Pulmonary vascular air embolism is a rare and, universally, almost a fatal complication of positive pressure ventilation in newborn infants. Here, we report a case of this unusual complication in a very-low-birth-weight infant who showed the clinical and radiological features of this complication along with pulmonary hypoplasia and massive hydrops. The possible pathogenesis has been discussed and a brief review of related literature has been presented.
Edema ; Embolism, Air ; Humans ; Hydrops Fetalis ; Infant, Newborn ; Infant, Very Low Birth Weight ; Positive-Pressure Respiration ; Pulmonary Embolism

Poland syndrome is characterized by unilateral absence or hypoplasia of the pectoralis muscle and variable degree of ipsilateral hand anomalies. Mobius syndrome is a congenital neurological disorder characterized by complete or partial facial paralysis. Although the pathogeneses of these diseases are not well-characterized, diminished blood flow to the affected side is thought to play a role. A male infant weighing 2.670 g was born at 38+3 weeks of gestation with left facial paralysis, left chest wall defect with dextrocardia, and symbrachydactyly between the second and third fingers. The combination of Poland-Mobius syndrome is rare, and only 2 cases associated with dextrocardia have been reported worldwide. Here, we report the first case of Poland-Mobius syndrome associated with dextrocardia in Korea.
Dextrocardia ; Facial Paralysis ; Fingers ; Hand ; Humans ; Infant ; Korea ; Male ; Mobius Syndrome ; Nervous System Diseases ; Pectoralis Muscles ; Poland Syndrome ; Pregnancy ; Thoracic Wall

We report a case of an 18-month-old girl with glycogen storage disease type Ib (GSD Ib). Her neutrophil counts had gradually decreased to less than 500/microL by the age of 3 years. However, there were no recurrent bacterial infections. Mutation analysis of the glucose-6-phosphate translocase (G6PT) gene revealed a compound heterozygous missense mutation (Ala148Val/Gly273Asp).
Bacterial Infections ; Glucose-6-Phosphate ; Glycogen ; Glycogen Storage Disease ; Humans ; Infant ; Mutation, Missense ; Neutropenia ; Neutrophils

PURPOSE: The gonadotropin-releasing hormone (GnRH) test results of girls with precocious puberty were analyzed to determine whether this test can efficiently and clearly differentiate between central precocious puberty (CPP) and other disorders. METHODS: Clinical and laboratory data of 54 girls with precocious pubertal signs were reviewed. Intravenous GnRH test was performed with blood samples obtained at 0, 30, 60, and 90 minutes. A peak luteinizing hormone (LH) level of > or =5.0 IU/L was indicative of CPP. RESULTS: Of the 40 girls with CPP, 36 (90.0%), 3 (7.5%), and 1 (2.5%) showed peak LH levels at 30, 60, and 90 minutes, respectively. A percentage of girls whose peak LH > or =5.0 IU/L up to 30, 60, and 90 minutes was 92.5%, 100%, and 100%, respectively. The peak LH/follicle stimulating hormone (FSH) ratio of girls with CPP was 0.89+/-0.49 and was <1 in 16 of the 40 girls (40.0%). Girls with peak LH/FSH ratio of >1.0 showed higher chronological age (CA) (8.3+/-0.6 vs. 7.7+/-1.0 years, P=0.033), bone age (BA) (10.9+/-0.8 vs. 9.7+/-1.1 years, P=0.001), and BA-CA difference (2.6+/-0.7 vs. 2.0+/-0.7 years, P=0.009) than those of girls with peak LH/FSH ratio of < or =1.0. Higher percentage of girls with peak LH/FSH ratio of >1.0 showed advanced breast development (> or =Tanner III) (93.7% vs. 41.7%, P=0.001). CONCLUSION: LH levels after 30 and 60 minutes of intravenous GnRH administration are the most useful for diagnosing CPP in girls.
Breast ; Gonadotropin-Releasing Hormone ; Luteinizing Hormone ; Piperazines ; Puberty, Precocious

