Isisombululo kwiingxaki zomjikelo we-urea? Masifunde ngeepilisi zeSodium Phenylbutyrate

Ngaba wena okanye umntu omaziyo wakha waxelelwa ngugqirha ukuba unesifo sokujikeleza kwe-urea , okanye i-Urea Cycle Disorder ( UCD ) ? Xa oku kusenzeka, inqanaba lento enobuthi ebizwa ngokuba yi-ammonia emzimbeni wethu inokunyuka ngendlela eyingozi. Le yimeko embi kakhulu. Ngoko ke, elinye iyeza elikhethekileyo elicetyiswa ngoogqirha ngamaxesha anje yiSodium Phenylbutyrate . Namhlanje, siza kuthetha ngale yeza, ukuba liyintoni, lisebenza njani, kunye namanyathelo okuthintela xa ulisebenzisa, ngendlela elula onokuyiqonda.

Luhlobo luni lonyango oluyiSodium Phenylbutyrate?

Ngamafutshane, iSodium Phenylbutyrate liyeza elinikwa abantu abane -Urea Cycle Disorders (UCDs) . Kule meko ibizwa ngokuba yi-UCD, umzimba wethu awukwazi ukususa i-ammonia ngokufanelekileyo, imveliso yenkunkuma eveliswa xa iproteni ityiwa. Khawuthelekelele oku, kufana nomzi-mveliso emzimbeni wethu onenkqubo yokulahla inkunkuma evalekileyo. Ngoko ke, xa le ammonia iqokelelana emzimbeni, iyingozi kakhulu, ingakumbi engqondweni, kwaye inokubangela iingxaki ezinkulu zempilo.

Ngoko ke, into eyenziwa leli yeza leSodium Phenylbutyrate kukunceda umzimba wakho unciphise le ammonia igqithisileyo. Ngokuchanekileyo, eli yeza livuselela ezinye iindlela emzimbeni ezisusa iammonia. Oku kunciphisa umngcipheko wokufumana ezo ngxaki zempilo ziyingozi.

Into ebalulekileyo kukuba eli yeza aliyinyangi ngokupheleleyo i-UCD. Nangona kunjalo, liyanceda ukulawula iimpawu kwaye liphile ubomi obuqhelekileyo.

Ungalifumana eli yeza phantsi kwegama lophawu lweBuphenyl . Kwakhona, ngamanye amaxesha linokusetyenziswa kwezinye iimeko. Ngoko ke ukuba unemibuzo, kungcono ubuze ugqirha wakho okanye ingcali yezonyango.

Zeziphi izinto ekufuneka uzixelele ugqirha wakho ngaphambi kokuba usebenzise eli yeza?

Ngaphambi kokuba uqale ukusebenzisa eli yeza, kubalulekile ukuxelela ugqirha wakho okanye iqela lezonyango elikunyangayo ngezi zinto. Ngaba ezi zinto zibalulekile?

• Ukuba unesifo seswekile .
• Ukuba utya ukutya okungenatyuwa ingako .
• Ukuba unesifo sentliziyo .
• Ukuba unesifo sezintso .
• Ukuba unezinga eliphantsi le-potassium egazini lakho.
• Ukuba unesifo sesibindi .
• Ukuba uvuvukile amaqatha, iinyawo, izandla .
• Ukuba wakha waba ne-allergy okanye i-reaction engaqhelekanga kweli yeza , iSodium Phenylbutyrate, naluphi na olunye unyango, ukutya, iidayi, izithinteli, okanye nayiphi na enye into.
• Ukuba ukhulelwe okanye uzama ukukhulelwa .
• Ukuba ungumama oncancisayo .

Xa ugqirha esazi ngezi zinto, kuya kuba lula kuye ukugqiba ngokuchanekileyo ukuba eli yeza lifanelekile kuwe okanye akunjalo, kunye nendlela yokulinika.

Ndingayisebenzisa njani kanye kanye le yeza?

Eli yeza liza njengepilisi yokulisela ngomlomo.

• Ufanele usele amayeza akho yonke imihla ngexesha elimiselweyo, kanye njengoko ugqirha esitsho kwileyibhile kagqirha. Musa ukuwasela ngamaxesha ahlukeneyo.
• Eli yeza lifanele lithathwe kunye nokutya. Musa ukulithatha ungenanto esiswini.
• Musa ukuyeka ukuthatha eli yeza ngequbuliso ngaphandle kwengcebiso kagqirha wakho. Ukwenza njalo kunokubangela iingxaki.

