Search

Hepatocellular adenoma is a rare benign liver tumor that occurs predominantly in young women who use exogenous estrogens. We describe a case of a 40-year-old woman on birth control pill who presented with acute right hypochondrial pain and signs of hemodynamical instability triggered by a large bleeding tumor in the right liver lobe. Arterial embolization was performed with cessation of bleeding. To determine etiology, magnetic resonance imaging was conducted with findings suggestive of a hepatocellular adenoma. The tumor was surgically resected, and histologic examination made the definite diagnosis of an inflammatory hepatocellular adenoma. This is a noteworthy case of a rare but potentially fatal complication of liver tumors, whose diagnosis requires a high index of suspicion. Clinicians should consider this diagnosis in young women on oral contraceptive pills who present with severe unexplained abdominal pain and hemodynamical instability. This case aimed to raise awareness of this condition, while reviewing important aspects concerning the management of hepatocellular adenomas.

Introduction: The outbreak of coronavirus disease 2019 (COVID-19) had affected clinical practice in several ways, including the restriction of nonessential endoscopic procedures. Therefore, our aim was to evaluate how colorectal cancer (CRC) diagnosis and management was affected during the first year of pandemics in Portugal. Methods: This is a Portuguese substudy of the French retrospective multicentric study ETICC (Etude de l’Impact de la pandémie COVID-19 sur le diagnostic et la prise en charge du Cancer Colorectal). We compared patients’ characteristics, clinical manifestations, CRC staging at diagnosis, delay to first medical appointment, histological diagnosis, surgical and medical treatments between the year previous to the pandemics (control) and the first year of pandemics. Results: We included 766 patients: 496 in the control group and 270 in the COVID group. There was no significant difference in CRC staging at diagnosis between both groups, with 21% being diagnosed as metastatic in the control group and 22% in the first year of pandemics (p = 0.770). Contrary to what happened in France, there was a significant decrease in CRC diagnosis in asymptomatic patients (25–8.4%; p < 0.001) and after a positive fecal immunochemical test (20.8–11.3%; p = 0.002) during the pandemics. Although the increase in the overall complication rate at diagnosis was nonsignificant, in Portugal, there was a significant increase in diagnosis of abdominal occlusion (12.1–18.1%; p = 0.033). In Portugal, time between the beginning of symptoms and the first medical appointment significantly increased from a median of 50 days to 64 days during COVID (p < 0.001). On the contrary, time between histological diagnosis and tumor resection had significantly decreased from a median of 65 to 39 days (p < 0.001). Time between histological diagnosis and neoadjuvant treatment was not statistically different (median of 64–67 days; p = 0.590), as was time between histological diagnosis and palliative chemotherapy (median of 50–51 days; p = 1.000). Time from CRC resection and adjuvant treatment has significantly decreased from a median of 54 to 43 days (p = 0.001). Discussion: We found a significant impact in CRC diagnosis in the first year of pandemics, more pronounced than what was found in France. These are likely related not only with the closing of endoscopy units but also with the difficulties patients had in finding an appointment with their general practitioners. On the other hand, both in France and Portugal, the first year of pandemics did not worsen CRC staging at diagnosis and did not significantly affect medical and surgical treatments once the diagnosis was made.

Severe acute liver injury (ALI) is mostly triggered by viral infections and hepatotoxic drugs; however, it can also be seen in systemic diseases. Hemophagocytic lymphohistiocytosis (HLH) is a rare, immune-mediated syndrome that presents as a life-threatening inflammatory disorder affecting multiple organs. Secondary causes occur mainly in the set of malignancy, infection, and autoimmune disease, and are seldom triggered by vaccination. Although liver involvement is common, presentation as severe ALI is rare. We describe a case of a 65-year-old male with history of low-risk chronic lymphocytic leukemia and rheumatoid arthritis treated with prednisolone who presented with persistent fever and jaundice 1 week after COVID-19 vaccination. The diagnosis was challenging given the predominant liver impairment, characterized by hyperbilirubinemia, transaminases over 1,000 U/L, and prolonged INR, which prompted an extensive investigation and exclusion of autoimmune, toxic, and viral causes of hepatitis. Laboratory workup revealed bicytopenia, hyperferritinemia, which together with organ failure and evidence of hemophagocytosis in bone marrow suggested the diagnosis of HLH. After excluding infectious etiologies, flare of rheumatological disease, and the progression of hematological disease, HLH was diagnosed. He was successfully treated with etoposide and corticosteroids, with dramatic improvement of liver tests. After exclusion of other causes of secondary HLH, the recent vaccination for COVID-19 was the likely trigger. We report a case of double rarity of HLH, as it presented with severe liver dysfunction which was probably triggered by vaccination. In this case, the predominant liver involvement urged extensive investigation of liver disease, so a high index of suspicion was required to make an early diagnosis. Clinicians should consider HLH in patients with unexplained signs and symptoms of systemic inflammatory response and multiorgan involvement, including severe liver involvement as the first presentation.

Cholestatic liver diseases may be associated with increased plasmatic cholesterol due to an abnormal lipoprotein – lipoprotein X (LpX). Correcting the underlying cause of cholestasis is the critical treatment of LpX-associated hypercholesterolemia without any proven benefit from conventional lipid-lowering agents. In some situations, plasma exchange may apply to prevent associated complications, such as hyperviscosity syndrome. The authors present the case of a 44-year-old man with orbital inflammatory pseudotumor on prednisolone, admitted due to hepatocellular and cholestatic lesion and severe hypercholesterolemia. Laboratory investigation established that hepatitis E virus was responsible for liver injury and showed that LpX mediated the severe hypercholesterolemia. Reduction of the immunosuppressive load contributed to virus clearance. The consequent resolution of cholestasis and cholesterol removal by plasmapheresis allowed lipid profile normalization. The authors report the first case of LpX-associated hypercholesterolemia in a patient with hepatitis E-induced cholestasis and revisit the role of the liver in lipid metabolism.

