Cut te tak te pawhin thisen a chhuah loh theihna condition i nei bawk em? (Factor VII tlakchhamna) 1.1.

A chang chuan cut te tak te pawhin thisen a chhuah loh nan hun rei tak a ngai niin i hria em? A nih loh leh blue leh bruise mai mai i ni em? Heng thilte hi kan hriat loh chhan a awm mai thei. Chu chu Factor VII tlakchhamna chu a ni.

Factor VII Deficiency hi eng nge ni?

A awlsam zawngin Factor VII tlakchhamna hi genetic condition a ni a, i thisen chu a clot tha lo a, chu chu thisen chhuah a titawp nghal vat lo tihna a ni. Kan thisenah hian protein chi hrang hrang a awm a, chu chu ‘blood clotting factors’ emaw ‘coagulation factors’ emaw an ti a ni. Factor VII hi chutiang protein pakhat a ni. Heng protein zawng zawng hian i platelet te nen thawk hovin thisen zam an siam a, i hliam hunah thisen chhuah a titawp thei a ni. Hetiang kalphung hi brick leh cement hmanga bang siam ang mai a ni. Chuvangin, he protein pawimawh tak Factor VII an tih hi i taksaah i neih tlem chuan emaw, a thawk tha lo a nih chuan i thisen chu a pangngai aia rei a ni. Hei vang hian midang aiin i hliam a, thisen i chhuak awlsam zawk.

Hei hi a hming dang chu Alexander natna leh proconvertin tlakchhamna te hi a ni.

He factor VII tlakchhamna hi engtiang chiahin nge a awm?

Hei hi a takah chuan thil awm lo tak a ni . Khawvel pumah mi 500,000 zinga pakhat velin a nghawng a ni . Doctor-te'n "rare" tia an ngaih thisen zam natna (inherited blood clotting disorder) tam tak a awm. Factor VII tlakchhamna hi heng natna hmuh tur awm lo zingah hian a tam ber pakhat a ni.

Hei hi eng symptoms nge ni?

Factor VII tlakchhamna hian thisen a chhuah rei thei a, awlsam takin a hliam thei bawk. A lan chhuah dan ber chu:

• Gums thisen chhuak.
• Hnar thisen chhuak (epistaxis) , leh tihtawp harsat.
• Hmeichhia tan chuan thlasik laiin thisen chhuak nasa (menorrhagia).
• Zun chhunga thisen (hematuria) emaw, ek chhunga thisen awm emaw.
• Hliam, operation emaw nau neih hnua thisen chhuak chhunzawm zel.
• Taksa chhungril lama thisen chhuak (entir nan gastrointestinal system, tissue, muscle, joint-ah).
• Intracranial bleeding pawh a awm thei bawk. Hei hi a pawi deuh deuh.
• Spontaneous bleeding hi chhan hriat loha thisen chhuah nghal a nih chuan a awm thin.

Heng symptoms nasat dan leh a lan chhuah hun hi mi tin a inang lo. Entirnan, naute Factor VII tlakchhamna nasa tak nei chu a pian tirh atangin hmuhchhuah a ni thei. Amaherawhchu, natna lanchhuahna na lo zawk nei mi chu an kum upat hma loh chuan hmuhchhuah a ni lo mai thei.

Factor VII tlakchhamna hi eng nge ni?

This is mainly a hereditary disease , he natna hi dam chhungin a lo awm theih dan pawh a awm bawk.

Factor VII tlakchhamna neia piang i nih chuan i F7 gene -ah mutation, a nih loh leh defect i rochun tihna a ni. He gene hian i taksain factor VII a siam dan a control a ni. Factor VII tlakchhamna nei tur chuan he gene mutation hi nu leh pa dik tak pahnih hnen atangin i rochun a ngai a ni . Hei hi autosomal recessive inheritance an ti a . Nu leh pa pahnih hian he mutation hi an neih chuan factor VII tlakchhamna nei fa neih theihna chance 25% vel an nei a ni.

Hun kal zelah Factor VII tlakchhamna lo awm thin chu ‘acquired factor VII deficiency’ an ti a. A chhan awm thei engemaw zat a awm a:

• Liver natna: I thin hian Factor VII protein a siam a. Thin natna khirh tak i neih chuan i thin hian he hna hi a thawk tha thei lo mai thei.
• Vitamin K tlakchhamna: Factor VII siam nan taksa tan Vitamin K hi a pawimawh hle. I ei tlem chuan Factor VII tlakchhamna i nei thei a ni.
• Thisen tihthianghlimna damdawi: Thisen tihthianghlimna damdawi Warfarin (Coumadin®) hman hunah pawh he natna hi a awm thei bawk.
• Thisen cell lama harsatna leh cancer: Factor VII tlakchhamna hi aplastic anemia leh leukemia ang chi natna vei te zingah hmuhchhuah a ni tawh a ni.

Factor VII tlakchhamna hian eng harsatna nge a thlen?