Ngokwesiqhelo, eli yeza lisetyenziswa kunye nokutya okukhethekileyo okuneproteni ephantsi . Oku kungenxa yokuba abantu abane-UCD badla ngokuvelisa i-ammonia eninzi xa besitya iiproteni ezingaphezulu. Ke ngoko, kufuneka uqaphele kakhulu ngento oyityayo. Ukongeza, abanye abaguli banokucetyiswa ngugqirha wabo ukuba bathathe izongezo zokutya . Kubalulekile ukulandela yonke le miyalelo ngononophelo.

Izinto ekufuneka uzilumkele xa unika abantwana abancinci eli yeza

Eli yeza linokunikwa abantwana abancinci kwiimeko ezithile ezikhethiweyo. Nangona kunjalo, kukho izinto ekufuneka uzilumkele. Ukuba unika umntwana eli yeza, thetha nogqirha wakho ngalo ngononophelo kwaye ulandele imiyalelo yakhe ngokuchanekileyo. Umlinganiselo weyeza unokwahluka ngokuxhomekeke kubunzima bomntwana kunye nobudala bakhe.

Yintoni omawuyenze ukuba uthatha amayeza amaninzi kakhulu? (Ukusebenzisa amayeza amaninzi kakhulu)

Ukuba ucinga ukuba usele kakhulu eli yeza, fowunela iziko lolawulo lwetyhefu okanye igumbi longxamiseko ngokukhawuleza. Kubaluleke kakhulu. Musa ukwenza nantoni na ovakalelwa kukuba uyafuna ukuyenza.

Enye into, eli yeza lelakho wedwa. Musa ukulinika omnye umntu, nokuba uneempawu ezifanayo nezakho. Eli yeza lisebenza ngendlela eyahlukileyo kumntu ngamnye.

Yintoni omawuyenze ukuba uphoswe yidosi yeyeza?

Ukuba ulibele ukuthatha idosi, yithathe kwangoko xa ukhumbula. Nangona kunjalo, ukuba sele kusondele ixesha lokusebenzisa idosi yakho elandelayo, yitsibe idosi oyiphosileyo uze uthathe idosi yakho elandelayo ngexesha elicwangcisiweyo. Musa ukuthatha iidosi eziphindwe kabini okanye ezongezelelweyo ukuze ubuyisele idosi oyiphosileyo. Ukwenza njalo kunokunyusa umngcipheko weziphumo ebezingalindelekanga.

Ezinye izinto ezinokusebenzisana nale mithi (Intsebenziswano)

Kukho amayeza athile anokubangela iingxaki xa esetyenziswa nge-sodium phenylbutyrate. Oku kuthetha ukuba ukusebenza kakuhle kwale mayeza kunokuncipha, kwandiswe, okanye iziphumo ebezingalindelekanga zinyuke. Ke ngoko, kufuneka ulumke ngakumbi xa usebenzisa la mayeza ukuba usebenzisa la mayeza alandelayo:

• IHaloperidol (iyeza lokugula ngengqondo)
• I-Probenecid ( iyeza elisetyenziselwa iimeko ezifana ne-gout)
• Amayeza e-steroid (umzekelo , i-Prednisone okanye i-Cortisone )
• I-Valproic acid (iyeza elisetyenziselwa izifo ezifana nesifo sokuwa)

Olu luhlu luyimizekelo embalwa nje. Alulo uluhlu olupheleleyo. Ngoko ke, xelela ugqirha wakho ngawo onke amayeza owasebenzisayo (kuquka iivithamini, amayeza emifuno, kunye nezongezo), kwaye ukuba uyatshaya, usela utywala, okanye usebenzisa ezinye iziyobisi. Ezinye izinto zinokuchaphazela amayeza akho.

Ezinye izinto ekufuneka uziqwalasele xa usebenzisa amayeza

• Ukuba iimpawu zakho aziphucuki, okanye ukuba ziya zisiba mandundu, xelela ugqirha wakho ngoko nangoko.
• Ngelixa usebenzisa eli yeza, ugqirha wakho unokuyalela uvavanyo lwegazi ukujonga amanqanaba e-ammonia yakho kunye nokujonga ukuba iyeza lisebenza kakuhle na. Musa ukulibaleka eli yeza.

Zithini iziphumo ebezingalindelekanga ezinokubakho zale yeza?

Njengayo nayiphi na iyeza, eli yeza le-sodium phenylbutyrate linokubangela iziphumo ebezingalindelekanga kwabanye abantu. Asinguye wonke umntu ozifumanayo, kodwa zinokwenzeka.

