Increased Urine Albumin-to-Creatinine Ratio-a Harbinger of Renal Dysfunction after Repair of Congenital Heart Disease?

No abstract available.

Long Term Outcomes of Left Atrial Reservoir Function in Children with a History of Kawasaki Disease

BACKGROUND: Decreased left atrial (LA) reservoir function is reported to be associated with elevated left ventricular (LV) end diastolic pressure and LV diastolic dysfunction. Echocardiographic parameters that reflect LA reservoir function include LA total emptying fraction [(maximum LA volume - minimum LA volume) / maximum LA volume], peak LA longitudinal strain (PLALS) at systole, and LA stiffness index (E/E´/PLALS). We aimed to investigate the long-term outcomes of LV diastolic function in children with a history of Kawasaki disease (KD) (KDHx group) by assessing LA reservoir function. METHODS: Retrospectively, echocardiograms performed at a mean follow-up period of 5 years after the acute phase of KD in 24 children in the KDHx group were compared to those from 20 normal control subjects. LA total emptying fraction, PLALS, LA stiffness index, LV peak longitudinal systolic strain (ε), and strain rate (SR) were evaluated with conventional echocardiographic parameters. RESULTS: The mean age at long term follow-up echocardiography in children in the KDHx group was 6.8 years. Five children (20.8%) had coronary artery lesions (CALs) in the acute stage of KD. No children showed CALs at a mean follow-up period of 5 years after the acute phase of KD. There were no significant differences in the conventional echocardiographic parameters and in LA total emptying fraction, PLALS, LA stiffness index, LV peak longitudinal systolic ε, and SR, between the children in the KDHx and control group. CONCLUSION: LV diastolic function assessed by LA reservoir function parameters at long-term follow-up in children in the KDHx group appears to be favorable.

Outcomes of Left Ventricular Function According to Treatment Response for a Patent Ductus Arteriosus in Preterm Infants

BACKGROUND: To evaluate the outcomes of left ventricular (LV) function according to treatment response for a hemodynamically significant patent ductus arteriosus (hsPDA) in preterm infants. METHODS: Echocardiograms of 21 preterm infants born at gestational age < 31 weeks obtained at term-equivalent age were retrospectively studied. Among preterm infants with a hsPDA, 9 underwent ligation after failure of pharmacological closure (ligation group) and 6 experienced successful pharmacological closure (medication group). Six preterm infants without hsPDA (no-hsPDA group) were studied as controls. LV peak longitudinal systolic strain (ε) of each infant was retrospectively obtained from echocardiograms using velocity vector imaging, along with neonatal outcomes. RESULTS: Pharmacological closures were attempted at postnatal day 2–3. In the ligation group, the median postnatal age at ligation was 20 days. In the ligation group, LV peak longitudinal systolic ε was significantly decreased at term-equivalent age compared to the other groups. Between the medication and no-hsPDA groups, LV peak longitudinal systolic ε did not differ significantly. Among the neonatal outcomes, infants who experienced necrotizing enterocolitis (NEC) showed significantly decreased LV peak longitudinal systolic ε compared to the infants who did not experience NEC . CONCLUSION: We speculate that in preterm infants with an hsPDA, in cases of medical treatment failure, early PDA ligation at less than 20 days of postnatal age would be beneficial for preserving LV systolic function.

Association of Toll-like receptor 2-positive monocytes with coronary artery lesions and treatment nonresponse in Kawasaki disease / 소아과

