[Show abstract][Hide abstract] ABSTRACT: 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.
[Show abstract][Hide abstract] ABSTRACT: 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.
Journal of Korean medical science 02/2012; 27(2):194-9. · 0.84 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: 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.
Journal of Korean medical science 06/2011; 26(6):797-802. · 0.84 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: 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.
Journal of Korean medical science 09/2010; 25(9):1296-304. · 0.84 Impact Factor
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[Show abstract][Hide abstract] ABSTRACT: A 12-year-old boy visited the emergency room for severe dyspnea. He was diagnosed with tuberous sclerosis at 5 years of age, and right ventricular dysplasia was detected at 11 years of age. Thromboemboli in right ventricle and bilateral pulmonary arteries were confirmed with two-dimensional echocardiography and computed tomography. We report the case of a patient with tuberous sclerosis who presented with huge thrombi in a dysplastic right ventricle and massive bilateral pulmonary thromboemboli without evidence of a cardiac tumor.
[Show abstract][Hide abstract] ABSTRACT: Hypertension (HTN) is no longer viewed as an adult disease. The purpose of the present study was to understand how hypertensive children are evaluated and managed, by surveying pediatricians in Japan and South Korea.
A questionnaire was mailed to 109 Japanese (JA) and 159 Korean (KO) pediatric cardiologists, pediatric nephrologists, and other pediatricians.
A total of 127 replies were received (response rate 47%). Most of respondents did not check blood pressure (BP) routinely in outpatient clinics (JA 77%, KO 88%). A mercury sphygmomanometer was the most commonly used method for BP measurements (JA 72%, KO 62%). BP treatment goals were usually set at the 95th percentile for age, gender, and height (JA 47%, KO 54%). More KO used a lower goal in children with primary HTN than JA. KO respondents preferred angiotensin-converting enzyme inhibitors (ACEI) as initial agents regardless of underlying diseases whereas JA respondents chose various medications, that is, calcium channel blockers, diuretics, and ACEI. For BP monitoring, self-monitoring was found to be most frequent in both countries (JA 80%, KO 57%). Ambulatory BP monitoring was not frequently utilized in both countries (JA 33% KO 34%).
The current assessment, management and differing trends in pediatric HTN in Japan and Korea have been identified in the present study. Pediatricians should be aware of the growing implications of HTN in children.
Pediatrics International 04/2009; 52(1):1-5. · 0.73 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: 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.
[Show abstract][Hide abstract] ABSTRACT: A 16-month-old boy with previous repair of a critical pulmonary stenosis had persistence of a right modified Blalock-Taussig shunt. Transcatheter occlusion of the modified Blalock-Taussig shunt was achieved using the Amplatzer vascular plug with the catheter-snare technique.
[Show abstract][Hide abstract] ABSTRACT: Because the major problems of respiratory difficulties in newborn infants are due to cardiopulmonary problems, improving the early detection and referral of newborn infants with cardiovascular problems has been considered one of the primary goals of care in the neonatal intensive care unit.
To evaluate whether rapid plasma B-type natriuretic peptide (BNP) assay could be used as a screening test to detect the cardiovascular problems in newborn infants with respiratory difficulties.
We studied 73 newborn infants >or=34 weeks gestational age presenting with respiratory difficulties during the first few days after birth; they were divided into a cardiovascular problem group (CP group, n = 32) and a noncardiac problem group (NP group, n = 41) according to the presence of cardiovascular problems by clinical and/or echocardiographic studies in newborn infants with respiratory difficulties.
On admission, the median (25-75%) BNP concentration of the CP group was significantly higher than that of the NP group [1,038 (578-1,435) vs. 240 (118-388) pg/ml, p < 0.001]. The best cutoff BNP values for differentiating the CP group were 346.0, 421.0, 570.5 and 191.5 pg/ml within 18 h, at 18-36 h, at 36-60 h and after 60 h of life, respectively. Although the plasma BNP measurement was not a single confirmative test, it was found to have a high sensitivity and a high negative predictive value for rapidly ruling out serious cardiovascular problems in neonatal respiratory difficulties.
A rapid plasma BNP assay may be useful for detection of cardiovascular problems in newborn infants with respiratory difficulties during the first few days after birth.
[Show abstract][Hide abstract] ABSTRACT: This study was done to determine whether recombinant human erythropoietin (rhEPO) treatment could attenuate hyperoxia-induced lung injury, and if so, whether this protective effect is mediated by the down-modulation of inflammation in neonatal rats. Newborn Sprague Dawley rat pups were subjected to 14 days of hyperoxia (>95% oxygen) within 10 hr after birth. Treatment with rhEPO significantly attenuated the mortality and reduced body weight gain caused by hyperoxia. With rhEPO treatment, given 3 unit/gm intraperitoneally at 4th, 5th, and 6th postnatal day, hyperoxia- induced alterations in lung pathology such as decreased radial alveolar count, increased mean linear intercept, and fibrosis were significantly improved, and the inflammatory changes such as myeloperoxidase activity and tumor necrosis factor-alpha expression were also significantly attenuated. In summary, rhEPO treatment significantly attenuated hyperoxia-induced lung injury by down-modulating the inflammatory responses in neonatal rats.
