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He aha ka Hyperammonemia?
I ka ʻōlelo maʻalahi, ʻo ka hyperammonemia ka wā e kiʻekiʻe loa ai kou mau pae ammonia koko. ʻO Ammonia, a i ʻole NH3, kahi huahana ʻōpala i hana ʻia i loko o ko kākou ʻōpū i ka wā e ʻeli ai kākou i nā protein a kākou e ʻai ai. ʻO ka maʻamau, hele kēia ammonia i ko kākou akepaʻa a hana ʻia ma laila. Kāhea mākou i kēia kaʻina hana ʻo ke kaʻina hana urea . Ma o kēia kaʻina hana, hoʻololi ʻia ka ammonia i kahi mea ʻino ʻole i kapa ʻia ʻo urea a hoʻokuʻu ʻia i loko o ka mimi. Eia naʻe, inā hewa kekahi mea me kēia kaʻina hana urea, a inā ʻaʻole hiki i ke akepaʻa ke hana pono i kēia ammonia, hoʻomaka ka ammonia e hōʻiliʻili i loko o ke koko. ʻO kēia hoʻonui ʻana o ka ammonia i loko o ke koko he mea ʻawahia loa ia i kā mākou Central Nervous System (CNS) , ʻo ia ka lolo a me ke kaulahao spinal. ʻO ia ke kumu he ʻano maʻi ka hyperammonemia e pono ai ke kōkua koke ʻana i ke kauka a hiki ke hoʻoweliweli i ke ola .ʻO wai ka mea hiki ke hoʻomohala nui i kēia?
ʻO kēia kūlana, i kapa ʻia ʻo hyperammonemia, hiki ke hoʻopilikia i nā keiki hānau hou, nā keiki ʻōpio, a me nā pākeke.- Ma waena o nā pākeke, ma kahi o 90% o ka poʻe i loaʻa i kēia maʻi ʻo ia ka poʻe me ka cirrhosis o ke ake .
- Hiki i nā pēpē hānau hou ke loaʻa i kēia no ka mea ua hānau ʻia lākou me kahi kīnā i ka pōʻaiapuni urea (congenital urea cycle disorder) . He mau kūlana genetic kēia.
- Eia kekahi, ʻo ka hemahema nui o ke akepaʻa a me nā maʻi metabolic hoʻoilina hiki ke hana i ka hyperammonemia i nā keiki ʻōpio.
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Check Availability →He aha nā kumu o ka hyperammonemia?
Hiki ke nui nā kumu no kēia. Hiki ke loli kēia mau kumu me ka makahiki. Akā ma ke ʻano laulā, ʻelua o nā kumu nui, ʻo ia nā pilikia o ke akepaʻa a me nā maʻi pōʻaiapuni urea (UCD) .Ma muli o nā pilikia o ke akepaʻa
ʻO ko kākou akepaʻa ke kino nui e hoʻololi i ka ammonia i urea. No laila, inā ua hōʻino ʻia a maʻi paha ke akepaʻa, ʻaʻole hiki iā ia ke hana pono i ka ammonia. ʻO kēia ka manawa e hōʻiliʻili ai ka ammonia i loko o ke koko. Eia kekahi mau maʻi e pili ana i ke akepaʻa e hiki ai ke hoʻoulu i ka hyperammonemia:- Maʻi akepaʻa : Ke hōʻino ʻia ke akepaʻa , ua kaupalena ʻia kona hiki ke hana i ka ammonia. He mea maʻamau kēia i nā maʻi akepaʻa koʻikoʻi, akā i nā poʻe me ka maʻi akepaʻa paʻa, hiki i nā pae ammonia ke piʻi koke i kekahi manawa, no ka laʻana, inā hana kekahi mea i loko o ka ʻōpū a i ʻole nā naʻau ( ke kahe ʻana o ke koko ma ka ʻōpū) a i ʻole inā he kaulike ʻole o ka electrolyte . ʻO kēia ke kumu nui o nā pae ammonia kiʻekiʻe i nā pākeke a me nā keiki.
- ʻO ka encephalopathy hepatic: Hana ʻia kēia ke hōʻino ʻia ke akepaʻa a ʻaʻole hiki ke hana pono i ka ammonia. Hōʻiliʻili ka ammonia i loko o ke koko a hele i ka lolo. Hiki i kēia ke hana i ka huikau, ka disorientation , a me ka coma. I kekahi manawa hiki ke make.
