Une ammonia yakawandisa muropa rako here? Ngatidzidzei nezvengozi ye hyperammonemia!

Kune zvinhu zvakawanda zviri kuitika mumiviri yedu, handiti? Dzimwe nguva, kana maitiro aya apera zvishoma, matambudziko makuru anogona kumuka. Semuenzaniso, hyperammonemia chirwere chakakomba chinoitika kana tsvina inonzi ammonia ikawedzera muropa redu. Zvakakosha kuziva izvi nekuti zvinogona kukanganisa hupenyu hwako zvikuru kana zvikasatarisirwa zvakanaka.

Chii chinonzi Hyperammonemia?

Zvichitaurwa zviri nyore, hyperammonemia inoitika kana huwandu hweammonia muropa rako hwakwira zvisingawanzoitiki. Ammonia, kana kuti NH3, tsvina inogadzirwa muura hwedu patinogaya mapuroteni atinodya. Kazhinji, ammonia iyi inoenda kuchiropa chedu uye inogadziriswa ipapo. Tinodana maitiro aya kuti urea cycle . Kuburikidza nedenderedzwa iri, ammonia inoshandurwa kuita chinhu chisina kukuvadza chinonzi urea uye inoburitswa muweti. Zvisinei, kana chimwe chinhu chikakanganisika nedenderedzwa iri reurea, kana chiropa chikatadza kugadzirisa ammonia iyi nemazvo, ammonia inotanga kuungana muropa. Kuwedzera uku kweammonia muropa kune chepfu zvikuru kuCentral Nervous System yedu (CNS) , inova uropi nemusana. Ndosaka hyperammonemia iri chirwere chinoda kurapwa nekukurumidza uye chinogona kutotyisidzira hupenyu .

Ndiani anogona kukudziridza izvi zvakanyanya?

Dambudziko iri, rinonzi hyperammonemia, rinogona kukanganisa vacheche, vana vadiki, nevakuru.

• Pakati pevanhu vakuru, vanenge 90% yeavo vane chirwere ichi ndeavo vane cirrhosis yechiropa .
• Vana vacheche vanogona kukura nechirwere ichi nekuti vanozvarwa vaine dambudziko mu urea cycle (congenital urea cycle disorder) . Izvi zvirwere zvemajini.
• Uyewo, kutadza kushanda zvakanaka kwechiropa uye zvirwere zvemuviri zvinogarwa nhaka zvinogona kukonzera hyperammonemia muvana vadiki.

Zvakaoma kutaura kuti izvi zvakajairika sei, sezvo zvichigona kukonzerwa nezvinhu zvakasiyana-siyana. Asi vaongorori vanofungidzira kuti matambudziko emudumbu, chimwe chezvikonzero, anokanganisa mwana mumwe chete pavana 250,000 vanozvarwa muUnited States uye mumwe chete pavana 440,000 vanozvarwa pasi rose.

Ndezvipi zvikonzero zve hyperammonemia?

Panogona kuva nezvikonzero zvakawanda zveizvi. Zvikonzero izvi zvinogona kuchinja nekukura. Asi kazhinji, zvikonzero zviviri zvikuru zvinetso zvechiropa uye urea cycle disorders (UCDs) .

Nekuda kwematambudziko echiropa

Chiropa chedu ndicho chikamu chikuru chinoshandura ammonia kuita urea. Saka kana chiropa chakakuvara kana kuti chiine chirwere, hachigone kugadzirisa ammonia nemazvo. Apa ndipo apo ammonia inoungana muropa. Heano mamwe matambudziko ane chekuita nechiropa anogona kukonzera hyperammonemia:

