PURPOSE: To evaluate the findings and the role of CT in plexiform neuro-fibromatosis of the mediastinum. MATERIALS AND METHODS: We retropectively reviewed the CT scans of five patients with plexiform neurofibromatosis of the mediastinum. The CT scans were reviewed with attention to the distribution of the lesions, appearance and attenuation of mediastinal lesions, enhancement pattern after intravenous contrast infusion and associated findingssuch as intercostal neurofibroma. RESULTS: In all five patients CT scans demonstrated fusiform low attenuated masses which were oriented longitudinally and extended over multiple contiguous scans along the distribution of major mediastinal nerves. In four patients, mediastinal lesions appeared infiltrative, obliterating adjacent mediastinal fat plane. One patient had well defined fusiform masses along the major mediastinal nerves. Postcontrast enhanced CT scans revealed slight central enhancement in two patient and no contrast enhancement in three patients. Associated findings such as neurofibromas of intercostal nerves and sympathetic trunk, or subcutaneous neurofibromas were detected on CTscans in all five patients. CONCLUSION: Characteristic CT findings of low attenuation masses along the major mediastinal nerves are helpful to differentiate plexiform neurofibromatosis from mediastinal lymphadenopathy and to prevent from misreading as a malignant disease.
Humans ; Intercostal Nerves ; Lymphatic Diseases ; Mediastinum* ; Neurofibroma ; Neurofibromatoses* ; Tomography, X-Ray Computed

BACKGROUND: Various tools for the acute response system (ARS) predict and prevent acute deterioration in pediatric patients. However, detailed criteria have not been clarified. Thus we evaluated the effectiveness of bradycardia as a single parameter in pediatric ARS. METHODS: This retrospective study included patients who had visited a tertiary care children's hospital from January 2012 to June 2013, in whom ARS was activated because of bradycardia. Patient's medical records were reviewed for clinical characteristics, cardiologic evaluations, and reversible causes that affect heart rate. RESULTS: Of 271 cases, 261 (96%) had ARS activation by bradycardia alone with favorable outcomes. Evaluations and interventions were performed in 165 (64.5%) and 13 cases (6.6%) respectively. All patients in whom ARS was activated owing to bradycardia and another criteria underwent evaluation, unlike those with bradycardia alone (100.0% vs. 63.2%, p = 0.016). Electrocardiograms were evaluated in 233 (86%) cases: arrhythmias were due to borderline QT prolongation and atrioventricular block (1st and 2nd-degree) in 25 cases (9.2%). Bradycardia-related causes were reversible in 202 patients (74.5%). Specific causes were different in departments at admission. Patients admitted to the hemato-oncology department required ARS activation during the night (69.3%, p = 0.03), those to the endocrinology department required ARS activation because of medication (72.4%, p < 0.001), and those to the gastroenterology department had low body mass indexes (32%, p = 0.01). CONCLUSIONS: Using bradycardia alone in pediatric ARS is not useful, because of its low specificity and poor predictive ability for deterioration. However, bradycardia can be applied to ARS concurrently with other parameters.
Arrhythmias, Cardiac ; Atrioventricular Block ; Body Mass Index ; Bradycardia* ; Electrocardiography ; Endocrinology ; Gastroenterology ; Heart Arrest ; Heart Rate ; Hospital Rapid Response Team ; Humans ; Medical Records ; Pediatrics ; Retrospective Studies ; Sensitivity and Specificity ; Tertiary Healthcare

PURPOSE: Bisphosphonates are widely used for the management steroid-induced osteoporosis (SIO) in children. With the increasing use of bisphosphonates, there have been reports of abnormal radiological findings in the growing skeleton. Therefore, their use in pediatric patients remains controversial. The present study was conducted to evaluate the long-term follow-up radiographic features, particularly metaphyseal sclerotic lines, in children who receive pamidronate therapy for nephropathy. METHODS: Twenty-four children with nephropathy treated with oral calcium and pamidronate (mean duration, 9 months; dose, 100 mg daily), were evaluated retrospectively. All patients had SIO secondary to chronic glucocorticoid therapy for treating nephropathy. Long bone radiographic imaging was performed before treatment with pamidronate, and at follow-up, several years later. Physeal growth rates were estimated by measuring the distance that the sclerotic lines moved on the radiographs during the corresponding time intervals. RESULTS: The mean follow-up period was 138 months. Long bone radiographs showed well-defined sclerotic lines at the metaphyseal ends, progressively moving from the physeal plate to the diaphysis, in all patients. The mean rate of movement of the sclerotic line was 6.21 mm per year. In 12 patients, the lines disappeared. The mean rate of growth in height was 7.33 cm per year. CONCLUSIONS: Results of long-term follow-up suggest that the metaphyseal sclerotic lines associated with pamidronate treatment tend to disappear without affecting overall skeletal growth. Bisphosphonate treatment for SIO in children with nephropathy seems to be safe, although further studies in larger number of patients are needed.
Calcium ; Child* ; Diaphyses ; Diphosphonates ; Follow-Up Studies* ; Humans ; Osteoporosis ; Retrospective Studies ; Skeleton

Isopropylantipyrine is one of the pyrazolon derivatives which are nonsteroidal anti-inflammatory drugs (NSAIDs) with potent antipyretic and pain-relieving properties. It has been known that pyrazolon-hypersensitive patients, in contrast to ASA-sensitive patients, could safely use other NSAIDs. We describe two Korean patients with isopropylantipyrine hypersensitivity without ASA/NSAID hypersensitivity.
Anti-Inflammatory Agents, Non-Steroidal ; Humans ; Hypersensitivity*

