Search

Background: The appropriateness of percutaneous coronary intervention (PCI) for chronic total occlusion (CTO) lesions has rarely been investigated. Methods and Results: The Japanese CTO-PCI Expert Registry enrolled consecutive patients undergoing CTO-PCI carried out by highly experienced Japanese CTO specialists who performed more than 50 CTO-PCIs per year and 300 CTO-PCIs in total. This study included patients undergoing CTO-PCI between January 2014 and December 2019. The appropriateness, trends, and differences among the procedures performed by the operators using the 2017 appropriate use criteria were analyzed. Furthermore, we performed a logistic regression analysis to assess whether the appropriateness was associated with in-hospital major adverse cardiovascular and cerebrovascular events (MACCE). Of the 5, 062 patients who underwent CTO-PCI, 4, 309 (85.1%) patients who did not undergo the non-invasive stress test were classified as having no myocardial ischemia. Of the total cases, 3, 150 (62.2%) were rated as “may be appropriate, ” and 642 (12.7%) as “rarely appropriate” CTO-PCI cases. The sensitivity analyses showed that the number (%) of “may be appropriate” ranged from 4, 125 (57.8%) to 4, 744 (66.4%) and the number of “rarely appropriate” ranged from 843 (11.8%) to 970 (13.6%) among best and worst scenarios. Conclusions: In a large Japanese CTO-PCI registry, approximately 13% of CTO-PCI procedures were classified as “rarely appropriate”. Substantial efforts would be required to decrease the number of “rarely appropriate” CTO-PCI procedures.

Introduction Although the mortality rates associated with Japanese spotted fever (JSF) are unknown, advances in testing technology have led to an increase in JSF-induced mortality reported in clinical practice. Up-to-date clinical information is essential for accurate diagnosis and prompt treatment of JSF. Methods This retrospective descriptive study included patients with JSF who were treated at the Ise Red Cross Hospital between 2006 and 2019. Diagnostic criteria included positive results of molecular-based tests during the acute phase and/or increased serum-specific antibody titers. This study was performed based on the clinical findings, clinical course, treatment, and prognosis in confirmed cases of JSF. Results We investigated 239 patients with a confirmed diagnosis of JSF (48.1% men, mean age 69.2 years). Notably, 237 patients received tetracycline antibiotics, and eight patients died (one patient was misdiagnosed and died without adequate treatment). Four of the remaining patients had a multi-organ failure at the time of admission. However, among the 155 consecutive patients who received effective antibiotic therapy after 2012, we observed two deaths; one patient died of hemorrhage secondary to non-steroidal anti-inflammatory drug-induced duodenal ulcer. Conclusions Our study showed a case fatality rate of 3.3%, which indicates that JSF is a severe illness. Although a few cases of the fulminant disease are reported, early initiation of therapy was shown to improve JSF-induced mortality by approximately 1%. Prompt initiation of antibiotic therapy (even in the absence of genetic test results) is warranted in cases of suspected JSF.

In this study, we report a case of a patient on dialysis who presented necrotic lesions on the legs and penile ulceration 7 years after a mechanical aortic valve replacement. The diagnosis of calciphylaxis was not confirmed even after skin biopsy, and multidisciplinary management was not initiated until the patient was admitted with septic shock. Cardiovascular surgeons should be aware of warfarin-induced calciphylaxis, whose pathophysiology differs from that of atherosclerosis. Considering poor long-term survival of dialysis patients, mechanical valves should be reserved only for those patients whose estimated survival is longer than the time taken for a biological valve to deteriorate.

BACKGROUND In-stent chronic total occlusion (CTO) presents various occlusion patterns, which complicate percutaneous coronary intervention (PCI). AIMS The aim of the study was to investigate the initial outcome and strategy of PCI for in-stent CTO according to the angiographic occlusion patterns. METHODS This study assessed 791 in-stent CTOs from the Japanese CTO-PCI Expert Registry from 2015 to 2018. They were divided into four patterns: pattern A (n=419), CTO within the stent segment; pattern B (n=196), CTO beyond the distal edge; pattern C (n=85), CTO beyond the proximal edge; and pattern D (n=69) CTO beyond both the proximal and distal edges. RESULTS There were significant differences in the technical success rates (96.2%, 86.2%, 92.9%, and 75.4% for patterns A-D, respectively; p

Pathogenic-activating variants of interferon induced with Helicase C domain 1 (IFIH1) cause Singleton-Merten (S-M) syndrome, which accompanies acro-osteolysis, loss of permanent teeth, and aortic calcification, as well as causing Aicardi-Goutieres (A-G) syndrome, which shows progressive encephalopathy, spastic paraplegia, and calcification of basal ganglia. Recently, patients with overlapping syndromes presenting with features of S-M syndrome and A-G syndrome were reported. However, progression of clinical features of this condition has not been fully understood. We report a Japanese boy with a novel pathogenic IFIH1 variant who presented with clinical features of S-M syndrome and A-G syndrome.

We report a case of acute ischemic nephropathy in a patient with severe renal artery stenosis and bradycardia due to sick sinus syndrome. An 83-year-old Japanese woman with a history of hypertension was diagnosed with sick sinus syndrome and scheduled for pacemaker implantation. Four days prior to admission for the procedure, she experienced sudden-onset severe right flank pain that persisted for 1 day. On the day of admission, her serum creatinine level increased from 1.35 mg/dL, measured 2 weeks earlier, to 7.04 mg/dL. Laboratory examinations showed elevated C-reactive protein and lactate dehydrogenase levels. A computed tomography scan showed a severely atrophied left kidney, suggesting that it was non-functioning. Doppler ultrasonography of the right renal artery showed an extended acceleration time, suggesting proximal stenosis. Magnetic resonance imaging showed no enhancement in the proximal portions of the right renal artery, consistent with severe stenosis or occlusion. The patient developed severe bradycardia with lightheadedness; as a result, pacemaker implantation was performed on post-admission day 7. On day 10, digital subtraction angiography revealed diffuse severe stenosis of the right renal artery; intravascular ultrasonography suggested plaque rupture. Percutaneous transluminal renal angioplasty (PTRA) was performed and a drug-eluting stent was placed. On day 11, hemodialysis was performed owing to deteriorating renal function. The patient's renal function dramatically improved shortly thereafter. This case highlights the importance of PTRA for select patients, as it can potentially save some patients from chronic dialysis, and outlines the possible implications of bradycardia in the pathogenesis of ischemic nephropathy.

