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.

Sclerosing Mesenteritis and Disturbance of Glucose Metabolism: A New Relationship? A Case Series.

BACKGROUND: Sclerosing mesenteritis is an idiopathic inflammatory and fibrotic disease that affects the mesentery. It is a rare disease, with the total number of reported cases in the literature ranging from 122 to 300. It mainly affects men in the sixth decade of life, and its etiology remains unknown. Clinical presentation is variable, but it is frequently asymptomatic. Diagnosis is often made by computed tomography (CT) scan, although biopsy may be needed for confirmation. An association between other diseases (e.g., neoplasms) and sclerosing mesenteritis has been described, but the relationship between the latter and glucose changes is not disclosed in the currently available literature. CASE REPORT: Five cases of sclerosing mesenteritis and glucose metabolism disorders (impaired fasting glucose and type 2 diabetes mellitus) were retrospectively collected and analyzed. The mean age was 65 ± 9.3 years, 80% were male, and all patients were white. Three patients were asymptomatic and the other 2 (40%) had non-specific chronic abdominal pain. Blood tests revealed normal inflammatory parameters (mean HbA1c was 6.4% and fasting blood glucose was 140 mg/dL). The diagnosis was made by abdominal CT scan. The 2 symptomatic patients underwent therapy with colchicine 1 mg/day, with clinical improvement. During the mean 43-month follow-up period, there was no symptomatic progression, thereby maintaining the usual benign course of this condition. CONCLUSIONS: Sclerosing mesenteritis has only been described in small series and isolated cases, but its diagnosis is becoming more common due to greater access to diagnostic methods and higher awareness of the disease in the medical community. Furthermore, despite the small sample size, we describe a possible association between glucose metabolism impairment and sclerosing mesenteritis.

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.