Electro‐vectorcardiographic demonstration of bifascicular block associated with ventricular preexcitation

Down syndrome occurs more frequently in the offsprings of older pregnant women and may be associated with atrioventricular septal defect. This refers to a broad spectrum of malformations characterized by a deficiency of the atrioventricular septum and abnormalities of the atrioventricular valves caused by an abnormal fusion of the superior and inferior endocardial cushions with the midportion of the atrial septum and the muscular portion of the ventricular septum.     

Outcomes of Primary Total Knee Arthroplasty in Patients With Ehlers-Danlos Syndromes

Background

Ehlers-Danlos syndromes (EDS) are connective tissue disorders that cause defects in collagen synthesis or processing, resulting in joint hypermobility. Following total knee arthroplasty (TKA), concern exists that hypermobility will affect the outcome as prosthesis relies on soft tissues for stability. The purpose of this study is to investigate the outcome of TKA in the setting of EDS compared to patients undergoing TKA for osteoarthritis (OA).

Treatment of generalized infantile myofibromatosis with sorafenib and imatinib: A case report

Infantile myofibromatosis (IM) is characterized by solitary musculoskeletal nodules presenting during infancy but can manifest as multiple lesions with visceral involvement. Multicentric IM with visceral involvement carries a high risk of mortality and there is no consensus on treatment. We present a case of a patient with multicentric IM and pulmonary involvement who progressed on several chemotherapeutic regimens and subsequently had a complete response to sorafenib and later imatinib. This report describes the novel use of sorafenib and imatinib to treat generalized IM and the role of continued tyrosine kinase inhibitor therapy to maintain remission.     

Multiple Symmetric Lipomas: A Case Report

Multiple symmetric lipomas are a rare disease characterized by multiple symmetrical deposits of adipose tissue associated with alcohol abuse. A 68-year-old man was admitted to our hospital for being operated on for a mass growth on the neck and back. Based on the patient's medical history and preoperative magnetic resonance imaging findings, we performed surgical resection. There was no recurrence at one year postoperatively.     

Unilateral bamboo node of the vocal fold associated with anti-SS-A and anti-SS-B antibody

Voice disorder is occasionally associated with systemic autoimmune diseases. Bamboo nodes of the vocal fold have a characteristic bamboo-shaped appearance and strongly indicate the presence of an underlying autoimmune disorder. Both mechanical and immunologic mechanisms are assumed to be involved in the pathogenesis of vocal disorder. We present a 27-year-old woman with hoarseness, sore throat, and a unilateral bamboo node of the vocal fold. Serum anti-SS-A and -SS-B antibodies were positive, but she had no systemic signs or symptoms suggestive of Sjögren’s syndrome. Oral systemic glucocorticoid treatment was not effective, but surgical resection improved her hoarseness. Histopathologic findings of the resected vocal node revealed fibrosis with hyaline degeneration. Thereafter, she had no recurrence of hoarseness for 2 years. Bamboo nodes of the vocal fold may occur without definitive autoimmune diseases, although immunologic abnormalities such as autoantibody-positivity may occur.