Loaʻa i kāu keiki liʻiliʻi ke kuni, nā ʻeha waha, a i ʻole ka ʻāʻī ʻeha i nā lā like i kēlā me kēia mahina? Nalo aku kekahi maʻi, a hoʻi hou mai i ʻelua a ʻekolu paha pule ma hope? Ke noʻonoʻo nei paha ʻoe, "Ua nāwaliwali ka ʻōnaehana pale o kaʻu keiki." ʻAe, he ʻoiaʻiʻo paha kēlā. Akā ʻo ke kumu ma hope o kēia maʻi cyclical he maʻi kakaikahi loa ia. I kēia lā, ke kamaʻilio nei mākou e pili ana i kekahi o ia ʻano maʻi, ʻo ia hoʻi ka cyclic neutropenia.
I ka ʻōlelo maʻalahi, he aha ka neutropenia cyclic?
I mea e hoʻomaopopo ai i kēia, pono mua kākou e ʻike e pili ana i kahi koa i loko o ko kākou koko. ʻO ka inoa o kēlā koa ʻo ka neutrophil . ʻO kēia neutrophil kahi ʻano o ke koko keʻokeʻo e pale iā kākou mai nā maʻi ma ke kaua ʻana i nā germs, ʻoiai nā bacteria, e komo i loko o ko kākou kino. Ua like ia me kahi pūʻali koa i loko o ko kākou kino.
ʻO ka Neutropenia ka wā e hāʻule ai ka nui o nā koa neutrophil i loko o ke koko ma lalo o nā pae maʻamau. E like me ka nāwaliwali o kahi pūʻali koa ke emi iho kāna mau koa, ke emi iho nā neutrophils, e nāwaliwali ka ʻōnaehana pale o ko mākou kino a hoʻomaka mākou e loaʻa i nā maʻi me ka maʻalahi.
I kēia manawa, e nānā kākou i ke ʻano o ka huaʻōlelo cyclic . ʻO ke ʻano o Cyclic he "cyclical." ʻO ke ʻano kēia, ʻaʻole haʻahaʻa mau ka pae neutrophil. Piʻi a iho ia i kahi papa hana maʻamau, i loko o kahi pōʻaiapuni. I ka pololei, ʻo ka maʻamau i kēlā me kēia ʻekolu pule (ma kahi o 21 mau lā) , hāʻule nui ka pae neutrophil no ʻekolu a ʻelima mau lā. A laila hoʻi ia i ka maʻamau, a laila hāʻule hou no nā pule ʻekolu e hiki mai ana. Hana ʻia kēia pōʻaiapuni ma ke ʻano hiki ke wānana ʻia.
I ka ʻōlelo maʻalahi, ʻo ka neutropenia cyclic kahi kūlana kahi e emi ai ka helu o nā cell neutrophil, ka mea e pale ai i ko mākou kino mai ka maʻi, e like me kahi pōʻaiapuni i hoʻonohonoho ʻia i kēlā me kēia lā, e hoʻonui ana i ka pilikia o ka maʻi.
He mea laha ʻole kēia maʻi. Wahi a nā kānaka noiʻi hoʻokahi wale nō i loko o hoʻokahi miliona mau kānaka ma ka honua i loaʻa i kēia maʻi.
He aha nā hōʻailona o kēia maʻi?
ʻO ke ʻano kūikawā o kēia maʻi, ʻo ia ka ʻike ʻia ʻana o nā hōʻailona e like me ke kaʻina o ka emi ʻana o nā pae neutrophil. ʻO ia hoʻi, ʻike ʻia kēia mau hōʻailona i loko o ʻekolu a ʻehā mau lā o ka emi ʻana o nā neutrophils, a i ka wā e hoʻi ai nā pae neutrophil i ka maʻamau, nalo nā hōʻailona.
| Hōʻailona | Wehewehena |
|---|---|
| Kuni a me ka luhi | Ke haʻahaʻa nā neutrophils, ʻo ka hōʻailona mua o kahi maʻi i loko o ke kino, ʻo ia paha ke kuni. Me kēia, hiki i ke kino ke manaʻo ua make. |
| ʻEha waha a me ka ʻāʻī ʻeha | He hōʻailona maʻamau kēia. Hiki iā ia ke hana i nā ʻeha ʻeha i loko o ka waha a ma nā ʻāʻī. Hiki iā ia ke hana i ka ʻāʻī ʻeha. |
| Maʻi periodontal | Hiki ke kahe pinepine ke koko a me ka pehu ʻana o nā ʻāʻī. |
| Nā maʻi hoʻi hou | Hiki ke loaʻa pinepine nā maʻi ʻōpū hanu (anu, ʻū), nā maʻi ʻōpū hoʻoheheʻe (ʻeha ʻōpū), a me nā maʻi ʻili (cellulitis). |
Hiki ke loli ke ʻano o kēia mau hōʻailona i ke ola holoʻokoʻa. No nā kānaka he nui, emi iho ka koʻikoʻi o nā hōʻailona i ko lākou hiki ʻana i ka wā makua . He mea maʻamau ka ʻeha waha a me nā pilikia niho i nā poʻe ʻōpio, ʻoiai ʻoi aku ka maʻamau o ke poʻo a me nā maʻi sinus (sinusitis) i nā poʻe ʻelemakule.
