• Tuberculosis and Respiratory Diseases
• /
• v.40 no.6
• /
• pp.730-735
• /
• 1993

BOOP is a clinopathologic entity consisting of a flu-like illness, late inspiratory crackles, and pathologically granulation tissue plugs within lumens of small airways sometimes with complete obstruction of small airways and granulation tissue extending into alveolar ducts and alveoli with a variable degree of interstitial infiltration of mononuclear cells and accumulation of foamy macrophages in alveolar spaces in a patch distribution, and preservation of background architecture of the lung. It has patch infiltrates roentgenographically, and restrictive ventilatory defect pysiologically such as decreased vital capacity. and diffusing capacity. The BOOP has been observed in the context of collagen vascular disease, and other autoimmune disease secondary to treatment with penicillamine, bleomycin, acebutolol and amiodarone, following the inhalation of toxic fumes, after several infections including measles, pertussis and influenza and idiopathic. Clinically, response to coricosteroid therapy is good and relapse dose not occur if sufficient theraphy is good. A flu-like illness occurs in one third, cough in one third, cough with dyspnea in the remaining patients. Hemoptysis are rare. The physical examination reveales dry crackles in the majority of the patients with BOOP but rarely associated with wheezing. The duration of illness is less than 2 months in 75% of patients. With a brief review of literature, we report a case of the BOOP which is good response to steroid, but frequent relapse and assoicated with wheezing.

• Tuberculosis and Respiratory Diseases
• /
• v.39 no.6
• /
• pp.536-541
• /
• 1992

Bronchiolitis obliterans organizing pneumonia (BOOP) is a type of diffuse interstitial lung disease that primarily affects the small conducting airways and characterized by the presence of granulation tissue plugs within the lumen of small airways often extending into alveolar ducts. It is associated with a number of different causes, including a variety of infections, fume exposures, drugs, collagen diseases and idiopathic. Recently we have experienced one patient with idiopathic BOOP. The patient was a 58 year old man presented with 2 months' history of dry cough and exertional dyspnea. The phyical examination showed inspiratory crackles at both lower lung field. Chest X-ray showed bilateral multiple patchy alveolar density. Pulmonary function studies showed a moderate degree of restrictive lung disease. Open lung biopsy carried out and revealed findings characteristic of BOOP. There was a dramatic response clinically and radiologically to high dose predinisolone therapy. Chest X-ray and pulmonary function test under-taken one year later showed marked improvement. New lesion on chest PA was developed during the period of tapering of prednisolone dose, but it was soon disappeared after increasing of prednisolone dose. One year later, he is well without steroid therapy.

• Childhood Kidney Diseases
• /
• v.14 no.1
• /
• pp.51-61
• /
• 2010

Purpose : Previous studies have suggested that Chemokine (C-C motif) ligand-2 (CCL-2; also known as MCP-1) and CCL-5 (also known as RANTES) are possibly associated with the pathogenesis of various inflammatory and non-inflammatory renal diseases. The present study was conducted to investigate association of polymorphisms of CCL-2 and CCL-5 genes with childhood IgA nephropathy (IgAN). Methods : The authors analyzed six single nucleotide polymorphisms (SNPs) of CCL-2 and CCL-5 in 196 pediatric IgAN patients and in 285 healthy controls. We compared variations in SNPs between two several sets of IgAN subgroups, allocated by presence of proteinuria (>4 mg/$m^2$/hour), podocyte foot process effacement, and pathologically advanced disease markers, such as interstitial fibrosis, tubular atrophy, or global sclerosis. Results : Genotypic data of IgAN patients and controls showed no significant SNP frequency difference in both of of CCL-2 and CCL-5. Even though two linkage disequilibrium blocks were formed, there was no significance in the haplotype analysis. In the patient subgroup analysis, no SNP of CCL-2 and CCL-5 was found to be associated with the presence of proteinuria, podocyte foot process effacement, and pathologically advanced disease markers. Conclusion : Our data indicate that no association exists between CCL-2 and CCL-5 SNPs and childhood IgAN susceptibility, and presence of proteinuria, podocyte foot process effacement, and pathologic progression of IgAN.

