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Cholangiopathy has been described in survivors of severe COVID-19, presenting significant clinical parallels to the pre-pandemic condition of secondary sclerosing cholangitis in critically ill patients (SSC-CIP). We aimed to examine the liver histopathology of individuals with persistent cholestasis after severe COVID-19. METHODS: We subjected post-COVID-19 cholestasis liver samples to routine staining techniques and cytokeratin 7 immunostaining and semi-quantitatively analyzed the portal and parenchymal changes. RESULTS: All ten patients, five men, had a median age of 56, an interquartile range (IQR) of 51-60, and required intensive care unit and mechanical ventilation. The median and IQR liver enzyme concentrations proximal to biopsy were in IU/L: ALP 645 (390-1256); GGT 925 (664-2169); ALT 100 (86-113); AST 87 (68-106); and bilirubin 4 (1-9) mg/dL. Imaging revealed intrahepatic bile duct anomalies and biliary casts. We performed biopsies at a median of 203 (150-249) days after molecular confirmation of infection. We found portal and periportal fibrosis, moderate-to-severe ductular proliferation, and bile duct dystrophy in all patients, while we observed hepatocyte biliary metaplasia in all tested cases. We observed mild-to-severe parenchymal cholestasis and bile plugs in nine and six cases. We also observed mild swelling of the arteriolar endothelial cells in five patients. We observed a thrombus in a small portal vein branch and mild periductal fibrosis in one case each. One patient developed multiple small biliary infarctions. We did not observe ductopenia in any patient. CONCLUSIONS: The alterations were like those observed in SSC-CIP; however, pronounced swelling of endothelial cells, necrosis of the vessel walls, and thrombosis in small vessels were notable.
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Introduction: Post-COVID-19 cholangiopathy is a novel condition characterized by biliary tract sclerosis and elevated alkaline phosphatase levels in critically ill patients. This case series aims to describe the experience of a Latin American reference hospital in managing this condition. Methods: This case series includes patients with confirmed coronavirus disease 2019 (COVID-19) who exhibited subsequent elevation of alkaline phosphatase levels exceeding three times the normal value. The patients also had documented bile duct abnormalities observed through cholangioresonance or endoscopic retrograde cholangiopancreatography (ERCP). The clinical presentation, imaging findings, complications, and treatment approaches are described. Results: Eight patients (56.5 ± 9.2 years old, 62.5% male) were included in the study. All patients had previously experienced severe acute respiratory syndrome coronavirus type 2 (SARS-CoV-2) pneumonia and required mechanical ventilation. Four patients (50%) received sedoanalgesia with ketamine, and all eight patients (100%) received propofol. All patients developed infections, such as cholangitis or liver abscesses, caused by gram-negative bacteria. The peak alkaline phosphatase level during follow-up averaged 1646.12 ± 611.3. Imaging findings revealed intrahepatic (100%) and extrahepatic (87.5%) bile duct dilation. In 75% of cases, bile molds with a black appearance were extracted. Seven patients experienced recurrent cholangitis, and three patients were referred for pre-liver transplant consultation. Conclusions: Post-COVID-19 cholangiopathy is characterized by severe cholestasis, intra- and extrahepatic bile duct dilation, formation of bile molds, and recurrent cholangitis. In our study, a possible association between sepsis caused by gram-negative bacteria and the use of sedative medications is hypothesized. Further studies are necessary to establish the most appropriate management strategies for these patients, as they currently face unfavorable long-term morbidity and mortality outcomes.
Introducción: la colangiopatía pos-COVID-19 es una entidad nueva caracterizada por esclerosis del tracto biliar y elevación de fosfatasa alcalina en el paciente críticamente enfermo. Esta serie de casos describe la experiencia de un hospital de referencia en Latinoamérica. Métodos: serie de casos que incluye a pacientes con enfermedad por coronavirus de 2019 (COVID-19) confirmada y elevación posterior de fosfatasa alcalina > 3 veces del valor normal, asociados a anormalidades en las vías biliares documentada por colangiorresonancia o colangiopancreatografía retrógrada endoscópica (CPRE). Se describe la presentación clínica, hallazgos imagenológicos, complicaciones y tratamiento. Resultados: se incluyeron a 8 pacientes (56,5 ± 9,2 años, 62,5% hombres). Todos presentaron neumonía por coronavirus del síndrome respiratorio agudo grave de tipo 2 (SARS-CoV-2) previo a desarrollo de colangiopatía, y requirieron ventilación mecánica. Cuatro pacientes (50%) recibieron sedoanalgesia con ketamina y ocho (100%) con propofol. Todos presentaron infecciones (colangitis/abscesos hepáticos) por bacterias gramnegativas. El nivel pico de fosfatasa alcalina durante el seguimiento fue en promedio 1646,12 ± 611,3. Los hallazgos imagenológicos incluyeron dilatación de la vía biliar intrahepática (100%) y extrahepática (87,5%). En el 75% se extrajo el molde biliar de apariencia negra. Siete pacientes presentaron recurrencia de colangitis y tres pacientes fueron referidos a consulta de pretrasplante hepático. Conclusiones: la colangiopatía pos-COVID-19 está caracterizada por colestasis grave con dilatación de la vía biliar intra- y extrahepática, formación de moldes biliares y colangitis recurrente. En nuestro estudio se plantea como hipótesis una posible relación con sepsis por bacterias gramnegativas y uso de medicamentos sedativos. Se requieren nuevos estudios para establecer el manejo más adecuado para estos pacientes, que hasta el momento presentan una morbimortalidad desfavorable a largo plazo.
