Nukawoe nye ayiku me kpe siawo siwo hã vaa dziwò? (Cystine Stones) Mina míasrɔ̃ nu tso wo ŋu bɔbɔe!

Ðe woxɔ wò ɖe kɔdzi kpɔ le ayiku me kpewo kple vevesese si womate ŋu ado dzi atsɔ o le wò akɔta, ƒodo ƒe anyime, alo axadzi kpɔa? Alo ɖe nèse ame aɖe si nènya si to kuxi sia tɔgbe dzia? Ayiku me kpewo nye nuteƒekpɔkpɔ si me vevesese le vavã. Kpe siawo te ŋu dzɔna le mɔ vovovo geɖe nu. Egbea míele nu ƒom tso ayiku me kpe ƒomevi aɖe si mebɔ vie o, gake ate ŋu ahe kuxiwo vɛ ne womewɔ atike nɛ nyuie o, vevietɔ le sɔhɛwo dome, si woyɔna be cystine stones.

Nukawoe nye cystine kpe siawo?

Kpuie ko la, cystine kpewo nye ayiku me kpe ƒomevi aɖe. Ayiku me kpewo nye nu sesẽ siwo dzɔna ne atike vovovo siwo le wò aɖuɖɔ me ƒo ƒu, abe kpe suesuesuewo ene. Atike aɖe si woyɔna be cystine ye wɔa cystine kpewo . Cystine sia nye amino acid si le míaƒe ŋutilã me. Zi geɖe la, nusi wòle be wòadzɔe nye be ayikuawo gaxɔa cystine sia ɖe ŋutilãa me ake. Gake ɣeaɖewoɣi la, ne esia medzɔ nyuie o la, cystine la ƒoa ƒu ɖe aɖuɖɔa me eye wòwɔa kpe.

Zi geɖe la , cystine kpe siawo lolona ŋutɔ . Eyata ɣeaɖewoɣi la, ehiãna be woawɔ dɔ na wo be woaɖe wo ɖa be ayiku alo aɖuɖɔtoewo nagagblẽ o. Nublanuinyae nye be ame geɖe siwo ŋu cystine stones le la ate ŋu axɔ kpe siawo zi geɖe le woƒe agbenɔɣi. Gake mègatsi dzi o, mɔ aɖewo li siwo dzi woato ada nɔnɔme sia amekoko manɔmee. Le kpɔɖeŋu me, àte ŋu aɖu nɔnɔme sia dzi ne ètrɔ wò nuɖuɖu alo nèzã atike siwo naa wò aɖuɖɔ ƒe pH da sɔ .

Eyata nukae nye Cystinuria?

Cystinuria nye dɔléle si mebɔ o, si ƒe dome wonyi . Esiae nye ne atike aɖe si woyɔna be cystine ƒo ƒu ɖe wò aɖuɖɔ me. Dɔléle sia le ame siwo ade ɖeka le akpe ewo ɖesiaɖe me. Ne cystinuria le ŋuwò la, esesẽna na wò ŋutilã be wòaɖe cystine ɖa. Esiae nye esime wòƒo ƒu eye wòwɔa cystine kpewo. Cystinuria ate ŋu ana ayiku me kpewo nadze dziwò zi geɖe le wò agbemeŋkekewo katã me.

Domenyiŋusẽfianu ƒomevi aɖe si woyɔna be (autosomal recessive gene) gbɔe esia tsona . Esia fia be ne ɖevi nyi domenyiŋusẽfianu sia ƒe dome tso dadaa kple fofoa siaa gbɔ la , ɖevia axɔ cystinuria. Esia nye agbemeŋkekewo katã ƒe nɔnɔme eye womate ŋu adae keŋkeŋ o. Gake woate ŋu aɖu edzi nyuie ne wodae nyuie .

Aleke gbegbee ayiku me kpe siwo woyɔna be cystine mebɔ o?