- ʻO ka Cirrhosis o ke akepaʻa: ʻO ka Cirrhosis ka hoʻololi ʻana o nā ʻiʻo akepaʻa olakino me nā ʻiʻo ʻeha. Hiki i kēia ke hana pono ʻole i ke akepaʻa a hiki ke alakaʻi i ka hyperammonemia. He maʻi akepaʻa mau ka Cirrhosis.
- ʻO ka hāʻule ʻana o ke akepaʻa koʻikoʻi: ʻO kēia ka manawa e pau koke ai ka hana ʻana o ke akepaʻa. Hiki ke hana ʻia e nā ʻano maʻi like ʻole a me nā kūlana. No ka laʻana, ʻo ka hōʻeha ʻana o ke akepaʻa i hoʻoulu ʻia e ka lāʻau lapaʻau i hoʻokumu ʻia e kekahi mau lāʻau lapaʻau, e like me ka acetaminophen , he 50% o nā hihia āpau o ka hāʻule ʻana o ke akepaʻa koʻikoʻi ma ʻAmelika Hui Pū ʻIa. He 64% ia o nā hihia o ka hyperammonemia i nā keiki.
- Ka emi ʻana o ke kahe koko i kou akepaʻa: Inā ʻaʻole kahe pono ke koko i kou akepaʻa, ʻaʻole hiki i ke kino ke hoʻouna i ka ammonia i ke akepaʻa e hana ʻia. ʻOiai i kēlā manawa, hōʻiliʻili ka ammonia i loko o ke koko.
- ʻO ka maʻi Reye:He maʻi laha ʻole kēia. Hoʻopilikia ia i ke koko, ka lolo, a me ke akepaʻa. Hoʻonui pinepine ia i nā pae ammonia koko a me nā pae glucose koko haʻahaʻa. Hoʻopilikia pinepine ia i nā keiki ʻōpio a me nā ʻōpio e hoʻōla ana mai nā maʻi viral e like me ka chickenpox a i ʻole ka maʻi flu/influenza a ua lawe i ka aspirin no kēlā mau hōʻailona. ʻAʻole ʻike ʻia ke kumu pololei o ka maʻi Reye. Eia nō naʻe, ma muli o kēia pilikia, ʻaʻole pono e hāʻawi ʻia ka aspirin i nā keiki ʻōpio a me nā ʻōpio ke ʻole i ʻōlelo kūikawā ʻia e ke kauka.
Ma muli o nā maʻi pōʻaiapuni Urea (UCDs)
E like me kā mākou i kūkākūkā ai ma mua, ʻo ke kaʻina hana urea ke kaʻina hana e hoʻololi ʻia ai ka ammonia ʻawahia i urea a hoʻokuʻu ʻia i loko o ka mimi. He nui nā ʻanuʻu o kēia kaʻina hana, a ʻo kēlā me kēia mea e pono ai kahi ʻano enzyme like ʻole. ʻO kēia mau enzymes:- N-acetyl-glutamate synthase (NAGS)
- ʻO ka Carbamoyl phosphate synthetase (CPS)
- ʻO ka Ornithine transcarbamylase (OTC)
- ʻO Argininosuccinate synthetase (AS)
- ʻO ka lyase waikawa Argininosuccinic (ASL)
- ʻArginase (ARG1)
Nā kumu ʻē aʻe
Ma waho aʻe o kēia, aia paha kekahi mau kumu ʻē aʻe:- ʻO ka hāʻule ʻana o ka puʻupaʻa (renal): Inā ʻaʻole hiki i nā puʻupaʻa ke hoʻokuʻu pono i ka urea ma muli o ka hāʻule ʻana o ka puʻupaʻa, hiki iā ia ke hoʻoulu i ka ammonia e hōʻiliʻili i loko o ke koko.
- ʻO kekahi mau maʻi koko: Hiki ke hana ʻia ka Hyperammonemia e nā maʻi koko e like me ka myeloma maha a me ka leukemia koʻikoʻi.
- ʻO kekahi mau maʻi: nā meaola e hana ana i ka urease e like me Proteus mirabilis , Escherichia coli (E. coli) a me KlebsiellaʻO nā maʻi bacteria e like me ka streptococci, ʻoi aku hoʻi i nā keiki me nā kīnā hānau o ke ala mimi a me nā pākeke me ka paʻa ʻana o ka mimi ma muli o nā maʻi ala mimi (UTI), hiki ke hana i ka hyperammonemia koʻikoʻi.
