No abstract available.
Chronic Disease* ; Risk Factors*

Churg-Strauss syndrome(CSS) is a systemic vascular disorder that has an unknown cause with multiorgan involvement and diverse presentations. The three main histologically distinct phases were necrotizing vasculitis, tissue eosinophilia and extravascular granulomas. A diagnosis of CSS can be made on four or more of the following six criteria : 1) asthma, 2) peripheral eosinophilia >10% on the differential leukocyte count, 3) mononeuropathy (including multiple) or polyneuropathy, 4) paranasal sinus abnormalities, 5) nonfixed pulmonary infiltrates, and 6) biopsy evidence of extravascular eosinophils in the skin, the nerves, or the lungs. CSS has a good prognosis with systemic steroid therapy. The 5 year survival is approximately 70 %. We experienced a 66-year-old man who presented with cough, sputum, edema and numbness in both legs. He presented with all of the 6 CSS criteria. A nerve and muscle biopsy confirmed the diagnosis. Here, we report this case with a review of the relevant literatures.
Male ; Humans ; Biopsy

Churg-Strauss syndrome(CSS) is a systemic vascular disorder that has an unknown cause with multiorgan involvement and diverse presentations. The three main histologically distinct phases were necrotizing vasculitis, tissue eosinophilia and extravascular granulomas. A diagnosis of CSS can be made on four or more of the following six criteria : 1) asthma, 2) peripheral eosinophilia >10% on the differential leukocyte count, 3) mononeuropathy (including multiple) or polyneuropathy, 4) paranasal sinus abnormalities, 5) nonfixed pulmonary infiltrates, and 6) biopsy evidence of extravascular eosinophils in the skin, the nerves, or the lungs. CSS has a good prognosis with systemic steroid therapy. The 5 year survival is approximately 70 %. We experienced a 66-year-old man who presented with cough, sputum, edema and numbness in both legs. He presented with all of the 6 CSS criteria. A nerve and muscle biopsy confirmed the diagnosis. Here, we report this case with a review of the relevant literatures.
Male ; Humans ; Biopsy

PURPOSE: Dyspnea is the cardinal symptom of asthma, but it is difficult to quantify clinically. Although modified Borg (mBorg) scale has been successfully used in adult, but there has been some difficulties to apply in children. Recently, Pediatric Dyspnea Scale (PDS) was adequately designed and has been widely used. The aim of this study is to compare 2 evaluating scales of dyspnea provoked by induced-bronchoconstriction in childhood asthma. METHODS: Seventy-three clinically suspected children with asthma were enrolled in this study. Each ‘fractional exhaled nitric oxide (FeNO)’ was documented. Forced expiratory volume in 1 second (FEV₁), mBorg score and PDS score were recorded during methacholine provocation test. RESULTS: Mapping using canonical plot demonstrated global similarity between 2 scales with some distinctive features. Whereas mBorg score showed more diverse categories in low level of dyspnea, PDS score did in medium level of it. A distribution of dyspnea perception score at a 20% decrease in FEV₁ relative to baseline (PS₂₀), a perception score of dyspnea at 20% fall in FEV1 of 2 scales represented similar wide, biphasic feature. Statistical relevance was verified with spearman correlation (R(s)=0.903, P<0.001) and Bland-Altman analysis. PS₂₀ of both scores and FeNO had no statistical relationship. While relationship between PS20 by mBorg score and the concentration of methacholine at 20% fall in FEV₁ (PC₂₀) was not significant (R(s)=0.224, P=0.154), that between PS₂₀ by PDS and PC₂₀ was weak positive (R(s)=0.29, P=0.063). CONCLUSION: PDS had similar pattern to assess the dyspnea with the mBorg scale suggesting adequacy of PDS in evaluating pediatric clinical asthma. We expect these scales to help clinical practice in complementary ways.
Adult ; Asthma* ; Bronchial Provocation Tests ; Bronchoconstriction* ; Child* ; Dyspnea* ; Forced Expiratory Volume ; Humans ; Methacholine Chloride ; Nitric Oxide ; Weights and Measures

