A 59-year-old woman has presented symptoms of fatigue since January 1996. Atypical coarctation due to aortitis syndrome had been diagnosed 8 years earlier. Her upper-limb blood pressure was 200mmHg and antihypertensive drugs were administered. An aortogram showed severe stenoses of the aorta at the level of the diaphragm and renal artery. A computed tomogram showed extensive calcification of the aorta below the origin of the left subclavian artery. She underwent a bypass operation with a 16-mm-diameter prosthetic graft from the ascending aorta to the infrarenal abdominal aorta. She has progressed well after the bypass and her upper-limb blood pressure is almost normal.

Advances in genetic medicine has rapidly been applied to clinical practice. However, many medical students have not studied biology or genetics in high school. There is little chance to think in Japan medical education about how to treat genetic information appropriately in the setting of clinical medicine. The timing and contents of a clinical genetics education program in medical school has hardly been discussed in Japan. This paper discusses the clinical genetics educationduring the medical-science and clinical-medicine stages at Nippon Medical School.
1) An exercise on information gathering and role-play (for 180 minutes) about color vision deficiency were performed during the second-year molecular genetics course.
2) A clinical genetics course (45 minutes 18 classes) in the fourth year was started in 2002 as a part of an integrated medical curriculum with courses classified by organ system.
3) This clinical genetics course included systematic lectures for knowledge acquisition, lectures by patient support groups, exercises in drawing pedigrees, role-play, and discussions of ethical issues. Students evaluated this course favorably.
4) Some topics in clinical genetics can be effectively presented at an early stage of medical education as part of an introduction to medicine. To maximize the educational effects and increase the possibility that students understand the importance of medical genetics, clinical genetics education in medical school will be performed after the student have grasped a basic understanding of diseases through lectures about clinical subjects.

NIPPV provides positive pressure ventilation through a face mask without intubation. We performed NIPPV for 2 patients with acute respiratory failure following cardiovascular surgery. (Case 1) A 63-year-old man, who had had COPD (Hugh-Jones class III), underwent replacement of the aortic arch. He was extubated after 5 days. However, he was re-intubated under controlled ventilation because of deterioration of his respiratory condition. The patient had NIPPV after extubation on postoperative day 14 because he was alert and had no cardiovascular compromise. On the 18th postoperative day he was weaned from NIPPV. (Case 2) A 67-year-old man underwent coronary artery bypass surgery. On the next day he was extubated, but he suffered from hypoxemia due to impaired respiratory condition on postoperative day 3. The patient underwent NIPPV instead of conventional mechanical ventilation because his condition was stable except for respiration. Respiratory condition improved quickly and he was weaned from NIPPV on the 7th postoperative day. NIPPV is an effective method for managing patients with acute respiratory failure after cardiovascular surgery.

A 71-year-old woman underwent mitral valve replacement with Carpentier-Edwards Pericardial Xenograft for mitral regurgitation on January 8, 1987. She had nocturnal hematuria and paroxysmal nocturnal hemoglobinuria was diagnosed in November, 1992. She had congestive heart failure in February, 1993. Cineradiographical analysis revealed a fracture of the wireform in three locations of the Xenograft and the stent was distorted inward. A second mitral valve replacement was successfully performed on March 16, 1993. She was discharged 45 days after operation after an uneventful course.

A 31-year-old woman complained of dizziness. Preoperative aortogram revealed a saccular type of aneurysm of the aortic arch and abnormal origin of the left subclavian artery. She underwent reconstruction of the aortic arch through 4th intercostal thoracotomy on August 10th, 1996. The aneurysm originated distally to the left common carotid artery and its wall was very thin. Aortic arch replacement with woven Dacron prosthesis was performed between the left common carotid artery and the left subclavian artery using the inclusion method under hypothermic circulatory arrest. The post operative course was uneventful. Cystic medial necrosis of the aneurysmal wall, and normal arterial findings of the left femoral artery were recognized by the pathohistological examinations. Kinking of the aorta is a congenital disease due to abnormal embryonal development. The aortic arch elongates between the left common carotid artery and the left subclavian artery, and arch aneurysm originates in this portion, because the aneurysmal wall is very thin and fragile when accompanied with cystic medial necrosis. Therefore, it is important that the left subclavian artery originating distally to the arch aneurysm in this category of the aortic arch aneurysm be recognized. There are 11 cases which were successfully operated for aortic arch aneurysm associated with congenital kinking of the aorta in Japan.

