A Case of Macropharge Activation Syndrome Successfully Treated with Combination Therapy Including Etanercept

Macrophage activation syndrome (MAS) is a severe, potentially life-threatening complication of childhood systemic inflammatory disorder, primarily systemic onset juvenile rheumatoid arthritis (SoJRA). It is characterized by pancytopenia, liver insufficiency, coagulopathy, and neurologic symptoms. The clinical manifestations are caused by the activation and uncontrolled proliferation of T lymphocytes and macrophages, leading to cytokine overproduction including tumor necrosis factor-alpha (TNF-alpha). Methylprednisolone pulse therapy and cyclosporine A have made a considerable progress in the treatment of MAS. However, the mortality rate remains high suggesting the need of another therapeutic agent. Several cases of MAS successfully treated with TNF-alpha inhibitor (etanercept) have been reported. We report the first Korean case of MAS successfully treated with combination therapy of corticosteroid, cyclosporine A and etanercept.

Clinical Characteristics and Psychological Analysis in Children with Tic Disorder / 대한소아신경학회지

PURPOSE: Tic disorder is a neurodevelopmental disorder which begins in early childhood and continues into adolescence and adults. Tic disorder affects 1-2% of the population. In order to make a correct diagnosis and treatment of tic disorder, the clinical psychological analysis is needed because children with tic disorder tend to have higher rates of emotional and behavioral difficulties than those of the general population. The aim of this study was to evaluate the clinical and psychological characteristics of child with tic disorder. METHODS: The sample consisted of 69 patients with tic disorder who visited to the pediatric neurology clinic of Kangnam sacred heart hospital from January 2007 to June 2010. Fifty patients who visited our clinic without tic disorder were included in the control group. Patients were classified as the DSM-IV, Tourette's disorder, chronic motor or vocal tic disorder, transient tic disorder and tic disorder not otherwise specified. Korean child behavior checklist (K-CBCL), K-WISC-III, ADS, K-ARS was conducted in the tic disorder patients and T score of K-CBCL was compared with the control group. RESULTS: Male to female ratio was 2.5:1 in the study group and the mean age was 9.5+/-3.2 years old. The symptom period was 16.7+/-17.2 months at their first visit. Twelve subjects (18%) presented with Tourette syndrome, 14 subjects (20%) had multiple chronic motor or phonic tic disorder, 20 subjects (29%) had transient tic disorder and 23 subjects (33%) had tic disorder not otherwise specified. The Medications were given in 26 patients and risperidone was the primary drug for most of them. The mean T scores of K-CBCL were 55.5+/-6.1 for attention scale, 56.9+/-6.9 for anxiety-depression scale, 56.5+/-7.4 for withdrawn scale, 52.0+/-3.4 for emotional lability scale. All of them were higher than control group (P<0.05). CONCLUSION: In order to make a correct diagnosis and treatment of tic disorder, the clinical psychological assessment is mandatory due to their higher rates of emotional and behavioral difficulties.