Matupi athu amagwira ntchito nthawi zonse mseri, sichoncho? Nthawi zina, pamene njira zovutazi zamoyo zasokonekera, zimatha kubweretsa mavuto aakulu pa thanzi. Vuto limodzi lofunika kwambiri ndi Hyperammonemia , lomwe limachitika pamene mankhwala oopsa otchedwa ammonia akuunjikana m'magazi mwanu. Chifukwa vutoli likhoza kukhala loopsa ngati silikuthandizidwa mwachangu, ndikofunikira kudziwa zambiri za vutoli.
Kodi Hyperammonemia ndi chiyani?
Mwachidule, hyperammonemia ndi kukwera koopsa kwa ammonia (NH3) m'magazi mwanu. Ammonia ndi chinthu china chomwe chimapezeka m'matumbo mwanu pamene thupi lanu likugaya mapuloteni omwe mumadya. Nthawi zambiri, chiwindi chanu chimagwiritsa ntchito ammonia iyi kudzera mu njira yotchedwa Urea Cycle . Njira imeneyi imasintha ammonia kukhala urea, chinthu chopanda poizoni chomwe chimatulutsidwa m'thupi mwanu mumkodzo wanu.
Komabe, ngati pali kusokonezeka kwa urea kapena ngati chiwindi chanu sichingathe kukonza bwino ammonia, chimayamba kudziunjikira m'magazi anu. Ammonia wambiri m'magazi ndi woopsa kwambiri ku Central Nervous System (CNS) - ubongo wanu ndi msana wanu. Ichi ndichifukwa chake hyperammonemia ndi vuto lachipatala lomwe limafuna chithandizo mwachangu ndipo likhoza kukhala pachiwopsezo cha moyo.
Ndani amene ali pachiwopsezo chachikulu?
Hyperammonemia ingakhudze aliyense, kuyambira makanda obadwa kumene ndi ana mpaka akuluakulu.
- Mwa akuluakulu, pafupifupi 90% ya milandu imakhudzana ndi matenda a chiwindi otchedwa Cirrhosis .
- Mwa makanda obadwa kumene, izi zimatha kuchitika chifukwa cha matenda a congenital urea cycle , omwe ndi matenda obadwa nawo a majini.
- Kwa ana aang'ono, izi zitha kuchitikanso chifukwa cha kulephera kwakukulu kwa chiwindi kapena matenda ena obadwa nawo a kagayidwe kachakudya.
Ngakhale kuti nthawi yeniyeni ndi yovuta kudziwa chifukwa cha zifukwa zosiyanasiyana, kafukufuku akusonyeza kuti monga vuto lalikulu, matenda a Urea Cycle Disorders (UCDs) amakhudza pafupifupi mwana mmodzi mwa 250,000 obadwa ku United States, ndipo pafupifupi mwana mmodzi mwa 440,000 obadwa padziko lonse lapansi.
Kodi n’chiyani chimayambitsa Hyperammonemia?
Pali zifukwa zingapo, zomwe zingasiyane malinga ndi zaka. Komabe, nthawi zambiri, magulu awiri akuluakulu ndi kulephera kugwira ntchito kwa chiwindi ndi matenda a Urea Cycle Disorders (UCDs) .
Nkhani Zokhudzana ndi Chiwindi
Chiwindi chanu ndicho chiwalo chachikulu chomwe chimasintha ammonia kukhala urea. Ngati chiwindi chanu chawonongeka kapena chadwala, chimataya mphamvu yogwiritsira ntchito ammonia bwino, zomwe zimapangitsa kuti chiziunjikana m'magazi anu.
Zinthu zomwe zingayambitse hyperammonemia ndi izi:
- Matenda a Chiwindi : Chiwindi chanu chikawonongeka , mphamvu yake yogwiritsira ntchito ammonia imachepa. Ngakhale kuti nthawi zambiri chimachitika m'matenda oopsa, ngakhale anthu omwe ali ndi matenda a chiwindi osatha amatha kukwera mwadzidzidzi mu ammonia chifukwa cha zinthu monga kutuluka magazi m'mimba kapena kusalinganika kwa electrolyte (monga, zokhudzana ndi kuchuluka kwa mchere ). Matenda a chiwindi akadali chifukwa chachikulu cha kuchuluka kwa ammonia mwa ana ndi akulu.
