To determine normal values for Doppler parameters of left ventricular function, ascending aortic blood flow velocity was measured by pulsed wave Doppler echocardiography in 63 healthy children with body surface area (BSA) <1 m(2) (age <10 yr). Peak velocity was independent of sex, but increased with body size. Mean acceleration was related to peak velocity (r=0.75, p<0.0001). Both stroke distance and ejection time had strong negative correlations with heart rate and positive correlations with BSA, suggesting that these parameters should be evaluated in relation to heart rate and body size. Mean intra- and interobserver variability for peak velocity, ejection time, stroke and minute distance ranged from 3 to 7%, whereas variability for acceleration time was 9 to 13%. These data may be used as reference values for the assessment of hemodynamic states in young children with cardiac disease.
Age Factors ; Aorta/*physiology ; Blood Flow Velocity ; Body Constitution ; Child ; Child, Preschool ; Echocardiography, Doppler/*standards/statistics & numerical data ; Female ; Heart Rate ; Human ; Infant ; Infant, Newborn ; Male ; Observer Variation ; Reference Values ; Stroke Volume

PURPOSE: The purpose of this study was to determine whether N-terminal fragment of B-type natriuretic peptide(NT-proBNP) may be used to differentiate acute Kawasaki disease(KD) from other clinically similar diseases. METHODS: Using electrochemiluminescence immunoassay, NT-proBNP concentrations were measured in the acute phase within 10 days after the onset of KD(n=58) and in the convalescent phase, 60 to 81 days after the onset(n=51), and also in patients with acute febrile disease as a control(n=34). Echocardiography was performed to detect pericardial effusion(PE) and coronary artery lesions(CAL), and to measure the left ventricular dimension at diastole(LVIDd) and ejection fraction(LVEF). The cutoff value of NT-proBNP for separating KD from other diseases was determined. RESULTS: NT-proBNP concentration in the acute phases of KD was significantly higher than that in the control group(1,501.6+/-2,132.6 vs. 139.0+/-88.8 pg/mL, P<0.0001). In KD patients, NT-proBNP was elevated in the acute phase and was lowered in the convalescent phase(1,466.0+/-2,173.2 vs. 117.5+/-95.5 pg/mL, P<0.0001). The cutoff value of 260 pg/mL discriminated KD patients from other patients, with a sensitivity of 93 percent and a specificity of 88 percent. The NT-proBNP was higher in patients with PE(n=17) compared with those without PE(n=41)(1,784.2+/-1,903.1 vs. 1,384.4+/-2,232.6 pg/mL, P=0.52). Comparison of NT-proBNP could not be done between patients with CAL and those without, owing to a small number of patients with CAL(n=3). There was no correlation between NT-proBNP and LVEF index(r=0.104, P=0.46) or LVIDd index(r=0.171, P=0.22). CONCLUSION: NT-proBNP increases in the acute phase of KD and decreases to within normal range in the convalescent phase. NT-proBNP >260 pg/mL may be highly suggestive of acute KD. NT-proBNP may be used as a diagnostic tool for KD.
Coronary Vessels ; Echocardiography ; Humans ; Immunoassay ; Mass Screening* ; Mucocutaneous Lymph Node Syndrome* ; Reference Values ; Sensitivity and Specificity

No abstract available.
Truncus Arteriosus*

No abstract available.
Angioplasty* ; Child* ; Humans ; Takayasu Arteritis*

PURPOSE: The adverse reactions of prostaglandin E1(PGE1) are troublesome in the preoperative management of critical patients with ductus dependent congenital heart disease, and a preparation with less adverse reactions is preferable. The effects of Lipo PGE1, a new preparation of PGE1 contained in lipid microspheres, were compared with those of conventional PGE1(PGE1-CD). METHODS: Lipo PGE1 was infused at a rate of 5 ng/kg/min in 19 patients, PGE1-CD at a rate between 10 and 50 ng/kg/min in 15 patients. The effects of drugs were assessed in terms of clinical response rate and overall safety. RESULTS: Clinically, both treatment were effective in relieving cyanosis and hypoxemia except in patients already having either a closed ductus or severe hypoxemia and acidosis. The increments of PaO2 1 hour after infusion were 10.9 and 6.2 mmHg (p>0.1), respectively and those 4 hours postinfusion were 16.0 and 7.8 mmHg(p<0.05), respectively. Even though there was no significant difference in clinical response rate(78.9 vs 60.0%, p>0.1), the mean dose of Lipo PGE1 at appearance of response was about 1/5 of that of PGE1-CD in overall patients and also in patients with ductus dependent pulmonary circulation(6.7 vs 31.7 ng/kg/min, p<0.005). The adverse reactions occurred in 52.6% of the patients given Lipo PGE1, while it was 86.7% in those administered PGE1-CD(p<0.05). The adverse reactions in Lipo PGE1 group was much less severe than that in PGE1-CD group. There was a significant difference in overall safety between the two drugs(84.2 vs 40%, p<0.01). As the incidence of the adverse reactions increased at dose over 5 ng/kg/min, the initial dose of 5 ng/kg/min seemed to be appropriate for Lipo PGE1. CONCLUSIONS: Lipo PGE1 was effective at a lower dose than was PGE1-CD, and was associated with fewer or less severe adverse reactions, and is therefore judged to be more suitable for clinical use than conventional PGE1-CD.
Acidosis ; Alprostadil ; Anoxia ; Cyanosis ; Heart Defects, Congenital* ; Humans ; Incidence ; Microspheres

