Chronological Echocardiographic Changes in Healthy Term Neonates within Postnatal 72 Hours Using Doppler Studies

BACKGROUND: This study evaluated echocardiographic changes in full-term healthy neonates during early transitional period from postnatal 0–72 hours at 12-hour intervals by echocardiography. METHODS: This was a prospective, observational, and longitudinal single-center cohort study. Morphometric, functional, systolic, diastolic, and tissue Doppler imaging (TDI) parameters (patent ductus arteriosus [PDA], aorta, superior vena cava [SVC], stroke volume [SV], cardiac output [CO], cardiac index [CI], early diastolic flow velocity [E], late diastolic flow velocity [A], early filling in TDI [E′], peak systolic annular velocity in TDI [S′], late velocity peak in TDI [A′], and myocardial performance index [MPI]) were evaluated in left ventricle (LV) and right ventricle (RV) with 56 newborns. RESULTS: Sizes and peak velocities of PDA before postnatal 24 hours were significantly changed than those after postnatal 24 hours. Aortic velocity time integral (VTI), systolic blood pressure (BP), LV SV/kg, LV CO/kg, LV CI, and SVC flow/LV CO before 24 hours showed significantly changes than those after 24 hours. Also, LV and RV MPI before 24 hours were significantly higher than those after 24 hours. LV E/E′ was significantly higher than RV E/E′. CONCLUSION: Postnatal 24 hours is critical time for hemodynamic closure of PDA because aortic VTI, systolic BP, LV SV, LV CO, LV CI, and SVC flow/LV CO showed simultaneously significant changes after 24 hours at the same time as 24 hours of physiological closure of PDA. Chronological and dramatic changes of systolic, diastolic, and TDI parameters during early postnatal period can be used to compile normal baseline data of healthy full-term neonates.

Laboratory Markers in Incomplete Kawasaki Disease according to Coronary Artery Outcome

BACKGROUND AND OBJECTIVES@#We defined laboratory marker profiles typical of incomplete Kawasaki disease (iKD) during illness, especially with respect to the presence of a coronary artery abnormality such as coronary artery dilation or aneurysm.@*METHODS@#This retrospective study examined the clinical and laboratory markers of patients with iKD over time, along with those of patients with complete KD (cKD) and febrile controls.@*RESULTS@#Of 795 patients, 178 had iKD, 504 had cKD and 113 were febrile controls. During the transition from the acute to subacute phase, the age-adjusted hemoglobin levels and platelet counts were significantly lower and higher, respectively, in the subacute phase than in the acute phase in both iKD and cKD patients, which differed from those of febrile controls. Lower levels of acute and subacute age-adjusted hemoglobin levels in iKD patients (odds ratio [OR], 0.538 and 0.583; p=0.006 and 0.018, respectively) and higher subacute platelet counts in cKD patients (OR, 1.004; p=0.014) were correlated with the risk of coronary dilation. A higher acute neutrophil-to-lymphocyte ratio was associated with aneurysm only in cKD patients (OR, 1.059; p=0.044).@*CONCLUSIONS@#The iKD patients share KD-specific laboratory marker profiles in terms of complete blood cell counts and acute phase reactant levels with cKD patients. However, the factors predicting coronary dilation differ according to the phenotype; lower acute and subacute age-adjusted hemoglobin levels predict coronary dilation only in iKD patients.

Laboratory Markers in Incomplete Kawasaki Disease according to Coronary Artery Outcome

BACKGROUND AND OBJECTIVES: We defined laboratory marker profiles typical of incomplete Kawasaki disease (iKD) during illness, especially with respect to the presence of a coronary artery abnormality such as coronary artery dilation or aneurysm. METHODS: This retrospective study examined the clinical and laboratory markers of patients with iKD over time, along with those of patients with complete KD (cKD) and febrile controls. RESULTS: Of 795 patients, 178 had iKD, 504 had cKD and 113 were febrile controls. During the transition from the acute to subacute phase, the age-adjusted hemoglobin levels and platelet counts were significantly lower and higher, respectively, in the subacute phase than in the acute phase in both iKD and cKD patients, which differed from those of febrile controls. Lower levels of acute and subacute age-adjusted hemoglobin levels in iKD patients (odds ratio [OR], 0.538 and 0.583; p=0.006 and 0.018, respectively) and higher subacute platelet counts in cKD patients (OR, 1.004; p=0.014) were correlated with the risk of coronary dilation. A higher acute neutrophil-to-lymphocyte ratio was associated with aneurysm only in cKD patients (OR, 1.059; p=0.044). CONCLUSIONS: The iKD patients share KD-specific laboratory marker profiles in terms of complete blood cell counts and acute phase reactant levels with cKD patients. However, the factors predicting coronary dilation differ according to the phenotype; lower acute and subacute age-adjusted hemoglobin levels predict coronary dilation only in iKD patients.

