A Case of Porto-Sinusoidal Vascular Disease.

Introduction: Porto-sinusoidal vascular syndrome is characterised by specific histological changes that do not include cirrhosis, with or without portal hypertension. Patients are usually asymptomatic until development of portal hypertension complications. Case description: A 69-year-old female with history of JAK2 positive essential thrombocythemia (ET) was referred to internal medicine consultation due to elevated liver enzymes. The patient had no previous history of liver disease. Seven months earlier, she had an ischaemic stroke and started treatment with atorvastatin. After discontinuing medication, liver enzymes returned to normal and atorvastatin-related drug-induced liver disease (DILI) was presumed.During a follow-up visit, iron deficiency anaemia was detected and an endoscopic study was performed. It revealed a gastric varix actively bleeding, which was successfully treated with cyanoacrylate.Two months later, the patient was admitted due to a new episode of variceal bleeding, and a portal hypertension complementary study was made. Discussion: Although the pathogenesis of porto-sinusoidal vascular disease (PSVD) remains poorly understood, vascular changes within the liver have been associated with several predisposing conditions, such as hypercoagulable states. Patients with ET, especially those with JAK2 mutation, are known to be at increased risk of non-cirrhotic vein thrombosis. Concerning PSVD, the association is not clear but it is believed that both PSVD and myeloproliferative neoplasms share a common denominator: a state characterised by hypercoagulability, inflammation, endothelial dysfunction and, in some cases, portal hypertension. Conclusion: Portal hypertension without cirrhosis is a rare condition, presenting diagnostic challenges and significant impact on the patient's prognosis. LEARNING POINTS: The suspicion of PSVD should be raised when signs of portal hypertension are present with normal or mildly elevated liver enzymes and normal liver stiffness measurement. A liver biopsy should be performed in this situation.Although the pathogenesis of PSVD is not clearly understood, it is based on the development of vascular changes within the liver and there might be several predisposing conditions such as coagulation disorders.

Infection of Multiple Tunneled Dialysis Catheters Resulting From the Contamination of the Chlorhexidine Solution by Serratia marcescens.

Tunnelled dialysis catheters continue to be a choice in several patients as hemodialysis access. According to Kidney Disease Outcomes Quality Initiative guidelines, its handling implies disinfection, that can be performed using chlorhexidine solutions. Theoretically, these solutions have bactericidal capacity at concentrations greater than 0.12%. We present a curious situation of failure of the antiseptic process due to contamination of the chlorhexidine solution 4% of aqueous base. In this hemodialysis clinic, three cases of infections by the bacteria Serratia marcescens were identified over 2 weeks - in two of the cases, identified in blood culture, and in the other case in the exudate from the exit site of the catheter. Considering the abnormal number of infections by this agent and the fact that these patients were on different shifts, were treated in different rooms, and handled by different nurses, the antiseptic solutions used in the different hemodialysis rooms were analyzed, as well as a closed package from the same batch. After microbiological tests were performed on the antiseptic solution, we identified the growth of Serratia marcescens. This result identified the culprit as being the contamination of the 4% chlorhexidine solution. The competent authorities were notified, and the disinfection method was changed to use a chlorhexidine alcohol-based solution.

An Incidental Myxoma Hidden in Chest Pain.

Chest pain is a very common symptom in an emergency context. Its differential diagnosis is extensive and includes some conditions that require immediate recognition and intervention. It can also be a symptom associated with rarer diagnostic possibilities. Here, we report the case of a 53-year-old woman admitted to the emergency department due to chest pain and initially diagnosed with non-ST elevation acute myocardial infarction. A transthoracic echocardiogram revealed a large hyperechogenic round mass, suggestive of a left atrial cardiac myxoma. Coronary angiography showed no significant lesions. The patient underwent cardiac surgery with excision of the mass, whose histological diagnosis was atrial myxoma. The immediate postoperative period was uneventful, and the patient was discharged asymptomatic and without echocardiographic changes. Cardiac tumors are a rare finding, of which myxomas are the most common. Symptoms can typically result from embolism, obstruction, or constitutional symptoms. A myxoma presenting as acute chest pain and mimicking an acute coronary syndrome is an uncommon finding. This case reminds us of an extremely rare differential diagnosis of chest pain and awakens us to the usefulness and importance of using echocardiography as a diagnostic tool.

