Congenital coloboma which is characterized by an absence of a part of normal ocular tissues involving iris, lens, ciliary body, choroid, and optic nerve. And choroidal coloboma is a rare disease in which there are defects of a part or all parts of choroid and retinal pigment epithelium. Retinal detachment has been reported in 23-42% of the patients with choroidal coloboma, and when retinal breaks are within the area of coloboma, conventional scleral buckling technique has resulted in low rates of anatomic success. We report a case of choroidal coloboma combined by retinal detachment which was treated by pars, plana vitrectomy, fluid-gas exchange, and laser photocoagulation.
Choroid* ; Ciliary Body ; Coloboma* ; Humans ; Iris ; Light Coagulation ; Optic Nerve ; Rare Diseases ; Retinal Detachment* ; Retinal Perforations ; Retinal Pigment Epithelium ; Retinaldehyde* ; Scleral Buckling ; Vitrectomy

PURPOSE: To assess whether the Modified Early Warning Score (MEWS) predicts the need for intensive care unit (ICU) transfer for patients with severe sepsis or septic shock admitted to general wards. METHODS: A retrospective chart review of 100 general ward patients with severe sepsis or septic shock was implemented. Clinical information and MEWS according to point of time between ICU group and general ward group were reviewed. Data were analyzed using multivariate logistic regression and the area under the receiver operating characteristic curves with SPSS/WIN 18.0 program. RESULTS: Thirty-eight ICU patients and sixty-two general ward patients were included. In multivariate logistic regression, MEWS (odds ratio [OR] 2.02, 95% confidence interval [CI] 1.43-2.85), lactic acid (OR 1.83, 95% CI 1.22-2.73) and diastolic blood pressure (OR 0.89, 95% CI 0.80-1.00) were predictive of ICU transfer. The sensitivity and the specificity of MEWS used with cut-off value of six were 89.5% and 67.7% for ICU transfer. CONCLUSION: MEWS is an effective predictor of ICU transfer. A clinical algorithm could be created to respond to high MEWS and intervene with appropriate changes in clinical management.
APACHE ; Aged ; Blood Pressure/physiology ; Female ; Hospital Mortality ; Humans ; Intensive Care Units ; Lactic Acid/analysis ; Logistic Models ; Male ; Middle Aged ; Odds Ratio ; Patients' Rooms ; ROC Curve ; Reproducibility of Results ; Retrospective Studies ; Risk Assessment/methods ; Sepsis/*pathology ; Shock, Septic/*pathology

PURPOSE: This study was done to examine factors influencing nurses' perception of patient safety culture in reporting of patient safety events. METHODS: Structured questionnaires were used to collect data from 305 nurses who were involved in direct patient care. Data were analyzed using descriptive statistics, t-test, ANOVA, Pearson correlation coefficients and multiple linear regressions with SPSS/WIN version 24.0. RESULTS: Patient safety events were reported as follows: 4.60±0.63 for harmful incidents, 4.02±0.82 for no harm incidents, and 3.59±0.97 for near misses. Patient safety event reporting was significantly positively correlated with patient safety culture. Regression analysis showed, factors influencing reports of harmful incidents were ‘feedback and communication about error’, ‘supervisor/manager expectations’ and ‘carrier of hospital’. Factors influencing reports of no harm incidents were ‘feedback and communication about error’. Factors influencing reports on near-misses were ‘teamwork across units’, ‘overall perceptions of safety’, and ‘feedback and communication about error’. CONCLUSION: Findings show that reports of near misses are relatively low and need to be strengthened. These results provide evidence that reporting on patient safety events would be enhanced through improved patient safety culture. Hospital managers could identify factors that affect reporting of each patient safety event and use it to develop intervention programs for risk management.
Humans ; Linear Models ; Patient Care ; Patient Safety ; Risk Management

Patients with Kennedy syndrome, which progresses more slowly than amyotrophic lateral sclerosis show a mild degree of motor fluctuation but rarely show significant decremental responses to repetitive nerve stimulations. Even in a patient with decremental responses to repetitive nerve stimulations, there is usually no significant improvements in motor symptoms to anticholinesterases. We experienced a patient with Kennedy syndrome, who showed significant decremental responses to repetitive nerve stimulations and a marked degree of motor fluctuation. His motor fluctuation responded dramatically to anticholinesterase. (J Korean Neurol Assoc 19(5):544~546, 2001)
Amyotrophic Lateral Sclerosis ; Bulbo-Spinal Atrophy, X-Linked* ; Cholinesterase Inhibitors ; Humans ; Myasthenia Gravis*