Correlation of B-type natriuretic peptide levels and echocardiographic parameters in preterm infants with patent ductus arteriosus / 소아과

PURPOSE: This study aimed to evaluate the correlation, according to postnatal age, between plasma B-type natriuretic peptide (BNP) levels and echocardiographic parameters for the assessment of patent ductus arteriosus (PDA) in preterm infants with respiratory distress. METHODS: We enrolled 42 preterm infants with respiratory distress who underwent serial echocardiographic evaluation with simultaneous plasma BNP measurements until ductal closure. The correlations between BNP levels and the following 4 representative echocardiographic parameters were studied: diameter of the ductus arteriosus (DA), ratio of the left atrial diameter to the aortic diameter (LA/Ao), ratio of the PDA diameter to the infant's left pulmonary artery diameter (PDA/LPA), and the antegrade diastolic flow of LPA (DFLPA). RESULTS: BNP levels were significantly correlated to the magnitude of the ductal shunt, comprising the DA diameter, PDA/LPA ratio, LA/Ao ratio, and antegrade DFLPA for the overall study period. The earliest significant correlation, starting from postnatal day 2, was observed between the LA/Ao ratio and BNP levels. The PDA/LPA ratio and the antegrade DFLPA showed significant correlations with BNP levels postnatal day 3 onward, and with the DA diameter, postnatal day 5 onward. CONCLUSION: BNP levels and echocardiographic parameters showed a positive correlation, but the significance of the correlations differed according to the postnatal age, especially during the first few days of life.

A Case of Congenital Hypertrophic Cardiomyopathy

Congenital hypertrophic cardiomyopathy (HCMP) is a very rare congenital heart disease. Here, we report a case of neonatal HCMP, which was confirmed by two-dimensional echocardiography and autopsy. The HCMP rapidly progressed and the patient's condition deteriorated, despite the treatment for congestive heart failure.

Myocardial Injury in Newborn Infants with Severe Metabolic Acidosis at the First Day of Life

PURPOSE: Severe acidosis by various causes can lead to multi-organ dysfunction including cardiac dysfunction (CD) due to myocardial injury. The aims of this study are to investigate CD in newborn infants with severe acidemia at the first day of life and to evaluate the effect on myocardial injury according to the type and the severity of acidosis. METHODS: This was a retrospective study of infants > or =34 weeks of gestational age with severe acidemia (pH60 mmHg) and non-RA group (pCO2 or =-10) were classified based on the degree of BE. RESULTS: The levels of CK-MB and myoglobin had significant negative relation with BE. CD group had higher incidences of seizure and mortality and higher levels of CK-MB and cTnI than those of non-CD group. Severe BE group had higher incidences of CD and seizure and higher levels of CK-MB, myoglobin and cTnI than those of mild BE group. Non-RA group had higher levels of CK-MB and myoglobin than those of RA group. CONCLUSION: At the first day of life, the newborn infants with severe metabolic acidosis have high incidences of CD and myocardial injury. Aggressive monitoring and appropriate treatment for CD according to myocardial injury should be recommended in the newborn infants with severe metabolic acidosis.