A Compelling Case of Autoimmune Hemolytic Anemia and Its Potential Association With SARS-CoV-2.

Autoimmune hemolytic anemia can be caused by infections, lymphoproliferative disorders, autoimmune disorders, or triggered by drugs or toxins. We present the case of a 92-year-old man admitted with gastrointestinal symptoms. He presented with autoimmune hemolytic anemia. The etiologic study was negative for autoimmune conditions or solid masses. Viral serologies were negative, and RT-PCR for SARS-CoV-2 was positive. The patient began treatment with corticoid, with resulted in cessation of hemolysis and improvement of the anemia. A few cases of autoimmune hemolytic anemia have been reported in COVID-19 patients. In this case, the infection seems to coincide with the hemolysis period, and we found no other cause for this event. So, we highlight the need to search for SARS-CoV-2 as a possible infective cause of autoimmune hemolytic anemia.

A Rare Image of Bladder Herniation Concealed in a Frequent Diagnosis.

Herniation of the urinary bladder is a relatively uncommon condition, and when it herniates to the scrotum, it can also be called a scrotal cystocele. The incidence of inguinal herniation is estimated to vary between 1% and 3 % of all inguinal hernias. However, scrotal cystocele is believed to occur in less than 1% of cases and only 7% are diagnosed preoperatively. We present a case of a 91-year-old male who presented to our hospital with dysuria, fever, right scrotal pain, and tumefaction. In the course of the patient's evaluation, a CT scan of the pelvis was performed, which showed a right inguinal hernia constituting the bladder. A hernia reduction was performed, and it led to immediate symptomatic relief. After the procedure, antibiotics were initiated and administrated for the urinary tract infection. Bladder hernia and its extension to the scrotum is rare; therefore, this is a relevant case that may contribute to additional knowledge in the medical community.

A Rare Cause of Low Back Pain: A Challenging Diagnosis.

One of the rarest causes of low back pain is septic arthritis of a lumbar facet joint. We report the case of a 92-year-old diabetic woman with a history of four days of back pain, dysuria, and fever. Due to a sudden worsening of lumbar pain, she went to the emergency department. Physical exam revealed pain with pressure over the D12 vertebral apophyses and the lower-left paraspinal musculature. Laboratory data showed a normochromic normocytic anemia with a hemoglobin of 9.3 g/dL, white cell count of 14.61x10e3/µL (83.1% neutrophils), serum creatinine 1.46 mg/dL and C-reactive protein of 32.11 mg/dL. In urinalysis, nitrites and leukocyturia were identified. CT scan showed an acute D12 fracture and fat stranding at L5, with no irregularities in the discs or in other lumbar spaces. Escherichia coli was isolated in blood culture. Lumbar MRI confirmed the diagnosis of septic arthritis of an L5-S1 facet joint and L5 vertebrae osteomyelitis. The patient was successfully treated with intravenous ceftriaxone for eight weeks. As far as we know, this is the second report of septic arthritis of the facet joint caused by Escherichia coli.

Early Lung Ultrasound Findings in Patients With COVID-19 Pneumonia: A Retrospective Multicenter Study of 479 Patients.

OBJECTIVES: Lung ultrasound (LUS) holds the promise of an accurate, radiation-free, and affordable diagnostic and monitoring tool in coronavirus disease 2019 (COVID-19) pneumonia. We sought to evaluate the usefulness of LUS in the diagnosis of patients with respiratory distress and suspicion of interstitial severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pneumonia, in comparison to other imaging modalities. METHODS: This was a multicenter, retrospective study. LUS was performed, on Emergency Department (ED) arrival of patients presenting for possible COVID-19 evaluation, by trained emergency physicians, before undergoing conventional radiologic examination or while waiting for the report. Scans were performed using longitudinal transducer orientation of the lung regions. CXR was interpreted by radiologists staffing ED radiology. Subjects were divided into two group based on molecular test results. LUS findings were compared to COVID test results, nonlaboratory data, and other imaging for each patient. Categorical variables were expressed as percentages and continuous variables as median ± standard error. RESULTS: A total of 479 patients were enrolled, 87% diagnosed with SARS-CoV-2 by molecular testing. COVID positive and COVID negative patients differed with respect to sex, presence of fever, and white blood cells count. Most common findings on lung point of care ultrasound (POCUS) for COVID-positive patients were B-lines, irregular pleural lines, and small consolidation. Normal chest X-ray was found in 17.89% of cases. CONCLUSIONS: This 479 patient cohort, with COVID-19, found LUS to be noninferior to chest X-ray (CXR) for diagnostic accuracy. In this study, COVID-positive patients are most likely to show B lines and sub-pleural consolidations on LUS examination.

