FMF` an tih hi chutiang bawk chuan a upa tawh mai thei. Zirchiangtu thenkhat chuan he natna hi chutih laia natna lian tham tak tak `tuberculosis` te dona kawnga puitu atan a lo awm niin an ngai. Dik tak chuan taksaa inflammation neih hian hlawkna engemaw zat a nei a, mahse a tam lutuk chuan harsatna a siam thin. Vanneihthlak takin tunah hian he natna hi control turin damdawi tha tak tak a awm ta a ni. Chuvangin `FMF` nei mah la, nun pangngaiin i nung thei a ni.

I hriat reng tur pawimawh ber chu! (In lama hruai tur thuchah)

Okay, chuti a nih chuan tunah chuan vawiin a kan sawi tawh, `FMF` hi i hrethiam zawk tawh a ni. A pawimawh ber chu nangmah emaw, i chhungte zingah emaw, chhan awm lova khawsik, pum na, ruh na, etc. i neih chuan khawsik mai mai anga ngaih loh tur a ni a, thil awmdan dik tak hriat tumin doctor pan rawh. Natna i hriat chian a, enkawl tan hma chuan harsatna tam tak i veng thei a, nun pangngaiin i nung thei bawk. I doctor-in damdawi a pek dik tak, a tih angin hman a pawimawh hle bawk tih hre reng ang che. Zawhna emaw, ngaihtuahna emaw i neih chuan i doctor nen inbiakna neih theihnghilh suh.

` FMF, familial fever syndrome, khawsik fo, pum na, ruh na, genetic disease, colchicine, amyloidosis