ʻO ka mea maʻamau loa e hana ʻia ana i loko o ko kākou kino, ʻo ia ka meaʻai a kākou e ʻai ai e hāʻawi mai iā kākou i ka ikehu. Ua like ia me ke kau ʻana i ke kinoea i loko o ke kaʻa. Maanei, loaʻa i ko kākou kino ka ikehu mai ke kōpaʻa (glucose). Hoʻomalu ʻia kēia kaʻina holoʻokoʻa e kahi hormone i kapa ʻia ʻo
insulin . ʻO kēia insulin ka mea e haʻi aku i ke kōpaʻa i loko o ke koko e hoʻouna ʻia i loko o nā pūnaewele e pono ai ka ikehu. Ua like ia me ka wehe ʻana i nā puka o nā pūnaewele a me ka hoʻokuʻu ʻana i ke kōpaʻa i loko. Eia naʻe, i loko o ke kino o ke kanaka me ka Rabson-Mendenhall Syndrome, ʻaʻole hana pono kēia kaʻina hana. ʻO ia hoʻi, ʻaʻole hiki i ko lākou kino ke hoʻohana pono i ka insulin. He maʻi laha loa kēia.
No laila he aha maoli kēia syndrome?
Ke hiki ʻole i ka insulin ke hana pono i kāna hana, pili pololei ia i ka ulu ʻana o ke keiki. E noʻonoʻo, hoʻomaka ka hopena o kēia ma mua o ka hānau ʻana o ke keiki, ʻo ia hoʻi, ʻoiai ʻo ia i loko o ka ʻōpū o ka makuahine. ʻO nā pēpē me kēia maʻi he liʻiliʻi ka nui. ʻOiai ma hope o ka hānau ʻana, lohi loa
ka piʻi ʻana o ko lākou kaumaha a me ka ulu ʻana o ke kino . Ma ke ʻano lapaʻau, ʻo ka maʻi Rabson-Mendenhall kahi maʻi e pili ana i kahi hui nui i kapa ʻia ʻo nā syndromes kūʻē nui o ka insulin. ʻO ka maʻi Donohue a me ka maʻi kūʻē insulin type A ʻelua mau maʻi ʻē aʻe e pili ana i kēia hui.
He aha ke kumu o kēia kūlana?
I ka ʻōlelo maʻalahi, he ʻano hoʻoilina kēia. ʻO ia hoʻi,
ua hoʻoilina ʻia mai nā mākua a i ke keiki . Hana ʻia kēia e kahi hewa i loko o ka gene i kapa ʻia ʻo `INSR` i loko o ko kākou kino. I kēia manawa, e nānā kākou pehea e hana ai kēia. I loko o kēlā me kēia cell o ko kākou kino, aia ʻelua kope o kēlā me kēia gene. Hoʻokahi mai ka makuahine, a hoʻokahi mai ka makuakāne. I mea e loaʻa ai i ke keiki ka maʻi Rabson-Mendenhall, pono i nā kope ʻelua o ka gene `INSR` i loaʻa i ke keiki ke kīnā i ʻōlelo ʻia. Inā hoʻokahi wale nō kope i loaʻa ka hemahema, ʻaʻole e ulu ka maʻi. I nā huaʻōlelo ʻē aʻe, no ke keiki e loaʻa ai kēia maʻi, pono i ka makuahine a me ka makuakāne ke kope hoʻokahi o kēia gene hemahema a me hoʻokahi kope o ka gene olakino. A laila, pono i ke keiki ke loaʻa nā kope hemahema ʻelua mai iā lāua ʻelua ma ka manawa kūpono. ʻO ia ke kumu he mea kākaʻikahi loa ia no ka mea ʻaʻole ia e hana pinepine ʻekolu manawa mai ka ʻehā manawa.
He aha nā hōʻailona o kēia maʻi?
Hoʻomaka pinepine nā hōʻailona e ʻike ʻia
i loko o ka makahiki mua o ke ola o kahi keiki. Eia kekahi, hiki ke ʻokoʻa ke koʻikoʻi o kēia mau hōʻailona mai kēlā kanaka a kēia kanaka. E nānā kākou i nā hōʻailona nui e ʻike ʻia.
| ʻĀpana/ʻōnaehana kino | Nā hiʻohiʻona i ʻike ʻia |
|---|
| Poʻo a me ka maka | ʻIli ʻāʻī, maka ākea, nā ʻauwai hohonu ma ke alelo, nā pepeiao, nā lehelehe, a me ka ʻauwae i ʻoi aku ka nui ma mua o ka maʻamau. |
| Nā kui | Mānoanoa ma mua o ka maʻamau. |
| ʻIli | ʻIli maloʻo. Ka ʻeleʻele a me ka mānoanoa o kekahi mau wahi o ka ʻili (ʻoi aku hoʻi ma nā pelu e like me nā ʻāʻī a me ka ʻāʻī). Ua kapa ʻia kēia ma ke ʻano lapaʻau he acanthosis nigricans . Hiki i kēia mau wahi ke ʻano velvet ke pā. |
| Nā niho | ʻOi aku ka nui ma mua o ka maʻamau, paʻapū i kekahi i kekahi, a i ʻole ka ulu ʻana o nā niho i ka wā ʻōpiopio. |
| Nā kino kūloko | ʻOi aku ka nui o nā puʻupaʻa, ka puʻuwai, a me nā ʻōpū moekolohe (pele i nā keikikāne, vagina i nā kaikamahine) ma mua o ka maʻamau. |
| Nā hiʻohiʻona maʻamau ʻē aʻe | Ka ulu nui ʻana o ka lauoho o ke kino, ka lohi o ka ulu ʻana ma mua a ma hope o ka hānau ʻana, ka pehu ʻana o ka ʻōpū, ka momona liʻiliʻi ma lalo o ka ʻili, a me ka nāwaliwali o nā ʻiʻo. |
Nā maʻi ʻē aʻe e hiki ke kū mai ma muli o kēia
Ma waho aʻe o kēia mau hōʻailona maʻamau, hiki i kēia syndrome ke hana i nā kūlana koʻikoʻi ʻē aʻe.
