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We present an extremely rare case of mitral Ebstein's anomaly that resulted in severe mitral regurgitation (MR). A 41-year-old woman with a history of tuberous sclerosis underwent surgery. Preoperatively, it was assumed that MR had occurred due to leaflet tethering related to left ventricular posterior wall motion asynergy due to a scarred rhabdomyoma. However, surgical inspection revealed a dysplastic posterior leaflet adhering to the ventricular wall, which was completely covered by the endocardium. Both congenital mitral Ebstein's anomaly and acquired wall motion abnormality due to a scarred rhabdomyoma may have contributed to the development of severe MR in this case.We herein report a 65-year-old man with progressive multifocal leukoencephalopathy (PML) after 2-year remission from acute myeloid leukemia who developed recurrent episodes of left hemiparesis with gadolinium enhancement on magnetic resonance imaging. Steroid pulse therapy for each exacerbation induced clinical and radiological improvement, suggesting that exacerbations are an excessive immune response to the JC virus and distinct from immune reconstitution inflammatory syndrome (IRIS). Although glucocorticoids are recommended only for IRIS, steroid pulse treatment should be considered as a therapeutic option in cases of exacerbation of hematologic malignancy-associated PML. Importantly, neuroimaging is not sufficient to differentiate excessive inflammation from a controlled inflammatory response, for which steroids are not recommended.We herein report a rare case of intraductal papillary mucinous neoplasm with a pancreatogastric fistula in an elderly Japanese man admitted to our hospital. The pancreatogastric fistula was confirmed using endoscopic retrograde pancreatography via a cannulated guidewire placed in the stomach. Six months after admission, the patient was diagnosed with intraductal papillary mucinous carcinoma. A pancreatogastric fistula is generally a rare complication of intraductal papillary mucinous neoplasm. It was caused by mechanical penetration in this case. Interestingly, we also observed endoscopic and histochemical mucosal changes in the fistula.Procalcitonin (PCT), a marker of the inflammatory response during infections, can be elevated by diabetic ketoacidosis (DKA). A male patient in his 50s with diabetic nephropathy on hemodialysis presented with vomiting and a reduced level of consciousness and was diagnosed with DKA. TL12-186 His PCT level was markedly elevated, but bacterial cultures (blood, urine, and stool) were negative. The PCT level decreased after DKA improvement. In this patient, DKA probably enhanced the PCT levels. As DKA can increase the PCT levels, an elevation of the PCT levels in DKA patients may not be indicative of infectious diseases, and non-infectious causes of DKA should therefore be considered.Objective Sarcopenia and osteoporosis often coexist in older adults. Sarcopenia is diagnosed using the skeletal muscle mass index (SMI), which is calculated as the appendicular skeletal muscle mass (ASM)/(present height)2, although patients with osteoporosis frequently have a loss of body height. We therefore investigated whether the present height or maximum height is more useful for calculating the SMI in the evaluation of physical abilities. Methods We conducted a cross-sectional study to investigate the association of the SMI with physical abilities, such as the grip strength and gait speed, in 587 postmenopausal women. The SMI was evaluated using whole-body dual-energy X-ray absorptiometry (DXA). The SMI [(ASM)/(present height)2], modified SMI (mSMI) [(ASM)/(maximum height)2], and SMI difference (ΔSMI) (mSMI - SMI) were calculated. Results Age and body mass index (BMI)-adjusted regression analyses showed that the SMI (β=0.30, p less then 0.001 and β=0.14, p=0.034) and mSMI (β=0.40, p less then 0.001 and β=0.29, p less then 0.001) were positively associated while the ΔSMI was negatively associated with the grip strength and gait speed (β=-0.15, p less then 0.001 and β=-0.24, p less then 0.001, respectively). Furthermore, the age, BMI, and presence of osteoporotic fractures-adjusted logistic regression analyses showed that a low mSMI ( less then 5.4 kg/m2) was significantly associated with a low grip strength ( less then 18 kg) and slow gait speed (1.0 m/s) (odds ratio [OR]=2.45, 95% confidence interval [CI]=1.52-3.95 per SD increase, p less then 0.001; and OR=1.73, 95% CI=1.01-2.96, p=0.042, respectively), although a low SMI showed no such relationship (p=0.052 and p=0.813, respectively). Conclusion The mSMI using the maximum height is more useful for evaluating physical abilities than conventional SMI estimation in postmenopausal women.High-altitude cerebral edema (HACE) is a rare condition of acute mountain sickness that manifests as consciousness disturbance and truncal ataxia. Neuroimaging shows vasogenic edema with microbleeds in the white matter and the corpus callosum. We herein report a case of HACE in which the patient showed widespread hyperintense signals with extensive microbleeds in the white matter and corpus callosum on MRI, as well as cognitive dysfunction. Rehabilitation to improve the higher brain function facilitated the recovery of the patient's cognitive impairment and was accompanied by improved MRI findings.A 65-year-old man presented with a slight headache and transient visual disturbance. Magnetic resonance imaging (MRI) revealed occlusion of the left internal carotid artery (ICA) and acute brain infarctions in both hemispheres, and a blood examination indicated inflammation. Gadolinium enhancement was observed in the walls of the temporal arteries and ICAs. After we diagnosed giant cell arteritis (GCA) by a temporal artery biopsy, aspirin and corticosteroids were administered. The typical symptoms of GCA, such as jaw claudication and temporal artery tenderness, were absent during the entire clinical course, and the findings of contrast-enhanced MRI contributed to the diagnosis.Hermansky-Pudlak syndrome (HPS) is an autosomal recessive hereditary disease that may be complicated by progressive and potentially fatal interstitial pneumonia. We herein report a 64-year-old woman with interstitial pneumonia associated with HPS type 4 whom we treated with nintedanib after pirfenidone proved ineffective. To our knowledge, there have been no previous reports of nintedanib being used to treat a patient with HPS type 4. There is a need for clinical trials of antifibrotic agents, including nintedanib, pirfenidone, and new therapeutic agents with different mechanisms of action in these patients.Objective Patients with hematological malignancies, particularly those with multiple myeloma, often suffer from pathological vertebral compression fractures (VCFs). Consequent and significant spinal pain and paralysis impair the activities of daily living and quality of life and delay subsequent chemotherapy. Balloon kyphoplasty (BKP), which is less invasive than conventional therapies, is a type of percutaneous vertebroplasty in which cement is injected into the broken vertebrae to stabilize the spinal column. The present study assessed the effect of BKP on hematological tumors. Methods We retrospectively analyzed five myeloma patients and one lymphoma patient who underwent BKP for pathological VCFs in our institution. Results The median age was 74 years old. The spinal operation level ranged from T2 to L4. BKP was performed at the diagnosis in two cases, after first-line chemotherapy in one case, and after subsequent chemotherapy in three cases. After approximately 1 month, the patients' average Eastern Cooperative Oncology Group performance status score rapidly improved from 3.2 to 1.3. The numeric rating scale score decreased from 8.8 to 2.0, and the Karnofsky Performance Status score increased from 35 to 75. No severe complications were observed. All patients became able to walk unassisted and underwent early subsequent chemotherapy. Conclusion BKP can be a safe and effective treatment option for pathological VCFs in patients with hematological malignancies and allows for rapid induction with subsequent chemotherapy.

