He nui hoki te toto e puta mai ana i tētahi patunga iti? Me kōrero tātou mō te Mate Bernard-Soulier (BSS)!

Kua kite koe i ētahi tāngata e toto nui ana ahakoa he rakuraku iti noa iho, he maru noa iho rānei, he ngawari rānei te maru, ā, he roa te wā e ora ai? I ētahi wā, tera pea nā tētahi āhuatanga kāore anō kia rongohia, engari he mea nui kia mōhiotia. Ko tētahi o aua āhuatanga ko te Bernard-Soulier Syndrome, ko te ``(BSS)`` rānei. Me kōrero tātou mō tēnei i tēnei rā.

He aha te mate Bernard-Soulier (BSS)?

Ki te kī māmā noa, he tino onge te mate Bernard-Soulier `(BSS)`. Ko te mea ka tupu i roto i tēnei ko te tukanga toto totoka i roto i tō tātou tinana, arā, ka mutu te toto ina whara tātou. Whakaarohia, he patunga iti tō ringa. I muri i tētahi wā, ka mutu te toto, tika? Āe, ko te mea e āwhina ana i tēnā ko ngā pereti toto, he momo pūtau iti i roto i ō tātou toto, me te plasma, te wāhanga wai o te toto.

He iti ake ngā pereti toto o te tangata whai mate Bernard-Soulier (BSS) i te mea noa. Ka kiia tēnei mate e ngā tākuta he thrombocytopenia. Waihoki, he nui ake ngā pereti toto i ngā pereti toto noa. Nō reira, nā ēnei take, ka totohu, ka maru ngāwari hoki te hunga whai mate BSS.

E hia te onge o tēnei āhuatanga?

He tino onge tēnei mate. E ai ki ngā tohunga hauora, kotahi anake i roto i te miriona tāngata o te ao e pāngia ana e tēnei mate. Ko te tikanga, ahakoa i Sri Lanka, he tokoiti noa iho te hunga e pāngia ana e tēnei mate.

He aha ngā tohu o te Mate Bernard-Soulier (BSS)?

He maha ngā wā ka tīmata ngā pēpi whai BSS ki te whakaatu i ēnei tohu i te tīmatanga o te oranga. Tirohia mēnā kei a koe, kei tētahi atu rānei e mōhio ana koe, tētahi o ēnei tohu:

• He ngawari te maru, ā, he roa te wā e ora ai: Ahakoa he tukinga noa iho i runga i te tūru ka waiho he maru nui. E hia wiki pea te roa e ora ai taua maru.
• Te toto nui ahakoa he motu me he patunga iti: Ahakoa he motu iti kei te heu koe, he tapahi rānei i tō ringa ki te maripi i a koe e tapatapahi ana i ngā huawhenua, he uaua ki te whakamutu i te toto.
• Te toto ihu auau: E kīia ana tēnei e ētahi he `(epistaxis)`. Mēnā he toto ihu auau tōu, kāore he take, he tohu anō hoki tēnā.
• Te toto i raro i te kiri: Ka puta ēnei hei ira whero, papura rānei i runga i te mata o te kiri. Ka kiia ēnei e ngā tākuta he "petechiae".
• Te toto nui i te wā o te paheketanga i roto i ngā wāhine: Mena ka roa ake te toto mō ētahi rā i te tikanga, he nui rānei (menorrhagia), he mea nui anō hoki tēnā.

Kaua e wehi i te BSS nā te mea kei a koe tētahi, e rua rānei o ēnei tohu. Engari ki te mau tonu ēnei tohu, he pai ake te rapu tohutohu hauora.

He aha te take o te mate Bernard-Soulier (BSS)?

Koinei te mea tino nui. He mate ira te `(BSS)`.Arā, he āhuatanga ka puta mai i te whānautanga mai o te tangata nā te huringa (whakarerekētanga ira) o ō tātou ira. He mea tuku iho tēnei, arā, ka taea e ngā tamariki te whiwhi i tēnei huringa ira mai i ō rātou mātua.

Ko tēnei āhuatanga (BSS) i puta mai i ngā whakarerekētanga ira o ngā ira `GP1BA`, `GP1BB`, `GP9` rānei. He mea nui katoa ēnei ira mō te tukanga toto totoka i roto i tō tātou tinana. Nō reira, ina he raruraru ki ēnei ira, kāore e tika te totoka.

Me pēhea koe e mōhio ai mēnā kei a koe te Mate Bernard-Soulier (BSS)?

Ki te pā mai ēnei tohu ki a koe, ko te mea tuatahi ka mahia e te tākuta ina kite koe i ēnei tohu ko te pātai atu i tētahi pātai taipitopito ki a koe. Kātahi ka whakahaua e rātou ētahi whakamātautau toto hei whakaū i te āhua.

