Kodi mwana wanu ali ndi PKU? (Phenylketonuria) - Tiyeni tikambirane izi mwatsatanetsatane!

Kodi ndinu mayi wa mwana wakhanda? Kapena mwamvapo kuti winawake m'banja mwanu kapena mwana wa mnzanu ali ndi vutoli? Ngati ndi choncho, nkhaniyi ikhala yofunika kwambiri kwa inu. Lero tikambirana za Phenylketonuria , kapena PKU mwachidule, vuto lomwe anthu ambiri adamvapo ndipo sanazolowere, koma ndi lofunika kwambiri kudziwa. Musadandaule, tikambirana za izi mosavuta, m'njira yomwe mungamvetse.

Kodi PKU (Phenylketonuria) ndi chiyani? Tiyeni timvetse mosavuta!

Mwachidule, PKU ndi matenda a majini . Izi zikutanthauza kuti, amayamba chifukwa cha kusintha pang'ono kwa majini athu. Izi zimapangitsa kuti thupi lathu lisagwire bwino chinthu chotchedwa phenylalanine .

Tsopano mwina mukudabwa kuti phenylalanine ndi chiyani. Ndi amino acid . Ma amino acid ali ngati tinthu tating'onoting'ono tomwe timapanga mapuloteni. Phenylalanine imapezeka muzakudya zambiri zomwe timadya, makamaka zomwe zili ndi mapuloteni ambiri. Imapezekanso mu zotsekemera zina zopangidwa, monga aspartame .

Kawirikawiri, mwa munthu wathanzi, phenylalanine iyi imagawika mpaka kuchuluka kofunikira ndikukonzedwa mwanjira yoti thupi lizitha kuyamwa. Komabe, mwa munthu yemwe ali ndi PKU, izi sizichitika bwino. Kenako, phenylalanine iyi imayamba kusonkhana m'thupi. Ili ngati chitoliro chotsekedwa mu thanki yamadzi chomwe chimadzaza ndi madzi. Kuchulukana kumeneku ndi komwe kumayambitsa mavuto. Makamaka, kungakhudze kukula kwa ubongo . Ngati sichichiritsidwa, matenda monga 'kulephera kwa nzeru' angachitike.

Kodi pali mitundu yosiyanasiyana ya PKU?

Inde, pali mitundu ingapo ya PKU, kutengera kuopsa kwake. Zizindikiro zimasiyananso kutengera mtundu wake, makamaka kwa iwo omwe salandira chithandizo.

Mitundu itatu ikuluikulu ingathe kuzindikirika:

• PKU Yachikale: Iyi ndi mtundu woopsa kwambiri .
• PKU yapakati kapena yocheperako: Iyi ndi yochepa pang'ono.
• Hyperphenylalaninemia yochepa: Iyi ndi mtundu wochepa kwambiri wa vutoli. Ngakhale popanda chithandizo, anthuwa ali ndi chiopsezo chochepa kwambiri chokhala ndi vuto la maganizo.

Ndani amene amakhudzidwa kwambiri ndi vuto la PKU ili?

Popeza PKU ndi majini, aliyense amene ali ndi kusintha kwa majini m'makopi onse awiri a PAH akhoza kukhala ndi vutoli. Kafukufuku wina akusonyeza kuti anthu ochokera ku America kapena ku Europe ali pachiwopsezo chachikulu.

Chinthu china chofunika ndi chakuti mayi woyembekezera amene ali ndi PKU yosalamulirika, kutanthauza kuchuluka kwambiri kwa phenylalanine m'magazi mwake, akhoza kubereka mwana amene alibe PKU.Zingakhudze kukula kwa nzeru za mwana, kuyambitsa zilema zobadwa nazo, komanso kuyambitsa mavuto ena. Chifukwa chake, ndikofunikira kwambiri kuwongolera PKU panthawi ya mimba.

Kodi PKU ndi yofala bwanji?

