Objective: Both acyclovir (ACV) and valacyclovir (VACV) can cause drug-induced encephalopathy, which occurs primarily in patients with renal impairment because of delayed drug metabolism. Here, we report a case of ACV-associated encephalopathy precipitated by the addition of nonsteroidal anti-inflammatory drugs (NSAIDs).Patient: A 97-year-old woman with a 1-d history of altered consciousness was admitted to our hospital. After treatment with ACV and VACV for herpes zoster, NSAIDs were introduced for pain 5 d prior to admission. VACV and NSAIDs were subsequently administered by her primary care physician 3 d before admission.Results: The patient presented with altered consciousness and acute kidney injury, leading to a suspicion of ACV-associated encephalopathy. Her consciousness improved rapidly with hemodialysis. We diagnosed ACV-associated encephalopathy based on a significantly elevated ACV blood level of 32.7 µg/dL.Conclusion: The addition of NSAIDs during ACV or VACV administration may precipitate ACV-associated encephalopathy. When combining ACV or VACV with NSAIDs for the treatment of herpes zoster, careful monitoring of consciousness level and renal function is recommended.

This study reports the case of a 0-day-old girl who was transferred to our hospital for suspected total anomalous pulmonary venous return due to her postnatal cyanosis. After she was presented at our hospital, pulmonary vein stenosis was diagnosed and emergency surgery was planned. Preoperative computed tomography showed abnormal perfusion of the pulmonary veins into the left and right superior vena cava separately on the left and right sides. Given that the persistent left superior vena cava was refluxing into the coronary sinus, the coronary sinus was enlarged greatly, and the left atrial volume was small. In the neonatal period, the right pulmonary vein was anastomosed to the right atrium, and the left pulmonary vein was anastomosed to the unroofed coronary sinus. Thereafter, at 4 months of age, the patient underwent two-stage intracardiac repair with re-routing of the right pulmonary vein and extracardiac ligation of the left vena cava. The postoperative course was good. Intracardiac repair via a two-stage surgery was effective for total anomalous pulmonary venous return (Ib+Ib) with a persistent left superior vena cava and an enlarged coronary sinus.

The patient was an 8-years- and 4-months old girl. At the age of one, she visited a previous doctor with hepatomegaly and liver dysfunction. As a result of the examination, she was diagnosed with geleophysic dysplasia. Other than the heart, she was followed in genetics, ophthalmology, orthopedics, endocrinology, and otolaryngology. At 3 years and 5 months, she was first examined by the cardiology department and was found to have mild mitral regurgitation and aortic valve stenosis, and was followed up once a year. The patient was referred to our hospital at 7 years and 11 months, and the cardiac catheterization performed at 8 years and 2 months showed mild mitral valve regurgitation, but the mean pressure gradient was 16 mmHg and the mitral valve area was 0.60 cm2 (MVAi 0.97 cm2/m2), and mitral valve stenosis was observed. The left atrial pressure was as high as 25 mmHg and the average pulmonary artery pressure was as high as 36 mmHg, and pulmonary hypertension was also observed. Intraoperative findings demonstrated that the mitral valve had a marked thickening of the leaflet, the papillary muscles and chordae were also thickened, and the effective valve opening area was narrowed. The leaflet and subvalvular tissue were resected as much as possible and mechanical valve replacement was performed. Postoperatively, the patient recovered satisfactorily and was discharged on the 16th postoperative day. Pathological findings showed no major disturbance in the layered structure of the papillary muscle or the leaflet itself, but it was highly thickened due to mucous degeneration of the leaflet. We report our experience with the rare case described above.

