• Journal of Genetic Medicine
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• v.10 no.2
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• pp.113-116
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• 2013

Alexander disease is a rare degenerative leukodystrophy caused by dominant mutations in glial fibrillary acidic protein (GFAP). The neonatal form of Alexander disease may manifest as frequent and intractable seizures or obstructive hydrocephalus, with rapid progression leading to severe disability or death within two years. We report a case of a 50-day-old male who presented with intractable seizures and obstructive hydrocephalus. His initial magnetic resonance imaging (MRI) suggested a tumor-like lesion in the tectal area causing obstructive hydrocephalus. Despite endoscopic third ventriculostomy and multiple administrations of antiepileptic drugs, the patient experienced intractable seizures with rapid deterioration of his clinical status. After reviewing serial brain MRI scans, Alexander disease was suspected. Subsequently, we confirmed the de novo missense mutation in GFAP (c.1096T>C, Y366H). Although the onset was slightly delayed from the neonatal period (50 days old), we concluded that the overall clinical features were consistent with the neonatal form of Alexander disease. Furthermore, we also suspected that a Y366 residue might be closely linked to the neonatal form of Alexander disease based on a literature review.

• Physical Therapy Korea
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• v.14 no.1
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• pp.28-36
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• 2007

The aim of this study was to identify the effect of suspension unloading (SU) and electrical stimulation upon the development of neonatal muscular system. For this study, the neonatal rats were randomly divided into three groups: a control group, an experimental group I, and an experimental group II. The SU for experimental group I and II was applied from postnatal day (PD) 5 to PD 30. The electrical stimulation for soleus muscle of experimental group IIwas applied from PD 16 to PD 30 using neuromuscular electrical stimulation (NMES), which gave isometric contraction with 10 pps for 30 minutes twice a day. In order to observe the effect of SU and ES, this study observed myocyte enhancer factor 2C (MEF2C) and vascular endothelial growth factor (VEGF) immunoreactivity in the soleus muscles at PD 15 and PD 30. In addition, the motor behavior test was performed through footprint analysis at PD 30. The following is the result. At PD 15, the soleus muscles of experimental group Iand II had significantly lower MEF2C, VEGF immunoreactivity than the control group. It proved that microgravity conditions restricted the development of the skeletal muscle cells at PD 15. At PD 30, soleus muscles of the control group and experimental group II had significantly higher MEF2C, VEGF, immunoreactivity than experimental group I. It proved that the NMES facilitated the development of the skeletal muscle cells. At PD 30, it showed that SU caused the decrease in stride length of parameter of gait analysis and an increase in toe-out angle, and that the NMES decreased these variations. These results suggest that weight bearing during neonatal developmental period is essential for muscular development. They also reveal that NMES can encourage the development of muscular systems by fully supplementing the effect of weight bearing, which is an essential factor in the neonatal developmental process.

• Clinical and Experimental Pediatrics
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• v.49 no.9
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• pp.966-971
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• 2006

Purpose : 'Programming' describes the process that stimulus at a critical period of development has lifelong effects. The fact that low birth weight links to the risk of elevated blood pressures in adult life is well known. This study aims to examine whether this link is evident in the newborn by investigating the relationship of the intrauterine growth indices and neonatal blood pressure(BP). Methods : We studied 127 neonates who were born at Ewha Womans' Hospital and their mothers enrolled our cohort study during pregnancy. Data on the mothers and details of the birth records were tracked and collected from medical charts. Neonatal BP was measured within 24 hours after birth. Results : Neonatal SBP was positively correlated to intrauterine growth indices; birth weight(BW)(r=0.4), head circumference(HC)(r=0.4), and birth height(r=0.3). However, an inverse relationship existed, between HC/BW ratio and neonatal SBP(r=-0.4). After adjusting for the baby's sex, maternal BP, and gestational age, neonatal SBP still associated with intrauterine growth indices. SBP was 7 mmHg higher in the highest BW group(${\geq}90percentiles$) compared to the lowest group(<10 percentiles). On the other hand, SBP was 17 mmHg lower in the highest HC/BW group(${\geq}90percentiles$) compared in the lowest group(<10 percentiles). Conclusion : This study could not find the evidence that intrauterine growth retardation affect on elevated neonatal BP. It suggests that the initiating events of BP programming may occur during postnatal growth period. To identify the critical starting period that intrauterine growth retardation leads to elevated BP, a study tracking BP changes from birth to childhood is required.

