Dubin-Johnson syndrome: incazelo, uphethwe, izimpawu kanye nokwelashwa

Naphezu kweqiniso lokuthi inqubo yokwelapha yesimanje seyenze inqubekelaphambili enkulu kanye nezifo eziningi Akasekho isigwebo for the iziguli, ezinye zalezi zifo akukaqondakali ngokugcwele. Omunye wabo Dubin-Johnson syndrome. Umtholampilo, ukuxilongwa kanye nokwelashwa yalesi sifo edalwa bezempilo kwezinye isithakazelo. Cabanga ngalo mbuzo kabanzi.

Rotor syndrome ne Dubin-Johnson syndrome - ezingavamile lezinceku isifo sesibindi Okufanayo jaundice sixhumene.

izimbangela

Dubin-Johnson syndrome linenye igama: enzimopaticheskaya jaundice isibindi. It kuvela ngokuqondene ukuhlukumeza we ukulethwa phakathi Bile emangqamuzaneni kwesibindi bilirubin.

Le nto ivela emzimbeni ukuze kususwe therefrom erythrocyte noma icekelwe phansi ngokuphelele. Isiphetho bilirubin kwenzeka ngezindlela ezimbili:

• ukukhulisa inani lemithi iza ngokusebenzisa Bile;
• nengcosana umchamo ukuya.

Dubin-Johnson syndrome kwenzeka uma kwaphazamisa ukuthuthwa bilirubin nge Bile. Lena isifo ezingavamile kakhulu ekuthonyweni ngaphezulu izinsizwa (mayelana 20-30 iminyaka). amacala lesi sifo kusukela ekuzalweni ngezinye izikhathi zenzeka. Ingozi yokutheleleka ngemva kweminyaka engu-umuntu eminyakeni engu-50 noma ngaphezulu incane.

Imbangela zofuzo yalesi sifo, Dubin-Johnson kungenxa kuguquka isakhi sofuzo, bahlanganyele nokukhishwa bilirubin esibindini namaprotheni ikhodi.

izimpawu

Dubin-Johnson syndrome, izimpawu zazo yilezi nobuntu ezinye izifo ezifana, isibonelo, ukukhathala kanye inezici ayo esiwuphawu:

• ubuhlungu izimbambo ngakwesokudla;
• ukungabakhathaleli nhlobo ukudla;
• kungenzeka kube khona ukulunywa okuncane kwesikhumba;
• isibindi lakhulisiwe;
• colic, ngezinye izikhathi kwandisa;
• ukuhlanza;
• lokushisa ukwanda.

Izenzo kuthiwa iyakhula ngenxa yezifo ezithathelwanayo, sindisa obukhulu emzimbeni nangokomzwelo. Ukuze abesifazane, kungenzeka okubi kakhulu ngemva edonsa nokukhandela imbeleko.

iqembu ingozi

Ngisiphi isiqhema sabantu kufanele uqaphele sifo?

Lesi sifo sitholwa njengefa. Uma umndeni nginezingane nalesi sifo, umbhangqwana at the stage of ukuhlela yokukhulelwa kuyadingeka ukuba kudlule bonke ukuhlolwa, ovivinyweni olubangelwa ezidingekayo futhi uthintane nge onguchwepheshe. Labo izincomo ezifanelekayo labo imibhangqwana, kuhlanganise omunye abangane bawo bomshado noma zabo eziseduze baye bahlupheka Dubin-Johnson syndrome.

Inqubo zokuxilonga

Ayisebenzi, lesi sifo singumbulali ezivamile phakathi kwamaJuda Iranian. Isibalo sawo sifinyelela 70% isisindo isamba neziguli.

Kubalulekile ukuthi cishe akukho ezisongela ukuphila Dubin-Johnson syndrome. iziguli ISIBONELO iyakufakazela lokhu. Odokotela bahlola isilinganiso sobude bokuphila iziguli lesi sifo futhi waphetha ngokuthi awusebenzi ziholele ekufeni.

Mayelana 25% eziguli ukuzwa ubuhlungu hypochondrium kwesokudla. Isakhiwo isibindi engabathinti, akukho ukusonteka e sifo. Kodwa ngesikhathi esifanayo ezicutshini zayo avele namabala amnyama, okuholela umzimba kushintsha umbala yayo nsundu luhlaza noma amnyama. Lesi sici yalesi sifo libizwa chocolate chip cookies. Lo mphumela ibangelwa a uketshezi ukwephulwa metabolites.

Lesi sifo has a Izibikezelo evumayo, yize-ke ungaqhubeka kuyo yonke impilo isiguli.

Ithebula elingezansi ibonisa idatha ngesimo izibilini iziguli.

ukuhlola

Dubin-Johnson syndrome, sitholakele ebanjwa ngezigaba eziningana, kunqunywa yizenzo.

• Kuphoqelekile ukuthi ultrasound esiswini.
• ukuhlolwa kwegazi Jikelele kanye nomchamo.
• Ukuhlaziya egazi noma omchamo ukuze bilirubin. Njengomthetho, ziphakeme kakhulu.
• Amasampula fenobarbetalom.
• Abamakayo zokusha kwesibindi.
• bromsulfaleinova Sample.
• Coproporphyrin ukubaluleka izinkomba umchamo ngosuku.
• Okuqukethwe enzyme egazini.

