Ua ʻike anei ʻoe i kekahi poʻe e kahe nui ana ke koko mai kahi ʻōpala liʻiliʻi a i ʻole kahi palapū maʻalahi, a i ʻole he maʻalahi nā palapū a lōʻihi ka manawa e ho'ōla ai? I kekahi manawa hiki ke kumu kēia o kahi kūlana i lohe ʻole ʻia e mākou, akā he mea nui loa ia e ʻike. ʻO kekahi o ia ʻano kūlana ʻo Bernard-Soulier Syndrome, a i ʻole ``(BSS)`` no ka pōkole. E kamaʻilio kākou e pili ana i kēia me nā kikoʻī hou aku i kēia lā.
He aha ka Bernard-Soulier Syndrome (BSS)?
I ka ʻōlelo maʻalahi, he maʻi laha ʻole ka Bernard-Soulier Syndrome `(BSS)`. ʻO ka mea e hana ʻia ma kēia, ʻo ia ke kaʻina hana o ke kāpili ʻana o ke koko i loko o ko mākou kino, ʻo ia hoʻi, ua pau ke kahe ʻana o ke koko ke hōʻeha ʻia mākou. E noʻonoʻo, he ʻeha liʻiliʻi kou ma kou lima. Ma hope o kekahi manawa, pau ke kahe ʻana o ke koko, ʻeā? ʻĀ, ʻo ka mea e kōkua ana i kēlā, ʻo ia nā platelets, kahi ʻano o nā cell liʻiliʻi i loko o ko mākou koko, a me ka plasma, ʻo ia ka ʻāpana wai o ke koko.
ʻOi aku ka liʻiliʻi o nā platelets o ke kanaka me Bernard-Soulier Syndrome (BSS) ma mua o ka maʻamau. Ua kapa nā kauka i kēia maʻi he thrombocytopenia. Eia kekahi, ʻoi aku ka nui o nā platelets i loaʻa ma mua o nā platelets maʻamau. No laila, no kēia mau kumu, kahe ke koko a me ka palapū maʻalahi o ka poʻe me BSS.
Pehea ka laha ʻole o kēia maʻi?
He ʻano maʻi laha ʻole loa kēia. Wahi a nā loea lapaʻau, hoʻokahi wale nō o ka miliona o ka poʻe ma ka honua holoʻokoʻa e hoʻopilikia nei kēia maʻi. ʻO ia hoʻi, ma Sri Lanka hoʻi, he kakaikahi loa ka poʻe e ʻeha nei i kēia maʻi.
He aha nā hōʻailona o ka Bernard-Soulier Syndrome (BSS)?
I ka manawa pinepine, hoʻomaka nā pēpē me BSS e hōʻike i kēia mau hōʻailona i ka wā ʻōpiopio o ke ola. E nānā e ʻike inā loaʻa iā ʻoe a i ʻole kekahi āu i ʻike ai kekahi o kēia mau hōʻailona:
- He maʻalahi kou palapū, a he lōʻihi ka manawa e ho'ōla ai: ʻOiai he puʻupuʻu maʻalahi ma ka noho hiki ke waiho i kahi palapū nui. Hiki ke hala nā pule no ka ho'ōla ʻana o kēlā palapū.
- Ke kahe nui nei ke koko mai nā ʻoki liʻiliʻi a me nā ʻeha: Inā loaʻa iā ʻoe kahi ʻoki liʻiliʻi i ka wā e kahi ai a ʻoki paha i kou lima me ka pahi i ka wā e ʻoki ana i nā mea kanu, he paʻakikī ke hoʻōki i ke kahe ʻana o ke koko.
- Ka kahe pinepine ʻana o ka ihu: Kapa kekahi poʻe i kēia he `(epistaxis)`. Inā he pinepine kou kahe koko ihu me ke kumu ʻole, he hōʻailona nō hoʻi kēlā.
- Ke kahe ʻana o ke koko ma lalo o ka ʻili: ʻIke ʻia kēia mau mea ma ke ʻano he mau kiko liʻiliʻi ʻulaʻula a poni paha ma ka ʻili o ka ʻili. Kapa nā kauka i kēia mau mea he "petechiae".
- Ke kahe koko nui i ka wā o ka menstruation i nā wahine: Inā he mau lā ka lōʻihi o ke kahe koko ma mua o ka maʻamau, a inā he kaumaha (menorrhagia), he mea hopohopo nō hoʻi ia.
