• Clinical and Experimental Pediatrics
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• v.46 no.11
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• pp.1073-1079
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• 2003

Purpose : Recently there has been a decrease in ventilator care rate and duration of very low birth weight infants(VLBWI) in Fatima Hospital. The aims of this study were to survey the frequency and duration of ventilation in VLBWI and to develop a non-invasive neonatal intensive care unit (NICU) policy. Methods : We performed a retrospective study of 284 newborn of infants less than 1,500 gm admitted to NICU and discharged from January 1998 to December 2001. Patients were intubated or applied continuous positive airway pressure(CPAP) via nasal prong immediately after presenting signs of respiratory distress. We analyzed epidemiologic data to study the changes in ventilator care rate, duration and outcome of ventilator care groups. Results : Of 284 newborn infants, 146 required invasive management, such as endotracheal intubation and assisted ventilation. The characteristics, the severity of clinical symptoms and laboratory findings in ventilator care groups at birth showed no significant differences. The annual proportion of infants requiring assisted ventilation decreased according to increasing gestational age. The median duration of ventilation decreased markedly from 6.0 days in 1998 to 2.7 days in 2001. Final complications and outcomes in ventilator care groups showed no significant differences. Conclusion : Our study shows a significant reduction in the invasiveness of the treatment of VLBW infants, which was not associated with an increased mortality or morbidity. A non-invasive strategy for the VLBW infant with minimal to moderate respiratory distress after birth in NICU is better than immediate invasive management. Non-invasive nasal CPAP is a simpler and safer method than invasive assisted ventilation.

• Clinical and Experimental Pediatrics
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• v.46 no.12
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• pp.1207-1211
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• 2003

Purpose : This study was performed to observe the relationship of plasma L-arginine level and the severity of disease and pulmonary artery pressure in respiratory distress syndrome of premature infants. Methods : Peripheral blood samples were obtained at 1st, 3rd and 7th day from 21 premature infants with respiratory distress syndrome to analyze the L-arginine concentration. Oxygenation index (OI), an indicator of the severity of the disease, was calculated at the same time of the blood sampling. And pulmonary artery pressure was measured by Doppler echocardiography at each period. Plasma L-arginine level, OI and right ventricular systolic time interval(RVSTI) were analyzed. Results : Plasma L-arginine concentration of the 1st day was lower than 3rd and 7th day. OI and RVSTI were significantly correlated with each other(r=0.772, P<0.01). Plasma L- arginine level was correlated with oxygenation index(r=-0.346, P<0.01) and RVSTI(r=-0.416, P<0.01). Conclusion : Plasma L-arginine level was lowest in the 1st day during the study period. Plasma L-arginine concentration correlated significantly with the severity of respiratory distress syndrome and pulmonary artery pressure in premature infants.

• Journal of the korean academy of Pediatric Dentistry
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• v.35 no.2
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• pp.319-323
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• 2008

Edward's syndrome is a genetic disorder of 18th chromosomal trisomy. Main clinical features of this syndrome include systemic conditions, such as mental retardation, growth retardation, dyspnea, congenital heart disease, and orthopedic disorders, such as flexion deformity and rocker-bottom feet. The characteristics of fetal abnormality are hydramnios, small placenta, common umbilical artery. Infants with Edward's syndrome have very low survival rate. Almost half of them die during fetal stage. Fifty percent of them survive 2 months, and 5 to 10 percent of them survive about 1 year. A 3-year and 7 month old girl visited our dental hospital for the treatment of dental caries. Considering her systemic disease, low body weight, medical history of cardiac surgery, and difficulty in airway management, dental procedure was performed under general anesthesia. We report this case with review of literatures.

• Neonatal Medicine
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• v.17 no.1
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• pp.116-122
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• 2010

Kasabach-Merritt syndrome is a rare thrombocytopenic consumptive coagulopathy associated with a giant hemangioma. We experienced a case of unexplained ascites with thrombocytopenia in a 32 week premature infant. An exploratory laparotomy was performed to determine the cause of the refractory ascites and thrombocytopenia. An intestinal hemangioma was found, but, surgical removal was not performed due to the extensive involvement. Hemangioma was confirmed by SPECT (single-photon emission computed tomography) and the thrombocytopenia was treated with steroid therapy. It is recommended that hemangioma of the visceral organs should be suspected when unexplained thrombocytopenia and disseminated intravascular coagulopathy persist.

• Journal of Chest Surgery
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• v.34 no.11
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• pp.879-882
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• 2001

advancement in the treatment of esophageal perforation due to the development of ICU care and antibiotics. There were controversies in the treatment of esophageal perforation when diagnosed after 24hrs. From 1995 to 2000, we performed a buttressed primary repair and mediastinal drainage in 6 Boerhaave\`s syndrome patients among 13 esophageal perforation patients. Two patients died(33%). They died because of pneumonia, ARDS and sepsis on 38th, 39th post-operative day respectively. Two patients had leak at the site of repair which was treated completely with conservative treatment. We report on the result of a buttressed primary repair and mediastinal drainage for 6 Boerhavve\`s syndrome patients.

