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Menene Hyperammonemia?
A taƙaice dai, yawan sinadarin ammonia a cikin jinin mutum yana ƙaruwa sosai. Ammonia, ko NH3, wani abu ne da ake samarwa a cikin hanjinmu lokacin da muke narkar da sunadaran da muke ci. Yawanci, wannan sinadarin ammonia yana shiga cikin hanta kuma ana sarrafa shi a can. Muna kiran wannan tsari da tsarin urea . Ta wannan tsarin, ammonia yana canzawa zuwa wani abu da ba shi da illa da ake kira urea kuma yana fita a cikin fitsari. Duk da haka, idan wani abu ya faru da wannan tsarin urea, ko kuma idan hanta ba ta iya sarrafa wannan sinadarin ammonia yadda ya kamata ba, ammonia yana fara taruwa a cikin jini. Wannan ƙaruwar ammonia a cikin jini yana da guba sosai ga Tsarin Jijiyoyi na Tsakiya (CNS) , wanda shine kwakwalwa da kashin baya. Shi ya sa hyperammonemia yanayi ne da ke buƙatar kulawar likita nan take kuma yana iya zama barazana ga rayuwa .Wa zai iya haɓaka wannan?
Wannan yanayi, wanda ake kira hyperammonemia, zai iya shafar jarirai, yara ƙanana, da manya.- A tsakanin manya, kusan kashi 90% na waɗanda ke fama da wannan yanayin su ne waɗanda ke fama da cutar cirrhosis na hanta .
- Jarirai jarirai na iya kamuwa da wannan saboda an haife su da lahani a cikin zagayowar urea (cutar zagayowar urea ta haihuwa) . Waɗannan yanayi ne na kwayoyin halitta.
- Haka kuma, gazawar hanta mai tsanani da cututtukan da suka gada na iya haifar da hauhawar ammonemia a cikin ƙananan yara.
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Check Availability →Mene ne ke haifar da hyperammonemia?
Akwai dalilai da dama da ke haifar da wannan. Waɗannan dalilai na iya canzawa da shekaru. Amma gabaɗaya, manyan dalilai guda biyu sune matsalolin hanta da kuma matsalolin zagayowar urea (UCDs) .Saboda matsalolin hanta
Hantarmu ita ce babbar gaɓar da ke canza ammonia zuwa urea. Don haka idan hanta ta lalace ko kuma ta kamu da rashin lafiya, ba za ta iya sarrafa ammonia yadda ya kamata ba. Wannan shine lokacin da ammonia ke taruwa a cikin jini. Ga wasu cututtuka da suka shafi hanta waɗanda za su iya haifar da hyperammonemia:- Cutar Hanta : Idan hanta ta lalace , ikon sarrafa ammonia yana da iyaka. Wannan ya fi yawa a cikin cututtukan hanta masu tsanani, amma har ma a cikin mutanen da ke da cutar hanta mai ɗorewa, matakan ammonia wani lokacin na iya ƙaruwa kwatsam, misali, idan wani abu ya faru a cikin ciki ko hanji ( zubar da jini a cikin hanji) ko kuma idan akwai rashin daidaiton electrolyte . Wannan shine babban dalilin yawan ammonia a cikin manya da yara.
- Ciwon hanta: Wannan yana faruwa ne lokacin da hanta ta lalace kuma ba ta iya sarrafa ammonia yadda ya kamata ba. Ammonia yana taruwa a cikin jini kuma yana tafiya zuwa kwakwalwa. Wannan na iya haifar da rudani, rashin fahimta , har ma da suma. Wani lokaci yana iya zama mai kisa.
- Ciwon hanta: Ciwon hanta shine maye gurbin kyallen hanta mai lafiya da tabo. Wannan na iya sa hanta ta kasa aiki yadda ya kamata kuma yana iya haifar da hyperammonemia. Ciwon hanta cuta ce ta hanta mai ɗorewa.
- Matsalar hanta mai tsanani: Wannan shine lokacin da hanta ta daina aiki ba zato ba tsammani. Ana iya haifar da shi ta hanyar cututtuka da yanayi daban-daban. Misali, raunin hanta da miyagun ƙwayoyi ke haifarwa sakamakon wasu magunguna, kamar acetaminophen , shine ke haifar da kashi 50% na dukkan lamuran gazawar hanta mai tsanani a Amurka. Yana haifar da kashi 64% na lokuta na hyperammonemia a cikin yara.
