Search

Introduction The proximal femur (PF) is one of the most common locations of benign cystic lesions. A fracture after bone biopsy is a rare but severe complication. However, the risk of fracture after biopsy of this lesion has not been well-studied. Computed tomography (CT)-based finite element (FE) modeling estimates the elastic modulus and compressive strength enables fracture prediction. This study investigated strength of PF after biopsy by CT-FE modeling and determined the optimum biopsy level and size. Materials and methods Six male bone tumor patients’ (15–38 years) total femur CT data (slice thickness, 0.8–1.0 mm) of the healthy side were obtained. Three different cylindrical bone defect (BD) diameters (10, 15, and 20 mm) were set on the lateral surface of PF at the following levels: level 1, insertion of the gluteus minimums; level 2, lower end of the greater trochanter (GT); level 3, origin of the vastus lateralis; level 4, center of the lesser trochanter (LT); and level 5, lower end of LT using Mechanical Finder software (version 8.0). Virtual loads were applied with incremental increases of 100 N until fracture occurred and the fracture load (FL) was evaluated. Results For BD with a diameter of 15 and 20 mm, there was a significant difference in the decrease of the mean FL, with an average of 22% at level 4 and 5, and 33%–44% at levels 3 to 5, respectively. At level 1 and 2, no significant decrease in the mean FL was observed regardless of the diameter of BD. Conclusion Biopsies at level 1 and 2 showed no significant decrease in bone strength. However, biopsy at level 1 may contaminate the GT bursas. Therefore, biopsy at level 2 (lower end of GT) can avoid contamination and minimize the effect on bone strength.

Depression, anxiety, and binge eating are common psychiatric symptoms among people with obesity. Although many studies seek to understand the mechanisms of association between these psychiatric symptoms, there is no still consensus about the longitudinal association.155 patients (124 women) were recruited from a university-based bariatric center and evaluated over three waves (T0-T1-T2). In the last period the sample comprised 126 (104 women) participants. Trained clinicians assessed psychiatric symptoms by telephone interview using measurement scales. Partial Least Squares (PLS) was applied to investigate the path effects between anxiety, depression and binge eating symptoms over time.The results of path coefficients (β) showed that the effect of anxiety on depression was constantly significant in all periods T0 (β = 0.74), T1 (β = 0.71), and T2 (β = 0.67). Anxiety had an effect on binge eating in T0 (β = 0.39) and T2 (β = 0.26) but not in T1. Binge eating affected depressive symptoms only in T2 (β = 0.22). Two carry-over-effects were significant binge eating in T0-T1 (β = 0.41) and T1-T2 (β = 0.19).Telephone interviews, social isolation due to the pandemic and the social desirability may have contributed to collection and information biases.Anxiety has significant path effects on depression and binge eating. Binge eating was shown to be the most unstable symptom over time. The time factor seems to contribute to path effects between the psychiatric symptoms. The results draw attention to the fact that psychiatric symptoms must be evaluated and treated in association with each other, and investigated over time.

A 67-year-old man was admitted to our hospital with cough and fatigue. He had had long-term exposure to silica due to cement processing. Chest computed tomography showed bilateral centrilobular nodules, and hilar and mediastinal lymphadenopathy with calcification, suggesting chronic silicosis. Within a few months, these nodules enlarged, and bilateral patchy consolidations appeared. A lung biopsy revealed sarcoid-like granulomas with birefringent particles under polarized light without malignancy or infection. He was diagnosed with silicosis-associated sarcoid-like granulomatous lung disease, rather than sarcoidosis, according to the clinicopathological findings. His pulmonary manifestations improved after the discontinuation of silica exposure and combination therapy of corticosteroid and azathioprine.

Immune checkpoint inhibitors (ICIs) treatment can result in endocrine immune-related adverse events (irAEs), including pituitary dysfunction. Quick diagnosis of secondary adrenal insufficiency (AI) is challenging because no universal definition of ICI-induced secondary AI has been agreed. The aim of this study was to clarify the clinical features of ICI-induced secondary AI that can be used for screening in standard clinical practice. This retrospective study was performed using the medical records of patients who received ICIs at Hirosaki University Hospital between 1 September 2014 and 31 January 2021. Longitudinal clinical data of patients who developed AI were analyzed and compared with the data of thyroid irAEs. Regression analysis showed a significant correlation between ICI-induced secondary AI and absolute or relative eosinophil counts at pre-onset of AI, as well as differences or rate of increase in eosinophil counts at baseline and at pre-onset. Absolute eosinophil counts > 198.36/µL or relative eosinophil counts > 5.6% at pre-onset, and a difference of 65.25/µL or a rate of eosinophil count increase of 1.97 between the baseline and at pre-onset showed the best sensitivity and specificity. This is the first report to demonstrate that eosinophil counts can be a predictor of ICI-induced secondary AI.

Lower gastrointestinal perforation is rare and challenging to diagnose in patients presenting with an acute abdomen. However, no study has examined the frequency and associated factors of diagnostic errors related to lower gastrointestinal perforation. This large-scale multicenter retrospective study investigated the frequency of diagnostic errors and identified the associated factors. Factors at the level of the patient, symptoms, situation, and physician were included in the analysis. Data were collected from nine institutions, between January 1, 2015 and December 31, 2019. Timely diagnosis was defined as diagnosis at the first visit in computed tomography (CT)-capable facilities or referral to an appropriate medical institution immediately following the first visit to a non-CT-capable facility. Cases not meeting this definition were defined as diagnostic errors that resulted in delayed diagnosis. Of the 439 cases of lower gastrointestinal perforation identified, delayed diagnosis occurred in 138 cases (31.4%). Multivariate logistic regression analysis revealed a significant association between examination by a non-generalist and delayed diagnosis. Other factors showing a tendency with delayed diagnosis included presence of fever, absence of abdominal tenderness, and unavailability of urgent radiology reports. Initial misdiagnoses were mainly gastroenteritis, constipation, and small bowel obstruction. In conclusion, diagnostic errors occurred in about one-third of patients with a lower gastrointestinal perforation.

Background Takayasu arteritis (TA) is a chronic inflammatory disease that induces stenosis, occlusion, or aneurysmal degeneration of the aorta and its major branches. Though rarely reported, proximal aneurysmal lesions from the aortic root to the arch are more common in Asian populations than in Western populations. In the surgical treatment of TA, anastomotic aneurysm can be problematic. Furthermore, atherosclerotic complications should be considered in surgical treatment for elderly TA patients. Case presentation Here, we report a case of brachiocephalic artery (BCA) aneurysm with TA for which surgical treatment was successful. Though it was solely a lesion of the brachiocephalic artery, after considering the patient’s clinical background and the features of TA, we chose a partial arch replacement. Further, for avoidance of anastomotic aneurysm, both distal and proximal anastomosis were reinforced with Teflon felt strips. Preoperative computed tomography detected severe atherosclerotic changes in the arch vessels. The patient underwent partial arch replacement using isolated cerebral perfusion (ICP) for brain protection and recovered without any neurological deficits. Conclusions In avoidance with anastomotic aneurysm, reinforcement of the anastomosis was introduced. ICP was effective for brain protection in case with severe atherosclerotic changes.