Purpose To evaluate whether the bony impingement lesion in elbow osteoarthritis can be removed accurately, as planned during arthroscopy, by using the computer-aided navigation system and performing mock surgery using 3-dimensional (3D)-printed bone models for clinical applications. Methods We performed mock surgery using 3D-printed plaster bone models of the humerus of 15 actual patients with elbow osteoarthritis. Two types of experiments were conducted to evaluate the surgical accuracy. Three surgeons performed the mock surgery, each with 15 bone models (total, 45 trials). Surgical accuracy was based on the mean of 45 trials. The differences in surgical accuracy among the 3 surgeons were also evaluated (mean 15 trials). The same surgeon performed 30 trials, and the difference in surgical accuracy between the first and the second halves was also evaluated (mean 15 trials). Results The spatial error in the entire elbow joint was 1.13 mm. In terms of resection volume, a mean of 8% more volume was resected than was planned, and 85% of the planned area was resected. In our experiments, the surgical accuracy was significantly lower in the anterior than in the posterior joint. Intrarater reliability was intraclass correlation (ICC)2,1 0.81 and inter-rater reliability was ICC1,1 0.87. Conclusions Surgery using computer-aided navigation systems for arthroscopic debridement of the elbow provided accuracy comparable to that in other joints. Arthroscopic debridement of elbow osteoarthritis requires advanced surgical skills because accurate identification of the bony impingement legion is difficult during surgery. Surgery using computer-aided navigation systems for arthroscopic debridement of the elbow will provide real-time tracking of both the surgical instruments and bony impingement lesions as well as solve the technical difficulties of arthroscopic surgery of the elbow joint. Clinical Relevance Level Basic Science Study; Computer Modeling.

Endobronchial resection using a bronchoscope is often selected as treatment for carcinoid tumors located in the central airways. However, massive bleeding is one of the most serious complications during bronchoscopic surgery. Here, we report the case of a 77‐year‐old female with a typical carcinoid tumor located in the right truncus intermedius who underwent bronchial artery embolization (BAE) one day before endobronchial intervention using a flexible bronchoscope. The tumor was successfully resected without bleeding. BAE prior to endobronchial resection of carcinoid tumors may be useful for reducing the risk of bleeding., We report a female case with a typical carcinoid tumor. She underwent bronchial artery embolization (BAE) before endobronchial intervention using a flexible bronchoscope. BAE prior to endobronchial resection of carcinoid tumors is useful for reducing the risk of bleeding.

Background: The efficacy of sodium-glucose cotransporter 2 (SGLT2) inhibitors in elderly patients with heart failure with preserved ejection fraction (HFpEF) remains unclear. Methods and Results: In a multicenter, controlled trial, the CANONICAL study, we enrolled 82 HFpEF (left ventricular ejection fraction [LVEF] ≥50%) patients with type 2 diabetes (T2D) aged ≥65 years, with plasma B-type natriuretic peptide (BNP) ≥100 pg/mL or plasma N-terminal pro BNP (NT-proBNP) ≥400 pg/mL or history of HF. Patients were randomly assigned to 2 groups and were administered either the SGLT2 inhibitor canagliflozin (100 mg/day) for 24 weeks or standard therapy. The primary endpoints were changes in body weight (BW) and BNP concentrations. Mean (±SD) patient age, body mass index, and LVEF were 75.7±6.5 years, 25.0±3.6 kg/m2 and 61.5±7.6%, respectively. At 24 weeks, BW was significantly lower in the canagliflozin than standard therapy group. The extent of BNP reductions at 4 weeks was significantly greater in the canagliflozin than standard therapy group (P

The validity of the Academic Research Consortium for High Bleeding Risk (ARC-HBR) definitions of early (1 year), late (1-4 years), and very late (4 years) bleeding events is unknown.Methods and Results:This study was performed on patients (n=3,453) implanted with second-generation drug-eluting stents (DES) between 2010 and 2013. Data on all criteria of the ARC-HBR definition were collected retrospectively. The primary endpoint was Bleeding Academic Research Consortium Type 3 or 5 bleeding events; the ischemic endpoint was a composite of cardiac death, myocardial infarction, and ischemic stroke. The mean follow-up period was 7.5 years. Compared with non-high bleeding risk (HBR) patients, HBR patients (n=1,840; 53.3%) had an increased risk of the primary endpoint (early events, 3.6% vs. 0.5% [P0.0001]; late events, 5.3% vs. 2.5% [P0.0001]; very late events, 5.5% vs. 2.1% [P0.0001]) and of ischemic events during follow-up. The discrimination ability of the ARC-HBR definition for late and very late bleeding events was comparable to that of early bleeding events (C statistics 0.679, 0.621, and 0.620, respectively) with high negative predictive value (96.6%, 95.1%, and 93.1%, respectively). Multivariate analysis revealed the different effects of individual criteria on bleeding events in each follow-up period.The ARC-HBR definition consistently identified patients at risk of long-term bleeding and ischemic events after second-generation DES implantation.

Background Endoscopic papillectomy of duodenal papillary tumors (PT) is indicated for adenomas or well-differentiated adenocarcinomas that do not involve the sphincter of Oddi. However, there is currently no reliable pre-operative method to diagnose the infiltration in the sphincter of Oddi.’ Insulin-like growth factor 2 mRNA protein 3 (IMP3) staining is reportedly associated with advanced disease stage and clinical outcomes in many carcinomas. The aim of this retrospective study was to investigate the ability of diagnosing sphincter of Oddi involvement in PT and predicting the prognoses using IMP3 immunohistochemistry. Methods Twenty-five resected specimens from patients with PT and 24 biopsy specimens from the same patients excluding one were immunostained for IMP3. The percentage of positive cells in the tumor was evaluated and compared with the final pathological diagnosis and prognosis. Results The final pathological diagnoses were adenoma in 5 patients and adenocarcinoma in 20 patients (no sphincter of Oddi involvement in 5 and involvement in 15). The ability to diagnose sphincter of Oddi involvement based on the percentage of IMP3-positive cells in resected specimens and tissue biopsies was the area under the curve 0.8 and 0.78, respectively, of the receiver operating characteristic curve, and the accuracies were 80.0% and 75.0% (cutoff value: 10%), respectively. Moreover, patients with an IMP3-positive cell rate of ≥ 10% had a significantly worse prognosis (log-rank test P = 0.01). Conclusion IMP3 immunostaining of resected and biopsy specimens from PT patients enables the diagnosis of sphincter of Oddi involvement objectively and is also effective in predicting the prognosis.

We report a case of immunotactoid glomerulonephritis (ITG) in a patient with cold agglutinins. An 86-year-old Japanese male with a history of hypertension, dyslipidemia, and gastric malignancy presented to our hospital for the evaluation of proteinuria and hematuria. He had an elevated blood pressure of 200/77 mmHg and edema of the lower extremities. Initial blood test results revealed an impaired renal function (creatinine, 1.37 mg/dL) and hypoalbuminemia (albumin, 2.6 g/dL). His estimated daily urinary protein was 5.89 g/g creatinine, meeting the diagnostic criteria for nephrotic syndrome. The selectivity index for proteinuria indicated low selectivity (0.329). We conducted a renal biopsy to identify the cause of nephrotic syndrome. Immunofluorescence microscopy demonstrated positive staining of IgM, C4, and C1q. Electron microscopy exhibited mesangial expansion with inflammatory cells and a lobular structure, suggesting membranoproliferative glomerulonephritis. Subendothelial deposits containing microtubular structures with a diameter of approximately 30–200 nm were found, concurrent with the criteria for the diagnosis of ITG. Screening for lymphoproliferative diseases and immunological abnormalities revealed a positive direct Coombs test result and the presence of cold agglutinins. Paraproteinemia was absent. The similarities between cold agglutinin disease and ITG, including the production of autoantibodies and involvement of complement pathways, raise the possibility that cold agglutinins played a role in the development of ITG; however, we were unable to prove it due to difficulties in detecting cold agglutinins on renal histology. We discuss the possible implications for pathogenesis considering prior reports on nephrotic syndrome being potentially associated with cold agglutinins.