PURPOSE: Treatment of precocity with gonadotropin releasing hormone analogue (GnRHa) might theoretically exert a detrimental effect on the bone mass during pubertal development. We investigated the short-term changes in bone mineral density (BMD) during GnRHa treatment and the enhancement in the changes with the co-administration of GnRHa and human growth hormone (hGH). METHODS: Forty girls with precocious or early puberty who were using GnRHa for more than 1 year were enrolled. Of them, 14 concurrently received hGH. Lumbar bone mineral density was measured before and after the treatment, and bone mineral density-standard deviation scores (BMD-SDSs) were compared according to chronologic age (CA) and bone age (BA), as well as according to the administration of GnRHa alone (Group I) or the co-administration of hGH and GnRHa (Group II). RESULTS: BMDs before and after treatment were in the normal range according to CA but were significantly lower according to BA (P<0.05). During treatment, BMD-SDSs did not change according to CA but significantly increased according to BA (P<0.05). BMD-SDSs in group I did not change during treatment according to CA or BA, while those in group II increased significantly according to BA (P<0.05), but not according to CA. CONCLUSION: Lumbar BMD was adequate according to CA at initial manifestation of precocity but was lower if compared to BA, that is, BMD did not increase with BA. Because co-treatment with hGH significantly increased BMD-SDSs according to BA, hGH co-treatment could be considered during GnRHa therapy.
Bone Density ; Gonadotropin-Releasing Hormone ; Human Growth Hormone ; Puberty ; Puberty, Precocious ; Reference Values

PURPOSE: To analyze abnormal ventricular activation in childhood congenital heart disease induced by postoperative changes in ventricular volume and pressure and ventricular scar formation using signal-averaged electrocardiography (SAECG). METHODS: Fifty-two patients who had undergone open heart surgery (OHS) were enrolled. Patients were divided into the following 3 groups: right ventricular volume overload (atrial septal defect, group1), left ventricular volume overload (ventricular septal defect, group2), and right ventricular pressure overload (tetralogy of Fallot, group 3). The patients were monitored by standard 12-lead ECG and SAECG before and 2 months after the operation. QRS duration, QT and QTc intervals, filtered QRS (f-QRS), high frequency low amplitude potential (HFLA), and root mean square (RMS) voltage in the terminal 40 ms of SAECG were determined. RESULTS: In the preoperative period, group1 showed significant increase in QRS (P=0.011) compared to those of the other 2 groups. In the postoperative period, group3 showed significant increase in the QTc interval (P=0.004) compared to those in the other 2 groups. SAECG parameters showed no significant differences among the groups in the pre- or postoperative period. Of the 52 patients, 12 (23%) in the preoperative period and 21 (40%) in the postoperative period had at least 1 SAECG abnormality. The prevalence of SAECG abnormalities was significantly higher in the postoperative group 2 and group 3 (preoperative: 20% versus postoperative: 28%, P<0.001, preoperative: 14% versus postoperative: 64%, P<0.001, respectively). CONCLUSION: Abnormal SAECG patterns may be attributed to postoperative scars, OHS itself, and/or ventricular overload.
Cicatrix ; Electrocardiography ; Heart ; Heart Diseases ; Humans ; Postoperative Period ; Preoperative Period ; Prevalence ; Thoracic Surgery ; Ventricular Pressure

PURPOSE: The aim of this study was to determine the prevalence of asymptomatic nasopharyngeal carriages in children using a multiplex reverse transcriptase-polymerase chain reaction (mRT-PCR) assay kit. METHODS: We obtained nasopharyngeal swabs from 33 children without any underlying disease from July 25 to July 28, 2008. The children were free from the signs of respiratory tract infections at the time of sampling. DNA was extracted from the swabs and subjected to multiplex RT-PCR using a primer set for the detection of pneumococci (Seeplex(R) PneumoBacter ACE Detection Seegene, Seoul, Korea). The amplified PCR products were separated on 2% agarose gels and stained with either ethidium bromide or screen tape system (Lab901 Scotland, UK). RESULTS: A total of 33 children (male, 15 female, 18) aged between 3.2 and 16.3 (median, 8.2) years were included in this study. The mRT-PCR detected colonized bacteria (Streptococcus pneumoniae, Hemophilus influenzae, Chlamydia pneumoniae, and Bordetella pertussis) in 30 children (90.9%). Of these, 13 children (39.4%) showed more than 2 bacteria: 12 children were positive for 2 bacteria (S. pneumoniae and H. influenzae) and 1 child was positive for 3 bacteria (S. pneumoniae, H. influenzae, and C. pneumoniae). CONCLUSION: mRT-PCR was found to be a sensitive tool for the detection of asymptomatic nasopharyngeal carriages. Clinical significances of the bacteria detected by mRT-PCR will have to be evaluated in the future.
Aged ; Bacteria ; Bordetella ; Child ; Chlamydophila pneumoniae ; Colon ; DNA ; Ethidium ; Female ; Gels ; Haemophilus influenzae ; Humans ; Influenza, Human ; Nasopharynx ; Pneumonia ; Polymerase Chain Reaction ; Prevalence ; Respiratory Tract Infections ; Scotland ; Sepharose ; Streptococcus pneumoniae