• Journal of the Korean Society of Radiology
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• v.81 no.6
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• pp.1478-1485
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• 2020

Leigh syndrome or subacute necrotizing encephalomyelopathy is a rare, rapidly progressive neurodegenerative disorder. In general, symptoms such as shortness of breath and decreased cardiac function usually occur within 1 year of life. It is a serious disease with a mortality rate of 75% in 2-3 years. The cause of Leigh syndrome is DNA mutation. Approximately 75% of patients have nuclear DNA mutations while 25% have mitochondrial DNA mutations. Clinical symptoms vary depending on the affected brain area. Neuroimaging plays an important role in diagnosing patients with Leigh syndrome. Late-onset Leigh syndrome is rarer and progresses more slowly compared to the classic form. Here, we report a case of late-onset Leigh's syndrome mimicking Wernicke's encephalopathy.

• Clinical and Experimental Pediatrics
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• v.48 no.2
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• pp.148-153
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• 2005

Purpose : The aim of this study was to determine the reference ranges of serum albumin levels depending on the gestational ages of preterm infants. We also intended to compare the mean serum albumin levels between groups of preterm infants that did not develop clinical disorders later, and groups that did develop clinical disorders such as respiratory distress syndrome, intraventricular hemorrhage, retinopathy of prematurity, apnea and bronchopulmonary dysplasia. We also examined the significance of serum albumin as a predictor for the development of clinical disorders. Methods : The records of 208 neonates with gestational ages from 23 weeks to 41 weeks were reviewed retrospectively. The mean albumin concentrations with reference ranges by gestational ages were determined. Statistics for each two of group were compared. Logistic regression analysis was used to model odd ratio, and 95 percent confidence interval as a mean of the association between predictors and outcome. Results : Serum albumin levels were at 23-24 weeks gestation was 2.36 g/dL, rising to 3.43 g/dL in full term babies. There were significant mean differences between the clinical groups and control groups for each clinical disorder such as respiratory distress syndrome, intraventricular hemorrhage, retinopathy of prematurity and apnea in premature babies of 30-36 weeks of gestation. Low serum albumin appeared to be associated with increased risks of clinical disorders. Conclusion : The normal serum albumin levels in preterm infants should be defined according to the gestational ages. Lower albumin levels increase the risks of the later development of clinical disorders, which are common in premature infants.

• Neonatal Medicine
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• v.18 no.2
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• pp.395-398
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• 2011

Sick sinus syndrome (SSS) is a disorder characterized by sinus node dysfunction. Although the condition is most common in the elderly, it can occur in children including neonates and its recognition and treatment are important. The diagnosis of SSS is based on the presence of sinus bradycardia, sinus arrest or exit block, combinations of sinoatrial and atrioventricular nodal conduction disturbances, and atrial tachyarrhythmias documented in the Holter recordings. In most children with SSS, previous history of congenital heart malformation or cardiac surgery is noted. SSS is also seen in the children including neonates without heart disease or other contributing factors, however SSS is most often idiopathic. The treatment of SSS depends on the basic rhythm problem, but generally involves the placement of a cardiac pacemaker. We report a case of SSS in extremely low birth weight infant without congenital heart disease and suggest that the treatment system is necessary for preterm infants with SSS.

• Journal of Chest Surgery
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• v.34 no.9
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• pp.720-723
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• 2001

A case of Marfan’s syndrome with atresia of right coronary artery is reported. A 45-year-old woman, who was diagnosed as Marfan’s syndrome 1 year ago, came to the hospital complaining of acute chest pain. The patient showed arachynodactyly, pectus carinatum, and long and slender extremities. In echocardiography there were severe aortic regurgitation measured grade IV and aortic dilatation of ascending aorta maximally 5.9 cm in diameter. Mitral regurgitation was mild, but there were also moderate left ventricular dilation and moderately decreased ejection fraction of left ventricle. At operation, atresia of right coronary artery was found. We performed Bentall type operation with SJM 27mm valved conduit for left coronary artery, and Piehler’s modification for right coronary artery bypass using 6mm PTFE graft. The atretic portion of right coronary artery from the suspected right coronary ostium to distal coronary flow was about 4 cm in length. The combination of right coronary artery atresia and Marfan’s syndrome is very rare. The author describes the rare case, which is treated with combined technique of Bentall and Piehler modification for reconstruction of coronary circulation.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.7 no.2
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• pp.228-238
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• 2004

Purpose: Obesity is rapidly increasing in Korean children. Obesity is a risk factor for cardiovascular morbidity and is frequently associated with hypertension, diabetes mellitus and coronary artery disease. This study was designed to evaluate risk factors of the metabolic syndrome in obese children. Methods: From February 2000 to June 2004, eighty eight obese (body mass index ${\geq}95th$ percentile) children aged 4 to 15 years were included. We measured serum lipid levels (total cholesterol, triglyceride, HDL cholesterol, LDL cholesterol), fasting sugar levels and insulin levels. Insulin resistance was determined by homeostasis model assessment, fasting insulin/glucose ratio and quantitative insulin sensitivity check index. Results: Clustering of risk factors for the metabolic syndrome in obese children demonstrated that 60.2% had more than one risk factors. Hypertension (14.8%), hypertriglyceridemia (14.8%), HDL-hypocholesterolemia (14.8%), LDL-hypercholesterolemia (12.5%) and hyperinsulinemia (12.5%) were observed. As BMI increased, there was statistically significant increase in systolic blood pressure, insulin and insulin resistance values. Insulin resistance was correlated to systolic blood pressure, serum lipid and insulin levels. The more risk factors for the metabolic syndrome obese children had, the higher was their insulin resistance. Conclusion: The increase in insulin resistance and clustering of risk factors for the metabolic syndrome are already apparent in obese children. Monitoring these risk factors for the metabolic syndrome should become a part of routine medical care for obese children.