Background The current guidelines have recommended to shorten the dual antiplatelet therapy duration in patients at high bleeding risk (HBR) to minimize the bleeding complications. The impact of ischemic risk factors (IRF) on long-term ischemic events in patients with and without HBR after percutaneous coronary intervention remains unclear. Methods The study population comprised 1219 patients who were treated with everolimus-eluting stents between 2010 and 2011. The mean follow-up period was 2996 ± 433 days. HBR was defined as set by the Academic Research Consortium. IRF was defined as high-risk features of stent-driven recurrent ischemic events endorsed in the 2017 European Society of Cardiology Guidelines. Major bleeding was defined as the occurrence of Bleeding Academic Research Consortium type 3 or 5 bleeding. Primary ischemic events included myocardial infarction, definite stent thrombosis, and cardiac death. Results The 1219 patients were divided into two groups: 614 patients at HBR (HBR group) and 605 patients at no HBR (non-HBR group). The rate of IRF patients was significantly higher in the HBR group than in the non-HBR group (81.4% versus 47.6%, P 0.001). The cumulative rate of ischemic events in the HBR group was significantly higher in IRF patients than in non-IRF patients (21.0% versus 7.0%, log rank P 0.001), whereas that in the non-HBR group was not significantly different between IRF and non-IRF patients (10.1% versus 6.3%, log rank P = 0.09). Conclusions More than 80% of HBR patients treated with everolimus-eluting stents were at IRF. A combination of HBR and IRF may increase the risk of long-term ischemic events.

Aim Patients with major depression present with an increased serum insulin-like growth factor-1 (IGF-1) concentration. However, the longitudinal relationship between serum IGF-1 levels and depression development remains unclear. This study aimed to investigate the longitudinal association between the serum IGF-1 concentration in the first trimester of pregnancy and postpartum depression development using data obtained from the Japan Environment and Children's Study (JECS). Methods The JECS included 97 415 pregnant women; among them, 8791 were enrolled in this study. Data regarding depression in the first trimester, postpartum depression development at 1 month after childbirth, and other covariates were collected using a self-administered questionnaire. Serum IGF-1 levels were measured in the first trimester of pregnancy. The participants were divided into four groups according to the serum IGF-1 level. Results In the first trimester, serum IGF-1 levels were not significantly associated with psychological distress in pregnant women. In the longitudinal analyses, however, postpartum depression development in mothers within the highest quartile for serum IGF-1 concentration in the first trimester was significantly less common than in those within the lowest quartile (odds ratio 0.48, 95% confidence interval 0.30-0.79). Conclusion Pregnant women with a high serum IGF-1 concentration in the first trimester were less likely to develop postpartum depression than those with a low concentration. A high serum IGF-1 concentration during pregnancy may help to protect against postpartum depression development.

Introduction Given that few studies investigated the efficacy of catheter ablation (CA) in patients with paroxysmal atrial fibrillation (AF) and atrial septal defect (ASD), this study evaluated its effectiveness in patients with paroxysmal AF and ASD. Methods and results Of the 216 patients who underwent ASD device closure at two hospitals, 36 patients had paroxysmal AF. After April 2012, CA for AF was performed before ASD device closure (ASD-CA group; n = 20). The ASD-CA group had a significantly higher AF-free survival rate after ASD device closure compared to patients without CA for AF before ASD device closure (ASD-non-CA group; n = 16) (ASD-CA group: 2 patients vs. ASD-non-CA group: 9 patients; follow-up period: 4.2 ± 2.5 years; log-rank p = 0.01). In addition, the AF-free survival rates were similar between the ASD-CA group and 80 paroxysmal AF patients who underwent CA without any detectable structural heart disease (non-SHD-CA group). The two groups were matched by propensity scores for age, sex, and left atrium dimension (ASD-CA group: 2 patients vs. non-SHD-CA group: 5 patients; follow-up period: 3.3 ± 1.8 years; log-rank p = 0.28). Conclusion CA for AF before ASD device closure might be an effective treatment option for patients with paroxysmal AF and ASD. This article is protected by copyright. All rights reserved.

Herein, we report two girls with a neonatal screening (NS)-negative 21-hydroxylase deficiency (21-OHD) requiring treatment with hydrocortisone due to virilization that developed in late childhood. Patient 1 was born prematurely on the 30th gestational week with normal external genitalia at birth. She passed the NS for 21-OHD. At 6 yr of age, she was referred to a hospital for evaluation of premature pubarche and clitoromegaly. Her diagnosis was central precocious puberty, and GnRH agonist was initiated. However, her symptoms did not improve despite treatment for over 4 years. She was then referred to our hospital where she was diagnosed with 21-OHD. Although she was started on hydrocortisone therapy, her adult height reached only 140 cm (−3.4 SD). Patient 2 was delivered at 37 weeks of gestation and passed the NS for 21-OHD. She was referred to a hospital because of premature pubarche at the age of 6 yr. She was diagnosed with 21-OHD, and hydrocortisone replacement therapy was initiated. Her present height at 13 yr of age is 148 cm (−1.3 SD). These cases reminded us that the possibility of 21-OHD should be considered when patients show premature pubarche or precocious puberty, even if they passed the NS test for 21-OHD.

We report a case of nail-patella syndrome (NPS) with unusual thinning of the glomerular basement membrane (GBM) associated with a novel heterozygous variant in the LMX1B gene. A 43-year-old female patient with a previous diagnosis of NPS, referred to our hospital for persistent proteinuria, underwent a renal biopsy, which revealed minor glomerular abnormalities. She underwent a second renal biopsy at the age of 56 owing to the presence of persistent proteinuria and decline in serum albumin, meeting the diagnostic criteria for nephrotic syndrome. Light microscopy demonstrated glomerulosclerosis and cystic dilatation of the renal tubules. Notably, electron microscopy revealed unusual thinning of the GBM, which is quite different from typical biopsy findings observed in patients with NPS, characterized by thick GBM with fibrillary material and electron-lucent structures. Comprehensive genetic screening for 168 known genes responsible for inherited kidney diseases using a next-generation sequencing panel identified a novel heterozygous in-frame deletion-insertion (c.723_729delinsCAAC: p.[Ser242_Lys243delinsAsn]) in exon 4 of the LMX1B gene, which may account for the disrupted GBM structure. Further studies are warranted to elucidate the complex genotype-phenotype relationship between LMX1B and proper GBM morphogenesis.

