Transcatheter aortic valve implantation (TAVI) is an effective way to treat patients with symptomatic severe aortic valve stenosis, who are deemed high risk or inoperable. TAVI was started in June 2015 at our hospital.
A total of 15 consecutive patients undergoing TAVI were included by January 2016. Of them, 10 patients (67%) were more than 85 years old and 12 patients (80%) had a high degree of frailty.
Peak aortic valve velocity significantly decreased to 2.2±0.5 meters per second from 4.9±0.6 meters per second with TAVI (p＜0.001), and aortic valve area increased to 1.34±0.42cm2 from 0.47±0.14cm2 (p＜0.001).
As a complication, there was acute aortic valve insufficiency after balloon aortic valvuloplasty and cardiogenic shock in one patient. However, the use of an extracorporeal circulation assisting device was avoided by rapid transcatheter heart valve implantation.
During the perioperative period, there were no serious complications, such as death or dissection of the aortic annulus, ventricular perforation or coronary occlusion. However, one patient required permanent pacemaker implantation after TAVI, and rehospitalization due to heart failure was necessary for three patients.
TAVI was useful for elderly and high-risk patients with severe aortic stenosis. We were able to safely perform TAVI by building up a good heart team.
We report a case of relapsing polychondritis who underwent F-18 fluorodeoxyglucose (FDG) positron-emission tomography/computed tomography (PET/CT), and the F-18 FDG uptake in the larynx, central airway and rim cartilages was observed. After corticosteroid and immunosuppressant drug therapy, clinical findings were improved and F-18 FDG uptake almost disappeared. F-18 FDG PET/CT is a useful tool in the diagnosis of relapsing polychondritis and the follow-up after therapy.
The main clinical management for Fabry disease is enzyme replacement therapy (ERT) with genetically recombinant agalsidase-β. ERT for Japanese Fabry disease was started in 2004. This is a report of a classical Fabry patient treated with ERT for 12 years.
The patient is a 49-year-old Japanese male having a nonsense mutation (p. A227X) in the α-GAL gene. He had sweating abnormality (anhydrosis), mild vascular cutaneous lesions (angiokeratomas) and proteinuria in childhood, but no periodic crises of pain in the extremities. ERT was initiated at the age of 37 in 2004 with 1mg/kg algalsidase-beta for 2 weeks.
There have been no severe clinical cardiac, cerebrovascular or renal events throughout. Echocardiography shows mild hypertrophic change of interventricular septum (10.5mm at baseline, 13.0mm at the end of follow-up), but normal left-ventricular thickness and volume. Brain MRI and MR angiography show normal white matter and cerebral arteries. However, gradual deterioration of renal function has occurred during these 12 years. He had stage 1 CKD at the baseline. The urine protein-to-creatinine ratio (UP/Cr), and the estimated glomerular filtration rate calculated from creatinine (eGFRcre) were 0.41 and 121.3ml/min per 1.73m2 before ERT, and 0.74 and 52.0ml/min per 1.73m2 recently. The slope for eGFRcre is -3.65ml/min per 1.73m2 per year, which is a faster rate than the rate of decline in a normal individual (approximately -1.00ml/min per 1.73m2 per year). In the most recent analysis the slope for eGFR calculated from cystacin C (eGFRcys) is -6.16ml/min per 1.73m2 per year, which seems to show more rapidly progressive kidney disease. With regard to other clinical features, right-ear hearing loss appeared at the age of 42 and angiokeratomas increased with age, but anhydrosis disappeared in the early stage of ERT.
This report indicates that, compared to the natural history of Fabry disease, ERT has the effect of delaying the onset of severe events such as cardiac and cerebrovascular disease and end-stage renal disease. However, the therapy has not been as highly effective as expected.
We report a case of neuroendocrine carcinoma of the ascending colon with an intramural metastasis that was treated by adjuvant chemotherapy using FOLFOX after surgery. A 62-year-old man complained of abdominal pain. An egg-sized tumor was detected in the right lower abdomen. CT showed ileus due to a tumor of the ascending colon, and an emergency operation was performed. The tumor was located in the ascending colon, and right hemicolectomy with regional node dissection was performed. Macroscopically, two tumors were observed in the ascending colon : a type 5 tumor (50×30mm ; Lesion A) and a 0-Ip tumor (30×30mm ; Lesion B). Histopathologically, Lesion A was diagnosed as neuroendocrine carcinoma (pT3 pN0 cM0 : pStage II), and Lesion B was diagnosed as an intramural metastasis from Lesion A. These lesions macroscopically and histologically were separate from each other. FOLFOX as an adjuvant chemotherapy was performed after surgery, and the patient is alive without recurrence or metastasis 5 years after surgery.
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