To investigate the muscle activity patterns of the glenohumeral joint during internal rotation both with the arm at 0° and 90° of abduction using 2-deoxy-2-[18F] fluoro-d-glucose (FDG) positron emission tomography (PET) and magnetic resonance imaging (MRI). Six healthy male volunteers underwent PET examination after performing active glenohumeral internal rotation exercise using an elastic band both with the arm at 0° and 90° of abduction. As a control, PET scan under resting condition was also performed. The exercise was performed before and after 18 fluorodeoxyglucose injection. Each PET image was fused to the corresponding MRI to identify each muscle. The standardized uptake value (SUV) of each muscle was compared between the two arm positions. With the arm at 0° of abduction, the SUV increased significantly after exercise both in the middle and inferior 1/3 of the subscapularis, which were significantly higher than that of the superior 1/3 of the subscapularis (P

A 60-year-old man presented with postoperative recurrence of intrahepatic cholangiocarcinoma with right portal vein tumor thrombosis (PVTT). After failure of standard chemotherapy, a liver biopsy showed that his microsatellite instability (MSI) status was high. Treatment with the immune checkpoint inhibitor (ICI) pembrolizumab was commenced, which resulted in a partial response and resolution of the PVTT. There were no significant immune-related adverse events. According to recently published reports, the frequency of MSI-high biliary tract cancer (BTC) is about 0%-2.1%, which is extremely rare. However, ICIs may be effective in patients with MSI-high BTC, such as the present patient.

Background Preoperative chemotherapy is widely applied to high-grade localized soft tissue sarcomas (STSs); however, the prognostic significance of histological response to chemotherapy remains controversial. This study aimed to standardize evaluation method of histological response to chemotherapy with high agreement score among pathologists, and to establish a cut-off value closely related to prognosis. Methods Using data and specimens from the patients who had registered in the Japan Clinical Oncology Group study, JCOG0304, a phase II trial evaluating the efficacy of perioperative chemotherapy with doxorubicin (DOX) and ifosfamide (IFO), we evaluated histological response to preoperative chemotherapy at the central review board. Results A total of 64 patients were eligible for this study. The percentage of viable tumor area ranged from 0.1% to 97.0%, with median value of 35.7%. Regarding concordance proportion between pathologists, the weighted kappa coefficient (κ) score in all patients was 0.71, indicating that the established evaluation method achieved substantial agreement score. When the cut-off value of the percentage of the residual tumor area was set as 25%, the p-value for the difference in overall survival showed the minimum value. Hazard ratio of the non-responder with percentage of the residual tumor p = 0.070). Conclusion The standardized evaluation method of pathological response to preoperative chemotherapy showed a substantial agreement in the weighted κ score. The evaluation method established here was useful for estimating of the prognosis in STS patients who were administered perioperative chemotherapy with DOX and IFO. Trial registration UMIN Clinical Trials Registry C000000096. Registered 30 August, 2005 (retrospectively registered).

The refined disease risk index (R-DRI) is a well-designed prognostic parameter that is based on only the disease type and status and is used for stratifying patients undergoing allogeneic hematopoietic stem cell transplantation (allo HSCT) into 4 risk groups. However, the application of the R-DRI for rare diseases has remained unclear.We evaluated 135 patients who underwent allo HSCT for hematological malignancies including rare diseases, such as acute leukemia of ambiguous lineage, acute T-cell leukemia/lymphoma, extranodal natural killer T-cell lymphoma, and lymphoblastic lymphoma, at our institute.According to the R-DRI, overall survival (OS) and progression-free survival at 2 years for patients with the low, intermediate, high, and very high groups were 66.7% and 66.7%, 60.8% and 56.0%, 27.1% and 23.7%, and 5.9% and 5.1%, respectively (P .0001 and P .0001, respectively). OS showed no significant difference between B-cell non-Hodgkin lymphoma (B-NHL) and T-cell non-Hodgkin lymphoma (T-NHL) (P = .71). Moreover, OS at 1 year was 80%, 14.3%, 60%, and 0% for the intermediate risk group, the very high-risk group of B-NHL, the intermediate risk group, and the high-risk group of T-NHL, respectively (P = .035).We showed the applicability of the R-DRI for hematological malignancies, including rare disorders. However, we suggest that T-NHL patients may be better to be assigned between the nodal group and the extranodal group in the R-DRI.

A 52-year-old man presented with temporal haemoptysis and chest pain 6 months after radiofrequency catheter ablation for atrial fibrillation. Computed tomography revealed severe stenosis in the left upper pulmonary vein (PV) and complete occlusion of the left lower PV. A modified sutureless repair of the left PV obstruction was successfully performed. This modified procedure provides a feasible, safe and effective means of treating PV obstruction, even in cases with distal extension of stenosis.

A 72‒year‒old man with hepatocellular carcinoma(HCC)was treated with transarterial chemoembolization(TACE)and radiofrequency ablation(RFA). Six months after RFA, gadolinium ethoxybenzyl diethylenetriamine pentaacetic acid(Gd‒ EOB‒DTPA)‒enhanced magnetic resonance imaging(MRI)revealed multiple metastatic recurrences in the liver. TACE was performed for the recurrent HCC. However, the treatment response on the Gd‒EOB‒DTPA‒enhanced MRI showed that the lesions had advanced and that the liver metastatic nodules had ring‒shaped contrast effects. We suspected metastatic liver cancer based on the MRI findings and performed colonoscopy. Finally, we diagnosed the patient with multiple hepatic metastases of sigmoid colon cancer based on the results of the endoscopic colon biopsy and percutaneous liver tumor biopsy. In conclusion, we had a teachable case of the treatment of HCC.

Objectives Acute pulmonary thromboembolism is a fatal condition with high mortality rate in patients with hemodynamic collapse. Early diagnosis and aggressive treatment after circulatory collapse due to pulmonary thromboembolism are important. Although catheter-directed therapy or surgical thromboembolectomy could be considered, the prognosis of such cases is poor. Methods We reviewed nine consecutive patients who underwent surgical intervention for acute pulmonary thromboembolism requiring cardiopulmonary resuscitation from March 2010 to May 2017. The patients’ demographics, risk factors, operative and postoperative outcomes were retrospectively analyzed. All nine patients who experienced cardiopulmonary arrest or pulseless electrical activity had received cardiopulmonary resuscitation, including endotracheal intubation with cardiac massage. Computed tomography revealed massive pulmonary thromboembolism. Percutaneous cardiopulmonary support was initiated in six cases. Pulmonary thromboembolectomy was performed on a beating heart without aortic cross-clamping in all patients Results The mean operation time was 251.9 ± 74.7 min. Among the six patients who received preoperative percutaneous cardiopulmonary support, three were able to wean off cardiopulmonary bypass during the surgery. The other three cases were able to wean off within 3 days postoperatively. The mean postoperative intubation time was 56.3 h. No mortality was observed. All patients were discharged with New York Heart Association functional class I without any neurological complications. No patients developed major and residual thrombi in the pulmonary artery upon postoperative computed tomography. Conclusions Pulmonary thromboembolectomy on a beating heart for acute pulmonary thromboembolism may be beneficial for minimizing right ventricular damage and improving clinical outcomes.