The Trifecta valve has been reported to have valve dysfunction months to years after operation. However there were no reports of intraoperative valve dysfunction. A 73-year-old man with aortic stenosis underwent aortic valve replacement using a 21-mm Trifecta valve with Glide Technology (Trifecta GT; Abbot). Hemodynamics were collapsed after aortic declamp because of severe regurgitation, and we replaced it with another biologic valve. We noticed that the removed valve's mount post was open outwardly more than usual. Despite the progress of the biologic valve, dysfunction like this case can occur during operation, and it is important to reoperate immediately even if there is no obvious tear or deformity.

Background Selective use of distal filter protection during percutaneous coronary intervention (PCI) for acute coronary syndromes (ACS) decreased the incidence of no-reflow phenomena and in-hospital serious adverse cardiac events compared with conventional PCI in patients with attenuated plaque ≥5 mm; however, its long-term clinical outcome remains unknown.Methods and Results:Patients who had ACS with attenuated plaque ≥5 mm were assigned to receive distal protection (DP) (n=98) or conventional treatment (CT) (n=96). The rate of major adverse cardiovascular events (MACE), a composite of death from any cause, non-fatal myocardial infarction, or target vessel revascularization (TVR) at 1 year, was the pre-specified secondary endpoint of the trial. MACE at 1 year occurred in 12 patients (12.2%) in the DP group and 3 patients (3.1%) in the CT group (P=0.029), which was driven by a higher risk of TVR (11 [11.2%] vs. 2 [2.1%], P=0.018). In patients treated with bare-metal stents (n=42), MACE occurred in 25.0% of the patients in the DP group and in none of the patients in the CT group (P=0.029), whereas in patients treated with drug-eluting stents (n=151), rates of MACE were similar in the groups (8.1% vs. 3.9%, P=0.32). Conclusions In ACS patients with attenuated plaque ≥5 mm, the 1-year rates of MACE were higher in the DP group than in the CT group. This effect might be mitigated by the use of drug-eluting stents.

Purpose People with dementia exhibit disturbed rest-activity rhythms and extended sleep duration issues throughout their disease. Little is known about the effects of these issues on clinical problems for those with moderate and severe dementia. This cross-sectional study aimed to examine the associations of disturbed rest-activity rhythms and extended sleep duration with activities of daily living (ADL). Methods Sleep parameters were measured using an actigraphy device. Cognitive function was assessed using the Mini-Mental State Examination and Cognitive Test for Severe Dementia, the Hyogo Activities of Daily Living Scale was used to assess ADL, and behavioral and psychological symptoms of dementia were assessed using the Neuropsychiatric Inventory-Nursing Home scale. Associations among rest-activity rhythms, sleep duration, and other clinical variables were analyzed with multiple linear regression. Clinical variables were compared between 2 groups categorized by onset timing of rest peak. Patients Sixty-four participants with moderate and severe dementia were assessed. Results In the correlation analysis, unstable daily rest-activity rhythm was associated with lower ADL. In the multiple linear regression analysis, low intradaily variability, and long daytime sleep duration were associated with low ADL. Aberrant rest peak timing showed lower ADL compared with nonaberrant timing. Conclusions Abnormal rest-activity rhythm and sleep duration in persons with moderate and severe dementia may affect ADL.

Introduction: Increased serum procalcitonin (PCT), a well-known biomarker for sepsis, has been reported in several cancer types. We aimed to investigate the prognostic impact of PCT in non-small cell lung cancer (NSCLC). Methods: Medical records of 51 consecutive patients with NSCLC (Aichi Medical University Hospital) admitted between July 2017 and July 2018 were retrospectively reviewed. The patients were divided into PCT-low (PCT < 0.1 ng/mL) and PCT-high (PCT ⩾ 0.1 ng/mL) groups, and their clinical characteristics and survival were compared. Results: In contrast to the PCT-low group (n = 24), the PCT-high group (n = 27) showed significantly worse Performance Status (PS) and overall survival (OS) (PS 0–2/3–4, 16/8 versus 12/15, p = 0.034; median OS, not reached versus 127 days, p < 0.001), irrespective of the presence of infection ( p = 0.785). Multivariate analysis showed that the disease stage (IV versus I–III) and high PCT level (⩾0.1 versus Conclusion: The results suggest that serum PCT in NSCLC was elevated regardless of the presence of infection. Higher PCT levels are associated with poor PS and shorter OS in NSCLC.

Objectives Osteogenesis imperfecta (OI) is a skeletal dysplasia characterized by recurrent fractures due to congenital bone fragility. The only bisphosphonate approved for OI in Japan is pamidronate (PAM). To investigate whether monthly intravenous alendronate (ALN) infusions can maintain bone strength in OI children following cyclical PAM treatment. Methods A prospective and non-inferiority study was conducted. Eight school-age OI patients aged 8.5±2.0 years who were treated with cyclical PAM for 6.0±2.3 years were enrolled and switched to monthly intravenous ALN (0.030 mg/kg/month). Changes in L1-4 bone mineral density (BMD) Z-scores, fracture rates, and bone turnover markers for 12 months were analyzed. Results Average BMD Z-scores were −3.0±1.9, −2.9±2.0, and −2.2±2.0 in 12 months before enrollment, at enrollment, and after 12 months of ALN treatment, respectively. BMD Z-scores increased significantly during treatment with both PAM and ALN (p=0.012), and the effect of ALN was not inferior to that of PAM (p=0.67). There was no change in fracture rates (p=0.86) and bone turnover markers during the 12 months before and after enrollment. Additionally, ALN showed no remarkable side effects. Conclusions Our results suggest that monthly intravenous ALN can maintain bone strength after primary usage of cyclical PAM. We concluded that monthly intravenous ALN as a maintenance treatment following cyclical PAM administration can be an option for OI children.

Background: Takotsubo syndrome occasionally occurs in patients with fever due to underlying diseases. However, the impact of body temperature on inhospital prognosis of patients with takotsubo syndrome remains unknown. Methods: Using the patient cohort in the Tokyo Cardiovascular Care Unit Network Registry from 2013 to 2015, we identified 421 eligible patients whose data on body temperature at admission were available and classified them into three groups: high body temperature group (≥37.5°C; n=27), normal body temperature group (36.0–37.4°C; n=319), and low body temperature group (≤35.9°C; n=75). We compared the patient characteristics and inhospital outcomes among the three groups. Results: On admission, the high body temperature group showed a higher proportion of men and preceding physical triggers, higher heart and respiratory rates, and higher C-reactive protein level than the other groups. Inhospital all-cause mortality was significantly higher in the high body temperature group than in the normal or low body temperature group (18.5% vs. 2.2% vs. 4.0%, respectively, P Conclusions: Our findings suggest that high body temperature at admission is a strong predictor of inhospital mortality in patients with takotsubo syndrome. Febrile takotsubo syndrome patients may need to be managed with recognition of life-threatening conditions from the time of diagnosis, no matter what the causes of fever are.

