• Clinical and Experimental Pediatrics
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• v.45 no.3
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• pp.383-389
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• 2002

We experienced a case of partial DiGeorge syndrome in a $35^{＋5}$ week premature female infant presented with micrognathia, fish-shaped mouth, beaked nose, nasal regurgitation, obstructive sleep apnea, velopharyngeal insufficiency and late onset hypocalcemic seizures. The chromosome 22q11 microdeletion was found by the FISH method. The lab findings showed serum calcium level of 4.4 mg/dL, ionized calcium level of 0.49 mg/dL, phosphorous level of 7.5 mg/dL, magnesium level of 1.3 mg/dL and PTH-RIA level of <1 pq/mL. Initial treatment was done with 10% calcium gluconate infusion and magnesium sulfate followed by oral calcium gluconate and low phosphorousformula milk feeding. The serum calcium level was normalized in 6 days. Nasal regurgitation, desaturation with obstructive sleep apnea continued. T-cell functions & numbers(CD 3, CD 4, CD 8)were decreased but Ig G/A/M levels were normal. No visible signs of thymus shadow were seen in either chest X-ray & chest MRI. Electrocardiography and echocardiography showed normal heart. Kidney ultrasonographby showed right side mild hydronephrosis. Neurosonography was normal but EEG showed electrical partial seizure. Hearing assessment by BERA showed mild to moderate hearing impairment. Velopharyngoplasty is scheduled for further treatment. A brief review of literature was made.

• Journal of Chest Surgery
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• v.47 no.5
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• pp.444-450
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• 2014

Background: We aimed to evaluate the efficacy and safety of early surgical ligation (within 15 days of age) over late surgical ligation (after 15 days of age) by a comparative analysis of very low birth weight (VLBW) infants undergoing surgical correction for symptomatic patent ductus arteriosus (PDA) over the course of 6 years in our hospital. Methods: We retrospectively reviewed all the medical records in the neonatal intensive care unit at Hanyang University Seoul Hospital, from March 2007 to May 2013, to identify VLBW infants (<1,500 g) who underwent surgical PDA ligation. Results: The gestational age (GA) in the late ligation (LL) group was significantly younger than in the early ligation (EL) group (p=0.010). The other baseline characteristics and preoperative conditions did not differ significantly between the two groups. The intubation period before surgery (p<0.001) and the age at surgery (p<0.001) were significantly different. The postoperative clinical outcomes of the study patients, including major morbidity and mortality, are summarized. There were no significant differences in bronchopulmonary dysplasia, sepsis, or mortality between the EL and the LL groups. However, the LL group was significantly associated with an increased risk of necrotizing enterocolitis (p=0.037) and with a prolonged duration of the total parenteral nutrition (p=0.046) after adjusting for GA. Conclusion: Early surgical ligation for the treatment of PDA that failed to close after medical treatment or in cases contraindicated for medical treatment might be desirable to reduce the incidence of necrotizing enterocolitis and to alleviate feeding intolerance in preterm infants.

• Child Health Nursing Research
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• v.10 no.2
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• pp.165-172
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• 2004

The main purpose of this study is to examine the concepts appeared on research and provide future research directions in field of child health nursing. 205 studies of the total 318 studies had been analyzed first for the concepts of the client domain and this time 113 studies were analyzed for the practice, the client-nurse and the environmental domain in nursing. The practice domain includes mentalistitic, enactment, knowledge utilization, role related phenomenon, the client-nurse domain includes touch, communication, interaction phenomenon, and the environmental domain includes physical, social, symbolic environment. All were originally published between 1990 and 2000 in Korea. An analysis of concepts for this study was used the metaparadigm framework for nursing proposed by H. S. Kim(2000). 1. 103 studies belonged to the practice domain. Among them, 56(54％) studies used concepts related to enactment phenomenon like education(21.4％), giving information(7.1％), breast feeding(5.4％), caring(5.4％), airway suction(5.4％), nonnutritive sucking(5.4％). 44(43％) studies used concepts related to knowledge utilization like program development and evaluation of smoking, mother-infant interaction, home health nursing, obesity management. And only 3(3％) studies used role related concepts like quality of nursing, direction of health education, contents of child health nursing education. 2. Only 2(0.006％) studies belonged to the client-nurse domain. One concept is empathy in communication phenomenon and the other concept is role conflict in interaction phenomenon. 3. 8(0.02％) studies belonged to the environmental domain. Among them, 3 studies related to physical environment like space, noise and 5 studies related to social environment like social support, home environment. But the concept of symbolic environment was not used. The findings of this study provide the evidence that research related to the client-nurse domain and the environmental domain should be conducted actively to improve the practice of child health nursing. So that the research in field of child health nursing should be dealt with the concepts of four domains to develop knowledge systematically.