- Matenda a chiwindi: Izi zimachitika pamene kuwonongeka kwa chiwindi kumapangitsa kuti ammonia iunjikane ndikufikira muubongo, zomwe zimapangitsa kuti pakhale zizindikiro monga chisokonezo, kusokonezeka , komanso ngakhale chikomokere. Izi zitha kupha ngati sizikuthandizidwa.
- Matenda a chiwindi:Matenda a cirrhosis amakhudza kusintha kwa minofu ya chiwindi yathanzi ndi minofu ya zipsera, zomwe zimawononga kwambiri ntchito ya chiwindi ndipo zimatha kubweretsa hyperammonemia.
- Kulephera kwa chiwindi mwachangu: Uku ndi kutayika kwadzidzidzi kwa ntchito ya chiwindi. Mwachitsanzo, kuvulala kwa chiwindi chifukwa cha mankhwala, monga kumwa acetaminophen mopitirira muyeso, kumabweretsa 50% ya milandu yolephera kwa chiwindi mwachangu ku US. Mwa ana, 64% ya milandu ya hyperammonemia imagwirizanitsidwa ndi kulephera kwa chiwindi mwachangu.
- Kuchepa kwa magazi kupita ku chiwindi chanu: Ngati chiwindi sichilandira magazi okwanira, sichingathe kusefa bwino ndikugwiritsa ntchito ammonia, zomwe zimapangitsa kuti thupi lonse liziunjikana.
- Matenda a Reye: Uwu ndi matenda osowa komanso oopsa omwe amayambitsa kutupa m'chiwindi ndi muubongo. Nthawi zambiri amayambitsa kuchuluka kwa ammonia komanso shuga m'magazi. Amaonekera kwambiri mwa ana ndi achinyamata omwe akuchira matenda opatsirana ndi mavairasi monga chickenpox kapena chimfine, makamaka ngati aspirin yagwiritsidwa ntchito. Chifukwa cha chiopsezo ichi, musapatse ana kapena achinyamata aspirin pokhapokha ngati dokotala wakuuzani mwachindunji.
Matenda a Urea Cycle (UCDs)
Monga tanenera, kayendedwe ka urea ndi njira yosinthira ammonia woopsa kukhala urea. Njirayi imaphatikizapo masitepe angapo, iliyonse imafuna enzyme inayake, kuphatikizapo:
- N-acetyl-glutamate synthase (NAGS)
- Carbamoyl phosphate synthetase (CPS)
- Ornithine transcarbamylase (OTC)
- Argininosuccinate synthetase (AS)
- Argininosuccinic acid lyase (ASL)
- Arginase (ARG1)
Ngati ma enzyme ena aliwonsewa akusowa, kayendedwe kake kamalephera, ndipo ammonia imasonkhana. Matendawa a majini, omwe amadziwika kuti UCDs, angayambitse hyperammonemia yoopsa komanso yosatha.
Ma UCD amapezeka kuyambira kubadwa. Makanda obadwa kumene omwe ali ndi vuto la kusowa kwa ma enzyme nthawi zambiri amakhala ndi hyperammonemia mkati mwa maola 24 mpaka 72 kuchokera kubadwa. Ngati kusowako kuli kochepa kapena kochepa, zizindikiro zimatha kuyamba mtsogolo chifukwa cha matenda kapena nkhawa. Ma UCD ndi omwe amachititsa 23% ya milandu ya hyperammonemia mwa ana odwala kwambiri.
Zina Zoyambitsa
Kupatula zinthu zomwe zatchulidwa pamwambapa, palinso zifukwa zina zingapo zomwe zingachitike:
- Kulephera kwa impso (impso): Impso zanu zikalephera kugwira ntchito, sizingathe kusefa urea m'thupi lanu. Izi zimapangitsa kuti ammonia ichuluke m'magazi mwanu.
- Matenda ena a magazi: Matenda monga multiple myeloma ndi acute leukemia angayambitsenso hyperammonemia.
- Matenda ena: Matenda oyambitsidwa ndi tizilombo toyambitsa matenda tomwe timatulutsa urease—monga Proteus mirabilis , Escherichia coli (E. coli) , ndi Klebsiella —angayambitse hyperammonemia yoopsa. Izi zimachitika makamaka kwa ana obadwa ndi vuto la mkodzo kapena kwa akuluakulu omwe ali ndi vuto la mkodzo chifukwa cha matenda a UTI.