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The case of a 57-year-old male patient with jaundice, high-grade fever, and upper abdominal pain who was recovering from a mild coronavirus disease-19 (COVID-19) infection is reported. Laboratory analysis showed liver injury with high levels of AST and ALT, as well as an elevated serum ferritin level. The patient underwent a bone marrow biopsy which showed features of hemophagocytic lymphohistiocytosis (HLH), a systemic syndrome caused by immune activation. The patient was successfully treated with etoposide and dexamethasone and kept on maintenance therapy with cyclosporine, with resolution of the HLH. The discussion highlights that COVID-19 infection may cause liver injury, and in severe cases, patients may develop HLH as a cause for liver injury. The incidence of HLH in adults with severe COVID-19 infection is estimated to be lower than 5%. The association between HLH and COVID-19 infection has been studied due to immunological hyperactivation. Signs such as persistent high fever, hepatosplenomegaly, and progressive pancytopenia should raise suspicion for the diagnosis of overlapping HLH. A specific approach using steroids and etoposide, followed by maintenance therapy with cyclosporine, is proposed in the HLH-94 protocol as the mainstay of treatment. It is suggested that HLH should be suspected in patients with laboratory signs of liver injury following COVID-19 infection, especially in patients with high-grade fever and a history of rheumatic conditions.
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COVID-19 , Ciclosporinas , Linfo-Histiocitose Hemofagocítica , Masculino , Adulto , Humanos , Pessoa de Meia-Idade , Linfo-Histiocitose Hemofagocítica/diagnóstico , Linfo-Histiocitose Hemofagocítica/tratamento farmacológico , Linfo-Histiocitose Hemofagocítica/etiologia , COVID-19/complicações , Etoposídeo/uso terapêutico , Medula Óssea , FebreRESUMO
Since the spread of the first cases of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection much progress has been made in understanding the disease process. However, we are still facing the complications of coronavirus disease 19 (COVID-19). Multiple sequelae may appear as a consequence of acute infection. This set of entities called post-COVID-19 syndrome involves a wide variety of new, recurrent or persistent symptoms grouped together as a consequence of the acute disease process. One of those that has attracted the most attention is the liver and bile duct involvement called post-COVID-19 cholangiopathy. This is characterized by elevation of liver markers such as alkaline phosphatase, bilirubin and transaminases as well as alterations in the bile ducts in imaging studies. Thus, a narrative review of the cases reported until the end of 2021 was carried out. From the findings found, we concluded that patients who have had COVID-19 or during the process have required hospitalization should remain under follow-up for at least 6 months by a multidisciplinary team.
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COVID-19 , Colangite Esclerosante , Humanos , Colangite Esclerosante/diagnóstico , Síndrome de COVID-19 Pós-Aguda , COVID-19/complicações , SARS-CoV-2 , Ductos BiliaresRESUMO
BACKGROUND: The global coronavirus disease 2019 (COVID-19) pandemic has caused more than 5 million deaths. Multiorganic involvement is well described, including liver disease. In patients with critical COVID-19, a new entity called "post-COVID-19 cholangiopathy" has been described. CASE SUMMARY: Here, we present three patients with severe COVID-19 that subsequently developed persistent cholestasis and chronic liver disease. All three patients required intensive care unit admission, mechanical ventilation, vasopressor support, and broad spectrum antibiotics due to secondary infections. Liver transplant protocol was started for two of the three patients. CONCLUSION: Severe COVID-19 infection should be considered a potential risk factor for chronic liver disease and liver transplantation.
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Liver compromise in critically ill patients with coronavirus disease 2019 (COVID-19) is common but usually transient and self-limited. However, liver tests on some patients continue to show abnormal results. Herein, a 29-year-old patient with clinical and histological features of cholangiopathy is presented. Despite treatment with ursodeoxycholic acid and cholestyramine, bilirubin and transaminase levels remained elevated. This case report raises awareness of the difficulty of managing this condition in patients with COVID-19.
