ʻO ka maʻi maʻi ʻo Brugada kahi maʻi kakaʻikahi i hōʻike ʻia e ke komo ʻana o ka naʻau. ʻO ka hopena maʻamau ka piʻi ʻana o ka puʻuwai puʻuwai (arrhythmia). ʻO kēia piʻi ʻana o ka puʻuwai puʻuwai hiki ke hopena i ke alo o ka palpitations, maule a make paha. (2)

ʻAʻole loaʻa nā hōʻailona o kekahi mau maʻi. Eia nō naʻe, ʻoiai kēia ʻoiaʻiʻo a me ka maʻamau o ka ʻiʻo o ka puʻuwai, hiki ke hoʻololi koke i ka hana uila o ka puʻuwai.

He pathology ia e hiki ke hoʻoili ʻia mai kēlā hanauna a i kēia hanauna.

ʻAʻole ʻike ʻia ka prevalence pololei (ka nui o nā hihia o ka maʻi i ka manawa i hāʻawi ʻia, i ka heluna kanaka). Eia naʻe, ʻo kāna kuhi he 5 / 10. ʻO ia ka mea he maʻi kakaʻikahi hiki ke make i nā maʻi. (000)

Hoʻopilikia ka maʻi Brugada i nā poʻe ʻōpio a i ʻole waena o nā makahiki. ʻIke ʻia ka mana nui o ke kāne i kēia pathology, me ka ʻole o ka maʻemaʻe o ke ola. ʻOiai ka nui o nā kāne, hiki i nā wahine ke hoʻopilikia ʻia e Brugada syndrome. ʻO kēia helu nui o nā kāne i loaʻa i ka maʻi i wehewehe ʻia e nā ʻōnaehana hormonal kāne / wahine like ʻole. ʻOiaʻiʻo, ʻo ka testosterone, he hormone kāne wale nō, he kuleana koʻikoʻi i ka hoʻomohala ʻana i nā pathological.

ʻO kēia kane / wahine predominance i hypothetically i wehewehe ʻia e ka ratio 80/20 no nā kāne. I loko o ka heluna kanaka he 10 mau maʻi me Brugada Syndrome, 8 mau kāne a he 2 mau wahine.

Ua hōʻike ʻia nā haʻawina Epidemiological ua loaʻa kēia maʻi me ka pinepine o nā kāne ma Iapana a me Asia Hema. (2)

Ma Brugada syndrome, ʻike pinepine ʻia nā hōʻailona mua ma mua o ka hoʻomaka ʻana o ka puʻuwai kiʻekiʻe kiʻekiʻe. Pono e ʻike ʻia kēia mau hōʻailona mua i ka hikiwawe i mea e pale aku ai i nā pilikia, a me ka hopu ʻana i ka naʻau.

ʻO kēia mau hōʻailona hōʻailona mua:

• nā mea ʻino uila o ka puʻuwai;
• palpitations;
• nā kauā.

ʻO ka ʻoiaʻiʻo he kumu hoʻoilina o kēia maʻi a me ka loaʻa ʻana o nā hihia o kēia maʻi maʻi i loko o kahi ʻohana hiki ke hāpai i ka nīnau no ka hiki ʻana o ka maʻi i ke kumuhana.

Hiki i nā hōʻailona ʻē aʻe ke kāhea i ka ulu ʻana o ka maʻi. ʻOiaʻiʻo, kokoke i ka 1 i loko o 5 mau maʻi e loaʻa ana i ka maʻi Brugada maʻi i loaʻa i ka atrial fibrillation (ke ʻano o kahi hana desynchronized o ka ʻiʻo puʻuwai) a i ʻole ke hōʻike nei i kahi kiʻekiʻe kiʻekiʻe o ka puʻuwai puʻuwai.

ʻO ka loaʻa ʻana o ke kuni i nā mea maʻi e hoʻonui i ko lākou pilikia o ka hoʻonui ʻana i nā hōʻailona pili me Brugada syndrome.

