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Objective Supraventricular arrhythmias are commonly detected in patients with anti-mitochondrial antibody M2 (AMA-M2)-associated myopathy. However, the prevalence of supraventricular arrhythmias in unselected AMA-M2-positive patients and the impact of AMA-M2 on supraventricular arrhythmias have yet to be fully investigated. Methods We analyzed 384 patients (116 men; age, 60 [48-69] years), who underwent AMA-M2 testing following the detection of elevated hepatobiliary enzymes. Supraventricular arrhythmias involving atrial fibrillation, atrial flutter, atrial tachycardia, sick sinus syndrome, and atrial standstill were confirmed by a 12-lead electrocardiogram, 24-hour ambulatory monitoring, and physician-assigned diagnoses within the three years before and two years after the AMA-M2 test. Results Seventy-seven (20%) patients were positive for AMA-M2. The prevalence of supraventricular arrhythmias among AMA-M2-positive patients was higher than that among AMA-M2-negative patients (14% vs. 6%, p=0.008). A univariate analysis showed that supraventricular arrhythmias were associated with AMA-M2 positivity, aging, congestive heart failure, and the CHADS2 score. The multivariate analysis determined that AMA-M2 positivity was an independent risk factor for supraventricular arrhythmias (odds ratio 3.52, p=0.011). Among the AMA-M2-positive patients, the AMA-M2 titer did not differ to a statistically significant extent, regardless of the presence or absence of supraventricular arrhythmias. Multiple supraventricular arrhythmias with extremely low atrial deflections was a characteristic finding in AMA-M2-positive patients with supraventricular arrhythmias. Conclusion AMA-M2 enhances the risk of supraventricular arrhythmias, indicating the possible involvement of the atrial myocardium and the formation of an arrhythmogenic substrate. The results highlight the need for clinical attention to supraventricular arrhythmias in AMA-M2-positive patients.

OBJECTIVE The supplementation of oral branched-chain amino acid (BCAA) granules is known to improve energy metabolism in cirrhotic patients, but not those with hepatocellular carcinoma (HCC). We aimed to clarify whether BCAA granules improve energy metabolism in HCC patients after radiofrequency ablation (RFA). METHODS We performed a prospective cohort study (UMIN000004624) involving 40 HCC patients who underwent RFA at Kobe University Hospital. Indirect calorimetry and urinary/blood biochemical examinations were performed before and seven days after RFA. Blood biochemical examinations were also conducted three months after RFA. The patients treated with and without BCAA supplementation were compared, and univariate factors were statistically examined. RESULTS The non-protein respiratory quotient (npRQ) and albumin levels before RFA were significantly lower in the BCAA group than in the control group (p=0.024 and 0.005). The npRQ ratio (seven days after/before RFA) was significantly higher in the BCAA group than in the control group (p=0.019). In addition, the albumin ratio (three months after/before RFA) was significantly higher in the BCAA group than in the control group (p=0.018). CONCLUSION Supplementation with BCAA granules improves energy metabolism in addition to the liver function after RFA. Improvements in the liver function may result in consistently adequate treatment for HCC recurrence after RFA.

The patient was a 15-year-old girl with severe acute hepatitis. A liver biopsy showed the typical findings of autoimmune hepatitis (AIH). Subsequently, two lineages of cytopenia were found in the patient's peripheral blood. Hemophagocytosis by macrophages was observed in the bone marrow. Virus-, drug- and lymphoma-associated hemophagocytic syndrome (HPS) was ruled out. Therefore, the patient was diagnosed with autoimmune-associated HPS (AAHS). Following the administration of combination therapy with prednisolone and cyclosporine A, both the AAHS and AIH improved. This is the first report of AAHS originating from AIH. The patient was followed up for five years after treatment, and no disease recurrence was detected.

A liver neoplasm was found in a 63-year-old man with alcoholic liver disease. Sonazoid-enhanced ultrasonography (US) showed that the neoplasm was isoechoic at the early vascular phase and hypoechoic at the post-vascular phase. Gadolinium ethoxybenzyl-diethylenetriamine-enhanced magnetic resonance imaging (MRI) showed that the neoplasm was hypointense at the hepatobiliary phase. We suspected that it was a malignant tumor. By needle biopsy, however, the neoplasm was diagnosed as an inflammatory pseudotumor (IPT). We encountered a rare case of hepatic IPT, the differential diagnosis of which was difficult to distinguish from malignant tumor. Here, we report new US and MRI findings of hepatic IPT.

We encountered a case of portal vein thrombosis (PVT) after treatment for portal hypertension due to pancreatic arteriovenous malformation (PAVM). A 75-year-old man was admitted for the treatment of esophageal varices. Diffuse PAVM and aneurysm in the celiac and superior mesenteric arteries were detected via abdominal computed tomography and angiography. Although endoscopical sclerotherapy was performed, PVT was identified after the treatment and variceal bleeding continued. Autopsy was performed and the thrombus and malformation were pathologically confirmed. This case indicates that PVT can be associated with PAVM.

A 61-year-old woman, who was diagnosed in 1982 as having polycythemia vera, was admitted to our hospital in July 1998 because of a splenic tumor in an enlarged spleen due to myelofibrosis. As it was difficult to identify the etiology of the splenic tumor, partial splenectomy was carried out. The resected tumor proved to be an extremely proliferative lesion as the result of extramedullary hematopoiesis. Since it is difficult to diagnose the etiology of splenic tumor, the collection and analysis of reports of relevant cases may well facilitate diagnosis.