Ese ufite ammonia nyinshi mu maraso yawe? Reka twige ku kibazo cy’akaga cya hyperammonemia!

Hari ibintu byinshi biba mu mibiri yacu, sibyo? Hari igihe, iyo ibi bikorwa birangiye gato, hashobora kuvuka ibibazo bikomeye. Urugero, hyperammonemia ni indwara ikomeye ibaho iyo imyanda yitwa ammonia yiyongera mu maraso yacu. Ni ngombwa cyane kubimenya kuko bishobora kugira ingaruka zikomeye ku buzima bwawe iyo bitafashwe neza.

Hyperammonemiya ni iki?

Muri make, hyperammonemia ni igihe urugero rwa ammonia mu mara yawe ruri hejuru cyane. Ammonia, cyangwa NH3, ni imyanda ikorerwa mu mara yacu iyo turya poroteyine. Ubusanzwe, iyi ammonia ijya mu mwijima wacu igatunganyirizwa aho ngaho. Iyi nzira tuyita uruhererekane rwa urea . Muri uru ruhererekane, ammonia ihinduka ikintu kitangiza cyane cyitwa urea hanyuma igasohorwa mu nkari. Ariko, iyo hari ikintu kitagenda neza muri uru ruhererekane rwa urea, cyangwa niba umwijima udashobora gutunganya iyi ammonia neza, ammonia itangira kwirundanya mu maraso. Uku kwiyongera kwa ammonia mu maraso ni uburozi bukomeye kuri sisitemu yacu y'imitsi yo hagati (CNS) , ari yo ubwonko n'umugongo. Niyo mpamvu hyperammonemia ari indwara isaba ubuvuzi bwihuse kandi ishobora no gushyira ubuzima mu kaga .

Ni nde ushobora guteza imbere ibi kurusha abandi?

Iyi ndwara, yitwa hyperammonemia, ishobora kwibasira abana bavutse, abana bato, ndetse n'abantu bakuru.

• Mu bantu bakuru, hafi 90% by'abarwaye iyi ndwara ni abafite cirrhosis y'umwijima .
• Abana bavutse bashobora kubyibasira kuko bavukana inenge mu mikorere ya urea (congenital urea cycle disorder) . Izi ni indwara zikomoka ku ngirabuzimafatizo.
• Nanone, umwijima unanirwa cyane ndetse n'indwara z'imikorere y'umubiri zikomoka ku bana bato zishobora gutera hyperammonemia.

Biragoye kuvuga neza uburyo ibi bikunze kugaragara, kuko bishobora guterwa n’ibintu bitandukanye. Ariko abashakashatsi bavuga ko indwara za urea cycle, imwe mu mpamvu, zigira ingaruka ku mwana umwe mu 250.000 wavukiye muri Leta Zunze Ubumwe za Amerika n’umwana umwe mu 440.000 wavukiye ku rwego mpuzamahanga.

Ni izihe mpamvu zitera hyperammonemia?

Hari impamvu nyinshi zishobora gutera ibi. Izi mpamvu zishobora guhinduka uko imyaka igenda ikura. Ariko muri rusange, impamvu ebyiri z'ingenzi ni ibibazo by'umwijima n'indwara za UCD (UCDs) .

Bitewe n'ibibazo by'umwijima

Umwijima wacu ni wo mubiri w’ingenzi uhindura ammonia mo urea. Rero iyo umwijima wangiritse cyangwa urwaye, ntushobora gutunganya ammonia neza. Iki ni cyo gihe ammonia yirundanyije mu maraso. Dore zimwe mu ndwara ziterwa n’umwijima zishobora gutera hyperammonemia:

• Indwara y'umwijima : Iyo umwijima wangiritse , ubushobozi bwawo bwo gutunganya ammonia buragabanuka. Ibi bikunze kugaragara cyane mu ndwara zikomeye z'umwijima, ariko no ku bantu bafite indwara y'umwijima idahindagurika, urugero rwa ammonia rimwe na rimwe rushobora kwiyongera mu buryo butunguranye, urugero, iyo habaye ikintu mu gifu cyangwa mu mara ( kuva amaraso mu nda) cyangwa niba hari electrolyte imbalance . Iyi ni yo mpamvu nyamukuru ituma ammonia izamuka cyane mu bantu bakuru n'abana.
• Indwara y'umwijima: Ibi bibaho iyo umwijima wangiritse kandi udashobora gutunganya ammonia neza. Ammonia yiyongera mu maraso ikagera mu bwonko. Ibi bishobora gutera urujijo, kujijinganya , ndetse no kuri koma. Rimwe na rimwe bishobora kwica.
• Kurwara umwijima: Kurwara umwijima ni ugusimbuza ingirangingo z'umwijima zizima n'inkovu. Ibi bishobora gutuma umwijima udakora neza kandi bigatera hyperammonemia. Kurwara umwijima ni indwara idakira y'umwijima.
• Kunanirwa k'umwijima gukabije: Iki ni cyo gihe umwijima uhagarara gukora mu buryo butunguranye. Bishobora guterwa n'indwara zitandukanye n'ibindi bibazo. Urugero, kwangirika k'umwijima guterwa n'imiti biterwa n'imiti imwe n'imwe, nka acetaminophen , bingana na 50% by'indwara zose z'umwijima gukabije muri Leta Zunze Ubumwe za Amerika. Bingana na 64% by'indwara za hyperammonemia mu bana.
• Kugabanuka kw'amaraso ajya mu mwijima: Iyo amaraso adatembera neza mu mwijima, umubiri ntushobora kohereza ammonia mu mwijima ngo itunganywe. Nubwo bimeze bityo, ammonia irakomeza kwiyongera mu maraso.
• Indwara ya Reye:Iyi ni indwara ikunze kugaragara cyane. Igira ingaruka ku maraso, ubwonko, n'umwijima. Akenshi itera urugero rwinshi rwa ammonia mu maraso n'urugero ruto rw'isukari mu maraso. Ikunze kwibasira abana bato n'ingimbi barimo gukira indwara zandura nka grippe cyangwa grippe/grippe kandi bafashe aspirine kubera izo ndwara. Impamvu nyayo ya Reye's syndrome ntabwo izwi. Ariko, kubera iyi ngaruka, aspirine ntigomba guhabwa abana bato n'ingimbi keretse muganga abisabye by'umwihariko.

Bitewe n'indwara zo mu bwoko bwa Urea Cycle Disorders (UCDs)

Nkuko twabiganiriyeho mbere, uruhererekane rwa urea ni inzira aho amoniya irimo uburozi ihinduka urea hanyuma igasohorwa mu nkari. Uru ruhererekane rufite intambwe nyinshi, buri imwe muri zo ikaba isaba ubwoko butandukanye bwa enzyme. Izi enzyme ni:

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

Iyo imwe muri izi enzymes idafite ubushobozi bwo gukora neza, uruhererekane rw'amaraso rudakora neza. Ammonia yiyongera mu maraso. Ubu bwoko bw'ibura ry'imyunyu ngugu bwitwa indwara ya urea cycle disorder (UCD). UCDs zishobora gutera hyperammonemia, ishobora kuba itunguranye cyangwa ihoraho. UCDs ni indwara zivuka. Abana bavutse bafite ikibazo cyo kubura burundu enzyme ya urea cycle bakunze kugira hyperammonemia ikabije mu masaha 24 kugeza kuri 72 nyuma yo kuvuka. Ibura ry'imyunyu ngugu ryaba rito cyangwa rito, amonia ishobora kubaho igihe icyo ari cyo cyose mu buzima, mu gihe cy'uburwayi, stress, cyangwa ibindi bibazo bibabaza. Izi ndwara za UCDs zigize 23% by'abantu barwaye hyperammonemia ikabije mu bana bato barembye cyane.

Izindi mpamvu

Uretse ibi, hashobora kuba hari izindi mpamvu nyinshi:

• Kunanirwa kw'impyiko (impyiko): Iyo impyiko zidashobora gusohora urea neza bitewe n'uko impyiko zinaniwe, bishobora gutuma ammonia yirundanya mu maraso.
• Indwara zimwe na zimwe z'amaraso: Hyperammonemia ishobora no guterwa n'indwara z'amaraso nka multiple myeloma na acute leukemia.
• Utunyangingo tumwe na tumwe: udukoko dutera urease nka Proteus mirabilis , Escherichia coli (E. coli) na KlebsiellaIndwara za bagiteri nka streptococci, cyane cyane ku bana bavukanye ibibazo byo mu nzira y'inkari ndetse no ku bantu bakuru bafite ibibazo byo kugumana inkari bitewe n'indwara zo mu nzira y'inkari (UTIs), zishobora gutera hyperammonemia ikomeye.

