Prenatally diagnosed neuroblastomas have been reported in increasing numbers over the past several years. The vast majority are in favorable stages of the disease (stage I, II, IV-S). The authors experienced one case of stage IV-S neuroblastoma of the adrenal gland with liver metastasis, which regressed spontaneously after removal by adrenalectomy. This patient was noticed to have an abdominal mass at prenatal ultrasonography performed at 36weeks of gestation. This tumor was a neuroblastoma of the left adrenal gland with multiple liver metastases. Left adrenalectomy and liver biopsy were performed at 3 months of age. Thirty-eight months after surgery, an MRI demonstrated that the hepatic metastatic lesions had completely regressed without chemotherapy or radiation.
Adrenal Glands ; Adrenalectomy* ; Biopsy ; Drug Therapy ; Humans ; Liver* ; Magnetic Resonance Imaging ; Neoplasm Metastasis* ; Neuroblastoma* ; Pregnancy ; Prenatal Diagnosis ; Ultrasonography, Prenatal

OBJECTIVE: As an electronic health record system is implementing in Korean health care sectors, concerns about key functionalities of electronic health record systems are increasing. The purpose of this study was to identify core functions and set the priority in electronic health record systems under the Korean contexts in order to assure and improve the quality of the systems. METHODS: A survey was conducted using questionnaire developed by the study team based on literature review. The subjects were medical record administrators working at medical record department in general hospitals. RESULTS: The response rate was 59.8%(55/92). The functions which more than ninety percent of subjects responded as necessary right now and/or in near future related to 'drug alert', 'clinical guideline', 'chronic disease management', 'automated real-time surveillance', 'coded data', 'result reporting', 'de-identifying data', 'disease registry', and 'provider-provider communication and connectivity'. CONCLUSION: The results showed the high prioritized functions were decision support and health information/data management.
Electronic Health Records* ; Health Care Sector ; Hospitals, General* ; Humans ; Medical Record Administrators ; Medical Records ; Needs Assessment* ; Surveys and Questionnaires

Amyopathic dermatomyositis is defined by characteristic cutaneous manifestation of dermatomyositis without evidence of muscle involvement. There is no clinical difference between dermatomyositis and amyopathic dermatomyositis. Pulmonary involvement of dermatomyositis is so frequent than aggressive dianostic and therapeutic approach is needed. Early steroid or immunosuppresive treatment in pulmonary involvement of dermatomyositis gets better prognosis. Intravenous immunoglobulin treatment is an effective and safe alternative when the steroid or immunosuppresive treatment is ineffective or intolerable. We are reporting a case of amyopathic dermatomyositis with interstitial lung disease. This patient was improved with intravenous immunoglobulin treatment.
Dermatomyositis* ; Humans ; Immunoglobulins ; Lung Diseases, Interstitial* ; Prognosis

No abstract available.
Myocardial Bridging* ; Sleep Apnea, Obstructive* ; Spine*

No abstract available.
Myocardial Bridging* ; Sleep Apnea, Obstructive* ; Spine*

Background: Reduced lipid content in the stratum corneum is a major cause of skin-barrier dysfunction in various pathological conditions. Promoting lipid production is a potential strategy to improve skin-barrier function. Recent evidence supports the beneficial effects of adiponectin on lipid metabolism and senescence in keratinocytes. Objective: This study aimed to investigate whether plant extracts can enhance skin-barrier function. Methods: We screened fruit and herb extracts that enhance the lipid synthesis of keratinocytes via AMP-activated protein kinase (AMPK) activation and SIRT1 signaling in the adiponectin pathway. The levels of major lipid synthesis enzymes and transcription factors as well as epidermal barrier lipids involved in adiponectin-associated epidermal barrier formation were evaluated in the herbal extracts- or adiponectin-treated human epidermal keratinocyte and equivalent models. The mRNA expression of major lipid synthesis enzymes increased following treatment with Lycii Fructus, Prunus tomentosa, and Melia toosendan extracts. Results: The expression of transcription factors SIRT1, liver X receptor α, peroxisome proliferator-activated receptors (PPARs), and sterol regulatory element-binding proteins (SREBPs) were upregulated. Levels of free fatty acids, cholesterol, and ceramides were elevated. The expression of keratinocyte differentiation markers increased. In particular, among fruit extracts with a detectable effect, Melia toosendan induced the highest expression of lipid synthase. Conclusion These results indicate that Melia toosendan is a promising candidate for improving skin-barrier function.