Kodi zizindikiro za Hyperammonemia ndi ziti?
Zizindikiro zimatha kusiyana kwambiri kutengera zaka zanu komanso kuopsa kwa matenda anu.
Zizindikiro zofala kwambiri mwa ana ndi akuluakulu
- Kusanza ndi kusanza
- Kupweteka m'mimba
- Kukwiya
- Mutu
- Kuvuta ndi kulinganiza, kugwirizana, kapena kulankhula (ataxia)
- Kusintha kwa khalidwe
Zizindikiro zina zofatsa zomwe zimachitikira ana
- Kulephera kukula bwino
- Kufooka kwa minofu (hypotonia)
- Kuchedwa kwa chitukuko cha mitsempha
Zizindikiro zoopsa mwa ana ndi akuluakulu
Izi ndi zizindikiro zazikulu zochenjeza. Ngati mukukumana ndi chilichonse mwa izi, pitani kuchipatala nthawi yomweyo.
- Kusokonezeka ndi kusokonezeka maganizo
- Kusintha kwa maganizo
- Kugona tulo topitirira muyeso
- Kusintha kwa chidziwitso
- Kugwidwa ndi khunyu
- Kupuma mofulumira (kupuma mofulumira)
- Chikomokere
Chenjezo: Ngati inu kapena wokondedwa wanu mukukumana ndi zizindikiro izi, chonde imbani 911 nthawi yomweyo kapena pitani ku dipatimenti yapafupi yazadzidzidzi. Ammonia wambiri m'magazi ndi woopsa ndipo amafunika thandizo lachipatala mwachangu.
Zizindikiro mwa makanda obadwa kumene
Mwa makanda obadwa kumene, hyperammonemia nthawi zambiri imapezeka pakati pa maola 24 ndi 72 atabadwa:
- Kukwiya ndi kulira mopitirira muyeso
- Kusanza
- Kutopa
- Kugwidwa ndi khunyu
- Kung'ung'udza pamene mukupuma
- Kupuma movutikira
Chofunika: Ngati mwana wanu akuwonetsa zizindikiro izi mukadali kuchipatala, dziwitsani dokotala wanu nthawi yomweyo. Ngati muli kunyumba, imbani 911 kapena pitani ku chipinda chapafupi chadzidzidzi nthawi yomweyo. Musazengereze kufunafuna chithandizo.
Kodi gulu lachipatala la Nirogi Lanka limazindikira bwanji izi?
Madokotala nthawi zambiri amakayikira hyperammonemia poyang'ana kuchuluka kwa ammonia m'magazi mwanu.
- Kwa makanda osakwana mwezi umodzi: Kuchuluka kwa madzi m'thupi kopitirira 80 micromol/L n'kodetsa nkhawa.
- Kwa ana okulirapo: Mlingo woposa 55 micromol/L ndi wodetsa nkhawa.
- Kwa akuluakulu: Kuchuluka kwa shuga m'magazi kuposa 30 micromol/L ndi kodetsa nkhawa.
Micromol/L ndi muyezo womwe umagwiritsidwa ntchito poyesa kuchuluka kwa ammonia m'magazi anu.
Mayeso ozindikira matenda
Chida chachikulu chodziwira matenda ndi kuyeza magazi kuti aone kuchuluka kwa ammonia.
Ngati kuchuluka kwa magazi kwatsimikizika, dokotala wanu angakupatseni mayeso ena kuti adziwe chomwe chimayambitsa, monga mayeso a chiwindi , mayeso a impso , kapena kuyesa mkodzo .
Popeza hyperammonemia ingawononge dongosolo la mitsempha ndipo ikhoza kupha moyo, dokotala wanu angakulangizeninso mayeso ojambulira zithunzi monga CT (computed tomography) scan kapena MRI (magnetic resonance imaging) kuti aone ngati pali zovuta za mitsempha.
Kodi mankhwala a Hyperammonemia ndi ati?
Chithandizo chimadalira kwathunthu chomwe chimayambitsa vuto—kaya ndi matenda a chiwindi mwa munthu wamkulu amene ali ndi matenda a chiwindi, kapena matenda a urea cycle disorder (UCD) mwa mwana wakhanda.
Pankhani ya hyperammonemia yoopsa, cholinga chake ndi kuchepetsa kuchuluka kwa ammonia m'magazi mwachangu ndikuthana ndi mavuto monga kutupa kwa ubongo (kutupa kwa ubongo) ndi kuthamanga kwa magazi mkati mwa mutu.