Mai makaʻu i ka BSS no ka mea wale nō he hoʻokahi a ʻelua paha o kēia mau hōʻailona kou. Akā inā hoʻomau kēia mau hōʻailona, ʻoi aku ka maikaʻi o ka ʻimi ʻana i ke kōkua lapaʻau.
He aha ke kumu o ka Bernard-Soulier Syndrome (BSS)?
ʻO kēia ka mea nui loa. He maʻi hoʻoilina ka `(BSS)`.ʻO ia hoʻi, he kūlana e kū mai ana i ka wā e hānau ʻia ai kākou ma muli o kahi loli (mutation) i loko o kā kākou mau genes. He mea hoʻoilina kēia, ʻo ia hoʻi, hiki i nā keiki ke hoʻoilina i kēia loli genetic mai ko lākou mau mākua.
Hoʻokumu ʻia kēia kūlana (BSS) e nā mutations i loko o nā genes `GP1BA`, `GP1BB` a i ʻole `GP9`. He mea nui loa kēia mau genes a pau no ke kaʻina hana koko i loko o ko mākou kino. No laila, i ka wā e loaʻa ai ka pilikia me kēia mau genes, ʻaʻole hana pono ʻia ke koko koko.
Pehea ʻoe e ʻike ai inā loaʻa iā ʻoe ka Bernard-Soulier Syndrome (BSS)?
Inā loaʻa iā ʻoe kēia mau hōʻailona, ʻo ka mea mua a ke kauka e hana ai ke ʻike ʻoe iā lākou, ʻo ia ke nīnau iā ʻoe i kahi nīnau kikoʻī. A laila, e kauoha lākou i kekahi mau hoʻāʻo koko e hōʻoia i ke kūlana.
- Hoʻāʻo helu platelet: Nānā kēia i ka helu o nā platelet i loko o kou koko a inā he haʻahaʻa lākou.
- Nā hoʻokolohua hoʻololi gene: Hiki i kēia ke ʻike inā he mau mutations i loko o nā genes 'GP1BA', 'GP1BB', 'GP9' i ʻōlelo mua ʻia e hoʻoulu ai i ka '(BSS)'.
- Ka hoʻokolokolo ʻana o nā platelet ma ke ʻano microscopic: Lawe ʻia kahi hāpana o kou koko a nānā ʻia ma lalo o kahi microscope e ʻike ai inā nui loa nā platelets.
- ʻO ka cytometry kahe platelet: ʻOiai he inoa paʻakikī kona, ke ana nei kēia hoʻāʻo i ka nui o nā glycoproteins i kapa ʻia ʻo `GP1BA`, `GP1BB`, `GP9` ma ka ʻili o nā platelets. Inā haʻahaʻa kēia mau mea, he hōʻailona ia o `(BSS)`.
Ma muli o nā hopena o kēia mau hoʻokolohua e haʻi pololei ai ke kauka iā ʻoe inā he BSS kou a ʻaʻole paha.
He aha nā lāʻau lapaʻau no ka Bernard-Soulier Syndrome (BSS)?
ʻAʻohe lāʻau lapaʻau no ka BSS i kēia manawa, no ka mea, he maʻi hoʻoilina ia. Eia nō naʻe, nui nā mea hiki iā ʻoe ke hana e pale aku i ke kahe koko nui a hōʻemi i nā pilikia ʻē aʻe e kū mai ana. ʻO ke kumu nui o ka mālama ʻana, ʻo ia ke kau nui ʻana ma luna.
Nā mea hiki iā ʻoe ke hana e hōʻemi i ke kahe ʻana o ke koko:
He mau mea maʻalahi kēia, akā he mau mea nui loa hiki iā ʻoe ke hoʻohui i kou ola o kēlā me kēia lā.
- E pale i nā lāʻau lapaʻau e hoʻonui ai i ka pilikia o ke kahe ʻana o ke koko: ʻO kekahi mau lāʻau ʻeha, e like me nā lāʻau anti-inflammatory nonsteroidal (NSAID), aspirin, a me kekahi mau antihistamines hiki ke hoʻonui i ka pilikia o ke kahe ʻana o ke koko. No laila, mai lawe i kēia mau lāʻau lapaʻau me ka ʻole o ka nīnau ʻana i ke kauka.
- E hoʻohana i kahi palaki niho palupalu i ka palaki ʻana i kou mau niho: Hiki i kēia ke hōʻemi i ke kahe ʻana o nā ʻāʻī.
- E aʻo i ke ala kūpono e hoʻōki ai i ke kahe ʻana o ka ihu: e nīnau i kāu kauka e pili ana i ke ʻano o ka paʻa ʻana i kou ihu a pehea e paʻa ai i kou poʻo.