He aha nā hōʻailona o ka Hyperammonemia?
Hiki ke ʻokoʻa loa nā hōʻailona ma muli o ke ʻano o ka maʻalahi a i ʻole ke koʻikoʻi o ke ʻano, a me ka makahiki e ulu ai.Nā hōʻailona maʻalahi i maʻa mau i nā keiki a me nā pākeke
- Ka luaʻi a me ka luaʻi
- ʻEha ʻōpū
- Hoʻonāukiuki pinepine
- ʻEha poʻo (ʻeha poʻo)
- Ka paʻakikī me ke kaulike, ka hoʻonohonoho ʻana, a me ka ʻōlelo (ataxia)
- Nā loli ʻano
Nā hōʻailona ʻoluʻolu ʻē aʻe e hoʻopilikia wale ana i nā keiki
- ʻAʻole e holomua
- Ka nāwaliwali o nā ʻiʻo (hypotonia)
- Nā lohi o ka hoʻomohala ʻana o ka lolo
Nā hōʻailona koʻikoʻi i nā keiki a me nā pākeke
He mau hōʻailona weliweli loa kēia. Inā loaʻa iā ʻoe kekahi o kēia mau mea, pono ʻoe e ʻimi koke i ke kōkua lapaʻau.- Ka huikau a me ka nalowale ʻana o ke kuhikuhi
- Nā loli o ka naʻau
- Hiamoe nui
- Nā loli i ka ʻike
- Nā hopu ʻana
- Ka hanu wikiwiki ʻana (Hyperventilation)
- Koma
ʻŌlelo Aʻo: Inā loaʻa iā ʻoe a i ʻole kekahi mea kokoke iā ʻoe kēia mau hōʻailona, e ʻoluʻolu e kelepona koke i ka 911, a i ʻole e hele i ka ʻoihana pilikia o ka haukapila kokoke loa iā ʻoe. ʻO nā pae ammonia koko kiʻekiʻe he mea hoʻoweliweli i ke ola a pono e mālama koke ʻia e ke kauka!
Nā hōʻailona i nā pēpē hānau hou
Inā loaʻa i kahi pēpē hānau hou ka hyperammonemia, ʻike pinepine ʻia nā hōʻailona ma hope o 24 a 72 mau hola o ka hānau ʻana:- Hoʻonāukiuki pinepine, uē pinepine
- Ka luaʻi ʻana
- Luhi
- Nā hopu ʻana
- ʻUwū ʻana i ka wā e hanu ana
- Ka hanu wikiwiki ʻana (Hyperventilation)
He mea nui: Inā hōʻike kāu pēpē i kēia mau hōʻailona, e kāhea koke aku i ke kauka inā ʻoe ma ka haukapila. Inā ʻoe ma ka home, e kāhea koke aku i ka 911, a i ʻole e lawe iā lākou i ka haukapila kokoke loa. ʻAʻole kēia he mea e hoʻopaneʻe ai.
Pehea e ʻike ai nā kauka i kēia?
Hoʻomaopopo pinepine nā kauka i ka hyperammonemia ma ka hoʻāʻo ʻana i nā pae ammonia i loko o ke koko.- No nā pēpē a hiki i hoʻokahi mahina, inā ʻoi aku ka nui o ka ammonia i loko o ke koko ma mua o 80 micromol/lita (80 micromol/L).
- No nā keiki makua, inā ʻoi aku ia ma mua o 55 micromol/lita (55 micromol/L).
- I nā pākeke, inā ʻoi aku ia ma mua o 30 micromol/lita (30 micromol/L).
He aha nā hoʻokolohua e hana ʻia e ʻike ai i kēia?
ʻO ka hoʻāʻo nui he hoʻāʻo koko e ana i ka pae o ka ammonia i loko o kou koko. Inā kiʻekiʻe kou pae ammonia, hiki i kāu kauka ke hana i nā hoʻokolohua ʻē aʻe e ʻike ai i ke kumu. No ka laʻana, nā hoʻāʻo koko e nānā i ka hana o kou ake a me ka hana o nā puʻupaʻa , a me ka urinalysis . No ka mea hiki i ka hyperammonemia ke hōʻino i ka ʻōnaehana nerve waena a hoʻoweliweli i ke ola, hiki i kāu kauka ke hana i nā hoʻokolohua kiʻi, e like me ka CT (computed tomography) scan a i ʻole MRI (magnetic resonance imaging) scan, e nānā i nā pilikia e pili ana i ka lolo.He aha nā lāʻau lapaʻau no ka hyperammonemia?