OBJECTIVE To determine the prevalence of bovine leukemia virus (BLV) in beef bulls; evaluate the presence of BLV provirus DNA in blood, smegma, and semen samples; and analyze whether blood BLV proviral load was associated with differential blood cell counts. DESIGN Observational cross-sectional study. ANIMALS 121 beef bulls ≥ 2 years old from 39 Michigan herds. PROCEDURES Blood, smegma, and semen samples were collected from each bull during a routine breeding soundness examination. An ELISA was used to detect serum anti-BLV antibodies. A coordination of common motifs-quantitative PCR assay was used to detect BLV provirus DNA in blood, smegma, and semen samples. Bulls with positive results on both the BLV serum ELISA and coordination of common motifs-quantitative PCR assay were considered infected with BLV. RESULTS 19 of 39 (48.7%) herds and 54 of 121 (44.6%) bulls were infected with BLV. Provirus DNA was detected in the blood of all 54 and in smegma of 4 BLV-infected bulls but was not detected in any semen sample. Lymphocyte count was significantly greater in BLV-infected bulls than in uninfected bulls. The proportion of BLV-infected bulls with lymphocytosis (16/54 [29.6%]) was greater than the proportion of uninfected bulls with lymphocytosis (6/67 [9%]). Lymphocyte count was positively associated with BLV proviral load in BLV-infected bulls. CONCLUSIONS AND CLINICAL RELEVANCE Results indicated that almost half of beef bulls and herds were infected with BLV, and BLV provirus DNA was detected in the smegma of some BLV-infected bulls. Bulls may have an important role in BLV transmission in beef herds.

BackgroundHuman herpesvirus 6 (HHV-6) causes life-threatening central nervous system disorders after allogeneic hematopoietic stem cell transplantation (allo-HSCT). Recent studies implicated CD134 as a specific receptor of HHV-6B and demonstrated that its expression levels in CD4-positive T cells after allo-HSCT could be related to the reactivation of HHV-6. We prospectively evaluated the relationship between HHV-6 reactivation and CD134+ T cells in the recipients of allo-HSCT.MethodsHHV-6 viral load in plasma was quantitatively measured weekly after allo-HSCT by digital polymerase chain reaction in 34 patients. The ratio of CD134 in CD4+ T cells (CD134/CD4 ratio) was serially measured by flow cytometry before and after transplantation.ResultsHHV-6 reactivation was detected in 23 patients (68%). The CD134/CD4 ratio before conditioning was significantly higher in patients with HHV-6 reactivation than in those without (median, 3.8% vs 1.5%, P < .01). In multivariate analysis, a higher CD134/CD4 ratio before conditioning was significantly associated with the incidence of HHV-6 reactivation (odds ratio, 10.5 [95% confidence interval, 1.3–85.1], P = .03).ConclusionsA higher CD134/CD4 ratio before conditioning was associated with a higher risk of HHV-6 reactivation, suggesting that the rate may be a promising marker for predicting HHV-6 reactivation after allo-HSCT.

Background Left ventricular (LV) pseudo-false aneurysm is a rare complication secondary to myocardial infarction and is caused by intramyocardial dissecting hematoma due to fragile myocardium. Very occasionally, intramyocardial dissecting hematoma appears as a neocavitation entirely contained within the myocardial wall (so called “pseudo-false LV”) and is an unusual form of subacute cardiac rupture. Case presentation A 38-year-old male experienced chest discomfort 3 weeks ago, which improved within few days. However, after that episode, he presented at our hospital with rapidly deteriorating severe breathlessness in a preshock state with acute heart failure. Emergency coronary angiography revealed an occluded left anterior descending artery. An intra-aortic balloon catheter was inserted because of unstable hemodynamics. Enhanced computed tomography revealed extensive aneurysm formation in the LV anterior wall and contrast leakage from the inner cavity to the LV myocardium, with a moderately accumulated pericardial effusion. Emergency surgery revealed a large aneurysmal sac on the anterior wall, slightly attached to the pericardium. A 5-mm, slit-like, oozing-type, rupture site was detected in the LV after dissecting the pericardium. Conclusions To our knowledge, this is the first report of a pseudo-false aneurysm on the LV anterior wall. Subacute rupture of pseudo-false LV aneurysm is rare.

Purpose Renin-angiotensin system (RAS) inhibitors carry a risk of normotensive ischemic acute kidney injury in dehydration and concurrent nonsteroidal anti-inflammatory drug (NSAID) use. Although the estimated number of patients with chronic kidney disease (CKD) is 20 000, Fujieda, Japan, has only three nephrologists. On 25 March 2016, we reorganized the CKD network to include pharmacists and distributed a CKD manual. We assessed effects of pharmacist participation in the CKD network and CKD manual distribution on patient hospitalizations because of drug-related kidney injury. Methods Changes in the prevalence of RAS inhibitor-related estimated glomerular filtration rate (eGFR) declines of greater than or equal to 30% and hyperkalemia of greater than or equal to 6.0 mEq/L in 129 hospitalized CKD patients, drug prescriptions of 14 150 hospitalized patients, and annual medical checkup data in 36 042 citizens were investigated before and after pharmacist participation. Results After pharmacist participation, patient hospitalizations due to RAS inhibitor-related eGFR declines decreased (71.4% to 38.1%, P = .03) and hyperkalemia declined (38.1% to 9.5%, P = .03). Pharmacist participation influenced the decrease in RAS inhibitor-related eGFR declines (P = .03). NSAID prescriptions decreased (13.4% to 11.8%, P = .003) and acetaminophen prescriptions increased (6.6% to 8.0%, P = .002) among 14 150 hospitalized patients, whereas RAS inhibitor prescriptions decreased (43.2% to 39.4%, P = .002) among 6930 hospitalized patients with eGFR less than 60 mL/min/1.73 m2 . A significant number of citizens shifted from CKD stage G3a-3b to G1-2. Conclusions Pharmacist participation in the CKD network and CKD manual distribution decreased both hospitalizations due to RAS inhibitor-related kidney injury and citizens with CKD stage G3a-3b.

