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A delayed diagnosis of polyarteritis nodosa may lead to critical limb-threatening ischemia (CLTI). A 74-year-old woman presented with left-foot pain and was treated with oral vasodilators and antiplatelet agents. However, the distal ischemia progressed to CLTI, including gangrene of the fingers and toes, and bilateral foot dropping appeared because of peroneal nerve paralysis. Angiography of the extremities revealed obstruction and stenosis of medium-sized arteries. Based on the progressive distal gangrene, mononeuropathy multiplex, and pathological findings of necrotic vasculitis, polyarteritis nodosa was diagnosed, and the patient's condition improved. A biopsy and neurological examination are essential for the appropriate diagnosis of PAN and immediate treatment.

Neurofibromatosis type 1 (NF1) is an autosomal dominant multi-organ disease. The clinical manifestations include not only skin lesions and malignant tumors but also lung complications, including pulmonary arterial hypertension (PAH). However, the association between gene mutations in NF1 and the occurrence of PAH has not yet been elucidated. We herein report a case of isolated PAH in a 67-year-old woman with NF1, presumably caused by a novel heterozygous mutation, c.4485_4486delinsAT (p.Lys1496Ter), in the NF1 gene.

Myocardial calcification in myocarditis is rare and may be linked to poor outcomes. We herein report a case of fulminant myocarditis with massive myocardial calcification and its pathological outcomes at autopsy. A 49-year-old man experienced chest pain and was diagnosed with acute myocarditis. His cardiac function did not recover despite mechanical circulatory support in combination with V-A extracorporeal membrane oxygenation and IMPELLA CP ® . He eventually developed sepsis and gastrointestinal bleeding and died on day 27. Diffuse myocardial calcification was observed on computed tomography at autopsy. The pathological autopsy depicted that calcification filled every myocardial cell in the left ventricle.

Infective endocarditis (IE) may be acquired in the community as community-acquired (CA) IE or in the healthcare setting. In Japan, cases of CA-methicillin-resistant Staphylococcus aureus (MRSA) infection as skin infection have been increasing. CA-MRSA strains, including the USA300 clone, have higher pathogenicity and are more destructive to tissue than healthcare-associated MRSA strains because of the toxins they produce, including arginine-catabolic mobile element (ACME) and Panton-Valentine leukocidin (PVL). However, only a few IE cases induced by USA300 have been reported. We herein report a 64-year-old man who developed CA-IE from a furuncle caused by USA300 MRSA producing PVL and ACME, which resulted in complications of meningitis.

A 42-year-old woman visited our hospital due to syncope. Contrast-enhanced CT revealed portosystemic shunt, portal vein hypoplasia, and multiple liver nodules. The histological examination of a liver biopsy specimen exhibited portal vein hypoplasia and revealed that the liver tumor was positive for glutamine synthetase. The patient was therefore diagnosed with congenital extrahepatic portosystemic shunt type II, and with focal nodular hyperplasia (FNH)-like nodules. She had the complication of severe portopulmonary hypertension and underwent complete shunt closure by balloon-occluded retrograde transvenous obliteration (B-RTO). The intrahepatic portal vein was well developed at 1 year after B-RTO, and multiple liver nodules completely regressed. Her pulmonary hypertension also improved.

TEMPI syndrome, a disease entity comprising telangiectasia, erythrocytosis with high erythropoietin, monoclonal gammopathy, perinephric fluid collection, and intrapulmonary shunting, was first described by Sykes et al. in 2011. To our knowledge, only 15 cases have been reported worldwide, none of which were in Japan. We herein report a 47-year-old man who had intractable ascites for 2 and a half years and was referred to our department for a peritoneovenous shunt. In addition to ascites, he had telangiectasia, high erythropoietin, monoclonal gammopathy, and perinephric fluid collection. Thus, this is the first case of TEMPI syndrome in Japan.

Anti-myelin oligodendrocyte glycoprotein (MOG) antibodies have been associated with steroid-responsive cortical encephalitis and comorbid generalized epilepsy. A 44-year-old woman developed repeated epilepsia partialis continua (EPC) without generalized seizures and was anti-MOG antibody-positive. Radiological abnormalities were detected in the bilateral medial frontoparietal cortices, but there were no cerebrospinal fluid abnormalities. She achieved remission with anti-epileptic drugs alone. However, encephalitis recurred four months later when pleocytosis appeared, and steroid therapy was effective. Altogether, EPC without typical cerebrospinal fluid features can be an early sign of anti-MOG antibody-positive encephalitis. Thus, patients with EPC of unknown etiology need to be screened for anti-MOG antibodies.

A 69-year-old woman was admitted to our hospital with the chief complaints of fever and fatigue. We initially treated the patient for a tick-borne disease after noticing a pustule on her leg; however, abdominal computed tomography (CT) showed multiple low-density areas in the liver and Chromobacterium violaceum was isolated from a blood culture. We diagnosed her with multiple liver abscesses secondary to Chromobacterium violaceum bacteremia. The patient was successfully treated with ciprofloxacin.