PURPOSE: The aims of this study were to identify the clinical characteristics and determine the changes in the expression of cytokines and apoptosis-related genes in children with infectious mononucleosis. METHODS: Serological examinations of 15 pediatric patients diagnosed with infectious mononucleosis were performed prospectively. Peripheral blood from the patients was used to compare the composition of T cell subsets, cytokines, Epstein-Barr virus (EBV) DNA, and the expression of apoptosis-related genes with those in 10 healthy children. RESULTS: Mean age of the patient group was 5.7+/-3.4 (range, 3-9) years, and the male-to-female ratio was 1.5:1. Fever, sore throat, pharyngitis/tonsillitis, and cervical lymph node enlargement were the most common symptoms and signs. The proportions of CD3+ T cells, CD8+ suppressor cells, and CD56+ natural killer (NK) cells were higher in the patient group than in the control group (P<0.01). The IL-2, IL-6, and interferon (IFN)-gamma levels were higher in the early symptomatic period (P<0.01). Mean amount of EBV DNA in the patients was 10(2.38) copies/microg, and the amount was the highest at the beginning of the symptomatic period and normalized during the convalescent phase. Bcl-2 expression increased during the initial phase, while Bax expression increased during the convalescent phase. Further, FasL expression increased 1 week after symptom presentation and decreased during the convalescent phase. There was no significant change in Fas expression. CONCLUSION: We analyzed the clinical characteristics and changes in the expression ofcytokines and apoptosis-related genes in the patients with infectious mononucleosis.
Apoptosis ; Child ; Cytokines ; DNA ; Fever ; Herpesvirus 4, Human ; Humans ; Infectious Mononucleosis ; Interferons ; Interleukin-2 ; Interleukin-6 ; Lymph Nodes ; Pharyngitis ; Prospective Studies ; T-Lymphocyte Subsets ; T-Lymphocytes

PURPOSE: Taurine (2-aminoethanesulfonic acid) is a simple sulfur-containing amino acid. It is abundantly present in tissues such as brain, retina, heart, and skeletal muscles. Current studies have demonstrated the neuroprotective effects of taurine, but limited data are available for such effects during neonatal period. The aim of this study was to determine whether taurine could reduce hypoxic-ischemic (HI) cerebral injury via anti-apoptosis mechanism. METHODS: Embryonic cortical neurons isolated from Sprague-Dawley (SD) rats at 18 days gestation were cultured in vitro. The cells were divided into hypoxia group, taurine-treated group before hypoxic insult, and taurine-treated group after HI insult. In the in vivo model, left carotid artery ligation was performed in 7-day-old SD rat pups. The pups were exposed to hypoxia, administered an injection of 30 mg/kg of taurine, and killed at 1 day, 3 days, 1 week, 2 weeks, and 4 weeks after the hypoxic insult. We compared the expressions of Bcl-2, Bax, and caspase-3 among the 3 groups by using real-time polymerase chain reaction (PCR) and western blotting. RESULTS: The cells in the taurine-treated group before hypoxic insult, although similar in appearance to those in the normoxia group, were lesser in number. In the taurine-treated group, Bcl-2 expression increased, whereas Bax and caspase-3 expressions reduced. CONCLUSION: Taurine exerts neuroprotective effects onperinatal HI brain injury due to its anti-apoptotic effect. The neuroprotective effect was maximal at 1-2 weeks after the hypoxic injury.
Animals ; Anoxia ; Apoptosis ; Blotting, Western ; Brain ; Brain Injuries ; Carotid Arteries ; Caspase 3 ; Heart ; Ligation ; Muscle, Skeletal ; Neurons ; Neuroprotective Agents ; Pregnancy ; Rats ; Real-Time Polymerase Chain Reaction ; Retina ; Taurine

Increasing numbers of children and adolescents prefer undertaking physical exercise to overcome overweight or obesity. Children and adolescents are in the growth stage and require adequate nutrient supply. More calories and nutrients are required especially when they are engaged in physical exercise. Exercise is the only means to increase lean body mass and decrease body fat, but adequate nutrient supply is also essential. Lack of adequate nutrient supply causes muscle mass loss, menstruation irregularity, reduced bone density, fatigue, or frequent injury in children undertaking physical exercise. Here, I have introduced some guidelines on the nutrient requirement for child and adolescent athletes.
Adipose Tissue ; Adolescent ; Athletes ; Bone Density ; Child ; Exercise ; Fatigue ; Female ; Humans ; Menstruation ; Mortuary Practice ; Muscles ; Nutritional Requirements ; Obesity ; Overweight