Delirium is an independent predictor of death in patients undergoing dialysis for end-stage renal disease (ESRD). However, it is unknown whether delirium during hospitalization at the start of hemodialysis (HD) in elderly populations is associated with early mortality. We conducted a retrospective cohort study to investigate the association between delirium and early mortality in the elderly after starting HD. The cohort consisted of patients ≥ 75 years who started dialysis for ESRD at the National Center for Global Health and Medicine from 2010 to 2017 and at Yokosuka Kyosai Hospital from 2007 to 2011. Delirium was defined as patients who showed new symptoms of transient confused thinking and reduced awareness of their environment and were prescribed antipsychotic medications. The primary outcome was death within 1 year. Data were analyzed using Cox proportional hazard models with adjustments for baseline characteristics. A multinomial logistic regression was used to identify the determinants of patients developing delirium. We enrolled 259 patients (males, 60%); 33 patients were diagnosed with delirium. The primary outcome was observed in 19 patients with delirium (58%) and 24 patients without delirium (11%) (p

Tumor location and immunity play important roles in the progression of colorectal cancer (CRC). This study aimed to investigate the differences in the immunosurveillance pattern between right‐ and left‐sided CRC and analyze their association with clinicopathologic features, including mismatch repair (MMR) status. We included surgically resected stage II/III CRC cases and evaluated the immunohistochemical findings of HLA class I, HLA class II, programmed cell death‐ligand 1 (PD‐L1), PD‐1, CTLA‐4, CD3, CD4, CD8, TIA‐1, T‐bet, GATA3, RORγT, Foxp3, and CD163. A total of 117 patients were included in the analyses; of these, 30 and 87 had right‐ and left‐sided cancer, respectively. Tumor immunity varied according to the tumor location in the overall cohort. Analysis of the tumors excluding those with DNA mismatch repair (MMR) deficiency also revealed that tumor immunity differed according to the tumor location. In right‐sided colon cancer (CC), high expression of Foxp3 (P = .0055) and TIA‐1 (P = .0396) were associated with significantly better disease‐free survival (DFS). High CD8 (P = .0808) and CD3 (P = .0863) expression tended to have better DFS. Furthermore, in left‐sided CRC, only high PD‐L1 expression in the stroma (P = .0426) was associated with better DFS. In multivariate analysis, high Foxp3 expression in right‐sided CC was an independent prognostic factor for DFS (hazard ratio, 7.6445; 95% confidence interval, 1.2091‐150.35; P = .0284). In conclusion, the immunosurveillance pattern differs between right‐ and left‐sided CRC, even after adjusting for MMR deficiency., Comparison of Kaplan‐Meier curves of disease‐free survival (DFS) in each tumor location excluding DNA mismatch repair deficiency. In right‐sided colon cancer, high Foxp3 (P = .0055) and TIA‐1 expression (P = .0396) were associated with significantly better DFS. High CD8 (P = .0808) and CD3 (0.0863) expression showed a tendency towards better DFS. In left‐sided colorectal cancer, only high sPD‐L1 expression (P = .0426) was associated with better DFS.

Objective: Inflammatory response is central to pathogenesis of abdominal aortic aneurysm (AAA). Recently, we reported that Syk, a signaling molecule in inflammatory cells, promotes AAA development in a mouse model. In this study, we aimed to investigate the role of Syk in human AAA pathogenesis. Materials and Methods: We obtained human AAA wall samples during open surgical aortic repair at Kurume University Hospital. Immunohistochemical analyses of AAA samples were performed for Syk activation and cell type markers. Ex vivo culture of human AAA tissue was utilized to evaluate the effect of P505-15, a Syk inhibitor, on secretions of interleukin-6 (IL-6) and matrix metalloproteinases (MMPs). Results: Immunohistochemical analysis showed infiltration of B cells, T cells, and macrophages in AAA samples. Syk activation was localized mainly in B cells and part of macrophages. AAA tissue in culture secreted IL-6, MMP-9, and MMP-2 without any stimulation. The unstimulated secretions of IL-6, MMP-9, and MMP-2 were insensitive to P505-15. Secretions of IL-6 and MMP-9 were enhanced by exogenous normal human immunoglobulin G (IgG), which was suppressed by P505-15, whereas secretion of MMP-2 was insensitive to IgG or P505-15. Conclusion: These results demonstrate an important role of Syk for IgG-dependent inflammatory response in human AAA.

The management of prosthetic dialysis arteriovenous graft infection comprises antibiotic treatment and total or partial excision of infected grafts for infectious source control. Partial excision with graft bypass is an important graft preservation strategy for localized infection but carries a higher reinfection risk. Here, we report a case of prosthetic graft infection that was successfully treated with partial excision, a graft bypass procedure, and a portable negative pressure wound therapy system, PICO, applied to the open wound postoperatively. The combined approach may be a useful strategy that decreases reinfection risk, shortens the length of hospital stay, and preserves graft patency.

The currently approved dose of pazopanib (800mg) is being re-examined owing to its adverse effects. The aim of this study was to evaluate the relationships among starting or maintenance doses of pazopanib, estimated pazopanib C-min, and other clinical factors, including albumin and a-1 acid glycoprotein levels, in soft-tissue sarcoma and renal cell carcinoma. We also determined whether therapeutic drug monitoring of pazopanib concentrations may be used to improve its therapeutic efficacy and prevent adverse effects. Forty patients who received pazopanib for renal cancer or soft-tissue sarcoma at the Hokkaido Cancer Center were evaluated prospectively. C-min, for pazopanib was calculated based on the measured values from the plasma samples. The efficacy and time to treatment failure were then assessed. The pazopanib maintenance doses were 200 (n=4), 400 (n=34), 600 (n=4), and 800mg (m = 1). Most patients (65%) who received a 400 mg dose had an effective pazopanib concentration (>= 20//g/mL), whereas 35% of patients who received the 400mg dose had ineffective concentrations (

Cholesterol crystal embolization (CCE) is a rare, mainly iatrogenic condition. The proportion of CCE after cardiovascular procedures has not been fully elucidated. The purpose of this study was to determine the proportion of CCE diagnosed after cardiovascular procedures and to identify risk factors for CCE occurrence. Data on patients aged older than 40 years who underwent cardiovascular procedures between July 2010 and March 2017 were extracted from the Japanese Diagnosis Procedure Combination database. Inpatients diagnosed with CCE within 1 year after procedures in the same hospital were identified. Logistic regression analysis was performed to identify factors associated with the occurrence of CCE. There were 962 patients with CCE in 2,190,300 patients who underwent cardiovascular procedures. The overall proportion of CCE after cardiovascular procedures was 4.4 per 10,000 patients (95% confidence interval 4.1-4.7). The overall in-hospital mortality among patients with CCE was 11% (107/962). Older age, male sex, smoking, heart failure, peripheral vascular disease, cerebrovascular disease, renal insufficiency, diabetes mellitus, hypertension, and aortic aneurism and dissection were significantly associated with the higher occurrence of CCE. Compared with cardioangiography, several procedures were significantly associated with higher occurrence of CCE, including intra-aortic balloon pumping, percutaneous transluminal angioplasty of the renal artery, and transcatheter aortic valve implantation or balloon aortic valvuloplasty. CCE is rare but remains a severe complication of cardiovascular procedures. Atherosclerotic risk factors and certain cardiovascular procedures were associated with CCE.