Ni ibihe bimenyetso bya Hyperammonemia?

Ibimenyetso bishobora gutandukana cyane bitewe n'uburyo indwara imeze cyangwa ikomeye, ndetse n'imyaka ikura.

Ibimenyetso byoroheje bikunze kugaragara ku bana n'abantu bakuru

• Isesemi no kuruka
• Kubabara mu gifu
• Kurakara kenshi
• Kuribwa umutwe (umutwe)
• Ingorane zo kuringaniza, guhuza imikorere, no kuvuga (ataxia)
• Impinduka mu myitwarire

Ibindi bimenyetso byoroheje bigira ingaruka ku bana gusa

• Kunanirwa gutera imbere
• Ubukene bw'imitsi (hypotonia)
• Gutinda mu iterambere ry'imitsi

Ibimenyetso bikomeye ku bana no ku bantu bakuru

Ibi ni ibimenyetso biteje akaga cyane. Niba ufite kimwe muri ibi, ugomba kwihutira gushaka inama kwa muganga.

• Urujijo no kubura icyerekezo
• Guhindagurika kw'amarangamutima
• Gusinzira cyane
• Impinduka mu bwenge
• Indwara zo mu mutwe
• Guhumeka vuba (Hyperventilation)
• Koma

Icyitonderwa: Niba wowe cyangwa umuntu wawe wa hafi ufite ibi bimenyetso, nyamuneka hamagara 911 ako kanya, cyangwa ujye mu biro byihutirwa by’ibitaro biri hafi yawe. Urugero rwinshi rwa ammonia mu maraso rushyira ubuzima mu kaga kandi rusaba ubuvuzi bwihuse!

Ibimenyetso ku bana bavutse

Iyo umwana uvutse arwaye hyperammonemia, ibimenyetso bikurikira bikunze kugaragara mu masaha 24 kugeza kuri 72 nyuma yo kuvuka:

• Kurakara kenshi, kurira kenshi
• Kuruka
• Ubunebwe
• Indwara zo mu mutwe
• Gutaka iyo uhumeka
• Guhumeka vuba (Hyperventilation)

Icy'ingenzi: Niba umwana wawe agaragaje ibi bimenyetso, hamagara muganga ako kanya niba ukiri mu bitaro. Niba uri mu rugo, hamagara 911 ako kanya, cyangwa umujyane ku bitaro biri hafi. Ibi si ibintu byo gutinza.

Abaganga bapima iki kibazo gute ?

Ubusanzwe abaganga bakeka ko hari hyperammonemia bapima urugero rwa ammonia mu maraso.

• Ku bana bafite kugeza ku kwezi kumwe, niba urugero rwa ammonia mu maraso rurenze mikoromoli 80 kuri litiro (mikoromoli 80 kuri litiro).
• Ku bana bakuru, iyo birengeje micromol 55/litiro (55 micromol/L).
• Ku bantu bakuru, iyo birengeje mikoromoli 30 kuri litiro (mikoromoli 30 kuri litiro).

Iki gipimo (micromol/L) ni uburyo bwo gupima ingano ya ammonia mu maraso.

Ni ibihe bizamini bikorwa kugira ngo ibi bigaragare?

Ikizamini nyamukuru ni ikizamini cy'amaraso gipima urugero rwa ammonia mu maraso yawe. Niba urugero rwa ammonia ruri hejuru, muganga wawe ashobora gukora ibindi bizamini kugira ngo amenye impamvu. Urugero, ibizamini by'amaraso kugira ngo arebe imikorere y'umwijima wawe n'imikorere y'impyiko , ndetse no gupima inkari . Kubera ko hyperammonemia ishobora kwangiza uturemangingo tw'imitsi yo hagati kandi ikaba yashyira ubuzima mu kaga, muganga wawe ashobora kandi gukora ibizamini by'amashusho, nka scan ya CT (computed tomography) cyangwa MRI (magnetic resonance imaging), kugira ngo arebe niba hari ingorane zigira ingaruka ku bwonko.

Ni ubuhe buryo bwo kuvura hyperammonemia?

