Post-COVID-19 Secondary Sclerosing Cholangitis: A Rare but Severe Condition with no Treatment Besides Liver Transplantation.

BACKGROUND The incidence of abnormal liver function, mainly aspartate aminotransferase and alanine aminotransferase elevations, in patients with COVID-19 is not uncommon, but persistent liver damage after the acute phase of the disease is uncommon and has been recently recognized as a new entity named post-COVID-19 cholangiopathy. CASE REPORT We report a clinical case with progressive cholestatic disease following severe COVID-19. AST and ALT peaked at hospital admission and while its serum concentration went down, bilirubin and cholestatic liver enzymes started to increase, reaching the maximum at day 122. Magnetic resonance imaging (MRI) revealed a diffuse irregularity of intra- and extrahepatic bile ducts, with multiple focal strictures alternating with mild focal dilations of the biliary tree, suggesting a sclerosing cholangiopathy. A transjugular liver biopsy showed a prominent bile ductular reaction, cholangiocyte injury, inflammatory infiltrate rich in neutrophils, biliary infarctions, marked cholestasis, and portal fibrosis, suggesting the diagnosis of post-Covid-19 secondary sclerosing cholangitis. The patient evolved with a continuous deterioration of liver functions, but liver transplantation was not performed due to his poor clinical condition. CONCLUSIONS Post-COVID-19 SSC is a severe disease with no effective clinical treatment and has liver transplantation as the only treatment for a few selected patients.

Association between Metabolic Disorders and Cholangiocarcinoma: Impact of a Postulated Risk Factor with Rising Incidence.

Introduction and objectives: The incidence of cholangiocarcinoma (CCA) has been increasing globally. Although a concomitant increase in the incidence of metabolic disorders might suggest a causal relationship, the data are scarce. We aimed to describe the prevalence of metabolic disorders in patients with CCA and report the clinical features and outcomes. Patients and Methods: Retrospective study including patients with CCA. Patients were divided into: (1) past history of diabetes or/and overweight/obesity ("metabolic disorder group") and (2) without any of these features ("non-metabolic-disorder group"). A Cox regression model was used to determine the prognostic factors. Results: 122 patients were included. In total, 36 (29.5%) had overweight/obesity, 24 (19.7%) had diabetes, and 8 (6.6%) had both. A total of 29 (23.8%) patients had resectable disease and received upfront surgery. A total of 104 (85.2%) received chemotherapy for advanced/recurrent disease. The overall survival of the cohort was 14.3 months (95% CI: 10.1−17.3). ECOG-PS 0 (p < 0.0001), resectable disease (p = 0.018) and absence of vascular invasion (p = 0.048) were independently associated with better prognosis. The "metabolic disorder group" (n = 52) had a median survival of 15.5 months (95% CI 10.9−33.9) vs. 11.5 months (95% CI 8.4−16.5) in the "non-metabolic-disorder group" (n = 70) (HR: 1.10; 95% CI 0.62−1.94). Patients with resectable disease in the "metabolic group" had longer survival than patients in the "non-metabolic group" (43.4 months (95% CI 33.9-NR) vs. 21.8 months (95% CI 8.6−26.9); HR = 0.12, 95% CI 0.03−0.59). Conclusion: Metabolic disorders are frequent among CCA patients. Underlying metabolic comorbidities may be associated with prognosis in resectable CCA. There is a need to explore the mechanism that drives CCA carcinogenesis in a metabolic background.

Liver Surgery: Important Considerations for Pre- and Postoperative Imaging.