BACKGROUND Double-balloon endoscopic retrograde cholangiography (DB-ERC) is widely performed for biliary diseases after reconstruction in gastrointestinal surgery, but there are few reports on DB-ERC after hepatectomy or living donor liver transplantation (LDLT). AIM To examine the success rates and safety of DB-ERC after hepatectomy or LDLT. METHODS The study was performed retrospectively in 26 patients (45 procedures) who underwent hepatectomy or LDLT (liver operation: LO group) and 40 control patients (59 procedures) who underwent pancreatoduodenectomy (control group). The technical success (endoscope reaching the choledochojejunostomy site), diagnostic success (performance of cholangiography), therapeutic success (completed interventions) and overall success rates, insertion and procedure (completion of DB-ERC) time, and adverse events were compared between these groups. RESULTS There were no significant differences between LO and control groups in the technical [93.3% (42/45) vs 96.6% (57/59), P = 0.439], diagnostic [83.3% (35/42) vs 83.6% (46/55), P = 0.968], therapeutic [97.0% (32/33) vs 97.7% (43/44), P = 0.836], and overall [75.6% (34/45) vs 79.7% (47/59), P = 0.617] success rates. The median insertion time (22 vs 14 min, P < 0.001) and procedure time (43.5 vs 30 min, P = 0.033) were significantly longer in the LO group. The incidence of adverse events showed no significant difference [11.1% (5/45) vs 6.8% (4/59), P = 0.670]. CONCLUSION DB-ERC after liver operation is safe and useful but longer time is required, so should be performed with particular care.

Immersion pulmonary edema (IPE) is a rare condition observed in divers. We report a case of a 66-year-old man on maintenance dialysis who developed acute dyspnea and blood-tinged sputum after scuba diving. Vital signs on admission were significant for elevated blood pressure at 209/63 mmHg and hypoxia with an oxygen requirement of 6 L/min. Physical examination was remarkable for bilateral coarse crackles and systolic ejection murmur. Chest radiography revealed bilateral pulmonary edema. Echocardiography showed aortic stenosis and diffuse hypokinesis of left ventricular wall motion. We started bilevel positive airway pressure and administered nitroglycerin and nicardipine to maintain adequate oxygenation and reduce blood pressure. We started hemodialysis and extracorporeal ultrafiltration to remove excess fluid. His dyspnea subsided and oxygen was no longer required on Day 3. His long-standing hypertension, increased afterload due to vasoconstriction induced by cold water, increased capillary pressure due to impaired left ventricular motion and increased preload caused by exertion, and aortic stenosis probably contributed to pulmonary congestion. We propose maintenance dialysis as a novel risk factor for IPE due to its tendency to induce volume overload, increase pulmonary capillary pressure, and increase aortic stenosis risk. Patients on hemodialysis should refrain from diving to prevent this life-threatening condition.

Mycobacterium kansasii is one of the major non-tuberculous mycobacteria species that typically cause pulmonary diseases. M. kansasii is known to cause septic arthritis as an extrapulmonary disease in immunosuppressed patients or chronic skin disease. Herein, we present a case of M. kansasii arthritis involving the elbow of an immunocompetent patient, which was initially suspected to be a soft-tissue tumor. A 70-year-old man presented with a swollen left elbow that had progressed for 18 months with deteriorating arthralgia and limited range of motion. Magnetic resonance imaging revealed filling of the intra-articular space of the elbow and surrounding of the radial head with a soft tissue mass with mixed signal intensity. Initial incisional biopsy was performed via the lateral approach to the elbow joint, and pathological examination of the mass did not reveal any evidence of malignancy. One year after the first operation, arthroscopic surgery was performed to excise the mass following the recurrence of swelling and limited function of the elbow. Pathological examination of the resected synovium revealed epithelioid granulomas containing a multinucleated giant cell and inflammatory cell infiltration, characteristic of mycobacterial infection. M. kansasii was cultured after 2 weeks of incubation of the synovial sample. He experienced full resolution of the swelling and limited function following a combination of synovectomy and multidrug antimycobacterial treatment (rifampin 600 mg/day, clarithromycin 800 mg/day, and ethambutol 750 mg/day). This case highlights the need to consider this rare infection in the differential diagnosis of intra-articular soft tissue tumor-like lesions even in immunocompetent patients.

Serum anion gap (AG) has recently been proven to represent a biomarker for predicting prognosis in patients with end-stage renal disease (ESRD). However, whether change in AG (ΔAG) at the time of starting hemodialysis predicts mortality after starting hemodialysis in elderly patients with ESRD remains unknown. This retrospective cohort investigated the association between ΔAG and mortality after starting hemodialysis in the elderly. The cohort comprised patients ≥ 75 years old who started hemodialysis for ESRD at National Center for Global Health and Medicine between 2010 and 2017 and at Yokosuka Kyosai Hospital between 2007 and 2011. Patients were stratified into three groups (G1-3) based on ΔAG, calculated according to the equation: ΔAG = sodium − (chloride + bicarbonate) − 12. The primary outcome was death within 1 year of starting hemodialysis. Data were analyzed using Cox proportional hazard models with adjustments for baseline characteristics. We enrolled 254 patients (59% male). Median ΔAG was 2.6 (G1: > 3, n = 111; G2: 0–3, n = 103; G3

Background True thymic hyperplasia is a rare condition characterized by enlargement of the thymus while its normal structure is retained. True thymic hyperplasia is known to accompany Graves’ disease, but no association between true thymic hyperplasia and thyroid follicular tumor has been reported so far. We report a case of true thymic hyperplasia in a patient with a thyroid follicular tumor. Case presentation A 52-year-old Japanese man was referred to our hospital for evaluation of a thyroid mass and a mediastinal mass. His serum thyroglobulin level was high, and hemithyroidectomy was performed to remove the thyroid mass. The resected mass was diagnosed as a follicular tumor of uncertain malignant potential. After resection of the thyroid lesion, the patient’s serum thyroglobulin levels were markedly decreased. Seven months later, the patient underwent resection of the mediastinal mass. On pathological examination, the mass was found to consist of lobules, which formed a corticomedullary structure with Hassall’s bodies, indicating a normal thymic mass with hyperplastic thymic tissue, less organized cellular cords, and intermingled adipose tissue. Immunostaining for cytokeratin 19 and cytokeratin 7 indicated that the lesion was consistent with thymic tissue. The lesion was diagnosed as true thymic hyperplasia, and the histological findings suggested that secondary atrophy had occurred. No evidence of recurrence was observed at 24 months after surgery. Conclusions We present a case of a combination of true thymic hyperplasia and thyroidal follicular tumors that, to our knowledge, has not been reported previously. High serum thyroglobulin levels might play a role in hyperplasia of the thymus. Although true thymic hyperplasia is a rare disorder, it should be included in the differential diagnosis of a mediastinal mass in patients with thyroid disease.