A nasat chuan Factor VII tlakchhamna hian enkawl loh chuan hriselna lama harsatna dang a thlen thei a ni. Entir nan:

• Anemia: Taksa atanga thisen tam lutuk a bo chuan i red blood cell count nasa takin a tlahniam thei a ni.
• Joint damage: Joint chhunga thisen chhuak a awm chuan chuta tissue chu a chhe thei a, ruh chu a ben tha thei lo va, a dinglam thei lo bawk.
• Hematomas: Hematoma chu i vun hnuaia thisen zam awmkhawm a ni. Hematoma lian tak a lo awm chuan a bul hnaia taksa peng hrang hrangah pressure a siam thei a, natna lan chhuahna a thlen thei bawk.

A pawimawh ber chu thisen chhuak nasa tak hi enkawl loh chuan nunna atana hlauhawm tak a ni thei. Chuvang chuan thisen chhuah a reh loh chuan damdawi lam thurawn lak a pawimawh hle.

Engtin nge Factor VII tlakchhamna hi hriat theih?

I doctor chuan i natna lan chhuah dan leh i chhungte zingah thisen chhuahna lama harsatna nei tawh leh awm loh a zawt ang che. Factor VII tlakchhamna hi thisen test hmanga hriat theih a ni a, chu chuan i thisen a khalh that dan a enfiah thin.

Heng test hrang hrangte hi:

• Prothrombin time (PT/INR) test: Hei hian thisen zam thei thil pakhat prothrombin an tih hnathawh dan a zirin thisen a tikhawlo hun chhung a tehna a ni. Factor VII tlakchhamna i neih chuan thisen zam nan a pangngai aia rei a ngai a ni.
• Partial thromboplastin time (PTT) test: Hei hian prothrombin tih loh clotting factors dangte hnathawh dan atanga thisen zam hun chhung tehna a ni. He test result hi factor VII tlakchhamna nei tan chuan a pangngai tlangpui.
• Factor VII assay: He test hian Factor VII protein hnathawh dan a tarlang a.

Factor VII tlakchhamna hi engtin nge enkawl a nih?

Enkawlna atan chuan i thisenah factor VII hnathawk tha tak dah tel a ni tlangpui. Thisen lama harsatna nei doctor, hematologist tia koh nen i thawk dun mai thei a, chu chuan eng enkawlna nge i mamawh a, eng chen nge i mamawh dawn tih a rel ang.

Enkawl dan tlangpui engemaw zat a awm a:

• Recombinant factor VIIa (NovoSeven®): He damdawi hi US Food and Drug Administration (FDA) chuan factor VII tlakchhamna enkawlna atan a pawmpui a ni. Laboratory-a scientist-te siam a ni. Hei hian factor VII thlak danglamna damdawi dang ang lo takin mi thisen pek atanga siam a ni lo tihna a ni.
• Prothrombin complex concentrates (PCC): He enkawlnaah hian thisen pek atanga lak chhuah clotting factor pali, factor VII te pawh a tel a, a tam hle.
• Fresh frozen plasma (FFP): I Factor VII stores siam thar lehna tura pui turin plasma intravenous-in pek i ni thei bawk.

Enkawlna dang: 1.1.

• Antifibrinolytic agents: Heng damdawi te hian i taksaa thisen zam lo awmte chu a hmin loh nan a titawp a ni. I taksaa thisen zam awm tawhte venhimna hmangin thisen chhuah tam lutuk tur venna atan an pui thin. Entirnan damdawi aminocaproic acid leh tranexamic acid tih te hi a ni .
• Naupai venna damdawi: 1.1.Birth control pill chi thenkhat chuan thlasik laiin thisen chhuak nasa tak a veng thei.
• Vitamin K supplement emaw injection emaw: I natna hi vitamin K tlakchham vang a nih chuan vitamin K tam zawk ei hian i taksain factor VII tam zawk a siam thei a ni.

Enkawlna avanga harsatna/side effects awmte chu engte nge ni?

Thisenah factor VII dah belhna enkawlna hian a chang chuan harsatna a thlen thei a ni. Mahse, i doctor hian heng hlauhawmnate hi a hre chiang a, a venna atan hma a la mek tih hriat reng a pawimawh.

Factor VII therapy avanga harsatna awm thei te chu:

• Thisen zam chhiatna: Factor VII dah belh hian thisen kalna kawng tikhawlo thei thisen zam a tipung thei a ni. He hlauhawmna hi tihziaawmna turin i doctor chuan i damdawi ei zat a enfiah ang.
• Pathogens nena inzawmna: Thisen pek kan dawn hian thisenah hian natna hrik (e.g., virus emaw bacteria emaw) inkaichhawn theihna a awm fo thin. Hetiang a nih loh nan Doctor ten thisen atanga thil siamte uluk takin an screen thin.
• Transfusion reaction: Plasma donated-ah allergy i nei thei.
• Plasma overload: Factor VII hi a chhe rang lutuk a, i thisen atanga lakchhuah a ni. Hei hi ven nan chuan i taksaah factor VII tling tawk awm reng turin plasma tam tak i mamawh mai thei. Hei hian i system-a plasma a tipung thei a ni. Plasma overload hian thisen sang leh lung natna (pulmonary edema) a nghawng thei a ni.