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We present the case of a 28-year-old man with recently-diagnosed human immunodeficiency virus and hepatitis C virus infection. He developed obstructive cholangiopathy secondary to cytomegalovirus and Kaposi sarcoma, both diagnosed by endoscopic retrograde cholangiopancreatography and biopsies. He received antiretroviral therapy, chemotherapy and valganciclovir with full recovery.
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Infecções Oportunistas Relacionadas com a AIDS/diagnóstico , Terapia Antirretroviral de Alta Atividade/métodos , Doenças dos Ductos Biliares/etiologia , Infecções por HIV/tratamento farmacológico , Valganciclovir/uso terapêutico , Dor Abdominal/etiologia , Adulto , Doenças dos Ductos Biliares/diagnóstico , Biópsia , Colangiopancreatografia Retrógrada Endoscópica , Citomegalovirus , Diarreia , Febre/etiologia , Infecções por HIV/diagnóstico , Hepatite C/complicações , Humanos , Masculino , Sarcoma de Kaposi , Redução de PesoRESUMO
OBJECTIVES: To report baseline features and long-term medical/social outcomes of juvenile autoimmune liver disease, including autoimmune hepatitis (AIH) and autoimmune sclerosing cholangitis (ASC), managed in a single tertiary center. STUDY DESIGN: Retrospective study of children diagnosed in 2000-2004 with AIH/ASC followed up to date. Patients with abnormal cholangiogram were classified as ASC. Presentation and outcome features were compared. RESULTS: Eighty-three children were included (42 female, median age 12.1 years [8.5-14.1 years], AIH = 54, ASC = 29). Most (65%) had antinuclear and/or anti-smooth muscle autoantibodies; 6% presented with acute liver failure; 29% had histologic evidence of cirrhosis. The 1999 and simplified International Autoimmune Hepatitis Group criteria failed to diagnose up to 26% of patients with AIH and 48% with ASC, and the proposed the European Society for Pediatric Gastroenterology, Hepatology and Nutrition criteria were accurate. Response to treatment was excellent with 95% achieving normal transaminase levels. During follow-up, 31% had at least 1 relapse episode; 3 patients with AIH developed cholangiopathy and 5 patients with ASC developed progressive bile duct injury. At last follow-up (median of 14.5 years, 10.4-16.8), 99% were alive, 11 underwent transplantation and 1 is listed for transplant. Five-, 10-, and 15-year transplant-free survival rates were 95%, 88%, and 83%; patients with ASC and those relapsing being more likely to require transplant. Social outcome was excellent with 93% in employment/education. CONCLUSIONS: Seamless management of juvenile autoimmune liver disease leads to excellent clinical and social outcomes. Despite good response to immunosuppressive treatment, patients with ASC have a worse prognosis than those with AIH. Diagnostic models developed for adults are unsatisfactory to correctly diagnose juvenile autoimmune liver disease.
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Colangite Esclerosante/terapia , Hepatite Autoimune/terapia , Adolescente , Autoanticorpos/imunologia , Criança , Colangite Esclerosante/epidemiologia , Continuidade da Assistência ao Paciente , Emprego , Feminino , Seguimentos , Hepatite Autoimune/epidemiologia , Humanos , Terapia de Imunossupressão , Imunossupressores/uso terapêutico , Estimativa de Kaplan-Meier , Transplante de Fígado , Masculino , Equipe de Assistência ao Paciente , Período Pós-Operatório , Recidiva , Estudos Retrospectivos , Centros de Atenção Terciária , Fatores de Tempo , Resultado do TratamentoRESUMO
Kawasaki's disease (KD) is a systemic vasculitis often seen with viral and bacterial infections. Cholangitis is a known complication in biliary atresia patients post Kasai Portoenterostomy (KP). However KD, in a biliary atresia patient post KP has not been previously reported. A 1 years old girl who had previously undergone a KP for BA, presented with cholangitis which was presumed to be caused by a previous enterobacter infection that she had 2 months ago. However, on treating the cholangitis, the patient developed fever again after ten days which persisted even after changing the antibiotics. By this time she also displayed three of five characteristic features of KD in form of fever, strawberry tongue and cervical adenopathy. Investigations showed high ESR, high CRP, thrombocythemia and dilated coronary vessels on echocardiography. Treatment with intravenous immunoglobulin (IVIG) and steroids caused the symptoms to subside.