PURPOSE: Activation of Toll-like receptor 2 (TLR2) present on circulating monocytes in patients with Kawasaki disease (KD) can lead to the production of proinflammatory cytokines and interleukin-10 (IL-10). We aimed to determine the association of the frequency of circulating TLR2+/CD14+ monocytes (FTLR2%) with the outcomes of KD, as well as to compare FTLR2% to the usefulness of sIL-10. METHODS: The FTLR2% in patients with KD was measured by flow cytometry. Serum levels of IL-10 (sIL-10) were determined in 31 patients with KD before the initial treatment with intravenous immunoglobulin (IVIG) and in 21 febrile controls by using enzyme-linked immunosorbent assay. Patients were classified as having coronary artery lesions (CALs) based on the maximal internal diameters of the proximal right coronary artery and proximal left anterior descending coronary artery one month after the initial diagnosis. RESULTS: We found that FTLR2% greater than 92.62% predicted CALs with 80% sensitivity and 68.4% specificity, whereas FTLR2% more than 94.61% predicted IVIG resistance with 66.7% sensitivity and 71.4% specificity. Moreover, sIL-10 more than 15.52 pg/mL predicted CALs and IVIG resistance with 40% and 66.7% sensitivity, respectively, and 73.7% and 76.2% specificity, respectively. CONCLUSION: We showed that measuring FTLR2% before the initial treatment could be useful in predicting CAL development with better sensitivity than sIL-10 and with results comparable to sIL-10 results for the prediction of IVIG resistance in patients with KD. However, further studies are necessary to validate FTLR2% as a marker of prognosis and severity of KD.

Progression of Right Ventricular Systolic Dysfunction Detected by Myocardial Deformation Imaging in Asymptomatic Preterm Children

BACKGROUND: To detect progression of right ventricular (RV) systolic dysfunction (RVSD) in asymptomatic preterm children from infancy to 24-month corrected age, using velocity vector imaging (VVI). METHODS: Retrospective study comparing sequential RV longitudinal peak systolic strain (LPSS) from 24 children born at 16% at the first exam, and only 2/7 of these children had a history of moderate or severe bronchopulmonary dysplasia. CONCLUSION: In asymptomatic preterm children, routine echocardiographic screening using VVI could detect RVSD which could progress from 4–24 month corrected age.

Clinically Useful Predictors of Resistance to Intravenous Immunoglobulin and Prognosis of Coronary Artery Lesions in Patients with Incomplete Kawasaki Disease

BACKGROUND AND OBJECTIVES: The prevalence of incomplete Kawasaki disease (iKD) is progressively increasing. We aimed to retrospectively investigate the predictors of intravenous immunoglobulin (IVIG) resistance in iKD patients and compare them with those of IVIG resistance in complete Kawasaki disease (cKD) patients. We also compared the prognosis of coronary artery lesions (CALs) between the IVIG non-responders and responders in both iKD and cKD groups. SUBJECTS AND METHODS: A total of 234 cKD and 77 iKD patients were treated with IVIG between February 2009 and April 2012. Among these 311 patients, we reviewed the data of 77 iKD patients and 75 age-matched cKD patients. RESULTS: Patients with iKD having an elevated neutrophil count {percentage of segmented neutrophils (SEG%) > or =79.0} were at risk of IVIG resistance, while patients with cKD having SEG% > or =79.25 and serum total bilirubin (TB) > or =0.56 mg/dL were at risk of IVIG resistance as shown by multivariable logistic regression analysis. Fractional changes of laboratory data before and after IVIG treatment showed that Creactive protein (CRP) and N-terminal B type natriuretic peptide (NT-proBNP) levels were significantly elevated in IVIG non-responders of the iKD group, whereas erythrocyte sedimentation rate was significantly elevated in IVIG non-responders of the cKD group. Among the patients who had CALs at 10 months after the start of illness, the z scores of coronary arteries were higher in IVIG non-responders of the iKD group, when compared with IVIG non-responders of the cKD group. CONCLUSION: Elevated SEG%, changes in CRP and NT-proBNP levels may help in early detection of IVIG resistance in patients of the iKD group, which may aid in predicting the prognosis of CALs in these patients. Further studies with a larger number of patients are warranted.