- E kaupalena i kekahi mau meaʻai a me nā mea inu: ʻO nā meaʻai kiʻekiʻe i ka waiʻona a me nā kō i hoʻomaʻemaʻe ʻia e hiki ke hoʻopilikia i kāu helu platelet, no laila ʻoi aku ka maikaʻi o ka palena ʻana iā lākou i ka hiki.
- E kāinoa ma kahi kikowaena e hāʻawi ana i ka mālama ulia pōpilikia 24-hola: E kōkua kēia iā ʻoe e loaʻa koke ka lāʻau lapaʻau i kahi ulia pōpilikia, e like me ke kahe koko nui.
- E lawe i nā mea hoʻohui hao inā nele ʻoe i ka hao: Hiki i ke kahe pinepine ʻana ke alakaʻi i ka nele i ka hao. E lawe i nā papa hao e like me ke kuhikuhi a kāu kauka.
- E komo i kahi kūpeʻe lima a i ʻole kahi lepili hōʻoia lapaʻau: He mea nui loa i ka wā pilikia e komo i kahi kūpeʻe lima a i ʻole kahi lepili e haʻi maopopo ana he `(BSS)` kou.
Lapaʻau lapaʻau:
I kekahi mau hihia, ʻoi aku hoʻi i nā hihia o ke kahe koko koke a i ʻole i ka wā e hoʻomākaukau ai no ke ʻoki ʻana, hiki i nā kauka ke hāʻawi i ka lāʻau lapaʻau e like me:
- Nā hoʻoili koko platelet: ʻO kēia ka lāʻau lapaʻau mua no kahi pilikia kahe koko. Hāʻawi ʻia nā platelets mai kahi mea hāʻawi i loko o kou kino ma o kahi laina IV. Kōkua kēia mau platelets i kou ʻūlū koko.
- Lapaʻau Antifibrinolytic: He mau lāʻau lapaʻau kēia e kōkua ai i ke kāpili ʻana o ke koko. No ka laʻana, ʻo ka waikawa tranexamic, āu i lohe ai paha, loaʻa pū kekahi ma lalo o ka inoa inoa "Cyklokapron®". Kōkua kēia i ka kaohi ʻana i ke kahe ʻana o ke koko.
Hiki ke pale ʻia ka Bernard-Soulier Syndrome (BSS)?
ʻO ka mea pōʻino, ʻaʻohe ala e pale ai i ka ulu ʻana o ka BSS no ka mea he maʻi hoʻoilina ia.
Eia nō naʻe, inā loaʻa i kekahi o kou ʻohana ka BSS, a inā manaʻo ʻoe he loaʻa paha iā ʻoe kēia hoʻololi ʻana o ka gene, makemake paha ʻoe e noʻonoʻo i ka hoʻāʻo ʻana i ka genetic . He mea nui hoʻi e ʻimi i ke aʻoaʻo genetic . Hiki i kahi kākāʻōlelo genetic ke haʻi hou aku iā ʻoe e pili ana i ke kūlana a wehewehe i ka pilikia o ka hoʻoili ʻana i kāu mau keiki.
He aha kekahi mau mea e pono ai ke makaʻala i ka wā e noho ai me ka Bernard-Soulier Syndrome (BSS)?
Hiki i ka hapa nui o ka poʻe me BSS ke ola i kahi ola maʻamau. Eia nō naʻe, pono lākou e akahele iki aʻe e hōʻemi i ka pilikia o ka ʻeha.
Eia kekahi laʻana, ʻoi aku ka maikaʻi o ka noho mamao aku mai nā haʻuki pili, no ka mea, ʻoi aku ka nui o ka hōʻeha a me ke kahe koko nui.
He mea nui hoʻi e ʻike pinepine i kāu mea mālama ola mua e mālama i kou olakino holoʻokoʻa. Inā hana ʻoe i ke ʻoki kino, pono paha e hoʻonohonoho mua a loaʻa paha kahi hoʻoili platelet ma mua o ke kaʻina hana.
Inā loaʻa iā ʻoe ka Bernard-Soulier Syndrome (BSS), he aha nā nīnau āu e nīnau ai i kāu kauka?
Inā manaʻo ʻoe ua loaʻa iā ʻoe (BSS) a ua ʻike ʻia paha me ia, he mea kōkua nui ka nīnau ʻana i kāu kauka i nā nīnau e like me kēia:
- He aha nā hōʻailona mua o ka maʻi Bernard-Soulier?
