Dynamic electrophysiological examination of cervical flexion myelopathy

Cervical flexion myelopathy is thought to arise following compression of the spinal cord by vertebrae or intervertebral discs and dura mater, or from overstretching of the spinal cord induced by cervical spinal flexion. However, the influence of spinal flexion on the spinal cord and the detailed origins of this disease are unknown. In this article the authors report a case of cervical flexion myelopathy in which dynamic electrophysiological examination was performed using an epidural electrode. This investigation showed the real-time influence of flexion of the cervical spine on spinal cord function. This technique was considered to be useful for diagnosis and in decision making for treatment. Anterior fusion was the optimal surgical method for treating this disease.     

Thymus and activation-regulated chemokine (TARC/CCL17) predicts decline of pulmonary function in patients with chronic obstructive pulmonary disease

BackgroundThe deterioration of pulmonary function, such as FEV₁-decline, is strongly associated with poor prognosis in patients with chronic obstructive pulmonary disease (COPD). However, few investigations shed light on useful biomarkers for predicting the decline of pulmonary function. We evaluated whether thymus and activation-regulated chemokine (TARC), a Th2 inflammation marker, could predict rapid FEV₁-decline in COPD patients.MethodsWe recruited 161 patients with stable COPD and performed pulmonary function test once every six months. At the time of registration, blood tests, including serum levels of TARC were performed. We assessed the correlation between changes in parameters of pulmonary function tests and serum levels of TARC. The rapid-decline in pulmonary function was determined using 25th percentile of change in FEV₁ or FEV₁ percent predicted (%FEV₁) per year.ResultsIn the FEV₁-rapid-decline group, the frequency of exacerbations, the degree of emphysema, and serum levels of TARC was higher than in the non-rapid-decline group. When using %FEV₁ as a classifier instead of FEV₁, age, the frequency of exacerbations, the degree of emphysema and serum levels of TARC in the rapid-decline group was significantly greater than those in the non-rapid-decline group. In univariate logistic regression analysis, TARC was the significant predictive factor for rapid-decline group. In multivariate analysis adjusted for emphysema, serum levels of TARC are independently significant predicting factors for the rapid-decline group.ConclusionsTARC is an independent predictive biomarker for the rapid-decline in FEV₁. Measuring serum TARC levels may help the management of COPD patients by predicting the risk of FEV₁ decline.     

Myelodysplastic Syndrome in a Patient with IPEX Syndrome

Journal of Clinical Immunology -     

Sudden death of a child because of an intestinal obstruction caused by a large congenital mesenteric defect

Transmesenteric hernias are internal hernias caused by a congenital defect in the mesentery. They are rare causes of intestinal obstruction, but most commonly affect the small bowel. We report an unexpected death of an infant with a bowel obstruction caused by a congenital mesenteric defect, which was undiagnosed despite visits to three different hospitals. Mesenteric defects are usually 2-3 cm in diameter. At autopsy, we found an oval, 14 × 7 cm congenital defect in the ileal mesentery through which the small bowel had herniated. Diagnosis of such defects remains difficult, even with currently available imaging techniques. Diagnosis is particularly difficult in infants who usually have nonspecific symptoms. Therefore, it is important that sudden unexpected deaths in children undergo full forensic evaluation to establish the precise cause of death. It is also important for forensic physicians to inform clinicians of the risk of such diseases, particularly in emergency situations.     

Eltrombopag in Combination with Rabbit Anti-thymocyte Globulin/Cyclosporine A in Immunosuppressive Therapy-naïve Patients with Aplastic Anemia in Japan

Objective In Japan, immunosuppressive therapy (IST) with anti-thymocyte globulin (ATG), and cyclosporine A (CsA) is the standard of care in patients with aplastic anemia (AA) who are not indicated for stem-cell transplantation, although some patients may experience relapse. This study assessed the efficacy and safety of eltrombopag in combination with rabbit-ATG/CsA in IST-naïve patients with non-severe or severe AA in Japan. Methods In this non-randomized, open-label, single-arm, phase II study, rabbit-ATG/CsA and eltrombopag were initiated on Days 1 and 15 (±3 days), respectively, and continued for ≥26 weeks; rabbit-ATG was given for 5 days (Days 1 to 5). The primary endpoint was the overall response rate (ORR) at Week 26. Patients Patients with AA who were IST-naïve and ≤70 years old or between 71 and 75 years old based on the recommendation of the investigator were enrolled in Japan. Results Of the 11 enrolled patients, 10 started treatment with eltrombopag. The ORRs at Weeks 26 and 52 were 70.0% and 60.0%, respectively. The ORR at Week 26 was 100% (all 3 patients) in patients with non-severe AA and 57.1% (4/7) in patients with severe AA. Among transfusion-dependent patients, 66.7% (4/6) and 62.5% (5/8) became red blood cell- and platelet-transfusion independent, respectively. The most common adverse events were nausea and headache. No deaths or hematologic malignancies were reported. A cytogenetic abnormality was reported in one patient. Conclusion This study confirmed the clinical benefit of eltrombopag plus rabbit-ATG/CsA in IST-naïve patients with non-severe or severe AA in Japan.