Objective The purpose of this study was to identify the predictors of subsequent ischemic stroke events in patients with transient ischemic attack (TIA) attributable to intracranial arterial occlusive lesions.

Methods The study population included 82 patients (55 men; mean age, 69.3±12.1 years) with TIA caused by intracranial arterial occlusive lesions who were admitted to our stroke care unit within 48 h of the onset of a TIA between April 2008 and November 2015. TIA was diagnosed if focal neurological symptoms ascribable to a vascular etiology lasted less than 24 h, irrespective of the presence of ischemic insults on imaging. The primary endpoint was an ischemic stroke event within 90 days of the onset of a TIA.

Results The 90-day risk of ischemic stroke after the onset of a TIA was 14.6% [95% confidence interval (CI): 8.6-23.9%]. Cox proportional hazards multivariate analyses revealed that diffusion-weighted imaging (DWI) positivity [hazard ratio (HR), 8.73; 95%CI, 2.20-41.59; p=0.002], prior ischemic stroke (HR, 4.03; 95%CI, 1.07-15.99; p=0.040), and a high serum level of alkaline phosphatase (ALP) on admission (HR, 1.15; 95%CI, 1.05-1.26; p=0.002, for every +10 U/L) were significant independent predictors of ischemic stroke within 90 days after the onset of a TIA.

Conclusion Our results suggested that patients with a TIA attributable to intracranial artery disease who showed DWI lesions, prior ischemic stroke, or high serum levels of ALP on admission were at high risk of subsequent ischemic stroke events.

Objective The purpose of this study was to present the recent clinical profiles and the real-world management of infective endocarditis (IE).

Methods All medical records of patients with IE were reviewed retrospectively for their clinical data, including clinical presentation, laboratory results, blood cultures, echocardiographic findings, treatments and complications. Using the clinical data collected, we calculated the EuroSCORE II, the European risk score for adult cardiac surgery, the Charlson Comorbidity Index as a surrogate of comordibity, and the Katz Index as a surrogate of frailty.

Results Thirty-eight patients were identified as having IE (24 men, age: 71.8±13.1 years). Congestive heart failure occurred in 16 patients (42%), stroke in 14 (50%), and systemic embolism in 5 (13%). The EuroSCORE II and Charlson Comorbidity Index were high (7.7±5.8% and 5.5±2.8%, respectively). The Katz Index was fair (5.5±1.4) before the onset but deteriorated to 2.8±2.7 at the time of establishing the diagnosis of IE (p<0.001). Early surgery was performed in 22 cases (61%). In-hospital death occurred in 10 cases (26%). A EuroSCORE II ≥9%, Staphylococcus aureus etiology, and a Charlson Comorbidity Index were suggested as determinants of in-hospital death (hazard ratios: 173.60, 9.31, 1.57, respectively). In contrast, early surgery was suggested as a determinant of the survival (hazard ratio: 0.04). The Charlson Comorbidity Index was also suggested as a determinant for selecting conservative management (odds ratio: 1.40).

Conclusion Comorbidity may influence the treatment selection and outcome of elderly patients with IE.

Objective Heart failure is currently the most serious complication of muscular dystrophy. The transient receptor potential cation channel, subfamily V, member 2 (TRPV2) is a stretch-sensitive Ca channel. In damaged myocytes or cardiomyocytes, TRPV2 translocates to the cytoplasmic membrane and enhances Ca influx, triggering cell damage. Evidence suggests that the inhibition of TRPV2 may be a new therapeutic target in heart failure. We found that tranilast, which is widely used as an anti-allergic drug, inhibits TRPV2. A pilot study was conducted to assess the safety and efficacy of tranilast in muscular dystrophy patients with cardiomyopathy.

Methods After obtaining informed consent, two muscular dystrophy patients with advanced heart failure took tranilast (300 mg/day) for three months. Blood tests, echocardiography, electrocardiography (ECG), Holter ECG, analyses of the TRPV2 expression in peripheral mononuclear cells, and circulating micro ribonucleic acid profiling were performed to assess the safety and efficacy of tranilast.