Factors to Predict Positive Results of Gonadotropin Releasing Hormone Stimulation Test in Girls with Suspected Precocious Puberty

Sometimes, the clinical findings and the results of the gonadotropin-releasing hormone (GnRH) stimulation test are inconsistent in girls with early breast development and bone age advancement. We aimed to investigate the factors predicting positive results of the GnRH stimulation test in girls with suspected central precocious puberty (CPP). We reviewed the records of 574 girls who developed breast budding before the age of 8 yr and underwent the GnRH stimulation test under the age of 9 yr. Positive results of the GnRH stimulated peak luteinizing hormone (LH) level were defined as 5 IU/L and over. Girls with the initial positive results (n = 375) showed accelerated growth, advanced bone age and higher serum basal LH, follicle-stimulating hormone, and estradiol levels, compared to those with the initial negative results (n = 199). Girls with the follow-up positive results (n = 64) showed accelerated growth and advanced bone age, compared to those with the follow-up negative results. In the binary logistic regression, the growth velocity ratio was the most significant predictive factor of positive results. We suggest that the rapid growth velocity is the most useful predictive factor for positive results in the GnRH stimulation test in girls with suspected precocious puberty.

Association of Serum Retinol Binding Protein 4 with Adiposity and Pubertal Development in Korean Children and Adolescents

Retinol binding protein 4 (RBP4) has been postulated to provide a new link between obesity and insulin resistance. We aimed to assess the relationship between serum RBP4 and insulin resistance by investigating serum RBP4 levels in children and adolescents according to degree of obesity and pubertal stage. A total of 103 (30 lean, 39 overweight, 34 obese) were evaluated for serum RBP4, adiponectin, insulin, glucose and lipid profiles. RBP4 levels of obese and overweight groups were higher than those of lean group. RBP4 level was higher in pubertal group than in prepubertal group. RBP4 was positively correlated with age, height, weight, body mass index (BMI), abdominal circumference, systolic blood pressure, fasting insulin, homeostatic model assessment of insulin resistance (HOMA-IR), total cholesterol and triglyceride, and inversely with adiponectin. In the multiple linear regression analysis, RBP4 was found to be independently associated with pubertal stage, BMI and triglyceride but not with HOMA-IR. In conclusion, serum RBP4 level is related with degree of adiposity and pubertal development. The association of RBP4 with insulin resistance is supposed to be secondary to the relation between RBP4 and adipose tissue in children and adolescents.

Aldosterone Modulates Cell Proliferation and Apoptosis in the Neonatal Rat Heart

In the present study, we investigated whether and how the mineralocorticoid receptor antagonist spironolactone affects cardiac growth and development through apoptosis and cell proliferation in the neonatal rat heart. Newborn rat pups were treated with spironolactone (200 mg/kg/d) for 7 days. The cell proliferation was studied by PCNA immunostaining. The treatment with spironolactone decreased proliferating myocytes by 32% (P<0.05), and reduced myocytes apoptosis by 29% (P<0.05). Immunoblot and immunohistochemistry for the expression of p38, p53, clusterin, TGF-beta2, and extracellular signal-regulated kinase were performed. In the spironolactone group, p38, p53, clusterin, and TGF-beta2 protein expression was significantly decreased (P<0.05). These results indicate that aldosterone inhibition in the developing rat heart induces cardiac growth impairment by decreasing proliferation and apoptosis of myocytes.

Comparison of predicted adult heights measured by Bayley-Pinneau and Tanner-Whitehouse 3 methods in normal children, those with precocious puberty and with constitutional growth delay / 소아과

PURPOSE: This study compared bone ages measured by the Greulich-Pyle (GP) and Tanner-Whitehouse 3 (TW3 ) methods and investigated the differences in predicted adult heights measured by Bayley-Pinneau (BP) and TW3 methods. METHODS: Bone ages were assessed from left-wrist radiographs by two investigators, one for each GP and TW3 methods in 85 normal children, 30 precocious puberty girls, and 30 constitutional growth delay boys. The differences between the measured predicted adult heights using the BP and TW3 methods were compared in each group. RESULTS: The bone age measured by the TW3 method was less than that by the GP method in normal children. The predicted adult heights measured by the two methods showed no significant difference in normal boys, while the predicted adult height measured by the TW3 method was higher than that by the BP method for normal girls (156.4+/-4.7 cm vs. 158.9+/-3.8 cm, P<0.01) and for precocious puberty girls (156.3+/-4.0 cm vs. 159.3+/-4.2 cm, P<0.01). In contrast, the predicted adult height was higher from the BP method than from the TW3 method in constitutional growth delay boys (173.3+/-4.4 cm vs. 169.7+/-3.2 cm, P<0.01). CONCLUSION: There were significant differences in predicted adult heights between the BP and TW3 method in normal girls, precocious puberty girls, or constitutional growth delay boys. In precocious puberty and constitutional growth delay, the BP method might be preferred to predict adult height, but further studies on final adult height are needed.