Pleomorphic adenomas are the most common neoplasms of the salivary glands. A long-lasting pleomorphic adenoma can develop into a malignant tumor. Malignant mixed tumor of the salivary gland includes carcinoma ex pleomorphic adenoma, carcinosarcoma, and metastasizing pleomorphic adenoma. The metastasizing pleomorphic adenoma is to be distinct from the true malignant mixed tumor, because it remains histologically benign and can metastasize. We report the case of a 64-year-old female with a long-standing goose egg-sized neck mass and multiple small firm nodules on the trunk and extremities, which showed histological features metastasizing pleomorphic adenoma of the salivary gland.
Adenoma, Pleomorphic ; Carcinosarcoma ; Extremities ; Female ; Humans ; Middle Aged ; Mixed Tumor, Malignant ; Neck ; Neoplasm Metastasis* ; Salivary Glands ; Submandibular Gland*

A balance between production and degradation of reactive oxygen species has an important role in the cardiovascular homeostasis, and is known to contribute to hypertension. Under oxidative stress, an upregulation of inducible NOS (iNOS) induces ischemic-reperfusion injury, and is involved in the pathophysiology of the hypertension. Ischemic-reperfusion injury of the skeletal muscle results from reactive oxygen species, and overexpression of iNOS in the skeletal muscle increases the ischemic injury. Superoxide dismutase (SOD), antioxidant, is a major enzyme for degradation of reactive oxygen species (ROS). The purpose of this study was to observe the effect ischemic preconditioning (IP) of the lower limb on the expression of iNOS, CuZnSOD and MnSOD in the white and red muscle of the spontaneously hypertensive rat (SHR). Nine weeks old male normotensive rat (Wistar-Kyoto rat, WKY) and SHR were divided into control and IP groups. The IP group was further divided into 3 (3IP) and 10 (10IP) times of IP. Left common iliac artery was occluded 3 and 10 times for 5 min of ischemia-5 min of reperfusion using rodent vascular clamp. The animals were sacrificed at 0, 0.5, 1, and 3 hours after reperfusion and the Tibialis anterior and Soleus were removed. The expressions of iNOS, CuZnSOD and MnSOD in the skeletal muscle were examined with immunohistochemical methods and Western blot analysis. iNOS was expressed in Tibialis anterior, but in Soleus after IP. The expression of iNOS was increased in both WKY and SHR, it was higher in SHR than WKY. CuZnSOD and MnSOD were expressed in Tibialis anterior and Soleus, higher in Soleus, after IP. The expression of CuZnSOD and MnSOD were increased in both WKY and SHR, higher in WKY than SHR. It is consequently suggested that hypertensive individual and white muscle are more sensitive to ischemic injury of the skeletal muscle as considering their high expression of iNOS and low expression of SODs.
Animals ; Blotting, Western ; European Continental Ancestry Group ; Homeostasis ; Humans ; Hypertension ; Iliac Artery ; Ischemic Preconditioning ; Lower Extremity ; Male ; Muscle, Skeletal ; Muscles ; Oxidative Stress ; Rats ; Rats, Inbred SHR ; Reactive Oxygen Species ; Reperfusion ; Rodentia ; Superoxide Dismutase ; Superoxides ; Up-Regulation

Transient hyperammonemia of the newborn is an overwhelming disease manifestated by hyperammonemic coma in ill premature infant. This recognized metabolic disorder is chiefly characterized by severe hyperammonemia in the postnatal period, a comatous state, absence of abnormal organic aciduria, normal activity of urea cycle enzymes and, usually, complete recovery. The etiology is unknown. Infant had mild respiratory distress that progressed within 48 hours to deep coma requiring ventilatory assistance and had marked hyperammonemia. The degree of neurologic impairment and developmental delay in this disorder depends on the duration of the hyperammonemic coma. So, treatment of hyperammonemia should be initiated promptly and continued vigorously. We report of a preterm infant (34+5 weeks of gestation) presenting with respiratory distress, seizure, coma, and marked elevated plasma ammonia level.
Ammonia ; Coma ; Humans ; Hyperammonemia* ; Infant ; Infant, Newborn* ; Infant, Premature ; Plasma ; Seizures ; Urea

Both death certificates and postmortem examination certificates are used as proof of death. These certificates sometimes contain erroneous information but how frequently they do so is unknown. In particular, only a few studies have measured the accuracy of the cause and manner of death on Korea death documents. In this study, we compared the cause and manner of death on both kinds of certificates with those on autopsy reports to determine the frequency of errors, and to identify way to improve the accuracy of these certificates. In 2012, 528 autopsies were requested of out institute, and certificates were submitted in 241 of the cases. The manner of death was classified as natural, unnatural, or unknown. The cause of death in the autopsy report matched that on the death certificate in 37 of 63 cases (58.7%), and the manner of death matched in 40 of 63 cases (63.5%). The cause of death in the autopsy report matched that on the postmortem examination certificate in 62 of 178 cases (34.8%), and the manner of death matched in 74 of 178 cases (41.6%). Death certificates and postmortem examination certificates are important documents. We identified many incorrect reports of causes and manners of death on both kinds of documents, especially the postmortem death certificates. These inaccuracies are presumably due to a lack of forensic information and education, as well as lack of interest on the part of medical doctors.
Autopsy* ; Cause of Death ; Death Certificates* ; Education ; Korea