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1.
ABCD (São Paulo, Online) ; 36: e1740, 2023. graf
Article Dans Anglais | LILACS | ID: biblio-1447009

Résumé

ABSTRACT BACKGROUND: Liver transplantation represents the best therapeutic modality in end-stage chronic liver disease, severe acute hepatitis, and selected cases of liver tumors. AIMS: To describe a double retransplant in a male patient diagnosed with Crohn's disease and complicated with primary sclerosing cholangitis, severe portal hypertension, and cholangiocarcinoma diagnosed in the transplanted liver. METHODS: A 48-year-old male patient diagnosed with Crohn's disease 25 years ago, complicated with primary sclerosing cholangitis and severe portal hypertension. He underwent a liver transplantation in 2018 due to secondary biliary cirrhosis. In 2021, a primary sclerosing cholangitis recurrence was diagnosed and a liver retransplantation was indicated. Recipient's hepatectomy was very difficult by reason of complex portal vein thrombosis requiring extensive thromboendovenectomy. Intraoperative ultrasound with liver doppler evaluation was performed. Two suspicious nodules were incidentally diagnosed in the donor's liver and immediately removed for anatomopathological evaluation. RESULTS: After pathological confirmation of carcinoma, probable cholangiocarcinoma, at frozen section, the patient was re-listed as national priority and a new liver transplantation was performed within 24 hours. The patient was discharged after 2 weeks. CONCLUSIONS: The screening for neoplasms in donated organs should be part of our strict daily diagnostic arsenal. Moreover, we argue that, for the benefit of an adequate diagnosis and the feasibility of a safer procedure, the adoption of imaging tests routine for the liver donor is essential, allowing a reduction of the costs and some potential risks of liver transplant procedure.


RESUMO RACIONAL: O transplante de fígado representa a melhor modalidade terapêutica na doença hepática crônica terminal, hepatite aguda grave e casos selecionados de tumores hepáticos. OBJETIVOS: Descrever um retransplante duplo em paciente do sexo masculino, diagnosticado com doença de Crohn e complicado com colangite esclerosante primária, hipertensão portal grave e colangiocarcinoma diagnosticado no fígado transplantado. MÉTODOS: Paciente do sexo masculino, 48 anos, diagnosticado com doença de Crohn há 25 anos e complicado com colangite esclerosante primária e hipertensão portal grave. Foi submetido a um transplante de fígado em 2018 devido a cirrose biliar secundária. Em 2021, foi diagnosticada recidiva de colangite esclerosante primária e indicado retransplante hepático. A hepatectomia do receptor foi de alta complexidade devido à trombose complexa da veia porta, exigindo extensa tromboendovenectomia. Foi realizada ultrassonografia intraoperatória com doppler hepático. Dois nódulos suspeitos foram diagnosticados incidentalmente no fígado do doador e imediatamente removidos para avaliação anatomopatológica. RESULTADOS: Após confirmação patológica de carcinoma, provável colangiocarcinoma, pela congelação, o paciente foi relistado como prioridade nacional, e novo transplante hepático foi realizado em 24 horas. O paciente teve alta após 2 semanas. CONCLUSÕES: O rastreamento de neoplasias em órgãos doados deve fazer parte de nosso estrito arsenal diagnóstico diário. Além disso, defendemos que, em benefício de um diagnóstico correto e da viabilidade de um procedimento mais seguro, a adoção de uma rotina de exames de imagem é essencial em doadores hepáticos, permitindo a redução dos custos e alguns riscos potenciais do procedimento de transplante hepático.


Sujets)
Humains , Mâle , Adulte d'âge moyen , Tumeurs des canaux biliaires/chirurgie , Angiocholite sclérosante/chirurgie , Maladie de Crohn/complications , Transplantation hépatique , Cholangiocarcinome/chirurgie , Cholangiocarcinome/imagerie diagnostique , Réintervention , Tumeurs des canaux biliaires/anatomopathologie , Tumeurs des canaux biliaires/imagerie diagnostique , Conduits biliaires intrahépatiques , Angiocholite sclérosante/étiologie , Cholangiocarcinome/anatomopathologie , Échographie-doppler , Donneur vivant , Hypertension portale/étiologie
2.
Arch. argent. pediatr ; 114(3): e187-e191, jun. 2016. ilus
Article Dans Espagnol | LILACS, BINACIS | ID: biblio-838224

Résumé

Niña de 10 años con diarrea, abdominalgia, pérdida de peso y febrícula de un mes y medio de evolución. Los hallazgos analíticos y ecográficos hicieron sospechar una enfermedad inflamatoria intestinal. La endoscopía y la histología mostraron hallazgos compatibles con la enfermedad de Crohn. Se inició un tratamiento con mesalazina y nutrición enteral exclusiva, y se anadieron corticoides, inmunosupresores y ácido ursodesoxicólico por la persistencia de colestasis e hipergammaglobulinemia. La colangiorresonancia y la biopsia hepática confirmaron el diagnóstico de colangitis esclerosante primaria concomitante. La asociación entre la enfermedad de Crohn y la colangitis esclerosante primaria es muy poco frecuente; predomina en varones de entre los 20 y los 40 años de edad; y presenta una gran variabilidad clínica. El diagnóstico de confirmación requiere la realización de una colangiorresonancia o colangiopancreatografía retrógrada endoscópica. El pronóstico es malo y no existe ningún tratamiento capaz de frenar la progresión de la enfermedad.