BACKGROUND AND PURPOSE: Plasma D-dimer levels are elevated during the acute phase of cerebral infarction (CI). We investigated whether the D-dimer level on admission and other clinical characteristics could be used to predict the poor outcome of patients with acute CI. METHODS: The clinical characteristics and plasma D-dimer levels measured within 3 days of onset were compared according to outcome among patients with acute CI. RESULTS: In total, 359 consecutive patients (mean age, 71.8 years) were examined, of which 174 had a poor outcome [score on the modified Rankin scale (mRS) > or =3] at 30 days after hospitalization. The mean mRS score was higher and a poor outcome was observed more frequently among women than among men (p<0.001 for each). The proportions of women, cardioembolism, atrial fibrillation, advanced age (> or =75 years), prior history of CI or transient ischemic attack, and elevated D-dimer level (> or =1.0 microg/mL) were significantly higher among patients with a poor outcome than among those with a good outcome. A multivariate analysis showed that elevated D-dimer level [> or =1.0 microg/mL; odds ratio (OR), 2.45; 95% confidence interval (95% CI), 1.52-3.89; p<0.01], advanced age (OR, 1.93; 95% CI, 1.21-3.07; p<0.01), and female gender (OR, 1.75; 95% CI, 1.08-2.83; p=0.02) were independent predictors of a poor outcome. CONCLUSIONS: Certain clinical characteristics (gender and advanced age) and an elevated D-dimer level upon admission can be used to predict the outcome of patients with acute CI at 30 days after hospitalization.
Atrial Fibrillation ; Cerebral Infarction ; Female ; Fibrin Fibrinogen Degradation Products ; Hospitalization ; Humans ; Ischemic Attack, Transient ; Male ; Multivariate Analysis ; Odds Ratio ; Plasma ; Stroke

OBJECTIVES: As the population ages, the number of lumbar spinal surgeries performed on sarcopenic patients will increase. The purpose of this study was to investigate the prevalence of sarcopenia and evaluated its impact on the results of lumbar spinal surgery. METHODS: This study included 2 groups: One group consisted of patients who underwent whole-body dual-energy X-ray absorptiometry (DXA) scanning before the option of undergoing surgery for lumbar spinal disease (LSD group) and a second group consisted of patients underwent DXA scanning for osteoporosis screening under hospital watch at the geriatric medicine department (control group). In order to evaluate the impact of sarcopenia on the clinical outcome of lumbar spinal surgery, the Japanese Orthopedic Association (JOA) score, the recovery rate based on the JOA score, and visual analogue scale (VAS) scores for lower back pain, lower extremity pain, and lower extremity numbness were compared within the LSD group. RESULTS: The prevalence of sarcopenia showed no statistical difference between groups (control group, 50.7%; LSD group, 46.5%). In the LSD group, while the changes in VAS scores showed no statistical difference between the nonsarcopenia subgroup and sarcopenia subgroup, the sarcopenia subgroup demonstrated inferior JOA scores and recovery rates at the final follow-up when compared with the nonsarcopenia subgroup (P < 0.05). CONCLUSIONS: This study demonstrated a high prevalence of sarcopenia among the elderly populations in Japan and a negative impact of sarcopenia on clinical outcomes after lumbar spinal surgery.
Absorptiometry, Photon ; Aged ; Asian Continental Ancestry Group ; Follow-Up Studies ; Humans ; Hypesthesia ; Japan ; Low Back Pain ; Lower Extremity ; Lysergic Acid Diethylamide ; Mass Screening ; Orthopedics ; Osteoporosis ; Prevalence ; Sarcopenia ; Spinal Diseases

