No abstract available.
Anemia, Dyserythropoietic, Congenital*

Peliosis hepatis is characterized by the presence in the liver of blood-filled cavities, which may or may not be lined with sinusoidal cells. The individual cysts or cavities usually do not exceed several centimeters in diameter. The cysts are typically continuous with adjacent, more normal sinusoids, and they sometimes can be seen in continuity with hepatic venous tributaries. The lesion is usually diagnosed by gross or microscopic examination. When suspected, it can be diagnosed by percutaneous liver biopsy. In the past, peliosis hepatis is primarily associated with wasting diseases, such as tuberculosis, malignancy, and chronic suppurative infection. However, recently peliosis hepatis is seen most commonly in association with the administration of anabolic steroids or HIV infection. We report a case of peliosis hepatis that is diagnosed by peritoneoscopic live biopsy and not associated with known disease.
Biopsy* ; HIV Infections ; Laparoscopy ; Liver* ; Peliosis Hepatis* ; Steroids ; Tuberculosis ; Wasting Syndrome

Wiskott-Aldrich syndrome(WAS) is an X-linked recessive immunodeficiency characterized by thrombocytopenia with small platelet volume, eczema, and recurrent infections, and is also characterized by increased incidence of auto immune diseases and malignancies. The phenotype observed in this syndrome is caused by mutation in the Wiskott-Aldrich syndrome protein(WASP) gene localized to the proximal short arm of the X chromosome and recently isolated through positional cloning. The gene encodes a 502 amino acid protein, which contains 12 exons and spans 9 kb of genomic DNA. The function of the encoded protein is not well understood. The clinical diagnosis of WAS can be difficult and is usually confirmed by the detection of WASP gene mutations and the expression of WSAP in patient blood sample using genetic analysis. We reported a case of a 13-month old boy with WAS who was identified with the novel mutation in exon 2 of WASP gene by direct sequencing and the complete absence of WASP expression by immunoblotting.
Arm ; Blood Platelets ; Clone Cells ; Cloning, Organism ; Diagnosis ; DNA ; Eczema ; Exons* ; Humans ; Immune System Diseases ; Immunoblotting ; Incidence ; Infant ; Male ; Phenotype ; Thrombocytopenia ; Wasps* ; Wiskott-Aldrich Syndrome* ; X Chromosome

OBJECTIVE: The purpose of this study is to demonstrate the technical feasibility and clinical efficacy of emergent carotid angioplasty and stenting (CAS) for acute stroke due to athero-thrombotic occlusion of the cervical internal carotid artery (ICA). METHODS: Review of medical records identified 17 patients who underwent emergent CAS for treatment of athero-thrombotic occlusion of the cervical ICA with acute stroke between 2009 and 2013. Eleven patients (64.7%) presented with concomitant intracranial artery occlusion, which was treated primarily by mechanical thrombectomy after CAS. RESULTS: Successful revascularization of the cervical ICA with emergent CAS was achieved in all patients. After CAS, intracranial recanalization with Thrombolysis in Cerebral Infarction > or =2b flow was achieved in four of the 11 patients (36.4%). The overall recanalization rate (cervical ICA and intracranial artery) was 10 of 17 patients (58.8%). Symptomatic intracranial hemorrhage occurred in two patients (11.8%), resulting in death. Ten patients (58.8%) showed improvement (decrease in NIHSS score of > or =4 points) at seven days after recanalization. Nine patients (52.9%) showed a favorable outcome (mRS < or =2) at the last follow-up. A favorable outcome (mRS < or =2) was obtained in four of the six patients with isolated cervical ICA occlusion (4/6, 66.7%) and five of 11 patients with intracranial tandem occlusion (5/11, 45.5%). CONCLUSION: Emergent CAS for acute stroke due to athero-thrombotic occusion of the cervical ICA showed a good technical feasibility and favorable clinical outcome.
Angioplasty ; Arteries ; Carotid Artery, Internal* ; Cerebral Infarction ; Follow-Up Studies ; Humans ; Intracranial Hemorrhages ; Medical Records ; Stents* ; Stroke* ; Thrombectomy

OBJECTIVES: Emotion perception deficit has long been suggested to be one of the core features of schizophrenia. Although there have been several studies examining responses to facial expressions, few studies addressed music emotion recognition. The aim of this study was to examine the difference in emotion recognition in music between normal subjects and patients with schizophrenia. METHODS: Twenty pieces of music were presented to 43 patients with chronic schizophrenia and 82 healthy controls. The set of music consisted of ten pieces of sad music and ten pieces of cheerful music. The subjects were asked to answer whether each piece of music was sad or cheerful. RESULTS: The correct response rate of music emotion recognition was lower in patients with schizophrenia compared with that in healthy controls (76.67+/-19.03% vs. 93.54+/-8.18%, p<0.001). When subgroup analyses was done by type of music ('sad' or 'cheerful'), patients showed a significantly lower correct response rate in recognizing both sad and cheerful emotion in music than healthy controls (sad emotion : 65.12+/-31.15% vs. 91.95+/-15.27%, cheerful emotion : 88.0+/-16.83% vs. 95.12+/-8.64%). The significant correlations between the clinical symptom severity of schizophrenia and music emotion recognition were not found to be. CONCLUSION: The results suggest that patients with schizophrenia might have difficulties in recognizing musical nuances.
Facial Expression ; Humans ; Music ; Schizophrenia