Sebenzisa ezinye izindlela ukuxilongwa.

Ephambi kwezinduna Dubin-Johnson iziguli syndrome zinenkinga ukuvakashela owelapha.

Ukulapha nokuvikela

Dubin-Johnson syndrome, okuyinto ukwelashwa aluhileli ethola amalungiselelo akhethekile kulawulwa izinyathelo:

• ukunamathela ukudla okuqinile, ukusetshenziswa B amavithamini, okungezona nezivimbelakuvunda;
• ukunciphisa ezimweni ukucindezeleka;
• ukunciphisa umzimba;
• ukwenqaba uphuzo oludakayo;
• zokuqapha njalo amazinga bilirubin ngokuhamba kwesikhathi ekuphileni ukuphila kwesiguli.

Kuyadingeka ukukhumbula ukuthi ukukhulelwa kuphazamisa babhebhethekisa lesi sifo. Izidakamizwa ukusiza ukwelapha lesi sifo Dubin-Johnson, okwamanje athuthukile.

Ngokufanayo, ubunjalo sifo Rotor syndrome.

Incazelo emfushane

Rotor syndrome futhi kuyinto esiwufuzo esithinta isibindi. izinguquko asitholayo ekwethuleni bilirubin, enzyme imisebenzi ahlale evamile. Singasho ukuthi lesi sifo singumbulali ifomu engasindi Dubin-Johnson syndrome kuchaziwe ngenhla. Izimpawu Yaveza kancane ethambile.

Lesi sifo engaveza njengoba kusukela nokuzalwa nokukhula. Bilirubin egazini isiguli kungaba mahhala futhi lesibopho. Kukhona umehluko omkhulu we gallbladder.

Rotor syndrome libhekene iqiniso lokuthi hepatocytes akukho okuqukethwe zombala ensundu. Hhayi kukhona umphumela, okuyinto njengoba chocolate chip cookies ezaziwayo. Imbangela sifo ukuhluka Dubin-Johnson syndrome. Kulokhu, isibindi akakwazi ukubhekana ukumuncwa bilirubin. Ngenxa yalokho, ukwehluleka undigested imfuyo emuva lingene egazini. Kulesi sisekelo, Isiguli eba giperbulemiya. Izibikezelo Evumayo lesi sifo.

Ngesikhathi uphethwe lidofreninom kwesibindi, nenyongo sesinye futhi Amapayipi ziyabonakala.

izimpawu syndrome

Izimpawu Rotor syndrome kuyafana izimpawu sifo Dubin-Johnson:

• Ngokugcwele uzwakalise jaundice.
• Umuzwa umunyu umlomo.
• Ubuhlungu hypochondrium kwesokudla.
• Lapho Dubin-Johnson syndrome, iziguli isibindi anda ubukhulu. Endabeni Rotor syndrome luyanda kuphela kwezinye izimo.
• Amazinga aphakeme bilirubin egazini.
• umsebenzi ukuhlolwa isibindi ijwayelekile.
• ukuhlolwa kwegazi ngaphandle kwezinguquko.
• I bioptome isibindi kungekho ekuqongeleleni zombala.

Izibikezelo ngokuvamile evumayo, lesi sifo kwenzeka eminyakeni eminingi eyadlula, hhayi kakhulu kokuyekethisa empilweni yomuntu.

sitholakele lesi sifo

Lesi sifo angasethwa kuphela ngesikhathi uphethwe olunzulu umzimba.

Ucwaningo olwenziwa e-laboratory ukusiza wasembula okuqukethwe lelitfutfukile bilirubin egazini. Ukuze kuhlonzwe kubalulekile ukwazi isibalo coproporphyrin eziqukethwe umchamo.

In iziguli eziningi, isifo siqhubeka ngaphandle izimpawu.

Endabeni sitholakele Rotor syndrome ukuchitha isibindi luhlolo: wakhe X-ray kanye ultrasound. Ekuhlaziyeni efanayo ibhekane nenyongo sesinye futhi mapayipi.

ekuvimbeleni izifo

izinyathelo Jikelele zokuvimbela zimi kanje:

• ukunciphisa umzimba;
• ekwelapheni izifo ezihambisana Rotor syndrome;
• Gwema ukucindezeleka nokucindezeleka okungokomzwelo.

Ingabe Zikhona Izimo Ezenza ukwanda of bilirubin egazini

Bilirubin inyuka ngenxa yezizathu ezilandelayo:

• kwengcindezi enkulu;
• nokudla okungenamsoco, ukudla ukwephulwa;
• ukusetshenziswa kwezidakamizwa,
• ngokuphuza utshwala ngokweqile.

ekwelapheni lesi sifo

izindlela ezivamile ukuqeda isifo khona. Nokho, kumelwe uhambisane izinyathelo. Njengoba imithi udinga ukuthatha kusho ukuthi bilirubin okukhiphayo kusuka emzimbeni. Bile ngokuvamile asetshenziselwa izidakamizwa.

Khetha ukwelashwa okufanele ukusiza udokotela.