PURPOSE: To identify the clinical and epidemiological characteristics of human metapneumovirus infections (hMPV) in children compared to respiratory syncytial virus A (RSV A) and B (RSV B). METHOD: A retrospective review of medical records was performed in 36 patients with hMPV infection, 106 with RSV A infection, and 51 with RSV B infection, from September 2007 to July 2012. RESULTS: The peak incidence of hMPV infection was observed in May, whereas for RSV infections in November and December. hMPV infection occurred in older patients compared to RSV A and B infection (29.9+/-32.5 months vs. 13.6+/-15.4 months, P<0.001; 29.9+/-32.5 months vs. 12.1+/-13.5 months, P<0.001, respectively). hMPV infection was more often associated with fever compared to RSV A (97.2% vs. 67.9%, P<0.001), while wheezing was less frequent compared to RSV A and B infection (16.7% vs. 47.2%, P=0.001; 16.7% vs. 37.3%, P=0.037, respectively). hMPV infection was more often diagnosed as pneumonia compared to RSV A infection (72.2% vs. 50.0%, P=0.047) while bronchiolitis was less frequent than in RSV A (5.6% vs. 34.9%, P=0.001) or RSV B infection (5.6% vs. 29.4%, P=0.006). In addition, intravenous antibiotic was more often prescribed for patients with hMPV infection than those with RSV A and B (69.4% vs. 39.6%, P=0.002; 69.4% vs. 43.1, P=0.015, respectively). CONCLUSION: This study identified characteristics of hMPV infection compared to RSV A and B infection. Seasonality in spring, higher age group, and higher proportion of pneumonia in hMPV infections may be a useful guide for management of respiratory viral infections in children.
Bronchiolitis ; Child ; Fever ; Humans* ; Incidence ; Medical Records ; Metapneumovirus* ; Pneumonia ; Respiratory Sounds ; Respiratory Syncytial Viruses* ; Retrospective Studies ; Seasons

Transplantation-associated thrombotic microangiopathy (TA-TMA) is an uncommon but devastating complication in patients who undergo hematopoietic stem cell transplantation (SCT). However, the optimal treatment strategy for TA-TMA is unclear. We report a rare case of TA-TMA in a 39-month-old boy who underwent tandem autologous SCT (autoSCT) for high-risk medulloblastoma. TA-TMA developed 64 days after the second autoSCT with microangiopathic hemolytic anemia, fever, renal impairment, acute respiratory distress syndrome and posterior reversible encephalopathy syndrome. The patient recovered after plasmapheresis and methylprednisolone therapy. He had mild to moderate deficiency of a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS-13). The patient's clinical course would suggest that plasmapheresis and methylprednisolone therapy could be a treatment option for TA-TMA. Early intervention is needed to aid the recovery of the patient who is suspected for TA-TMA.
Anemia, Hemolytic ; Child, Preschool ; Early Intervention (Education) ; Fever ; Hematopoietic Stem Cell Transplantation ; Humans ; Male ; Medulloblastoma ; Methylprednisolone ; Pediatrics ; Plasmapheresis ; Posterior Leukoencephalopathy Syndrome ; Respiratory Distress Syndrome, Adult ; Stem Cell Transplantation ; Thrombospondins ; Thrombotic Microangiopathies

PURPOSE: Malignant rhabdoid tumor (MRT) is a rare and highly aggressive tumor that affects young children. Due to its extreme rarity, most of the available data are based on retrospective case series. To add to the current knowledge of this disease, we reviewed the patients treated for extra-cranial MRT in our institute. MATERIALS AND METHODS: A retrospective medical record review was conducted on children treated for pathologically confirmed extra-cranial MRT at Seoul National University Children's Hospital between January 2003 and May 2013. RESULTS: Eleven patients (7 boys, 4 girls) were diagnosed with extra-cranial MRT at a median age of 9 months old. INI1 staining was important in the pathological confirmation. Six patients (55%) had renal MRT and five (45%) had soft tissue MRT. Five patients (45%) had metastases at diagnosis. All patients underwent chemotherapy, eight patients (73%) underwent surgery, six patients (55%) received therapeutic radiotherapy, and four patients (36%) underwent high dose chemotherapy with autologous stem cell rescue (HDCT/ASCR) with melphalan, etoposide, and carboplatin. Five patients (45%) died of disease following progression (n=3) or relapse (n=2), however, there was no treatment related mortality. The overall survival of the cohort was 53.0% and the event-free survival was 54.5% with a median follow-up duration of 17.8 months (range, 2.3 to 112.3 months). CONCLUSION: Extra-cranial MRT is still a highly aggressive tumor in young children. However, the improved survival of our cohort is promising and HDCT/ASCR with melphalan, etoposide, and carboplatin may be a promising treatment option.
Carboplatin ; Child* ; Cohort Studies ; Diagnosis ; Disease-Free Survival ; Drug Therapy ; Etoposide ; Follow-Up Studies ; Humans ; Kidney Neoplasms ; Medical Records ; Melphalan ; Mortality ; Neoplasm Metastasis ; Radiotherapy ; Recurrence ; Retrospective Studies ; Rhabdoid Tumor* ; Seoul ; Soft Tissue Neoplasms ; Stem Cells