Pehea e hana ʻia ai ka hōʻailona?
ʻO kekahi o nā pilikia i ka ʻike ʻana i kēia maʻi, ʻo ia ka hoʻokaʻawale ʻana iā ia mai nā maʻi ʻē aʻe e like like ana (e like me ka maʻi Donahue). E hana
ke kauka o kāu keiki i kahi hoʻokolokolo kino piha, e nīnau e pili ana i nā hōʻailona o kāu keiki a me ka mōʻaukala olakino ʻohana, a e kauoha paha i kekahi mau hoʻāʻo koko e nānā i
ke kō koko a me nā pae insulin o kāu keiki. ʻO kēia wale nō ke ala e hana ai i kahi hōʻailona pololei.
Pehea e mālama ʻia ai?
ʻO ka mālama ʻana no ka maʻi Rabson-Mendenhall e pono ai ke kōkua o kahi hui nui, me nā loea like ʻole, nā kauka ʻokiʻoki, a me nā kauka niho. Hiki i ke aʻo ʻana ke lilo i mea nui loa no nā ʻohana e hoʻokele i ke kaumaha a me nā manaʻo e hele mai me ka loaʻa ʻana o kahi keiki me kēia maʻi laha ʻole.
ʻAʻohe lāʻau lapaʻau mau loa no kēia maʻi i kēia manawa. ʻO ke kumu o ka lāʻau lapaʻau ʻo ia ke kāohi a me ka mālama ʻana i nā hōʻailona.
Hoʻopili pinepine ʻia ka lāʻau lapaʻau i kēlā me kēia hōʻailona. No ka laʻana, ke ʻoki ʻana e wehe i nā cysts ovarian, ka mālama niho no nā pilikia niho, a pēlā aku. I kekahi mau hihia, kuhikuhi nā kauka i nā dosis kiʻekiʻe o ka insulin a i ʻole nā lāʻau lapaʻau ʻē aʻe e hoʻoulu ai i ka hoʻohana ʻana o ka insulin, akā ʻaʻole pinepine kēia mau mea i ka wā lōʻihi.
Nā lāʻau lapaʻau hou e noiʻi ʻia nei
Ke hoʻomau nei nā loea i ka noiʻi ʻana i nā koho lapaʻau maikaʻi aʻe no ke kiʻekiʻe o ke kō koko (hyperglycemia) e pili ana me ke kūʻē ʻana o ka insulin. ʻOiai ua hōʻike kēia mau lāʻau i kekahi mau hopena hoʻohiki, pono ka noiʻi hou aku.
- Nā Biguanides: He ʻano lāʻau lapaʻau kēia e hōʻemi ana i ka hana ʻana o ke kō e ke kino a hoʻonui i ka hoʻohana ʻana o ka insulin .
- Leptin: Ua ʻike ʻia kēia protein e kōkua i ka kaohi ʻana i ke kō koko a me nā pae insulin.
- rhIGF-I (Recombinant insulin-like growth factor I): Hoʻohana ʻia kēia protein e mālama i ka ketoacidosis, kahi kūlana i hoʻokumu ʻia e ke kūʻē koʻikoʻi o ka insulin .
Leka Lawe-Home
- ʻO ka Rabson-Mendenhall Syndrome kahi maʻi laha ʻole loa kahi i hiki ʻole ai i ke kino ke hoʻohana pono i ka insulin .
- ʻO ke kumu o kēia, he gene kīnā (ka gene `INSR`) i hoʻoilina ʻia e ke keiki mai nā mākua ʻelua.
- Hoʻomaka nā hōʻailona i ka wā kamaliʻi a hoʻopilikia i ka ulu ʻana o ke kino , ke ʻano o ka helehelena , ka ʻili, nā niho, a me nā ʻōpū o loko.
- ʻAʻohe lāʻau lapaʻau mau loa no kēia, a ke kuhikuhi ʻia nei ka mālama ʻana i nā hōʻailona. Pono kēia i ke kākoʻo o kahi hui o nā kauka loea.
- Inā hōʻike kāu keiki i kekahi o kēia mau hōʻailona, he mea nui loa ia e ʻike koke i ke kauka kūpono (kauka) no ka ʻōlelo aʻo.
ʻO ka maʻi Rabson-Mendenhall, ke kū'ē ʻana o ka insulin, nā maʻi genetic, nā maʻi pediatric, acanthosis nigricans, ketoacidosis, ka maʻi diabetes
💬 Comments (0)
ʻAʻohe manaʻo i kau ʻia. Hoʻohui i kāu manaʻo ma ʻaneʻi no ka manawa mua.
Hoʻohui i kāu manaʻo