Uburyo bwo kuvura buterwa n'impamvu. Urugero, ku muntu mukuru, biterwa n'indwara y'umwijima cyangwa indwara y'umwijima, cyangwa ku mwana ukiri muto, biterwa n'indwara ya urea cycle disorder (UCD). Mu gihe cy'ubukana bwa hyperammonemia, intego nyamukuru ni ukugabanya urugero rwa ammonia mu maraso no kugenzura ingorane zihariye nko kubyimba ubwonko no kwiyongera k'umuvuduko ukabije mu bwonko (hypertension intracranial).

• Iyo umwana uvutse afite hyperammonemia, abaganga bazahagarika kumuha poroteyine (kuko ammonia ikorwa iyo poroteyine igogorwa) ahubwo bakamuha isukari, isukari, kugira ngo imuhe karori. Bazakoresha kandi uburyo bwitwa hemodialysis . Ibi bikubiyemo gusukura amaraso y'umwana no gukuramo ammonia binyuze mu mashini ikoresha dialysis n'icyuma cyihariye (nk'impyiko z'ubukorano).
• Abantu bafite ikibazo cyo kubura enzyme ya urea cycle (UCD) barwara hyperammonemia bitewe n'stress, nko kuba bafite stress-induced hyperammonemia, nabo bahabwa poroteyine zo gukuraho glucose na calories. Hemodialysis ikorwa gusa iyo urugero rwa ammonia rwabo rutagabanutse mu masaha make nyuma yo kuvurwa bwa mbere.
• Uburyo bwo kuvura indwara y’umwijima ni ukugabanya ikorwa rya ammonia mu mara. Uburyo bwa mbere bwo kuvura ni imiti inyobwa irimo lactulose na lactitol . Iyi sukari igabanya ikorwa n’iyuzuzwa rya ammonia mu mara.

Abaganga bashobora gutanga inama ku mirire nk'iyi kugira ngo bafashe abantu bafite indwara ya urea cycle kugabanya inshuro z'ibimenyetso byabo:

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

Ese hari uburyo bwo kwirinda iki kibazo?

Kubera ko hari impamvu nyinshi zitera hyperammonemia, si indwara zose zishobora kwirindwa. Ariko, ushobora gufata ingamba zo kwirinda ubwoko bumwe na bumwe bw'indwara z'umwijima, cyane cyane izijyanye n'imirire yawe n'imibereho yawe. Niba uri mu kaga ko kurwara indwara z'umwijima, muganga wawe ashobora kukugira inama yo guhindura iyi mikorere y'ubuzima:

• Nta kibazo kirimo kwirinda cyangwa kugabanya inzoga.
• Irinde ibiryo n'ibinyobwa birimo ibinure bya trans cyangwa sirupe y'ibigori ifite fructose nyinshi.
• Kugira ngo wirinde kwangirika k'umwijima, witondere cyane iyo ukoresha imiti yandikiwe na muganga n'indi itangwa na muganga.
• Gukora imyitozo ngororamubiri buri gihe .
• Kugabanya kurya inyama zitukura.

Kugira ngo wirinde indwara ya Reye, indwara itera hyperammonemia ku bana no mu rubyiruko, ntugahe abana aspirine ibimenyetso by'ubwandu bwa virusi (nk'ibicurane cyangwa inkoko) keretse iyo muganga abigusabye by'umwihariko. Ubusembwa bwa Urea (UCDs) ntibukunze kubaho, ariko hari ibizamini bya genetiki kugira ngo urebe niba ufite genetiki zibitera. Vugana na muganga wawe kuri ibi.

Ni iki giteganyijwe kuri iyi ndwara?

Uko hyperammonemiya ihagaze, ni ukuvuga ubushobozi bwo gukira, biterwa n'ibintu byinshi:

• Uko urugero rwa ammonia mu mubiri wawe ruri hejuru.
• Igihe urugero rwa ammonia rumaze rwongerwa.
• Ni iki gitera hyperammonemia?

Muri rusange, icyizere cyo kurwara hyperammonemia ni gito. Iyo itavuwe cyangwa ngo ivurwe itinze, kubyimba ubwonko bidasubirwaho bifitanye isano n'umubare munini w'impfu. Ikibabaje ni uko nubwo umwana ukiri muto yavurwa vuba cyangwa mu buryo bukomeye gute na hyperammonemia ikomeye, nta cyizere cy'uko umusaruro uzaba mwiza. Muri Leta Zunze Ubumwe za Amerika, igipimo cy'imyaka 11 yo kubaho ku bantu bafite indwara ya urea cycle defects (UCDs) barwara hyperammonemia mu ntangiriro z'ubuzima ni hafi 35%. Ku bantu barwara hyperammonemia mu myaka yabo yo hasi, igipimo cyo kubaho ni hafi 87%. Igipimo cy'umwaka umwe n'imyaka itatu yo kubaho ku barwayi bafite indwara ikomeye y'umwijima iterwa na hyperammonemia byavuzwe ko ari 42% na 23%, uko bikurikirana.