Primary biliary cirrhosis (PBC) is characterized by histological findings of an immunoinflammatory destruction of small- and medium-sized bile ducts with progressive portal fibrosis, and the presence of anti-mitochondrial antibody (AMA) with a laboratory evidence of chronic cholestasis. The term "autoimmune cholangitis" (AIC) is used for a disease with the clinical and pathologic features of primary biliary cirrhosis (PBC) but with negative AMA and positive anti-nuclear antibody (ANA) tests. Eight cases of AIC and ten cases of PBC were reviewed in order to determine whether there was any difference between two diseases in clinico-pathologic aspects. All of the patients were female and the mean ages of AIC and PBC patients were 48 and 47 years, respectively. ANA test was positive in six of ten PBC paients and their mean titer was lower than that of AIC patients. IgM level was significantly higher in PBC group than in AIC group. No significant difference was found between two groups with respect to biochemical and histopathological features. Since the only consistently distinguishing features between these two conditions are the autoantibody profile (AMA vs ANA) and immunoglobulin level (IgM), these two conditions might be part of a spectrum. PBC can be considered to be the same as AMA-positive AIC or alternatively AIC to be the same as AMA-negative PBC.
Bile Ducts ; Cholangitis* ; Cholestasis ; Female ; Fibrosis ; Humans ; Immunoglobulin M ; Immunoglobulins ; Liver Cirrhosis, Biliary*

No abstract available.
Humans ; Living Donors*

No abstract available.
Humans ; Living Donors*

Purpose: The purpose of this study was to identify the experiences of insomnia symptoms in the elderly living alone in the community. Methods: Eleven of the elderly with insomnia symptoms living alone in the community were interviewed. Data were collected through individual interviews July-September 2018. Colaizzi’s phenomenological methodology was used. Results: Results showed that the experiences of insomnia symptoms of the elderly living alone comprised 11 themes clusters and five categories: 1) Insomnia symptoms triggered by various causes, 2) Physical symptoms exacerbated by insomnia, 3) Insomnia worsened by psychological symptoms, 4) Useless efforts to fix insomnia, 5) Living with adapted insomnia symptoms. Conclusion It is necessary to develop a sleep intervention program that includes insomnia symptoms awareness, sleep health education, and social support factors. Community nurses should focus on insomnia symptoms as well as listen and empathize with various psychological phenomena in caring for the elderly with insomnia living alone.

Cephalosporines are the most important beta-lactams inducing IgE-mediated reactions such as urticaria, angioedema and anaphylaxis. There have been a few reports that describes assays of serum specific IgE for cephalosporins. We experienced a case of cefaclor-induced anaphylaxis and detected serum specific IgE to cefalor-human serum albumin (HSA) conjugate. A 40-year-old man was hospitalized due to sudden dyspnea. generalized urticaria, facial edema 30 minutes after taking cefaclor (250mg, po) for an upper respiratory infection. His systolic blood pressure dropped to 50 mmHg at admission. He had been treated with cefaclor for chronic osteomyelitis of the right heel for 1 year without adverse reactions. He had no personal or family history of allergic disease nor penicillin hypersensitivity. Skin prick test with cefaclor showed a positive response and serum specific IgE to cefaclor-HSA conjugate was detected by ELISA inhibition test showed significant inhibitions with additions of cefaclor-HSA conjugate and cefaclor in a dose-dependent manner. In conclusion, we confirmed that cefaclor-induced anaphylaxis in this patient was an IgE-mediated reation to cefaclor-HSA conjugate.
Adult ; Anaphylaxis ; Angioedema ; beta-Lactams ; Blood Pressure ; Cefaclor* ; Cephalosporins ; Dyspnea ; Edema ; Enzyme-Linked Immunosorbent Assay ; Heel ; Humans ; Hypersensitivity ; Hypersensitivity, Immediate* ; Immunoglobulin E* ; Osteomyelitis ; Penicillins ; Serum Albumin ; Skin ; Urticaria

BACKGROUND: AEB071, an orally available PKC inhibitor, prevents organ rejection after transplantation in rodents and man. Furthermore, pro-inflammatory cytokines and inflammatory processes are important mediators of transplanted organ rejection. We therefore examined whether single or combination therapies of AEB071 and/or tacrolimus affect cytokine profiles in a rat cardiac allograft model. METHODS: AEB071 (60 mg/kg twice a day) and tacrolimus (0.6 or 1.2 mg/kg once a day) were orally administered daily after cardiac transplantation. Interferon (IFN)-gamma, interleukin (IL)-1beta, IL-2, IL-4, IL-6, IL-10, and tumor necrosis factor (TNF)-alpha levels in serum were subsequently measured 5 days after cardiac transplantation using a multiplex protein assay system. RESULTS: All cytokine levels were significantly depressed in cardiac transplanted rats treated with AEB071, whereas tacrolimus only reduced IFN-gamma, IL-2, IL-4, IL-6, and IL-10 levels. When administered in combination, AEB071 and low- or high-dose tacrolimus had additive effects on IFN-gamma, IL-4, IL-6, and TNF-alpha. CONCLUSIONS: These results suggest that AEB071 inhibits T cell activation by blocking the production of proinflammatory cytokines, and that tacrolimus combined with AEB071 can effectively regulate inflammatory cytokines in the transplantation setting.
Animals ; Cytokines ; Heart Transplantation ; Immunosuppression ; Interferons ; Interleukin-10 ; Interleukin-2 ; Interleukin-4 ; Interleukin-6 ; Interleukins ; Pyrroles ; Quinazolines ; Rats ; Rejection (Psychology) ; Rodentia ; Tacrolimus ; Transplantation, Homologous ; Transplants ; Tumor Necrosis Factor-alpha