Bone Mineral Density and Correlation Factors in Normal Children and Adolescence / 대한소아내분비학회지

PURPOSE:The mean height of Korean children has been increased recently, and new version of growth chart was made in 2007. Nevertheless, the reference value on bone mineral density (BMD) of normal children has not been established yet. The aims of this study were to obtain mean values of BMD in normal children and to investigate correlation between BMD and their relating factors. METHODS:One hundred thirty five normal children from 6 to 14 years old were included, who were 69 boys and 66 girls. Bone density was measured at the lumbar spine and the proximal femur by dual energy x-ray absorptiometry (Discovery A, Hologic, 2004). We assessed bone age, body mass index, bone alkaline phosphatase, insulin-like growth factor I (IGF-I), sex steroid level and analyzed their correlation with BMD. Results:The result showed a trend of an increase in BMD according to the age in both boys and girls. The mean value of BMD showed the greatest increase during age of 10 to 11 in girls and 12 to 13 in boys. A sudden increase of BMD in both boys and girls occurred during the overt puberty. Bone age and IGF-I level indicated the most significant relating factors of BMD of lumbar spine. The mean values of BMD of this study were similar to the previous studies about BMD of Korean children. CONCLUSION:The results could be utilized as fundamental data for normal BMD value of Korean children and larger population study is needed.

Congenital Cardiopulmonary Anomalies in Infants with Recurrent Stridor and/or Respiratory Distress: Report of 3 Cases / 소아알레르기및호흡기학회지

Although laryngomalacia is the most common cause of congenital stridor in infancy, it is a benign, self-limited disease which usually resolves by the age of 18 months with no long- term sequelae. Nevertheless, infants who suffered from recurrent stridor and respiratory distress should be evaluated for other causes of stridor such as cardiopulmonary anomalies. We report 3 cases of infants who had recurrent stridor and respiratory distress from their early infancy. Case 1 had a double aortic arch and a tracheomalacia, case 2 had a hypoplasia of the right lung, and case 3 had a horseshoe lung as well as scimitar syndrome. Physicians should be alert for the possibility of the congenital cardiopulmonary abnormalities in infants with recurrent stridor and/or respiratory distress.

An Autopsy Case of New Bronchopulmonary Dysplasia / 소아알레르기및호흡기학회지

Bronchopulmonary dysplasia (BPD) is a chronic lung disease that affects premature babies and contributes to their increased morbidity and mortality. The classic progressive stage with prominent fibroproliferation that first characterized BPD is generally less striking and the disease is currently predominantly defined by a disruption of distal lung growth, thus it has been termed "new BPD." We present here in a case of 5-month-old girl dying from BPD and pulmonary hypertension. Autopsy findings of the lung showed fewer and larger alveoli with less fibrosis, which is consistent with those of "new BPD."

Evaluating and managing hypertension in children: a survey of Korean cardiologists and nephrologists / 소아과

PURPOSE: Hypertension (HTN) is no longer an exclusively adult disease; the prevalence of pediatric HTN is increasing. To understand the evaluation and treatment of childhood HTN in Korea, we investigated, via a questionnaire, how hypertensive children are currently assessed and managed by pediatric cardiologists (CA) and nephrologists (NE). METHODS: We surveyed 82 pediatric CA and 77 NE, regarding how they manage hypertensive children in Korea. RESULTS: A total 75 replies were received to our questionnaire request (response rate: 47.15%). Routine blood pressure (BP) checks were more frequently performed by NE (CA: 5.7%, NE: 25%, P=0.03), but most respondents (86%) did not check BP routinely. Mercury sphygmomanometers were the most commonly used devices and ambulatory blood pressure monitoring was not frequently used. The goal BP in treated patients was set at the 95th percentile by 61% of respondents. NE used a lower BP goal in hypertensive children with renal disease (CA: 24%, NE: 64%, P=0.004) or DM (CA: 12.5%, NE: 50%, P=0.003). Angiotensin converting enzyme (ACE) inhibitors were the most commonly used agents (mean: 59%); following ACE inhibitors, CA preferred diuretics for hypertensive children with renal disease or cardiovascular disease, and NE preferred calcium channel blockers, regardless of underlying disease. Self-monitoring was the most frequent method for BP monitoring at home. CONCLUSION: In Korea, BP measurement is not yet a routine examination in treating hypertension. There are some differences in management strategies vis-a-vis hypertension, between pediatric cardiologists and nephrologists. For the appropriate management and prevention of secondary disease in long-term studies, standard guidelines and education are needed for pediatricians.