Malinga ndi ziwerengero zochokera kumayiko ngati United States, matenda a PKU amakhudza pafupifupi mwana m'modzi mwa makanda 10,000 mpaka 15,000 . Ngakhale kuti n'zovuta kupeza ziwerengero zenizeni za izi ku Sri Lanka, vutoli limapezeka kulikonse padziko lapansi.

Kodi zizindikiro za PKU ndi ziti? Musachite mantha, dziwani izi!

M'mayiko ambiri, kuphatikizapo zipatala zina ku Sri Lanka, kuyezetsa makanda obadwa kumene kumachitika kale kuti adziwe ngati ali ndi PKU, zomwe zimapangitsa kuti athe kuzindikira vutoli asanayambe zizindikiro. Chifukwa chake, kuchuluka kwa zizindikiro pakadali pano kuli kochepa kwambiri.

Komabe, ngati vutoli silikudziwika mwanjira ina kapena ngati silinachiritsidwe, zizindikiro zina zingawonekere. Izi ndi izi:

• Matenda a pakhungu monga eczema .
• Kusintha kwa khungu ndi/kapena mtundu wa tsitsi ( khungu ndi tsitsi zimakhala zopepuka kuposa achibale ena).
• Mutu ndi wocheperapo kuposa wachibadwa (microcephaly) .
• Fungo loipa lochokera mu mpweya, pakhungu, kapena mkodzo. Limanunkhiza ngati chinthu chakale.

Zizindikiro zoopsa kwambiri zomwe zingachitike ngati sizikuchiritsidwa ndi izi:

• Mavuto a khalidwe.
• Kuchedwa kwa kukula - Izi zikutanthauza kuti zochitika za mwana monga kumwetulira, kukweza mutu wake, ndi kuyenda, zimachedwa.
• Kulephera kwa nzeru .
• Kawirikawiri, khunyu monga khunyu limatha kuchitika.

Chofunika: Kumbukirani, zizindikiro zambirizi zimachitika pokhapokha ngati sizinachiritsidwe. Masiku ano, kuyezetsa makanda obadwa kumene kumatha kuzindikira izi msanga, kotero palibe chodetsa nkhawa.

Kodi chifukwa chenicheni cha PKU ndi chiyani?

Chifukwa chachikulu cha PKU ndi kusintha kwa majini onse awiri a PAH m'thupi lathu. Majini a PAH awa amalangiza thupi lathu kupanga enzyme yotchedwa phenylalanine hydroxylase . Enzyme iyi imasintha amino acid phenylalanine, yomwe timadya, kukhala mapuloteni omwe thupi lingagwiritse ntchito.

Choncho, pamene jini ya PAH iyi sikugwira ntchito bwino, enzymeyo siipangidwa, kapena sikugwira ntchito bwino. Kenako, phenylalanine yochokera ku chakudya imasonkhana m'thupi. Thupi lathu, makamaka ubongo, limakhala lokhudzidwa kwambiri ndi phenylalanine yochuluka. Ichi ndichifukwa chake ubongo ukhoza kuwonongeka.

Kodi PKU imafalikira bwanji kudzera m'majini?

Kodi PKU ndi chiyani?Matenda a majini omwe amatengera kwa makolo kupita kwa ana mu autosomal recessive pattern. Izi zingamveke ngati mawu asayansi, koma mwachidule ndi awa:

Tangoganizirani, jini yomwe imayambitsa PKU imatchedwa "p". Jini yathanzi imatchedwa "P".

Kuti mwana akhale ndi PKU, ayenera kulandira jini ya "p" kuchokera kwa mayi ndi bambo (ndiko kuti, iyenera kukhala "pp").

Nthawi zambiri, makolo akhoza kukhala ndi jini ya PKU iyi. Izi zikutanthauza kuti, akhoza kukhala ndi jini ngati "Pp". Alibe zizindikiro za PKU, chifukwa jini ya "P" yathanzi ikugwira ntchito. Koma, amatha kupatsira jini ya "p" kwa ana awo.