- Raguwar kwararar jini zuwa hanta: Idan jini bai kwarara yadda ya kamata zuwa hanta ba, jiki ba zai iya aika sinadarin ammonia zuwa hanta don a sarrafa shi ba. Ko da a lokacin, sinadarin ammonia yana taruwa a cikin jini.
- Ciwon Reye:Wannan yanayi ne da ba kasafai ake samunsa ba. Yana shafar jini, kwakwalwa, da hanta. Yawanci yana haifar da hauhawar matakan ammonia a cikin jini da ƙarancin glucose a cikin jini. Ya fi shafar yara ƙanana da matasa waɗanda ke murmurewa daga kamuwa da cututtukan ƙwayoyin cuta kamar su chickenpox ko mura/mura kuma waɗanda suka sha aspirin don waɗannan alamun. Ba a san ainihin dalilin cutar Reye ba. Duk da haka, saboda wannan haɗarin, bai kamata a ba wa yara ƙanana da matasa aspirin ba sai dai idan likita ya ba da shawarar musamman.
Sakamakon Matsalolin Zagayen Urea (UCDs)
Kamar yadda muka tattauna a baya, zagayowar urea shine tsarin da ake canza gubar ammonia zuwa urea sannan a fitar da ita a cikin fitsari. Wannan zagayowar tana da matakai da dama, kowannensu yana buƙatar nau'in enzyme daban-daban. Waɗannan enzymes sune:- N-acetyl-glutamate synthase (NAGS)
- Carbamoyl phosphate synthetase (CPS)
- Ornithine transcarbamylase (OTC)
- Sintetase na Argininosuccinate (AS)
- Argininosuccinic acid lyase (ASL)
- Arginase (ARG1)
Wasu dalilai
Bugu da ƙari, akwai wasu dalilai da yawa:- Matsalar koda ): Idan kodan ba za su iya fitar da urea yadda ya kamata ba saboda matsalar koda, zai iya sa ammonia ta taruwa a cikin jini.
- Wasu matsalolin jini: Hyperammonemia na iya faruwa ne sakamakon cututtukan jini kamar su multiple myeloma da cutar sankarar jini mai tsanani.
- Wasu cututtuka: ƙwayoyin cuta masu samar da urease kamar Proteus mirabilis , Escherichia coli (E. coli) da KlebsiellaCututtukan ƙwayoyin cuta kamar streptococci, musamman a cikin yara masu lahani a cikin fitsari da kuma tsofaffi waɗanda ke riƙe da fitsari saboda kamuwa da cututtukan fitsari (UTIs), na iya haifar da mummunan hyperammonemia.
Mene ne alamun cutar Hyperammonemia?
Alamomin na iya bambanta sosai dangane da yadda yanayin yake da sauƙi ko tsanani, da kuma shekarun da yake tasowa.Alamomi masu sauƙi da suka zama ruwan dare ga yara da manya
- Tashin zuciya da amai
- Ciwon ciki
- Yawan fushi
- Ciwon kai (ciwon kai)
- Wahala wajen daidaito, daidaitawa, da magana (ataxia)
- Canje-canje a Halayya
Ƙarin alamun rashin sauƙi waɗanda ke shafar yara kawai
- Rashin bunƙasa
- Raunin tsoka (hypotonia)
- Jinkirin ci gaban jijiyoyi
Alamomi masu tsanani a cikin yara da manya
Waɗannan alamu ne masu matuƙar haɗari. Idan kana da ɗaya daga cikin waɗannan, ya kamata ka nemi shawarar likita nan da nan.- Ruɗani da rashin fahimta
- Sauyin yanayi
- Barci mai yawa
- Canje-canje a cikin sani
- Kamawa
- Numfashi mai sauri (Hyperventilation)
- Komawa
Gargaɗi: Idan kai ko wani na kusa da kai yana da waɗannan alamun, da fatan za a kira 911 nan da nan, ko kuma a je sashen gaggawa na asibiti mafi kusa da kai. Yawan sinadarin ammonia a cikin jini yana barazana ga rayuwa kuma yana buƙatar kulawar likita nan take!