• Korean Journal of Radiology
• /
• v.24 no.11
• /
• pp.1081-1092
• /
• 2023

Objective: To investigate the incidence of microvascular myocardial ischemia in diabetic patients without obstructive coronary artery disease (CAD) and its relationship with angina. Materials and Methods: Diabetic patients and an intermediate-to-high pretest probability of CAD were prospectively enrolled. Non-diabetic patients but with an intermediate-to-high pretest probability of CAD were retrospectively included as controls. The patients underwent dynamic computed tomography-myocardial perfusion imaging (CT-MPI) and coronary computed tomography angiography (CCTA) to quantify coronary stenosis, myocardial blood flow (MBF), and extracellular volume (ECV). The proportion of patients with microvascular myocardial ischemia, defined as any myocardial segment with a mean MBF ≤ of 100 mL/min/100 mL, in patients without obstructive CAD (Coronary Artery Disease-Reporting and Data System [CAD-RADS] grade 0-2 on CCTA) was determined. Various quantitative parameters of the patients with and without diabetes without obstructive CAD were compared. Multivariable analysis was used to determine the association between microvascular myocardial ischemia and angina symptoms in diabetic patients without obstructive CAD. Results: One hundred and fifty-two diabetic patients (mean age: 59.7 ± 10.7; 77 males) and 266 non-diabetic patients (62.0 ± 12.3; 167 males) were enrolled; CCTA revealed 113 and 155 patients without obstructive CAD, respectively. For patients without obstructive CAD, the mean global MBF was significantly lower for those with diabetes than for those without (152.8 mL/min/100 mL vs. 170.4 mL/min/100 mL, P < 0.001). The mean ECV was significantly higher for diabetic patients (27.2% vs. 25.8%, P = 0.009). Among the patients without obstructive CAD, the incidence of microvascular myocardial ischemia (36.3% [41/113] vs. 10.3% [16/155], P < 0.001) and interstitial fibrosis (69.9% [79/113] vs. 33.3% [8/24], P = 0.001) were significantly higher in diabetic patients than in the controls. The presence of microvascular myocardial ischemia was independently associated with angina symptoms (adjusted odds ratio = 3.439, P = 0.037) in diabetic patients but without obstructive CAD. Conclusion: Dynamic CT-MPI + CCTA revealed a high incidence of microvascular myocardial ischemia in diabetic patients without obstructive CAD. Microvascular myocardial ischemia is strongly associated with angina.

• Childhood Kidney Diseases
• /
• v.13 no.1
• /
• pp.63-74
• /
• 2009

Purpose : The current nationally representative data on inpatient care are important to make the of the national public health policy because distributions and the prevalence of diseases among children and adolescents represent the socioeconomic status of the society. The prevalence of chronic disease is increasing now in Korea as the socioeconomic condition is improving. We analyzed a part of genitourinary tract disease of the cross-sectional hospital discharge survey data in Korea collected recently to delineate the trend of genitourinary tract diseases. Methods : Korean nationwide hospital discharge survey for pediatric inpatients in the period from 2004 to 2006 was analyzed. Diagnoses in the data were coded using ICD-10 classification. Totally 826,896 cases were collected from the 85 training hospitals. Selected data of genitourinary tract diseases (belonging to N00-N99 by ICD-10) among 826,896 cases of final inpatients data were analyzed for this study. Results : Among total patients of 826,896, diseases of the genitourinary system accounted for 4.1%. and four diagnostic categories accounted for 92.8%. These were other diseases of the urinary system (N30-39), 45.8%, disease of male genital organs (N40-51),19.1%, glomerular diseases (N00-08), 17.3%, renal tubulo-interstitial diseases (N10-16), 10.6%, respectively. Conclusion : Genitourinary tract disease in pediatric inpatient shows decreasing tendency but the prevalence of chronic diseases is increasing in Korea as the socioeconomic condition is improving. For further comprehensive analysis, regular and organized nationwide survey should be performed. Development of a new data collecting system will improve the performance of such nationwide survey.

• Clinical and Experimental Pediatrics
• /
• v.49 no.12
• /
• pp.1348-1353
• /
• 2006