Congenital ventricular diverticulum (CVD) in adults is a rare cardiac malformation, which includes fibrous type congenital ventricular aneurysm (CVA). CVA is often clinically asymptomatic and shows no abnormality in the electrocardiogram or chest X-ray. However, some cases of sudden death resulting from ventricular tachycardia, cardiac embolism or ventricular rupture have been reported. Therefore, physicians should perform further cardiac imaging studies to detect a CVA if ventricular arrhythmia originating from the left ventricle is observed. Here, we report two successfully followed cases of CVA which were diagnosed from premature ventricular contractions.

Pulmonary arterial hypertension (PAH) is a frequent complication in patients with systemic sclerosis. Bosentan is used in patients with symptomatic PAH; however, it has not been established whether or not bosentan ameliorates the progression of PAH in patients with no PAH-related symptoms. We present a case of systemic sclerosis with no PAH-related symptoms in which bosentan ameliorated exercise-induced PAH evaluated by 6-minute walk stress echocardiography, brachial flow-mediated dilation, and skin temperature of hands and feet. The results suggest that administration of bosentan in patients with early-stage PAH ameliorates pulmonary arterial vasodilatation through improvement of endothelial function.

Lactobacillus (LB) is a gram-positive rod-shaped bacterium that inhabits the oral cavity, gastrointestinal tract, vagina and nasal cavity. Although LB plays a role in the prevention of infections caused by pathogenic bacteria, it causes some critical infectious diseases such as infective endocarditis (IE). IE due to LB is rare; however, early diagnosis and early treatment are important because of its high mortality rate. We report the onset of IE after otologic treatment in a heavy drinker of alcohol, the second case of IE due to LB in Japan.

Objective: We prospectively evaluated the usefulness of the QuantiFERON TB-2G (QFT-2G) compared to that of tuberculin skin test (TST) as a supportive method of diagnosing pulmonary tuberculosis (TB) without invasive examinations., Methods: The subjects were 90 patients who required differentiation of pulmonary TB clinically and for whom a definitive diagnosis could not be obtained after admission. The final clinical diagnosis of TB infection in 28 patients and non-TB infection in 62 patients was established using bronchoscopic procedures., Results: In patients with TB infection, the positive response rate on QFT-2G (79%) was significantly higher than that on TST (57%). The QFT-2G negative rate (5%) was significantly lower than that on TST (48%) in patients with non-TB infection. For QFT-2G test, there was a positive response in 25 patients, a negative response in 49, and indeterminate findings in 16. Of the two patients with a false-negative result on QFT-2G, one had pulmonary TB during immunosuppressive treatment and one had pulmonary tuberculoma. Of the three non-TB patients with a positive result on QFT-2G, two had pneumonia and one had pulmonary mycosis. Four TB patients with an indeterminate result on QFT-2G included two elderly patients and two immunocompromised patients., Conclusions: We could confirm the usefulness of the QFT-2G test compared to TST in patients requiring a differential diagnosis between pulmonary TB and other pulmonary diseases in this series. Therefore, we recommend the QFT-2G test as one of the useful noninvasive diagnostic examinations for pulmonary TB.

AL amyloidosis is a disease in which immunoglobulin L chain is deposited in multiple organs, and the prognosis of cardiac amyloidosis is extremely poor. Although several treatments based on that for multiple myeloma, have been performed, there is no clear evidence that cardiac function is improved. We report a case of AL cardiac amyloidosis with moderate cardiac dysfunction for which we performed autologous peripheral blood stem cell transplantation (auto-PBSCT) in combination with high-dose melphalan therapy. This treatment resulted in significant improvement in cardiac function and good prognosis for about 3.5 years after the diagnosis. Therefore, auto-PBSCT is a possible option as up-front therapy for AL cardiac amyloidosis.

Object: To evaluate a newly developed enzyme-linked immunosorbent assay (ELISA) (Hitazyme C. pneumoniae) detecting Chlamydophila pneumoniae-specific immunoglobulin M (IgM) antibody, we compared the assay with culturing, immunoblotting and the microimmunofluorescence (MIF) test., Patients and Methods: Two hundred five patients with stable chronic lung diseases without acute respiratory tract infections (ARTIs) and 116 healthy volunteers without ARTIs were enrolled in this study. Nasopharyngeal swab specimens and sera were obtained from all subjects for isolation and serological testing of C. pneumoniae., Results: C. pneumoniae IgM-positive results were observed in 16.5% of patients with stable chronic lung diseases and in 8.6% of asymptomatic healthy subjects. However, there were no positive cases with cell culture, immunoblot or MIF test. In addition, no cases with a significant increase in IgA or IgG antibody titer for the ELISA kit and MIF test between paired sera were observed in the followed-up groups. IgM-positive cases were more frequent among patients with chronic obstructive pulmonary disease (p=0.1566), collagen disease-associated interstitial lung disease (p<0.0001) and cryptogenic organizing pneumonia (p=0.0199) than among the healthy subjects., Conclusion: Our results indicate that IgM-positive results with the ELISA kit do not always reflect acute C. pneumoniae infections. Further studies are needed, to determine an appropriate cut-off level and the possible causes of the false-positive results in the ELISA kit, such as other underlying conditions.

A patient with a history of adrenocortical carcinoma presented with massive pericardial effusion and a giant pericardial mass. Death resulted from cardiac tamponade. At autopsy a large necrotic mass was histologically similar to the original adrenocortical carcinoma. We present this case and review the literature on metastatic pericardial disease.