Targeted Ultrasound Imaging of Apoptosis with Annexin A5 Microbubbles in Acute Doxorubicin-Induced Cardiotoxicity

BACKGROUND: The aim of this study was to assess the feasibility of targeted ultrasound imaging on apoptosis with annexin A5 microbubbles (A5MB) in acute doxorubicin-induced cardiotoxicity. METHODS: Avidinated and octafluoropropan-filled phospholipid microbubbles were conjugated with biotinylated annexin A5. To confirm the specific binding of A5MB, flow cytometry was performed with hydrogen peroxide induced apoptosis in rat aorta smooth muscle cells incubated with fluorescein-5-isothiocyanate (FITC) labeled annexin A5 and A5MB. Adult male rats were injected intraperitoneally with 5 mg/kg doxorubicin weekly for 3 weeks (n = 5). Control rats were injected with normal saline (n = 5). At 24 hours after the final treatment, triggering imaging was performed 15 min after an intravenous bolus injection of A5MB for washout of freely circulating microbubbles. After echocardiography, the heart was isolated for histological detection of apoptosis by terminal deoxynucleotidyl transferase-mediated dUTP nick-end labeling (TUNEL) assay. RESULTS: In the in vitro tests, fluorescence intensity was low for healthy cells and high for apoptotic cells when incubated with FITC-labeled annexin A5 and A5MB. Rats treated with doxorubicin showed significant contrast opacification of the myocardium on contrast echocardiography using A5MB. However, no opacification was observed in control rats. Apoptosis was confirmed by TUNEL assay in doxorubicin treated rats. CONCLUSION: Acute doxorubicin-induced cardiomyopathy based on early apoptosis can be assessed and imaged with targeted ultrasound imaging using A5MB in rats.

Assessment of Time Interval Between Onset of Ventricular Inflow and Onset of Early Diastolic Velocity by Tissue Doppler in Normal Infants

PURPOSE: Recently, time interval between onset of transmitral early inflow (E) and onset of early diastolic (Ea) velocity of the mitral annulus (TE-Ea) has been used to identify diastolic dysfunction in adults, but to date, no studies have been done in infants. The purpose of our study was to evaluate the normal values of TE-Ea in infants under 1 year of age, and to assess the influence of age, heart rate and cardiac growth on this index. METHODS: Thirty healthy children (mean+/-SD, age; 5.4+/-2.3 months) underwent echocardiography. Mitral and tricuspid inflow and tissue Doppler velocities were obtained from the leaflet tips and lateral site of the annulus, respectively, in the apical four-chamber view. The time intervals between the peak of R wave and onset of mitral and tricuspid E velocity (T(E)) and between peak of R wave and onset of Ea velocity (T(Ea)) were measured. The differences between these time intervals were calculated as T(E-Ea), which were compared with demographic and echocardiographic variables. RESULTS: Mean heart rate was 125.27+/-10.78 (bpm). Mitral T(E-Ea) was shorter than tricuspid T(E-Ea) (mean+/-SD, 24.89+/-10.33 msec versus 32.43+/-12.72 msec, P=0.016). Both mitral and tricuspid T(Ea) were significantly greater than T(E) (both: P<0.01). Age and heart rate did not show correlation with T(Ea-E). When correlated with echocardiographic variables, mitral T(E-Ea) tended to decrease as mitral ring area increased. CONCLUSION: In normal infants, T(E-Ea) values differ between both ventricles, which suggest differences in ventricular adaptation in the first year of life.

Therapeutic Efficacy of Meropenem for Treatment of Experimental Penicillin-Resistant Pneumococcal Meningitis