- Kwa makanda obadwa kumene, madokotala amasiya kudya mapuloteni (popeza kugaya mapuloteni kumapanga ammonia) ndipo amapereka ma calories kudzera mu njira zothetsera shuga. Hemodialysis ingagwiritsidwe ntchito kuyeretsa magazi pogwiritsa ntchito makina apadera ndi fyuluta (yomwe imagwira ntchito ngati impso yochita kupanga).
- Kwa iwo omwe ali ndi vuto la kuchepa kwa ma enzyme a UCD pang'ono chifukwa cha kupsinjika, kudya mapuloteni kumakhala kochepa pomwe shuga imapereka chithandizo cha caloric. Kuyeretsa magazi kumachitika pokhapokha ngati chithandizo choyamba sichikuchepetsa kuchuluka kwa ammonia mkati mwa maola ochepa.
- Pofuna kuchiza matenda a chiwindi, cholinga chachikulu ndi kuchepetsa kupanga ammonia m'matumbo. Mankhwala oyamba ndi monga mankhwala akumwa okhala ndi lactulose kapena lactitol , omwe amathandiza kuti thupi lisayamwe ammonia.
Kwa iwo omwe ali ndi vuto la urea cycle, madokotala angapereke zakudya zinazake zowonjezera kuti asabwererenso, kuphatikizapo:
- L-carnitine
- L-ornithine-L-aspartate
- Arginine
Kodi vutoli lingapewedwe?
Popeza hyperammonemia imayambitsa zinthu zosiyanasiyana, sizingatheke nthawi zonse kupewa. Komabe, mutha kuchitapo kanthu kuti muteteze chiwindi chanu komanso thanzi lanu lonse:
- Pewani kapena chepetsani kumwa mowa.
- Chepetsani kudya mafuta a trans ndi madzi a chimanga okhala ndi fructose yambiri.
- Samalani ndi mankhwala omwe amaperekedwa ndi dokotala komanso omwe amaperekedwa ndi dokotala kuti mupewe kupsinjika kwa chiwindi.
- Pitirizani kuchita masewera olimbitsa thupi nthawi zonse.
- Chepetsani kudya nyama yofiira.
Pofuna kupewa matenda a Reye mwa ana ndi achinyamata—vuto lomwe lingayambitse hyperammonemia —musapereke aspirin ngati pali zizindikiro za kachilombo (monga malungo kapena nthenda ya nkhuku) pokhapokha ngati dokotala wakuuzani momveka bwino.
Matenda a Urea Cycle (UCD) ndi osowa, koma mayeso a majini alipo kuti adziwe ngati muli ndi majini omwe amayambitsa matendawa. Chonde funsani dokotala wanu kuti mukambirane ngati kuyezetsa uku ndikoyenera kwa inu.
Kodi chiyembekezo cha matendawa ndi chiyani?
Kuyezetsa kwa hyperammonemia—kapena chiyembekezo chanu chochira—kumadalira zinthu zingapo zofunika:
- Kuopsa kwa kuchuluka kwa ammonia m'thupi lanu.
- Nthawi yomwe kuchuluka kwa ammonia m'magazi anu kwakhalabe kokwera.
- Chifukwa chachikulu cha matenda a hyperammonemia.
Kawirikawiri, nthawi yoti munthu ayambe kulandira chithandizo cha hyperammonemia ikhoza kutetezedwa. Ngati chithandizo chachedwa kapena sichiperekedwa, kutupa kwa ubongo kosatha kungayambitse imfa zambiri. Mwatsoka, mwa makanda obadwa kumene omwe ali ndi hyperammonemia yayikulu, ngakhale kuchitapo kanthu mwachangu komanso mwachangu sikutsimikizira zotsatira zabwino nthawi zonse.
Ku United States, odwala omwe ali ndi matenda a UCD omwe ali ndi vuto la hyperammonemia ali aang'ono amakhala ndi moyo wa pafupifupi 35%. Kwa iwo omwe ali ndi vuto la hyperammonemia akakula, mwayi wopulumuka uwu umakwera kufika pafupifupi 87%.
Kwa anthu omwe ali ndi vuto lalikulu la chiwindi chifukwa cha hyperammonemia, kuchuluka kwa anthu omwe apulumuka chaka chimodzi ndi zitatu kwanenedwa kuti ndi 42% ndi 23% motsatana.