Background/Aims: Esophageal motility disorders (EMDs) contribute to the pathophysiology of gastroesophageal reflux disease. However, the causes of EMDs and their impact on gastroesophageal reflux disease-associated symptoms remain unknown. This study aims to elucidate clinical features associated with various types of EMDs in patients with heartburn symptoms. Methods: Of the 511 patients who underwent high-resolution manometry, 394 who were evaluated for heartburn symptoms were examined. Patients subjected to high-resolution manometry were classified into 4 groups: outflow obstruction group, hypermotility group, hypomotility group, and normal motility group. Symptoms were evaluated using 3 questionnaires. Patient characteristics and symptoms for each EMD type were compared with those of the normal motility group. Results: Of the 394 patients, 193 (48.9%) were diagnosed with EMDs, including 71 with outflow obstruction, 15 with hypermotility, and 107 with hypomotility. The mean dysphagia score was significantly higher in each of the 3 EMD groups compared with those with normal motility. The mean acid reflux and dyspepsia scores were significantly lower in the outflow obstruction group (P < 0.05). The mean body mass index and median Brinkman index were significantly higher in the hypermotility group (P = 0.001 and P = 0.018, respectively), whereas the mean diarrhea and constipation scores were significantly lower in the hypomotility group (P < 0.05). Conclusions The results of our study indicate that different EMDs have distinct characteristics. Cigarette smoking and high body mass index were associated with esophageal hypermotility. Assessment of the dysphagia symptom scores may help identify patients with EMDs.

Background/Aims: Esophageal motility disorders (EMDs) contribute to the pathophysiology of gastroesophageal reflux disease. However, the causes of EMDs and their impact on gastroesophageal reflux disease-associated symptoms remain unknown. This study aims to elucidate clinical features associated with various types of EMDs in patients with heartburn symptoms. Methods: Of the 511 patients who underwent high-resolution manometry, 394 who were evaluated for heartburn symptoms were examined. Patients subjected to high-resolution manometry were classified into 4 groups: outflow obstruction group, hypermotility group, hypomotility group, and normal motility group. Symptoms were evaluated using 3 questionnaires. Patient characteristics and symptoms for each EMD type were compared with those of the normal motility group. Results: Of the 394 patients, 193 (48.9%) were diagnosed with EMDs, including 71 with outflow obstruction, 15 with hypermotility, and 107 with hypomotility. The mean dysphagia score was significantly higher in each of the 3 EMD groups compared with those with normal motility. The mean acid reflux and dyspepsia scores were significantly lower in the outflow obstruction group (P < 0.05). The mean body mass index and median Brinkman index were significantly higher in the hypermotility group (P = 0.001 and P = 0.018, respectively), whereas the mean diarrhea and constipation scores were significantly lower in the hypomotility group (P < 0.05). Conclusions The results of our study indicate that different EMDs have distinct characteristics. Cigarette smoking and high body mass index were associated with esophageal hypermotility. Assessment of the dysphagia symptom scores may help identify patients with EMDs.

The patient was a 4-year-old boy, with no indication of heart disease at birth. When he was 4 years and 3 months old, he underwent surgery for cryptorchidism, and persistent changes after crying, consisting of ST-segment depression, were noted on the ECG. Therefore, it was decided that a detailed examination would be carried out. Aortography revealed that a small left coronary cusp had formed a pouch and that there was a delayed washout of the contrast material in the left coronary artery compared with the right coronary artery. Right coronary angiography showed retrograde flow to the left coronary artery. There was no significant aortic regurgitation, and no stenosis in the left coronary artery. Intraoperative findings demonstrated that the hypoplastic left coronary cusp had adhered to the aortic wall and covered the left sinus of Valsalva. Enlargement of the Valsalva was performed by placing a patch from the aortic transection site to an area directly above the coronary artery ostium. Postoperatively, the patient recovered satisfactorily and was discharged on the eleventh postoperative day. Cardiac catheterization performed 6 months after surgery showed no aortic regurgitation, no significant stenosis in the left coronary artery, and no retrograde flow from the right coronary artery. We report our experience with the rare case described above.

The patient was a 50-year-old man who had undergone ascending aortic replacement and coronary artery bypass grafting at another hospital for acute type A aortic dissection 4 years before. He was diagnosed with hemolytic anemia 1 year after surgery for his progressive anemia and high serum lactate dehydrogenase level. He was referred to our hospital because frequent transfusion was required. A computed tomography showed severe kinking of the graft (110°), which we considered to be the cause of hemolysis. Peak pressure gradient was 60 mmHg. To remove the cause of hemolysis and to precipitate thrombosis of the residual false lumen, we performed re-ascending aortic replacement and total arch replacement with a frozen elephant trunk. The postoperative course was uneventful and hemolysis resolved soon after the operation. Surgeons should be aware that severe kinking of a Dacron graft can be a cause of hemolysis.