A 10 year old girl with diarrhea, abdominal pain, weight loss and fever of one month and a half of evolution. Analytical and sonographic findings raised the possibility of inflammatory bowel disease. Endoscopy and histology showed findings consistent with Crohn's disease. Treatment was initiated with mesalazine and exclusive enteral nutrition. Later corticosteroid treatment, immunosuppressive drugs and ursodeoxycholic acid were added due to cholestasis and persistent hypergammaglobulinemia. Magnetic resonance cholangiography and liver biopsy confirmed the diagnosis of concomitant primary sclerosing cholangitis. The association between Crohn's disease and primary sclerosing cholangitis is rare, predominantly in males between 20 and 40 years old and it presents a great clinical variability. The confirmation of the diagnosis requires magnetic resonance cholangiography or endoscopic retrograde cholangiopancreatography. The prognosis is poor and there is no treatment to slow the progression of the disease.


Sujets)
Humains , Femelle , Enfant , Angiocholite sclérosante/étiologie , Maladie de Crohn/complications
3.
The Korean Journal of Gastroenterology ; : 49-54, 2013.
Article Dans Anglais | WPRIM | ID: wpr-46503

Résumé

BACKGROUND/AIMS: Benign biliary strictures (BBSs) have been endoscopically managed with plastic stent placement. However, data regarding fully covered self-expandable metal stents (FCSEMSs) in BBS patients remain scarce in Korea. METHODS: Forty-one patients (21 men, 65.9%) with BBSs underwent FCSEMS placement between February 2007 and July 2010 in Asan Medical Center. Efficacy and safety were evaluated retrospectively. Patients were considered to have resolution if they showed evidence of stricture resolution on cholangiography and if an inflated retrieval balloon easily passed through the strictures at FCSEMS removal. RESULTS: The mean FCSEMS placement time was 3.2 (1.9-6.2) months. Patients were followed for a mean of 10.2 (1.0-32.0) months after FCSEMS removal. The BBS resolution rate was confirmed in 38 of 41 (92.7%) patients who underwent FCSEMS removal. After FCSEMS removal, 6 of 38 (15.8%) patients experienced symptomatic recurrent stricture and repeat stenting was performed. When a breakdown by etiology of stricture was performed, 14 of 15 (93.3%) patients with chronic pancreatitis, 17 of 19 (89.5%) with gall stone-related disease, 4 of 4 (100%) with surgical procedures, and 2 of 2 (100%) with BBSs of other etiology had resolution at FCSEMS removal. Complications related to stent therapy occurred in 12 (29%) patients, including post-ERCP pancreatitis (n=4), proximal migration (n=3), distal migration (n=3), and occlusion (n=2). CONCLUSIONS: Temporary FCSEMS placement in BBS patients offers a potential alternative to plastic stenting. However, because of the significant complications and modest resolution rates, the potential benefits and risks should be evaluated in further investigations.


Sujets)
Adulte , Sujet âgé , Femelle , Humains , Mâle , Adulte d'âge moyen , Maladies des canaux biliaires/thérapie , Cholangiopancréatographie rétrograde endoscopique , Angiocholite sclérosante/étiologie , Sténose pathologique , Calculs biliaires/étiologie , Pancréatite/étiologie , Études rétrospectives , Endoprothèses/effets indésirables , Facteurs temps
5.
Rev. chil. radiol ; 18(4): 184-189, 2012. ilus
Article Dans Espagnol | LILACS | ID: lil-665609

Résumé

In HIV-infected patients, liver and biliary tract may be affected by different entities, such as AIDS-cholangiopathy, which is usually associated with CD4+T lymphocytes count below 100 cells/mm³ along with non-specific symptoms. Although imaging studies play a major diagnostic role they should always be evaluated according to patients clinical context. While ultrasound and CT scans provide relevant diagnostic information, specialized studies such as MRI and MRCP have become increasingly valuable due to their ability to demonstrate parietal as well as stenotic biliary changes. Despite ERCP remains the gold-standard for diagnosis of AIDS-related cholangiopathy, currently it is usually reserved to clearly exclude the presence of malignancy, or as a specific invasive therapeutic procedure, when indicated.


