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

Salicornia herbacea L. is one of the halophytes that can grow in salt marshes, or salt fields along the seashores in Korea. The objective of this study is to investigate the mechanism by which Salicornia Polysaccharide, (SPS) activates macrophages. To analyze macrophage activation and iNOS gene expression, we performed nitrite generation assay, immunohistochemistry, and reverse transcriptase-polymerase chain reaction. A polysaccharide isolated from the Salicornia herbacea L. significantly induces nitric oxide (NO). Immunohistochemical staining of inducible NO synthase (iNOS) showed that the increase of NO was due to the induction of iNOS production. RT-PCR analysis showed that SPS produced significant induction of iNOS gene expression. Immunohistochemical staining of p65 showed that SPS produced strong induction of NF-kappa B/Rel nuclear translocation. Electrophoretic mobility shift assay further confirmed the activation of NF-kappaB/Rel by SPS. In conclusion, we demonstrate that SPS stimulates the macrophages to express iNOS gene via the activation of NF-kappa B/Rel.
Chenopodiaceae* ; Electrophoretic Mobility Shift Assay ; Gene Expression ; Immunohistochemistry ; Korea ; Macrophage Activation ; Macrophages* ; Nitric Oxide ; Nitric Oxide Synthase ; Salt-Tolerant Plants ; Wetlands

No abstract available.
Immunization, Passive ; Immunoglobulins ; Lambert-Eaton Myasthenic Syndrome

BACKGROUND: The pathogenesis of ADPKD is still unknown but the proliferation of cystic epithelia and the fluid secretion to cystic lumen are thought to be important. Cytokines play a pivotal role in growth, differentiation, and apoptosis in general, but there were few reports about the cytokine profile in ADPKD cysts. METHODS: In this study, we measured cytokine content in aerobic culture-negative cystic fluids from 23 patients with symptomatic normal to end-stage (n=3) ADPKD in order to elucidate the possibility that cytokines are related to the development and progression of disease. Enzyme-linked immunosorbent assays (ELISAs) were used to detect IL-1beta, IL-2, IL-4, IL-6, IL-10, and IFN-gamma with commercial kits. RESULTS: Male to female ratio was 6 : 17 and the median age at examination was 52 years (range 36 to 78). IL-1beta was present in 18 of 23[78%] (11 to 173 pg/mL), IL-2 in 18 of 23[78%] (5 to 159 pg/ mL), IL-4 in 9 0f 23[39%] (8 to 156 pg/mL) and IL-6 in 10 of 23[43%] (16 to 1498 pg/mL). IL-10, and IFN-gamma were not detected. IL-1beta concentrations correlated directly with those of IL-2 (r=0.7671). IL- 6 levels in patients with azotemia (n=7) [288.4+/-26.2 (mean+/-S.D.)] were significantly higher than those of normal renal function group (98.3+/-413.9)(p<0.01). Such difference was not found in other cytokines. Cytokine concentrations did not correlate with sodium concentrations, nor with cystic fluid osmolality, indicating that differences in concentrations among fluids could not be explained by differences in water content. And, there was no significant correlation between the intracystic concentrations of these cytokines and the corresponding cyst diameters. CONCLUSION: These data identify proinflammatory cytokines as possible mediators to the morbidity of ADPKD. Especially, IL-6 levels of cystic fluid were elevated in the azotemic ADPKD patients.
Apoptosis ; Azotemia ; Cytokines ; Enzyme-Linked Immunosorbent Assay ; Female ; Humans ; Interleukin-10 ; Interleukin-2 ; Interleukin-4 ; Interleukin-6 ; Male ; Osmolar Concentration ; Polycystic Kidney, Autosomal Dominant* ; Sodium ; Water

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

PURPOSE: To discribe the radiologic findings of idiopathic portal hypertension and to find the points of differentiation between idiopathic portal hypertension and liver cirrhosis. MATERIALS AND METHODS: Four portogramsin five patients who for four years had suffered from pathologically confirmed idiopathic portal hypertension were retrospectively analyzed and compared with a portogram obtained from a cntrol subject with liver cirrhosis. RESULTS: Portographic findings of idiopathic portal hypertension were paucity of medium-sized portal branches, irregular and obtuse-angled division of peripheral branches, abrupt interruption and an avascular area beneath the liver margin. CONCLUSION: A portogram of idiopathic portal hypertension may be useful in differentiating this andliver cirrhosis.
Fibrosis ; Humans ; Hypertension, Portal* ; Liver Cirrhosis ; Portography

OBJECTIVES: Polymethylmethacrylate(PMMA) is often used to reconstruct the spine after total corpectomy, but the exothermic curing of liquid PMMA poses a risk of thermal injury to the spinal cord. The purposes of this study are to analyze the heat blocking effect of pre-polymerized PMMA sheet in the corpectomy model and to establish the minimal thickness of PMMA sheet to protect the spinal cord from the thermal injury during PMMA cementation of vertebral body. MATERIALS AND METHODS: An experimental fixture was fabricated with dimensions similar to those of a T12 corpectomy defect. Sixty milliliters of liquid PMMA were poured into the fixture, and temperature recordings were obtained at the center of the curing PMMA mass and on the undersurface(representing the spinal cord surface) of a pre-polymerized PMMA sheet of variable thickness(group 1:0mm, group 2:5mm, or group 3:8mm). Six replicates were tested for each barrier thickness group. RESULTS: Consistent temperatures(106.8+/-3.9degreesC) at center of the curing PMMA mass in eighteen experiments confirmed the reproducibility of the experimental fixture. Peak temperatures on the spinal cord surface were 47.3degreesC in group 2, and 43.3degreesC in group 3, compared with 60.0degreesC in group 1(p<0.00005). So pre-polymerized PMMA provided statistically significant protection from heat transfer. The difference of peak temperature between theoretical and experimental value was less than 1%, while the predicted time was within 35% of experimental values. The data from the theoretical model indicate that a 10mm barrier of PMMA should protect the spinal cord from temperatures greater than 39degreesC(the threshold for thermal injury in the spinal cord). CONCLUSION: These results suggest that pre-polymerized PMMA sheet of 10mm thickness may protect the spinal cord from the thermal injury during PMMA reconstruction of vertebral body.
Cementation ; Hot Temperature ; Models, Theoretical ; Polymethyl Methacrylate* ; Spinal Cord* ; Spine

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

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