- He aha nā hoʻokolohua e pono iaʻu e hana e ʻike pono ai inā loaʻa iaʻu ka Bernard-Soulier Syndrome?
- He aha nā koho lapaʻau no ka Bernard-Soulier Syndrome?
- Inā loaʻa iaʻu ka Bernard-Soulier Syndrome, he aha kaʻu e hana ai e pale aku ai i ke kahe nui ʻana o ke koko?
- He aha nā hana e pono ʻole iaʻu e hana no ka mea he Bernard-Soulier Syndrome koʻu?
- He aha nā manawa kūpono e hoʻoilina ai kaʻu mau keiki i ka Bernard-Soulier Syndrome mai iaʻu?
Ma waho aʻe o kēia mau nīnau, mai makaʻu e kamaʻilio me ke kauka e pili ana i nā mea a pau e hiki mai ana i kou manaʻo.
He mea weliweli anei ka loaʻa ʻana o ka Bernard-Soulier Syndrome (BSS) i ka wā hāpai i ka pēpē?
Inā hāpai ʻoe a loaʻa iā ʻoe ka BSS, pono ʻoe i ka mālama lapaʻau kūikawā (mālama obstetric a me hematology kiʻekiʻe) . Pono e nānā mau ʻia kou helu platelet a me kou olakino holoʻokoʻa. I ka wā hānau, ʻaʻole paha hiki iā ʻoe ke loaʻa i kekahi mau ʻano anesthesia e hoʻonui ai i ka pilikia o ke kahe ʻana o ke koko.
ʻOiai ma hope o ka hānau ʻana o ka pēpē, pono e nānā mau ʻia ka helu platelet o ka pēpē. No ka mea, aia kahi manawa e hoʻoilina ai kāu pēpē i ka hoʻololi genetic e hoʻoulu ai i ka "(BSS)" mai iā ʻoe. No laila, he mea nui e kamaʻilio me kāu kauka e pili ana i kēia a hana i nā hana e pono ai.
He aha ka pilikia nui i hoʻokumu ʻia e ka Bernard-Soulier Syndrome (BSS)?
I ka pōkole, ʻo ka pilikia nui i hoʻokumu ʻia e ka "BSS" ʻo ia ka hiki ʻole o ke koko ke kāpili . ʻO ia ke kumu o ke kahe nui ʻana o ke koko a me ka maʻalahi o ka palapū nā hōʻailona maʻamau.
He aha nā kūlana ʻē aʻe e hoʻoulu ai i nā hōʻailona like me ka Bernard-Soulier Syndrome (BSS)?
Aia kekahi mau kūlana ʻē aʻe e hoʻoulu ai i nā pilikia kahe koko a me nā hōʻailona like me BSS. He mea maikaʻi ke ʻike i kēia mau mea ma ka inoa wale nō:
- ʻO ka hemophilia
- ʻAnomalia o Mei-Hegglin
- ʻO ka maʻi o ka loko wai mālama
- ʻO ka maʻi Glanzmann thrombostenia
- ʻO ka maʻi ʻo Von Willebrand
Ma muli o kāu mau hōʻailona, hiki i ke kauka ke hoʻoholo pololei i kahi o kēia mau maʻi.
ʻO kā mākou mau ʻōlelo hope loa
ʻO ka Bernard-Soulier Syndrome `(BSS)` he maʻi laha ʻole loa ia i hoʻoilina ʻia mai kou mau mākua mai. Inā loaʻa iā ʻoe kēia maʻi, loaʻa paha iā ʻoe kekahi mau pilikia me ke kāpili ʻana o ke koko. Eia naʻe, ʻaʻole ia he manaʻo ʻaʻole hiki iā ʻoe ke ola i kahi ola maʻamau. ʻO ka mea nui loa, ʻo ia ka pale ʻana i nā kūlana pilikia e hiki ai ke alakaʻi i ka ʻeha a e hahai mau i ka ʻōlelo aʻo a kāu kauka.
E ʻike pinepine i kāu kauka ʻohana. Hiki iā ia ke pane i kāu mau nīnau, kōkua iā ʻoe e hoʻokele i kēia kūlana, a hana pū me ʻoe e mālama i kou olakino holoʻokoʻa. Mai makaʻu, ʻo ka ʻike a me ka mālama ko mākou mau ikaika nui loa i ka wā e noho pū ai me kēia mau kūlana.
ʻO ka Bernard-Soulier Syndrome, BSS, ke koko ʻana, nā platelets, ke kahe nui ʻana o ke koko, nā maʻi genetic, ka palapū
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