• Journal of Yeungnam Medical Science
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• v.16 no.1
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• pp.60-68
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• 1999

A nationwide survey was conducted to investigate the annual occurrence rate of neonatal sepsis, maternal risk factors in neonatal sepsis, localized infection in neonates, causative organisms in nosocomial infection and the most common causative organism for neonatal sepsis in Korea. Clinical and bacteriological data wele collected from 37 neonatal units to perform retrospective review of the medical records of the newborn infants who were confirmed as having neonatal sepsis and whose blood culture was collected to isolate organisms for one year study period from January to December in 1997. 78,463 neonates were born at 37 hospital in 1997, and 20,869 neonates were admitted to the neonatal units, During this period, 772 episodes of neonatal sepsis were recorded in 517 neonates. The occurrence rate of neonatal sepsis was 0.73%(0~2.95%). Male to female ratio was 1.15:1, and 303 cases(42.1%) were born prematurely. The main pathogens of early onset of sepsis were S. aureus(20%), S. epidermidis(14.4%) and coagulase negative staphylococcus(14. 4%). Gram negative bacilli including Enterobacter spp (7.2%), E. coli(5.1%), Klepstella(4.5%), Pseudomonas(3.7%) and Enterobacter faectum(3.6%) accounted for 24.1% of sepsis. Group B beta-hemolytic streptococcus were isolated only in two cases. Common obstetric factors were PROM(21.1%), difficulty delivery(18.7%), fetal tachycardia(5.3%), chorioamnionitis(4.9%), and maternal fever(4.7%). The main pathogens of late-onset sepsis were S. aureus(22.3%), S. epidermidis(20.4%) and CONS(9.9%). There were 6 cases(1.0%) of Candida sepsis, Frequent focal infections accompanying sepsis were pneumonia(26.1%), urinary tract infection(10.5%), meningitis(8.2%), and arthritis(3.6%), S. epidermidis(22.0%) and s. aureus(21.7%) were also the most common pathogens in 373 nosocomial infection.

• Clinical and Experimental Pediatrics
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• v.52 no.8
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• pp.881-887
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• 2009

Purpose : The survival rate of very low birth weight infant (VLBWI) had increased as a result of advances in neonatal intensive care. We evaluated the changes in outcomes of VLBWI who admitted to the neonatal care unit of Hallym University Kangnam Sacred Heart Hospital. Methods : Retrospective review of 339 VLBWI who were born from 1st January 1997 to 31th December 2008 were performed. Outcomes including survival rate, birth weight (BW), gestational age (GA), morbidities, and mortality between period I (1997- 2003) and period II (2004-2008) were compared. Results : Overall incidence of VLBWI was 2.3% and it was significantly higher in period II(3.3%). Mean BW and GA were significantly decreased in period II (P<0.001, P=0.01). The survival rate increased from period I (59.1%) to period II (74.2%). BW-specific survival rate increased in 1,000-1,249 gm and GA-specific survival rate significantly increased in 27-28 weeks and 29-30 weeks. The incidences of respiratory distress syndrome (RDS), retinopathy of prematurity (ROP), sepsis, bronchopulmonary dysplasia (BPD), intraventricular hemorrhage, periventricular leukomalacia, and necorotizing enterocolitis were same except patent ductus arteriosus. Conclusion : The survival rate of VLBWI was increased in period II, especially in less than 1,000 gm and below 27 weeks. This may be due to recent dramatic improvement of neonatal care. But more efforts are needed to improve outcome during initial phase and to reduce long term complication such as BPD and ROP.

• Clinical and Experimental Pediatrics
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• v.45 no.4
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• pp.535-539
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• 2002

Incontinentia pigmenti is a rare neurocutaneous syndrome characterized by vesiculobullous skin disease in neonates and infants, a noninfectious disease that should be distinguished from infectious diseases with the neonatal seizure or encephalopathy. This disease is X-linked dominant with Xq28 region abnormalities and often associated with developmental defects of the ocular, skeletal, dental, and central nervous system. Central nervous system involvement in the neonatal period, or complicated by encephalopathy, may cause severe neurologic impairment, retardation or even death. We experienced a case of incontinentia pigmenti in a three-day-old female patient who had characteristic papulovesicular skin lesions and partial seizures with secondary generalization. Histopathological examination favored the diagnosis of incontinentia pigmenti and a brain MRI showed undifferentiated white matters with periventricular nodular lesions.