Results The brain natriuretic peptide levels decreased after treatment. The expression of TRPV2 on the cytoplasmic membrane of peripheral mononuclear cells was enhanced before treatment and was decreased after treatment. Some heart-related micro ribonucleic acids (miR-208a-5p, miR-223-3p) were elevated and then decreased after treatment. Some adverse events, including the potentiation of warfarin, the worsening of renal dysfunction, an increased heart rate and premature ventricular contractions, were observed.

Conclusion Tranilast can inhibit TRPV2 and can be effective for treating heart failure, even in patients with muscular dystrophy. Although careful attention is needed, the inhibition of TRPV2 can be a new treatment target for cardiomyopathy. A multi-center trial is planned.

Objective The present study was designed to identify the clinical characteristics that permit the differential diagnosis of hereditary angioedema (HAE) and mast cell-mediated angioedema (Mast-AE) during the first consultation.

Methods The medical histories and laboratory data of 46 patients with HAE and 41 patients with Mast-AE were compared.

Results The average age of onset in the HAE group (19.8±9.0 years) was significantly lower than that in the Mast-AE group (35.2±12.0 years). The incidence of familial angioedema (AE) in the HAE group (73.9%) was significantly higher than that in the Mast-AE group (9.7%). The frequency of history of AE in the extremities, larynx, or gastrointestinal tract was significantly higher in the HAE group. The frequency of AE episodes of the lips and eyelids was significantly lower in the HAE group. The serum C4 concentration and CH50 titer were lower than the normal limit in 91.3% and 45.6% of the patients in the HAE group, respectively; in Mast-AE group the serum C4 concentration and CH50 titer were significantly lower than the normal limit in 4.8% and 0% of the patients, the difference between the two groups was statistically significant. A C1-inhibitor (C1-INH) activity level of <50% was observed in all of the HAE patients, but none of the Mast-AE patients. The mean serum IgE titer in the HAE group (120.8±130.5 IU/mL) was significantly lower than that in the Mast-AE group (262.2±314.9 IU/mL).

Conclusion The parameters within the patients' medical histories, such as the age at the onset of AE, a family history of AE, and the locations of past AE episodes are critical for the successful diagnosis of the disease. Measurements of the C4 and C1-INH activity are very useful for differential diagnosis of HAE from Mast-AE.

Objective Chikungunya fever (CHIK) is a re-emerging arboviral disease that is transmitted through the bite of infected Aedes mosquitoes. There is limited information regarding the epidemiology and clinical information of imported CHIK in Japan. The objective of this study was to review the epidemiology and clinical information of imported CHIK patients treated at the National Center for Global Health and Medicine (NCGM).

Methods We evaluated all patients (n=16) who were diagnosed with imported CHIK and treated at the NCGM between October 1, 2005 and March 31, 2016.

Results The primary complaint of 7 patients who presented to the NCGM after 31 days from disease onset was persistent arthritis, and the primary complaints of 9 patients who presented within 30 days after disease onset were a fever, headache, arthralgia, and rash. Eleven patients experienced a rash during the first week of illness. The median duration of the arthralgia was 75 days, and the joint pain lasted for >2 months in 8 patients and >6 months in 3 patients. Persistent arthralgia was not significantly associated with an age of >35 years (p=0.13) or patient sex (p=0.69). All 16 patients exhibited positive results for CHIK IgM, although only 4 exhibited positive real-time polymerase chain reaction results.

Conclusion Physicians should consider CHIK in patients with a fever who have returned from areas where CHIK is endemic.

Self-expandable metallic stents (SEMSs) are used for the management of malignant colorectal obstruction. A patient who underwent colonic uncovered SEMS insertion for extraluminal stenosis in the splenic flexure of the transverse colon due to advanced gastric cancer is herein reported. The patient presented with a fracture of the colonic SEMS 494 days after SEMS insertion. Although various complications of stenting have previously been reported, the details of fractures of colonic SEMSs have not yet been reported. Because the improvement in the prognosis for patients who undergo palliative SEMS insertion leads to long-term SEMS placement, diverse complications can thus be expected, and new events like stent fracture are expected to increase in the future.