No ke aha lā kēia e hana nei?
Hoʻokumu ʻia ka neutropenia cyclic e kahi mutation genetic . ʻO ke kumu nui ka loli o ka gene ELANE i loko o ko mākou kino.
E noʻonoʻo i kahi gene ma ke ʻano he hoʻonohonoho o nā kuhikuhi e haʻi i nā cell i loko o ko mākou kino pehea e hana ai. Aia i loko o ka gene ELANE nā kuhikuhi no ka hana ʻana i kahi enzyme e kōkua i nā neutrophils e hana pono. Ke loaʻa kahi kīnā a i ʻole ka mutation i loko o kēia gene, ʻaʻole hiki i nā neutrophils ke hana pono.
Hiki ke hoʻoili ʻia kēia maʻi mai nā mākua a i nā keiki, a i ʻole hiki ke ulu i ka wā o ka fetal.
Ma muli o kēia hemahema genetic,
- ʻAʻole paha e hana ke kino i nā neutrophils lawa.
- A i ʻole, e make koke nā neutrophils.
- A i ʻole ʻaʻole paha e hana pono nā neutrophils.
E ke kauka, pehea ʻoe e ʻike ai i kēia?
ʻIke pinepine ʻia kēia mau hōʻailona mai ka wā hānau mai, no laila ua ʻike pinepine ʻia ka maʻi i ka wā kamaliʻi. E noʻonoʻo ke kauka i kekahi mau mea e hōʻoia i kēia maʻi.
- Moʻolelo ʻohana o ka maʻi: ʻOiai he maʻi hoʻoilina kēia, nānā ʻia e ʻike inā loaʻa i kekahi o ka ʻohana (nā mākua, nā kaikaina) kēia maʻi.
- Nā hoʻāʻo koko: ʻO kēia ka mea nui loa. E nānā kāu kauka i kou pae neutrophil i loko o kou koko ʻelua a ʻekolu paha manawa i ka pule no kahi o ʻeono pule . E hana ʻia kēia e ʻike inā e emi ana kāu mau pae neutrophil i ke ʻano cyclically.
- ʻAno o nā hōʻailona: E nānā pono ke kauka i ke alapine (ma kahi o kēlā me kēia 3 pule). Kōkua kēia ʻano cyclical e hoʻokaʻawale i kēia mai nā ʻano neutropenia ʻē aʻe.
- Ka hoʻāʻo ʻana i ka genetic: I kekahi manawa, hiki ke ʻōlelo ʻia ka hoʻāʻo genetic e hōʻoia inā he mutation i loko o ka gene ELANE.
I ka wā e ʻike ai i kēia kūlana, pono ke kauka e kāpae i nā ʻano neutropenia ʻē aʻe i loaʻa nā hōʻailona like, e like me 'Severe congenital neutropenia' (i ʻike mua ʻia ʻo 'Kostmann syndrome'), 'Autoimmune neutropenia', a me 'Idiopathic neutropenia'.
He aha nā lāʻau lapaʻau no kēia?
ʻElua mau pahuhopu nui o ka mālama ʻana. ʻO kekahi, ʻo ia ka hoʻōla ʻana i nā maʻi e kū nei. ʻO kekahi, ʻo ia ka hoʻonui ʻana i nā pae neutrophil a pale i nā maʻi e hiki mai ana.