Alamomin cutar a jarirai
Idan jariri ya kamu da cutar hyperammonemia, waɗannan alamun yawanci suna bayyana cikin awanni 24 zuwa 72 na haihuwa:- Yawan fushi, yawan kuka
- Amai
- Baƙin ciki
- Kamawa
- Guraren murya lokacin numfashi
- Numfashi mai sauri (Hyperventilation)
Muhimmi: Idan jaririnka ya nuna waɗannan alamun, kira likita nan take idan har yanzu kana asibiti. Idan kana gida, kira 911 nan take, ko kuma ka kai su asibiti mafi kusa. Wannan ba abu ne da za a jinkirta ba.
Ta yaya likitoci ke gano wannan?
Likitoci yawanci suna zargin hyperammonemia ta hanyar gwada matakan ammonia a cikin jini.- Ga jarirai har zuwa wata ɗaya, idan matakin ammonia a cikin jini ya fi micromol/lita 80 (micromol/L).
- Ga yara manya, idan ya fi micromol/lita 55 (55 micromol/L).
- A cikin manya, idan ya fi micromol/lita 30 (micromol/L).
Wadanne gwaje-gwaje ake yi don gano wannan?
Babban gwajin shine gwajin jini wanda ke auna matakin ammonia a cikin jininka. Idan matakin ammonia ɗinka ya yi yawa, likitanka zai iya yin wasu gwaje-gwaje don gano musabbabin. Misali, gwaje-gwajen jini don duba aikin hanta da aikin koda , da kuma gwajin fitsari . Saboda yawan ammonia na iya lalata tsarin jijiyoyi na tsakiya kuma yana iya zama barazana ga rayuwa, likitanka zai iya yin gwaje-gwajen hoto, kamar CT (computed tomography) scan ko MRI (magnetic resonance imaging), don duba matsalolin da ke shafar kwakwalwa.Menene hanyoyin magance hyperammonemia?
Maganin ya dogara ne da dalilin. Misali, ga babba, yana faruwa ne saboda cutar hanta ko cutar hanta, ko kuma ga jariri, yana faruwa ne saboda matsalar zagayowar urea (UCD). A cikin tsananin hyperammonemia, babban burin shine a rage yawan ammonia a cikin jini da kuma magance wasu matsaloli kamar kumburin kwakwalwa da kuma ƙaruwar matsin lamba a kusa da kwakwalwa (hawan jini a cikin kwakwalwa).- Idan jariri ya kamu da cutar hyperammonemia, likitoci za su daina ba shi furotin (saboda ammonia ana samar da shi ne lokacin da aka narkar da furotin) maimakon haka sai su ba shi glucose, wani maganin sukari, don samar da adadin kuzari. Haka kuma za su yi amfani da wata hanya da ake kira hemodialysis . Wannan ya ƙunshi tsaftace jinin jariri da kuma cire ammonia ta hanyar injin dialysis da kuma matattarar musamman (kamar koda ta wucin gadi).
- Mutanen da ke da ƙarancin sinadarin enzyme na urea cycle (UCD) waɗanda ke da hyperammonemia saboda damuwa, kamar hyperammonemia da damuwa ke haifarwa, ana kuma ba su damar cire furotin da glucose a matsayin adadin kuzari. Ana yin aikin hemodialysis ne kawai idan matakan ammonia ɗinsu ba su ragu ba cikin 'yan awanni bayan fara magani.
- Maganin cutar hanta (hepatic encephalopathy) shine rage samar da ammonia a cikin hanji. Maganin farko shine magungunan baki da ke ɗauke da lactulose da lactitol . Waɗannan sukari suna rage samarwa da shayewar ammonia a cikin hanji.
- L-carnitine
- L-ornithine-L-aspartate
- Arginine
Akwai hanyoyin da za a kauce wa wannan yanayin?
