Objective Cardiovascular disease increases the risk of maternal mortality. This study examined the risk factors for cardiovascular events in pregnant women with cardiovascular disease.

Methods This was a case-control study conducted in 2 phases at Japanese maternal and fetal care centers. The primary survey, using an interviewer-administered questionnaire, investigated whether the institutions had managed pregnant women with cardiovascular disease from April 2014 to March 2016. From 424 individual facilities surveyed, 135 facilities were found to have experience in managing pregnant women. In the secondary survey, the 135 institutions were asked to complete a web-based questionnaire, which collected detailed clinical information about cases, including cardiovascular disease, cardiovascular events, maternal background, and the perinatal outcome.

Results Information on 302 pregnant women with cardiovascular disease was collected. None of the 302 patients died. There were 25 women with cardiovascular events (cardiovascular event group) and 277 women without cardiovascular events (non-cardiovascular event group); the two groups were compared. No significant differences were found in the perinatal outcomes. Medication use before pregnancy was identified as a risk factor for cardiovascular events (adjusted odds ratio, 23.28; 95% confidence interval, 8.15-66.47; p<0.001). In pregnant women with cardiovascular disease, New York Heart Association (NYHA) functional class II or III before pregnancy was associated with a higher risk of cardiovascular events in comparison to NYHA functional class I (p<0.001 for both).

Conclusion Medication use before pregnancy and NYHA functional class >I were risk factors for cardiovascular events in pregnant women with cardiovascular disease.

Objective Although acute coronary syndrome (ACS) is an uncommon entity in young patients, it constitutes an important problem due to the devastating effects of the disease on the more active lifestyle of young patients. At present, there are no guidelines regarding the prevention of ACS in young patients.

Methods We performed a retrospective study of ACS patients between 2014 and 2017. Epidemiological data, clinical findings, and short-term outcomes were evaluated between young ACS patients (≤50 years old) and elderly ACS patients (>50 years old).

Results Of a total of 361 consecutive ACS patients, 37 were young ACS patients (10.2%). Compared with elderly ACS patients, young ACS patients showed a higher prevalence of males (94.6% vs. 73.8%, p<0.001), current smoking (70.3% vs. 29.9%; p<0.001), and overweight persons (67.6% vs. 27.8%, p<0.001). The eicosapentaenoic acid (EPA)/arachidonic acid (AA) ratio was significantly lower in young ACS patients than in elderly ACS patients [0.17 (0.12-0.25) vs. 0.25 (0.18-0.37), p=0.002]. The prevalence of cardio-pulmonary arrest and percutaneous cardiopulmonary support use was higher in young ACS patients than in elderly ACS patients (24.3% vs. 8.6%, p=0.003, 16.2% vs. 3.1%, p<0.001).

Conclusion The features were markedly different between young ACS patients and elderly ACS patients. In young ACS patients, smoking, being overweight, and a low EPA/AA ratio were distinctive risk factors, and more serious clinical presentations were observed at the onset of ACS than in older patients.

Objective Intragastric balloon (IGB) therapy is a low-invasion treatment for obesity. Recently, a low-carbohydrate diet has shown effectiveness for encouraging weight loss, but whether or not a low-carbohydrate diet improves the efficacy of IGB therapy remains unclear. Therefore, we examined the effectiveness of a low-carbohydrate diet compared with a calorie-restricted diet in combination with IGB therapy.

Methods A prospective study was conducted on 51 patients who had undergone IGB therapy from October 2012 to December 2017. Overall, 31 of the 51 patients were included in this study (12-month assessment after IGB placement). These 31 cases consisted of 18 IGB plus low-carbohydrate diet and 13 IGB plus calorie-restricted diet. We compared the two groups with respect to body weight loss as outcomes.

Results At 12 months after IGB placement, the body weight was significantly lower than that observed at baseline in both the IGB plus low-carbohydrate diet group (baseline 101.9±25.8 kg, 12 months 88.2±21.9 kg) (p<0.0001) and the IGB plus calorie-restricted diet group (baseline 103.5±17.0 kg, 12 months 89.1±6.2 kg) (p<0.005). The percentage of excess weight loss in the IGB plus low-carbohydrate diet group was slightly higher than that in the IGB plus calorie-restricted diet group, but there was no significant difference between the 2 groups at 12 months after IGB placement (IGB plus low-carbohydrate 49.9±60.0%, IGB plus calorie-restricted diet 33.1±27.0%).