Liver surgery may be a curative treatment option not only for primary liver neoplasms but also for liver metastases in selected patients. The number of liver surgeries performed worldwide has increased, but surgical morbidity associated with these surgeries remains significant. Therefore, radiologists need to understand the terminology, surgical techniques, resectability and unresectability criteria, and possible postoperative complications as these are part of the decision-making process. Because vascular and biliary variations are common, an adequate preoperative anatomic evaluation determines the best surgical technique, helps identify patients in whom additional surgical steps will be required, and reduces the risk of inadvertent injury. The surgeon must ensure that the future liver remnant is sufficient to maintain adequate function, aided by the radiologist who can provide valuable information such as the presence of steatosis, biliary dilatation, signs of cirrhosis, and portal hypertension, in addition to the volume of the future liver remnant. Postoperative complications must also be understood and evaluated. The most common postoperative complications are vascular (bleeding, thrombosis, and ischemia), biliary (fistulas, bilomas, and strictures), infectious (incisional or deep), those related to liver failure, and even tumor recurrence. An invited commentary by Winslow is available online. Online supplemental material is available for this article. ©RSNA, 2022.

Fatores associados ao controle da asma em pacientes pediátricos em centro de referência / Factors associated with asthma control in a pediatric reference center

OBJETIVO:Descrever os dados epidemiológicos, clínicos e os fatores associados ao controle da asma em pacientes asmáticos seguidos em um ambulatório pediátrico especializado. MÉTODOS: Estudo transversal de pacientes asmáticos ambulatoriais, para os quais eram fornecidas medicações profiláticas. A classificação da asma, as etapa de tratamento e a avaliação do controle seguiram a IV Diretrizes Brasileiras para o Manejo da Asma, de 2006. Os fatores avaliados foram: outras alergias, obesidade, imunodeficiências, refluxo gastresofágico e sensibilização alérgica, sendo comparados pacientes com asma controlada ou não com relação à etapa do tratamento, à obesidade e à sensibilização alérgica. RESULTADOS: Foram analisados 300 pacientes com asma (1,38M:1F), com mediana de idade de 10,8 anos, e de início dos sintomas de 1,0 ano. A atopia estava presente em 78 por cento dos pais e/ou irmãos. Antecedentes pessoais de doenças alérgicas ocorreram em 292 pacientes (96 por cento rinite, 27 por cento dermatite atópica, 18 por cento conjuntivite alérgica, 6 por cento alergia alimentar). Foram diagnosticados sete casos de deficiência de IgA (DIgA) e quatro de IgG2; obesidade em 37/253 (15 por cento), sendo que sobrepeso e obesidade estiveram associados à falta de controle (p=0,023). Em 118 pacientes com multissensibilização, predominaram casos não controlados (22 (19 por cento) casos; p=0,049; OR 1,9; IC95 por cento 1,03-3,50). Entre os 180 casos (60 por cento) em tratamento nas etapas 3 e 4, 122 (45 por cento) estavam controlados e 112 (41 por cento) parcialmente controlados. CONCLUSÕES: A rinite foi a alergia mais associada à asma e a prevalência de DIgA foi 20 vezes maior do que na população geral. O controle parcial ou total dos sintomas da asma foi obtido em 85 por cento dos casos. Obesidade/sobrepeso e multissensibilização foram associadas à falta de controle da asma.

OBJECTIVE:To describe the epidemiological and clinical data and factors associated with asthma control of asthmatic patients followed at a pediatric reference center. METHODS: Cross-sectional study including asthmatic outpatients receiving prophylactic medications from the institution. For classification of asthma, steps of treatment and control evaluation, the IV Brazilian Guidelines for the Management of Asthma (2006) were adopted. The presence of other allergies, obesity, immunodeficiency, gastroesophageal reflux and allergic sensitization were evaluated and compared between patients with or without asthma control according to the treatment step, obesity and allergic sensitization. RESULTS: 300 patients with asthma (1.38M:1F) were included; median age=10.8 years; median age at onset of symptoms=1.0 year. Among parents and/or siblings, 78 percent reported atopy. Personal history showed other allergic diseases in 292 patients (96 percent rhinitis, 27 percent atopic dermatitis, 18 percent allergic conjunctivitis, 6 percent food allergy). IgA deficiency was diagnosed in seven cases and IgG2 deficiency in four. Obesity was noted in 34/233 patients (15 percent) and overweight/obesity were associated with asthma poor control (p<0.023). Among 118 patients multisensitizated, the frequency of poor asthma control was greater (22 (19 percent) cases; p=0.049, OR 1.9; 95 percentCI 1.03-3.50). Among the patients, 180 (60 percent) were treated according steps 3 and 4; 122 children (45 percent) were considered controlled and 112 (41 percent) were partially controlled. CONCLUSIONS: Allergic rhinitis was the allergic disease more associated with asthma. The prevalence of IgA deficiency was 20 times higher than in the general population. Total or partial control of asthma symptoms was obtained in 85 percent of the cases. Overweight/obesity and multisensitization were associated to poor asthma control.