Iziphumo ebezingalindelekanga ekufuneka uzixele kugqirha wakho ngoko nangoko:

Ukuba ufumana naziphi na kwezi ziphumo ebezingalindelekanga, xelela ugqirha wakho okanye iqela lezonyango ngokukhawuleza:

• Iimpendulo ze-allergy: Ukurhawuzelela kwesikhumba, ukurhawuzelela, amaqhuqhuva, ukudumba kobuso, imilebe, ulwimi, okanye umqala. Ukuba oku kuyenzeka, kunokubangela ubunzima bokuphefumla, ngoko ke kufuneka wenze ngokukhawuleza.
• Ukudinwa, ukulahlekelwa kukulinganisela okanye ukulungelelana, ukudideka okanye ingxaki yokuthetha: Ezi zinokuba yimiphumo kwinkqubo yemithambo-luvo.
• Inqanaba eliphantsi le-potassium: Oku kunokubangela iintlungu okanye ukuqaqamba kwemisipha, ukudinwa okanye ubuthathaka obungaqhelekanga, ukubetha kwentliziyo okukhawulezayo okanye okungahambelaniyo, kunye nokuqhina.
• Ukudumba kwamaqatha, izandla, okanye iinyawo: Oku kusenokuba kungenxa yokugcinwa kwamanzi emzimbeni.

Iziphumo ebezingalindelekanga ezingadingi ngcebiso kagqirha, kodwa kufuneka zixelwe kugqirha wakho ukuba ziyaqhubeka okanye ziyakhathaza:

Ezi zinto zisenokwenzeka zize ziphele zodwa. Kodwa ukuba ziyaqhubeka, okanye ukuba ziyakukhathaza, xelela ugqirha wakho :

• Ivumba lomzimba
• Tshintsha incasa
• Abafazi banemijikelo yokuya exesheni engacwangciswanga okanye amabala angabonakaliyo.
• Ukulahlekelwa ngumdla wokutya

Olu luhlu alubandakanyi zonke iziphumo ebezingalindelekanga ezinokubakho. Ukuba ufumana nantoni na eyahlukileyo, thetha nogqirha wakho ngayo. Musa ukoyika iziphumo ebezingalindelekanga, kodwa kubalulekile ukuba uzazi.

Uligcina njani eli yeza?

Kubaluleke kakhulu ukugcina amayeza akho ngokufanelekileyo.

• Yigcine kude nabantwana abancinci kunye nezilwanyana zasekhaya.
• Gcina isiciko sebhotile yamayeza sivaliwe kakuhle.
• Gcina kubushushu begumbi , phakathi kwe-15 kunye ne-30 degrees Celsius (59 kunye ne-86 degrees Fahrenheit). Musa ukugcina kwiindawo ezishushu kakhulu okanye ezibandayo kakhulu.
• Musa ukusebenzisa amayeza aphelelwe lixesha. Walahle.

Umyalezo obalulekileyo onokuwuthatha ekhaya

Kulungile, ngoko ke, ngokwezinto esithethe ngazo, ezi zezona zinto zibalulekileyo ekufuneka uzikhumbule:

• I-Sodium Phenylbutyrate liyeza elinikwa abantu abaneengxaki zomjikelo we-urea (UCDs) ukulawula amanqanaba e-ammonia emzimbeni.
• Oku akuyonyango lwesifo, kodwa kunceda ukulawula iimpawu.
• Thatha idosi emiselweyo, ngexesha elimiselweyo, kunye nokutya, njengoko kuchazwe ngugqirha.
• Ukutya ukutya okuneproteni ephantsi kubaluleke kakhulu ngeli xesha lonyango.
• Xelela ugqirha wakho ngazo zonke ezinye iziyobisi ozisebenzisayo kunye nazo naziphi na ezinye izifo onazo.
• Lumka ngemiphumo emibi , kwaye uxelele ugqirha wakho ukuba ubona nantoni na engaqhelekanga.
• Gcina amayeza ngokufanelekileyo kwaye uwagcine kude nabantwana abancinci.

Khumbula, eli nqaku linika ulwazi oluqhelekileyo kuphela. Ngugqirha wakho kuphela onokukuxelela ngqo ukuba imeko yakho injani kwaye ingaba eli yeza likulungele na. Ngoko ke, ungoyiki ukuthetha nogqirha wakho ngayo nayiphi na imibuzo onokuba nayo. Ndikunqwenelela ukuchacha ngokukhawuleza!

I- Sodium Phenylbutyrate, Ukuphazamiseka koMjikelo we-Urea, i-UCD, i-Ammonia, i-Buphenyl, Amayeza, Iziphumo ebezingalindelekanga