YeeFom ze-Johnson syndrome: inkcazelo, sifo, iimpawu kunye nonyango

Nangona ukuba iyeza namhlanje wenze inkqubela enkulu kunye nezifo ezininzi azikho ixesha elide isivakalisi ukuba izigulane, ezinye zezi zifo akukaqondwa kakuhle. Enye yazo i yeeFom ze-Johnson syndrome. Ekliniki, isifo kwaye afumane unyango lwesi sifo edalwa iingcali zempilo ezithile umdla. Qwalasela lo mbuzo ngokucacileyo ngaphezulu.

Rotor syndrome kunye yeeFom ze-Johnson syndrome - iseliswelekile isifo njengelifa isibindi Afanayo senyongo ezihambelanayo.

oonobangela

YeeFom ze-Johnson syndrome nenye igama: enzimopaticheskaya senyongo esibindini. Ziphakama ngokunxulumene ukuhlukunyezwa unikezelo kwi enyongweni evela kwiiseli kwesibindi yibilirubin.

Oku kuvela emzimbeni ukuba ukususwa ngenxa yolo erythrocytes ezonakeleyo okanye zitshatyalaliswe ngokupheleleyo. Isiphelo yibilirubin kwenzeka ngeendlela ezimbini:

• ukwandisa imali wenkqu uphume nge enyongweni;
• neentwana umchamo ukuya.

YeeFom ze-Johnson syndrome kwenzeka xa ukuphazamisa zothutho yibilirubin nge enyongweni. Esi sisifo ezinqabe kakhulu basengozini abafana ngakumbi (malunga-20-30 iminyaka). iimeko zesi sifo ukususela ekuzalweni kuyenzeka. Ingozi yosulelo emva kweminyaka umntu engama-50 okanye nangaphezulu kuba ncinci.

Unobangela genetic syndrome, yeeFom ze-Johnson kungenxa kuguquka gene, senze ukuthunyelwa yibilirubin esibindini kunye neeproteni ikhowudi.

iimpawu

YeeFom ze-Johnson syndrome, iimpawu zazo uphawu ezinye izifo ezifana, ngokomzekelo, ukudinwa, kwaye iimpawu zayo zahluke:

• ubuhlungu ezimbanjeni ngasekunene;
• ukunqongophala epheleleyo emnyama;
• kusenokubakho ukurhawuzelela kancinane kwesikhumba;
• isibindi landile;
• kwesisu, nto leyo maxa wambi kwandisa;
• ukuphalaza;
• ukwanda lobushushu.

Obubonakala ziyanyuswa ngenxa kwizifo ezosulelayo, emzimbeni nasengqondweni sindisa kakhulu. Kuba namankazana, ukuqhubela kunokwenzeka emva kwe zokucwangcisa.

iqela ubungozi

Leliphi iqela labantu kufuneka uqaphele onaso?

Esi sifo ilifa. Ukuba usapho abantwana nesi sifo, isibini kwi nqanaba kwizicwangciso ukukhulelwa kuyimfuneko Kwathi zonke iimvavanyo, lihlaleni iimvavanyo eziyimfuneko athethane yincutshe. Abo neziphakamiso ezifanelekayo ezo zibini, kuquka omnye wabalingane okanye izihlobo zabo kufutshane ubunzima yeeFom ze-Johnson syndrome.

Le axilongwe

Indawo, esi sifo kuqhelekile kakhulu phakathi kwamaJuda Iranian. Inani efikelela 70% ye ubunzima zizonke izigulane zizifo.

Kubalulekile ukuba phantse akukho ezisongela ubomi yeeFom ze-Johnson syndrome. izigulane UMZEKELO iqinisekisa oku. Oogqirha bahlola iminyaka yobomi kwezigulane nesi sifo kwaye wagqiba ukuba akukho nto ikhokelele ekufeni.

Malunga ne-25% ezigulane iintlungu kwi hypochondrium ekunene. Ubume kwesibindi alichatshazelwa, akukho deformation kwi onaso. Kodwa ngexesha elifanayo ezintweni zayo kubonakala amabala amnyama, nto leyo umzimba utshintsha umbala wayo esintsundu eluhlaza okanye ezimnyama. Olu phawu lo sifo kuthiwa chocolate chip cookies. Le isiphumo kuzuzwa a secretion ukophulwa metabolites.

Isifo uba ALS kakuhle, nangona kunjalo uyakwazi ukuqhubeka kuyo ubomi umguli.

Le theyibhile ingezantsi ibonisa data malunga nobume izibilini zezigulane.

ekuxilongweni

YeeFom ze-Johnson syndrome, lokufunyaniswa leyo ethatha kuqhubeka ngokwezigaba eziliqela, kumiselwa ihlaziya.

• Kunyanzelekile ukuba ultrasound esiswini.
• igazi jikelele kunye nomchamo uvavanyo.
• Uhlalutyo igazi nomchamo ukuze yibilirubin. Ngenxa umthetho, ukuba phezulu kakhulu.
• Esampuli fenobarbetalom.
• Amagosa angabevi kwesibindi.
• bromsulfaleinova Sample.
• Coproporphyrin ixabiso izikhombisi kwi umchamo ngosuku.
• Umxholo enzyme egazini.

