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Lymphatic malformation (LM) is a congenital disorder resulting from an abnormal development of lymphatic vessels. LM may result in problems of cosmesis and functional impairment, including airway compression. An 11-year-old girl was referred to our department with increasing dysphagia caused by a large left cervical LM with a long history of treatment. Because of the LM location, surgical resection was not an option, and various therapies, including use of picibanil, had proven ineffective. Celecoxib treatment (100 mg/day) was initiated for local pain management. Softening of the lesion was observed 2 weeks after treatment initiation, and the dose was increased to 200 mg/day with additional shrinking of the LM over the next 2 weeks. With parental consent, celecoxib was continued, with a 65% reduction in volume achieved at 6 months. The patient discontinued treatment at 12 months, and the LM volume increased. Control over the LM was achieved with resumption of celecoxib treatment. After 2 years of treatment, the LM persists, but the size of the malformation is significantly smaller. No adverse effects of celecoxib treatment were observed. The anti-cyclooxygenase-2 effect of celecoxib prevented lymphatic vessel growth through an inhibition of cyclooxygenase-2 activity in the conversion of prostaglandin to prostaglandin E2. In conclusion, celecoxib may be a promising therapeutic agent for LM management. [ABSTRACT FROM AUTHOR]

Objective: To estimate target of treatment for long-term efficacy of the first biologic agent used to treat polyarticular juvenile idiopathic arthritis (pJIA). Methods: A retrospective cohort of patients with pJIA treated at six medical institutions in Japan between 1 March 2005 and 31 October 2014 was identified. The patients were divided by 2-year treatment periods with the first biologic agent into continuous treatment group and switching group. Three markers were examined: matrix metalloproteinase-3 (MMP-3), erythrocyte sedimentation rate (ESR), and disease activity score (DAS) 28-ESR. Results: Thirty-two pJIA patients (8 boys, 24 girls) from 43 recruited patients were included in this study. The treatment periods with the first biologic agent in continuous treatment group (24 patients, 75%) was 40 months (median, range 24-119) and switching group (8 patients; 25%) was 9.5 months (median, 6-18). Markers [odds ratio (95% confidence interval)] at 3 months were MMP-3 [1.02 (0.99-1.05), p = .219], ESR [1.00 (0.78-1.30), p = .998], and DAS28-ESR [13.9 (2.08-409.82), p = .035]. The cut-off point for DAS28-ESR at 3 months to distinguish the two groups was 2.49 (sensitivity, 87.5%; specificity, 87.5%). Conclusion: DAS28-ESR of 2.49 at 3 months after initiating the first biologic agent can be a target of sustained treatment in pJIA patients. [ABSTRACT FROM AUTHOR]

Background: The dose-limiting toxic effect of cyclophosphamide (CY) is cardiotoxicity. The pathogenesis of myocardial damage is poorly understood, and there is no established means of prevention. In previous studies, we suggested that for CY-induced cardiotoxicity, whereas acrolein is the key toxic metabolite, carboxyethylphosphoramide mustard (CEPM) is protective. We sought to verify that acrolein is the main cause of cardiotoxicity and to investigate whether aldehyde dehydrogenase (ALDH), which is associated with greater CEPM production, is involved in the protective effect for cardiotoxicity. We also evaluated the protective effect of N-acetylcysteine (NAC), an amino acid with antioxidant activity and a known acrolein scavenger. Methods: H9c2 cells were exposed to CY metabolites HCY (4-hydroxy-cyclophosphamide), acrolein or CEPM. The degree of cytotoxicity was evaluated by MTT assay, lactate dehydrogenase (LDH) release, and the production of reactive oxygen species (ROS). We also investigated how the myocardial cellular protective effects of CY metabolites were modified by NAC. To quantify acrolein levels, we measured the culture supernatants using high performance liquid chromatography. We measured ALDH activity after exposure to HCY or acrolein and the same with pre-treatment with NAC. Results: Exposure of H9c2 cells to CEPM did not cause cytotoxicity. Increased ROS levels and myocardial cytotoxicity, however, were induced by HCY and acrolein. In cell cultures, HCY was metabolized to acrolein. Less ALDH activity was observed after exposure to HCY or acrolein. Treatment with NAC reduced acrolein concentrations. Conclusions: Increased ROS generation and decreased ALDH activity confirmed that CY metabolites HCY and acrolein are strongly implicated in cardiotoxicity. By inhibiting ROS generation, increasing ALDH activity and decreasing the presence of acrolein, NAC has the potential to prevent CY-induced cardiotoxicity. [ABSTRACT FROM AUTHOR]

Objectives: This study aimed to evaluate the usefulness of S100A12 and vascular endothelial growth factor (VEGF) for predicting the stability of remission for discontinuing methotrexate (MTX) and/or biological agents in Japanese patients with oligo/polyarticular juvenile idiopathic arthritis (JIA). Methods: Forty-four patients with oligo/polyarticular JIA who received MTX with or without biological agents were enrolled. Serum concentration of both S100A12 and VEGF were simultaneously evaluated by ELISA in active and in remission phase determined by activity markers including DAS-28. Results: S100A12 and VEGF were correlated with DAS-28. Of the 22 patients with oligo/polyarticular JIA in clinical remission, 13 patients with low S100A12 and VEGF concentrations could discontinue treatment without relapse over 2 years. However, nine patients without low S100A12 and VEGF concentrations relapsed afterwards, even though they had been in clinical remission. The cut-off levels of S100A12 and VEGF for division into two groups of the maintenance remission and relapse groups were 177 ng/ml and 158 pg/ml, respectively. Conclusions: S100A12 and VEGF are useful markers for assessing disease activity of oligo/polyarticular JIA in remission phase. These markers should be kept low when clinicians consider tapering or discontinuing treatments in oligo/polyarticular JIA patients. [ABSTRACT FROM AUTHOR]

