Thisen zam chungchangah i lungkham em? Protein C tlakchhamna chungchang hi han sawi ila

Thisen chhuak tur ang maiin i inhriat ngai em? A nih loh leh i ke chu a hring leh na ang maiin i inhriat thut em? Heng thilte chhan hi i ngaihtuah aiin a danglam mai thei. Vawiin hian kan sawi tum chu thil awm lo tak, mahse hriat tur pawimawh tak a ni. Chu chu Protein C tlakchhamna a ni . A hming chu mak tak angin lang mah se, kan taksaa thisen zam (blood clotting process) nen a inzawm tlat a ni. Eng nge a nih, engvangin nge a lo awm tih leh eng nge i tih theih tih i lo en ang u.

Protein C tlakchhamna hi eng nge ni tak tak?

A awlsam zawngin sawi ila, Protein C tlakchhamna hi kan thisen a mamawh aia tam, a nih loh leh a pangngai lo taka a inzawm khawmna a ni. Hetah hian a sawifiahna tawi te kan rawn tarlang e.

I taksaah khawi emaw laiah hliam i nei ang tih han ngaihtuah teh. Tichuan, thisen chhuah tihtawp nan thisen thenkhat chu a inzawm a ngai a ni. Chu chu thil pangngai a ni. Kan taksa invenna mechanism a ni. Mahse, he thisen zam hi thunun theih loh leh a tam lutuk a nih chuan eng nge thleng ang? Chuta tang chuan buaina chu a intan ta a ni.

Kan thisenah hian he thisen zam dan hi thununtu thilsiam engemaw zat a awm a, chu chu car brake system ang chi hi a ni. Protein C hi chutiang zinga mi a ni a, thisen zam control tu protein pawimawh tak a ni. Hei hi natural ‘anti-clotting agent’ emaw anticoagulant emaw an ti a. Chu chu thisen zam lo tura vengtu thil tihna a ni.

Tunah chuan i hrethiam tawh a ni lawm ni? Protein C tlakchhamna tih awmzia chu i thisenah he protein hi i nei tlem tihna a ni. Chutiang a nih chuan thisen zam control tu brake chu a thawk tha lo. Chumi rah chhuah chu thisen kalna kawngah thisen zam (blood clots) a lo awm tan ta a ni. Hetianga thisen zam lo awm hian dinhmun hlauhawm tak, nunna atana hlauhawm tak pawh a thlen thei a ni. Entirnan, ke ruh thuk takah thisen zam (Deep Vein Thrombosis) a lo awm a, chutiang dinhmun chu thisen zam chu a inthen a, lungah a tang (Pulmonary Embolism)

Hetiang dinhmun hi engtiang chiahin nge a awm?

Hei hi a takah chuan hmuh tur awm tak a ni lo a, mahse kan ngaihtuah angin a tlem lo.

• Mild form: Hei hian mipui zawng zawng zinga mi 200 atanga 500 zinga pakhat chauh a nghawng thei niin an chhut.
• Severe form: Hei hi a tlem ber a ni. Mi 500,000 atanga 750,000 zinga pakhat velin a nghawng a ni. Mahse, a zat dik tak chu heng chhut dan aiin a tam zawk hle mai thei.

Hetiang dinhmun hian hmeichhia leh mipa a nghawng vek a ni.

Hei hi eng symptoms nge ni?

He natna lan chhuah dan hi a nasat loh leh a nasat dan azirin a inang lo. Hei hi chiang taka hrethiam turin a hnuaia table hi han en ila.

Protein C tlakchhamna chhante chu engte nge ni?

Hetiang dinhmun hi a awm chhan ber chu genetic influence a ni a, chu chu thlah khat atanga thlah danga inthlak danglam thin thil a ni tihna a ni.

Inherited Causes te chu a ni

Kan taksaah hian protein pakhat Protein C siam turin min hrilh a, chu chu PROC gene an ti a. He gene-ah hian mutation a awm a nih chuan Protein C hi siam that a ni lo mai thei a, siam a nih pawhin a thawk tha lo thei bawk.

• Type I Deficiency: Hei hi Protein C level tlahniam vang a ni.
• Type II Deficiency: Hei hi Protein C level a normal a, mahse protein hian hna a thawk tha lo a nih chuan a thleng thin.

Nu leh pa zinga pakhat hian he mutated PROC gene hi a nei ang tih han ngaihtuah teh. Tichuan an fa neih chuan chu mutated gene chu a rochun theihna chance 50% a awm. Chutiang a nih chuan naupang chuan he natna chi khat na lo tak a nei ang.

Tunah chuan nu leh pa pahnih hian he mutated gene hi an nei tih han ngaihtuah teh. Tichuan naupang pakhatin chu mutated gene pahnih chu a rochun theihna chance 25% a awm a ni. Chu naupang chuan a pian tirh atanga a lan chhuah dan natna khirh tak chu a nei ang.