Streptococcal Toxic Shock Syndrome: A Case Report.

Streptococcal toxic shock syndrome is a serious complication of group A Streptococcus infection with a high mortality rate. Rapid detection, early intensive care support, and surgical management are paramount in treating these patients. We present a case of a 65-year-old male, with a documented medical history of hypertension, type 2 diabetes mellitus, and peripheral arterial disease. The patient was evaluated in the emergency department with a chief complaint of pain, swelling in his left leg, and fever. Physical examination showed tachycardia, hypotension, and clear inflammatory signs in the left leg. After initial clinical and laboratory evaluation, the patient was admitted with a diagnosis of cellulitis and urinary tract infection. He presented progressive worsening with multi-organ dysfunction, requiring vasopressor support, invasive mechanical ventilation, and renal replacement therapy. Streptococcus pyogenes was isolated in blood cultures, and a streptococcal toxic shock syndrome was considered. Appropriate antibiotic therapy, immunoglobulins, hemoperfusion, and corticosteroid therapy were administered, with clinical improvement. During hospitalization, there was a progressive improvement in the skin lesion. Once clinically stabilized the patient was discharged with follow-up. The case presented shows the rapid evolution of cutaneous streptococcal infection with multiorgan dysfunction. Although these types of infections have an associated high mortality rate, this patient survived. The use of immunoglobulin and hemoperfusion technique, in this case, might have contributed to this positive outcome. Therefore, we highlight the need for high suspicion of this syndrome, especially in diabetic patients presenting with skin lesions. Once the diagnosis is established, these infections require close surveillance and rapid and intensive treatment.

A Case Report of an Emergency Hypertensive Episode During Trastuzumab Therapy.

Human epidermal growth factor receptor 2 (HER2+) targeted therapies, such as trastuzumab emtansine, has been shown to have a significant positive effect on the outcome of breast cancer. Rarely, this therapy can cause adverse cardiovascular effects such as cardiac dysfunction, arrhythmias and hypertension. We report a case of a 60-year-old woman who presented to the emergency department with an episode of hypertensive emergency. She had been recently diagnosed with hypertension which was found to be poorly controlled. The recent administration of trastuzumab together with this hypertensive episode led us to suspect trastuzumab as the cause for this rise in her blood pressure. With this case we intend to raise awareness of hypertension, a potentially preventable condition, as an adverse effect of human epidermal growth factor receptor 2 targeted therapies such as trastuzumab. Additionally we propose an appropriate and careful management of arterial hypertension among those receiving this first-in-class drug.

Prevalence and risk factors of fatty liver in Portuguese adults.

BACKGROUND: Prevalence of fatty liver (FL) and nonalcoholic fatty liver disease (NAFLD) depends mainly on obesity, diabetes and genetic factors. FL and NAFLD prevalence was evaluated in Portuguese adult population and correlated with several risk factors and related mortality data, within the same period. MATERIALS AND METHODS: A cross-sectional, population-based multicenter study, voluntary and randomly selected in 834 Portuguese adults (18-79 years). Participants were evaluated after 12-hour fasting. Anthropometric data, past history including alcohol consumption, and associated diseases were registered. Blood samples were collected for biochemical testing. Dietary intake was evaluated using a semi-quantitative food frequency questionnaire. Presence of FL was evaluated using ultrasound, and NAFLD was diagnosed after exclusion of other causes for liver disease. RESULTS: Adjusted prevalence of FL and NAFLD was 37.8% and 17.0%, respectively. FL individuals were older, more frequently males, with increased probability of having obesity, diabetes or harmful alcohol consumption (HAC). NAFLD individuals were also older, but had a similar sex distribution and an increased probability of obesity and diabetes. In both groups, no differences were found regarding dietary pattern or physical activity. During the same time period, nonalcoholic steatohepatitis (NASH) liver-related deaths in Portugal were 0.105/100 000, while alcohol-related liver disease mortality was 6.790/100 000. CONCLUSION: The large spectrum of FL was present in more than one third of the population, although only less than half could be classified as NAFLD. Other significant risk factors, such as HAC, are probably implicated in FL, explaining the low NASH-related mortality compared with the high alcohol-related mortality during the same time period.