I kekahi mau hihia, hiki ke hoʻomau ka puʻuwai ʻino a alakaʻi i ka ventricular fibrillation. ʻO ka hanana hope e pili ana i nā pūʻulu o ka wikiwiki a me ka hui pū ʻole ʻana o ka puʻuwai. ʻO ka mea maʻamau, ʻaʻole e hoʻi ka puʻuwai i ka maʻamau. Hoʻopilikia pinepine ʻia ke kahua uila o ka ʻiʻo puʻuwai e hoʻopau i ka hana o ka puʻuwai puʻuwai.

ʻO ka maʻi maʻi Brugada e alakaʻi pinepine i ka hopu puʻuwai koke a no laila i ka make ʻana o ke kumuhana. ʻO nā kumuhana i hoʻopilikia ʻia, ma ka hapanui o nā hihia, nā ʻōpio me ke ʻano ola olakino. Pono e hoʻokō koke ka hōʻailona i mea e hoʻokumu ai i ka mālama wikiwiki a no laila e pale aku i ka make. Eia naʻe, paʻakikī pinepine kēia hōʻailona e hoʻokumu mai ka manaʻo kahi iʻikeʻoleʻia ai nā hōʻailona. ʻO kēia ka wehewehe ʻana i ka make koke ʻana o kekahi mau keiki me ka maʻi Brugada ʻaʻole hōʻike i nā hōʻailona weliweli. (2)

He mea maʻamau ka hana ʻiʻo o ka puʻuwai o nā maʻi me Brugada syndrome. Aia nā anomalies i ka hana uila o ia mea.

Ma ka ʻili o ka puʻuwai, aia nā pores liʻiliʻi (nā ala ion). Loaʻa iā lākou ka hiki ke wehe a pani i ka manawa maʻamau e ʻae i ka calcium, ka sodium a me nā ion potassium e hele i loko o nā keʻena o ka puʻuwai. Aia kēia mau neʻe ionic i ke kumu o ka hana uila o ka puʻuwai. Hiki i ka hōʻailona uila ke hoʻolaha mai ka piko o ka ʻiʻo o ka puʻuwai i lalo a no laila e ʻae ai ka puʻuwai e ʻaelike a hana i kāna kuleana o ke "pump" koko.

ʻO ke kumu o ka maʻi Brugada he genetic. Hiki i nā hoʻololi genetic like ʻole ke kumu o ka ulu ʻana o ka maʻi.

ʻO ka gene i hoʻopili pinepine ʻia i ka pathology ʻo ka gene SCN5A. Hoʻokomo ʻia kēia gene i ka hoʻokuʻu ʻana i ka ʻike e ʻae ai i ka wehe ʻana o nā kahawai sodium. ʻO ka hoʻololi ʻana i loko o kēia moʻo hoihoi ke kumu i ka hoʻololi ʻana i ka hana ʻana o ka protein e hiki ai i ka wehe ʻana o kēia mau ala ion. I kēia manaʻo, ua hoʻemi nui ʻia ke kahe o nā ion sodium, e hoʻopilikia i ka paʻi ʻana o ka puʻuwai.

ʻO ka loaʻa ʻana o hoʻokahi wale nō o nā kope ʻelua o ka gene SCN5A e hiki ai ke hoʻopilikia i ka kahe ionic. A i ʻole, ma ka hapanui o nā hihia, loaʻa i ka mea maʻi kekahi o kēia mau mākua ʻelua i loaʻa ka genetic mutation no kēlā gene.

