We report a successful open heart reoperation of a 14-year-old girl with Alagille syndrome. The patient underwent a living related donor liver transplantation at the age of 9 years in another hospital because of liver failure due to a paucity of interlobular bile ducts. Two years later, because of progression of her aortic valve stenosis, Ross operation and CABG were performed in the same hospital. Afterwards, her neoaortic valve regurgitation developed due to aortic root dilatation and myocardial ischemia developed by anastomosis site stenosis. She started to experience frequent angina attacks. She underwent AVR and redo CABG in our institution in April 2002. Her pre- and postoperative liver function was normal and no special procedure for the liver was needed, and she was discharged on the 18th postoperative day with no complications. In this country, few open heart surgeries for liver transplant recipient have been performed, and no case of reoperation has yet been reported. If pre- and postoperative liver function are normal, pre- and postoperative management of open heart surgery for a transplant may be perfomed conventionally.

A 19-year-old girl was admitted to our hospital with nausea, vomiting, hiccups, constipation and syncope. After hiccups or vomiting sinus arrest developed and lasted more than 5-8 seconds. She lost consciousness every one hour. Based on an electrocardiographic diagnosis of sick sinus syndrome (SSS), a temporary pacemaker was implanted. The next day, although her syncope and bradycardia disappeared, she had orthostatic tachycardia of over 120 beats/minute and swelling of the legs, which led to a diagnosis of postural orthostatic tachycardia syndrome (POTS). Neurologically, she showed the right-sided tongue deviation and parasympathetic system disorders revealed by coefficient of variation of R-R interval (CV_R-R), the Achner eye-ball pressure test, the valsalva ratio, and the head-up-tilt test. Brain MRI disclosed a small hyperintense lesion on a T2-weighted image with gadolinium enhancement in the right dorsal medulla including the hypoglottis nucleus and the posterior nucleus of vagus. After steroid pulse therapy (methyl prednisolone 1 g/day×3 days, 5 times) was administered, this lesion became smaller and finally disappeared. Before the lesion disappeared, she was able to begin rehabilitation by wearing elastic stockings and treatment with midodrine hydrochloride. The following year, she developed other MRI-proven brain lesions, suggestive of demyelination. Such a spinal and temporal distribution of lesions led to a diagnosis of multiple sclerosis (MS). A case of POTS caused by MS has not been reported previously, however, MS often affects the medullary paraventricular regions associated with autonomic failures. Autonomic failures often prevent patients from experiencing early rehabilitations. We should promptly give symptomatic treatment against autonomic failures, which leads to good patient recovery not only in patient vitality but also functionality.

Cardiac tumors are rare, and multiple myxomas are even rarer. The latter phenomenon is mostly associated with the Carney complex, a dominantly inherited disease characterized by multiple primary cardiac myxomas, endocrinopathy, and spotty pigmentation of the skin. We report the rare case of a patient who did not have the Carney complex but had multiple primary cardiac tumors. A 78-year-old woman with a past history of breast cancer was referred to our hospital for further examination of multiple cardiac tumors. Echocardiography showed 4 tumors in the left atrium and left ventricle. We could not diagnose them preoperatively and decided to resect them surgically because they were mobile and could have caused embolism and obstruction. The postoperative pathological findings of all 4 tumors were myxomas, although the patient did not meet the diagnostic criteria of the Carney complex. Therefore, a rare case of multiple primary cardiac myxomas was diagnosed.
Aged ; Breast Neoplasms ; Carney Complex* ; Echocardiography ; Embolism ; Female ; Heart Atria ; Heart Neoplasms ; Heart Ventricles ; Humans ; Myxoma* ; Pigmentation ; Skin

OBJECTIVE: Galvanic vestibular stimulation (GVS) activates the vestibular afferents, and these changes in vestibular input exert a strong influence on the subject's posture or standing balance. In patients with Parkinson's disease (PD), vestibular dysfunction might contribute to postural instability and gait disorders. METHODS: Current intensity was increased to 0.7 mA, and the current was applied to the patients for 20 minutes. To perform a sham stimulation, the current intensity was increased as described and then decreased to 0 mA over the course of 10 seconds. The patient's status was recorded continuously for 20 minutes with the patient in the supine position. RESULTS: Three out of 5 patients diagnosed with PD with postural instability and/or abnormal axial posture showed a reduction in postural instability after GVS. The score for item 12 of the revised Unified Parkinson's Disease Rating Scale part 3 was decreased in these patients. CONCLUSIONS: The mechanism of postural instability is complex and not completely understood. In 2 out of the 5 patients, postural instability was not changed in response to GVS. Nonetheless, the GVS-induced change in postural instability for 3 patients in our study suggests that GVS might be a therapeutic option for postural instability.
Gait ; Humans ; Parkinson Disease* ; Posture ; Supine Position