Aia ka lāʻau lapaʻau ma ke kumu. No ka laʻana, i loko o kahi pākeke, ma muli ia o ka maʻi akepaʻa a i ʻole ka encephalopathy hepatic, a i loko o kahi keiki hānau hou, ma muli ia o kahi maʻi pōʻaiapuni urea (UCD). I ka hyperammonemia koʻikoʻi, ʻo ka pahuhopu nui ka hoʻemi ʻana i ka nui o ka ammonia i loko o ke koko a kaohi i nā pilikia kikoʻī e like me ka edema cerebral a me ka hoʻonui ʻia o ke kaomi a puni ka lolo (intracranial hypertension).- Inā loaʻa i kahi pēpē hānau hou ka hyperammonemia, e hoʻōki nā kauka i ka hāʻawi ʻana iā lākou i ka protein (no ka mea, hana ʻia ka ammonia i ka wā e ʻeli ʻia ai ka protein) a hāʻawi iā lākou i ka glucose, kahi hopena kō, e hāʻawi i nā calorie. E hoʻohana pū lākou i kahi kaʻina hana i kapa ʻia ʻo hemodialysis . Pili kēia i ka hoʻomaʻemaʻe ʻana i ke koko o ka pēpē a me ka wehe ʻana i ka ammonia ma o kahi mīkini dialysis a me kahi kānana kūikawā (e like me kahi puʻupaʻa hana).
- ʻO ka poʻe me ka hapa hapa o ka urea cycle enzyme deficiency (UCD) e hoʻomohala ana i ka hyperammonemia ma muli o ke kaumaha, e like me ka hyperammonemia i hoʻoulu ʻia e ke kaumaha, hāʻawi ʻia lākou i ka protein withdrawal a me ka glucose ma ke ʻano he calorie. Hana wale ʻia ka hemodialysis inā ʻaʻole e emi iho ko lākou pae ammonia i loko o kekahi mau hola o ka mālama mua ʻana.
- ʻO ka lāʻau lapaʻau no ka encephalopathy hepatic, ʻo ia ka hoʻēmi ʻana i ka hana ʻana o ka ammonia i loko o nā ʻōpū. ʻO ka lāʻau lapaʻau mua, ʻo ia nā lāʻau lapaʻau waha e loaʻa ana ka lactulose a me ka lactitol . Hoʻēmi kēia mau kō i ka hana ʻana a me ka omo ʻana o ka ammonia i loko o nā ʻōpū.
- ʻO L-carnitine
- ʻO L-ornithine-L-aspartate
- ʻArikinina
Aia kekahi mau ala e pale aku ai i kēia kūlana?
No ka mea, he nui nā kumu o ka hyperammonemia, ʻaʻole hiki ke pale ʻia nā kūlana āpau. Eia nō naʻe, hiki iā ʻoe ke hana i nā hana e pale aku ai i kekahi mau ʻano maʻi akepaʻa, ʻoiai nā mea e pili ana i kāu ʻai a me kou nohona. Inā pilikia ʻoe i ka maʻi akepaʻa, hiki i kāu kauka ke ʻōlelo aku e hana i kēia mau hoʻololi nohona:- He mea maikaʻi ke pale a kaupalena paha i ka inu ʻana i ka waiʻona.
- E hōʻalo i nā meaʻai a me nā mea inu i loaʻa nā momona trans a i ʻole ka syrup kulina fructose kiʻekiʻe.
- I mea e pale aku ai i ka hōʻino ʻana o ke akepaʻa, e makaʻala loa i ka wā e hoʻohana ai i nā lāʻau lapaʻau i kuhikuhi ʻia a me nā lāʻau lapaʻau i kūʻai ʻia ma luna o ke kauka.
- Hoʻoikaika kino mau .
- Ke kaupalena nei i ka ʻai ʻana i ka ʻiʻo ʻulaʻula.
He aha ka wānana no kēia kūlana?