Journal of Korean Medical Science 12/2007; 22(6):1042-7. · 1.25 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: To evaluate the short- and mid-term results and complications ensuing the transcatheter closure of patent ductus arteriosus (PDA). Between October 1999 and December 2005, 117 patients (34 males and 83 females) underwent attempted percutaneous closure of PDA with a minimum diameter of more than 3 mm. Follow-up evaluations were conducted at 1 day and 1, 3, 6, 12 months after the performance of the transcatheter closure. The median age of patients at catheterization was 11 yr (range, 0.6 to 68 yr), median weight was 30 kg (range, 6 to 74 kg), and the median diameter of PDA was 4 mm (range, 3 to 8 mm). This procedure was conducted successfully in 114 patients (97.4%), using different devices. Major complications were detected in 4 patients (3.4%); significant hemolysis (2), infective endocarditis (1), failed procedure due to embolization (1). Minor complications occurred in 6 patients (5.1%); mild narrowing of the descending aorta (2) and mild encroachment on the origin of the left pulmonary artery (4). Although the transcatheter closure of PDA may be considered to be effective, several complications, including hemolysis, embolization, infective endocarditis, and the narrowing of adjacent vessels may occur in certain cases.
Journal of Korean Medical Science 07/2007; 22(3):484-90. · 1.25 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: 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.
[Show abstract][Hide abstract] ABSTRACT: Pompe disease is a genetic disorder caused by a deficiency of acid α-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®, 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.
[Show abstract][Hide abstract] ABSTRACT: Histiocytic necrotizing lymphadenitis, which is also commonly referred to as Kikuchi's disease (KD), is a self-limiting disease of unknown etiology. It affects individuals of all ages, although it is usually seen in young women. However, only a few descriptions of this disease are available in the pediatric literature. KD is clinically characterized by cervical lymphadenopathy, high fever, myalgia, neutropenia and, rarely, cutaneous eruptions. Cutaneous manifestations have been reported in 16-40 percent of KD cases. The specific skin changes occurring in cases of KD have yet to be completely characterized. In most of the reported cases thus far, the lesions have been located on the face and upper extremities. In this report, we describe a case of pediatric Kikuchi's disease, occurring in a 9-year-old boy. The boy exhibited transient erythematous maculopapular skin lesions over the entirety of his body, including his lower extremities.
[Show abstract][Hide abstract] ABSTRACT: Hepatitis B viral (HBV) infection in early childhood is one of the leading causes of chronic hepatitis and liver cirrhosis that eventually lead to hepatic carcinoma. Despite the nationwide immunization programs to curtail the vertical transmission of HBV, childhood HBV infection through mothers is still occurring in Korea. As one of the efforts to understand the childhood HBV infection in Korea, four HBV promoter sequences in the sera of the chronically infected children were analyzed. Children harbored diverse viral variants as most of the chronically infected adult patients, but the deletion mutations were rare. The dominant viral sequences in the children were highly similar to the ones in the respective mothers, indicating that the maternal viruses were most likely transmitted to the children. The mutations in X, S1, S2/S promoters did not seem to show any correlation to the severity of the disease nor ages of the children. The mutations that showed some correlation to the severity of the disease were the mutations in C promoter, but the mutations did not seem to be vertically transmitted. Finally, the children with the elevated ALT/AST levels tended to have more child-specific variants suggesting that the accumulation of host-specific mutations might be associated with the development of clinical symptoms.
Archives of Virology 09/2005; 150(8):1639-51. · 2.28 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: In preterm infants, the rapid and accurate determination of the presence of a hemodynamically significant patent ductus arteriosus (PDA) is extremely important, but this is often difficult. Plasma B-type natriuretic peptide (BNP) measurement has been reported to be a helpful aid in the diagnosis of hemodynamically significant PDA in preterm infants. The aim of our study was to investigate the usefulness of a rapid BNP assay as a diagnostic marker of symptomatic PDA (sPDA) in preterm infants.
Sixty-six preterm infants, ranging from 25 to 34 gestational weeks of age, underwent clinical and echocardiographic examinations for PDA every other day from the third day of life until the disappearance of ductal flow. Blood samples were collected and plasma BNP concentrations were measured simultaneously using a commercial kit, (Triage BNP test kit; Biosite Diagnositics, San Diego, CA). When > or =2 clinically significant features of PDA were noted, and a large ductal flow was confirmed by color Doppler echocardiography, sPDA was diagnosed and treated with indomethacin.
On the third day after birth, the mean BNP concentration in the sPDA group (n = 23) was significantly higher than in the control group (n = 43) (2896 +/- 1627 vs 208 +/- 313 pg/mL). Seventeen infants (74%) in the sPDA group became asymptomatic after an initial course of indomethacin and their BNP levels concomitantly decreased. Moreover, BNP concentrations were significantly correlated with the magnitudes of the ductal shunt, such as the ratio of left atrial to aortic root diameter and the diastolic flow velocity of the left pulmonary artery (r = 0.726 and 0.877). The area under the receiver operator characteristic curve for the detection of sPDA was high: 0.997 (95% confidence interval: 0.991-1.004). The best cutoff of BNP concentration for the diagnosis of sPDA was determined to be 1110 pg/mL (sensitivity: 100%; specificity: 95.3%).
In preterm infants, the circulating BNP levels correlated well with the clinical and echocardiographic assessments of PDA. Although not a stand-alone test, the rapid BNP assay provides valuable information for the detection of infants with sPDA that require treatment. Moreover, serial BNP measurements may be of value in determining the clinical course of PDA in preterm infants.