Kodi mavuto omwe angakhalepo chifukwa cha hyperammonemia ndi ati?
Ngati vutoli silichiritsidwa mwachangu, kuchuluka kwa ammonia kungawononge dongosolo la mitsempha, zomwe zingayambitse mavuto omwe angawopseze moyo, kuphatikizapo:
- Kutupa kwa ubongo (cerebral edema)
- Kuthamanga kwa magazi m'mutu (kuwonjezeka kwa kuthamanga kwa magazi m'chigaza)
- Kutuluka kwa ubongo m'mimba - nthawi zambiri kumachitika chifukwa cha kutupa kwambiri kwa ubongo ndi kupanikizika kwakukulu.
- Chikomokere
- Imfa
Ngati muli ndi vuto la hyperammonemia yofatsa, mungakhale pachiwopsezo cha kulephera kwa nzeru kwa nthawi yayitali komanso mavuto osatha a khalidwe kapena amisala.
Kodi muyenera kupita kwa dokotala liti?
Ngati inu kapena wokondedwa wanu mukusonyeza zizindikiro za hyperammonemia, imbani 911 kapena pitani ku dipatimenti yadzidzidzi yapafupi nthawi yomweyo. Musazengereze kufunafuna chithandizo.
Ngati muli ndi matenda a chiwindi kapena kusowa pang'ono kwa ma enzymes a urea cycle, ndikofunikira kuti mupitirize kuyendera dokotala nthawi zonse kuti muthane ndi vuto lanu ndikuwunika bwino kuchuluka kwa ammonia m'thupi lanu.
Hyperammonemia ndi matenda oopsa omwe amayambitsa zifukwa zosiyanasiyana. Ngati inu, mwana wanu, kapena khanda likuwonetsa zizindikiro, funani thandizo lachipatala nthawi yomweyo. Ngati muli ndi matenda a chiwindi kapena mbiri ya banja lanu ya matenda a UCD, kambiranani ndi dokotala wanu za zinthu zomwe zingakubweretsereni chiopsezo. Nirogi Lanka ali pano kuti akuthandizeni paulendo wanu wathanzi.
Maganizo omaliza oti mukumbukire
Mwachidule, nazi mfundo zofunika kwambiri zomwe muyenera kukumbukira:
- Hyperammonemia imachitika pamene kuchuluka kwa ammonia m'magazi kukukwera kufika pamlingo woopsa, zomwe zimakhala zoopsa kwambiri ku ubongo wanu ndi dongosolo lanu la mitsempha.
- Zili ndi zifukwa zambiri zomwe zingayambitse vutoli, ndipo vuto la chiwindi ndi matenda a Urea Cycle Disorders (UCDs) ndizomwe zimayambitsa vutoli.
- Zizindikiro zimasiyana malinga ndi zaka ndi kuopsa kwake, kuyambira pamavuto ofooka monga nseru, kusanza, ndi mutu mpaka ku zizindikiro zoopsa monga chisokonezo, khunyu, ndi chikomokere. Nthawi zonse khalani maso ndi zizindikiro za makanda obadwa kumene.
- Ngati pali zizindikiro zoopsa, pitani kuchipatala nthawi yomweyo. Izi zitha kukhala vuto la moyo kapena imfa.
- Chithandizo chimapangidwira mogwirizana ndi chomwe chimayambitsa vutoli. Kuthandizira mwachangu kumayang'ana kwambiri kuchepetsa kuchuluka kwa ammonia ndikuwongolera zovuta zake.
- Ngakhale zifukwa zina sizingapewedwe, kukhala ndi moyo wathanzi komanso kuyang'anira thanzi la chiwindi chanu kungathandize kuchepetsa zoopsa zina.
- Ngati mukudwala matenda a chiwindi kapena muli ndi mbiri ya banja lanu ya matenda a UCD, kuyezetsa magazi nthawi zonse n'kofunika.
Kumbukirani, mumadziwa bwino thanzi lanu. Ngati muwona kusintha kulikonse kwachilendo kapena zizindikiro, musazengereze kukaonana ndi dokotala. Tili pano kuti tikuthandizeni.
Hyperammonemia, ammonia, chiwindi, kuzungulira kwa urea, ubongo, zizindikiro, chithandizo