Purpose : The reticuloendothelial system is composed of sinusoidal capillaries, through which even large protein molecules are freely movable between plasma and interstitial space, including the lymphatic system. Therefore, high-dose intravenous immunoglobulin (IVIG) would cause a redistribution of proteins between two compartments. To investigate this hypothesis, we measured plasma protein and lipid levels in patients with Kawasaki disease before and after high-dose IVIG treatment. Methods : Thirty four children with Kawasaki disease who had complete responses to high-dose IVIG treatment (1 g/kg/day for two consecutive days), were analyzed. Before and after the administration of IVIG, serum analyses were performed for such parameters as total protein, albumin, ${\gamma}$-globulins (IgG, IgM, IgA), ${\alpha}1-$, ${\alpha}2-$, and ${\beta}-$ globulin fractions, and lipid profiles (total cholesterol, HDL-cholesterol, LDL-cholesterol and triglyceride). Results : The levels of ${\gamma}$-globulins including IgG, IgM, IgA were significantly increased, and IgG was increased by $1,779{\pm}304mg/dL$ after two-dose of IVIG infusion. The levels of albumin, ${\alpha}1-$, ${\alpha}2-$, and ${\beta}$ globulin fractions were significantly decreased by 18 percent, 24 percent, 19 percent and 12 percent, respectively. HDL-cholesterol level was significantly decreased by 20 percent, while LDL-cholesterol and triglyceride levels were significantly increased by 21 percent and 50 percent, respectively. The total cholesterol level was not changed. Conclusion : High-dose IVIG treatment decreased the levels of a variety of proteins except immunoglobulins, and the increase of IgG after IVIG treatment was lower than expected. Our results suggest that a part of infused IVIG and plasma proteins, including etiologic proteins for Kawasaki disease, may be distributed to the extravascular compartments. The rapid improvement of symptoms induced by IVIG in Kawasaki disease might be explained by this mode of action of IVIG.

• Tuberculosis and Respiratory Diseases
• /
• v.42 no.4
• /
• pp.569-583
• /
• 1995

Background: The expression of the adhesion molecules on the cell surface is important in the movement of cells and the modulation of immune response. DILD starts as an alveolitis and progresses to pulmonary fibrosis. So adhesion molecules in these patients is expected to be increased. There are several reports about adhesion molecules in DILD in terms of the percentage of positive cells in immuno-stain, in which the interpretation is subjective and the data were variable. Methods: So we measured the relative median fluorescence intensity(RMFI) which is the ratio of the FI emitted by bound primary monoclonal antibody to FI emitted by isotypic control antibody of the cells in BALF of 28 patients with DILD(IPF:10, collagen disease:7, sarcoidosis:9, hypersensitivity pneumonitis:2) and 9 healthy control. Results: RMFI of the ICAM-1 on AM($3.30{\pm}1.16$) and lymphocyte($5.39{\pm}.70$) of DILD were increased significantly than normal control($0.93{\pm}0.18$, $1.06{\pm}0.21$, respectively, p=0.001, P=0.003). RMFI of the CD18 on lymphocyte was also higher($24.9{\pm}14.9$) than normal($4.59{\pm}3.77$, p=0.0023). And there was a correlation between RMFI of ICAM on AM and the % of AM(r=-0.66, p=0.0001) and lymphocyte(r=0.447, p=0.0116) in BALF. Also RMFI of ICAM on lymphocyte had a significant (r=0.593, p=0.075) correlation with the % of IL-2R(+) lymphocyte in BALF. The soluble ICAM(sICAM) in serum was also significantly elevated in DILD($499.7{\pm}222.2\;ng/ml$) compred to normal($199.0{\pm}38.9$) (p=0.00097) and sICAM in BAL fluid was also significantly higher than normal control group($41.8{\pm}23.0\;ng/ml$ vs $20.1{\pm}13.6\;ng/ml$). There was a Significant correlation between sICAM level in serum and the expression of ICAM-l on AM(r=0.554, p=0.0259).Conclusion: These data suggest that in DILD the expression of adhesion molecules is increased in the AM and BAL lymphocytes with elevated serum sICAM, and these parameter may be useful in determining disease activity.

• Tuberculosis and Respiratory Diseases
• /
• v.43 no.4
• /
• pp.613-622
• /
• 1996

