Treatment response in primary biliary cholangitis: The role of autoimmune hepatitis features.

INTRODUCTION AND AIM: Primary biliary cholangitis is a rare disease with scarce epidemiological data in Southern Europe. The authors aimed to evaluate treatment response in a cohort of patients. MATERIALS AND METHODS: This retrospective observational single-centre study included patients with diagnostic criteria of primary biliary cholangitis. Data on disease presentation, laboratory results, treatment and clinical endpoints were collected and analyzed. RESULTS: Fifty-three patients were included, 89% women, with mean age of 62±15 years at diagnosis. The majority was asymptomatic (49%), tested positive for antimitochondrial antibodies (96%) and had increased alkaline phosphatase (median=214U/L). 75% of the patients had liver histology and the majority were in Ludwig's stage I (42%). Autoimmune hepatitis (AIH) features were found in seven patients (13%). All were treated with ursodeoxycholic acid (UDCA) and 56% achieved biochemical response at one year; patients with AIH features exhibited steeper decreases in alkaline phosphatase (p=0.007) and reached the endpoint of 40% decrease in alkaline phosphatase more frequently (p=0.017). CONCLUSION: In conclusion a significant proportion of patients failed to achieve an adequate response to UDCA treatment. The response rate of patients with AIH features was better, which could be related to a different phenotype or to the potential impact of immunosuppressive agents.

A Case of a Large Malignant Pericardial Effusion on Computed Tomography Without Electrocardiographic Gating.

A 67-year-old female was admitted due to dyspnea. A computed tomography (CT) disclosed a suspicious pulmonary mass and a pericardial effusion. A transthoracic echocardiogram confirmed a large-volume circumferential pericardial effusion. A pericardiocentesis was performed, and the cytological and histochemical studies later confirmed the diagnosis of pulmonary adenocarcinoma. This case report highlights the casualty of having found a cardiac tamponade through a CT not synchronized with an electrocardiogram.

Apical hypertrophic cardiomyopathy, are low-risk patients really at low risk? A case report.

BACKGROUND: Hypertrophic cardiomyopathy (HCM) is a genetically determined myocardial disease that constitutes the main cause of sudden cardiac death (SCD) in young athletes. Apical HCM (ApHCM) represents a complex subset of patients, whose risk of SCD seems not negligible. Most applied scores likely underestimate the risk of heart events in this subset of patients. CASE SUMMARY: We report the case of a 55-year-old man who was admitted in the emergency department after an episode of aborted sudden death due to ventricular fibrillation. The electrocardiogram made at admission was noted for atrial fibrillation and a new-onset left bundle branch block. Emergency coronary angiography was normal. The electrocardiogram was repeated and showed symmetrical and profound inversion of T waves in the lateral leads. Transthoracic echocardiogram and cardiac magnetic resonance revealed left ventricular apical hypertrophy suggestive of apical variant of HCM. A cardiac defibrillator was implanted for secondary prevention of SCD. After 6 months of follow-up no further rhythmic events were noted. DISCUSSION: Although low, the risk of SCD of ApHCM patients is not negligible. This case illustrates the need for searching of new predictors of rhythmic risk in patients with ApHCM.