Emerging evidence advocates the use of restrictive transfusion strategies at hemoglobin (Hb) levels of approximately 7-8 g/dL in cardiac surgeries using cardiopulmonary bypass. Yet, it is unclear whether the same thresholds can be applied to off-pump coronary bypass (OPCAB) that accompanies cardiac displacement and warm regional ischemia-reperfusion injury without the aid of a bypass machine. The aim of this study is to investigate the relationship between perioperative nadir Hb level and outcome following OPCAB.Methods and ResultsMedical records of 1,360 patients were reviewed. Hb levels were serially assessed during and after surgery. The incidence of composite endpoints was 35%, which included myocardial infarction, stroke, acute kidney injury, sternal infection, reoperation, prolonged mechanical ventilation, and in-hospital mortality. The nadir Hb level was significantly lower in the morbidity group than in the non-morbidity group (8.1 [7.4-9.1] vs. 8.8 [7.9-9.8] g/dL, P<0.001). Multivariable logistic regression analysis revealed nadir Hb as an independent risk factor of adverse outcome (odds ratio 0.878, 95% confidence intervals 0.776-0.994, P=0.04), whereas preoperative anemia and perioperative transfusion were not. The critical value of Hb for predicting detrimental outcome was 8.05 g/dL.

A significant association is found between perioperative nadir Hb and adverse outcome after OPCAB. Although preoperative anemia was not associated with poor prognosisper se, it was the only modifiable risk factor that was closely linked to nadir Hb.

A significant association is found between perioperative nadir Hb and adverse outcome after OPCAB. Although preoperative anemia was not associated with poor prognosisper se, it was the only modifiable risk factor that was closely linked to nadir Hb.Congenital insensitivity to pain with anhidrosis (CIPA) is a rare disease caused by a mutation in the nerve growth factor (NGF) receptor, which results in an absence of Aδ and C fibers. It can be considered that this defect may also lead to deterioration of oral sensations. The aim of the present study was to clarify the ability of CIPA patients to perceive pungent, gustatory, and olfactory stimuli, which is essential for eating function, and the impact of the defect on dietary habits. Sensitivities to capsaicin and the five basic tastes were evaluated by measuring their threshold values, and dietary habits were examined using a questionnaire. Additionally, odor identification ability was evaluated using the odor stick method. The detection threshold for capsaicin and the recognition threshold for sour taste were significantly higher in the patients than in healthy volunteers. The questionnaire responses showed that the patients consumed spicy food more often. All patients were able to identify the tested odors, except those to which they had not been well accustomed. Since the abilities of CIPA patients to perceive taste and smell were not basically impaired, despite their lower sensitivity to capsaicin, it was suggested that their dietary habits were only minimally affected, except for intake of pungent foods.