ʻO ka hoʻomanawanui fructose hereditary

ʻO ka Hereditary fructose intolerance kahi maʻi e nele ai ke kanaka i ka protein e pono ai e wāwahi i ka fructose. ʻO Fructose kahi kō kō e kū maoli i ke kino. Hoʻohana ʻia ka fructose i hana ʻia e ke kanaka ma ke ʻano he meaʻono i nā meaʻai he nui, e like me ka meaʻai a me nā mea inu pēpē.

Kū kēia ʻano ke nalowale ke kino i kahi enzyme i kapa ʻia ʻo aldolase B. Pono kēia mea e wāwahi i ka fructose.

Inā ʻai kekahi kanaka me ka ʻole o kēia mea i ka fructose a i ʻole ka sucrose (kō kō a i ʻole beet kō, papa kō), e loli ka hoʻololi ʻana o nā kemika i loko o ke kino. ʻAʻole hiki i ke kino ke hoʻololi i kāna waihona kō (glycogen) i mālama ʻia i glucose. ʻO ka hopena, hāʻule ke kō kō a me nā mea weliweli e kūkulu i ke ake.

Hoʻoilina ka hoʻoilina hoʻoilina hoʻoilina, ʻo ia hoʻi hiki ke hoʻoili ʻia i waena o nā ʻohana. Inā halihali nā mākua ʻelua i kahi kope hana ʻole o ka ʻaoʻao aldolase B, he 25% (1 i ka 4) o kā lākou keiki e hoʻopilikia ʻia ai.

Hiki ke ʻike ʻia nā ʻōuli ma hope o ka hoʻomaka ʻana o ka pēpē e ʻai i ka meaʻai a i ʻole ka haʻina.

ʻO nā ʻōuli mua o ka intolerance fructose e like ia me nā galactosemia (hiki ʻole ke hoʻohana i ka galactose kō). Ma hope e pili hou nā ʻōuli i ka maʻi ate.

Pākuʻi ʻia paha nā ʻōuli:

• Nā manaʻo hoʻohoka
• Ka hiamoe nui loa
• Huhū
• ʻO kaʻili melemele a keʻokeʻo paha o nā maka (jaundice)
• ʻO ka hānai maikaʻi ʻole a me ka ulu ʻana ma ke ʻano he pēpē, ulu ʻole
• Nā pilikia ma hope o ka ʻai ʻana i nā hua a me nā meaʻai ʻē aʻe i piha i ka fructose a i ʻole ka sucrose
• ʻO ka luaʻi

Hōʻike paha ka hoʻokolokolo kino:

• Hoʻonui i ke ake a me nā ʻāpala
• ʻO Jaundice

ʻO nā hoʻāʻo e hōʻoia i ka ʻike me:

• Nā hoʻāʻo ʻana o ke kāpili koko
• Hōʻike kō kō
• Nā aʻo Enzyme
• Ke hoʻāʻo genetic
• Nā hoʻāʻo hana hakuʻala
• Nā hoʻāʻo hana ate
• ʻO ka biopsy ate
• Hōʻike koko ʻakika uric
• Urinalysis

E haʻahaʻa ke kō kō, ma hope o ka loaʻa ʻana o ka fructose a i ʻole ka sukrosa. E kiʻekiʻe nā pae waikawa Uric.

ʻO ka hemo ʻana i ka fructose a me ka sucrose mai ka papaʻai he lāʻau lapaʻau maikaʻi ia no ka hapanui o ka poʻe. E mālama ʻia paha nā pilikia. ʻO kahi laʻana, hiki i kekahi poʻe ke lawe i ka lāʻau e hoʻohaʻahaʻa i ka pae o ka uric acid i loko o ko lākou koko a hoʻoliʻiliʻi i ko lākou makaʻi no ka maʻi gout.

Hereditary fructose intolerance he akahai a ʻino hoʻi.

ʻO ka pale ʻana i ka fructose a me ka sucrose kōkua i ka hapa nui o nā keiki me kēia ʻano. Maikaʻi ka wānana i ka nui o nā hihia.

ʻO kekahi mau keiki me kahi ʻano koʻikoʻi o ka maʻi e hoʻomohala ʻia i nā maʻi ake ake. ʻOiai ʻo ka hemo ʻana i ka fructose a me ka sucrose mai ka papaʻai hiki ʻole ke pale aku i nā maʻi ake nui i kēia mau keiki.

Maikaʻi ka hilinaʻi ʻana o ke kanaka:

• Pehea ka manawa koke e hana ʻia ai ke kuhi
• Pehea koke e wehe ʻia ai ka fructose a me ka sucrose mai ka papaʻai
• Pehea ka maikaʻi o ka hana ʻana o ka enzyme i ke kino

Hiki ke hana i kēia mau pilikia:

• Ka hōʻalo ʻana i nā meaʻai i loaʻa i ka fructose ma muli o kā lākou hopena
• Kahena
• Goute
• ʻO ka maʻi mai ka ʻai ʻana i nā meaʻai me ka fructose a i ʻole ka sucrose
• Hoka kōpaʻa
• Ke kō koko haʻahaʻa (hypoglycemia)
• Ka hopu ʻana i nā maʻi
• Make

Kāhea i kāu kahu mālama ola inā loaʻa i kāu keiki nā ʻōuli o kēia ʻano ma hope o ka hoʻomaka ʻana o ka hānai ʻana. Inā loaʻa kēia maʻi i kāu keiki, paipai nā poʻe loea i ka ʻike ʻana i ke kauka i loea i ka genchemics genchemics a i ʻole ka metabolism.

ʻO nā kāne me ka moʻolelo o ka ʻohana o ka hoʻomanawanui fructose e makemake nei e loaʻa kahi pēpē e noʻonoʻo paha i ka ʻōlelo aʻo kūpuna.

ʻO ka hapa nui o nā hopena maikaʻi ʻole o ka maʻi i hiki ke pale ʻia e ka hōʻemi ʻana i ka fructose a me ka lawe ʻana i ka sucrose.

Fructosemia; ʻAno ʻole ʻo Fructose; ʻO Fructose aldolase B-nele; Ka hemahema o Fructose-1, 6-bisphosphate aldolase

Bonnardeaux A, Bichet DG. ʻO nā maʻi hoʻoilina o ka tubule renal. I: Skorecki K, Chertow GM, Marsden PA, Taal MW, Yu ASL, eds. ʻO Brenner a me Rector's The Kidney. 10 o ka lā. Piladelapia, PA: Elsevier; 2016: chap 45.

Kishnani PS, Chen Y-T. Nā ʻino ma ka metabolism o nā haʻalako. I: Kliegman RM, St. Geme JW, Schor NF, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. ʻO Puka Textbook of Pediatrics ʻo Nelson. 21st ed. Piladelapia, PA: Elsevier; 2020: chap 105.

Nadkarni P, Weinstock RS. Kalepona. I: McPherson RA, Pincus MR, hua. ʻO kā Henry's Clinical Diagnosis a me Management e nā Laboratory Methods. 23rd Ed. St Louis, MO: Elsevier; 2017: chap 16.

ʻO Scheinman SJ. Nā maʻi maʻi hōʻoki ʻōiwi. I: Gilbert SJ, Weiner DE, eds. ʻO ka National Primer Foundation ma ka maʻi maʻi maʻi. ʻEpekema 7 Piladelapia, PA: Elsevier; 2018: chap 38.