Background: Consideration of cultural aspects is important in medical care. We explored regional differences in cancer and palliative care among Okinawa, Tohoku, and Tokyo metropolitan area. Methods: We conducted a questionnaire survey of physicians involved in cancer medicine from September to November 2020. A total of 11 items related to physician experiences were rated using a 5-point Likert-type scale. Results: Responses were received from 553 physicians (187 in Okinawa, 219 in Tohoku, 147 in the Tokyo metropolitan area). In Okinawa, “When patients die, it is important that all family members are present at the last moment,” “Patients/family members primarily consult the elders of the family about the medical treatments,” “Family members hope the patients die at home, because the soul will not return when they die at the hospital,” “Patients/family members get advice from religious advisors about the medical treatments,” and “Family members wish to take the patient home when he/she is about to die and to confirm death at home” were significantly more frequently observed. In Tohoku, “Patients wish to be hospitalized at a specific season” was significantly more frequently reported. In Tohoku and Okinawa, “Patients hide cancer from neighbors and relatives” and “Elderly patients do not want treatment, because they cover the living expenses and education expenses for their children and grandchildren.” were significantly more frequently experienced. Conclusion: There are regional differences in cancer and palliative care in Japan. Being sensitive to the culture of the region is needed.

Objective: The antitumor effects of anti-PD-1 antibody against mismatch repair deficiency (MMR-D)-associated cancers have been reported. MMR-D is found in approximately 20%–30% of endometrial carcinomas (ECs) and frequently occurs due to MLH1 promoter hypermethylation (MLH1-PHM). ECs with MLH1-PHM are classified according to the molecular screening of Lynch syndrome (LS), but few detailed reports are available. The purpose of this study was to clarify the clinical features of EC with MLH1-PHM. Methods: Immunohistochemistry of MMR proteins (MLH1, MSH2, MSH6, and PMS2) was performed on specimens from 527 ECs treated at our university hospital from 2003 to 2018. MLH1 methylation analysis was added to cases with MLH1/PMS2 loss. ECs were classified as follows: cases that retained MMR proteins as “MMR-proficient;” cases with MLH1/PMS2 loss and MLH1-PHM as “met-EC;” and cases with other MMR protein loss and MLH1/PMS2 loss without MLH1-PHM as “suspected-LS.” The clinical features, including long-term prognosis, of each group, were analyzed. Results: Accordingly, 419 (79.5%), 65 (12.3%), and 43 (8.2%) cases were categorized as “MMR-proficient,” “suspected-LS,” and “met-EC,” respectively. Significantly, “met-EC” had a lower proportion of grade 1 tumors (37.5%) and a higher proportion of stage III/IV tumors (37.2%) than the other groups. The overall and progression-free survival of “met-EC” were significantly worse than those of “suspected-LS” in all cases. Conclusion In ECs with MMR-D, “met-ECs” were a subgroup with a poorer prognosis than “suspected-LS.” “Met-ECs” would be the main target for anti-PD-1 antibody treatment, and its clinical susceptibility should be verified individually.

Objective: To examine the efficacy and safety of an innovative, device-driven abdominal trunk muscle strengthening program, with the ability to measure muscle strength, to treat chronic low back pain (LBP) in elderly participants. Methods: Seven women with non-specific chronic LBP, lasting at least 3 months, were enrolled and treated with the prescribed exercise regimen. Patients participated in a 12-week device-driven exercise program which included abdominal trunk muscle strengthening and 4 types of stretches for the trunk and lower extremities. Primary outcomes were adverse events associated with the exercise program, improvement in abdominal trunk muscle strength, as measured by the device, and improvement in the numerical rating scale (NRS) scores of LBP with the exercise. Secondary outcomes were improvement in the Roland-Morris Disability Questionnaire (RDQ) score and the results of the locomotive syndrome risk test, including the stand-up and two-step tests. Results: There were no reports of increased back pain or new-onset abdominal pain or discomfort during or after the device-driven exercise program. The mean abdominal trunk muscle strength, NRS, RDQ scores, and the stand-up and two-step test scores were significantly improved at the end of the trial compared to baseline. Conclusion No participants experienced adverse events during the 12-week strengthening program, which involved the use of our device and stretching, indicating the program was safe. Further, the program significantly improved various measures of LBP and physical function in elderly participants.