Chylous Ascites due to Mantle Cell Lymphoma.

Chylous ascites is rare and results in accumulation of lymph in the abdominal cavity, due to several mechanisms. The ascitic liquid is milky because of the high concentration of triglycerides (>200 mg/dl). The higher incidence compared to the past is explained by increased survival of patients with cancer and more aggressive surgery. We describe the case of an 87-year-old man admitted to the geriatric ward due to general oedema, chylous ascites and loss of weight, explained by mantle cell lymphoma. LEARNING POINTS: Chylous ascites is more frequent in trauma, but in some cases may be related to obstruction of the thoracic duct by retroperitoneal fibrosis, pancreatitis or neoplasms.It is a progressive, difficult to manage condition, with a spectrum of treatment ranging from a special diet to surgery.Its prognosis depends fundamentally on the predisposing condition.

Polymyositis and the Spectrum of Scleroderma Disorders.

Polymyositis (PM) is usually associated to other autoimmune or connective tissue diseases. The authors report the case of a 59-year-old man with pulmonary fibrosis, who presented with constitutional symptoms and gradually developed proximal muscle weakness, Raynaud phenomenon, and dysphagia. Besides creatine kinase (CK) elevation, he had positive anti-Polymyositis-Scleromyositis (PM-Scl) and anti-Sjögren's-syndrome A (SSA) antibodies. Nailfold capillaroscopy showed a scleroderma pattern and muscle biopsy revealed necrosis, regeneration of muscle fibers, and inflammatory infiltrate. Prednisolone was started, with great improvement. Taking into account the overlap features between PM and systemic sclerosis sine scleroderma, it is important to closely monitor the patient for signs of pulmonary and cardiac decompensation. LEARNING POINTS: Polymyositis (PM) may be associated with connective tissue diseases such as systemic sclerosis, including its variant without skin involvement.Necrotizing muscle fibers are typically found in patients with overlap syndrome, in opposition to patients only with polymyositis.PM-Scl antibodies are associated to a good response to corticoids.

Chest pain in the emergency department: risk stratification with Manchester triage system and HEART score.

BACKGROUND: Fast and accurate chest pain risk stratification in the emergency department (ED) is critical. The HEART score predicts the short-term incidence of major adverse cardiac events (MACE) in this population, dividing it in three risk categories. We aimed to describe the population with chest pain, to characterize the subgroup of patients with acute coronary syndrome (ACS) and to assess the prognostic value of Manchester triage system and of HEART score. METHODS: Retrospective observational study including patients admitted to the ED of a tertiary hospital with chest pain as the presenting symptom. The primary outcome was a composite of all-cause mortality, myocardial infarction or unscheduled revascularization at 6 weeks. RESULTS: We enrolled 233 patients (age 58 ± 19; 55.4 % males). The most common final diagnosis was non-specific chest pain (n = 86, 36.9 %), followed by ACS (n = 22, 9.4 %). Male gender, smoking and chronic kidney disease were associated with higher risk of ACS. According to Manchester triage system, chest pain patients stratified with red or orange priority had a higher incidence of ACS (16.5 % vs. 3.8 %, p = 0.006). The application of HEART score showed that most patients were in low risk category (56.3 %). The six-week incidence of MACE in each category was 2 %, 15.6 % and 76.9 % (p < 0.001). HEART score accurately predicted the short-term incidence of MACE in chest pain patients (c-statistic 0.880; 95 % CI, 0.807-0.950, p < 0.001). CONCLUSIONS: Chest pain patients have very different levels of severity and the discriminatory power of Manchester triage system should be used in the assessment of this population. The HEART score seems to be an effective tool for risk stratification in the ED.

First report of chronic hepatitis E in renal transplant recipients in Portugal.

Hepatitis E virus (HEV) infection can be responsible for chronic hepatitis in immunocompromised patients, and can rapidly evolve into fibrosis and/or hepatic cirrhosis. We present two cases of chronic hepatitis E, emphasizing the need to be aware of this entity as a growing etiology of hepatitis in transplant and immunocompromised patients.