Ne wotsɔe sɔ kple ayiku me kpe ƒomevi bubuwo la, cystine kpewo dzɔna le 1% va ɖo 2% me ko. Ema ƒã ŋutɔ. Gake nukutɔe la, ayiku me kpe siwo le ɖeviwo me ƒe 6% va ɖo 8% nye cystine kpewo . Le nyateƒe me la, ame siwo ŋu cystine stones le la dometɔ 80% xɔa woƒe kpe gbãtɔ hafi xɔ ƒe 20.Gake anya wɔ be woatu kpe siawo dometɔ ɖeka alo esi wu nenema le ƒe ɖesiaɖe me.

Nukae nye cystine stones ƒe dzesiwo?

Cystine kpewo ƒe dzesiwo ɖi ayiku me kpe bubuwo tɔ ŋutɔ. Ne kpe aɖe do ko hafi nèdzea dzesiwo sese gɔme.

Nu siawo tɔgbe ate ŋu adzɔ:

• Vevesese sesẽ aɖe si ƒoa ame le wò ŋutilã ƒe akpa ɖeka (akɔta) .
• Vevesese le ame ƒe akɔta alo ƒodo ƒe akpa si le ete.
• Ʋu kpɔkpɔ le aɖuɖɔ me .
• Aɖuɖɔtoe kple dɔgbo ƒe dɔmawɔmawɔ nyuie (aɖuɖɔtoe).
• Vevesese ne wole aɖuɖɔ tom (esia dzɔna ne kpea te ɖe tsinyegoea ŋu).

Ɣeaɖewoɣi la, kpe si woyɔna be cystine ate ŋu atɔ eye wòana aɖuɖɔtoe me dɔléle (UTI) nadze ame dzi . Esi wònye be cystine-kpewo lolo vie wu kpe bubuwo ta la, woate ŋu ahe kuxi mawo vɛ.

Nukatae kpe siawo siwo woyɔna be cystine la dzɔna?

Abe alesi míegblɔe va yi ene la, anɔ eme be cystine kpewo dzena le amesiwo ŋu dɔléle aɖe si mebɔ o si woyɔna be cystinuria . domenyiŋusẽfianu siwo nye SLC3A1 kple SLC7A9 ƒe tɔtrɔ gbɔe cystinuria tsona. Nɔnɔme sia nana wò ŋutilã mete ŋu zãa amino acid cystine nyuie o. Emegbe eƒoa ƒu hewɔa kpewo. Kpe siawo ate ŋu adze wò aɖuɖɔtoe ƒe akpa ɖesiaɖe, abe wò tsinyegoe alo ayiku ene.

Kpuie ko la, domenyiŋusẽfianu ƒe kuxie nye esia. Ema fia be menye wò vodadae o.

Nukae wotsɔ wɔa cysteine ​​kpewoe?

Cystine kpewo nyea cystine . Abe alesi míegblɔe va yi ene la, cystine nye amino acid . Amino acid nye protein ƒomevi aɖe. Wokpena ɖe míaƒe ŋutilã ŋu wòdzraa lãmekawo ɖo, wòtua lãmekawo ɖo, eye wòwɔa avu kple dɔlékuiwo. Kuxiwo le amesiwo ŋu cystinuria le ŋu le woƒe ayikuwo ƒe amino acid sia xɔxɔ alo ezazã me.

Kuxi kawoe ate ŋu ado tso cystine kpewo me?

Cystinuria nye dɔléle si nɔa ame ƒe agbemeŋkekewo katã me. Wò ɖɔkta ate ŋu akpe ɖe ŋuwò nàtsɔ atikewo, tɔtrɔ le nuɖuɖu ŋu, kple ɖewohĩ amekoko dzi akpɔ dɔlélea gbɔ. Gake anɔ eme be cystine kpewo adze dziwò le wò agbemeŋkekewo katã me. Kpe siawo ate ŋu ave wo ŋutɔ ne wole yiyim.

Cystine kpewo ate ŋu ahe kuxiwo abe:

• Ayiku me dɔlékuiwo .
• Aɖuɖɔtoe (si nye mɔ̃ si tsɔa aɖuɖɔ tso ayiku me yia tsinyegoe me) ƒe xexe.
• Aɖuɖɔtoedɔlélewo (UTIs) .
• Ayikuwo ƒe gbegblẽ.