Conclusion Our study demonstrated that both a low-carbohydrate diet and a calorie-restricted diet were effective interventions for weight reduction in combination with IGB therapy.

Objective Uremic toxins are known risk factors for cancer in patients undergoing hemodialysis (HD). Although adequate removal of uremic toxins might reduce the cancer risk by improving subclinical uremia, the relationship between the dialysis dose and risk of cancer death in patients undergoing HD remains unclear.

Methods In this prospective observational study, 3,450 patients undergoing HD were followed up for 4 years. The primary outcome was cancer death. Patients were divided into quartiles according to their baseline Kt/V levels. The association between the Kt/V levels and risk of cancer death was estimated using the Kaplan-Meier method and Cox proportional-hazards model.

Results A total of 111 patients (3.2%) died from cancer during the 4-year observational period. The 4-year survival rate decreased linearly with decreasing Kt/V. The multivariable-adjusted hazard ratios (HRs) and 95% confidence intervals (CIs) for cancer death were 2.23 (95% CI, 1.13-4.56), 1.77 (0.88-3.63), and 1.89 (1.04-3.56) in quartile (Q) 1, Q2, and Q3, respectively, compared with patients in the highest Kt/V category (Q4) (p for trend = 0.06). Every 0.1 increase in Kt/V was associated with a reduction of 8% in cancer death (HR 0.92, 95% CI, 0.85-0.99).

Conclusion A lower dialysis dose might be associated with a higher risk of cancer death in patients undergoing HD. Kt/V is a simple indicator of dialysis dose used in clinical practice and might be a useful modifiable factor for predicting the risk of cancer death. Further basic and interventional studies are needed to confirm the apparent reduction in cancer death associated with increasing the dialysis dose.

Objective A randomized controlled trial has shown the efficacy of thalidomide against polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome; however, there are still refractory patients. We studied the effects of lenalidomide, a derivative of thalidomide, on patients refractory to thalidomide.

Methods This prospective single-arm trial evaluated the safety and efficacy of lenalidomide plus dexamethasone in refractory or recurrent patients with POEMS syndrome. The regimen was administered as six 28-day cycles with lenalidomide on days 1-21 (15 mg in cycle 1, and 25 mg in cycle 2-6) plus dexamethasone once a week (20 mg). The primary endpoints were the rate of reduction in the serum vascular endothelial growth factor (VEGF) level at 24 weeks and the incidence of adverse events. This trial was registered with ClinicalTrial.gov, NCT02193698.

Results Between July 2014 and December 2015, five men were enrolled. All patients had been refractory to thalidomide plus dexamethasone for more than 24 weeks. The mean rate of reduction in the serum VEGF level at 24 weeks was 59.6%±8.3% (p=0.0003). The mean serum VEGF level decreased from 2,466±771 pg/mL to 974±340 pg/mL. No serious adverse events were observed, and all patients completed six cycles treatment.

Discussion Lenalidomide is a therapeutic option for thalidomide-refractory patients with POEMS syndrome.

Objective To examine the degree of metabolic abnormalities and their association with the sociodemographic background or mental illness/cognitive disability among homeless men in Nagoya, Japan.

Methods We interviewed 106 homeless men (aged 54.2±12.7 years) and measured their metabolic parameters. Mental illness and cognitive disability were diagnosed using the Mini-International Neuropsychiatric Interview and Wechsler Adult Intelligence Scale-III test, respectively. Associations between metabolic abnormalities and the sociodemographic background or mental illness/cognitive disability were analyzed.

Results There were significant correlations of liver dysfunction (AST≥35 IU, ALT≥35 IU, γ-GTP≥75 IU), hypertension [systolic/diastolic blood pressure (BP) ≥140/90 mmHg], and dyslipidemia (HDL <40 mg/dL) with the history/duration of homelessness (over 2 times/year) and residence status (living on the streets). Although the mean body mass index (BMI), BP, HbA1c, and LDL in participants living in temporary residences were similar to those obtained from the general population data from National Health Nutrition Survey (NHNS) 2016, the systolic/diastolic BP in those living on the street was significantly higher than in the general population, and the HDL in those living in temporary residences was significantly lower than in those reported in the NHNS 2016 data. In the group with cognitive disability, the ALT, TG, and BMI values were significantly higher and the HDL level significantly lower in those living in temporary residences than in those living on the streets.