PURPOSE: Percutaneous closure with occluding coils has been recently described as a method of nonsurgical treatment of the small patent ductus arteriosus (PDA). The snare-assisted technique or detachable coil has been newly developed, improving coil delivery and eliminating the incidence of coil embolization. This method is also applicable to residual PDA following surgical ligation or device implantation. The study purpose is to discuss our experience with percutaneous closure of the small patent ductus arteriosus by occluding coils. METHODS: Between February 1995 and September 1996, 41 patients underwent coil occlusion. Thirty-one patients had native PDAs and 10 residual PDAs. Mean age was 5.0 +/- 3.2 years (1.5 to 14.0 years), and mean body weight 18.0 +/- 7.2kg (8.7 to 45kg). Mean ductal diameter was 1.9 +/- 0.6mm (1.0 to 3.5mm). Occlusion was performed by using the snare technique in 34 patients and by using a detachable coil in 6 patients. Follow-up was done at week 1, 3, 6, and a 12-month postprocedure was dont by echocardiography. RESULTS: Of the 41 patients with successful coil implantation, 32 patients (78%) had no residual shunting, 8 trace residual shunting, and 1 small residual shunting shown by angiogram immediately after coil embolization. All the patients except for one were followed up for 6.5 +/- 4.5 months (1 day to 12 months). Complete closure was confirmed in 38 patients (95%) at 6 months after implantation (34/40 at 1 month, 37/40 at 3 months, 38/40 at 6 months). There were no significant complications. CONCLUSION: Percutaneous occlusion of PDA can be safely and effectively performed in patients with small PDA, irrespective of native or residual nature, by using the snare technique or a detachable coil.
Body Weight ; Ductus Arteriosus, Patent* ; Echocardiography ; Embolization, Therapeutic* ; Follow-Up Studies ; Humans ; Incidence ; Ligation ; SNARE Proteins

PURPOSE: Kawasaki disease(KD) is rare in infants <3 months of age. In this younger group, the diagnosis may be delayed due to lack of most of the clinical criteria, resulting in a high risk of cardiac complications. We examined clinical characteristics in these patients for early recognition and treatment. METHODS: We conducted a retrospective study on the infants with KD aged three months or younger treated at our hospital from January 1998 to July 2002. RESULTS: Of a total of 291 patients treated during the study period, 11(3.8%) were three months old or younger. Of the 11 patients, 10 had atypical presentations. Infants had fewer of the accepted criteria, and the most common findings were fever(100%) and oral mucosal changes(72.7%). Erythema at the site of BCG inoculation was observed in six of the 11 patients(unknown in the remaining five). This feature proved a definite diagnostic clue in two patients in whom cardiac omplications developed in the subacute phase. Cardiac complications were found in six patients(54.5%) : three had coronary dilatation, two had coronary wall irregularity, and one had mitral valve prolapse with regurgitation. Defervescence occurred within 1.1+/-0.3 day in 10 of the 11 patients treated with intravenous immunoglobulin(IVIG) and one was given a second course of IVIG. Echocardiographic abnormality persisted in only one patient with mitral regurgitation at the 6-month follow-up. CONCLUSION: Most patients with KD younger than three months of age have atypical presentations and a high complication rate. For early diagnosis, erythema at the BCG inoculation site, if present, could be used as a valid diagnostic clue to atypical KD in this age group.
Diagnosis ; Dilatation ; Early Diagnosis ; Echocardiography ; Erythema ; Follow-Up Studies ; Humans ; Immunoglobulins, Intravenous ; Infant* ; Mitral Valve Insufficiency ; Mitral Valve Prolapse ; Mucocutaneous Lymph Node Syndrome* ; Mycobacterium bovis ; Retrospective Studies