Postoperative outcome of patients with prosthetic valve leak.

BACKGROUND: Prosthetic valve leak is a possible complication of surgical valve replacement. Although uncommon, its consequences may be serious. Few studies correlate the degree of prosthetic valve leak with clinical events. OBJECTIVE: To compare the postoperative outcome of patients with mild/moderate (Mi/Mo) or severe (Sev) prosthetic valve leak METHODS: A total of 185 patients with prosthetic valve leak were selected among 1350 patients undergoing heart valve surgery between 1999 and 2001. Of these, a sample of 58 patients (37 men) with prosthetic valve leak (36 with Mi/Mo versus 22 with Sev leak) in the pre and/or postoperative period of heart valve replacement had complete medical record data, so their clinical, laboratory and echocardiographic data could be retrospectively assessed. RESULTS: The incidence of reoperation was 11.1% in the Mi/Mo group, versus 22.7% in the Sev group (odds ratio = 2.35 [95% CI 0.56-9.94]). Endocarditis was the cause of reoperation in 75% of the patients of the Mi/Mo group and in 60% of the Sev group. Aortic bioprostheses were those most frequently related to leak (55.8% in the Mi/Mo group and 57.7% in the Sev group). Forty percent of the patients with previous Mi/Mo leak did not present prosthetic valve leak on postoperative day 2 versus 21.4% of the patients with Sev prosthetic valve leak. No significant differences were found regarding laboratory variables. CONCLUSIONS: (1) Patients with severe leak are more likely to undergo reoperation. (2) Endocarditis was the most frequent cause of reoperation for any leak degree. (3) Severe prosthetic valve leak is more difficult to fully resolve after surgical treatment.

Evolução pós-operatória de pacientes com refluxo protético valvar / Evolución postoperatoria de pacientes con reflujo protésico valvular / Postoperative outcome of patients with prosthetic valve leak

FUNDAMENTO: O refluxo de prótese valvar é uma possível complicação da cirurgia de troca valvar. Embora raro, suas consequências podem ser graves. Há poucos estudos que correlacionam o grau do refluxo de prótese valvar com os eventos clínicos dos pacientes. OBJETIVO: Comparar a evolução pós-operatória de pacientes com refluxo de prótese valvar discreto/moderado (D/M) ou importante (Imp). MÉTODOS: Dentre 1.350 pacientes submetidos a cirurgia valvar entre 1999 e 2001, foram selecionados 185 pacientes com refluxo de prótese valvar. Desses pacientes, foram avaliados retrospectivamente dados clínicos, laboratoriais e ecocardiográficos de uma amostra de 58 pacientes (37 homens) com refluxo de prótese valvar no pré e/ou no pós-operatório de troca valvar com dados completos em prontuários, sendo 36 com refluxo D/M versus 22 com refluxo Imp. RESULTADOS: A incidência de reoperação foi de 11,1 por cento no Grupo D/M versus 22,7 por cento no Grupo Imp (odds ratio = 2,35 [IC95 por cento 0,56-9,94]). Endocardite foi a causa de reoperação em 75 por cento dos pacientes do Grupo D/M e em 60 por cento do Grupo Imp. As biopróteses aórticas foram as mais acometidas por refluxo (55,8 por cento no Grupo D/M e 57,7 por cento no Grupo Imp). Evoluíram sem refluxo de prótese valvar no segundo pós-operatório 40 por cento dos pacientes com refluxo prévio D/M versus 21,4 por cento dos pacientes com refluxo de prótese valvar Imp. Não houve diferenças significantes nas variáveis laboratoriais. CONCLUSÕES: (1) Os portadores de refluxo importante têm maior probabilidade de reoperação. (2) Endocardite foi a causa mais frequente de reoperação para qualquer grau de refluxo. (3) O refluxo de prótese valvar importante é de mais difícil resolução completa após tratamento cirúrgico.