With the widespread emergence of antimicrobial resistance, combination regimens of ceftriaxone and vancomycin (C+V) or ceftriaxone and rifampin (C+R) are recommended for empirical treatment of pneumococcal meningitis. To evaluate the therapeutic efficacy of meropenem (M), we compared various treatment regimens in arabbit model of meningitis caused by penicillin-resistant Streptococcus pneumoniae (PRSP). Therapeutic efficacy was also evaluated by the final bacterial concentration in the cerebrospinal fluid (CSF) at 24 hr. Each group consisted of six rabbits. C+V cleared the CSF at 10 hr, but regrowth was noted in 3 rabbits at 24 hr. Meropenem monotherapy resulted in sterilization at 10 hr, but regrowth was observed in all 6 rabbits at 24 hr. M+V also resulted in sterilization at 10 hr, but regrowth was observed in 2 rabbits at 24 hr. M+V was superior to the meropenem monotherapy at 24 hr (reduction of 4.8 vs. 1.8 log10 cfu/mL, respectively; p=0.003). The therapeutic efficacy of M+V was comparable to that of C+V (reduction of 4.8 vs. 4.0 log10 cfu/mL, respectively; p=0.054). The meropenem monotherapy may not be a suitable choice for PRSP meningitis, while combination of meropenem and vancomycin could be a possible alternative in the treatment of PRSP meningitis.

Follow-up Study of Children with Anthracycline Cardiotoxicity

PURPOSE: We studied the relationship between anthracycline cumulative dose and anthracycline cardiotoxicity in childhood cancer and followed up 40 children with anthracycline cardiotoxicity. METHODS: A retrospective study was performed in 154 children who received anthracycline chemotherapy between January 1995 to December 2000. Cardiotoxicity was defined when the left ventricular fractional shortening(FS) was below 26%; it was divided into two groups, mild and severe cardiotoxicity, according to the FS. We followed up survivors with cardiotoxicity, and checked their present cardiac function by physical activity, echocardiography, electrocardiography(EKG) and chest X-ray. RESULTS: Of the 154 children treated with anthracyclines, forty(26.0%) were diagnosed as cardiotoxicity. The incidence of cardiotoxicity increased in exponential fashion with increases in the cumulative dose of anthracyclines. There was minimal increase of incidence until a dose of 300 mg/m2 after which the incidence increased rapidly. After mean 3.8+/-1.8 year follow-up of 23 survivors with cardiotoxicity, FS increased significantly. EKG and chest X-rays were not helpful for the diagnosis of cardiotoxicity because of their low sensitivity and specificity. CONCLUSION: Although convenient, non-invasive and inexpensive, EKG and chest X-rays were not helpful for the follow-up of anthracycline cardiotoxicity. Almost all survivors with anthracycline cardiotoxicity have improved in both physical activity and echocardiographic findings after discontinuation of anthracyclines.

Successful Treatment of Tachycardia-induced Cardiomyopathy with Radiofrequency Catheter Ablation

Tachycardia-induced cardiomyopathy is caused by chronic tachyarrhythmias. It is characterized by ventricular systolic dysfunction and dilatation and by clinical manifestations of heart failure. We experienced three children with tachycardia-induced cardiomyopathy. Two with ectopic atrial tachycardia and one with left ventricular tachycardia were treated successfully by radiofrequency catheter ablation. The clinical and echocardiographic features of cardiomyopathy induced by tachyarrhythmia were reversible after rhythm control. Tachycardia-induced cardiomyopathy should be considered in patient with unexplained systolic dysfunction and any form of tachyarrhythmia as a reversible etiology. Radiofrequency catheter ablation can be a safe and effective treatment in patients with tachyarrhythmia which is complicated by ventricular dysfunction.

Evaluation of Prognostic Factors in Corrected Transposition of the Great Arteries at Mid-term Follow-up