PURPOSE: Fecal incontinence as a result of anorectal malformation, Hirschsprung's disease, and spina bifida remains a major problem for patients, their families, and for surgeons. The aim of this study was to evaluate the results of an antegrade continence enema (ACE) in children suffering from fecal incontinence. METHODS: An ACE was performed in 18 patients with fecal incontinence due to meningomyelocele between January 1998 and May 2002. Three operative methods were applied: reversed appendicostomy (1/18), orthotopic appendicostomy (5/18) and neoappendicostomy with cecal flap (12/18). The enema solutions used in our patients were the Fleet enema solution, the solin enema solution and the Soft-soap enema solution etc. RESULTS: The overall success rate was 83%. The most common complication was abdominal pain (77.7%) with the other complications being stoma stricture (11.1%), stoma leakage (11.1%), as well as stoma infection, diarrhea, nausea, vomit ing and epigastric soreness. The success of the surgical techniques, the regulation of fecal soiling, and improvement in the quality of life for the patients and their families were evaluated according to the Johns Hopkins Hospital's scoring system. The improvement in the quality of life was 87% with a mean follow-up period of 24 months. CONCLUSION: An ACE procedure appears to be a safe and effective approach for the control of fecal incontinence in pediatric patients with menigomyelocele and significantly improves the quality of life for nearly all patients.
Abdominal Pain ; Child ; Constriction, Pathologic ; Diarrhea ; Enema* ; Fecal Incontinence* ; Follow-Up Studies ; Hirschsprung Disease ; Humans ; Meningomyelocele ; Nausea ; Quality of Life ; Soil ; Spinal Dysraphism

PURPOSE: To compare the findings of MR cholangiography with those of ultrasound and biochemistry in patients with suspected choledocholithiasis, and to evaluate the clinical utility and role of MR cholangiography prior to laparoscopic cholecystectomy. MATERIALS AND METHODS: We retrospectively reviewed the radiologic findings and clinical records of 103 consecutive patients in whom choledocholithiasis was suspected and who underwent both ultrasound and MR cholangiography. For MR imaging, a 1.5T unit was used, and axial T1-FLASH, True FISP, and oblique coronal HASTE and RARE images were obtained. Initial biochemical values (AST, ALT, total bilirubin) were correlated with the findings of MR cholangiography. RESULTS: Choledocholithiasis was present in 36 of 103 patients: overall, there were 34 true-positive, 63 truenegative, four false-positive, and two false-negative results. In the detection of choledocholithiasis, MR cholangiography showed the following characteristics: sensitivity, 94%; specificity, 94%; positive predictive value, 89%; negative predictive value, 96%; accuracy, 95%. Calculi in the common bile duct were detected in 3 of 33 patients (9%) in whom ultrasound showed that the caliber of the common bile duct was normal and whose laboratory findings were normal, and in 12 of 43 (28%) of those whose common bile duct was dilatated or whose laboratory values were abnormal. Calculi were present in the common bile duct of 21 of 27 patients (78%) with abnormal laboratory values and abnormal ultrasound findings. CONCLUSION: Choledocholithiasis was detected in 25% of patients without clinical suspicion and was not present in 25% of patients with strong clinical suspicion. In patients with this condition, MR cholangiography is noninvasive and accurate, and we suggest that in patients with suspected choledocholithiasis, it should be a routine diagnostic procedure prior to laparoscopic cholecystectomy.
Biochemistry ; Calculi ; Cholangiography* ; Cholecystectomy, Laparoscopic* ; Choledocholithiasis* ; Common Bile Duct ; Humans ; Magnetic Resonance Imaging ; Retrospective Studies ; Sensitivity and Specificity ; Ultrasonography

A 3-year-old girl with a primitive neuroectodermal tumor (PNET) and a 6-year-old girl with acute lymphoid leukemia were referred to us because of problems with their implantable central venous ports (Port-A-Cath(R)). On physical examination, the ports were upside-down, so a needle could notbe inserted through the membrane of the port. Right lateral side view of the chest radiogram confirmed port inversion in both cases. At operation, the ports were inverted and the transfixing sutures were totally absorbed. The ports were rotated 180 degrees and anchoring sutures placed.
Child* ; Child, Preschool ; Female ; Humans ; Membranes ; Needles ; Neuroectodermal Tumors, Primitive ; Physical Examination ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Sutures ; Thorax