Conclusion Stressful conditions while living on the streets may exacerbate hypertension and liver dysfunction, and unhealthy food habits when living in a temporary residence may exacerbate low HDL levels. In addition, an inability to self-manage due to cognitive disability may increase the ALT, TG, and BMI values. The provision of homeless people with the skills to sustain independent living conditions and ensure a healthy diet is required.

An 88-year-old man was admitted for elevated liver enzyme levels. Nine years earlier, the patient had been diagnosed with diffuse large B-cell lymphoma (DLBCL) and undergone rituximab, cyclophosphamide, doxorubicin hydrochloride, oncovin, prednisone (R-CHOP) therapy. This patient previously had had a hepatitis B virus (HBV) infection before chemotherapy. After the chemotherapy, he was administered an luteinizing hormone-releasing hormone (LHRH) agonist for prostate cancer. We diagnosed him with HBV reactivation because of positive serum HBV-DNA. HBV reactivation can occur a long time after chemotherapy, particularly if another treatment with immunity-altering drugs is added. In such cases, additional surveillance may be required to detect HBV reactivation.

A 53-year-old woman was admitted to a hospital for gradual left-ear hearing loss over 2 years. Head computed tomography revealed a 2-cm mass along the left jugular bulb and another at the right carotid bulb. The right tumor was resected; the pathological diagnosis was carotid body paraganglioma. Mutations of succinate dehydrogenase (SDH) were suspected, but SDHB staining remained in the tumor. Genetic testing identified a known SDHB mutation (L157X). The patient had head and neck paraganglioma with an SDHB mutation (L157X) more typical of an SDHD mutation. SDHB immunohistochemistry is useful for detecting SDHx mutations, but careful interpretation is needed.

We report a case in which diffusion-weighted magnetic resonance imaging (DWI) demonstrated renal artery stenosis-related renal ischemia and the therapeutic efficacy of revascularization. The patient was a 73-year-old man, who underwent descending thoracic aortic replacement due to DeBakey IIIb chronic aortic dissection, and who showed progressive renal dysfunction due to right renal artery stenosis caused by false lumen thrombosis. DWI demonstrated a decreased apparent diffusion coefficient (ADC) in the right kidney, indicating renal ischemia. Angioplasty with stenting restored renal perfusion and improved the renal function, resulting in the normalization of the decreased ADC in the treated kidney. Thus, DWI can be used to monitor renal ischemia in cases involving advanced renal artery stenosis.

We herein report a 50-year-old woman who suffered from tubulointerstitial nephritis with antimitochondrial M2 antibody, distal renal tubular acidosis, and Fanconi syndrome. Our case also had interstitial pneumonia. After initially successful glucocorticoid therapy, tubulointerstitial nephritis and interstitial pneumonia relapsed. After the second successful round of glucocorticoid therapy, tubulointerstitial nephritis relapsed again and responded to glucocorticoid and azathioprine. This case might indicate (1) the association between pulmonary involvement and tubulointerstitial nephritis with antimitochondrial antibodies and (2) the need for a maintenance dose of glucocorticoid and immunosuppressants in tubulointerstitial nephritis with antimitochondrial antibodies.

Invasive tracheobronchial aspergillosis (ITBA) complicated by nontuberculous mycobacteria (NTM) is rare. An 88-year-old man was admitted for hemoptysis. Bronchoscopy revealed bronchial ulcers, and a tissue biopsy showed Aspergillus fumigatus. He was diagnosed with ITBA, which improved with voriconazole. During treatment, infiltrative shadows appeared in his lungs, and bronchoscopy was performed once again. A non-necrotic epithelioid granuloma and Mycobacterium intracellulare were detected in the biopsy specimen. He was diagnosed with NTM disease. It is important to note that tracheobronchial ulcers may cause hemoptysis and to identify the etiology and treat it appropriately when multiple bacteria are found.

We herein report a case of refractory chronic eosinophilic pneumonia (CEP) complicated with uncontrolled bronchial asthma, in which remission was successfully induced with single dose of benralizumab, a monoclonal antibody against the alpha-chain of the interleukin-5 receptor. Resolution of the patient's symptoms and consolidation on chest X-ray were observed at 2 weeks and lasted for 8 weeks after the administration of benralizumab. Benralizumab would be a novel alternative choice of treatment for CEP patients who are at risk of potential toxicity due to long-term corticosteroid therapy.