PURPOSE: The prognosis of patients with corrected transposition of the great arteries(C-TGA) is variably affected by associated intracardiac defects, systemic right ventricular function, tricuspid valve competence, and conduction disturbances. This study aims to evaluate the importance of those factors at mid-term follow-up. METHODS: Medical records of 94 patients(males 58, females 36; mean age at last follow-up, 12+/-9 years; mean follow-up duration, 9+/-6.4 years) diagnosed between January 1980 and May 2002 at Seoul National University Children's Hospital were studied retrospectively. RESULTS: Among 94 patients, operations were performed in 72 patients(classic operations in 55; double switch operations in 17). Among prognostic factors including associated intracardiac anomalies(at least moderately severe tricuspid insufficiency(TI), ventricular septal defect, pulmonary stenosis and pulmonary atresia), intracardiac operation and complete atrioventricular block, TI was the only significant factor for death(P=0.001), and in turn, Ebstein anomaly and high grade atrioventricular block predicted TI. 20-year survival without TI was 77%, but only 35% with TI(P=0.0002); excluding perioperative death, the 20-year survival rates with and without TI were 48% and 87% respectively(P= 0.008). There was no statistical difference in 20-year survival rate or association with TI between classic and double switch operation. CONCLUSION: TI was the major prognostic factor for C-TGA and was associated with Ebstein anomaly and high grade atrioventricular block at mid-term follow-up. Long-term follow-up is required to evaluate other factors, including double switch operations and associated intracardiac defects more exactly.

A Practical Protocol for Titrating "Optimal" PEEP in Acute Lung Injury: Recruitment Maneuver and PEEP Decrement

This study was conducted to evaluate the effectiveness and safety of a practical protocol for titrating positive end-expiratory pressure (PEEP) involving recruitment maneuver (RM) and decremental PEEP. Seventeen consecutive patients with acute lung injury who underwent PEEP titration were included in the analysis. After baseline ventilation, RM (continuous positive airway pressure, 35 cm H2O for 45 sec) was performed and PEEP was increased to 20 cmH2O or the highest PEEP guaranteeing the minimal tidal volume of 5 mL/kg. Then PEEP was decreased every 20 min in 2 cmH2O decrements. The "optimal" PEEP was defined as the lowest PEEP attainable without causing a significant drop (>10%) in PaO2. The "optimal PEEP" was 14.5 +/- 3.8 cmH2O. PaO2 /FI O2 ratio was 154.8 +/- 63.3 mmHg at baseline and improved to 290.0 +/- 96.4 mmHg at highest PEEP and 302.7 +/- 94.2 mmHg at "optimal PEEP", both significantly higher than baseline (p<0.05). Static compliance was significantly higher at "optimal" PEEP (27.2 +/- 10.4 mL/ cmH2O) compared to highest PEEP (22.3 +/- 7.7 mL/cmH2O) (p<0.05). Three patients experienced transient hypotension and one patient experienced atrial premature contractions. No patient had gross barotrauma. PEEP titration protocol involving RM and PEEP decrement was effective in improving oxygenation and was generally welltolerated.

Pulmonary Hypertension in the Young

No Abstract available.

Evaluation of Resting Blood Pressures, Left Ventricular Function and Mass in Young Children with Successful Coarctoplasty in Infancy

BACKGROUND AND OBJECTIVES: Cardiovascular morbidities and mortalities due to persistent hypertension, left ventricular (LV) dysfunction and increased LV mass have been reported in adolescents and adults with successful coarctoplasty. We evaluated progressive properties by measuring resting blood pressures (BP), LV functions and the masses in young children with successful coarctoplasty in infancy. SUBJECTS AND METHODS: BP in the right arm and the left ankle, LV function and mass, by the use of M-mode and pulsed wave Doppler ultrasound, were measured in 25 patients, with a mean age of 6.4+/-3 years; and a mean age at the time of repair of 0.22+/-0.24 years, and in 22 control subjects, with a mean age of 5.8+/-2.4 years. All subjects were divided into two groups based on age (more than 5 years of age and less than 5 years of age) and were analyzed independently. RESULTS: BP in the right arm and left ankle, LV function and mass showed no consistent differences between the two groups. However, as age increased, there was a tendency of wide pulse pressure (PP) in the right arm of patients contrary to that in the control subjects. The relative increase in the PP was mainly due to a relative decrease in the diastolic BP. There was a significant increase in the LV mass index of patients with increasing age (p<0.01). The LV mass index showed significant positive correlations with the systolic BP (p<0.05) and the pulse pressure (p<0.05) of the right arm of the patients. CONCLUSION: We recommend careful long-term follow-up through out adulthood to reduce morbidity and mortality, even for children who received successful coarctoplasty in early infancy.