En pacientes infectados con VIH, el hígado y las vías biliares pueden presentar compromiso de distinta índole. Dentro de éstos, la colangiopatía asociada al SIDA se presenta con una sintomatología más bien inespecífica y habitualmente con recuentos de linfocitos T CD4 <100/mm³. En este escenario, si bien los estudios de imagen juegan un rol muy relevante, éstos siempre deben ser evaluados en relación al contexto clínico del paciente. Si bien la ecografía y la TC aportan valiosa información en el diagnóstico, en la actualidad la resonancia magnética de abdomen combinada con colangioresonancia tienen gran utilidad, por ser capaces de demostrar tanto las alteraciones parietales, como los cambios morfológicos estenóticos biliares de esta patología. Si bien la ERCP aún conserva su valor de gold standard para el diagnóstico, actualmente la tendencia se dirige a reservarla para el descarte de neoplasias, en casos dudosos o como procedimiento terapéutico invasivo específico, cuando sea indicado.


Sujets)
Jeune adulte , Angiocholite sclérosante/diagnostic , Angiocholite sclérosante/étiologie , Maladie des voies biliaires/diagnostic , Maladie des voies biliaires/étiologie , Syndrome d'immunodéficience acquise/complications , Imagerie par résonance magnétique , Infections opportunistes liées au SIDA , Cholangiopancréatographie rétrograde endoscopique
6.
GED gastroenterol. endosc. dig ; 27(4): 101-106, jul/ago 2008.
Article Dans Portugais | LILACS | ID: lil-506594

Résumé

A colangite esclerosante primária (CEP) é uma doença hepática colestática crônica caracteriza»da por estenoses da árvore biliar intra e extra»hepática, que pode causar cirrose e colangiocar»cinoma em 10% a 30% dos pacientes. Comu»mente está associada à doença inflamatória intestinal e pode causar morte por insuficiência hepática. A CEP é considerada doença hepática imunomediada, multifatorial, multigenética de causa desconhecida. CPRE e colangiopancreato»grafia-ressonância são recomendadas para o diag»nóstico de CEPo O clássico achado em "contas de rosário" é resultado de áreas multifocais de estenoses de duetos biliares intra ou extra-hepá»ticas, intercalados com segmentos normais ou di»latados. Citologia de duetos biliares com esteno»ses e dados histológicos de material obtidos por punção possibilitam o diagnóstico de colangio»carcinoma. Não há tratamento eficaz para a CEPo O ácido ursodesoxicólico pode reduzir as eleva»ções de enzimas hepáticas; seu efeito sobre a his»tologia hepática e o prognóstico são inconclusi»vos. O transplante hepático continua sendo o único tratamento comprovado a longo prazo para a CEP, porém, com recorrência em 20% a 40% dos pacientes.


Sujets)
Humains , Animaux , Mâle , Cholangiocarcinome/diagnostic , Angiocholite sclérosante/étiologie , Angiocholite sclérosante/thérapie , Cholangiographie , Cholangiopancréatographie par résonance magnétique , Angiocholite sclérosante/diagnostic , Hormones corticosurrénaliennes/usage thérapeutique , Tests hématologiques , Lithotritie , Transplantation hépatique , Pronostic , Tomoscintigraphie , Tomodensitométrie , Acide ursodésoxycholique
8.
Rev. Soc. Bras. Med. Trop ; 38(5): 438-441, set.-out. 2005. ilus
Article Dans Portugais | LILACS | ID: lil-409482

Résumé

Relatamos o caso de um paciente com icterícia obstrutiva cuja causa era colangite esclerosante secundária à síndrome da imunodeficiência adquirida. Na colangiopatia relacionada à síndrome da imunodeficiência adquirida, os pacientes apresentam dor no quadrante superior direito ou epigástrico, febre, diarréia e alterações biliares características. O paciente foi submetido à plastia transversa do ducto hepático comum por via laparoscópica.


Sujets)
Humains , Mâle , Adulte , Syndrome d'immunodéficience acquise/complications , Angiocholite sclérosante/étiologie , Cholangiographie , Angiocholite sclérosante/anatomopathologie , Angiocholite sclérosante/chirurgie , Conduit hépatique commun/chirurgie , Laparoscopie/méthodes
11.
Rev. colomb. gastroenterol ; 6(3): 195-8, jul.-sept. 1991. ilus, tab
Article Dans Espagnol | LILACS | ID: lil-221576

Résumé

Presentamos dos casos de colangitis esclerosante secundaria. En el primero, la enfermedad fue consecuencia de litiasis biliar y, en el segundo, la colangitis esclerosante se presentó en el contexto de un paciente con diarrea crónica y SIDA. Se discuten los principales aspectos etiológicos y fisipatológicos


Sujets)
Humains , Mâle , Adulte , Adulte d'âge moyen , Angiocholite sclérosante , Angiocholite sclérosante/étiologie , Angiocholite sclérosante/physiopathologie
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