Background/Aims: Intestinal Behçet disease is typically associated with ileocecal punched-out ulcers and significant morbidity and mortality. Intestinal ultrasound is a noninvasive imaging technique for disease monitoring. However, no previous reports have compared intestinal ultrasound with endoscopic ulcer activity or histopathological findings for intestinal Behçet disease. We evaluated the usefulness of intestinal ultrasound for assessing the activity of ileocecal ulcers in intestinal Behçet disease. Methods: We retrospectively compared intestinal ultrasound findings with 73 corresponding endoscopic images and 6 resected specimens. The intestinal ultrasound findings were assessed for 7 parameters (bowel wall thickness, vascularity [evaluated using the modified Limberg score with color Doppler], bowel wall stratification, white-plaque sign [strong hyperechogenic lines or spots], mesenteric lymphadenopathy, extramural phlegmons, and fistulas), and endoscopic ulcer activity was classified into active, healing, and scar stages. Histopathological findings were evaluated by consensus among experienced pathologists. Results: Bowel wall thickness (P< 0.001), vascularity (P< 0.001), loss of bowel wall stratification (P= 0.015), and white-plague sign (P= 0.013) were significantly exacerbated in the endoscopic active ulcer stage. Receiver operating characteristic curve analysis revealed that a bowel wall thickness of > 5.5 mm (sensitivity 89.7%, specificity 85.3%) was potentially useful for detecting active lesions. When compared with histopathological findings, an increase in bowel wall thickness reflected the ulcer marginal ridge, and the white-plaque sign reflected the ulcer bottom. Conclusions Intestinal ultrasound is useful for monitoring intestinal ulcer activity in intestinal Behçet disease.

We describe the case of a 1-year and 7-month-old girl who was born at 36 weeks and 6 days of pregnancy weighing 1,351 g. In addition to the diagnosis of Cornelia de Lange syndrome and Tetralogy of Fallot, we confirmed shunt blood flow from the lesser curvature of the aortic arch to the main pulmonary artery. Thus, we additionally diagnosed ectopic patent ductus arteriosus (PDA). Ultrasonography showed interruption and retrograde flow of the diastolic blood flow in the anterior cerebral artery. Therefore, we made a diagnosis of blood stealing due to an ectopic PDA, and we performed surgical intervention via a median sternotomy at 25 days. When we ligated the shunt blood vessel, the percutaneous oxygen saturation decreased from the high 90% range to the low 70% range, thus we temporarily released the ligation. We narrowed the ectopic PDA so that the percutaneous oxygen saturation could be maintained the high 80% range. Postoperative ultrasonography showed improvement of the pressure waveform in the anterior cerebral artery. After discharge, oxygen demand increased gradually with weight gain, and we performed intracardiac repair using a monocusp valve patch at 1 year and 7 months. We report a rare case of Cornelia de Lange syndrome wherein we achieved intracardiac repair of Tetralogy of Fallot after ectopic PDA banding in the neonatal period.

Wide resection of malignant bone and soft tissue tumors of the extremities may require resection of muscles, which correspondingly impairs limb movements. We describe a 67-year old man with a malignant soft tissue tumor of the right upper arm. Preoperatively, there was no impairment of right upper extremity function. The patient underwent wide resection of the tumor and triceps muscle. Postoperative rehabilitation included range of motion exercises, residual muscle strength exercises, and activities of daily living (ADL) exercises. One week postoperatively, the patient could independently perform the ADL exercises. Two weeks postoperatively, the patient scored 2 during manual muscle testing (MMT) for elbow extension, indicating a complete range of motion in a gravity-eliminated position. However, the patient could not raise the arm without bending it. Considering the needs of the patient, we prescribed an elbow extension brace to support the upper limb while being raised. With this brace, the patient was able to sustain elbow extension during upper limb elevation. Three months postoperatively, the patient's elbow joint extension remained MMT 2, grip strength was 28 kg, and the International Society of Limb Salvage and Musculoskeletal Tumor Society score was 76.7％．Although the triceps muscle was resected, there was no problem with the patient's ADL. However, the patient could not maintain elbow extension in an anti-gravity position while raising the upper limb. In such cases, prescribing an elbow brace may be useful.