Objectives Acute type A aortic dissection complicated with brain ischemia is associated with significantly higher mortality risks. Even if rescued with central aortic repair, some patients develop permanent postoperative neurological deficiency postoperatively. We recently introduced direct common carotid artery perfusion for acute type A aortic dissection involving the common carotid artery. This study introduced this technique to prevent postoperative neurological deficiency by comparing brain protection strategies. Methods Among 91 acute type A aortic dissection patients treated at our hospital during August 2015–October 2020, the common carotid artery was involved in 19 (21%), which had > 90% stenosis in either of the carotid arteries on preoperative contrast-enhanced computed tomography. Twelve patients underwent conventional selective cerebral perfusion during August 2015–December 2018 and seven patients underwent direct carotid artery perfusion during January 2019–October 2020. We assessed patient characteristics, surgical courses, clinical outcomes, and neurological outcomes. Results The mean age was 69 (range 39–84) years; 17 patients were male (89%). Eight patients (42%) had neurological symptoms. Concomitant procedures, postoperative neurological symptoms, and late mortality were significantly associated with conventional selective cerebral perfusion. Five selective cerebral perfusion patients experienced worsened neurological symptoms and two died of broad cerebral ischemia. No direct carotid artery perfusion patient died during hospitalization or experienced worsened neurological outcomes. Conclusions Direct carotid artery perfusion may be useful in aortic dissection with brain ischemia because it does not aggravate neurological symptoms and is not associated with intraoperative cerebral infarction. However, it may be ineffective when cerebral infarction has already developed.

Background: Many studies have reported specific adaptations to high altitude, but few studies have focused on physiological variations in high-altitude adaptation in Andean highlanders. This study aimed to investigate the relationships between SpO2 and related factors, including individual variations and sex differences, in Andean highlanders. Methods: The participants were community-dwelling people in La Paz, Bolivia, aged 20 years and over (age range 20-34 years). A total of 50 men and 50 women participated in this study. Height, weight, SpO2, hemoglobin concentration, finger temperature, heart rate, and blood pressure were measured. Information about lifestyle was also obtained by interview. Results: There were individual variations of SpO2 both in men (mean 89.9%, range 84.0-95.0%) and women (mean 91.0%, range 84.0-96.0%). On Student's t test, men had significantly lower heart rate (p = 0.046) and SpO2 (p = 0.030) than women. On the other hand, men had significantly higher SBP (p < 0.001), hemoglobin (p < 0.001), and finger temperature (p = 0.004). In men, multiple stepwise regression analysis showed that a higher SpO2 was correlated with a lower heart rate (β =-0.089, p = 0.007) and a higher finger temperature (β = 0.308, p = 0.030) (r 2 for model = 0.18). In women, a higher SpO2 was significantly correlated with a higher finger temperature (β = 0.391, p = 0.015) (r 2 for model = 0.12). A higher SpO2 was related to a higher finger temperature (β = 0.286, p = 0.014) and a lower heart rate (β =-0.052, p = 0.029) in all participants (r 2 for model = 0.21). Residual analysis showed that individual SpO2 values were randomly plotted.Conclusion: Random plots of SpO2 on residual analysis indicated that these variations were random error, such as biological variation. A higher SpO2 was related to a lower heart rate and finger temperature in men, but a higher SpO2 was related to finger temperature in women. These results suggest that there are individual variations and sex differences in the hemodynamic responses of high-altitude adaptation in Andean highlanders., Journal of Physiological Anthropology, 39(1), art.no.31; 2020

Hemophagocytic lymphohistiocytosis (HLH) is an uncontrolled hyperinflammatory disorder driven by an overactive immune system that results in high mortality. Post-transplant-associated hemophagocytic lymphohistiocytosis (PT-HLH) is a type of secondary HLH that occurs following allogeneic hematopoietic stem cell transplantation (allo-HSCT). The clinical features of PT-HLH remain unclear and diagnostic and prognostic tools have not yet been established. Here, we retrospectively evaluated the clinical manifestations and outcomes of PT-HLH in 94 patients who underwent allo-HSCT. According to our PT-HLH criteria (hyperferritinemia and increased macrophage count in bone marrow), PT-HLH occurred in 12 patients (12.8%). The PT-HLH patients showed splenomegaly (P = .001), a higher risk of engraftment failure (P = .013), and an increased percentage of macrophages and hemophagocytes in bone marrow aspirates (P = .0009 and P = .0006, respectively). Moreover, univariate and multivariate analyses revealed that the survival rate was lower in PT-HLH patients than non-PT-HLH patients (P = .0017 and P = .034, respectively). This study defines the clinical features of PT-HLH and PT-HLH criteria that could be useful tools for diagnosing PT-HLH.

Background and aim Mesenteric malperfusion is a complication with a higher risk of in-hospital mortality because diagnosing mesenteric ischemia before necrotic change is difficult, and when it occurs, the patient's condition has worsened. Although it contradicts the previous consensus on central repair-first strategy, the revascularization-first strategy was found to be significantly associated with lower mortality rates. This study aimed to present our revascularization-first strategy and the postoperative results for acute aortic dissection involving mesenteric malperfusion. Methods Among 58 patients with acute type A aortic dissection at our hospital between January 2017 and December 2019, mesenteric malperfusion was noted in six. Four hemodynamically stable patients underwent mesenteric revascularization with endovascular intervention in a hybrid operation room before central repair, and two hemodynamically unstable patients underwent central repair before mesenteric revascularization. Results No in-hospital mortality was recorded. All four patients with mesenteric revascularization-first strategy recovered with no symptoms related to mesenteric ischemia. Two patients with central repair-first strategy developed paralytic ileus for 1 week; one of them needed exploratory laparotomy, but no patients needed colon resection. Conclusion No in-hospital mortality was recorded. All four patients with mesenteric revascularization-first strategy recovered with no symptoms related to mesenteric ischemia. Two patients with central repair-first strategy developed paralytic ileus for 1 week; one of them needed exploratory laparotomy, but no patients needed colon resection.

Background Xylitol is an approved food additive that is widely used as a sweetener in many manufactured products. It is also used in pharmaceuticals. Secondary oxalosis resulting from high dietary oxalate has been reported. However, reported cases of oxalosis following xylitol infusion are rare. Case presentation A 39-year-old man with a 16-year history of organic psychiatric disorder was hospitalized for a laparoscopic cholecystectomy because of cholecystolithiasis. He had been treated with several antipsychotics and mood stabilizers, including lithium. The patient had polyuria (> 4000 mL/day) and his serum sodium levels ranged from 150 to 160 mmol/L. Urine osmolality was 141 mOsm/L, while serum arginine vasopressin level was 6.4 pg/mL. The patient was diagnosed with nephrogenic diabetes insipidus (NDI), and lithium was gradually discontinued. Postoperative urine volumes increased further to a maximum of 10,000 mL/day, and up to 10,000 mL/day of 5% xylitol was administered. The patient’s consciousness level declined and serum creatinine increased to 4.74 mg/dL. This was followed by coma and metabolic acidosis. After continuous venous hemodiafiltration, serum sodium improved to the upper 140 mmol/L range and serum creatinine decreased to 1.25 mg/dL at discharge. However, polyuria and polydipsia of approximately 4000 mL/day persisted. Renal biopsy showed oxalate crystals and decreased expression of aquaporin-2 (AQP2) in the renal tubules. Urinary AQP2 was undetected. The patient was discharged on day 82 after admission. Conclusions Our patient was diagnosed with lithium-induced NDI and secondary oxalosis induced by excess xylitol infusion. NDI became apparent perioperatively because of fasting, and an overdose of xylitol infusion led to cerebrorenal oxalosis. Our patient received a maximum xylitol dose of 500 g/day and a total dose of 2925 g. Patients receiving lithium therapy must be closely monitored during the perioperative period, and rehydration therapy using xylitol infusion should be avoided in such cases.