• Whakamātautau tatau pereti toto: Mā tēnei ka tirohia te maha o ngā pereti toto i roto i ō toto, mehemea rānei he iti.
• Ngā whakamātautau whakarerekētanga ira: Mā tēnei ka kitea mēnā he whakarerekētanga ira kei roto i ngā ira `GP1BA`, `GP1BB`, `GP9` kua whakahuatia ake nei e hua ai te `(BSS)`.
• Whakamātautau karuārai toto: Ka tangohia he tauira o tō toto ka tirohia i raro i te karuārai toto hei tirotiro mēnā he nui rawa ngā karuārai toto.
• Te ine i te rere o te toto i roto i ngā pereti toto: Ahakoa he ingoa uaua, ka ine tēnei whakamātautau i te nui o ngā glycoprotein e kiia nei ko `GP1BA`, `GP1BB`, `GP9` i runga i te mata o ngā pereti toto. Mena he iti ēnei, he tohu tēnei o te `(BSS)`.

I runga i ngā hua o ēnei whakamātautau ka mōhio ai te tākuta mēnā kei a koe te BSS.

He aha ngā maimoatanga mō te Mate Bernard-Soulier (BSS)?

Kāore anō kia kitea he rongoā mō te BSS i tēnei wā, nā te mea he mate ira. Heoi, he maha ngā mea ka taea e koe te mahi hei ārai i te toto nui me te whakaiti i ētahi atu raruraru ka puta. Ko te nuinga o te maimoatanga e aro ana ki tēnā.

Ngā mea ka taea e koe te mahi hei whakaiti i te toto:

He mea māmā noa iho ēnei, engari he mea tino nui ka taea e koe te tāpiri atu ki tō oranga o ia rā.

• Āraia ngā rongoā e whakanui ake ana i te mōrearea o te toto: Ka taea e ētahi rongoā whakamamae mamae, pērā i ngā rongoā anti-inflammatory kore-steroidal (NSAID), te aspirin, me ētahi antihistamine te whakanui ake i te mōrearea o te toto. Nō reira, kaua e tango i ēnei rongoā me te kore kōrero ki te tākuta.
• Whakamahia he paraihe niho ngohengohe ina paraihia ō niho: Ka taea e tēnei te whakaiti i te toto o ngā kapia.
• Akohia te huarahi tika hei whakamutu i te toto o te ihu: pātai atu ki tō tākuta mō te pupuri i tō ihu me te pupuri i tō mahunga.
• Whakawhāitihia ētahi kai me ētahi inu: Ka pāngia te maha o ngā pereti toto e ngā kai he nui te waipiro me te huka kua whakatikatikaina, nō reira he pai ake te whakawhāiti i aua kai i te mea ka taea.
• Rēhita ki tētahi pokapū e tuku ana i te tiaki ohorere 24-hāora: Mā tēnei koe e āwhina ki te whiwhi maimoatanga tere i roto i tētahi āhuatanga ohorere, pērā i te toto nui.
• Tangohia ngā tāpiringa rino mēnā he ngoikore tō rino: Mā te toto tonu ka arahi pea ki te ngoikore o te rino. Tangohia ngā pire rino kia rite ki ngā tohutohu a tō tākuta.
• Mauria he poroporo, he tohu rānei mō te tuakiri hauora: He mea tino nui i roto i te āhuatanga ohorere kia mauria he poroporo, he tohu rānei e whakaatu mārama ana kei a koe he `(BSS)`.

Maimoatanga hauora:

I ētahi wā, inā koa i ngā wā o te toto ohorere, i te wā rānei e whakarite ana mō te pokanga, ka whakaratohia pea e ngā tākuta he maimoatanga pēnei i:

• Ngā whakawhiti toto toto: Koinei te maimoatanga tuatahi mō te ohorere toto. Ka tukuna ngā toto toto mai i te kaituku ki roto i tō tinana mā te raina IV. Ka āwhina ēnei toto toto toto ki te totoka.
• Te rongoā ārai-fibrinolytic: He rongoā ēnei hei āwhina i te toto kia totoka. Hei tauira, ko te waikawa tranexamic, kua rongo pea koe mōna, kei te wātea hoki i raro i te ingoa waitohu "Cyklokapron®". Ka āwhina tēnei ki te whakahaere i te toto.

Ka taea te ārai i te mate Bernard-Soulier (BSS)?

Engari, kāore he huarahi hei aukati i te whanaketanga o te BSS nā te mea he mate ira.

Heoi, ki te mea he BSS tō tētahi o tō whānau, ki te whakaaro rānei koe kei a koe tēnei whakarerekētanga ira, me whakaaro pea koe ki te whakamātautau ira . He mea nui anō hoki kia rapu tohutohu ira . Ka taea e te kaitohutohu ira te kōrero atu ki a koe mō te āhua me te whakamārama i te mōrearea o te tuku atu ki ō tamariki.