Wide resection of malignant bone and soft tissue tumors of the extremities may require resection of muscles, which correspondingly impairs limb movements. We describe a 67-year old man with a malignant soft tissue tumor of the right upper arm. Preoperatively, there was no impairment of right upper extremity function. The patient underwent wide resection of the tumor and triceps muscle. Postoperative rehabilitation included range of motion exercises, residual muscle strength exercises, and activities of daily living (ADL) exercises. One week postoperatively, the patient could independently perform the ADL exercises. Two weeks postoperatively, the patient scored 2 during manual muscle testing (MMT) for elbow extension, indicating a complete range of motion in a gravity-eliminated position. However, the patient could not raise the arm without bending it. Considering the needs of the patient, we prescribed an elbow extension brace to support the upper limb while being raised. With this brace, the patient was able to sustain elbow extension during upper limb elevation. Three months postoperatively, the patient’s elbow joint extension remained MMT 2, grip strength was 28 kg, and the International Society of Limb Salvage and Musculoskeletal Tumor Society score was 76.7％．Although the triceps muscle was resected, there was no problem with the patient’s ADL. However, the patient could not maintain elbow extension in an anti-gravity position while raising the upper limb. In such cases, prescribing an elbow brace may be useful.

We report a case of a 64-year-old woman who presented with symptoms for the common cold, for a medical examination. The chest X-ray showed enlargement of the heart, and echocardiography detected a mobile mass suggestive of a tumor 21×14 mm in the left ventricle. Because there was a risk of embolism, we decided to perform an operation. The tumor was elastic and soft and had a stalk arising from the wall of the left ventricle. We considered that it would be difficult to observe and expose the left ventricular tumor by direct vision. Therefore, we chose to perform a complete endoscopic resection. The tumor was determined histopathologically to be a papillary fibroelastoma. Here, we report the relatively rare benign tumor, a papillary fibroelastoma, and include a discussion of the literature.

BACKGROUND/AIMS: Noninvasive liver fibrosis evaluation was performed in patients with nonalcoholic fatty liver disease (NAFLD). We used a quantitative method based on the hepatic volume acquired from gadoxetate disodium-enhanced (Gd-EOB-DTPA-enhanced) magnetic resonance imaging (MRI) for diagnosing advanced fibrosis in patients with NAFLD. METHODS: A total of 130 patients who were diagnosed with NAFLD and underwent Gd-EOB-DTPA-enhanced MRI were retrospectively included. Histological data were available for 118 patients. Hepatic volumetric parameters, including the left hepatic lobe to right hepatic lobe volume ratio (L/R ratio), were measured. The usefulness of the L/R ratio for diagnosing fibrosis ≥F3–4 and F4 was assessed using the area under the receiver operating characteristic (AUROC) curve. Multiple regression analysis was performed to identify variables (age, body mass index, serum fibrosis markers, and histological features) that were associated with the L/R ratio. RESULTS: The L/R ratio demonstrated good performance in differentiating advanced fibrosis (AUROC, 0.80; 95% confidence interval, 0.72 to 0.88) from cirrhosis (AUROC, 0.87; 95% confidence interval, 0.75 to 0.99). Multiple regression analysis showed that only fibrosis was significantly associated with the L/R ratio (coefficient, 0.121; p<0.0001). CONCLUSIONS: The L/R ratio, which is not influenced by pathological parameters other than fibrosis, is useful for diagnosing cirrhosis in patients with NAFLD.
Body Mass Index ; Fibrosis* ; Humans ; Liver Cirrhosis ; Magnetic Resonance Imaging ; Methods ; Non-alcoholic Fatty Liver Disease* ; Retrospective Studies ; ROC Curve