We here report a case of dermatomyositis associated with ureteral cancer. A 69-year-old male presented to our hospital complaining of a rash on his whole body, which resulted in a clinical diagnosis of dermatomyositis. Right ureteral cancer was suspected on computed tomographic CT examination during the investigation for the underlying malignancy. The patient was treated with prednisolone and right nephroureterectomy with bladder cuffing for dermatomyositis and ureteral cancer, respectively. One year after surgery, the dermatomyositis worsened, and CT examination showed local recurrence and lymph node metastasis. Chemotherapy was performed, and CT examination 3 months after treatment showed that the tumor had shrunk. Skin symptoms were also ameliorated. Chemotherapy was given intermittently thereafter. The tumor then increased and skin symptoms reappeared ; 3 years after surgery the patient's general condition deteriorated, resulting in death. Ureteral cancer with dermatomyositis is rare, but dermatomyositis complicated with malignancy has a poor prognosis. Careful whole body search may be useful for early detection of malignancy.

With the increasing incidence of dementia worldwide, the frequent use of amyloid and tau positron emission tomography imaging requires low-dose protocols for the differential diagnoses of various neurodegenerative diseases and the monitoring of disease progression. In this study, we investigated the feasibility to reduce the PET dose without a significant loss of quantitative accuracy in 3D dynamic row action maximum likelihood algorithm-reconstructed PET images using [11C]PIB and [18F]THK5351. Eighteen cognitively normal young controls, cognitively normal elderly controls, and patients with probable Alzheimer’s disease (n = 6 each), were included. Reduced doses were simulated by randomly sampling half and quarter of the full counts in list mode data for one independent realization at each simulated dose. Bias was evaluated between the reduced dose from the full dose of standardized uptake value ratio (SUVR), distribution volume ratio (DVR) from reference Logan, and non-displaceable binding potential (BPND) from simplified reference tissue model (SRTM). DVR yielded the least bias at low dose compared to SUVR and BPND, and thus, is highly recommended. The dose of [18F]THK5351 and [11C]PIB can be reduced to a quarter of the full dose using DVR for evaluation, whereas the dose can only be reduced to half and a quarter of the full dose for [18F]THK5351 and [11C]PIB using SUVR. BPND showed inconsistent trend and large bias at low dose. The feasibility of dose reduction was dependent on the selected parameters of interest, reconstruction algorithms, reference regions, and to a lesser degree by motion effects.

Situated at high positions on marine food webs, seabirds accumulate high concentrations of persistent organic pollutants (POPs), such as polychlorinated biphenyls (PCBs), dichlorodiphenyltrichloroethane and its metabolites (DDTs), and hexachlorocyclohexanes (HCHs). Our previous studies proposed the usefulness of seabirds preen gland oil as a nondestructive biomonitoring tool. The present study applied this approach to 154 adult birds of 24 species collected from 11 locations during 2005-2016 to demonstrate the utility of preen gland oil as a tool for global monitoring POPs, i.e., PCBs, DDTs, and HCHs. Concentrations of the POPs were higher in the Northern Hemisphere than in the Southern Hemisphere. In particular, ∑20PCBs and∑DDTs were highly concentrated in European shags (Phalacrocorax aristotelis) and Japanese cormorants (Phalacrocorax capillatus), explainable by a diet of benthic fishes. Higher concentrations of γ-HCH were detected in species from the polar regions, possibly reflecting the recent exposure and global distillation of ∑HCHs. We examined the relationship between age and POP concentrations in preen gland oil from 20 male European shags, aged 3-16 years old. Concentrations and compositions of POPs were not related to age. We also examined sex differences in the POP concentrations from 24 streaked shearwaters (Calonectris leucomelas) and did not detect a sex bias. These results underline the importance of the geographic concentration patterns and the dietary behavior as determinants species-specific POPs concentrations in preen gland oil.

Rapid immune recovery following allogeneic hematopoietic stem cell transplantation (allo-HSCT) is important for clinical outcome prediction. In most studies, immune recovery after allo-HSCT is monitored via peripheral blood. However, few reports regarding the status of absolute lymphocyte subsets in the bone marrow (BM) microenvironment have been undertaken. Therefore, we evaluated the clinical impact of immune recovery in the early period following allo-HSCT using BM samples. We showed that delayed natural killer cell recovery was independently associated with a poor prognosis for overall survival (hazard ratio [HR], 3.07; 95% confidence interval [CI], 1.37- 6.89; P = .007), progression-free survival (HR, 3.42; 95% CI, 1.47-7.94; P = .004), and nonrelapse mortality (HR, 6.68; 95% CI, 1.82-25.0; P = .004) by multivariate analysis. In addition, low NK cell counts were associated with the presence of 1 or more bacterial, viral, or fungal infections. Our results indicate that investigating absolute lymphocyte subsets in BM in the early phase following allo-HSCT can be useful for predicting and improving survival outcomes.

We report the successful repair in a 29-year-old man who experienced penetration of the ascending aorta and bronchus by a crossbow bolt. Imaging studies revealed the arrow penetrating the sternum, right lung, ascending aorta and right bronchus, with mediastinal emphysema. The top of the arrow (8 mm in diameter) was deeply embedded in the body of the thoracic vertebra. The arrow was successfully removed while maintaining a stable respiratory condition by performing differential lung ventilation. There was no need for cardiopulmonary bypass. To our knowledge, this is the first case of a through-and-through penetrating ascending aorta injury.

Background Glycosylation is a common posttranslational modification in protein biosynthesis that is implicated in several disease states. It has been reported that specific protein glycan structures are useful as biomarkers for cancer and some neuropsychiatric diseases; however, the relationship between plasma protein glycosylation and major depressive disorder (MDD) has not been investigated to date. The aim of this study was to determine whether plasma protein glycan structures are altered in depression using a stress-based mouse model and samples from patients with MDD. Methods We used chronic ultra-mildly stressed mice that were untreated or treated with imipramine as mouse models of depression and remission, respectively. We also made comparisons between samples from depressed and remitted patients with MDD. Protein glycosylation was analyzed using a lectin microarray that included 45 lectins with binding affinities for various glycan structures. Results Sia-alpha2-6Gal/GalNAc was a commonly altered glycan structure in both depression model mice and patients with MDD. Moreover, the expression of ST6GALNAC2 was decreased in leukocytes from patients with MDD. Limitations Our study samples were small and we did not identify specific alpha2-6Gal/GalNAc-sialylated proteins. Conclusions The glycan structure Sia-alpha2-6GalNAc in plasma protein and ST6GALNAC2 expression in peripheral leukocytes may have utility as candidate biomarkers for the clinical diagnosis and monitoring of MDD.