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Atresia Biliar/cirurgia , Colangite/etiologia , Síndrome de Linfonodos Mucocutâneos/etiologia , Portoenterostomia Hepática/efeitos adversos , Atresia Biliar/diagnóstico , Colangite/diagnóstico , Feminino , Glucocorticoides/uso terapêutico , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Lactente , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Síndrome de Linfonodos Mucocutâneos/tratamento farmacológico , Resultado do TratamentoRESUMO
Resumen El diagnóstico de las colangiopatías obstructivas por endosonografía biliopancreática (EUS, por sus siglas en inglés) se ha estudiado en profundidad, y existe un extenso reconocimiento de su papel para el diagnóstico de coledocolitiasis, tumores de la encrucijada biliopancreática y síndromes de compresión extrínseca de la vía biliar. La colangitis, la complicación más frecuente de la obstrucción biliar y responsable de gran parte de su morbimortalidad, no se ha relacionado en forma suficiente con los hallazgos endosonográficos. Nuestro estudio buscó definir la validez diagnóstica de la endosonografía en los pacientes con colangitis aguda con el fin de limitar la morbimortalidad del retraso diagnóstico. Se realizó un estudio descriptivo, analizando las historias clínicas de pacientes sometidos a endosonografía biliopancreática, por ictericia obstructiva de cualquier etiología, y quienes posteriormente fueron llevados a colangiopancreatografía retrógrada endoscópica (CPRE) para manejo de obstrucción biliar. Comparamos los hallazgos endosonográficos compatibles con colangitis aguda (engrosamiento de la vía biliar de 1,5 mm o más, presencia de halo pericolangítico de al menos 1,5 cm de longitud y presencia de contenido de ecogenicidad mixta en el interior de la vía biliar) con el drenaje purulento durante la CPRE. Se encontró una alta frecuencia de estos hallazgos en los pacientes con colangitis, y el engrosamiento de las paredes de la vía biliar fue el más común, en el 92,6% de los casos, seguido por la presencia de halo pericolangítico en el 59,3% de los casos y contenido ductal en el 66,7% de los casos. Igualmente, se encontró asociación ascendente de las frecuencias con grado de severidad de la colangitis. Resulta recomendable realizar estudios con un mayor poder estadístico que permitan validar nuestros resultados para una futura inclusión de la EUS en el algoritmo diagnóstico de las guías de práctica clínica en colangitis aguda.
Abstract Diagnosis of obstructive cholangiopathy with biliopancreatic endosonography (BUS) has been extensively studied, and its role in the diagnosis of choledocholithiasis, biliopancreatic junction tumors and extrinsic biliary compression syndromes is widely recognized. Endosonographic diagnosis of cholangitis, a more frequent complication of biliary obstruction which is responsible for much of its morbidity and mortality, has not been sufficiently studied. Our study's objective is to define the diagnostic validity of endosonography for patients with acute cholangitis in order to limit morbidity and mortality due to diagnostic delay. This is a descriptive study that analyzes clinical histories of patients who underwent biliopancreatic endosonography because of obstructive jaundice of any etiology and who later underwent endoscopic retrograde cholangiopancreatography (ERCP) to manage biliary obstruction. We compared endosonographic findings compatible with acute cholangitis: thickening of the bile duct of 1.5 mm or more, periportal halo sign of at least 1.5 cm in length, mixed echogenicity of bile duct content, and purulent drainage during ERCP. Patients with cholangitis most frequently presented thickened gallbladders (92.6% of cases) periportal halo signs (59.3% of cases) and mixed echogenicity of bile duct content (66.7% of cases). We also found an ascending association of frequencies with the degree of severity of cholangitis. Studies with greater statistical power are needed for validation of our results and for future inclusion of EUS in the diagnostic algorithm for clinical practice guidelines in cases of acute cholangitis.
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Colangite , Endossonografia , Colestase , Radioterapia Guiada por ImagemRESUMO
This paper describes a case of Caroli's disease in a female patient aged 32, who complained of nonspecific abdominal pain without cholesthasis or cholangitis. Liver resonance shows segment saccular dilations closely connected to intrahepatic biliary ducts, that differ from the Caroli's syndrome, which is more common and consists of multiple intrahepatic cystic dilatations, associated to congenital hepatic fibrosis. This patient has a congenital anomaly with an uncommon oligosymptomatic form of Caroli's disease that should be included in the differential diagnosis of patients with recurrent abdominal pain.
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The present report describes the case of a child that after blunt abdominal trauma presented with portal thrombosis followed by progressive splenomegaly and jaundice. Ultrasonography and percutaneous cholangiography revealed biliary dilatation secondary to choledochal stenosis caused by dilated peribiliary veins, characterizing a case of portal biliopathy. The present case report is aimed at presenting an uncommon cause of this condition.
Relata-se o caso de uma criança que após trauma abdominal fechado apresentou trombose portal, seguida por esplenomegalia progressiva e icterícia. Os achados da ultrassonografia e da colangiografia percutânea mostraram dilatação de vias biliares secundária à constrição do colédoco por veias pericoledocianas dilatadas, configurando caso de colangiopatia portal. O objetivo deste relato é a apresentação de causa incomum desta condição.