Objectives: To evaluate the safety and effectiveness of hepatitis B virus (HBV) vaccination in patients with juvenile idiopathic arthritis (JIA) controlled by treatment. Methods: Among 49 patients with juvenile idiopathic arthritis (JIA) at the outpatient clinic of Kagoshima University Hospital, we enrolled 25 who were controlled by treatment. All children were unimmunized and were vaccinated against HBV according to the schedule. Their responses to the vaccine and vaccine adverse events were examined during their visits. Results: Nineteen of the 25 patients with JIA controlled by treatment developed effective antibody responses (76%). All eight patients with JIA below 10 years of age achieved seroconversion. The seroconversion was not influenced by biologics. Five adverse events were observed (6.7%). The rate of all adverse events did not surpass that of a previous report, and all adverse events were immediately resolved. None of the patients with JIA experienced a flareup or clinical deterioration related to the vaccination. Conclusions: HBV vaccination is safe and effective. Pediatric rheumatologists should consider HBV vaccination for unimmunized patients with JIA, because the response to HBV vaccine might be influenced by age, and children have a higher risk for potential HBV infection than adults. [ABSTRACT FROM AUTHOR]

Background: Kawasaki disease (KD) is an acute febrile vasculitis in childhood. Currently, treatment with 2 g/kg of intravenous immunoglobulin (IVIG) is recommended. Previously we had encountered a patient with KD who showed persistent fever and a severe eruption after IVIG treatment. Using a drug-induced lymphocyte stimulation test (DLST), he was positive for an immunoglobulin product. The aim of this study was to clarify the importance of a positive value for the DLST for immunoglobulin products in KD patients. Methods: Subjects were 30 confirmed KD patients treated with IVIG at the Kagoshima Medical Association Hospital. DLST values were compared between patients with additional events and those without additional events using the stimulation index (SI = value of 3H-thymidine absorption with antigen/without antigen). Additional events were defined as symptoms observed after IVIG that were considered unexplainable by the symptoms of KD alone. Results: DLST results were evaluated in 13 patients with additional events and 17 patients without additional events. Elevated DLST values were observed not only in patients with additional events but also in those without additional events. Elevated SI values were observed in the initial 14 days after IVIG and the SI values in this period were significantly higher than those after day 14 (initial 14 days, n = 20, 194 ± 112%; after day 14, n = 10, 117 ± 66%, p = 0.010). Conclusions: Elevated SI values of DLST for immunoglobulin products are not related with additional events. Our results show they may represent one of the immunological abnormalities of KD. Copyright © 2010 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

The aberrant induction of proinflammatory cytokines is considered to be crucial in the pathogenesis of systemic juvenile idiopathic arthritis and adult‐onset Still's disease. Interleukin‐18 (IL‐18) in particular has been reported to be a candidate for the key cytokine in both diseases; however, the origin of IL‐18 is unclear. To clarify the origin, we investigated specimens from various organs obtained during autopsy of a child with systemic JIA and macrophage activation syndrome, using immunohistochemical staining. Our results showed a high number of cells expressing IL‐18 in the bone marrow but not in the other organs. This finding suggests that bone marrow is the origin of increased serum IL‐18 and raises the possibility that other proinflammatory cytokines are also induced by IL‐18 in bone marrow in this disease. Bone marrow may be an essential organ in the pathogenesis of systemic JIA. [ABSTRACT FROM AUTHOR]

Introduction We report the case of a patient who developed symptoms of acute cerebellar ataxia (ACA) after administration of the human papilloma virus (HPV)-16/18 vaccine. Patient and Method This patient developed symptoms of ACA, including nausea, vertigo, severe limb and truncal ataxia, and bilateral spontaneous continuous horizontal nystagmus with irregular rhythm, 12 days after administration of the HPV-16/18 AS04- adjuvanted cervical cancer vaccine. After this, the patient received methylprednisolone pulse and intravenous immunoglobulin (IVIG) therapies as well as immunoadsorption plasmapheresis. Results Severe ACA symptoms did not improve after methylprednisolone pulse and IVIG therapies, but the patient recovered completely after immunoadsorption plasmapheresis. Conclusion This temporal association strongly suggests that ACA was induced by the vaccination. [ABSTRACT FROM AUTHOR]

Abstract  We report herein of a 6-year-old boy with descending necrotizing mediastinitis (DNM) secondary to retropharyngeal abscess, who was successfully treated by the administration of antibiotics. DNM is very rare in children, and surgical drainage of the mediastinum is recommended in adult patients. DNM should be recognized as a severe complication of an oropharyngeal infection in children, and early and accurate diagnosis is important in ensuring appropriate clinical management. [ABSTRACT FROM AUTHOR]

Abstract  We describe a case of an 11-year-old girl who presented with osteomyelitis of the vertebrae and right femur due to Bartonella henselae. Her only symptom was prolonged fever without focal pain. Magnetic resonance imaging (MRI) and nested polymerase chain reaction (PCR) were useful for the diagnosis. Osteomyelitis due to B. henselae should be considered in cases of prolonged fever of unknown origin. [ABSTRACT FROM AUTHOR]