Background/Aims: The optimal length of the uncovered portion of partially covered self-expandable metal stents (PCSEMSs) used in endoscopic ultrasound-guided hepaticogastrostomy (EUS-HGS) remains unclear. This study investigated the safety and efficacy of PCSEMSs with different uncovered lengths, with a focus on stent migration and time to recurrent biliary obstruction (RBO). Methods: Outcomes of patients undergoing EUS-HGS using PCSEMSs with 5-mm and 20-mm uncovered portions at our institution from January 2016 to December 2021 were compared. Results: Sixty-two patients underwent EUS-HGS using PCSEMS (5/20-mm uncovered portions: 32/30). Stent migration occurred only in the 5-mm group. There were no differences in RBO rates (28.1% vs. 40.0%) or median time to RBO (6.8 vs. 7.1 months) between the two groups. Median overall survival (OS) was longer in the 20-mm group (3.1 vs. 4.9 months, p=0.037) due to the higher number of patients that resumed chemotherapy after EUS-HGS (56.7% vs. 28.1%, p=0.029). Good performance status, absence of hepatic metastases, and chemotherapy after EUS-HGS were independent predictors of longer OS. Conclusions No migration was observed in patients treated with PCSEMS with 20-mm uncovered portions. Patients treated with PCSEMS with 20-mm uncovered portions performed at least as well as those treated with 5-mm uncovered portions in all material respects.

BACKGROUND/AIMS: Decreased trough levels of infliximab (TLI) and antibodies to infliximab (ATI) are associated with loss of response (LOR) in Crohn's disease. Two prospective studies were conducted to determine whether TLI or ATI better correlates with LOR (Study 1), and whether TLI could become a predictor of mucosal healing (MH) (Study 2). METHODS: Study 1 was conducted in 108 patients, including those with LOR and remission to compare ATI and TLI in discriminating the 2 conditions based on receiver operating characteristic (ROC) curve analyses. Study 2 involved 35 patients who were evaluated endoscopically. RESULTS: In Study 1, there were no differences between the 2 assays in ROC curve analyses; the TLI cutoff value for LOR was 2.6 µg/mL (sensitivity, 70.9%; specificity, 79.2%), and the ATI cutoff value was 4.9 µg/mL (sensitivity, 65.5%; specificity, 67.9%). The AUROC (area under the ROC curve) of TLI was greater than that of ATI. AUROC was useful for discriminating between the 2 conditions. In Study 2, the TLI was significantly higher in the colonic MH group than in the non-MH group (2.7 µg/mL vs. 0.5 µg/mL, P=0.032). CONCLUSIONS: TLI is better than ATI for clinically diagnosing LOR, and a correlation was observed between TLI and colonic MH.
Antibodies ; Cohort Studies* ; Colon ; Crohn Disease* ; Humans ; Infliximab* ; Prospective Studies* ; ROC Curve ; Sensitivity and Specificity

BACKGROUND/AIMS: Colonic diverticular hemorrhage (DH) was a rare disease until the 1990s, and its incidence has increased rapidly since 2000 in Japan. In recent years, colonic DH has been the most frequent cause of lower gastrointestinal bleeding (LGIB). Nearly all cases of DH are mild, with the bleeding often stopping spontaneously. Some cases, however, require surgery or arterial embolization. In this study, using a cohort at Fukuoka University Chikushi Hospital, we investigated factors associated with severe colonic DH. METHODS: Among patients with LGIB who underwent colonoscopy at our hospital between 1995 and 2013, DH was identified in 273 patients. Among them, 62 patients (22.7%) were defined as having severe colonic DH according to recurrence of bleeding in a short period, and/or the necessity of transfusion, arterial embolization, or surgery. We then evaluated risk factors for severe DH among DH patients in this retrospective cohort. RESULTS: Among the 273 patients with DH, use of non-steroidal anti-inflammatory drugs (NSAIDs) (odds ratio [OR], 2.801; 95% confidence interval [CI], 1.164–6.742), Charlson Risk Index (CRI) ≥2 (OR, 3.336; 95% CI, 1.154–7.353), right-sided colonic DH (OR, 3.873; 95% CI, 1.554–9.653), and symptoms of cerebral hypoperfusion (such as light-headedness, dizziness, or syncope) (OR, 2.926; 95% CI, 1.310–6.535) showed an increased risk of severe DH even after controlling for other factors. CONCLUSIONS: Severe DH occurred in 23% of DH patients, and NSAID use, CRI ≥2, right-sided colonic DH, and symptoms of cerebral hypoperfusion are suggested to be predictors of severe DH.
Anti-Inflammatory Agents, Non-Steroidal ; Cohort Studies ; Colon* ; Colonoscopy ; Dizziness ; Hemorrhage* ; Humans ; Incidence ; Japan ; Rare Diseases ; Recurrence ; Retrospective Studies ; Risk Factors*