Aleke nàwɔ anya ne kpe siwo woyɔna be cystine le ŋuwò?

Wò ɖɔkta akpɔ cystine kpewo to toɖoɖo wò dzesiwo kple wò atikewɔwɔ ŋutinya bliboa xɔxɔ, si me nenye be dɔléle siawo nɔ wò ƒomea me dometɔ aɖe ŋu hã le. Tsɔ kpe ɖe eŋu la, àte ŋu awɔ nu siwo gbɔna:

• Nɔnɔmetatawo dodokpɔ , abe ultrasound scan kple CT scan , kple aɖuɖɔ dodokpɔ ene.
• Kpe ɖoɖo ɖe dodokpɔxɔ aɖe be woadoe kpɔ. Ne àte ŋui la, ƒo kpe aɖe si fa kple wò aɖuɖɔ la nu ƒu, eye wò ɖɔkta ate ŋu aɖoe ɖe dodokpɔxɔ me be woadae kpɔ. To mɔ sia dzi la, woate ŋu anya atike si tututu wotsɔ wɔ kpeae.

Aleke wodaa cystinuriae?

Nu vevitɔ kekeake ne wole cystine kpewo dam enye be woadze agbagba axe mɔ na wo be woagadzɔ le gɔmedzedzea me o . Mɔ nyuitɔ siwo dzi nàto awɔ esia dometɔ ɖekae nye be nàɖe cystine ƒe agbɔsɔsɔ si le wò aɖuɖɔ me dzi akpɔtɔ. Anɔ eme be wò ɖɔkta azã atikewo kple nuɖuɖu ƒe tɔtrɔ vovovowo atsɔ akpe ɖe ŋuwò nàɖe kpe siwo nèwɔna dzi akpɔtɔ. Ne atikewo kple nuɖuɖu ƒe tɔtrɔ mexe mɔ na kpeawo ƒe wɔwɔ o, alo ne kpeawo lolo ŋutɔ la, amekoko hã nye mɔnu bubu.

Atikewo zazã

Ne wò ɖɔkta susu be tsi geɖe nono akpe ɖe kpea ŋu wòato le eɖokui si la, ate ŋu aŋlɔ vevesesenutsitike si womeƒlena o (OTC) na wò ɖe vevesesea ta. Woate ŋu aŋlɔ atikewo abe tiopronin kple d-penicillamine ene hã be wòakpe ɖe ame ŋu be kpewo nagadzɔ le etsɔme o.

Nuɖuɖu Ŋuti Ðoɖowɔɖi

Wò ɖɔkta ate ŋu aɖo aɖaŋu na wò be nàtrɔ wò nuɖuɖu ƒe nɔnɔmewo be kpewo nagadzɔ o. Esiawo dometɔ aɖewoe nye:

• Tsi geɖe nono. Esia ate ŋu akpe ɖe ame ŋu be cystine kpewo nalɔ. Wò ɖɔkta ate ŋu agblɔ na wò be nàno tsi ounces 96 (anɔ abe lita 2.8 ene) gbesiagbe, ano tsi kplu ɖeka gaƒoƒo ɖesiaɖe, alo nàno tsi ne èfɔ le zã me.
• Ðe sodium (dze) agbɔsɔsɔme si nèɖuna dzi kpɔtɔ. Ema fia be ele be nàdzudzɔ nuɖuɖu siwo wofa tsikpe alo esiwo wotsɔ gaze me ɖuɖu. Menye dze si míetsɔ dea bli si míeɖuna me koe o, ke dze ɣaɣla hã le nuwo abe atadi, atadi, lãmi, nuɖuɖu siwo wobla ɖe agba me, kple nuɖuɖu siwo woƒlena le fiasewo me ene me. Eyata ele be nàɖɔ ŋu ɖo le ema hã ŋu.
• Lãwo ƒe protein , si fia be woaɖu lã ʋɛ aɖewo ko eye woaɖu protein si tso numiemiewo me boŋ. Le kpɔɖeŋu me, àte ŋu atsɔ nuwo abe chickpeas, lentils, kple soya ene akpe ɖe wò nuɖuɖu ŋu ɖe lã kple tɔmelã teƒe.