Constrictive bronchiolitis, one of small airway diseases, is very rare and occupies one of the two arms of bronchiolitis obliterans together with proliferative bronchiolitis. Proliferative bronchiolitis, presenting the prototype with bronchiolitis obliterans with organizing pneumonia(BOOP), can be easily taken into diagnostic consideration in terms of relatively rapid clinical course and radiologic presentation as if atypical pneumonia with interstitial and alveolar infiltrations. Meanwhile constrictive bronchiolitis is not only very Tare but also easily overlooked as chronic obstructive pulmonary diseases such as emphysema, because it usually shows normal chest radiographic finding and obstructive pattern in pulmonary function test. In the aspects of the response to treatment, proliferative bronchiolitis showed dramatic response to the corticosteroid while constrictive bronchiolitis is intractable, which is easily explained on the basis of the pathologic characteristics of cicartrical replacement of bronchiolar walls. The bronchiolitis, both proliferative and constrictive, can be associated with diverse conditions such as inhalational injury, postinfectious process, drug of chemical induced reactions, connective tissue diseases, and organ trasplantation. And there is idiopathic type which has no associated condition. There is one explanation that both types of bronchiolitis lie on the same disease spectrum because the different disease pattern can be evoked from the same etiology. In contrast, another explanation is suggested that both types of bronchiolitis are one of nonspecific tissue reaction rather than a disease specific histologic finding because the various types of causes can provoke the same histologic findings. These dilemma remains for further investigation. With literature investigation, the authors report a case of constrictive bronchiolitis proven by open lung biopsy in 47 year old female who was diagnosed as non-Hodgkin's lymphoma and simultaneously had relatively rapid progression of airflow obstruction and showed negative radiographic finding without the rise factors for the development of chronic obstructive lung disease. We consider it as idiopathic because we could not find any relationship between constrictive bronchiolitis and non-Hodgkin's lymphoma on the literature search and it requires further investigation.

• Clinical and Experimental Pediatrics
• /
• v.46 no.12
• /
• pp.1230-1234
• /
• 2003

Purpose : The aim of this study is to evaluate the value of lung biopsies for the management of children with lung disease. Methods : We retrospectively reviewed 19 lung biopsies done at Asan Medical Center, Seoul between 1993 and 2001. Data gathered included demographic information, underlying conditions, diagnosis before biopsy, final diagnosis, change in therapy, morbidity and mortality. Results : Nineteen patients underwent lung biopsy. Among them, 13 patients were male and six patients were female; the median age was 3.6 years(0.8 to 8.6 years). Twelve patients underwent open lung biopsies and seven patients had thoracoscopic biopsies. The overall diagnosis rate was 95 %. The most common diagnosis was interstitial lung disease(12 patients, 64%) and infection was detected in four patients(21%). The biopsy-proven bronchiolitis obliterance was confirmed in two of seven patients suspected by CT findings. Specific treatment was changed after biopsy in 16 patients (85%). The morbidity & overall mortality rates of the patients were 5%(one patient) and 21%(four patients) respectively. Only one complication was seen : empyema. The causes of death were acute respiratory distress syndrome(one patient), respiratory failure(two patients), and septicemia(one patient). Conclusion : The lung biopsy is a safe procedure and it contributes to more accurate diagnosis and proper management of pediatric lung diseases. We recommend lung biopsies should be considered more positively in the diagnosis of pediatric lung diseases.

• Childhood Kidney Diseases
• /
• v.17 no.2
• /
• pp.92-100
• /
• 2013

Purpose: To investigate the clinicopathologic effects of cyclosporine A (CsA) in children with diseases characterized by mesangial immunoglobulin A deposits such as immunoglobulin A nephropathy (IgAN) and Henoch-Sch$\ddot{o}$nlein purpura nephritis (HSPN). Methods: We retrospectively reviewed the clinicopathologic outcomes of 54 children (IgAN, 36; HSPN, 18) treated with CsA. The starting dose of CsA was 5 mg/kg per day, and it was administered in 2 divided doses. The degree of proteinuria and pathologic changes in renal biopsies were evaluated before and after CsA treatment. Results: The mean protein to creatinine ratio decreased from $3.7{\pm}1.5$ to $0.6{\pm}0.4$(P <0.001), and 32 (59.2%) children achieved complete remission of proteinuria after 1-year CsA treatment. Among the 54 children, 24 maintained normal renal function and 25 exhibited microscopic hematuria or proteinuria at the end of CsA treatment. In the HSPN group, 3 children whose initial biopsies indicated class IIIb disease showed class II disease on follow-up, and the follow-up biopsies of 2 children who had class II disease indicated the same class II disease. In the IgAN group, cortical tubular atrophy occurred in 1 child, and no child with IgAN had cortical interstitial fibrosis or tubular atrophy after 1-year CsA treatment. No significant complications were found in the children treated with CsA. Conclusion: Our findings indicate that CsA treatment is effective and beneficial in reducing massive proteinuria and preventing progression to end-stage renal failure in children with glomerular diseases characterized by IgA deposits, such as IgAN and HSPN, within 1 year of treatment.