Background/Aims: Inflammatory bowel disease (IBD)-related knowledge empowers patients, providing the development of adaptative coping strategies. Recently, a more comprehensive questionnaire for evaluating IBD-related knowledge was developed, the IBD-KNOW. The main aim of our study was to translate to Portuguese and validate the IBD-KNOW questionnaire. We also explored the predictors of high scores of disease-related knowledge and the effect of knowledge on health-related quality of life (HRQoL) and therapeutic adherence. Methods: This is an observational, unicentric, and cross-sectional study. We translated and adapted the original English version of the IBD-KNOW questionnaire into Portuguese. Afterwards, IBD patients in the outpatient clinics were invited to fill out a multimodal form including the Portuguese version of IBD-KNOW, a visual analogue scale (VAS) of self-perceived knowledge, the Portuguese version of Short IBD Questionnaire (SIBDQ) and the Portuguese version of Morisky Adherence Scale 8-item (MMAS-8). Demographic and disease characteristics were collected. We assessed validity (through discriminate validity among non-IBD volunteers and correlation between IBD-KNOW and VAS) and reliability (through internal consistency, test-retest, and intraclass correlation). Statistical analysis was performed using SPSS version 25.0. Results: The mean IBD-KNOW score was significantly different among non-IBD validation group (doctors: 23, nurses: 18, and non-medical volunteers: 12, p < 0.001). IBD-KNOW showed a high internal consistency (Cronbach’s α 0.78) and intraclass correlation (0.90). As expected, the IBD-KNOW score was positively correlated with VAS for self-perceived knowledge (r = 0.45, p < 0.001). One hundred and one patients with IBD (54 with ulcerative colitis and 47 with Crohn’s disease) completed the questionnaire at baseline. Multivariate analyses showed that a high IBD-KNOW score was associated with longer disease duration (OR: 2.59 [CI 1.11–5.74]; p = 0.04), previous hospitalization (OR: 3.63 [CI 1.301–9.96]; p = 0.01), current biologic treatment (OR: 3.37 [CI 1.31–8.65]; p = 0.02), and higher educational level (OR: 4.66 [CI 1.74–10.21]; p = 0.02). Moreover, there was no significant correlation between overall IBD-KNOW and SIBDQ, nor between IBD treatment adherence (MMAS-8 = 8) and a higher mean IBD-KNOW score (p = 0.552). Conclusion: The Portuguese version of IBD-KNOW is a simple, valid, and reliable tool for assessing IBD-related knowledge. Longer disease duration, hospitalization, use of biologics, and higher educational level are associated with higher levels of knowledge. Higher patient knowledge was not associated with higher HRQoL and adherence to therapy.

Hepatocellular carcinoma is a common malignancy usually associated with cirrhosis. Despite being a highly aggressive tumor with several cases of vascular invasion, metastatic disease to the heart is a rare condition. A 65-year-old male cirrhotic patient was admitted with dyspnea, ascites, and lower extremity edema. A transthoracic echocardiogram showed a large mass in the right atrium. Further imaging studies revealed the presence of hepatocellular carcinoma extending from the liver to the right atrium through the inferior vena cava. The cardiac mass was surgically removed to treat the symptoms of right heart failure, but unfortunately the patient died on the 30th day after surgery due to septic shock.

Abstract Background Primary hepatic lymphoma is a very uncommon disease. Due to its nonspecific clinical, laboratory, and imaging findings, it is often misdiagnosed. Liver biopsy is required to make a final diagnosis. Chemotherapy is the current gold standard of treatment. Case presentation An asymptomatic 65-year-old Caucasian man with Child-Pugh class A cirrhosis presented to our hospital with a nodular lesion seen on a routine surveillance abdominal ultrasound. His physical examination revealed hepatomegaly and no other significant findings. Magnetic resonance imaging of the abdomen showed a voluminous nodule on the left lobe with heterogeneous contrast enhancement. His liver biopsy was compatible with diffuse large B-cell lymphoma. Systemic staging showed no evidence of nodal or bone marrow involvement, confirming the diagnosis of primary hepatic lymphoma. He was treated with chemotherapy. However, he developed febrile neutropenia after one of the cycles and died. Conclusions In this article, we report a rare presentation of non-Hodgkin lymphoma and review the current literature on clinical features, diagnosis, and management.

Este estudo qualitativo teve como objetivo analisar as perceções de alunos adolescentes acerca dos aspetos que facilitam e dificultam a sua aprendizagem. O conhecimento das perceções dos alunos sobre a sua aprendizagem permite melhorar a qualidade dos processos de ensino-aprendizagem. A partir de um guião semiestruturado, foram realizados cinco grupos focais, em cinco escolas privadas do Norte de Portugal, tendo participado 32 alunos selecionados aleatoriamente. Os participantes eram alunos do 3º ciclo do Ensino Básico (7º, 8º e 9º anos), com idades compreendidas entre os 12 e os 15 anos. A análise dos dados seguiu um processo semi-indutivo de codificação descritiva. Os resultados realçam o papel fundamental do professor na aprendizagem; a importância da ação vs passividade dos alunos na sala de aula; a relevância da dimensão emocional e relacional para o envolvimento do aluno; e a relevância das estratégias de autorregulação da aprendizagem. Como implicações sugere-se a concretização de ações junto de professores e alunos assente nos dados recolhidos e analisados, que sejam promotoras da metacognição de ambos.