Kotero, ngati makolo onse awiri ali ndi kachilombo (Pp x Pp), akakhala ndi mwana:

• Pali mwayi wa 25% kuti mwana akhale ndi PKU (pp).
• Pali mwayi wa 50% kuti mwanayo akhale ndi kachilomboka (Pp) - palibe zizindikiro.
• Pali mwayi wa 25% kuti mwana adzalandira majini onse athanzi (PP) - opanda PKU, komanso opanda chonyamulira.

Ndicho chifukwa chake ndi chinthu chomwe chiyenera kuchokera kwa makolo onse awiri.

Kodi mungadziwe bwanji ngati muli ndi PKU?

Kawirikawiri, PKU imapezedwa kudzera mu mayeso owunikira makanda obadwa kumene, omwe amachitika pakati pa maola 24 ndi 72 mwana atabadwa . Izi zimaphatikizapo kubaya chidendene cha mwana ndi singano yaying'ono ndikutenga madontho ochepa a magazi . Izi zimayesa kuchuluka kwa phenylalanine m'magazi a mwana.

• Ngati kuchuluka kwa phenylalanine m'magazi a mwana kukupezeka kuti kuli kokwera, madokotala adzachita mayeso ena (magazi kapena mkodzo) kuti atsimikizire vuto la PKU, komanso kuti adziwe mtundu wa PKU.
• Popeza PKU ndi matenda a majini, mayeso a majini amatha kuchitidwa kuti adziwe kusintha kwa majini komwe kumayambitsa zizindikiro.

Ngati mayeso oyezetsa ana obadwa kumene sanachitike, PKU imatha kupezeka pa msinkhu uliwonse, koma kupezeka msanga, kumakhala bwino.

Ndi mankhwala ati omwe alipo a PKU?

Chithandizo cha matenda a PKU ndi njira ya moyo wonse . Koma musadandaule, ngati mutachilandira bwino, mutha kukhala ndi moyo wathanzi. Chithandizo chingaphatikizepo:

1. Zakudya zapadera: Ichi ndiye chinthu chofunikira kwambiri. Muyenera kudya zakudya zokhala ndi phenylalanine yochepa koma zokhala ndi michere ina yambiri .

2. Mavitamini, mchere, ndi zowonjezera zakudya: Izi ndizofunikira kuti mupeze michere yomwe mukufuna, chifukwa kuchepetsa kudya kungapangitse kuti musakhale ndi michere ina.

3. Mankhwala: Anthu ena angapatsidwe mankhwala monga sapropterin dihydrochloride (Kuvan®) . Izi zimathandiza thupi kuswa phenylalanine.

4. Mankhwala a Pegvaliase (Palynziq®):Anthu omwe amamwa mankhwalawa amatha kudya bwino popanda malamulo apadera a zakudya. Izi zimalowa m'malo mwa enzyme yomwe imaphwanya phenylalanine yomwe sikugwira ntchito bwino m'thupi la anthu omwe ali ndi PKU.

Ngati inu kapena mwana wanu ali ndi PKU, muyenera kugwira ntchito ndi dokotala wanu komanso katswiri wazakudya kuti mupange njira yabwino kwambiri yothandizira.

Ndi zakudya ziti zomwe zili ndi phenylalanine? Tiyeni tidziwe izi!

Anthu omwe ali ndi PKU (pokhapokha atamwa mankhwala otchedwa `Pegvaliase`) ayenera kuchepetsa zakudya zokhala ndi phenylalanine yambiri. Izi zimapezeka muzakudya zokhala ndi mapuloteni ambiri. Mwachitsanzo:

• Mkaka ndi zinthu za mkaka (tchizi, yogurt)
• Mazira
• Mtedza (monga mtedza, ma cashew)
• Nsomba
• Nkhuku
• Ng'ombe
• Nyemba monga nyemba ndi mphodza
• Aspartame, chotsekemera chopangidwa (chimapezeka mu zakumwa ndi zakudya zina zopanda shuga)

Kodi pali zakudya zapadera kwa munthu amene ali ndi PKU?