Acquired Causes te chu a ni

Hei hi rochan a ni ngai lo. Damdawi lam thil thenkhat emaw, thil dang vang emaw pawhin Protein C a tlahniam thei bawk.

• Vitamin K tlakchhamna
• Thisen tithianghlimtu Warfarin hman (hei hi a hnuah kan sawi leh ang)
• Liver natna na tak
• A natna pakhat chu Disseminated Intravascular Coagulation (DIC) tih a ni.
• Bacteria natna hlauhawm tak (Sepsis) .

He natna hi engtin nge kan hriat theih ang?

I doctor-in he natna hi i vei nia a rinhlelh chuan thil engemaw zat an test ang a, a finfiah ang.

• I mimal damdawi lam chanchin: Thisen zam chungchanga harsatna i neih tawh leh neih loh.
• I chhungkaw damdawi lam chanchin: I chhungkuaah hian hetiang thisen zam harsatna hi an nei em?
• Thisen test: Hei hi a pawimawh ber a ni. I thisena Protein C activity leh Protein C level te an teh thin.
• Genetic testing: PROC gene-a mutation awm leh awm loh enfiah nan Genetic testing tih theih a ni. Amaherawhchu, hei hi natna finfiah nan a tul lo.

Engtin nge enkawl a nih?

Enkawlna chu Protein C Deficiency chi hrang hrang i neih leh i natna lan chhuah dan nasat danah a innghat a ni.

Mild form tan chuan enkawlna

• Mi tam zawk chuan inenkawl an mamawh lo: Mi tam zawk chuan engmah enkawl ngai an awm lo. Mahse, operation neih lai te, naupai lai te, accident lian tak an neih (e.g., car accident), emaw, taksa tihchak loh hun lai (e.g., khumah an inkhung reng a nih chuan) ngaihven bik leh enkawl a ngai mai thei.
• Anticoagulants: Thrombotic event i neih tawh chuan i doctor chuan anticoagulants a pe ang che.

Form na tak a awm chuan enkawlna

Nau piang tharte he natna khirh tak hi Protein C concentrate (Ceprotin®) emaw fresh frozen plasma emaw tih ang chi enkawlna bik pek a ni.

Hei hian eng harsatna nge a thlen theih?

Hetiang dinhmun avang hian harsatna lian tham engemaw zat a awm thei.

• Warfarin kaihhnawih vun lama harsatna: Warfarin an hman tan hnuah mi thenkhat chuan natna, sen emaw, sen emaw an nei thei. Hengte hi taksa chunglam, kut leh ke emaw-ah a tam ber. A enkawl that loh chuan vun leh a hnuaia tissue te thihna a thlen thei a ni.
• Pulmonary Embolism: Kea thisen zam (deep vein thrombus) lo awm chu a chhuak thei a, thisen kal tlangin a kal a, lung chhunga vein pakhatah a awm thei bawk. Hei hi nunna atana hlauhawm tak a ni.
• Purpura Fulminans: Nausen piang thar taksa pum puia thisen zam (blood clotting problem) a awm. Enkawl loh chuan thihna a thlen thei.
• Plasma therapy thar avanga harsatna awm thei: Naute chu he enkawlna hi pek chhunzawm zel a nih chuan taksaa tui level sang (fluid overload) vangin harsatna a awm thei a ni.

Natna i vei tih hriatchhuah hnuah eng nge i beisei theih ang?

Chumi atanga lo chhuak chu natna nasat dan azirin a inang lo hle.

Naute Protein C Deficiency na tak nei tan chuan prognosis a tha lo hle. An pian hnu rei vak lovah pawh an thi mai thei. Hetiang natna khirh tak vei te hun rei tak chhunga an dam khawchhuah dan tur sawi tur hian thu chiang a la awm lo.

Mild form i neih chuan venous thromboembolism lo lang leh thei i ni. Tin, clots te hi lungah a kal theihna tur a awm bawk. Results tha ber tur chuan doctor follow-up appointment neih reng a pawimawh. Hei hian i dinhmun chu uluk takin a enfiah thei ang a, a tul angin i enkawlna chu a siamrem thei bawk ang.

Hei hi ven theih a ni em?

Protein C tlakchhamna hi a tam zawk chu thlahtute atanga lo chhuak a nih avangin ven theih a ni lo. Mahse, nu leh pa leh naupangte chuan hematologist an zawt thei a, chu chu an zir belh thei a, a tul ang ang test an ti thei bawk.

Tin, Damdawi dang avanga Protein C tlakchhamna hi a chang chuan ven theih a ni bawk. Entir nan,

• Hmeichhiain naupai venna pill thenkhata estrogen hormone a awm hian thisen zam a tipung thei a ni.
• He hlauhawmna hi zuk leh hmuam, thau lutuk, naupai, leh taksa tihchak lohna te hian a tipung lehzual a ni.

A then phei chuan i doctor chuan he hlauhawmna tihziaawmna turin anticoagulants preventive dose a pe thei bawk. Chuvangin, i risk factors chungchang hi doctor nen zalen taka inbiakna neih a pawimawh hle.