Although literature evidence suggests deficits in social and non-social cognition in patients with autistic spectrum disorder (ASD) and schizophrenia (SCZ), the difference in neural correlates of the impairments between the two disorders has not been elucidated. We examined brain function in response to a non-social cognition and a social cognition task using functional near-infrared spectroscopy (fNIRS) in 13 patients with ASD, 15 patients with SCZ, and 18 healthy subjects. We assessed the brain function of participants using a verbal fluency task and an emotional facial recognition task. The patients with ASD showed significantly reduced brain activation in the left frontotemporal area during both tasks compared to healthy subjects. The patients with ASD with larger score in ‘attention to detail’ in the autism spectrum quotient showed lower activation of the left frontotemporal area during the two tasks. The patients with SCZ showed significantly reduced activation, compared to healthy subjects, and greater activation, compared to patients with ASD, in the area during the verbal fluency task. The patients with SCZ with more severe symptoms had lower brain activation during the task in this area. Our results suggest that two distinct areas are involved in the distinctive brain pathophysiology relevant to cognitive processing in patients with ASD and SCZ.

Bendamustine has demonstrated favourable efficacy in relapsed or refractory indolent lymphoma and mantle cell lymphoma. We retrospectively evaluated the pre-treatment clinical and laboratory factors and their correlation with the clinical outcome of these lymphomas. We analysed 53 patients who had been treated with bendamustine alone (n = 6) or rituximab plus bendamustine (n = 47). The overall response rate was 81.1%, with a complete response (CR) rate of 39.6%. The CR rate was significantly low in patients who had elevated levels of soluble interleukin-2 receptor (p = 0.024) and C-reactive protein (CRP; p = 0.004). The 1-year overall survival (OS) rate was 79.3%. An elevated CRP was associated with a short OS (p = 0.056). The present findings suggest that the lymphoma microenvironment and immune response were involved in the effects of bendamustine. These findings are also important in order to understand the pathophysiology of refractory lymphoma and to find effective strategies using bendamustine.

Pulmonary hypertension (PH) with kyphoscoliosis-related alveolar hypoventilation is uncommon, so little is known about the effectiveness of treatments for this condition. A 66-year-old man with kyphosis who had been treated with nocturnal noninvasive positive-pressure ventilation developed PH with a mean pulmonary arterial pressure (PAP) of 32 mmHg and a pulmonary vascular resistance (PVR) of 5.95 Wood units. After addition of oxygen therapy and tadalafil, his condition improved. One year later, his mean PAP and PVR were 25 mmHg and 3.62 Wood units, respectively. This case shows the therapeutic potential of vasoactive medications for alveolar hypoventilation-related PH.

Strong evidence exists regarding multiple benefits of physical activity among cancer patients. Patients undergoing esophagectomy received counseling for physical activity by a nurse and instructions to keep a diary of physical activities before surgery, followed by 2 counseling sessions after surgery. Physical activity, body mass index, psychological distress, and quality of life were measured at baseline, 2-4 weeks, and 3 and 6 months after discharge. Of 29 participants (mean age = 65.9 years), 72.5% underwent thoracoscopic esophagectomy in the prone position with 3-field lymphadectomy. The results of the International Physical Activity Questionnaire indicated that postoperative physical activity returned to nearly preoperative level, and the number of inactive patients gradually decreased by half at 6 months after discharge. Quality of life scores, except those for role function and social function, tended to return to the baseline at 6 months after discharge. Psychological distress measured by the Kessler 6 remained lower than the baseline, whereas the mean score of body mass index gradually declined after discharge. Patients undergoing esophagectomy and receiving nurse counseling can return to preoperative physical activity level in their daily lives after surgery. Nurses should integrate physical activity promotion into cancer care.

Humans have spread out all over the world adapting to many different cold environments. Recent worldwide genome analyses and animal experiments have reported dozens of genes associated with cold adaptation. The uncoupling protein 1 (UCP1) gene enhances thermogenesis reaction in a physiological process by blocking ATP (adenosine triphosphate) synthesis on a mitochondrial membrane in brown adipose tissues. To our knowledge, no previous studies have shown an association between variants of the UCP1 gene and physiological phenotypes concerning non-shivering thermogenesis (NST) under the condition of low temperature in humans. We showed that the degree of NST for healthy subjects in an artificial climate chamber is significantly different among UCP1 genotypes. Defining the haplotypes covering the UCP1 region (39.4?kb), we found that the frequency of the haplotype with the highest NST was significantly correlated with latitudes and ambient temperature. Thus, the data in this study provide the first evidence that the UCP1 genotype alters the efficiency of NST in humans, and likely supports the hypothesis that the UCP1 gene has been related to cold adaptation in human evolutionary history., Scientific Reports, 7, 5570; 2017

Background Depression in old age is an increasing contributor to poor health and accompanying health care costs. Although there is an abundance of literature on later-life depression (LLD), the neural correlates have not been clarified. The aim of this study was to determine whether patients with LLD show abnormal gray matter volume (GMV) and white matter integrity by using multiple image analysis methods. Methods The study included 45 patients with LLD and 61 healthy participants who were matched for age, sex, years of education, and vascular risk factors. GMV was examined using voxel-based morphometry, while the white matter integrity was determined by tract-based spatial statistics and tract-specific analysis, which were obtained from high-resolution magnetic resonance images. Results Patients with LLD showed significantly less GMV in the orbitofrontal cortex, anterior cingulate, insula, amygdala, and temporal regions, as well as higher fractional anisotropy in the uncinate fasciculus, compared with healthy participants. Patients with LLD who had reduced orbitofrontal and insular GMV had more severe clinical variables. The reduced orbitofrontal GMV was associated with higher fractional anisotropy in the uncinate fasciculus. Limitation The effects of medication should also be considered when interpreting the results of this study. Conclusion Our results suggest that regional GMV is linked to white matter integrity of the uncinate fasciculus in the orbitomedial prefrontal limbic network, and the disruption of this network may be involved in the pathophysiology of LLD.

Although follicular lymphoma (FL) is a pathological entity characterized by relatively uniform histological and molecular findings, its clinical course is highly variable. Establishment of therapeutic strategies based on a simple and practical prognostic model is important. C-reactive protein (CRP) is an adverse prognostic marker for various tumors and aggressive lymphomas. However, the significance of serum CRP levels as a prognostic index in low-grade lymphomas, such as FL, has not been thoroughly investigated. We retrospectively analyzed the relationship between serum CRP levels at diagnosis and the prognosis in patients with FL (n = 61) undergoing rituximab-containing chemotherapy. Elevated CRP levels showed a significant association with elevated fibrinogen (P = 0.002) in univariate analysis. Patients with higher CRP levels (> 5 mg/L) had a significantly shorter progression-free survival in multivariate analysis (P = 0.044). We concluded that serum CRP levels are important in prognostic stratification of patients with FL.