We report the case of a 71-year-old man with afferent loop obstruction (ALO) after Roux-en-Y reconstruction due to gastric cancer. Computed tomography showed a distended afferent loop and a dilatated bile duct. We could not reach the stricture site in the afferent loop using a gastroscope. We performed percutaneous transhepatic biliary drainage (PTBD) and placed a self-expanding metallic stent (SEMS) in the duodenal stricture through the PTBD route. Although an endoscopic approach is preferable, when PTBD can be performed, percutaneous transhepatic SEMS placement might be an alternative option for treating ALO in cases in which it is not possible to reach the site of stenosis with an endoscope.

A 66-year-old Japanese male with a history of Behçet disease exhibited oral and genital ulcers, and a round deep ileocecal ulcer. He was treated with a combination of mesalazine and 20 mg/day of prednisolone (PSL), but was only partially responsive to PSL and we were not able to reduce the steroid dosage. Adalimumab was also administered. However, the ulcer was not completely responsive, and weaning the patient off PSL remained impossible. In contrast, additional treatment with clarithromycin completely healed the refractory active ulcer and left only a scar. Furthermore, the ulcer has since maintained the scar stage despite successfully weaning the patient from PSL.

Although S-1 chemotherapy is used widely as postoperative adjuvant chemotherapy for gastric cancer, some patients experience diarrhea during treatment. The patient was a 39-year-old woman who underwent distal gastrectomy for gastric cancer and who had started S-1 chemotherapy as postoperative adjuvant chemotherapy 1 week before her presentation. She experienced severe diarrhea immediately after starting the course of S-1 tablets. Capsule endoscopy revealed severe S-1-induced enteritis with extensive mucosal injury in the ileum and red intestinal fluid due to the oozing of blood in the ileum. After reducing the dosage of S-1, her diarrhea became milder, and she was able to continue S-1 chemotherapy.

Gastric metastasis from ovarian cancer has rarely been reported. We herein report the case of a 64-year-old woman with gastric metastasis from ovarian cancer that was diagnosed as surgical stage IA. Diagnostic and staging laparotomy showed mucinous carcinoma of the right ovary. At one month after surgery, bone metastasis was detected via scintigraphy. On esophagogastroduodenoscopy, a 10-mm elevated lesion with ulceration on the top was seen in the stomach. The immunohistochemical analysis of biopsy specimens showed that these metastases arose from ovarian cancer. We recommend that physicians remain aware of the possibility of gastric metastasis in patients with ovarian cancer.

An 85-year-old woman underwent endoscopic retrograde cholangiopancreatography (ERCP) for obstructive jaundice. Selective bile duct cannulation was unsuccessful because of periampullary diverticula (PAD). A pancreatic spontaneous dislodgement stent (PSDS) (5F diameter, 3 cm, straight type) was inserted to prevent post-ERCP pancreatitis. Three days after ERCP, she complained of abdominal pain, and computed tomography revealed retroperitoneal perforation because of PSDS migration to the PAD. If the papillary orifice is observed at the diverticular rim or in the diverticula, a pigtailed PSDS on the duodenal side or flanged stent on the pancreatic ductal side should be inserted in order to prevent this rare adverse event.

Pancreaticojejunostomy stricture (PJS) is a late complication of pancreaticoduodenectomy. The endoscopic treatment of PJS is very challenging due to the difficulty of locating the small anastomotic site and passing the stricture using a guidewire. We herein report two cases of severe PJS. These patients could not be treated using only double-balloon endoscopy or endoscopic ultrasound-guided puncture of the main pancreatic duct because of severe stenosis at the anastomotic site. However, we could treat them by the rendezvous technique using the rigid part of the guidewire to penetrate PJS. This method was useful and safe for treating severe PJS.

A 68-year-old man with a history of coronary artery bypass surgery was referred to our hospital because of pre-syncope on effort. During a treadmill exercise electrocardiogram test, the patient developed advanced atrioventricular block associated with dizziness. Coronary angiography revealed significant stenosis of the right coronary artery, which had not existed at the time of the bypass surgery. We implanted drug-eluting stents in the stenotic lesion, and an exercise test showed resolution of the atrioventricular block. Exercise-induced atrioventricular block is rare, and it is necessary to distinguish it from ischemic heart disease, especially in patients with a history of coronary artery disease.