Inde, ndithudi. Munthu amene ali ndi PKU (osamwa mankhwala a `Pegvaliase`) ayenera kutsatira zakudya zopanda mapuloteni ambiri . Zakudya zokhala ndi mapuloteni ambiri monga nyama, nsomba, mazira, ndi mkaka ziyenera kuchepetsedwa momwe zingathere, kapena kulekeratu.

Koma izi sizophweka monga momwe zikuonekera. Chifukwa mapuloteni ndi ofunikira m'thupi. Chifukwa chake, ndikofunikira kwambiri kulankhula ndi dokotala kapena katswiri wazakudya ndikupanga zakudya zoyenera zomwe zimapatsa michere ina yonse yomwe thupi limafunikira. Pali mitundu yapadera ya makanda omwe ali ndi PKU, yomwe ili ndi phenylalanine yochepa, koma imakhalabe ndi michere ina yofunika.

Tangoganizirani, muli ndi mwana wamng'ono kunyumba yemwe ali ndi PKU. Kodi muyenera kusamala bwanji ndi chakudya chomwe mumamupatsa? Muyenera kuyang'ana phukusi lililonse la chakudya ndikupeza kuchuluka kwa phenylalanine komwe kuli mkati mwake. Ndi nsembe yaikulu, koma ndiyofunika pa tsogolo la mwana.

Kodi PKU ingapewedwe?

Popeza PKU ndi ya majini, palibe njira yopewera vutoli.

Komabe, ngati mukukonzekera kutenga pakati, mutha kupatsidwa upangiri wa majini ndi kuyezetsa magazi kuti mudziwe ngati inu ndi mnzanu muli ndi jini ya PKU. Izi zikuthandizani kumvetsetsa chiopsezo chanu chokhala ndi mwana ndi PKU.

Kodi munthu amene ali ndi PKU angayembekezere tsogolo lotani?

Ili ndi vuto lomwe anthu ambiri ali nalo. Ngakhale kuti PKU ndi vuto lomwe limakhalapo kwa moyo wonse, ngati litathandizidwa bwino, anthu ambiri amatha kukhala ndi moyo wathanzi komanso wabwinobwino.

• Pambuyo pozindikira matendawa, kuyezetsa magazi nthawi zonse kumafunika kuti muwone kuchuluka kwa phenylalanine m'magazi . Umu ndi momwe mungadziwire ngati zakudya zikutsatiridwa bwino komanso ngati mankhwala akugwira ntchito bwino.
• Kwa iwo omwe amadya zakudya zochepa, katswiri wa zakudyaNdikothandiza kwambiri kugwira nawo ntchito limodzi. Amakuuzani zakudya zoyenera kudya, zomwe muyenera kupewa, komanso momwe mungapezere zakudya zomwe thupi lanu limafuna.
• Ngati mayi yemwe ali ndi PKU atenga pakati, dokotala wake ndi katswiri wa zakudya adzagwira ntchito limodzi kuti apange dongosolo lapadera la zakudya lomwe lidzaonetsetsa kuti mayiyo akupeza zakudya zomwe akufunikira komanso kuchepetsa kuvulaza kulikonse komwe kungachitike kwa mwana.

Kodi ndingadziteteze bwanji/ndingateteze bwanji mwana wanga ku matenda a PKU?

Kuzindikira matenda a PKU ndi vuto lomwe limakhalapo kwa moyo wonse, choncho ndikofunikira kuti mulumikizane ndi dokotala nthawi zonse ndikuyang'anira kuchuluka kwa phenylalanine m'magazi mwanu.

• Ngati mukudya zakudya zinazake zochepa, imwani mavitamini ndi zakudya zina zofunika kuti muchepetse kusowa kwa mapuloteni m'thupi.
• Tsatirani zakudya izi kwa moyo wanu wonse. Apo ayi, zizindikiro zingabwerenso n’kukhala zoopsa pa thanzi lanu.
• Ngati mukuganiza zoyambitsa banja, lankhulani ndi dokotala wanu za kuyezetsa majini , komwe kungakuthandizeni kumvetsetsa chiopsezo chanu chokhala ndi mwana wokhala ndi PKU.