Hematopoietic stem cells (HSCs) are quiescent cells in the bone marrow niche and are relatively dependent on glycolytic ATP production. On the other hand, differentiated cells, including hematopoietic progenitor cells (HPCs), preferentially generate ATP via oxidative phosphorylation. However, it is unclear how cellular differentiation and the cell cycle status affect nutritional requirements and ATP production in HSCs and HPCs. Using a newly developed culture system, we demonstrated that survival of HPCs was strongly dependent on glucose, whereas quiescent HSCs survived for a certain duration without glucose. Among HPCs, granulocyte/monocyte progenitors (GMPs) were particularly dependent on glucose during proliferation. By monitoring the ATP concentration in live cells, we demonstrated that the ATP level was maintained for a short duration without glucose in HSCs, possibly due to their metabolic flexibility. In addition, HSCs exhibited low ATP turnover, whereas HPCs including GMPs demonstrated high ATP turnover and required efficient ATP production from glucose. These findings show that ATP turnover and nutritional requirements differ between HSCs and HPCs according to the cell cycle and differentiation status.

Utilizing the Great Ape Information Network (GAIN), an open-access nationwide database containing the detailed life history information of all apes who have lived or currently reside in Japan, we present a robust chimpanzee life table by single year of age and sex including 821 individuals spanning nearly a century, current through March 11, 2019. While the demographic composition and status of captive chimpanzees in Japan has been previously reported, longevity and mortality statistics have not. We show that female and male survivorship do not differ significantly, and that a live-born chimpanzee in Japan can expect to live 28.3 years (females 26.3, males 30.3). Life expectancy increases to 34.6 years (females 33.4, males 35.7) for individuals who reach one year of age, and to 40.4 years (females 39.2, males 41.5) for those who survive to adulthood. The oldest chimpanzee in Japan, a wild-born male, lived an estimated 68 years. One in six infants are stillborn, and nearly 80% of all infants born alive survive beyond their first birthday. Finally, we report that a seasonal death pattern exists and chimpanzees in Japan are more likely to decease in the winter months (Dec–Feb) than in any other season.

BACKGROUND Immunoglobulin (Ig) G2 subclass deficiency is known to be associated with recurrent bacterial respiratory infections caused by capsulated bacteria and is found mostly in pediatric patients. However, its impact after allogeneic hematopoietic stem cell transplantation (HSCT) has not been fully assessed. METHODS We retrospectively evaluated the relationship between IgG2 subclass levels and bacterial pneumonia in 74 adult patients who survived longer than 2 years after allogeneic HSCT. RESULTS During the evaluation period, nine patients developed bacterial pneumonia. The median IgG2 level was significantly lower in patients with an infectious episode than in those without (143 mg/dL vs 287 mg/dL; P

Corticobasal syndrome (CBS) is pathologically characterized by tau deposits in neuronal and glial cells and by reactive astrogliosis. In several neurodegenerative disorders,Baseline and 1-year follow-up imaging were acquired using magnetic resonance imaging and positron emission tomography withThe 1-year follow-up scan images revealed thatLongitudinal increases in

Although patients with schizophrenia demonstrate abnormal processing of emotional face recognition, the neural substrates underlying this process remain unclear. We previously showed abnormal fronto-temporal function during facial expression of emotions, and cognitive inhibition in patients with schizophrenia using functional near-infrared spectroscopy (fNIRS). The aim of the current study was to use fNIRS to identify which brain regions involved in recognizing emotional faces are impaired in patients with schizophrenia, and to determine the neural substrates underlying the response to emotional facial expressions per se, and to facial expressions with cognitive inhibition. We recruited 19 patients with schizophrenia and 19 healthy controls, statistically matched on age, sex, and premorbid IQ. Brain function was measured by fNIRS during emotional face assessment and face identification tasks. Patients with schizophrenia showed lower activation of the right precentral and inferior frontal areas during the emotional face task compared to controls. Further, patients with schizophrenia were slower and less accurate in completing tasks compared to healthy participants. Decreasing performance was associated with increasing severity of the disease. Our present and prior studies suggest that the impaired behavioral performance in schizophrenia is associated with different mechanisms for processing emotional facial expressions versus facial expressions combined with cognitive inhibition.

Pneumonia is the third largest cause of death in Japan. Chest physicians have been struggling to improve the outcome of pneumonia treatment in acute care settings. However, a poor long-term prognosis after pneumonia has not been well recognized. Furthermore, the factors related to the poor prognosis, especially the possible involvement of senescence-related disability, have not been identified. In this study, long-term outcomes after discharge from hospital were retrospectively analyzed to identify factors related to the poor long-term prognosis. Outcomes of 958 pneumonia patients who were discharged from South Miyagi Medical Center (Miyagi, Japan) from June 1, 2008 to March 31, 2014 were determined through patient surveys or medical record reviews on September 26, 2014. Survival curves were constructed and compared according to various factors. Multivariate analysis revealed that all levels of decrease in physical activity, an age of 80 years old or more, the most severe status in Japanese Respiratory Society pneumonia severity grading system, the presence of antibiotic-resistant bacteria, and comorbid malignancy significantly reduced long-term survival. The effects of dementia, neuromuscular disease, heart disease, and nursing care residency on long-term survival were detected only with univariate analysis. Physical activity influenced the acute-phase and the long-term prognosis of pneumonia. This report provides information to assist physicians in giving better suggestions to disabled older patients when choosing pneumonia treatment options. In conclusion, we propose that death related to pneumonia can be prevented in the same way as non-communicable diseases by improving physical activity.

We investigated the efficacy, safety, and clinical significance of trafermin, a recombinant human fibroblast growth factor (rhFGF)-2, for periodontal regeneration in intrabony defects in Phase III trials. Study A, a multicenter, randomized, double-blind, placebo-controlled study, was conducted at 24 centers. Patients with periodontitis with 4-mm and 3-mm or deeper probing pocket depth and intrabony defects, respectively, were included. A total of 328 patients were randomly assigned (2:1) to receive 0.3% rhFGF-2 or placebo, and 323 patients received the assigned investigational drug during flap surgery. One of the co-primary endpoints, the percentage of bone fill at 36 weeks after drug administration, was significantly greater in the rhFGF-2 group at 37.131% (95% confidence interval [CI], 32.7502 to 41.5123; n = 208) than it was in the placebo group at 21.579% (95% CI, 16.3571 to 26.8011; n = 100; p < 0.001). The other endpoint, the clinical attachment level regained at 36 weeks, was not significantly different between groups. Study B, a multicenter, randomized, blinded (patients and evaluators of radiographs), and active-controlled study was conducted at 15 centers to clarify the clinical significance of rhFGF-2. Patients with 6-mm and 4-mm or deeper probing pocket depth and intrabony defects, respectively, were included. A total of 274 patients were randomly assigned (5:5:2) to receive rhFGF-2, enamel matrix derivative (EMD), or flap surgery alone. A total of 267 patients received the assigned treatment during flap surgery. The primary endpoint, the linear alveolar bone growth at 36 weeks, was 1.927 mm (95% CI, 1.6615 to 2.1920; n = 108) in the rhFGF-2 group and 1.359 mm (95% CI, 1.0683 to 1.6495; n = 109) in the EMD group, showing non-inferiority (a prespecified margin of 0.3 mm) and superiority of rhFGF-2 to EMD. Safety problems were not identified in either study. Therefore, trafermin is an effective and safe treatment for periodontal regeneration in intrabony defect, and its efficacy was superior in rhFGF-2 compared to EMD treatments.