Sebenzisa ezinye iindlela kokuxilongwa.

Phambi yeeFom ze-Johnson izigulane syndrome kufuneka atyelele therapist.

Treatment kunye nokuthintela

YeeFom ze-Johnson syndrome, apho unyango singabandakanyi abafumana ucwangciso olukhethekileyo elawulwa amanyathelo othintelo:

• ukunamathela ukutya engqongqo, ukusetyenziswa iivithamini B, non-kwisigcinakaliso;
• ukuncipha kwiimeko uxinzelelo;
• ukuncitshiswa umsebenzi ngokomzimba;
• ukwala iziselo ezinxilisayo;
• esweni rhoqo yamanqanaba yibilirubin ngaphezulu kobomi le ubomi yomguli.

Kuyimfuneko ukuba ukukhumbula ukuba ukukhulelwa ukuze andzise sifo. Iziyobisi ukunceda ukunyanga esi sifo yeeFom ze-Johnson, okwangoku kuphuhliswa.

Ngokufanayo, nohlobo sifo Rotor syndrome.

inkcazelo emfutshane

Rotor syndrome kwakhona sisifo ilifa esichaphazela isibindi. utshintsho ivavanywa xa ukunikezelwa yibilirubin, imisebenzi enzyme kuhlala eqhelekileyo. Sinokuthi isifo luhlobo okulula yeeFom ze-Johnson syndrome echazwe apha ngasentla. Iimpawu kwakhona wavakalisa kancinci othambileyo.

Isifo ngazo ukususela ekuzalweni kunye ebuntwaneni. Yibilirubin egazini yomguli kunokuba simahla, wabopha. Kukho umahluko le senyongo.

Rotor syndrome luphawulwa yokuba hepatocytes akukho kokuqulathwe zombala brown. Akukho kukho into ethile, nto leyo eyaziwa njenge chocolate chip cookies. Unobangela sifo yahluke kancinane yeeFom ze-Johnson syndrome. Kulo mzekelo, isibindi abakwazi ukumelana ukufunxeka yibilirubin. Ngenxa yoko, ukusilela undigested kweziyobisi emva egazini. Ngenxa yesi sizathu, kule isiguli giperbulemiya. abantu abane esihle esi sifo.

Ngelo xesha loxilongo lidofreninom kwesibindi, inyongo lwesinyi imibhobho ziyabonakala.

zesifo syndrome

Gns Rotor syndrome sifana kakhulu iimpawu zesifo syndrome yeeFom ze-Johnson:

• Ngokupheleleyo wavakalisa senyongo.
• Imvakalelo ubukrakra emlonyeni.
• Intlungu hypochondrium ekunene.
• Xa yeeFom ze-Johnson syndrome, izigulane zesibindi akhula ukusuka kwisayizi. Kwimeko Rotor syndrome yaligqiba kuphela kwezinye iimeko.
• imigangatho ephakamileyo yibilirubin egazini.
• Iimvavanyo umsebenzi isibindi kuqhelekile.
• iimvavanyo zegazi ngaphandle utshintsho.
• Le bioptome isibindi akukho beqokelela zombala.

Abantu abane ngokubanzi kakuhle, syndrome kwenzeka iminyaka emininzi, hayi kakhulu esichengeni impilo yabantu.

lokufunyaniswa syndrome

Isifo zingasetwa kuphela ebudeni uxilongo olunzulu eziphilayo.

Studies olwenziwa elebhu ukuze atyhile i isiqulatho nangakumbi yibilirubin egazini. Kuba uphethwe kubalulekile ukwazi inani coproporphyrin eziqulethwe yokuchama.

Xa izigulane ezininzi, isifo siqhubela kungekho naziphi na iimpawu.

Kwimeko une Rotor syndrome bachitha uviwo isibindi: wakhe X-ray ultrasound. Kuhlalutyo efanayo baye inyongo lwesinyi imibhobho.

zokuthintela izifo

amanyathelo jikelele othintelo zezi zilandelayo:

• ukuncitshiswa umsebenzi ngokomzimba;
• unyango lwezifo ezinxulumene plane syndrome;
• Xiphephana noxinezeleko ngokweemvakalelo.

Imiba ye ukwanda kwe yibilirubin egazini

ukunyuka yibilirubin ngenxa yezi zizathu zilandelayo:

• okukhulu;
• yokutya engekho mpilweni, ukutya ukungathobeli;
• ukusetyenziswa kweziyobisi;
• ukusetyenziswa ngokugqithisileyo utywala.

unyango lwesifo

iindlela eziqhelekileyo ukuphelisa esi sifo apho. Nakuba kunjalo, kufuneka athobele imiqathango zokuthintela. Njengoko amachiza kufuneka uthathe kuthetha ukuba yibilirubin Imveliso esuka emzimbeni. Bile Kudla machiza asetyenziswa.

Khetha unyango olufanelekileyo ukunceda ugqirha.