Evaluating and managing hypertension in children: a survey of Korean cardiologists and nephrologists / 소아과

PURPOSE: Hypertension (HTN) is no longer an exclusively adult disease; the prevalence of pediatric HTN is increasing. To understand the evaluation and treatment of childhood HTN in Korea, we investigated, via a questionnaire, how hypertensive children are currently assessed and managed by pediatric cardiologists (CA) and nephrologists (NE). METHODS: We surveyed 82 pediatric CA and 77 NE, regarding how they manage hypertensive children in Korea. RESULTS: A total 75 replies were received to our questionnaire request (response rate: 47.15%). Routine blood pressure (BP) checks were more frequently performed by NE (CA: 5.7%, NE: 25%, P=0.03), but most respondents (86%) did not check BP routinely. Mercury sphygmomanometers were the most commonly used devices and ambulatory blood pressure monitoring was not frequently used. The goal BP in treated patients was set at the 95th percentile by 61% of respondents. NE used a lower BP goal in hypertensive children with renal disease (CA: 24%, NE: 64%, P=0.004) or DM (CA: 12.5%, NE: 50%, P=0.003). Angiotensin converting enzyme (ACE) inhibitors were the most commonly used agents (mean: 59%); following ACE inhibitors, CA preferred diuretics for hypertensive children with renal disease or cardiovascular disease, and NE preferred calcium channel blockers, regardless of underlying disease. Self-monitoring was the most frequent method for BP monitoring at home. CONCLUSION: In Korea, BP measurement is not yet a routine examination in treating hypertension. There are some differences in management strategies vis-a-vis hypertension, between pediatric cardiologists and nephrologists. For the appropriate management and prevention of secondary disease in long-term studies, standard guidelines and education are needed for pediatricians.

Complications of transcatheter closure of atrial septal defects using the amplatzer septal occluder / 소아과

PURPOSE: Transcatheter closure of atrial septal defects (ASD) is currently established therapy as an alternative to surgery. But rarely, complications are reported in some studies. We report early and intermediate term complications associated with transcatheter closure of atrial septal defects using the Amplatzer septal occluder (ASO). METHODS: From June 2003 to May 2006, 64 patients underwent transcatheter closure of secundum ASD or patent foramen ovale using the ASO. The ratio of male to female was 1:2.4, the median age was 17 years (range: 2.6-64 years) and their median weight was 47.5 kg (range: 2.6-64 kg). RESULTS: The median diameter of ASD measured with transthoracic or transesophageal echocardiography was 15 mm (range: 6-28 mm), the median balloon stretched diameter was 18 mm (range: 6.5-34 mm), and the median size of device was 19.5 mm (range: 6-36 mm), was little difference with balloon stretched diameter. There were 10 cases of complications: arrhythmia (2), device malformation (2), aorta to right atrial fistula (1), hemolytic anemia (1), mitral valve encroachment (1), malposition (1), residual shunt (1), and inferior vena cava perforation (1). CONCLUSION: Transcatheter closure of ASD using ASO is effective and safe therapy. However, significant complications such as aorta to atrial fistula, atrial erosion, or device embolization can happen, so an appropriate selection of patient and device in relevance to size and anatomy of ASD is important for successful closure.

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery : Report of an Adult Case

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), also termed Bland-White-Garland syndrome, is a rare congenital cardiac anomaly characterized by myocardial ischemia which becomes symptomatic during early infancy leading to infarction, left heart failure or even death. More than 90% of patients can be dead within first year of life if untreated, so adult type ALCAPA without surgical correction is quitely rare. We present a case of a 20-year-old man with ALPACA syndrome whose diagnosis took several years to be made because he was mistaken to have organic mitral regurgitation.