Diagnostic Assessment of Pulmonary Atresia with Ventricular Septal Defect; Comparison of Echocardiogram with Cardiac Angiography

PURPOSE: Patients with pulmonary atresia with ventricular septal defects(PAVSD) have been a formidable surgical challenge. The source of pulmonary blood flow and vascular architecture are important in managing the surgical process. This study aimed to evaluate the usefulness of echocardiography in this process. METHODS: This study was prospectively designed to define the role of echocardiography in PAVSD. Non-invasive evaluations including echocardiography were done, catheterization/angiography was performed the next day and the results were compared with those of echocardiography. The study population consisted of 9 patients, diagnosed as PAVSD in our hospital, from Jan. 1995 to Dec. 1997. RESULTS: Pulmonary blood was supplied via ductus arteriosus, in 3 patients(group 1), and in the other 6 patients, via major aortopulmonary collateral arteries(MAPCA)(group 2). The characteristic findings of group 2 were cardiac murmur heard in the back area(3 cases), and abnormal pulmonary arborization pattern(2 cases). The echocardiographic diagnosis of source of pulmonary blood flow was correct in all cases except one case-who had had two previous shunt surgeries and additional MAPCAs. In group 2, the presence of pulmonary confluence was predicted in 3 out of 4 cases. The mean number of MAPCAs found by echocardiography was 2.3+/-1.2 and by angiography, 3.5+/-1.4. CONCLUSION: In evaluation of PAVSD patients, near complete characteristics of pulmonary blood supply were identified by echocardiogram. Using it, practitioners can make decisions for shunt surgery or the need for further evaluations, including cardiac catheterization which can be more easily executed with previous echocardiographic data.

Isolated Noncompaction of the Ventricular Myocardium : Clinical features and Outcomes

PURPOSE: Isolated noncompaction of the ventricular myocardium(INVM) can present as heart failure or arrhythmias in a child. It is a rare disorder, characterized by prominent trabecular meshwork and deep intertrabecular recesses. We still know little about the diagnosis, symptoms, and clinical outcomes of INVM. METHODS: We included in our study 6 patients who showed ventricular noncompaction on echocardiography. Patients were diagnosed as INVM were excessively prominent trabeculations with deep intertrabecular recesses were found on echocardiography. Patients who had other complex heart lesions such as pulmonary atresia with intact ventricular septum in addition to ventricular noncompaction, were excluded. RESULTS: Age at presentation ranged from 1 day 7 years, with follow up being as long as 6 years. Symptoms at initial presentation were heart murmur, paroxysmal supraventricular tachycardia, cyanosis, feeding intolerance, ventricular tachycardia, and cardiomegaly at fetal screening. Prominent trabeculations and intertrabecular recesses were observed at left ventricular apex in all six patients. All patients were alive at last follow-up. One patient showed WPW syndrome on electrocardiography. Echocardiography revealed decreased systolic function in 4 patients, and decreased systolic and diastolic function in 1 patient. One patient is currently asymptomatic. CONCLUSION: Six patients were diagnosed with INVM with various symptoms at initial presentation. Echocardiography is the most important tool in the diagnosis of INVM due to its morphological characteristics. INVM can rarely be the cause of long term systolic dysfunction, and early detection by echocardiographic screening may be beneficial.