Factor VII tlakchhamna ka neih chuan eng nge ka beisei theih ang?

Factor VII tlakchhamna hi dam chhung zawnga awm thin a ni . Damdawi awm lo mah se, i doctor nen thawkdunin i enkawl thei a ni.

Natna hi engtiang taka na nge a nih dawn tih hriatna kawnga harsatna lian ber pakhat chu i Factor VII level a hniam dan a zirin a natna lanchhuahna nasat dan tur sawi lawk theih a nih loh vang a ni. Chu ai chuan doctor-te chuan he tlakchhamna nei te hi mimal takin an thawk ho thin. I thlirna chu i thil tawn, i tlakchham chhan, enkawlna i dawn dan, leh i thisen chhuahna a nasat dan te ang chi thil danglam takah a innghat ang.

Engtin nge ka in enkawl ang?

I dinhmun enkawltu healthcare provider zawng zawngte hrilh vek a pawimawh . I fain factor VII tlakchhamna a neih chuan a enkawltu tu pawh (zirtirtu leh daycare worker te pawh tel) hnenah hrilh rawh.

Nitin i in enkawl dan tur i doctor zawt thin ang che. Amah chuan hetiang hi a rawt thei a ni:

• Risk sang tak nei thil tih loh tur:Hliam hlauhawmna tipung thei thil (e.g., contact sports) tih loh i duh mai thei.
• Damdawi thenkhat ei loh tur: I thisen tithianghlim thei damdawi (e.g., aspirin , NSAIDs , SSRI antidepressants ) lakah i inthiarfihlim a ngai mai thei. Fish oil, vitamin E, leh turmeric awmna supplement pawh ei loh i duh mai thei, hengte hian thisen chhuahna a tipung thei bawk.
• I enkawlna tur ruahmanna copy vawng la: Emergency a lo thlen hunah healthcare professional-te’n an enkawl dan tur chungchanga i hematologist hnen atanga documentation i neih chu vawn reng a pawimawh.
• Genetic counseling emaw testing emaw la rawh: Nau neih i tum hmain i fain factor VII tlakchhamna a neih theih dan tur ngaihtuah turin genetic counselor i biak thei ang.

Engtikah nge ka doctor ka hmuh ang?

Nangmah emaw i fa emaw chuan thisen chhuak reng emaw, blueness awm fo emaw chhinchhiahna i hmuh chuan doctor pan rawh. Hnar thisen chhuak leh a chang chuan blueness hi thil pangngai a ni. Mahse, a tlangpui aia tam an lo thleng a nih chuan thisen lama harsatna a awm tih chhinchhiahna a ni thei.

Engtikah nge Emergency Treatment Unit (ETU) ah ka kal ang ?

I thisen chhuah hi i hriselna atana hlauhawm a nih chuan emergency room-ah kal rawh. Hetiang chhinchhiahna hi zawng rawh:

• Darkar khat emaw a aia rei emaw chhung hnar thisen chhuak.
• Darkar khatah pad emaw tampon pahnih aia tam darkar hnih emaw a aia tam (hmeichhia tan) hnim hnim.

Doctor hnenah eng zawhna nge ka zawh ang?

Doctor i zawh theih tur zawhna thenkhat chu hetiang hi a ni:

• Ka Factor VII tlakchhamna hi thlahtute atanga lo chhuak nge a hnu lama lo awm?
• Eng ang enkawlna nge ka mamawh?
• Eng chen nge ka enkawl a ngai dawn?
• Enkawl lai hian eng side effect nge ka hriat tur?
• Ka dinhmun enkawl turin eng nunphung tihdanglam nge ka neih?

Factor VII tlakchhamna hi ‘hemophilia’ nen a inang em?

Factor VII tlakchhamna leh hemophilia hi thisen lama natna inzawm, mahse a hran, rochun chhuah pahnih a ni. Factor VII tlakchhamna hi protein Factor VII tlakchham vang a ni (7), hemophilia A , hemophilia chi khat, protein Factor VIII tlakchham vang a ni thung (8).

Hemophilia A hi factor VII tlakchhamna aiin a tam zawk a, mi 100,000 zinga pakhat velin a vei a ni. Mipaah ​​pawh a tam zawk .

Mahse a pawimawh ber chu heng pahnih hian thisen chhuah tam lutuk a thlen thei a, chuvangin doctor puihnain uluk taka enkawl a ngai a ni.

Hriat reng tur pawimawh ber berte

Thisen zam natna factor VII tlakchhamna neia piang tur ven dan tur a awm lo. Mahse chutiang a nih chuan nang (or i naute) hian he natna nen hian nun hrisel, active takin i hmang thei lo tihna a ni lo. I hematologist hian thisen chhuah loh leh enkawl danah a pui thei che a ni. Thisen chhuak tam lutuk a nih chuan eng nge i tih tur tih ruahmanna siam turin a pui thei bawk che. Lungngai suh, he dinhmun hi hlawhtling takin i nung thei a ni.

` Factor VII tlakchhamna, Thisen zam, Thisen chhuah, Hriselna (Hereditary Diseases), Alexander natna, Hematologist, Thisen natna