• Chirwere chechiropa : Kana chiropa chakakuvara , kugona kwacho kugadzira ammonia kushoma. Izvi zvinowanzoitika muzvirwere zvechiropa zvakakomba, asi kunyangwe kune vanhu vane chirwere chechiropa chakagadzikana, huwandu hweammonia dzimwe nguva hunogona kukwira kamwe kamwe, semuenzaniso, kana chimwe chinhu chikaitika mudumbu kana muura ( kubuda ropa mudumbu) kana kana paine kusawirirana kwe electrolyte . Ichi ndicho chikonzero chikuru chehuwandu hwakawanda hweammonia muvakuru nevana.
• Hepatic encephalopathy : Izvi zvinoitika kana chiropa chakakuvara uye chikasakwanisa kugadzirisa ammonia nemazvo. Ammonia inoungana muropa yoenda kuuropi. Izvi zvinogona kukonzera kuvhiringidzika, kuvhiringidzika , uye kutoita coma. Dzimwe nguva zvinogona kuuraya.
• Kurwara kwechiropa: Kurwara kwechiropa kunotsiva mavanga echiropa ane hutano. Izvi zvinogona kukonzera kuti chiropa chisashande zvakanaka uye zvinogona kukonzera hyperammonemia. Kurwara kwechiropa chirwere chisingaperi chechiropa.
• Kukundikana kwechiropa zvakanyanya: Apa ndipo apo chiropa chinomira kushanda kamwe kamwe. Izvi zvinogona kukonzerwa nezvirwere zvakasiyana-siyana nemamiriro ezvinhu. Semuenzaniso, kukuvara kwechiropa kunokonzerwa nemishonga kunokonzerwa nemimwe mishonga, yakaita seacetaminophen , kunokonzera 50% yezviitiko zvese zvekukundikana kwechiropa zvakanyanya muUnited States. Izvi zvinokonzera 64% yezviitiko zvehyperammonemia muvana.
• Kuderera kweropa rinoyerera muchiropa: Kana ropa rikasayerera zvakanaka muchiropa, muviri haugone kutumira ammonia kuchiropa kuti igadziriswe. Kunyangwe zvakadaro, ammonia inoungana muropa.
• Reye's syndrome: chirwere chepfungwaIchi chirwere chisingawanzoitiki. Chinokanganisa ropa, uropi, nechiropa. Chinowanzo kukonzera kuwanda kweammonia muropa uye kuderera kweshuga muropa. Chinowanzo bata vana vadiki nevechiri kuyaruka vari kupora kubva kuhutachiona hwakadai sechickenpox kana furuwenza/furuwenza uye vakatora aspirin nekuda kwezviratidzo izvozvo. Chikonzero chaicho cheReye's syndrome hachizivikanwe. Zvisinei, nekuda kwenjodzi iyi, aspirin haifanirwe kupihwa kuvana vadiki nevechiri kuyaruka kunze kwekunge chiremba ataura zvakananga.

Nekuda kweUrea Cycle Disorders (UCDs)

Sezvatakambokurukura, urea cycle inzira iyo ammonia ine chepfu inoshandurwa kuita urea yoburitswa muweti. Iyi cycle ine matanho akati wandei, imwe neimwe inoda mhando yakasiyana yeenzyme. Aya ma enzyme ndeaya:

• N-acetyl-glutamate synthase (NAGS)
• Carbamoyl phosphate synthetase (CPS)
• Ornithine transcarbamylase (OTC)
• Argininosuccinate synthetase (AS)
• Argininosuccinic acid lyase (ASL)
• Arginase (ARG1)

Kana imwe yema enzyme aya isina kukwana, urea cycle haishande nemazvo. Ammonia inoungana muropa. Rudzi urwu rwekushomeka kwema enzyme runonzi urea cycle disorder (UCD). MaUCD anogona kukonzera hyperammonemia, iyo inogona kuva acute kana chronic. MaUCD chirwere chekuzvarwa nacho. Vana vacheche vane kushomeka kwakakwana kwe urea cycle enzyme vanowanzova acute hyperammonemia mukati memaawa 24 kusvika 72 ekuzvarwa. Kunyangwe kushomeka kwema enzyme kuri kudiki kana kuti kushoma, ammonia inoungana inogona kuitika chero nguva muhupenyu, panguva yekurwara, kushushikana, kana zvimwe zviitiko zvinoshungurudza. MaUCD aya anokonzera 23% yezviitiko zve acute hyperammonemia muvana vadiki vanorwara zvakanyanya.

Zvimwe zvikonzero

Kunze kweizvi, panogona kunge paine zvimwe zvikonzero zvakawanda:

• Kukundikana kweitsvo (itsvo): Kana itsvo dzikatadza kubvisa urea zvakanaka nekuda kwekutadza kushanda kweitsvo, zvinogona kukonzera kuti ammonia iungane muropa.
• Zvimwe zvirwere zveropa: Hyperammonemia inogonawo kukonzerwa nezvirwere zveropa zvakaita se multiple myeloma uye acute leukemia.
• Zvimwe zvirwere: zvipembenene zvinogadzira urease zvakaita seProteus mirabilis , Escherichia coli (E. coli) uye KlebsiellaUtachiona hwemabhakitiriya hwakadai sestreptococci, kunyanya kuvana vane dambudziko rekuzvarwa naro renzira yeweti uye kune vakwegura vane weti inochengetwa nekuda kwehutachiona hwenzira yeweti (UTIs), hunogona kukonzera hyperammonemia yakanyanya.