We report a patient displaying several symptoms of myelitis associated with atopic dermatitis. The patient, a 35-year-old female, initially suffered from gait titubation that gradually developed into motor disturbance. She underwent many tests (blood, cerebrospinal fluid, electromyography, and brain, cervical, and lumbar spinal cord and muscle MRIs) at several hospitals, including a university institution, over the course of two years until she finally came to our hospital. However, her disease was never identified by these tests, and she was referred to our hospital to start rehabilitation for her disability which was believed to be caused by disuse syndrome. On first examination, muscle hypertonia of both legs, hypoesthesia of all extremities, difficulty sitting, and a low performance level of ADL were observed. Judging by the factors (e.g. age, neurological symptoms), we considered that the patient was not suffering disuse syndrome, and recommended that she attend another medical college hospital for a thorough investigation. The disease was finally diagnosed as atopic myelitis, as indicated by the test results (e.g. hyperIgEemia). She could sit without a back rest and undertake gait training between parallel bars after steroid pulse therapy at the hospital. While she undertook rehabilitation at our hospital, we applied some approaches to adapt to her fluctuating symptoms (e.g. making short leg braces for both legs, injection of botulinum toxin A (BOTOX^®) in the leg). We recognized we should gather as much information as possible given the rarity of the disease.

Dynamic knee valgus is considered a risk factor of non-contact anterior cruciate ligament (ACL) injury. To identify athletes at a higher risk, we developed a two-dimensional (2D) video-based screening test that determines hip abductor function as well as dynamic hindfoot and knee valgus. The purpose of this study was to validate the accuracy of the indices for dynamic knee valgus derived knee-in distance (KID) and hip-out distance (HOD) from the 2D-video.Twenty healthy university students agreed to participate in this study. Subjects were asked to step off a 30-cm box and land on one leg. This procedure was recorded simultaneously using a 2D video camera in the frontal plane and the Vicon motion capture system. Pearson's correlations examined associations between KID, KID normalized by height (KID/H), HOD, as well as HOD normalized by height (HOD/H) and 3D-valgus (knee valgus) or 3D-IR (tibial internal rotation).Significant correlations were found between the KID and 3D-valgus (r=0.72, p<0.01) and KID/H and 3D-valgus (r=0.73, p<0.01). Associations were not significant between KID and 3D-IR (r=0.08) and between KID/H and 3D-IR (r=0.03). A positive moderate correlation between HOD and 3D-valgus (r=0.46, p<0.05) and HOD/H and 3D-valgus (r=0.50, p<0.05), as well as a negative moderate correlations between HOD and 3D-IR (r=-0.52, p<0.05) and between HOD/H and 3D-IR (r=-0.51, p<0.05) were also observed.We conclude that KID is a reliable alternative for the 3D-valgus and the HOD is for the 3D-valgus and tibial external rotation.

Dynamic knee valgus is considered a risk factor of noncontact ACL injuries. The aim of this study was to determine the association between hip and hindfoot dynamic alignment and dynamic knee valgus.This cross-sectional study involved 88 high school female basketball players (175 legs). Subjects performed single-leg squatting and drop landing which provided a hip-out distance (HOD) and a knee-in distance (KID) via 2D video images. Hip and hindfoot dynamic alignment was evaluated by a dynamic Trendelenburg test (DTT) and a heel-floor test (HFT), respectively, during squatting and landing.The DTT-positive group (dynamic hip mal-alignment with lower non-weightbearing pelvis) demonstrated greater HOD and KID values for both squatting (p＜0.001) and landing (p＜0.001) than the DTT-negative group. The HFT-positive group (5^o or greater hindfoot valgus) demonstrated greater KID values for both squatting (p＜0.01) and landing (p＜0.001), but smaller HOD value for squatting (p＜0.01) than the HFT-negative group.In conclusion, dynamic hip mal-alignment may be associated with both greater HOD and KID, but hindfoot valgus only with greater KID.

We experienced the coronary artery bypass grafting (CABG) with essential thrombocythemia (ET). A case is a man of 73 years old. As for him, 3 vessel disease including left main trunk was recognized on coronary angiography, and it was planned CABG. However, we recognized blood cell aberration in blood examination, and it was diagnosed as ET. ET is classed as a chronic myeloproliferative disorder. It has two opposite tendencies, a bleeding tendency and thrombus tendency. Ischemic heart disease to merge ET is acute myocardial infarction by thrombus in case of most, and there are a few cases to need blood circulation reconstruction of coronary artery for angina pectoris. Perioperative hemorrhage and postoperative graft closure become a problem in CABG with ET. With the hydroxycarbamide which is DNA synthesis inhibitor of a platelet count, a function controlled it, and enforced CABG. He doesn't have any cardiac events and complications due to ET for 7 years post CABG. We report this case with a review of the literature.