Budd-Chiari syndrome

Budd-Chiari syndrome kwenzeka ngenxa nokwaphakade lesichubekako (ukuvalwa noma ukuvaleka) mafutha anciphisa isikhala esingaphakathi we vein nkinga esibindini. Chiari babekholelwa ukuthi isizathu esiyinhloko - yisishiso eyinhloko ngemithambo hepatic. Kodwa kamuva ke kwahlonzwa kwezinye izimo, kuyilapho thrombosis emithanjeni hepatic nabaphansi vena cava.

Lezo zincwadi uchaza cishe 200 amacala abhubhisa endoflebita e hepatic emithanjeni (isifo , Budd-Chiari syndrome).

With ukuthuthukiswa thrombophlebitis arantsieva imbobo, okuyinto ku kwesokunxele vein hepatic uthululela ngqo, umthambo enkabeni, futhi lokhu syndrome zingase ukuthuthukisa ekuqaleni kuka ebuntwaneni. Igobolondo engaphakathi emithanjeni hepatic noma vena phansi kunabaphostoli Vienna (eduze confluence kwesibindi) likhula, ngezinye izikhathi bajoyine amahlule. Konke lokhu kubangela elivalayo noma ukuvaleka mafutha anciphisa isikhala esingaphakathi we vein, okuyinto yena, kubangela ukuthuthukiswa yokujama tsi futhi kokucindezelwa lamaseli isibindi.

Budd-Chiari syndrome futhi ezinye pathologies: neoplasms abulalayo kwesibindi, amanyikwe, inso, njll, izifo semithambo generalized nemvelo. Isifo sokusha nazo zingaba nomthelela the ukuvaleka imithambo hepatic. Chiari syndrome zingase zibangele ngesikhathi kwasendaweni ezithile Gunma echinococcosis, actinomycosis, abscesses nokunye.

Emtholampilo, Budd-Chiari syndrome ububonisa izimpawu zesifo main, yavusa elikhulu ukubonisa eminye futhi sincike ubude occlusion emithanjeni. Ivelele abukhali futhi ukuvela kancane kancane lesi sifo.

Acute hepatic vein occlusion kubangela ukuvela ngasikhathi sinye ukuhlanza, ubuhlungu ngakwesokudla quadrant engenhla noma epigastrium ngenxa ukuvuvukala kwesibindi, kanye hyperextension Glisson capsule, eba ascites, kukhona yellowness encane.

nomfutho wegazi ophezulu Portal eba ngesikhathi esifanayo ngenxa ukwanda okusheshayo e ukugandeleleka sinusoids hepatic kanye emithanjeni.

Ascites nalesi sifo asilapheki nge isisu, uketshezi luyakha kakhulu ngokushesha futhi ngemva paracentesis, ngezinye izikhathi kugujwa engene wegazi lakhe, angagcina hydrothorax. Uma ijoyina thrombosis ukuthi yenzeka bakhiphe elezinga eliphansi vena cava ke kukhona nokuvuvuka wemikhawulo aphansi, "ekhanda Medusa", futhi nge ingcindezi ukukhanya isetshenziswa ke, igazi liya phezulu.

With ukuthuthukiswa hepatic vein occlusion Imininingwane ijoyina thrombosis mesenteric, kukhona ubuhlungu uhudo, ukuxakeka zezinzwa avele ngenxa isibindi cell ukulimala ukuthi ekufeni ezinsukwini ezimbalwa.

Ngezinye izikhathi lesi sifo Budd - Chiari, nokuvalwa olungaphelele mafutha anciphisa isikhala esingaphakathi we vein kungenzeka njengoba isifo esingelapheki isikhathi eside. Nemvelo ezinjalo ukugeleza isikhathi kwenzeka kwegazi eseduze: uvuleke olukha phezulu isikhumba vein, umfutho ophakeme wegazi portal livela, bese ejoyina futhi ngomphimbo varicose emithanjeni.

Ukuze uphethwe khona ebalulekile ingcindezi kwehliswa ngemithambo occlusal isibindi ukwanda ngesikhathi esisodwa ingcindezi portal, okuyinto ibanjiwe esebenzisa izindlela hepato- futhi splenomanometrii. Isetshenziswa rentgenogepatografiya retrograde lapho Ngokuphambene ejenti yethulwe vein hepatic. Ultrasound ukuhlola kusekelwe izimpawu ngqo - ezithwetshuliwe ihlule legazi ngemithambo.

Ngaphandle umbala doplerrovskogo ucwaningo lusikisela Budd-Chiari syndrome ku:

• Ukuba khona izinguquko ngokukhukhumala kwentamo esibindini, nobuntu ezinzima okunamafutha isibindi noma cystic ijwabu isibindi,

• Isithombe kungekho emithanjeni hepatic, uquqaba isibonelo, ngenxa yokuba khona e inesikhala yabo senanele-HIV mafutha anciphisa isikhala esingaphakathi,

• ukuvaleka bakhiphe elezinga eliphansi vena cava,

• izimpawu nomfutho wegazi ophezulu portal, uma lesi sifo isikhathi eside.