BACKGROUND: Prosthetic valve leak is a possible complication of surgical valve replacement. Although uncommon, its consequences may be serious. Few studies correlate the degree of prosthetic valve leak with clinical events. OBJECTIVE: To compare the postoperative outcome of patients with mild/moderate (Mi/Mo) or severe (Sev) prosthetic valve leak METHODS: A total of 185 patients with prosthetic valve leak were selected among 1350 patients undergoing heart valve surgery between 1999 and 2001. Of these, a sample of 58 patients (37 men) with prosthetic valve leak (36 with Mi/Mo versus 22 with Sev leak) in the pre and/or postoperative period of heart valve replacement had complete medical record data, so their clinical, laboratory and echocardiographic data could be retrospectively assessed. RESULTS: The incidence of reoperation was 11.1 percent in the Mi/Mo group, versus 22.7 percent in the Sev group (odds ratio = 2.35 [95 percent CI 0.56-9.94]). Endocarditis was the cause of reoperation in 75 percent of the patients of the Mi/Mo group and in 60 percent of the Sev group. Aortic bioprostheses were those most frequently related to leak (55.8 percent in the Mi/Mo group and 57.7 percent in the Sev group). Forty percent of the patients with previous Mi/Mo leak did not present prosthetic valve leak on postoperative day 2 versus 21.4 percent of the patients with Sev prosthetic valve leak. No significant differences were found regarding laboratory variables. CONCLUSIONS: (1) Patients with severe leak are more likely to undergo reoperation. (2) Endocarditis was the most frequent cause of reoperation for any leak degree. (3) Severe prosthetic valve leak is more difficult to fully resolve after surgical treatment.

FUNDAMENTO: El reflujo de prótesis valvular es una posible complicación de la cirugía de reemplazo valvular. Aunque raras, sus consecuencias pueden resultar severas. Hay pocos estudios que correlacionan el grado del reflujo de prótesis valvular con los eventos clínicos de los pacientes. OBJETIVO: Comparar la evolución postoperatoria de pacientes con reflujo de prótesis valvular leve/moderado (L/M) o severo (S). MÉTODOS: Teniendo en cuenta a los 1.350 pacientes sometidos a la cirugía valvular entre el 1999 y el 2001, se seleccionaron a 185 pacientes con reflujo de prótesis valvular. De ellos, se evaluaron retrospectivamente datos clínicos, laboratoriales y ecocardiográficos de una muestra de 58 pacientes (37 varones) con reflujo de prótesis valvular en el pre y/o en el postoperatorio de reemplazo valvular con datos completos en prontuarios, con 36 presentado reflujo L/M versus 22 con reflujo S. RESULTADOS: La incidencia de reoperación fue del 11,1 por ciento en el Grupo L/M versus el 22,7 por ciento en el Grupo S (odds ratio = 2,35 [IC95 por ciento 0,56-9,94]). La Endocarditis fue la causa de reoperación en el 75 por ciento de los pacientes del Grupo L/M y en el 60 por ciento del Grupo S. Las bioprótesis aórticas fueron las más afectadas por reflujo (el 55,8 por ciento en el Grupo L/M y el 57,7 por ciento en el Grupo S). Evolucionaron sin reflujo de prótesis valvular en el segundo postoperatorio el 40 por ciento de los pacientes con reflujo previo L/M versus el 21,4 por ciento de los pacientes con reflujo de prótesis valvular S. No hubo diferencia significantes en las variables laboratoriales. CONCLUSIÓN: 1) Los portadores de reflujo severo tienen mayor probabilidad de reoperación. 2) Endocarditis fue la causa más frecuente de reoperación para cualquier grado de reflujo. 3) El reflujo de prótesis valvular severo es de más difícil resolución completa tras tratamiento quirúrgico.