BACKGROUND: The t (8;21) (q22;q22), which produces the fusion gene AML1/ETO, is associated with relatively good prognosis and, in particular, with a good response to cytosine arabinoside. Analysis of t (8;21) positive leukemic blasts has shown characteristic morphological and immunological features. We performed this study to investigate the incidence of AML1/ETO rearrangement in adult AML, especially in M2 subtype, to make a comparison of morphologic, immunophenotypic and clinical characteristics between AML1/ETO rearrangement positive and negative group in patient with AML and to analyze the correlation with other biological parameters. METHODS: From May 1995 to Sep. 2000, fifty-nine patients with AML including twenty-nine AML-M2 were studied. RNAs were extracted from leukemic cells and reverse transcriptase mediated polymerase chain reaction (RT-PCR) for AML1/ETO fusion transcript was done. Chromosome study, immunophenotypic, and clinical characteristics were analysed and statistical analysis was done. RESULTS: The male to female ratio was 32:27 in AML and 17:12 in AML-M2. The median age was 43 years (range 14-86) in AML and 43 years (range 14-77) in AML-M2. The incidence of AML1/ETO fusion transcripts was 22.0% in AML and 44.8% in AML-M2. The morphologic finding of bone marrow in AML-M2 showed higher incidence of Auer rods, large blast with prominent golgi and abnormal granules in AML1/ETO positive patients. There was no significant difference of immunophenotype. AML patients with AML1/ETO rearrangement had a tendency of higher complete remission rate (81.8% vs 56.6%, p=0.13). The overall survival (median 82.2 weeks vs 34.4 weeks, p=0.02) and progression free survival (median 50.9 weeks vs 20.4 weeks, p=0.02) of AML1/ETO positive group were longer than those of negative group in AML. AML-M2 patients with AML1/ETO rearrangement had also a tendency of longer overall survival and progression free survival, although there was no significant difference between both group (median OS 82.4 weeks vs 15.6 weeks, p=0.07, median PFS 50.9 weeks vs 16.0 weeks, p=0.09). CONCLUSION: Our data suggest that AML1/ETO rearrangement is detected frequently in AML, especially M2, and is a favorable prognostic factor. Thus, molecular diagnostic approaches should be used routinely to identify patients with this genetic subtype of AML.
Adult ; Bone Marrow ; Cytarabine ; Disease-Free Survival ; Female ; Humans ; Incidence ; Leukemia, Myeloid, Acute* ; Male ; Pathology, Molecular ; Polymerase Chain Reaction ; Prognosis ; RNA ; RNA-Directed DNA Polymerase

PURPOSE: The incidence of inflammatory bowel disease (IBD) is rapidly increasing, and several reports have described the renal complications of IBD. We sought to evaluate the clinical manifestations of renal complications in children with IBD in order to enable early detection and prompt treatment of the complications. METHODS: We retrospectively reviewed the medical records of 456 children and adolescents aged < 20 years who had been diagnosed with IBD since 2000. We analyzed patient age, sex, medication use, IBD disease activity, and clinical manifestations of renal symptoms. RESULTS: Our study comprising 456 children with IBD included 299 boys (65.6%) and 157 girls (34.4%). The study included 346 children with Crohn disease and 110 children with ulcerative colitis. The incidence of kidney-related symptoms was 14.7%, which was significantly higher than that in normal children. We observed 26 children (38.8%) with isolated hematuria, 30 children (44.8%) with isolated proteinuria, and 11 children (16.4%) with hematuria and concomitant proteinuria. A renal biopsy was performed in 7 children. Histopathological examination revealed immunoglobulin A nephropathy in 5 children (71.4%). All children presented with mild disease and well-controlled disease activity of IBD. CONCLUSION: Children with IBD are more likely to show kidney-related symptoms than healthy children and adolescents are. Therefore, regular screening of urine and evaluation of renal function in such children are necessary for early detection of renal complications.
Adolescent* ; Biopsy ; Child* ; Colitis, Ulcerative ; Crohn Disease ; Female ; Glomerulonephritis, IGA ; Hematuria ; Humans ; Incidence ; Inflammatory Bowel Diseases* ; Kidney ; Mass Screening ; Medical Records ; Proteinuria ; Retrospective Studies