Predictors for Benign Solitary Pulmonary Nodule in Tuberculosis-Endemic Area

BACKGROUND: Solitary pulmonary nodule (SPN) may show different pre- sentation in tuberculosis (TB)-endemic countries. The aim of this study was to identify clinical and radiological predictors favoring benign or malignant SPN in TB-endemic region. METHODS: Two hundred one SPNs in 201 consecutive Korean patients were included (< 3 cm in diameter, all confirmed by pathology or bacteriology, 93 benign and 108 malignant diseases). For clinical parameters, age, sex, smoking status and amount, and past history of pulmonary tuberculosis and diabetes mellitus were investigated retrospectively. For radiological parameters, size, location, margin characteristics, presence of calcification, pleural tag, surrounding satellite nodule, cavitation, internal low attenuation, open bronchus sign, surrounding ground-glass opacity, enhancement pattern of the SPNs and mediastinal lymph node (LN) enlargement were analyzed on chest CT scans. RESULTS: Patients with a older age (60.7+/-9.6 vs 56.2+/-13.1, p=0.008) and more than 40-pack years smoking (27.8% vs 14.0%, p=0.017) were more frequently related with malignant than benign SPN. On chest CT scans, spiculated margin, contrast enhancement more than 20 Hounsfield unit and presence of pleural tag and mediastinal LN enlargement were more frequently observed in malignant than benign SPNs. In contrast to previous studies, satellite lesions (21.5% vs 1.9%, p < 0.001) and cavitation (20.4% vs 5.6%, p=0.001) were more frequently seen in benign than malignant SPN. Positive predictive values of benignity were 90.9% and 76.0%, respectively, when satellite lesions and cavitation were found in cases of SPN. CONCLUSION: Satellite lesions and cavitation on chest CT scan could be useful predictors for benign SPN in TB-endemic areas.

A Case of Hypertrophied Left Ventricle After Successful Coarctoplasty

Coarctation of aorta(CoA) has been recognized to be cured by corrective operation, so many clinicians have discontinued follow-up early. However, high incidences of cardiovascular morbidity and mortality, of which causes have been known as persistent resting hypertension and exercise-induced hypertension after corrective operation, have been reported during long-term follow-up. And left ventricular mass increase associated with persistent resting hypertension and exercise-induced hypertension has been known as an independent risk factor of cardiovascular disease. So, even for successfully operated CoA patients, increased left ventricular mass as well as resting hypertension and exercise-induced hypertension must be detected early and normalized through persistent observation to adulthood.

The Clinical Characteristics of Diffuse Alveolar Hemorrhage : A Retrospective Study of 21cases / 결핵

BACKGROUND: Diffuse alveolar hemorrhage (DAH) is rare but often fatal. To determine the clinical manifestations of DAH, its etiology, clinical course and prognosis were studied. METHODS: A retrospective analysis was performed in 21 patients that were diagnosed as DAH. Diagnosis of DAH was based on the presence of the "classical triad" of hemoptysis, anemia, and rapidly progressive infiltrates on chest X-ray and a finding of bronchoalveloar lavage or lung biopsy. RESULTS: Thirteen patients (61.9%) had collagen vascular diseases (CVDs) as underlying disease and 10 patients had systemic lupus erythematosus. Females were more prevalent in CVD than in non-collagen vascular disease (NCVD). Otherwise, there were no significant differences between the two groups in terms of clinical manifestations. Dyspnea (95.2%), cough (76.2%), hemoptysis (61.9%), and fever (33.0%) were frequent symptoms. The initial creatinine level was higher in CVD than in NCVD (3.27±3.15 mg/dl vs. 1.19±0.94 md/dl, p=0.030). The corresponding drop in hemoglobin level was 2.69±1.26 g/dl. Maximal drop in hemoglobin preceded the progression of infiltrates on the chest radiograph by 1.38±4.22 days. The mortality rate was higher in the patients with NCVD than in those with CVD (50.0% vs. 23.1%). CONCLUSION: The DAH can occur not only in patients with CVD but also in those with NCVD. Higher creatinine level CVD in patients is associated with renal involvement in conjunction with DAH. The maximal drop in hemoglobin preceeding the progression of infiltrates on the chest radiograph suggests that the drop in hemoglobin is important for diagnosing DAH.