Ni izihe ngorane zishobora guterwa na hyperammonemia?

Iyo iki kibazo kidavuwe vuba, urugero rwinshi rwa ammonia rushobora kwangiza uturemangingo tw’imitsi yo hagati ndetse bigatera ingorane zishobora gushyira ubuzima mu kaga. Izo ngorane zirimo:

• Kubyimba k'ubwonko
• Ubwiyongere bw'umuvuduko mu bwonko (Intracranial hypertension)
• Kubyimba k'ubwonko - Ibi akenshi biterwa no kubyimba k'ubwonko no kwiyongera k'umuvuduko hirya no hino mu bwonko.
• Koma
• Urupfu

Iyo umuntu arwaye hyperammonemia idakira igihe kirekire, ashobora kugira ubumuga bwo mu mutwe ndetse n'ibibazo by'imyitwarire n'iby'indwara zo mu mutwe bidashira.

Ni ryari twagombye kubonana na muganga?

Niba wowe cyangwa umuntu ukunda afite ibimenyetso bya hyperammonemia, hamagara 911 ako kanya cyangwa ujye kwa muganga uri hafi. Ibi si ibintu byo gutinda. Niba ufite indwara y'umwijima cyangwa ikibazo cy'ibura rya enzymes ya urea mu gice kimwe, ni ngombwa cyane kujya kwa muganga wawe buri gihe kugira ngo agufashe kandi akurikirane urugero rwa ammonia mu mubiri wawe.

Hyperammonemia ni indwara ikomeye ishobora guterwa n'ibintu byinshi. Niba wowe, umwana wawe, cyangwa umwana wawe mufite ibi bimenyetso, shaka ubufasha vuba. Niba ufite indwara y'umwijima, cyangwa niba hari umuntu wo mu muryango wawe ufite indwara zo mu bwoko bwa urea cycle disorders (UCDs), vugana na muganga wawe ku byago byawe byo kurwara hyperammonemia. Barahari kugira ngo bagufashe.

Amaherezo, ibuka ibi.

Noneho rero, dore bimwe mu bintu by'ingenzi ugomba kwibuka mu byo twavuze:

• Hyperammonemia ni urugero rukabije rwa ammonia mu maraso. Ibi byangiza cyane ubwonko bwacu n'imitsi.
• Hari impamvu nyinshi zishobora kubitera. Ibibazo by'umwijima n'indwara za UCD (UCDs) ni byo bitera.
• Ibimenyetso biratandukanye bitewe n'uburemere bw'indwara n'imyaka yayo. Bishobora kuva ku bimenyetso byoroheje nko kugira isesemi, kuruka, no kuribwa umutwe kugeza ku bimenyetso bikomeye nko kwitiranya ibintu, gupfukama, no kwishimagura. Abana bavutse bagomba kandi kwita cyane ku bimenyetso byabo.
• Niba ufite ibimenyetso bikomeye, ugomba gushaka inama kwa muganga vuba. Ibi bishobora kugutwara ubuzima cyangwa urupfu.
• Ubuvuzi buterwa n'impamvu. Ubuvuzi bwihuse bushobora kugabanya urugero rwa amoniya no kugenzura ingorane.
• Nubwo hari impamvu zidashobora kwirindwa, zimwe mu ngaruka zishobora kugabanuka uramutse ukomeje kugira ubuzima bwiza kandi ukita ku mwijima wawe .
• Waba ufite indwara y'umwijima cyangwa umuntu wo mu muryango wawe ufite UCDs, ni ngombwa ko ujya kwisuzumisha kwa muganga buri gihe.

Wibuke ko uzi ubuzima bwawe neza. Niba wumva ikintu gitandukanye cyangwa kidasanzwe, ntutinye kuvugana na muganga. Hyperammonemia, ammonia, umwijima, uruhererekane rwa urea, ubwonko, ibimenyetso, uburyo bwo kuvura