We herein report the case of a 25-year-old woman who presented with severe headache and visual field defects after childbirth. Magnetic resonance imaging revealed marked swelling of the pituitary gland, and an endocrinological examination revealed panhypopituitarism and diabetes insipidus. An immunohistological analysis of a transsphenoidal biopsy sample of the pituitary gland showed the significant accumulation of an immunogloblin G4 (IgG4)-positive population, leading to the diagnosis of IgG4-related hypophysitis. The patient was treated with prednisolone, which markedly reduced the swelling of the pituitary gland, in association with recovery of the pituitary function. This is a rare case of biopsy-proven IgG4-related hypophysitis with a postpartum onset.

Development of aspergilloma is common in cases with a fungus ball-like shadow in cavities due to old tuberculosis. Some reports have shown that blood clots tend to appear as a fungus ball-like shadow. A 71-year-old man with a history of pulmonary tuberculosis presented with a fungus ball-like shadow in an old cavity and hemoptysis. There was no evidence of aspergillus infection on various examinations. We confirmed a blood clot and aneurysm of an artery under direct vision by bronchoscopy. A lateral thoracic artery aneurysm was detected by angiography. Transcatheter arterial embolization was performed. After treatment, the artery aneurysm disappeared.

A 67-year-old man was admitted to our hospital complaining of dry cough. Chest computed tomography showed diffuse infiltrates and ground-glass opacities in the bilateral lung fields. Transbronchial lung biopsy specimens showed alveoli filled with yeast-like fungi. With a diagnosis of pneumocystis pneumonia (PCP), he was given oral sulfamethoxazole/trimethoprim, to which he responded well. However, seven months later, PCP relapsed. Analyses revealed a low bronchoalveolar lavage fluid CD4/CD8 ratio of 0.04 and CD4+ lymphocytopenia (250/μL). Despite intensive work-up, we were unable to detect the underlying cause of CD4+ lymphocytopenia; therefore, a final diagnosis of idiopathic CD4+ T-lymphocytopenia was made.

We report the case of a 61-year-old woman with rapidly progressive interstitial lung disease caused by clinically amyopathic dermatomyositis. Both the serum ferritin and anti-melanoma differentiation-associated gene 5 (MDA5) antibody levels were elevated at the time of admission. Despite intensive treatment with corticosteroids, immunosuppressants, immunoglobulins and polymyxin B direct hemoperfusion, the patient died 75 days after symptom onset. Over the course of treatment, the anti-MDA5 antibody level continually decreased, while the serum ferritin level increased, suggesting that sequential measurements of the serum ferritin level might be useful for evaluating the treatment responsivity, irrespective of the anti-MDA5 antibody level.

Acquired coagulation factor inhibitor is a rare coagulation disorder. We herein report a patient with acquired factor V inhibitor showing a decrease in multiple coagulation factor activities. A high titer of factor V inhibitor presumably led to a marked inhibition of factor V activity in the specific factor-deficient plasma used in coagulation factor activity assays based on either an activated partial thromboplastin time (APTT) or prothrombin time (PT) clotting assay, resulting in false low values of the coagulation activity. We re-examined the coagulation factor activity using several dilutions of the patient's plasma and confirmed that the high factor V inhibitor titer had caused an apparent decrease in multiple coagulation factor activities.

A 50-year-old man was diagnosed with multiple myeloma complicating AL amyloidosis. Splenic rupture was complicated during autologous stem cell transplantation (auto-SCT). Granulocyte colony-stimulating factor (G-CSF) was not administered. A pathological examination of the spleen revealed that CD34-positive cells were concentrated in the ruptured part of the splenic capsule. Hematopoietic cells were engrafted in the small gap between the capsule and amyloid protein deposition area of the spleen, which might have caused the splenic rupture in the absence of G-CSF administration. Special attention is thus required for amyloidosis patients undergoing auto-SCT, even when G-CSF is not administered.