Amekoko

Kpe gãwo ate ŋu agblẽ ne wometo aɖuɖɔtoea me o. Kpe siawo ate ŋu ave ame ŋutɔ eye woate ŋu axe mɔ na aɖuɖɔ ƒe sisi tso ŋutilãa me. Mɔnu geɖe li siwo dzi wò ɖɔkta ate ŋu azã atsɔ aɖe kpe aɖe ɖa:

• Wotsɔa fotoɖemɔ̃ sue aɖe si te ŋu trɔna bɔbɔe la yia wò tsinyegoe me, to tsinyegoe kple tsinyegoe me, be woake ɖe kpea ŋu ahaɖee ɖa (ureteroscopy) .
• Woɖea nu sue aɖe ɖe wò akɔta ƒe ŋutigbalẽ me, wotsɔa mɔ̃ aɖe toa eme dea ayiku me, eye woɖea kpea ɖa.(nephrolithotomy to ŋutigbalẽ me) .

Aleke nàwɔ anya ne atikewɔwɔa kpɔ dzidzedze?

Wò ɖɔkta ate ŋu abia be woado aɖuɖɔtoe me kpɔ ɣeaɖewoɣi be wòalé ŋku ɖe wò cystine ƒe agbɔsɔsɔ ŋu. Dodokpɔ sia akpe ɖe eŋu wòanya nenye be wò atikewɔwɔ ƒe ɖoɖoa le dɔ wɔm alo mele dɔ wɔm o.

Ðe woate ŋu alɔ cystine kpewo mea?

Ẽ, woate ŋu ato cystine-kpe aɖe ŋu. Gake ebia be woalé ŋku ɖe eŋu nyuie eye woayi edzi awɔ atike nɛ . Le esi teƒe be wòaʋuʋu keŋkeŋ la, woate ŋu ana wòalolo eye wòato eme nanɔ bɔbɔe.

Nuɖuɖu kawoe kpena ɖe ame ŋu le cystinuria me?

Numekukuwo ɖee fia be amesiwo ŋu cystinuria le ate ŋu aɖe vi ne wowɔ tɔtrɔ le woƒe nuɖuɖu ŋu. Esiawo dometɔ aɖewoe nye tsinono geɖe, atikutsetse kple amagbe geɖe ɖuɖu, kple nuɖuɖu si me dze mesɔ gbɔ ɖo o ɖuɖu. Citrus-kutsetsewo abe amititsetse kple lime, amagbe damawo, kple aŋgba damawo nyo ŋutɔ.

Ðe woate ŋu axe mɔ ɖe kpe siwo woyɔna be cystine nua?

Cystinuria nye dɔléle si ƒe dome míenyi, eyata womate ŋu axe mɔ ɖe enu o. Gake ne èwɔ ɖe atikewɔwɔ ƒe ɖoɖo si wò ɖɔkta na wò dzi la , àte ŋu axe mɔ na cystine kpewo ƒe dzɔdzɔ . Eyae nye be àte ŋu akpɔ kpewo ƒe wɔwɔme dzi.

Ðe cystinuria ate ŋu ade ame ƒe agbe afɔku mea?

Zi geɖe la, esia medea ame ƒe agbe afɔku me o . Ame akpa gãtɔ mexɔa nɔnɔme sesẽwo abe ayiku ƒe dɔmawɔmawɔ nyuie ene o. Nu vevitɔe nye be nàwɔ dɔ kple wò ɖɔkta akpɔ atike si awɔ dɔ na wò. Ame akpa gãtɔ wɔa nu ɖe ​​atikewɔwɔ ŋu nyuie. Ate ŋu adzɔ hã be woakpɔ atike si magblẽ nu le ayiku, tsinyegoe, alo aɖuɖɔtoe ŋu o.

Nukae nye etsɔme mɔkpɔkpɔ na sistin-kpewo?