BACKGROUND: Calcified nodules (CN) have been reported as being associated with stent failure including in-stent restenosis (ISR). However, there is no systematic study of this condition. AIMS: We aimed to clarify the prevalence, predictors, and midterm results of ISR lesions with CN. METHODS: We examined the clinical characteristics of 651 ISR lesions in patients who underwent percutaneous coronary intervention (PCI) with optical coherence tomography (OCT) between October 2008 and July 2016, and their 6- to 8-month follow-up angiography results. CN was defined as a high backscattering mass with small nodular calcium depositions which protruded into the vessel lumen. RESULTS: Thirty-two ISR lesions (4.9%) had CN. Multivariable analysis showed that calcified lesion (odds ratio [OR] 12.441, p

Coronary ostial aneurysm is a complication of aortic root replacement, particularly in Marfan syndrome. Reconstructing the coronary artery is an important problem during reoperation. Herein, we report a case of coronary artery bypass grafting using radial artery to repair bilateral coronary ostial aneurysms after aortic root replacement in a patient with Marfan syndrome.

Panitumumab, a fully human anti-epidermal growth factor receptor (EGFR) monoclonal antibody, has been shown to be useful in treating either advanced or recurrent KRAS/NRAS/BRAF wild-type colorectal cancer. We herein report the case of a 60-year-old man with short bowel syndrome who developed hematochezia due to panitumumab-induced colitis with vitamin K deficiency during third-line chemotherapy. The cause of vitamin K deficiency was the lack of intravenous vitamin K supplementation following a change from central venous nutrition to peripheral venous nutrition. We advise clinicians to carefully check for colitis and manage the infusions of chemotherapy patients with short bowel syndrome.

Background Although the automatic diagnosis of fractures using artificial intelligence (AI) has recently been reported to be more accurate than those by orthopedics specialists, big data with at least 1000 images or more are required for deep learning of the convolutional neural network (CNN) to improve diagnostic accuracy. The aim of this study was to develop an AI system capable of diagnosing distal radius fractures with high accuracy even when learning with relatively small data by learning to use bi-planar X-rays images. Methods VGG16, a learned image recognition model, was used as the CNN. It was modified into a network with two output layers to identify the fractures in plain X-ray images. We augmented 369 plain X-ray anteroposterior images and 360 lateral images of distal radius fractures, as well as 129 anteroposterior images and 125 lateral images of normal wrists to conduct training and diagnostic tests. Similarly, diagnostic tests for fractures of the styloid process of the ulna were conducted using 189 plain X-ray anteroposterior images of fractures and 302 images of the normal styloid process. The distal radius fracture is determined by entering an anteroposterior image of the wrist for testing into the trained AI. If it identifies a fracture, it is diagnosed as the same. However, if the anteroposterior image is determined as normal, the lateral image of the same patient is entered. If a fracture is identified, the final diagnosis is fracture; if the lateral image is identified as normal, the final diagnosis is normal. Results The diagnostic accuracy of distal radius fractures and fractures of the styloid process of the ulna were 98.0 ± 1.6% and 91.1 ± 2.5%, respectively. The areas under the receiver operating characteristic curve were 0.991 {n = 540; 95% confidence interval (CI), 0.984–0.999} and 0.956 (n = 450; 95% CI 0.938–0.973). Conclusions Our method resulted in a good diagnostic rate, even when using a relatively small amount of data.

Background Trans-jejunal nutrition via a post-pyloric enteral feeding tube has a low risk of aspiration or reflux; however, placement of the tube using the blind method can be difficult. Assistive devices, such as fluoroscopy or endoscopy, are useful but may not be suitable for patients with hemodynamic instability or severe respiratory failure. The aim of this study was to explore factors associated with first-pass success in the blind placement of post-pyloric enteral feeding tubes in critically ill patients. Methods Data were obtained retrospectively from the medical records of adult patients who had a post-pyloric enteral feeding tube placed in the intensive care unit between January 1, 2012, and December 31, 2018. Logistic regression analysis was performed to assess the association between first-pass success and the independent variables. For logistic regression analysis, the following 13 variables were defined as independent variables: age, sex, height, fluid balance from baseline, use of sedatives, body position during the procedure, use of cardiac assist devices, use of prokinetic agents, presence or absence of intestinal peristalsis, postoperative cardiovascular surgery, use of renal replacement therapy, serum albumin levels, and position of the greater curvature of the stomach in relation to spinal levels L1 − L2. Results Data obtained from 442 patients were analyzed. The first-pass success rate was 42.8% (n = 189). Logistic regression analysis demonstrated that the position of the greater curvature of the stomach cephalad to L1 − L2 was only associated with successful placement (odds ratio for first-pass success, 0.62; 95% confidence interval: 0.40 − 0.95). Conclusions In critically ill patients, the position of the greater curvature of the stomach caudal to L1 − L2 may be associated with a lower first-pass success rate of the blind method for post-pyloric enteral feeding tube placement. Further studies are needed to verify our results because the position of the stomach was estimated by radiographs after enteral feeding tube placement. Trial registration: University Hospital Medical Information Network Clinical Trials Registry (UMIN000036549; April 20, 2019).

Background A comprehensive understanding of the relevant factors involved in improving quality of life (QoL) is essential in patients with severe dementia; however, rating scales used in previous studies may not adequately reflect the factors that affect these patients. The purpose of this study was to identify factors that contribute to QoL using an evaluation scale suitable for patients with severe dementia. Methods The current cross-sectional study was conducted at a hospital for recuperation in Hyogo prefecture in Japan. The measurement scales included the QoL in Late-Stage Dementia Japanese version (QUALID-J), Cognitive Test for Severe Dementia, Neuropsychiatric Inventory-Nursing Home (NPI-NH), Physical Self-Maintenance Scale (PSMS), Pain Assessment in Advanced Dementia (PAINAD), and Special Care Unit Environment Quality Scale (SCUEQS). Multiple regression analyses were performed. Results We assessed a total of 105 patients with severe dementia (80 women; aged 87.3 ± 6.3 years). Multiple regression demonstrated that the QUALID-J total score was significantly affected by the NPI-NH and PAINAD scores. Factors 1 (expression of comfort) and 2 (expression of discomfort) of the QUALID-J were significantly affected by the PSMS and PAINAD, and the NPI-NH and PAINAD scores, respectively. Conclusion Our results indicate that behavioural and psychological symptoms of dementia and pain are important factors in influencing the QoL of patients with severe dementia.

Negative blood culture and pathological findings are helpful to diagnose non‐bacterial thrombotic endocarditis. The treatment strategy, including lifelong anticoagulation or surgery, should be individualized based on patients' underlying diseases.

