Matenda a mapulo matenda amkodzo

Matenda a mkodzo (MSUD) ndimatenda pomwe thupi silimatha kuwononga magawo ena a mapuloteni. Mkodzo wa anthu omwe ali ndi vutoli amatha kununkhiza ngati madzi a mapulo.

Matenda amkodzo (MSUD) amatengera cholowa, zomwe zikutanthauza kuti amapitilira m'mabanja. Zimayambitsidwa ndi chilema m'modzi mwa majini atatu. Anthu omwe ali ndi vutoli sangathe kuwononga amino acid leucine, isoleucine, ndi valine. Izi zimabweretsa kuchuluka kwa mankhwalawa m'magazi.

Pamavuto akulu kwambiri, MSUD imatha kuwononga ubongo nthawi yamavuto amthupi (monga matenda, malungo, kapena kusadya nthawi yayitali).

Mitundu ina ya MSUD ndi yofatsa kapena kubwera ndikupita. Ngakhale atakhala ochepetsetsa, kupsinjika kwakanthawi kwamthupi kumatha kuyambitsa kupunduka kwamaganizidwe komanso kuchuluka kwa leucine.

Zizindikiro za matendawa ndi monga:

• Coma
• Kudyetsa zovuta
• Kukonda
• Kugwidwa
• Mkodzo womwe umanunkhiza ngati madzi a mapulo
• Kusanza

Mayesowa atha kuchitidwa kuti athetse vutoli:

• Mayeso a Plasma amino acid
• Mayeso a organic acid
• Kuyesedwa kwachibadwa

Padzakhala zizindikiro za ketosis (kuchuluka kwa ma ketoni, opangidwa ndi mafuta oyaka) ndi asidi owonjezera m'magazi (acidosis).

Matendawa akapezeka, komanso munthawi yamagawo, chithandizo chimaphatikizapo kudya zakudya zopanda protein. Zamadzimadzi, shuga, ndipo nthawi zina mafuta amaperekedwa kudzera mumtsempha (IV). Dialysis kudzera m'mimba mwanu kapena mtsempha ungachitike kuti muchepetse kuchuluka kwa zinthu zosazolowereka m'magazi anu.

Chithandizo cha nthawi yayitali chimafuna chakudya chapadera. Kwa makanda, chakudyacho chimaphatikizapo chilinganizo chotsika kwambiri cha amino acid leucine, isoleucine, ndi valine. Anthu omwe ali ndi vutoli ayenera kukhalabe ndi chakudya chochepa mu ma amino acid amoyo wonse.

Ndikofunikira kutsatira nthawi zonse chakudyachi kuti muchepetse kuwonongeka kwamanjenje. Izi zimafuna kuyezetsa magazi pafupipafupi komanso kuyang'aniridwa ndi dokotala wazamankhwala, komanso mgwirizano wa makolo a ana omwe ali ndi vutoli.

Matendawa akhoza kukhala owopsa ngati atapanda kuchiritsidwa.

Ngakhale ndimankhwala othandizira kudya, zovuta komanso matenda zimatha kuyambitsa ma amino acid ambiri. Imfa imatha kuchitika munthawi izi. Ndi chithandizo chamankhwala chokhwima, ana amakula mpaka kukhala achikulire ndipo amatha kukhala athanzi.

Zovuta izi zitha kuchitika:

• Kuwonongeka kwamitsempha
• Coma
• Imfa
• Kulemala m'maganizo

Itanani omwe akukuthandizani ngati muli ndi mbiri ya banja la MSUD ndipo mukukonzekera kuyambitsa banja. Komanso itanani ndi omwe amakupatsani nthawi yomweyo ngati muli ndi mwana wakhanda yemwe ali ndi zizindikiro za matenda amkodzo a mapulo.

Upangiri wamtunduwu umaperekedwa kwa anthu omwe akufuna kukhala ndi ana komanso omwe ali ndi mbiri yabanja yamatenda a nthenda ya mapulo. Ambiri akuti tsopano amawunika ana onse akhanda ndikuyesa magazi kwa MSUD.

Ngati kuyezetsa magazi kukuwonetsa kuti mwana wanu atha kukhala ndi MSUD, kuyezetsa magazi kwa ma asidi a amino kuyenera kuchitidwa nthawi yomweyo kuti atsimikizire matendawa.

MSUD

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Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM. Zofooka zamagetsi zama amino acid. Mu: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, olemba. Nelson Textbook of Pediatrics. Wolemba 21. Philadelphia, PA: Elsevier; 2020: chap 103.

Merritt JL, Gallagher RC. Zolakwika zobadwa ndi zimam'patsa mphamvu, ammonia, amino acid, ndi organic acid metabolism. Mu: Gleason CA, Juul SE, olemba. Matenda a Avery a Mwana Wongobadwa kumene. 10th ed. Philadelphia, PA: Elsevier; 2018: mutu 22.