Saboda akwai dalilai da yawa da ke haifar da hyperammonemia, ba dukkan yanayi za a iya hana su ba. Duk da haka, za ku iya ɗaukar matakai don hana wasu nau'ikan cututtukan hanta, musamman waɗanda suka shafi abincinku da salon rayuwarku. Idan kuna cikin haɗarin kamuwa da cutar hanta, likitanku zai iya ba da shawarar yin waɗannan canje-canjen salon rayuwa:- Babu laifi a guji ko iyakance shan barasa.
- A guji abinci da abin sha da ke ɗauke da kitsen trans ko kuma syrup masara mai yawan fructose.
- Domin gujewa lalacewar hanta, a yi taka tsantsan sosai lokacin amfani da magungunan da likita ya rubuta da kuma waɗanda ba a rubuta ba.
- Motsa jiki akai-akai .
- Rage cin jan nama.
Menene hasashen wannan yanayin?
Hasashen hyperammonemia, wato, ikon murmurewa, ya dogara da dalilai da yawa:- Yaya girman matakan ammonia ɗinku yake.
- Tsawon lokacin da matakin ammonia ya karu.
- Me ke haifar da hyperammonemia?
Mene ne yiwuwar rikitarwa na hyperammonemia?
Idan ba a yi maganin wannan matsalar cikin gaggawa ba, yawan sinadarin ammonia zai iya lalata tsarin jijiyoyi na tsakiya kuma ya haifar da matsaloli masu barazana ga rayuwa. Irin waɗannan matsalolin sun haɗa da:- Ciwon kwakwalwa
- Ƙara matsin lamba a kusa da kwakwalwa (hauhawar jini a cikin kwakwalwa)
- Hawan kwakwalwa - Wannan galibi yana faruwa ne sakamakon kumburin kwakwalwa da kuma ƙaruwar matsin lamba a kusa da kwakwalwa.
- Komawa
- Mutuwa
Yaushe ya kamata mu ga likita?
Idan kai ko wani da kake damuwa da shi yana da alamun hyperammonemia, kira 911 nan da nan ko kuma ka je asibiti mafi kusa. Wannan ba abu ne da za a jinkirta ba. Idan kana da cutar hanta ko kuma rashin isasshen sinadarin urea cycle, yana da matukar muhimmanci ka ga likitanka akai-akai don kula da lafiyarka da kuma sa ido kan matakan ammoniya.Hyperammonemia wata cuta ce mai tsanani wadda abubuwa da dama ke iya haifarwa. Idan kai, ɗanka, ko jaririnka kuna da waɗannan alamun, nemi taimako nan da nan. Idan kana da cutar hanta, ko kuma idan wani a cikin iyalinka yana da matsalar urea cycle (UCDs), yi magana da likitanka game da haɗarin kamuwa da hyperammonemia. Suna nan don taimaka maka.
A ƙarshe, ku tuna da wannan.
To, ga wasu daga cikin muhimman abubuwan da ya kamata ku tuna daga abin da muka tattauna:- Hyperammonemia wani nau'in ammonia ne mai matuƙar haɗari a cikin jini. Wannan yana da matuƙar illa ga kwakwalwarmu da tsarin jijiyoyi.
- Akwai dalilai da dama da ke haifar da hakan. Matsalolin hanta da kuma matsalolin zagayowar urea (UCDs) su ne manyan abubuwan da ke haifar da hakan.
- Alamomin sun bambanta dangane da tsananin yanayin da shekaru. Suna iya kamawa daga ƙananan alamu kamar tashin zuciya, amai, da ciwon kai zuwa manyan alamu kamar ruɗani, farfadiya, da suma. Ya kamata jarirai su ma su damu da alamun su.
- Idan kana da alamun cutar mai tsanani, ya kamata ka nemi shawarar likita ba tare da ɓata lokaci ba. Wannan na iya zama yanayi na rai ko mutuwa.
- Maganin ya dogara ne da musabbabin. Maganin gaggawa zai iya rage yawan sinadarin ammonia da kuma magance matsalolin da ke tattare da shi.
- Duk da cewa ba za a iya hana wasu dalilai ba, wasu haɗari za a iya rage su ta hanyar kiyaye lafiyayyen salon rayuwa da kuma kula da hanta .
- Ko kana da cutar hanta ko wani a cikin iyalinka yana da UCDs, yana da muhimmanci a yi gwajin lafiya akai-akai.
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