ObjectivesTo report the impact of burosumab on patient-reported outcomes (PROs) and ambulatory function in adults with X-linked hypophosphataemia (XLH) through 96 weeks.MethodsAdults diagnosed with XLH were randomised 1:1 in a double-blinded trial to receive subcutaneous burosumab 1 mg/kg or placebo every 4 weeks for 24 weeks (NCT02526160). Thereafter, all subjects received burosumab every 4 weeks until week 96. PROs were measured using the Western Ontario and the McMaster Universities Osteoarthritis Index (WOMAC), Brief Pain Inventory-Short Form (BPI-SF) and Brief Fatigue Inventory (BFI), and ambulatory function was measured with the 6 min walk test (6MWT).ResultsSubjects (N=134) were randomised to burosumab (n=68) or placebo (n=66) for 24 weeks. At baseline, subjects experienced pain, stiffness, and impaired physical and ambulatory function. At week 24, subjects receiving burosumab achieved statistically significant improvement in some BPI-SF scores, BFI worst fatigue (average and greatest) and WOMAC stiffness. At week 48, all WOMAC and BPI-SF scores achieved statistically significant improvement, with some WOMAC and BFI scores achieving meaningful and significant change from baseline. At week 96, all WOMAC, BPI-SF and BFI achieved statistically significant improvement, with selected scores in all measures also achieving meaningful change. Improvement in 6MWT distance and percent predicted were statistically significant at all time points from 24 weeks.ConclusionsAdults with XLH have substantial burden of disease as assessed by PROs and 6MWT. Burosumab treatment improved phosphate homoeostasis and was associated with a steady and consistent improvement in PROs and ambulatory function.Trial registration numberNCT02526160.

Introduction: The aim of this study was to investigate the early changes in alpha-fetoprotein (AFP) and des-γ-carboxy prothrombin (DCP) levels in patients with advanced hepatocellular carcinoma (HCC) treated with atezolizumab plus bevacizumab and to evaluate the relationship between changes in these tumor markers and treatment efficacy. Methods: Of 58 consecutive patients who started atezolizumab plus bevacizumab at our institution, 50 patients with information on antitumor response obtained at 6 weeks after therapy were enrolled in this study and their treatment outcomes were retrospectively evaluated. Results: According to the Response Evaluation Criteria in Solid Tumors at 6 weeks, the objective response (OR) rate was 22.0% and the disease control (DC) rate was 78.0%. In patients who achieved OR at 6 weeks, median AFP and DCP ratios at weeks 1, 2, 3, and 6 were significantly lower than those in patients who did not achieve OR. AFP ratios in patients who did not achieve DC at 6 weeks (Non-6W-DC group) were significantly higher than in those who achieved DC at week 6 (6W-DC group). Median overall survival in the Non-6W-DC group was significantly shorter than in the 6W-DC group (156 days vs. not reached, p = 0.0008). An AFP ratio of 1.4 or higher at 3 weeks had a specificity of 88.0% and a sensitivity of 88.9% for predicting Non-6W-DC. Median progression-free survival was significantly shorter in patients with an AFP ratio of 1.4 or higher at 3 weeks than in those with an AFP ratio of p = 0.0003). Conclusion: Early changes in AFP might be useful for predicting the antitumor efficacy of atezolizumab plus bevacizumab in patients with advanced HCC. An AFP ratio of 1.4 or higher at 3 weeks might be an early predictor of refractoriness to atezolizumab plus bevacizumab therapy.

We report a rare case of recurrent solitary fibrous tumor (SFT) of the pleura with suspicious malignant transformation. A 78-year-old man had undergone prior surgical resection of the primary and recurrent SFT tumors at 11 and 2 years before the current presentation. Although his primary tumor had a round shape and did not show invasive growth, the current recurrent tumor extended through the neural foramen and had an osteoclastic progression into the thoracic spine. A computed tomography (CT) guided needle biopsy was performed and the pathological diagnosis of the tumor was confirmed as the recurrence of SFT. Immunohistochemically, the MIB-1 proliferation index (Ki-67) of the primary tumor and the current tumor was 1.74 and 30.00%, respectively. These clinical and immunohistochemical findings were strongly suspected the malignant transformation of SFT from benign. He was treated with radiotherapy, and a response was observed.

Background Rhabdomyomas comprise the majority of cardiac tumors in fetuses and are found in association with tuberous sclerosis complex. More than 90% of fetuses and neonates with multiple cardiac rhabdomyomas have signs of tuberous sclerosis complex. However, solitary cardiac rhabdomyoma cases are largely unrelated to tuberous sclerosis complex. Here, we report a case involving multiple cardiac rhabdomyomas not associated with tuberous sclerosis complex in a dizygotic twin. Case presentation A 36-year-old Japanese woman was diagnosed with a dizygotic twin pregnancy in the first trimester. Consistent with dizygosity, the fetal sex was discordant (male and female). At 27 weeks of gestation, hydrops and multiple echogenic cardiac masses were noted in the male baby, with the largest mass measuring 34 × 30 mm. The female fetus appeared normal. The cardiac masses enlarged gradually with the progression of the hydrops. At 32 weeks of gestation, intrauterine death of the male fetus was confirmed. The next day, autopsy of the male fetus was performed after cesarean section. Three well-demarcated white-tan-colored nodules were formed in the ventricular walls and interventricular septum, with the largest nodule (40 × 30 mm) in the left ventricular wall. Histologically, these lesions were diagnosed as rhabdomyomas. Conclusions We encountered a case involving multiple cardiac rhabdomyomas arising in one of dizygotic twin fetuses. Unlike most reported cases of multiple cardiac rhabdomyomas, this case was not accompanied by tuberous sclerosis complex. To the best of our knowledge, this is the first case report of multiple cardiac rhabdomyomas that developed in only one of dizygotic twins in the English literature.

Paclitaxel has the potential for inducing lumen enlargement by vessel enlargement, healing of dissection, and plaque regression. This study was carried out to determine the possibility of and the relevant factors of delayed stenosis regression after drug-coated balloon (DCB) angioplasty for femoropopliteal (FP) artery lesions. A total 105 de novo FP lesions were finalized with DCB angioplasty in our institute between May 2018 and June 2020. Among them, cases in which residual stenosis was detected by duplex ultrasonography (DUS) after the procedure were included in this study. Significant stenosis was defined as peak systolic velocity ratio ≥ 2.4 by DUS. Follow-up DUS was routinely performed 6 months after the procedure, and we defined cases without stenosis as cases of delayed stenosis regression according to the follow-up DUS. DUS showed that 26 (25.5%) of 102 lesions had residual stenosis after DCB angioplasty, and delayed stenosis regression was observed in 12 (57.1%) of 21 lesions 6 months after the procedure. The percentage of lesions containing calcified plaque as detected by intravascular ultrasound analysis was significantly higher in the non-regression group than in the regression group (18.2% vs. 77.8%, p = 0.02). Vessel remodeling and dissection patterns were not associated with delayed stenosis regression. The results of our analyses indicate that delayed stenosis regression may occur after DCB angioplasty for FP lesions in more than half of cases with residual stenosis. Delayed stenosis regression may be difficult in cases of calcified lesions.