Clinical application of D-dimer in Kawasaki Disease / 소아과

PURPOSE: Vascular endothelial cell damage and alteration of a fibrinolytic system was suggested to play a role in the development of coronary artery abnormalities in Kawasaki disease (KD). D-dimer is one of the markers of endothelial damage and fibrinolysis. We evaluated the clinical usefulness of D- dimer to differentiate KD from other febrile diseases and predict coronary artery abnormalities in KD. METHODS: Sixty eight patients diagnosed as KD and twenty eight patients presented with acute febrile illnesses other than KD from September 2005 to July 2006 were included. Blood levels of D- dimer and various inflammatory markers were measured before treatment and the clinical course of KD was followed. Serial echocardiography was performed at the onset of disease and thereafter at a monthly interval for at least 2 months. RESULTS: KD patients showed a higher D-dimer level than febrile controls, but the difference was not significant (1.21+/-0.77 mg/mL vs 0.92+/-0.71 mg/mL, P=0.083). Neither was the difference between KD patients who had coronary artery abnormalities and those who had not (1.49+/-0.98 mg/mL vs 1.15+/-0.71 mg/mL, P=0.169). D-dimer was significantly correlated with other inflammatory markers, such as C-reactive protein and erythrocyte sedimentation rate in both KD patients and febrile controls. CONCLUSION: D-dimer was not specific for KD. But it may be useful as an inflammatory marker to assess the severity of KD.

Clinical improvement in a case of atypical infantile onset Pompe disease with enzyme replacement therapy / 소아과

Pompe disease is a genetic disorder caused by a deficiency of acid alpha-glucosidase (GAA). Infantile onset Pompe disease is uniformly lethal. Affected infants generally present in the first few months of life with hypotonia, generalized muscle weakness, and a hypertrophic cardiomyopathy, which is rapidly followed by death, usually by the age of one. The late-onset form is characterized less severe symptoms and prognosis. Therapy for Pompe disease is intended to directly address the underlying metabolic defect via intravenous infusions of recombinant human GAA to replace the missing enzyme. We report a case of atypical infantile-onset Pompe disease that presented symptoms in infancy but had less severe clinical manifestations and improved after GAA enzyme replacement (Myozyme(R), Genzyme Co., MA, USA) therapy. It is very important that pediatricians become aware of signs and symptoms of Pompe disease, such as a nasal voice or a waddling gait at an early stage so that these patients can benefit from appropriate GAA replacement therapy as soon as possible.

Carotid artery intima media thickness in obese children: relation with cardiovascular risk factors / 소아과

PURPOSE: The of common carotid artery intima media thickness (IMT) is an acknowledged noninvasive marker for early atherosclerotic changes. We investigate whether common carotid IMT is different between obese and normal-weight children and also evaluate the relationships IMT with cardiovascular risk factors. METHODS: We collected the clinical data (age, sex, pubertal stage, body mass index) and measured blood pressure, glucose, insulin, lipid profiles and adiponectin in 49 obese children (mean age, 12 years) and 24 nonobese children as controls. The control group was composed of 24 nonobese children of the same age, sex and pubertal stage. We measured the carotid IMT of all subjects by B-mode ultrasound with a 7.5-MHz linear transducer and analyzed. RESULTS: Obese children demonstrated a significantly thicker intima media (mean, 0.34 mm, peak, 0.42 mm) compared to the control group (mean, 0.31 mm, peak, 0.38 mm, P<0.01). IMT was significantly correlated to the BMI (r=0.431, P<0.01), age (r=0.317, P<0.01), total cholesterol (r=0.377, P<0.01), triglyceride (r=0.253, P<0.05) and low-density lipoprotein cholesterol (r=0.289, P<0.05). Serum adiponectin was significantly lower in obese children than in controls (11.2 ng/mL vs. 14.7 ng/mL, P<0.05) and negatively related with IMT (r=-0.267, P<0.05). CONCLUSION: Obesity is associated with increased carotid artery IMT in children. Our results suggest vascular changes in obesity seem to occur already in childhood and vascular ultrasonography may helpful for screening cardiovascular complications in obese children.