Radiation recall dermatitis (RRD) is an inflammatory reaction that occurs at previously irradiated skin regions after drug administration. We herein report a patient with non-small-cell lung cancer treated previously with thoracic radiotherapy who developed severe RRD induced by atezolizumab [anti-programmed death 1 ligand 1 (PD-L1) antibody]. Immunohistochemistry of the skin biopsy showed dermatitis with infiltration of CD8+ lymphocytes, suggesting that atezolizumab might provoke an immune-related inflammatory reaction at previously irradiated skin regions. When administering anti-PD-L1 antibody to patients who have undergone radiotherapy previously, physicians should carefully monitor the irradiated skin for the potential occurrence of RRD.

A 69-year-old man with palpitations and decreased blood pressure was referred. Echocardiography showed a mass in the right atrium and cardiac septum. The serum IgG4 level was 1,450 mg/dL. A biopsy of the cardiac mass showed fibrosis with inflammatory cells and increased IgG4-positive plasma cells and lymphocytes. Flow cytometry and polymerase chain reaction of the immunoglobulin heavy chain did not demonstrate monoclonality. He was diagnosed with IgG4-related disease (IgG4-RD). IgG4-RD with a cardiac mass is rare and it is difficult to distinguish it from malignant lymphoma by a pathological examination alone. We therefore performed a biopsy and analyzed the clonality in order to make an accurate diagnosis of IgG4-RD.

Myositis generally affects the proximal muscles. However, we herein report a case of a 48-year-old woman with intractable myositis affecting the intrinsic muscles of the hands. Her myositis, which developed in childhood, was refractory to treatment with steroids and several immunosuppressants, causing walking disability. After experiencing pain and swelling in the hands for six months, she was diagnosed with myositis of the intrinsic muscles of the hands and tested positive for the anti-signal recognition particle antibody. Intravenous immunoglobulin therapy improved the myositis of the hands. This case suggests that inflammation caused by intractable myositis can extend to the hands.

Guillain-Barré syndrome (GBS) comprises a group of polyneuropathies characterized by rapid progression of limb paralysis. Various subtypes of GBS have been reported. The oculopharyngeal subtype of GBS is currently understood to be primarily a cranial polyneuropathy without limb weakness or cerebellar ataxia. In our case of 62-year-old man, gastrointestinal infection was followed by paranesthesia of the hands. He had bilateral ptosis, pharyngeal disorder, and tongue and bifacial weakness. We diagnosed oculopharyngeal subtype of GBS. It responded to intravenous immunoglobulin. This case highlights the need for further characterization of unusual GBS subtypes.

We herein report the case of a 65-year-old woman diagnosed with myasthenia gravis (MG) after complaining of double vision. The patient had anti-low-density lipoprotein receptor-related protein 4 (LRP4) antibody in her serum, although antibodies against the acetylcholine receptor and muscle-specific tyrosine kinase were not detected. Chest computed tomography showed an anterior mediastinal tumor with a high uptake on fluorodeoxyglucose-positron emission tomography. Endoscopic thymectomy successfully ameliorated her ocular symptoms and showed the lesion to be thymoma. The present case revealed that anti-LRP4 antibody-associated MG can be associated with thymoma, which has been regarded as a rare complication of this disease thus far.

A 72-year-old woman was admitted to our hospital with bilateral pleural effusions. She had a 31-year history of systemic lupus erythematosus and had been treated with prednisolone and azathioprine. Pleural fluid culture revealed Salmonella enterica subsp. arizonae infection. This pathogen rarely infects humans but is commonly found in the gut flora of reptiles, especially snakes. Our patient had not come in contact with reptiles. Despite antibiotic therapies and negative pleural cultures, the pleural effusion persisted. Colon cancer was detected concomitantly, and she finally died. The autopsy revealed that the pleuritis was due to underlying diffuse large B cell lymphoma.

Liposomal-amphotericin B (L-AmB) is used for cutaneous leishmaniasis (CL); however, its treatment failure has not yet been described in detail. A 58-year-old man returned from the Republic of Venezuela with a cutaneous ulcer on his left lower leg. The causative pathogen was Leishmania braziliensis. We started L-AmB 3 mg/kg/day for 6 days; however, the ulcer did not resolve. The patient was successfully retreated with a higher dose L-AmB 4 mg/kg/day 9 times (total, 36 mg/kg). If L-AmB fails to treat CL and other therapeutics cannot be used, increasing the L-AmB dose is a viable option.