Eia kekahi, hiki i nā genes ʻē aʻe a me nā kumu o waho ke kumu o kahi imbalance i ke kiʻekiʻe o ka hana uila o ka puʻuwai puʻuwai. Ma waena o kēia mau mea, ʻike mākou: kekahi mau lāʻau lapaʻau a i ʻole ke kaulike ʻole o ka sodium i loko o ke kino. (2)

Lawe ʻia ka maʻi by he hoʻoili mana autosomal. A i ʻole, ʻo ka loaʻa ʻana o hoʻokahi wale nō o nā kope ʻelua o ka gene of interest ua lawa ia no ke kanaka e hoʻomohala i ka phenotype pili i ka maʻi. ʻO ka mea maʻamau, loaʻa i ka mea maʻi kekahi o kēia mau mākua ʻelua i loaʻa ke ʻano mutated. Eia nō naʻe, i nā hihia liʻiliʻi, hiki ke ʻike ʻia nā hoʻololi hou i kēia gene. ʻO kēia mau hihia hope e pili ana i nā kumuhana i loaʻa ʻole ka maʻi i loko o ko lākou ʻohana. (3)

ʻO nā kumu pilikia e pili ana i ka maʻi he genetic.

ʻO ka ʻoiaʻiʻo, ʻo ka lawe ʻana i ka maʻi Brugada he autosomal dominant. A i ʻole, ʻo ka loaʻa ʻana o hoʻokahi wale nō o nā kope ʻelua o ka gene mutated e pono ai ke kumuhana e hōʻike i ka maʻi. Ma kēia manaʻo, inā hōʻike kekahi o nā mākua ʻelua i ka hoʻololi ʻana i ka gene o ka hoihoi, hiki ke hoʻoili ʻia i ka maʻi.

Hoʻokumuʻia ka hōʻailona o ka maʻi ma kahi hōʻailonaʻokoʻa mua. ʻOiaʻiʻo, ke hahai nei ia i kahi hoʻokolohua lapaʻau a ke kauka lapaʻau, e ʻike ana i nā hōʻailona ʻano o ka maʻi ma ke kumuhana, hiki ke hoʻāla ʻia ka ulu ʻana o ka maʻi.

Ma hope o kēia, hiki ke ʻōlelo ʻia ke kipa ʻana i kahi cardiologist e hōʻoia a ʻaʻole paha i ka ʻike ʻokoʻa.

ʻO ka electrocardiogram (ECG) ka maʻamau gula i ka ʻike ʻana i kēia maʻi. ʻO kēia hoʻokolohua e ana i ka puʻuwai puʻuwai a me ka hana uila o ka puʻuwai.

Inā kānalua ʻia ka maʻi Brugada, ʻo ka hoʻohana ʻana i nā lāʻau lapaʻau e like me: ajmaline a i ʻole flecainide hiki ke hōʻike i kahi kiʻekiʻe o ka māhele ST i nā maʻi i manaʻo ʻia he maʻi.

Pono paha kahi echocardiogram a / a i ʻole Magnetic Resonance Imaging (MRI) e nānā i ka hiki ʻana mai o nā pilikia puʻuwai ʻē aʻe. Eia kekahi, hiki i ka ho'āʻo koko ke ana i nā pae o ka pālolo a me ka calcium i loko o ke koko.

Hiki i nā ho'āʻo genetic ke ʻike i ka hiki ʻana mai o kahi mea ʻino i ka moʻo SCN5A i komo i ka maʻi Brugada.

Hoʻokumu ʻia ka lāʻau maʻamau no kēia ʻano o ka pathology ma ke kau ʻana o kahi defibrillator cardiac. ʻO ka hope e like me ka pacemaker. Hiki i kēia mea hana ke hoʻopuka i nā haʻalulu uila e hiki ai i ka mea maʻi ke loaʻa hou ka puʻuwai maʻamau.

I kēia manawa, ʻaʻohe lāʻau lapaʻau no ka mālama ʻana i ka maʻi. Eia kekahi, hiki ke lawe ʻia kekahi mau mea e pale aku ai i nā pilikia rhythmic. ʻO kēia ka hihia me ka hoʻokuke ʻana ma muli o ka maʻi maʻi (e hoʻopilikia i ke koena sodium i loko o ke kino) a i ʻole ke kuni, ma ka lawe ʻana i nā lāʻau lapaʻau kūpono. (2)