| ʻAno lapaʻau | Hana |
|---|---|
| Nā lāʻau lapaʻau | Hāʻawi ʻia kēia mau mea e hakakā i nā maʻi bacteria e kū mai ana i nā wā o nā neutrophils haʻahaʻa. |
| Ka mālama ʻana iā G-CSF | ʻO kēia ka lāʻau lapaʻau nui a maikaʻi loa. ʻO G-CSF (Granulocyte colony-stimulating factor) kahi lāʻau lapaʻau e hoʻoulu ai i ko mākou iwi iwi e hana i nā neutrophils hou aʻe (e.g. filgrastim (Neupogen®)). Hāʻawi ʻia ia ma ke ʻano he injection a he lāʻau lapaʻau palekana hiki ke hoʻohana ʻia no ka wā lōʻihi. |
| Hoʻoili pūnaewele kumu | He ʻano hana kēia i hoʻohana wale ʻia no ka poʻe me ka maʻi koʻikoʻi loa a ʻaʻole i pane i ka lāʻau G-CSF. Ma kēia ʻano hana, ua hoʻoili ʻia nā pūnaewele kumu olakino e pani i nā pūnaewele maʻi. |
Aia kekahi mau hopena ʻaoʻao o ka lāʻau lapaʻau?
Hiki i ka mālama ʻana iā G-CSF ke hana i nā hopena ʻaoʻao liʻiliʻi. Eia nō naʻe, hoʻomaka pinepine nā kauka i ka mālama ʻana no kēia maʻi me kahi mahele haʻahaʻa, no laila ua liʻiliʻi nā hopena ʻaoʻao.
- ʻEha poʻo
- ʻEha i nā iwi a me nā hono
- Ka luaʻi a me ka maʻi pākī
Hiki ke hana ʻia nā mea e like me kēia. E kamaʻilio pono me kāu kauka e pili ana i ka lāʻau āu e loaʻa nei a me kona mau hopena ʻaoʻao.
I ka manawa hea e pono ai iaʻu ke kāhea aku i ke kauka?
Inā loaʻa iā ʻoe a i ʻole kāu keiki ka neutropenia cyclic, he mea nui loa e ʻimi i ke aʻo lapaʻau i ka wā e ʻike ai ʻoe i kekahi mau hōʻailona o ka maʻi .
E hoʻolohe pono i nā hiʻohiʻona ma lalo nei:
* Kuni
* ʻEha ma kekahi wahi o ke kino
* Ka pehu ʻana a i ʻole ka ʻulaʻula o ka ʻili
Inā ʻike ʻoe i kekahi o kēia mau hōʻailona, e ʻike koke i kāu kauka. Mai kānalua e hele i ka Emergency Department (ETU) inā pono.
He aha kā kākou e hana ai e pale aku iā kākou iho mai nā maʻi?
Ma waho aʻe o ka pale ʻana iā ʻoe iho mai nā maʻi, pono ʻoe e makaʻala i ka wā e haʻahaʻa ai kāu helu neutrophil.
- E holoi pono i kou mau lima, ʻoiai ma mua o ka ʻai ʻana a ma hope o ka hoʻohana ʻana i ka lua.
- E mālama i ke olakino waha maikaʻi. E palaki i kou mau niho i kēlā me kēia lā a e ʻike mau i ke kauka niho.
- E kiʻi i nā lāʻau pale āpau i ʻōlelo ʻia i ka manawa kūpono.
- Mai hoʻopā i kou maka, maka, a me kou ihu me ka holoi ʻole i kou mau lima.
- E lawe i kāu mau lāʻau lapaʻau e like me ke kauoha a kāu kauka a e hana i kāu mau hoʻāʻo koko i ka manawa kūpono.
ʻOiai ke ola holoʻokoʻa kēia ʻano, ua ʻae nā lāʻau lapaʻau holomua e like me G-CSF i nā poʻe he nui me ka cyclic neutropenia e ola i nā ola maʻamau . ʻO ka mea nui, ʻoiai kekahi mau ʻano neutropenia ʻē aʻe e lawe i ka pilikia o ka loaʻa ʻana o ka maʻi kanesa, e like me ka leukemia, ʻaʻole ka cyclic neutropenia.
Leka Lawe-Home
- ʻO ka neutropenia cyclic kahi maʻi genetic laha ʻole kahi e emi ai ka helu o nā cell neutrophil e hakakā nei i ka maʻi.
- ʻIke pinepine ʻia nā hōʻailona (kuni, ʻeha waha) ma kahi ʻano o hoʻokahi manawa i kēlā me kēia 3 pule.
- No ka hōʻoia ʻana, pono e hana mau ʻia nā hoʻāʻo koko no kekahi mau pule e hōʻoia i ke kaʻapuni o nā pae neutrophil.
- Ma muli o ka loaʻa ʻana o nā lāʻau lapaʻau kūpono loa e like me G-CSF, hiki i nā mea maʻi ke ola maʻamau.
- He mea nui loa e ʻimi i ke kōkua lapaʻau i ka wā e ʻike ai ʻoe i kekahi mau hōʻailona o ka maʻi (kuni, ʻeha, pehu).











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