Ndezvipi zviratidzo zveHyperammonemia?

Zviratidzo zvinogona kusiyana zvakanyanya zvichienderana nekuti chirwere chacho chakanyanya kuderera kana kuti chakanyanya sei, pamwe nezera rachinotanga.

Zviratidzo zvishoma zvinowanzoonekwa kuvana nevakuru

• Kusvotwa nekurutsa
• Kurwadziwa kwedumbu
• Kushatirwa kazhinji
• Musoro (musoro)
• Kuoma nekuenzanisa, kubatana, uye kutaura (ataxia)
• Kuchinja kwemaitiro

Zvimwe zviratidzo zvishoma zvinongobata vana chete

• Kukundikana kubudirira
• Kushaya simba kwetsandanyama (hypotonia)
• Kunonoka kwekuvandudzwa kwetsinga dzeuropi

Zviratidzo zvakakomba muvana nevakuru

Izvi zviratidzo zvine njodzi zvikuru. Kana uine chimwe cheizvi, unofanira kutsvaga rubatsiro rwechiremba nekukasika.

• Kuvhiringidzika uye kuvhiringidzika
• Kuchinja-chinja kwemanzwiro
• Kurara zvakanyanya
• Kuchinja mukuziva
• Kubatwa nepfari
• Kufema nekukurumidza (Hyperventilation)
• Kushaya simba

Yambiro: Kana iwe kana mumwe munhu ari pedyo newe aine zviratidzo izvi, ndapota fonera 911 nekukasika, kana kuenda kuchipatara chiri pedyo newe. Kuwanda kweammonia muropa kunogona kutyisidzira hupenyu uye kunoda kurapwa nekukurumidza!

Zviratidzo muvana vacheche

Kana mwana achangoberekwa akabatwa ne hyperammonemia, zviratidzo zvinotevera zvinowanzoonekwa mukati memaawa makumi maviri nemana kusvika makumi manomwe nemaviri ekuzvarwa:

• Kugumbuka kakawanda, kuchema kakawanda
• Kurutsa
• Kuneta
• Kubatwa nepfari
• Kugunun'una kana uchifema
• Kufema nekukurumidza (Hyperventilation)

Zvakakosha: Kana mwana wako akaratidza zviratidzo izvi, fonera chiremba nekukasika kana uchiri muchipatara. Kana uri kumba, fonera 911 nekukasika, kana kuti umuendese kuchipatara chiri pedyo. Izvi hazvisi zvekunonotsa.

Vanachiremba vanoongorora sei izvi?

Vanachiremba vanowanzofungidzira kuti ane hyperammonemia nekuongorora huwandu hweammonia muropa.

• Kune vacheche kusvika pamwedzi mumwe chete, kana huwandu hweammonia muropa hwakakura kupfuura 80 micromol/litre (80 micromol/L).
• Kune vana vakura, kana ichipfuura 55 micromol/lita (55 micromol/L).
• Muvanhu vakuru, kana ichipfuura 30 micromol/lita (30 micromol/L).

Iyi yuniti yekuyera (micromol/L) inzira yekuyera huwandu hweammonia muropa.

Ndedzipi bvunzo dzinoitwa kuti zvionekwe izvi?

Kuongororwa kukuru kweropa kunoyera huwandu hweammonia muropa rako. Kana huwandu hweammonia yako hwakakwira, chiremba wako anogona kuita mimwe miedzo kuti awane chikonzero. Semuenzaniso, kuongororwa kweropa kuti aone mashandiro echiropa chako uye mashandiro eitsvo , pamwe nekuongorora weti . Nekuti hyperammonemia inogona kukuvadza sisitimu yetsinga uye inogona kutyisidzira hupenyu, chiremba wako anogonawo kuita bvunzo dzekufungidzira, dzakadai seCT (computed tomography) scan kana MRI (magnetic resonance imaging) scan, kuti aone kana paine matambudziko anokanganisa uropi.

Ndeipi mishonga yekurapa hyperammonemia?