He aha ētahi mea hei mōhio māu ina noho tahi koe me te Mate Bernard-Soulier (BSS)?

Ka taea e te nuinga o te hunga whai BSS te noho noa i te ao. Heoi, me tino tupato ake rātou kia iti ake ai te tūponotanga o te whara.

Hei tauira, he mea pai kia kaua e takaro i ngā hākinakina pā atu ki te kiri, nā te mea he nui ake te mōrearea o te whara me te toto nui.

He mea nui anō hoki kia kite i tō kaiwhakarato ratonga hauora tuatahi i ia wā, i ia wā, kia mau tonu ai tō hauora whānui. Mēnā ka pokangahia koe, me whakarite pea i mua, ā, me whiwhi pea koe i te whakawhiti toto ki te toto i mua i te pokanga.

Mena kei a koe te Mate Bernard-Soulier (BSS), he aha ngā pātai me pātai atu koe ki tō tākuta?

Ki te whakaaro koe kei a koe (BSS) kua kitea rānei kei a koe, he mea tino āwhina te pātai atu ki tō tākuta i ēnei pātai:

• He aha ngā tohu tuatahi o te Mate Bernard-Soulier?
• He aha ngā whakamātautau me mahi e au kia mōhio ai mēnā kei a au te mate Bernard-Soulier?
• He aha ngā kōwhiringa maimoatanga mō te Matepukupuku Bernard-Soulier?
• Mena kei a au te Mate Bernard-Soulier, me aha ahau hei ārai i te toto nui rawa?
• He aha ngā mahi kāore e tika kia mahia e au nā te mea kei a au te mate Bernard-Soulier?
• He aha ngā tūponotanga ka riro mai i aku tamariki te mate Bernard-Soulier i ahau?

Haunga ēnei pātai, kaua e wehi ki te kōrero ki te tākuta mō ngā mea katoa e puta ake ana ki tō hinengaro.

He mōrearea ki te pēpi te pāngia e te mate Bernard-Soulier (BSS) i te wā e hapu ana?

Mena kei te hapu koe, ā, he BSS tō pēpi, me whiwhi koe i te tiaki hauora motuhake (tiaki whānau me te tiaki toto mōrearea nui) . Me aroturuki tonu tō tatau pereti toto me tō hauora whānui. I te wā e whānau ana, tērā pea kāore e taea e koe te whakamahi i ētahi momo rongoā whakamoe ka whakanui ake i te mōrearea o te toto.

Ahakoa kua whānau te pēpi, me tirotiro tonu te maha o ngā pereti toto o te pēpi. Nā te mea tera pea ka riro mai i tō pēpi te whakarerekētanga ira e puta ai te "(BSS)" mai i a koe. Nō reira, he mea nui kia kōrero koe ki tō tākuta mō tēnei mea, kia mahi hoki i ngā mahi e tika ana.

He aha te raruraru matua i puta mai i te Mate Bernard-Soulier (BSS)?

I roto i te poto, ko te raru matua e puta mai ana i te "BSS" ko te kore e taea e te toto te totoka . Koia te take ko te toto nui me te ngawari o te marutanga ngā tohu tino kitea.

He aha atu ngā āhuatanga e puta ai ngā tohu e rite ana ki te Mate Bernard-Soulier (BSS)?

He maha atu anō ngā āhuatanga e puta ai ngā raruraru toto me ngā tohu e rite ana ki te BSS. He mea pai kia mōhiotia ēnei mā te ingoa anake:

• 'Hemophilia'
• `Te rerekētanga o Mei-Hegglin`
• 'Mate puna kaukau rokiroki'
• Te totohu o Glanzmann
• 'Te mate a Von Willebrand'

I runga i ō tohu, ko te tākuta anake ka taea te whakatau ko tēhea o ēnei mate.

Ā mātou kupu whakamutunga

He tino onge te mate Bernard-Soulier `(BSS)` e tukuna iho ana i ō mātua. Mēnā kei a koe tēnei mate, tera pea ka pāngia koe e ētahi raruraru ki te toto totoka. Heoi, ehara i te mea kāore e taea e koe te noho i tētahi oranga noa. Ko te mea nui ko te karo i ngā āhuatanga morearea ka arahi pea ki te whara, me te whai tonu i ngā tohutohu a tō tākuta.

Tirohia tō tākuta whānau i ia wā, i ia wā. Ka taea e ia te whakautu i ō pātai, te āwhina i a koe ki te whakahaere i tēnei mate, me te mahi tahi me koe ki te pupuri i tō hauora whānui. Kaua e mataku, ko te mōhio me te manaaki ko ō tātou kaha nui rawa atu i a tātou e noho tahi ana me ēnei mate.

Te Mate Bernard-Soulier, te BSS, te toto totoka, ngā pereti toto, te toto nui, ngā mate ira, te marō