Ukungabekezelelani okubangelwa ifa le-fructose

Ukungabekezelelani kwe-Hereditary fructose kuyisifo lapho umuntu engenawo amaprotheni adingekayo ukuze adilize i-fructose. I-Fructose ushukela wezithelo owenzeka ngokwemvelo emzimbeni. I-fructose eyenziwe ngumuntu isetshenziswa njengesiphuzo ekudleni okuningi, kufaka phakathi ukudla kwezingane neziphuzo.

Lesi simo senzeka lapho umzimba ulahlekelwe yi-enzyme ebizwa nge-aldolase B. Le nto iyadingeka ukuze kudilizwe i-fructose.

Uma umuntu ongenayo le nto edla i-fructose noma i-sucrose (umoba noma ishukela le-beet, ushukela wetafula), izinguquko eziyinkimbinkimbi zamakhemikhali zenzeka emzimbeni. Umzimba awukwazi ukushintsha uhlobo olugcinwe lukashukela (i-glycogen) lube yi-glucose. Ngenxa yalokho, ushukela wegazi uyawa bese izinto eziyingozi zakha esibindini.

Ukungabekezelelani ngefa lika-fructose kuzuzwe njengefa, okusho ukuthi kungadluliselwa emindenini. Uma bobabili abazali bephethe ikhophi engasebenziyo yofuzo lwe-aldolase B, ingane ngayinye inethuba lokuthinteka elingu-25% (1 in 4).

Izimpawu zingabonakala ngemuva kokuthi ingane iqale ukudla ukudla noma ifomula.

Izimpawu zokuqala zokungabekezelelani kwe-fructose ziyefana nalezi ze-galactosemia (ukungakwazi ukusebenzisa ushukela galactose). Izimpawu zakamuva zihlobene kakhulu nesifo sesibindi.

Izimpawu zingafaka:

• Ukudlikizela
• Ukulala ngokweqile
• Ukuthukuthela
• Isikhumba esiphuzi noma abamhlophe bamehlo (i-jaundice)
• Ukondla okungafanele nokukhula njengengane, ukwehluleka ukukhula
• Izinkinga ngemuva kokudla izithelo nokunye ukudla okuqukethe i-fructose noma i-sucrose
• Ukuhlanza

Ukuhlolwa komzimba kungakhombisa:

• Isibindi esikhulisiwe nobende
• I-jaundice

Ukuhlolwa okuqinisekisa ukuxilongwa kufaka:

• Ukuhlolwa kwegazi
• Ukuhlolwa kukashukela egazini
• Izifundo ze-enzyme
• Ukuhlolwa kofuzo
• Ukuhlolwa kokusebenza kwezinso
• Ukuhlolwa kokusebenza kwesibindi
• I-biopsy yesibindi
• Uric acid ukuhlolwa kwegazi
• Ukuhlolwa komchamo

Ushukela wegazi uzoba phansi, ikakhulukazi ngemuva kokuthola i-fructose noma i-sucrose. Amazinga e-uric acid azobe ephakeme.

Ukususa i-fructose ne-sucrose ekudleni kuyindlela yokwelapha esebenzayo kubantu abaningi. Izinkinga zingalashwa. Isibonelo, abanye abantu bangathatha umuthi ukwehlisa izinga le-uric acid egazini labo futhi banciphise ubungozi babo bokutholwa yi-gout.

Ukungabekezelelani ngefa lika-fructose kungaba mnene noma kube nzima.

Ukugwema i-fructose ne-sucrose kusiza izingane eziningi ezinalesi simo. Isibikezelo sihle ezimweni eziningi.

Izingane ezimbalwa ezinesimo esibi sesifo zizoba nesifo esibi sesibindi. Ngisho nokususa i-fructose ne-sucrose ekudleni kungahle kungavimbeli izifo ezinzima zesibindi kulezi zingane.

Ukuthi umuntu wenza kahle kangakanani kuya ngokuthi:

• Ukuxilongwa kwenziwa ngokushesha kangakanani
• Kungasuswa ngokushesha kangakanani i-fructose ne-sucrose ekudleni
• Isebenza kahle kanjani i-enzyme emzimbeni

Lezi zinkinga zingenzeka:

• Ukugwema ukudla okuqukethe i-fructose ngenxa yemiphumela yako
• Ukopha
• Gout
• Ukugula ngokudla ukudla okuqukethe i-fructose noma i-sucrose
• Ukuhluleka kwesibindi
• Ushukela wegazi ophansi (i-hypoglycemia)
• Ukuquleka
• Ukufa

Shayela umhlinzeki wakho wezokunakekelwa kwezempilo uma ingane yakho iba nezimpawu zalesi simo ngemuva kokuqala kokondla. Uma ingane yakho inale nkinga, ochwepheshe bancoma ukuthi ubonane nodokotela ogxile ku-biochemical genetics noma ku-metabolism.

Imibhangqwana enomlando womndeni wokungabekezelelani kwe-fructose efisa ukuba nengane ingabheka ukwelulekwa ngezofuzo.

Imiphumela eminingi elimazayo yalesi sifo ingavinjelwa ngokuncipha kokudla kwe-fructose kanye ne-sucrose.

I-Fructosemia; Ukungabekezelelani kwe-Fructose; Ukushoda kwe-Fructose aldolase B; Ukushoda kwe-Fructose-1, 6-bisphosphate aldolase

UBonnardeaux A, uBichet DG. Ukuphazamiseka okuzuzwe njenge-tubula yezinso. Ku: Skorecki K, Chertow GM, Marsden PA, Taal MW, Yu ASL, ama-eds. IBrenner neRector's The Kidney. Umhlaka 10. IPhiladelphia, PA: Elsevier; 2016: isahluko 45.

IKishnani PS, Chen YT. Amaphutha ku-metabolism ye-carbohydrate. Ku: Kliegman RM, St Geme JW, Schor NF, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Incwadi kaNelson Yezingane. Umhlaka 21. IPhiladelphia, PA: Elsevier; 2020: isahluko 105.

UNadkarni P, Weinstock RS. Ama-carbohydrate. Ku: McPherson RA, Pincus MR, abahleli. UHenry’s Clinical Diagnosis and Management by Laboratory Methods. Umhla ka-23. ISt Louis, MO: Elsevier; I-2017: isahluko 16.

Isikimu SJ. Izinkinga zokuthuthwa kwezinso ezisekelwe kwizakhi zofuzo. Ku: Gilbert SJ, Weiner DE, ama-eds. I-Primer yezifo zezinso iNational Kidney Foundation. Umhla wesi-7. IPhiladelphia, PA: Elsevier; 2018: isahluko 38.