Kurapwa kunoenderana nechikonzero chacho. Semuenzaniso, kumunhu mukuru, kunokonzerwa nechirwere chechiropa kana hepatic encephalopathy, kana kumwana achangoberekwa, kunokonzerwa ne urea cycle disorder (UCD). Mu acute hyperammonemia, chinangwa chikuru ndechekuderedza huwandu hwe ammonia muropa uye kudzora matambudziko akadai se cerebral edema uye kuwedzera kwekumanikidzwa kwakatenderedza uropi (intracranial hypertension).

• Kana mwana achangoberekwa aine hyperammonemia, vanachiremba vanorega kumupa mapuroteni (nekuti ammonia inogadzirwa kana mapuroteni agayiwa) asi vanomupa glucose, mushonga weshuga, kuti ape macalorie. Vachashandisawo nzira inonzi hemodialysis . Izvi zvinosanganisira kuchenesa ropa remwana uye kubvisa ammonia kuburikidza nemuchina we dialysis uye firita yakakosha (senge itsvo yekugadzira).
• Vanhu vane partial urea cycle enzyme deficiency (UCD) vanova ne hyperammonemia nekuda kwekushushikana, zvakaita se stress-induced hyperammonemia, vanopihwawo protein offensive uye glucose semacalorie. Hemodialysis inongoitwa chete kana huwandu hwavo hweammonia husina kudzikira mukati memaawa mashoma ekurapwa kwekutanga.
• Kurapa kwechirwere chehepatic encephalopathy ndekuderedza kugadzirwa kweammonia muura. Kurapa kwekutanga mishonga inonwiwa ine lactulose ne lactitol . Shuga idzi dzinoderedza kugadzirwa nekutorwa kweammonia muura.

Vanachiremba vanogona kukurudzira mishonga yekuwedzera yakadai seyi kubatsira vanhu vane chirwere che urea cycle kuderedza kuwanda kwezviratidzo zvavo:

• L-carnitine
• L-ornithine-L-aspartate
• Arginine

Pane nzira dzekudzivirira mamiriro ezvinhu aya here?

Nekuti kune zvakawanda zvinokonzera hyperammonemia, hazvisi zvese zvirwere zvinogona kudzivirirwa. Zvisinei, unogona kutora matanho ekudzivirira mamwe marudzi ezvirwere zvechiropa, kunyanya izvo zvine chekuita nekudya kwako uye mararamiro ako. Kana uri panjodzi yekubatwa nechirwere chechiropa, chiremba wako anogona kukurudzira kuti uchinje mararamiro aya:

• Hazvina kuipa kudzivisa kana kuderedza kunwa doro.
• Dzivisa zvekudya nezvinwiwa zvine mafuta e trans kana manyuchi echibage ane fructose yakawanda.
• Kuti udzivise kukuvara kwechiropa, chenjerera zvikuru pakushandisa mishonga inonyorwa nachiremba uye inotengeswa pasina kunyoreswa nechiremba.
• Kurovedza muviri nguva dzose .
• Kuderedza kudya nyama tsvuku.

Kuti udzivise Reye's syndrome, chirwere chinokonzera hyperammonemia muvana nevechidiki, usape vana aspirin kana vaine zviratidzo zvehutachiona (senge furuu kana chickenpox) kunze kwekunge wakurudzirwa zvakananga nachiremba. Urea cycle defects (UCDs) haiwanzoitiki, asi kune bvunzo dzemajini kuti uone kana uine majini anozvikonzera. Taura nachiremba wako nezveizvi.

Chii chiri kufanotaura nezvechirwere ichi?

Maonero e hyperammonemia, kureva kuti, kugona kupora, zvinoenderana nezvinhu zvakati wandei:

• Kuti huwandu hweammonia yako hwakakwira sei.
• Nguva yakareba sei ammonia yakwira.
• Chii chinokonzera hyperammonemia?

Kazhinji, tarisiro ye hyperammonemia haina kunaka. Kana ikasarapwa kana kurapwa kunonoka, kuzvimba kweuropi kusingadzoreki kunobatanidzwa nehuwandu hwerufu rwakanyanya. Zvinosuwisa kuti, zvisinei nekuti mwana achangoberekwa anorapwa nekukurumidza kana zvine simba sei ne hyperammonemia yakanyanya, hapana vimbiso yekuti mhedzisiro yacho ichave yakanaka. MuUnited States, huwandu hwevanhu vane urea cycle defects (UCDs) vanowana hyperammonemia vachiri vadiki hunosvika 35%. Kune avo vanowana hyperammonemia gare gare muhupenyu, huwandu hwevanhu vanorarama hunosvika 87%. Huwandu hwevanhu vanorarama kwegore rimwe chete nemakore matatu vane hepatic encephalopathy yakanyanya nekuda kwe hyperammonemia hwakataurwa kuti huri 42% uye 23%, zvichiteerana.