A 35-year-old woman with fever, edema and rash was admitted. Pleural effusion and cardiomegaly were observed. A laboratory analysis revealed anemia with iron deficiency and elevated human parvovirus B19 (B19V) immunoglobulin M. The patient's hepcidin-25 and erythroferrone levels were not elevated compared to those observed later in her clinical course. On the other hand, her growth differentiation factor-15 (GDF-15) levels were elevated. She was diagnosed to have heart failure symptoms and anemia with specific iron metabolism abnormalities due to a B19V infection. After providing supportive treatment, the heart failure symptoms disappeared and her anemia had improved. This case emphasizes the need to include a B19V infection in the differential diagnosis when we encounter cases demonstrating reversible heart failure with anemia.

A 79-year-old woman with a history of Guillain-Barré syndrome (GBS) developed somnolence and tetraparesis after pneumonia. Based on clinical and laboratory findings, she was diagnosed with complications of acute inflammatory demyelinating polyneuropathy (AIDP) and acute disseminated encephalomyelitis (ADEM). Anti-galactocerebroside (Gal-C) IgG antibodies were detected in her serum. Cases of recurrent GBS in patients who are positive for this antibody are extremely rare. The anti-Gal-C IgG antibodies likely played an important role in the pathogenesis of the AIDP and ADEM.

Although central nervous system manifestations seem common in primary Sjögren's syndrome, hypertrophic pachymeningitis is rare. We herein describe a case of Sjögren's syndrome that was associated with hypertrophic pachymeningitis. Sjögren's syndrome should be considered as a cause of hypertrophic pachymeningitis.

A 65-year-old man was diagnosed with granulomatosis with polyangiitis (GPA) based on the detection of high myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA), vasculitis and granulomas in a lung biopsy specimen and crescentic glomerulonephritis in a kidney biopsy specimen. Soon after the initiation of intravenous methylprednisolone pulse therapy (mPSL pulse) and intravenous cyclophosphamide pulse therapy (IVCY), the patient experienced cough and hemoptysis. Based on emerging anemia and bilateral diffuse lung consolidation on computed tomography, we judged that diffuse alveolar hemorrhage (DAH) was complicated by GPA. The patient's DAH improved following additional mPSL pulse and IVCY. Physicians should be aware of the possible occurrence of DAH, even when a patient's symptoms improve after mPSL pulse and IVCY.

Mycobacterium colombiense (M. colombiense) is a member of the Mycobacterium avium complex (MAC). To our knowledge, this is the third case report of an M. colombiense infection. An 80-year-old man, immunocompromised by myelodysplastic syndrome (MDS), developed a skin rash with exfoliation and eruption on his face and scalp. Mycobacteria were detected in pus samples. Broad-range polymerase chain reaction (PCR) revealed the mycobacteria to be M. colombiense. The lesions resolved after daily administration of rifampicin, ethambutol, and clarithromycin. In conclusion, broad-range PCR identified this rare mycobacterium, allowing for the administration of appropriate combination antibiotic therapy.

Mycobacterium bovis infection after intravesical Bacillus Calmette-Guérin (BCG) therapy is rare. A 65-year-old Japanese man with history of bladder cancer and intravesical BCG therapy, presented with low-grade fever. An aneurysm with perianeurysmal fluid was suspected and endovascular aortic repair was performed. After 160 days, he developed blood-streaked sputum and computed tomography images revealed that the perianeurysmal fluid area was increasing in size. A multiplex polymerase chain reaction using sputum identified M. bovis. Treatment with anti-tuberculosis drugs reduced the size of the perianeurysmal fluid area. After intravesical BCG therapy, the possibility of M. bovis infection should be considered, thus further investigations are required.

A previously healthy 31-year-old man was referred to us with refractory septic shock accompanied by bilateral conjunctival congestion and erythema of his right lower limb. Nine days after admission, he had bilateral desquamation of the fingertips, and his presentation satisfied the criteria for Kawasaki disease. A serological examination was positive for Yersinia pseudotuberculosis, and he was diagnosed with Far East scarlet-like fever (FESLF). Interestingly, his 11-month-old baby boy had similar symptoms around the same time, indicating the intrafamilial transmission of the pathogen. We should consider FESLF when we encounter a familial occurrence of systemic manifestations of Kawasaki disease.