OBJECTIVE: The provisional diagnosis of progressive supranuclear palsy (PSP) depends on a combination of typical clinical features and specific MRI findings, such as atrophy of the tegmentum in the midbrain. Atrophy of the superior cerebellar peduncle (SCP) distinguishes PSP from other types of parkinsonism. Histological factors affect the conventional fluid-attenuated inversion recovery (FLAIR) signals, such as the extent of neuronal loss and gliosis. METHODS: We investigated patients with PSP to verify the percentage of patients with various PSP phenotypes presenting a high signal intensity in the SCP. Three interviewers, who were not informed about the clinical data, visually inspected the presence or absence of a high signal intensity in the SCP on the FLAIR images. We measured the pixel value in the SCP of each patient. Clinical characteristics were evaluated using the Mann-Whitney test, followed by the χ² test. RESULTS: Ten of the 51 patients with PSP showed a high signal intensity in the SCP on FLAIR MRI. Higher pixel values were observed within the SCP of patients with a high signal intensity in the SCP than in patients without a high signal intensity (p < 0.001). The sensitivity and specificity of the high signal intensity in the SCP of patients with PSP was 19.6% and 100%, respectively. This finding was more frequently observed in patients with PSP with Richardson's syndrome (PSP-RS) (25.7%) than other phenotypes (6.2%). CONCLUSION: The high signal intensity in the SCP on FLAIR MRI might be an effective diagnostic tool for PSP-RS.
Atrophy ; Diagnosis ; Gliosis ; Humans ; Magnetic Resonance Imaging ; Mesencephalon ; Neurodegenerative Diseases ; Neurons ; Parkinsonian Disorders ; Phenotype ; Sensitivity and Specificity ; Supranuclear Palsy, Progressive

Several prospective studies on laparoscopy-assisted distal gastrectomy for early gastric cancer have been initiated, but no prospective study evaluating laparoscopy-assisted total gastrectomy or laparoscopy-assisted proximal gastrectomy has been completed to date. A non-randomized confirmatory trial was commenced in April 2015 to evaluate the safety of laparoscopy-assisted total gastrectomy and laparoscopy-assisted proximal gastrectomy for clinical stage I gastric cancer. A total of 245 patients will be accrued from 42 Japanese institutions over 3 years. The primary endpoint is the proportion of patients with anastomotic leakage. The secondary endpoints are overall survival, relapse-free survival, proportion of patients with completed laparoscopy-assisted total gastrectomy or laparoscopy-assisted proximal gastrectomy, proportion of patients with conversion to open surgery, adverse events, and short-term clinical outcomes. The UMIN Clinical Trials Registry number is UMIN000017155.
Anastomotic Leak ; Asian Continental Ancestry Group ; Conversion to Open Surgery ; Gastrectomy* ; Humans ; Japan* ; Laparoscopy ; Medical Oncology* ; Non-Randomized Controlled Trials as Topic ; Prospective Studies ; Stomach Neoplasms*

OBJECTIVEPrevious studies on the association between physical activity (PA) and neighborhood environments (NE) focused on either objectively measuring the NE or the residents' perception of NE. Here, we investigate which actual or perceived NE is associated with residents' PA in Japan.

METHODSTwo regions with an objectively assessed high and low residential density, land use mix-diversity, and street connectivity, respectively, were identified in one city. The subjects were selected using a stratified random sampling method by sex and age in each region. The NE of the subjects was objectively measured using the Geographic Information System (GIS), and the subjects' perception of the NE was assessed using a questionnaire. The daily total number of walking steps was measured with an accelerometer, and walking and cycling time were assessed by a questionnaire.

RESULTSFor the female subjects, the mean cycling time, subjectively assessed as a means of transport, was significantly longer in the group with a high GIS score for the number of land use types, while the score for total number of walking steps was significantly higher among those who were aware of places to walk to, and cycling time for transport was longer for those who perceived an accessibility to post offices, banks/credit unions, gymnasiums/fitness facilities, and amusement facilities in their neighborhood. For the male subjects, the score for walking time for leisure was longer for those who perceived aesthetics and an accessibility to parks, and the score for total walking steps was significantly higher for those who perceived an accessibility to bookstores or rental video stores in their neighborhood.

CONCLUSIONSThe results to this study demonstrate that daily PA was high among female subjects living in a NE with land use mix-diversity, and who had an awareness of places to walk to and the accessibility to facilities for daily necessities in their neighborhood. For male subjects, daily PA was high among those who perceived the aesthetics of and accessibility to facilities for pleasure in their neighborhood. Further research is needed to determine the association between PA and NE on the basis of sex differences.

The purpose of this study is to address what undergraduate students of professional healthcare learned in interprofessional education (IPE), and to explore a way to develop interprofessional work (IPW) competencies effectively in IPE. A qualitative analysis was conducted on the portfolios of 9 medical, 8 dental, 16 pharmaceutical, and 16 nursing and rehabilitation students. 6 elements were identified in the undergraduate IPE; 【Patient/Family-Centered care】, 【Value/Ethics for IPW】, 【Communication Necessary for IPW】, 【Roles/Responsibilities as Healthcare Professionals】, 【Understanding of their own profession】, 【Team/Team work】. In order to acquire these capabilities, undergraduate education programs based on IPE are required.

The purpose of this study is to address what undergraduate students of professional healthcare learned in interprofessional education (IPE), and to explore a way to develop interprofessional work (IPW) competencies effectively in IPE. A qualitative analysis was conducted on the portfolios of 9 medical, 8 dental, 16 pharmaceutical, and 16 nursing and rehabilitation students. 6 elements were identified in the undergraduate IPE; 【Patient/Family-Centered care】, 【Value/Ethics for IPW】, 【Communication Necessary for IPW】, 【Roles/Responsibilities as Healthcare Professionals】, 【Understanding of their own profession】, 【Team/Team work】. In order to acquire these capabilities, undergraduate education programs based on IPE are required.