Previous studies have reported a paradox that cigarette smoking reduces stress psychologically; however, it increases the arousal level physiologically. To examine this issue, our study aimed to investigate whether cigarette smoking relieves stress by measuring the late positive potential (LPP), a component of the event-related potential (ERP). In Experiment 1, participants first watched emotionally neutral images; second, they received a break; and finally, they watched emotionally neutral images again. In the break, they smoked a cigarette (smoking condition) or simply rested without smoking (non-smoking condition). The procedure of Experiment 2 was the same as that of Experiment 1, except that the participants watched unpleasant images as stress stimuli before the break. In Experiment 1, the LPP decreased from before to after the break in the smoking condition, but not in the non-smoking condition, suggesting that smoking cigarettes in the neutral state reduces the arousal level. In Experiment 2, the LPP for 400–600ms decreased from before to after the break, both in the smoking and non-smoking conditions; however, the LPP for 200–400ms decreased from before to after the break only in the smoking condition. This suggests the possibility that cigarette smoking in the unpleasant state may facilitate a decrease in the arousal level faster than with non-smoking. In both Experiments 1 and 2, the subjective rating results also suggested that cigarette smoking decreased anxiety. Taken together, both the physiological (LPP) and the psychological responses from our study suggest that cigarette smoking perhaps relieves stress.

A 26-year-old man presented with fever, multiple lymphadenopathies, polyclonal hypergammaglobulinemia, and an elevated serum interleukin-6 (IL-6) level. Multicentric Castleman disease (MCD) was diagnosed by lymph node biopsy. Treatment with prednisolone (PSL) was initiated; however, its efficacy was limited. During PSL tapering, rapidly progressive anemia and thrombocytopenia developed concurrently with increased reticulocyte level, elevated serum LDH level, decreased haptoglobin level, and positive direct Coombs test. Based on these findings, Evans syndrome, which is a concurrent development of autoimmune hemolytic anemia and immune thrombocytopenia, was confirmed. The PSL dose was increased but was ineffective. Therefore, treatment with tocilizumab was initiated, and the clinical findings of both MCD and Evans syndrome improved. The clinical course of this case suggests that tocilizumab could be a treatment option for Evans syndrome complicated with MCD. Three other cases of Evans syndrome complicated with MCD have also been reported; however, this is the first case that shows the efficacy of tocilizumab as treatment for both MCD and Evans syndrome.

The prognosis of acute promyelocytic leukemia (APL) has been improved by the combination of all-trans retinoic acid (ATRA) with chemotherapy. Nonetheless, relapse occurs in a certain proportion of patients, mostly within three to four years after treatment. We herein report a patient treated with ATRA and chemotherapy achieving remission who relapsed approximately 17 years after the treatment. A literature review identified 5 additional reported cases of APL relapse after more than 10 years. None of them presented with generally established risk factors for relapse, such as a high leukocyte count. The potential for late relapse of APL occurring more than 10 years after treatment should be recognized.

Recent positron emission tomography (PET) studies have demonstrated the accumulation of tau PET tracer in the affected region of progressive supranuclear palsy (PSP) cases. To confirm the binding target of radiotracer in PSP, we performed an imaging-pathology correlation study in two autopsy-confirmed PSP patients who underwent [18F]THK5351 PET before death. One patient with PSP Richardson syndrome showed elevated tracer retention in the globus pallidus and midbrain. In a patient with PSP-progressive nonfluent aphasia, [18F]THK5351 retention also was observed in the cortical areas, particularly the temporal cortex. Neuropathological examination confirmed PSP in both patients. Regional [18F]THK5351 standardized uptake value ratio (SUVR) in antemortem PET was significantly correlated with monoamine oxidase-B (MAO-B) level, reactive astrocytes density, and tau pathology at postmortem examination. In in vitro autoradiography, specific THK5351 binding was detected in the area of antemortem [18F]THK5351 retention, and binding was blocked completely by a reversible selective MAO-B inhibitor, lazabemide, in brain samples from these patients. In conclusion, [18F]THK5351 PET signals reflect MAO-B expressing reactive astrocytes, which may be associated with tau accumulation in PSP. Electronic supplementary material The online version of this article (10.1186/s40478-018-0556-7) contains supplementary material, which is available to authorized users.

Aberrant expression of the interleukin-3 receptor alpha chain (IL3RA or CD123) is frequently observed in patients with a subset of leukemic disorders, including acute myeloid leukemia (AML), particularly in leukemia stem cells. We analyzed the relationships between immunohistochemical (IHC) expression, including that of CD123, and clinical outcomes. This study involved a retrospective analysis of 48 patients diagnosed with de novo AML (M0–M5, n = 48) at our hospital between February 2008 and September 2015. Among patients with de novo AML, CD123 expression was associated with a failure to achieve complete response (CR) to initial induction chemotherapy (P = 0.044) and poor overall survival (OS) (P = 0.036). This is the first study using IHC to demonstrate that CD123 expression is associated with a poor CR rate and poor OS in de novo AML patients. These results support previous reports using flow cytometry (FCM). CD123 expression may thus be useful for assessing AML patients’ prognoses. At the time of diagnosis, CD123 expression analysis using IHC may represent a clinically useful assessment for de novo AML patients.

Department of Rheumatology, Tokyo Metropolitan TamaMedical Center, Fuchu City, Tokyo, Japan; Division of Infectious Diseases, Tokyo Metropolitan TamaMedical Center, FuchuCity, Tokyo, Japan; Division of Rheumatology, NagoyaMedical Center, Nagoya, Japan; University of California, San Francisco, San Francisco, CA, USA; Faculte de Medecine, Unite des Rickettsies, Marseille, France; Japan Community Healthcare Organization, Tokyo, Japan.

Visualization of the spatial distribution of neurofibrillary tangles would help in the diagnosis, prevention and treatment of dementia. The purpose of the study was to evaluate the clinical utility of [(18)F]THK-5117 as a highly selective tau imaging radiotracer.We initially evaluated in vitro binding of [(3)H]THK-5117 in post-mortem brain tissues from patients with Alzheimer's disease (AD). In clinical PET studies, [(18)F]THK-5117 retention in eight patients with AD was compared with that in six healthy elderly controls. Ten subjects underwent an additional [(11)C]PiB PET scan within 2 weeks.In post-mortem brain samples, THK-5117 bound selectively to neurofibrillary deposits, which differed from the binding target of PiB. In clinical PET studies, [(18)F]THK-5117 binding in the temporal lobe clearly distinguished patients with AD from healthy elderly subjects. Compared with [(11)C]PiB, [(18)F]THK-5117 retention was higher in the medial temporal cortex.These findings suggest that [(18)F]THK-5117 provides regional information on neurofibrillary pathology in living subjects.