Ndezvipi zvinetso zvinogona kuitika nekuda kwe hyperammonemia?

Kana chirwere ichi chikasarapwa nekukasika, huwandu hwakakwira hweammonia hunogona kukuvadza sisitimu yetsinga uye hunokonzera matambudziko anotyisidzira hupenyu. Matambudziko akadaro anosanganisira:

• Kuzvimba kweuropi
• Kuwedzera kwekumanikidzwa kweuropi (Intracranial BP)
• Kubuda kweuropi - Izvi zvinowanzokonzerwa nekuzvimba kweuropi uye kuwedzera kwekumanikidzwa kwakakomberedza uropi.
• Kushaya simba
• Rufu

Kana munhu aine chirwere chehyperammonemia chisingaperi kwenguva yakareba, anogona kuva nedambudziko repfungwa uye matambudziko ekugara kwenguva refu ehunhu nepfungwa.

Tinofanira kuona chiremba rini?

Kana iwe kana mumwe munhu waunoda aine zviratidzo zvehyperammonemia, fonera 911 nekukasika kana kuenda kuchipatara chiri pedyo. Izvi hazvisi zvekunonotsa. Kana uine chirwere chechiropa kana kushomeka kwe enzyme ye urea cycle, zvakakosha kuti uone chiremba wako nguva nenguva kuti ugadzirise mamiriro ako uye utarise huwandu hwe ammonia yako.

Hyperammonemia chirwere chakakomba chinogona kukonzerwa nezvinhu zvakawanda. Kana iwe, mwana wako, kana mwana wako muine zviratidzo izvi, tsvaga rubatsiro nekukasika. Kana uine chirwere chechiropa, kana kana mumwe munhu mumhuri yako aine urea cycle disorders (UCDs), taura nachiremba wako nezvenjodzi yako yekuva nehyperammonemia. Varipo kuti vakubatsire.

Pakupedzisira, yeuka izvi.

Zvakanaka, saka hezvino zvimwe zvezvinhu zvakakosha zvaunofanira kuyeuka kubva pane zvatataura nezvazvo:

• Hyperammonemia ihuwandu hwakawanda hweammonia muropa. Izvi zvinokuvadza zvikuru uropi hwedu netsinga dzedu.
• Panogona kuva nezvikonzero zvakasiyana-siyana zveizvi. Matambudziko echiropa uye zvirwere zvemudumbu (UCDs) ndizvo zvinonyanya kukonzera izvi.
• Zviratidzo zvinosiyana zvichienderana nekuoma kwechirwere uye zera. Zvinogona kusiyana kubva pazviratidzo zvisina kunyanya kuoma zvakaita sekusvotwa, kurutsa, uye musoro kusvika pazviratidzo zvakanyanya zvakaita sekuvhiringidzika, pfari, uye coma. Vana vacheche vanofanirawo kunyanya kunetseka nezvezviratidzo zvavo.
• Kana uine zviratidzo zvakakomba, unofanira kutsvaga rubatsiro rwechiremba nekukurumidza. Izvi zvinogona kureva hupenyu kana rufu.
• Kurapwa kunoenderana nechikonzero. Kurapwa nekukasika kunogona kuderedza huwandu hweammonia uye kudzora matambudziko.
• Kunyange hazvo zvimwe zvikonzero zvisingadzivisike, dzimwe njodzi dzinogona kuderedzwa nekugara uine mararamiro ane hutano uye kutarisira chiropa chako .
• Kunyangwe uine chirwere chechiropa kana kuti mumwe munhu mumhuri mako ane UCDs, zvakakosha kuti ugare uchiongororwa nachiremba.

Usakanganwa, unoziva hutano hwako zvakanyanya. Kana uchinzwa chimwe chinhu chakasiyana kana chisingawanzoitiki, usatya kutaura nachiremba. Hyperammonemia, ammonia, chiropa, urea cycle, uropi, zviratidzo, kurapwa