Cystine kpewo ate ŋu adzɔ zi geɖe le wò agbenɔɣi. Ne cystinuria le ŋuwò la, ahiã be nàyi edzi anɔ tɔtrɔ wɔm le nuɖuɖu ŋu eye nàno atikewo be cystine kpewo nagadzɔ o. Wò ɖɔkta alé ŋku ɖe ŋuwò eye wòakpɔ egbɔ be èle cystine kpe yeye ɖesiaɖe si dona la dam nyuie. Esia nye be wòaxe mɔ ɖe nusiwo gblẽ le wò aɖuɖɔtoe, ayiku, kple tsinyegoe ŋu nu.

Ɣekaɣie wòle be makpɔ ɖɔkta?

Ne ayiku me kpe ƒe dzesi aɖewo le ŋuwò la, kpɔ egbɔ be yeyi ɖɔkta gbɔ . Dzesi siwo bɔ wu dometɔ aɖewoe nye esi:

• Vevesese le dɔgbo, akɔta ƒe axadzi, alo akɔta.
• Aɖuɖɔtoe kple ʋuʋudedi.
• Vevesese ne wole aɖuɖɔ tom.

Cystinuria nye dɔléle si nɔa ame ƒe agbemeŋkekewo katã me si nana cystine kpewo dzɔna. Wɔ dɔ kple wò ɖɔkta nàwɔ atikewɔwɔ ƒe ɖoɖo aɖe si ana kpewo nagadzɔ o. Esi wònye be domenyiŋusẽfianu gbɔe dɔlélea tso ta la, amesiwo ŋu cystinuria le ate ŋu abu domenyiŋusẽfianu ŋuti aɖaŋuɖoɖo nana ŋu ne yewole ƒome ɖoɖo ŋu bum.Ate ŋu aɖe vi hã be wòaxɔe. Nya nyuiae nye be zi geɖe la, woate ŋu aɖu nɔnɔme sia dzi to atikewɔwɔ me eye mehea ayiku ƒe dɔmawɔmawɔ nyuie alo aɖuɖɔtoe me gblẽ vevie o.

Nu vevitɔ kekeake siwo dzi wòle be woaɖo ŋkui (Take-Home Message) .

Enyo, eyata mina míaƒo nu tso nusiwo dzi wòle be nàɖo ŋkui tso nusiwo ŋu míeƒo nu tsoe me la dometɔ aɖewo ŋu kpuie:

• Cystine kpewo nye ayiku me kpe ƒomevi aɖe si dzɔna le domenyiŋusẽfianu ƒe nɔnɔme aɖe si woyɔna be cystinuria ta .
• Esiawo ate ŋu alolo ŋutɔ eye woate ŋu adzɔ zi geɖe le ame ƒe agbenɔɣi katã.
• Dzesiawo ate ŋu anye vevesese sesẽ, ʋu si le aɖuɖɔ me, kple ʋuʋudedi .
• Atikewɔwɔa dometɔ aɖewoe nye tsi geɖe nono, dze kple lãwo ƒe protein dzi ɖeɖe kpɔtɔ, eye ne ehiã la, atikewo kple amekoko .
• Togbɔ be womate ŋu ada cystinuria keŋkeŋ o hã la, ne wodae nyuie la, woate ŋu aɖu kpe ƒe wɔwɔ dzi eye woate ŋu anɔ agbe si sɔ.
• Ne ayiku me kpe ƒe dzesiwo le ŋuwò la , mègagblẽ ɣeyiɣi aɖakpɔ ɖɔkta o.

Taflatse mide dzesii be nyatakakawo nana koe nyatakaka siawo nye eye mele be woabu wo be wonye atikewɔlawo ƒe aɖaŋuɖoɖo o. Anyo wu ɣesiaɣi be nàkpɔ ɖɔkta le lãmesẽnya ɖesiaɖe ŋu.

` Ayiku me kpewo, cystine kpewo, cystinuria, aɖuɖɔtoe me kpewo, ayikudɔlélewo, domenyiŋusẽfianu ƒe dɔlélewo, aɖuɖɔtoe me dɔlékuiwo