Background/Aims: Scoring systems play an important role in predicting intensive care unit (ICU) admission or estimating the risk of death in critically ill patients with hematological malignancies. We evaluated the modified early warning score (MEWS) for predicting ICU admissions and in-hospital mortality among at-risk patients with hematological malignancies and developed an optimized MEWS. Methods: We retrospectively analyzed derivation cohort patients with hematological malignancies who were managed by a medical emergency team (MET) in the general ward and prospectively validated the data. We compared the traditional MEWS with the MEWS plus SpO2/FiO2 (MEWS_SF) score, which were calculated at the time of MET contact. Results: In the derivation cohort, the areas under the receiver-operating characteristic (AUROC) curves were 0.81 for the MEWS (95% confidence interval [CI], 0.76 to 0.87) and 0.87 for the MEWS_SF score (95% CI, 0.87 to 0.92) for predicting ICU admission. The AUROC curves were 0.70 for the MEWS (95% CI, 0.63 to 0.77) and 0.76 for the MEWS_SF score (95% CI, 0.70 to 0.83) for predicting in-hospital mortality. In the validation cohort, the AUROC curves were 0.71 for the MEWS (95% CI, 0.66 to 0.77) and 0.83 for the MEWS_SF score (95% CI, 0.78 to 0.87) for predicting ICU admission. The AUROC curves were 0.64 for the MEWS (95% CI, 0.57 to 0.70) and 0.74 for the MEWS_SF score (95% CI, 0.69 to 0.80) for predicting in-hospital mortality. Conclusions Compared to the traditional MEWS, the MEWS_SF score may be a useful tool that can be used in the general ward to identify deteriorating patients with hematological malignancies.

BACKGROUND/AIMS: The modified early warning score (MEWS) is used to predict patient intensive care unit (ICU) admission and mortality. Lactate (LA) in the blood lactate (BLA) is measured to evaluate disease severity and treatment efficacy in patients with severe sepsis/septic shock. The usefulness of a combination of MEWS and BLA to predict ICU transfer in severe sepsis/septic shock patients is unclear. We evaluated whether use of a combination of MEWS and BLA enhances prediction of ICU transfer and mortality in hospitalized patients with severe sepsis/septic shock. METHODS: Patients with severe sepsis/septic shock who were screened or contacted by a medical emergency team between January 2012 and August 2012 were enrolled at a university-affiliated hospital with ~2,700 beds, including 28 medical ICU beds. RESULTS: One hundred patients were enrolled and the rate of ICU admittance was 38%. MEWS (7.37 vs. 4.85) and BLA concentration (5 mmol/L vs. 2.19 mmol/L) were significantly higher in patients transferred to ICU than those in patients treated in general wards. The combination of MEWS and BLA was more accurate than MEWS alone in terms of ICU transfer (C-statistics: 0.898 vs. 0.816, p = 0.019). The 28-day mortality rate was 19%. MEWS was the only factor significantly associated with 28-day mortality rate (odds ratio, 1.462; 95% confidence interval, 1.122 to 1.905; p = 0.005). CONCLUSIONS: The combination of MEWS and BLA may enhance prediction of ICU transfer in patients with severe sepsis/septic shock.
Adult ; Aged ; Biomarkers/blood ; *Decision Support Techniques ; Female ; Health Status ; *Health Status Indicators ; Hospital Bed Capacity ; Hospital Mortality ; Hospitals, University ; Humans ; *Intensive Care Units ; Lactic Acid/*blood ; Male ; Middle Aged ; *Patient Transfer ; Predictive Value of Tests ; Prognosis ; Retrospective Studies ; Risk Assessment ; Risk Factors ; Sepsis/blood/*diagnosis/mortality/therapy ; Shock, Septic/blood/*diagnosis/mortality/therapy ; Time Factors

OBJECTIVES: This study, as a preliminary one attempting to develop the Korean version of Integrated Therapy for the rehabilitation of schizophrenic patients, was designed to investigate the results of Integrated Psychological Therapy(IPT) and review the problems during the program application. METHODS: Cognitive Differentiation Scale, Problem-Solving Scale, and PANSS(Positive And Negative Syndrome Scale) were administered to schizophrenic inpatients before and after IPT incorporating cognitive differentiation, social perception, verbal communication, social skill and interpersonal problem-solving skill subprograms. The therapy groups were offered activities three times per week during the 12-week program. The control groups were attention-placebo groups for control ling factors such as group interaction and social attention, and were offered psychoeducation. RESULTS: The 12-week IPT groups, compared with the control groups, demonstrated significant improvements in the general psychopathology and the total symptoms of PANSS, but did not in cognitive functions and problem-solving skill. Significant improvements in cognitive functions were found only after cognitive differentiation subprogram and social skill subprogram had limited effects on social skills. But within 12-week IPT groups significant improvements in the positive symptoms, negative symptoms and general psychopathology were found. CONCLUSION: These findings support some effectiveness of IPT, but do not address the unique contribution by cognitive components. Applied to schizophrenic patients in this country, this modality had the problems such as differences in sociocultural and lingual background, duration of treatment, homogeneity in the level of the functioning among group members, management of emotions in activities, and the therapists' skill in the training of cognitive components. So the modification of this modality will be required.
Humans ; Inpatients ; Psychopathology ; Rehabilitation ; Schizophrenia ; Social Perception