Maple manyuchi mkodzo matenda: matenda kawirikawiri zimabweretsa matenda aakulu minyewa

Disease zokometsera - ndi matenda majini amene amagwirizana ndi matenda kagayidwe kachakudya la amino zidulo monga leucine, isoleucine ndi valine. maganizo awo mu madzi a ukuwonjezeka thupi, kuchititsa poizoni, ketoacidosis, kupweteka ndipo ngakhale chikomokere.

nkhani

Kwa nthawi yoyamba mu mabuku a zachipatala matenda zokometsera akuluakulu limafotokonzera 1954 ndi dokotala Menkes. Iwo nalo dzina lake chifukwa cha fungo achilendo ya mkodzo odwala. Akatswiri zimawoneka ngati yopsereza shuga kapena matabwa madzi. Wina, dzina sayansi - matenda zidulo nthambi.

Zimachitika pafupi kamodzi ana zana limodzi zikwi makumi asanu, chifukwa mtundu wa cholowa cha mbewu imeneyi - autosomal recessive. Chimaposa matenda zovuta ndi zambiri zimabweretsa anafa ku ubwana.

etiology

Pa chitukuko cha matenda makolo onse ayenera kukhala onyamula jini zosalongosoka ntchito dehydrogenase Alpha keto zidulo ya nthambi-unyolo. Mu chikhalidwe, enzyme izi mu zakudya zomanga thupi kulemera monga mazira, mkaka, cheese, nyama, nkhuku ndi ena. akhanda A akufotokozera atsidemiya organic, lomwe ndi loopsa kwambiri kwa wamanjenje dongosolo.

Maple manyuchi mkodzo matenda ambiri mwa Ayuda, Achiamishi, Amenoni. Ichi ndi chifukwa chakuti amakhala ndi gulu chatsekedwa zachikhalidwe, ndi mabanja amapezeka zimachitika pakati distantly kwambiri ndi nkhaniyi, kutanthauza kuti Mwina pamaso pa makolo a jini mutated udindo nzeru kwa amino zidulo ukuwonjezeka kwambiri.

zizindikiro

Pofika sabata chachiwiri pambuyo pa kubadwa akhoza molondola kudziwa matenda zokometsera. Zizindikiro ndi khalidwe atsopano mwanayo kuti: iye nthawi zonse kulira mwakachetechete, sadya, ndipo nthawi zambiri akusanza kwambiri, kusanza Mwina zimachitika. Ndi kupitirira kuledzera kuoneka kupweteka kwa minofu kamvekedwe kumawonjezera. Izi anawonetseredwa mu thupi la mwanayo ali anakoka, titero, "chala mzere" miyendo anawoloka pa mwamba pa nsapato. Mpaka chitukuko cha opisthotonos.

Ngati makolo tipitiriza kunyalanyaza matenda ndi musamatchule dokotala, gawo lotsatira la matenda - kuphwanya kupuma ndi chikumbumtima. Ana aulesi, kupanda kanthu, kugwa n'kufika poledzera kenako chikomokere. matenda a ubongo, ngakhale ndi zotsatira zabwino kukhala moyo. amalipiritsa ichi alibe yake matenda matenda zokometsera. Odwala Photos kukhumudwitsa, ndi kuti wachisoni kwambiri, ambiri a iwo ndi ana akusonyezera.

Matendawa zachokera kusanthula kukhalapo mu mkodzo zidulo sanali thovu kwa amino, komanso pa mawonetseredwe matenda.

Gawo

Malinga ndi mphamvu ya zizindikiro ndi digiri ya inertness dehydrogenase mitundu yambiri ya matenda:

1. Zachikhalidwe. Atangochoka mwana zikuoneka athanzi, monga masiku angapo, zizindikiro kuonekere. Choyamba ndi kusafuna kudya ndi kukana bere, ndiye kuwonda, nthawi apnea. Ndiye wosakwatiwa clonuses kenako zopweteka clonic-zimandilimbikitsa. Izo ukutha ndi chikomokere. Puloteni ntchito - osachepera awiri pa zana.
2. Zodziwitsa. Matendawa alibe kuonekera mpaka miyezi isanu ndi umodzi, ndipo ngakhale mpaka zaka ziwiri za moyo. The choyambitsa limagwirira ndi anasamutsidwa tizilombo toyambitsa matenda tizilombo, katemera kapena kuwonjezeka kwambiri mu kuchuluka kwa mapuloteni mu chakudya. Zizindikiro kukhala incrementally. Puloteni ntchito - peresenti makumi awiri.
3. Tiaminzavisimaya. Mu mawonekedwe ake matenda ofanana ndi maonekedwe m'mbuyomu. Kusiyana kofunika ndi kuti ntchito mankhwala a vitamini B1, amene kwambiri amachepetsa ndende ya amino zidulo mu mkodzo ndi magazi.

chithandizo

Popeza munthu anavomereza kuchipatala mu mkhalidwe poyizoni kwambiri, ndiye kuyamba kuchitira mapulo matenda madzi mkodzo m'pofunika detoxification. Chifukwa chaichi, plasmapheresis, peritoneal dialysis, kuikidwa zigawo zikuluzikulu magazi, komanso kukakamizidwa diuresis ndi hemosorption.

Pambuyo olimba wodwalayo kusintha matenda kagayidwe kachakudya. Choyamba ndi zakudya ndi kuchepetsedwa mapuloteni okhutira, nthawi zina amalangiza kupeŵa yoyamwitsa. mwambo okhwima ufulu chakudya kuthandiza kupewa kuwononga dzikoli kwa wamanjenje dongosolo.

zamakono walola pafupifupi kuchiza matenda zokometsera. Science zikusonyeza ndi zipangizo mu mankhwala mchitidwe kuti m'malo zofunika amino zidulo. Iwo amakhala mlingo wa kagayidwe mu osiyanasiyana bwinobwino, kupewa poizoni ngakhale ndi zakudya zabwinobwino.

Kugwilizana ndi malangizo, ndi chithandizo yake ku chipatala munthu angakhale moyo wathunthu. Mwatsoka, matenda a ubongo ana zikukwaniritsidwa mofulumira, ndipo makolo alibe nthawi kanthu moyenerera.