BACKGROUND: The imaging system that's currently being used in the field of dermatology is based on such instruments as the dermoscope, phototrichograph and camera. In recent years, the use of an image magnification system based on polarization has become popular. OBJECTIVE: In this study, optical quantification was performed based on the multiwavelength imaging analysis of the structures that form dermatologic diseases, and an attempt was made to enhance the image quality by using polarization technology. METHODS: The lesions of three patients who were clinically diagnosed with cherry angioma, melanocytic nevus and inflammatory lesions in acne and freckles were measured at the outpatient clinic of the Department of Dermatology of the authors' hospital. All the patients were female, and their mean age was 29.3 years. RESULTS: The optical characteristics of the patients' various skin lesions, including cherry angioma, melanocytic nevus and inflammatory lesions in acne and freckles, were distinguishable by their wavelength. CONCLUSION: The use of different kinds of information may be helpful for measuring and diagnosing various skin lesions that have not been differentiated with using the previous modalities. Further, if the various environmental factors that may be generated during the measurement process can be controlled, then these study results can be applied to a standard diagnostic modality in the field of dermatology.
Acne Vulgaris ; Ambulatory Care Facilities ; Dermatology ; Female ; Hemangioma ; Humans ; Melanosis ; Nevus, Pigmented ; Prunus ; Skin

BACKGROUND/AIMS: Cytomegalovirus (CMV) reactivations are frequently observed in patients with active ulcerative colitis (UC), and ganciclovir therapy is effective in patients with steroid-refractory UC. This study aimed to determine the long-term outcomes of CMV reactivation and the long-term therapeutic efficacy of ganciclovir treatment. METHODS: This retrospective multicenter study included a cohort of 72 patients with moderate-to-severe UC who were evaluated for CMV reactivation at the time of their initial UC flare. Colectomy, disease relapse, and the recurrence rate of CMV reactivation were investigated. RESULTS: The mean duration of follow-up for the 72 patients was 43.16+/-19.78 months (range, 1 to 67 months). The cumulative colectomy (log-rank, p=0.025) and disease flare-up rates (log-rank, p=0.048) were significantly higher in the CMV-positive group. Of the 11 patients who were successfully treated with ganciclovir in the initial treatment, three patients (27.3%) experienced CMV reactivation, and six patients (54.5%) experienced poor outcomes, such as the need for colectomy or a steroid-dependent state. CONCLUSIONS: The patients who had CMV-reactivated UC showed poor outcomes at the long-term follow-up, and the long-term efficacy of ganciclovir therapy was marginal. Careful assessment is necessary for patients who exhibit evidence of CMV reactivation.
Antiviral Agents/*therapeutic use ; Case-Control Studies ; Cohort Studies ; Colectomy/utilization ; Colitis, Ulcerative/complications/*drug therapy ; *Cytomegalovirus ; Cytomegalovirus Infections/complications/*drug therapy ; Ganciclovir/*therapeutic use ; Humans ; Longitudinal Studies ; Remission Induction ; Retrospective Studies ; Severity of Illness Index ; Treatment Outcome ; *Virus Activation

The purpose of this paper is to introduce standardization activities of nursing documentation for special nursing units following standardization efforts for general nursing units last year. Modified Delphi approach with expert panel was used to identify essential nursing documents and data set for each units. Expert panel was consisted of head nurses or charge nurses of each special nursing unit from 8 tertiary hospitals with more than 500 beds in Seoul. the secretary-general of Clinical Nurses Association and a faculty of College of Nursing. The exiting nursing forms of seven special nursing units, which include Emergency room, Intensive care unit, Operating room, Respiratory intensive care unit. Delivery floor. Nursery and Dialysis room, were analyzed and prototypes of the standard nursing forms and guidelines were developed. The clinical field test was done with the help of Clinical Nurses Association. At the field test 3.744 clinical staff nurses from 20 tertiary hospitals with more than 500 beds in Korea were involved and provided feedback. Finally public hearing was held and more than 600 nurses from 116 hospitals attended and provided feedback. Through these process consensus of nursing community was attained for standard documents and data items. The result is available at http://nursing.snu.ac.kr/standard/ through internet.
Consensus ; Dataset ; Dialysis ; Emergency Service, Hospital ; Hearing ; Intensive Care Units ; Internet ; Korea ; Nurseries ; Nursing* ; Nursing, Supervisory ; Operating Rooms ; Seoul ; Tertiary Care Centers

Leptospirosis is a spirochetal infectious disease caused by Leptospira interrogans, and may vary in degree from an asymptomatic infection to a severe and fatal illness. The kidney is one of the principal target organs of Leptospira. Renal disorders caused by Leptospira infection vary from an abnormality in urinalysis to acute kidney injury (AKI). Incidence of AKI in severe leptospirosis varies from 40% to 60%. AKI reflects the severity of leptospirosis and is generally accompanied by cholestatic jaundice. The pathophysiology of AKI in leptospirosis consists of hypovolemia, direct tubular toxicity, and rhabdomyolysis. Most patients with acute leptospirosis experience severe myalgias, and show laboratory evidence of mild rhabdomyolysis. However, occurrence of severe rhabdomyolysis is rare. We report here on a patient with leoptospirosis, who had severe rhabdomyolysis and acute kidney injury without jaundice.
Acute Kidney Injury ; Asymptomatic Infections ; Communicable Diseases ; Humans ; Hypovolemia ; Incidence ; Jaundice ; Jaundice, Obstructive ; Kidney ; Leptospira ; Leptospira interrogans ; Leptospirosis ; Rhabdomyolysis ; Urinalysis