Kodi muyenera kupita kwa dokotala liti?

• Ngati inu kapena mwana wanu mukupeza zizindikiro zokhudzana ndi PKU (zomwe takambirana kale), pitani kwa dokotala nthawi yomweyo kuti akakufufuzeni mokwanira.
• Musanayambe kutenga mimba, kapena kumayambiriro kwa mimba yanu, mutha kufunsa dokotala wanu za kuyezetsa magazi kwa omwe ali ndi kachilomboka , zomwe zingakuthandizeni inu ndi mnzanu kudziwa ngati muli pachiwopsezo chokhala ndi mwana wokhala ndi PKU.
• Ngati muli ndi PKU, pitani kwa dokotala nthawi zonse kuti akayeze magazi ndi kuyang'aniridwa.

Ndi mafunso ati omwe ndiyenera kufunsa dokotala wanga?

Ngati muli ndi mafunso okhudza matenda a PKU kapena momwe mwana wanu alili ndi matenda a PKU, musaope kufunsa. Nazi zitsanzo zina:

• "Ndingatsimikize bwanji kuti mwana wanga akupeza zakudya zoyenera kuti akhale wathanzi?"
• "Kodi ine/mwana wanga ndiyenera kumwa mavitamini ena kapena zakudya zowonjezera?"
• "Kodi kudya zakudya zopanda mapuloteni okwanira kuchepetsa kuchuluka kwa phenylalanine m'thupi la mwana wanga/wanga, kapena kodi ndiyeneranso kumwa mankhwala?"
• "Ndi zinthu ziti zomwe ndiyenera kusamala nazo pokonzekera chakudya chapaderachi?"
• "Ngati mwana akupita kusukulu, kodi sukulu iyenera kudziwitsidwa bwanji za chakudya ndi zakumwa zake?"

Poyamba, kusankha zakudya zopanda phenylalanine kungakhale kovuta pang'ono, koma mukamagwira ntchito ndi dokotala wanu komanso katswiri wa zakudya, mudzazolowera.

Pomaliza, zinthu zofunika kukumbukira (Uthenga Wopita Nawo Kunyumba)

Ngakhale kuti PKU ndi matenda omwe munthu amakhala nawo kwa moyo wake wonse, imatha kuchiritsidwa bwino. Chofunika kwambiri ndi kuzindikira msanga ndikuthana nawo bwino.

• Kuyezetsa makanda obadwa kumene n'kofunika kwambiri. Kungathe kuzindikira matenda ena monga PKU msanga.
• Mukangodziwa kuti muli ndi PKU, onetsetsani kuti mwapeza upangiri wa dokotala komanso malangizo a katswiri wa zakudya .
• Kutsatira kwambiri zakudya, komanso ngati kuli kofunikira, mankhwala pa moyo wonse ndikofunikira kwambiri kuti ubongo wanu kapena wa mwana wanu ukule bwino.
• Yang'anirani kuchuluka kwa phenylalanine yanu ndi kuyezetsa magazi nthawi zonse .
• Simuli nokha. Kulumikizana ndi anthu ena omwe ali ndi PKU ndi mabanja awo kungakhale gwero lalikulu la mphamvu ndi chilimbikitso kwa inu m'magulu othandizira .
• Ngati ndinu kholo la mwana yemwe ali ndi PKU, phunzitsani mwana wanu kukhala ndi vutoli moleza mtima komanso mwachikondi .

Ndikukhulupirira kuti izi zakuthandizani kumvetsetsa bwino za PKU (Phenylketonuria). Kumbukirani, kudziwa bwino matenda ndi chida chabwino kwambiri cholimbana ndi vuto lililonse la thanzi!

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PKU , Phenylketonuria, phenylalanine, matenda a majini, makanda obadwa kumene, zakudya, zakudya zopanda mapuloteni ambiri, kuyezetsa makanda obadwa kumene