ʻO ka nānā ʻana no ka hyperammonemia, ʻo ia hoʻi, ka hiki ke ho'ōla, hilinaʻi ia i kekahi mau kumu:- Pehea ke kiʻekiʻe o kou mau pae ammonia.
- Pehea ka lōʻihi o ka hoʻokiʻekiʻe ʻia ʻana o ka pae ammonia.
- He aha ke kumu o ka hyperammonemia?
He aha nā pilikia e hiki mai ana mai ka hyperammonemia?
Inā ʻaʻole mālama koke ʻia kēia kūlana, hiki i nā pae ammonia kiʻekiʻe ke hōʻino i ka ʻōnaehana nerve waena a alakaʻi i nā pilikia e hoʻoweliweli ai i ke ola. ʻO ia mau pilikia:- ʻEdema lolo
- Hoʻonui ʻia ke kaomi a puni ka lolo (Intracranial hypertension)
- ʻO ka herniation o ka lolo - ʻO kēia ka mea i hoʻoulu pinepine ʻia e ka pehu ʻana o ka lolo a me ka hoʻonui ʻia o ke kaomi a puni ka lolo.
- Koma
- Make
Āhea mākou e ʻike ai i ke kauka?
Inā loaʻa iā ʻoe a i ʻole kekahi āu e mālama nei nā hōʻailona o ka hyperammonemia, e kelepona koke i ka 911 a i ʻole e hele i ka haukapila kokoke loa. ʻAʻole kēia he mea e hoʻopaneʻe ai. Inā loaʻa iā ʻoe ka maʻi ate a i ʻole ka hemahema hapa o ka enzyme urea cycle, he mea nui loa ia e ʻike pinepine i kāu kauka e mālama i kou kūlana a nānā i kāu mau pae ammonia.He maʻi koʻikoʻi ka Hyperammonemia i hiki ke hana ʻia e kekahi mau mea. Inā loaʻa iā ʻoe, kāu keiki, a i ʻole kāu pēpē kēia mau hōʻailona, e ʻimi koke i ke kōkua. Inā loaʻa iā ʻoe ka maʻi akepaʻa, a inā he maʻi pōʻaiapuni urea (UCDs) kekahi o kou ʻohana, e kamaʻilio me kāu kauka e pili ana i kou pilikia o ka loaʻa ʻana o ka hyperammonemia. Aia lākou ma laila e kōkua iā ʻoe.
ʻO ka mea hope loa, e hoʻomanaʻo i kēia.
ʻAe, eia kekahi o nā mea nui e pono ai ʻoe e hoʻomanaʻo mai nā mea a mākou i kamaʻilio ai:- ʻO ka Hyperammonemia kahi pae kiʻekiʻe loa o ka ammonia i loko o ke koko. He mea ʻino loa kēia i ko kākou lolo a me ka ʻōnaehana nerve.
- Nui nā kumu no kēia. ʻO nā pilikia o ke akepaʻa a me nā maʻi pōʻaiapuni urea (UCD) nā kumu nui.
- ʻOkoʻa nā hōʻailona ma muli o ke koʻikoʻi o ke kūlana a me ka makahiki. Hiki iā lākou ke ʻano mai nā hōʻailona liʻiliʻi e like me ka nausea, ka luaʻi ʻana, a me ke poʻo ʻeha i nā hōʻailona koʻikoʻi e like me ka huikau, nā hopu ʻana, a me ka coma. Pono hoʻi nā pēpē hānau hou e hopohopo nui e pili ana i kā lākou mau hōʻailona.
- Inā loaʻa iā ʻoe nā ʻōuli koʻikoʻi, pono ʻoe e ʻimi koke i ke kōkua lapaʻau. Hiki i kēia ke lilo i kūlana ola a make paha.
- Aia ka lāʻau lapaʻau ma ke kumu. Hiki i ka lāʻau lapaʻau koke ke hōʻemi i nā pae ammonia a kāohi i nā pilikia.
- ʻOiai ʻaʻole hiki ke pale ʻia kekahi mau kumu, hiki ke hoʻemi ʻia kekahi mau pilikia ma ka mālama ʻana i kahi nohona olakino a me ka mālama ʻana i kou ake .
- Inā he maʻi akepaʻa kou a i ʻole he UCD ko kekahi o kou ʻohana, he mea nui e loaʻa nā hoʻokolohua lapaʻau maʻamau.
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