• Asian-Australasian Journal of Animal Sciences
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• v.15 no.7
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• pp.1022-1025
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• 2002

Effect of chemical stress of daily administration of glucocorticoid (dexamethasone @0.125 mg./calf/day) injections on plasma zinc levels, Zn status of body tissues and its distribution in sub cellular fractions, was studied in neonatal buffalo calves. Daily i/m injections of dexamethasone, starting at the completion of 1 week of age and continued till 8th week, led to a significant decline in plasma Zn concentration from 3rd week onwards, which then persisted throughout the rest of the experimental period. In control group, liver had the highest concentration of zinc, followed by heart, muscle, spleen, kidney and testis. In all these tissues, cytosolic fractions had the highest (>60%) zinc levels followed by nuclear, mitochondrial and microsomal fractions. In dexamethasone treated calves, there was a significant increase in the Zn uptake by the tissues of liver and muscle. This increase in zinc concentration was observed in all the sub cellular fractions of liver and muscle, however about 80% of this increase was in cytosolic fraction. It was concluded that glucocorticoid-induced stress caused increase in Zn levels of liver/muscles and decrease in blood plasma zinc, thus indicating a redistribution of Zn in body.

• Childhood Kidney Diseases
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• v.1 no.2
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• pp.161-165
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• 1997

Renal venous thrombosis (RVT) in neonatal period is a rare disease and usually complicated to clinical situations with reduced renal blood flow and hypercoagulability ; like acute blood loss, sepsis, shock, and birth asphyxia. RVT should be suspected in sick babies with hematuria, anemia, thrombocytopenia, enlarged kidney and acute renal failure. And the diagnosis can be confirmed by renal ultrasonography. We report two cases of neonatal renal venous thrombosis with review of literatures. One case, associated with E. coli sepsis, recovered completely, and the other, follwed respiratory distress in the neonate, revealed permanent renal functional impairment.

• Advances in pediatric surgery
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• v.3 no.1
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• pp.41-46
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• 1997

Perforation of the gastrointestinal tract in neonatal period has been associated with a grim prognosis. Recently there has been some improvement in survival. To evaluate the remaining pitfalls in management, 19 neonatal gastrointestinal perforation cases from May 1989 to July 1996 were analysed retrospectively. Seven patients were premature and low birth weight infants. Perforation was most common in the ileum(56.3%). Mechanical or functional obstruction distal to the perforation site was identified in 7 cases; Hirschsprung's disease 3, small bowel atresia 3, and anorectal malformation 1. These lesions were often not diagnosed until operation. Five cases of necrotizing enterocolitis and 1 of muscular defect were the other causes of perforation. In six cases, the cause of the perforation was not identified. Perinatal ischemic episodes were associated in five cases. Overall mortality was 15.1%. Because a considerable number of gastrointestinal perforations resulted from distal obstruction, pediatric surgeon should be alert for early identification and intervention of gastrointestinal obstruction, particularly in patients that are premature and have a history of ischemia.

• Journal of Korean Critical Care Nursing
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• v.10 no.2
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• pp.45-55
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• 2017

Purpose: This study was intended to standardize the Kangaroo care protocol for fathers, and to determine the effect of fathers' Kangaroo care experience on paternal attachment. Methods: The data was collected from February to April, 2013. The study subjects were 34 fathers (17 experimental group subjects, 17 control group subjects) of premature babies, bornatatertiaryhospitalinSeoul, who agreed to participate . The standardized Kangaroo care protocol, which consisted of at least three 60-minutes sessions during the hospitalization period in a neonatal intensive care unit, was carried out with the experimental group. The data was analyzed by a $x^2$-test and Mann-Whitney U test. Results: The results were as follows: 1) There were no between-group differences in the general characteristics of babies and their fathers. 2) The Kangaroo care fathers showed higher scores of paternal attachment than the control group (Z=-3.657, p=0.008). Conclusion: Fathers who attended the Kangaroo care sessions showed stronger paternal attachment than those who did not. Therefore, use of a Kangaroo care program for fathers of premature babies at neonatal intensive care units is recommended.