• Neonatal Medicine
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• v.17 no.2
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• pp.254-261
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• 2010

Little is known about neonatal mitochondrial disease, though mitochondrial metabolic disorders may often present in the neonatal period because of the high energy requirement of neonate. In newborn period, common presentations are not specific and the disease course may be rapid and fatal. In this study, we report three cases of neonatal mitochondrial disease. The first case was strongly suspected because of sudden seizure and mental change with severe lactic acidosis, and multiorgan failure. Plasma lactate/pyruvate (L/P) ratio was increased to 55.6 with marked lactic aciduria and increased plasma alanin up to 2,237 nmol/mL. In the second patient, a peritoneal dialysis was performed for acute adrenal and renal failure, but metabolic acidosis persisted. Plasma L/P ratio was increased to 23.9, and MRC I (mitochondrial respiratory chain defect) was diagnosed through the enzymatic analysis of the muscles. The third case showed repetitive episode of lactic acidosis during the first two months of life, hypotonia, failure to thrive and feeding difficulties. We found markedly increased cerebrospinal fluid L/P ratio up to 57 though plasma L/P ratio(19.4) was borderline with increased plasma lactate. The lactate peak was prominent in brain magnetic resonance spectroscopy (MRS). MRC II was confirmed through muscle biopsy. Plasma lactate level and lactate peak of brain MRS were normalized after conservative treatment.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.12 no.2
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• pp.87-91
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• 2016

Pierre Robin syndrome (PRS) is characterized by the triad of congenital mandibular hypoplasia, glossoptosis and cleft palate. Infant PRS patients are frequently suffering from upper airway obstruction, gastroesophageal reflux and growth retardation caused by above mentioned problems. We report a dental caries treatment of 3-year old girl with Pierre Robin syndrome with multiple caries. The cause of multiple caries was mainly presumed as patient's eating habit caused by her general condition. She had some feeding problems and had history of gastric tube. She was still using milk bottle and took more than an hour to finish a meal. The treatment was performed under general anesthesia considering patient's condition; mild autism, poor cooperation and respiratory problem due to micrognathia. Severely affected upper incisors were treated with pulp treatment and restored with zirconia crown for esthetic purpose. Lower incisors were treated with pulp treatment and restored with composite resin. Upper right first primary molar was restored with stainless steel crown and other primary molars were treated with composite resin. There were no postoperative complications. According to her parents, the patient's compliance to oral hygiene management was greatly improved after the treatment since she was very pleased with the esthetic result and highly motivated by her looks. The treatment without sedation or general anesthesia would be possible once the airway is improved as the mandible grows.

• Korean Journal of Community Nutrition
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• v.3 no.4
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• pp.533-541
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• 1998

This study compares the growth performance(weight-for-age Z-score, height-for-age Z-score, weight-for-height Z-score) of one- to three- month-old Korean infants(n=232) with the Korean standard(1994) and NCHS reference(1983). The weight-for-age Z-scores(WAZ) by the Korean standard were -2∼1,5 for males and -3∼1 for females. The NCHS reference, WAZ results were -1∼2.5 and -2.5∼3.5 for males and females, respectively. The WAZ compared Korean standard showed no subjects with overnutrition. But 1.3% of the infants showed signs of malnutrition. Comparison with NCHS reference revealed that 0.4% of the infants were malnourished and 7.0% of the infants were overnourished. The WAZ of formula-fed infants were distributed higher than breast-fed infants, but that was not significant. The height-for-age Z-score(HAZ) by the Korean standard were -4.5%∼1.5% for males and -4∼1.5% for females. According to the NCHS reference, HAZ were -2.5%∼2.5% and -3∼3 for males and females, respectively. When the WAZ was compared with the Korean standard, there was no overnutrition but 16% of the infants showed signs of malnutrition. The NCHS reference, revealed that 6.9% of the infants were malnourished and 1.8% of the infants were ovemourished. The weight-for-height Z-scores(WHZ) by the Korean standard were -2∼5 for males and -2.5∼5 for females. The NCHS reference WAZ scores were -1∼4 and -1.5∼4.5 for males and females, respectively. When the WHZ was compared with the Korean standard, 2.2% of infants were malnourished and 19.5% were overnourished. There were no malnourished subjects according to the NCHS reference and 19.1% of the infants were overnourished. When the three Z-scores are considered together, 92.0% of the infants should a normal growth status, there was no malnutrition, and 8.0% of the infants were overnourished. The growth performance was evaluated differently according to the type of standards. Thus, it is necessary to set proper growth standards for infants, according to which classification of feeding methods is chosen. A longterm and careful assessment of infants's growth performances to develop any group of standards.

• Clinical and Experimental Pediatrics
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• v.48 no.9
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• pp.953-959
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• 2005

Purpose : The administration of total parenteral nutrition(TPN) has become a standard procedure in the management of nutritionally deprived and critically low birth weight neonates. Sepsis remains the most frequent serious complication during TPN, resulting in increased morbidity, mortality and health care costs. This study was performed to evaluate the clinical efficacy and complications of percutaneous central venous catheterization(PCVC) in very low birth weight infants. Methods : A total of 56 very low birth weight infants below 1,500 g during the period from January 1998 to December 2003 were enrolled and their medical records reviewed. Study group(n=32) included the babies who had undergone PCVC and a control group(n=24) included babies who had not undergone PCVC. We compared the study group with the control group for factors such as subject characteristics and catheter-related complications. Results : There was no difference in subject characteristics, such as birth weight, gestational week, respiratory distress syndrome, duration of ventilator therapy, duration from tube to complete oral feeding, days at TPN and its total duration, body weight at discontinuation of TPN and the days taken to reach to 2,000 g. However, the morbidity rate due to patent ductus arterious, chronic lung disease, necrotizing enterocolitis, osteopenia, cholestasis, and sepsis showed no difference. The study group with infants below 1,000 g showed a higher incidence of sepsis compared to the control group of the same weight group. The study group with infants between 1,000 to 1,500 g showed significantly higher incidences of intraventricular hemorrhage and took longer reach the a body weight of 2,000 g. Conclusion : Considering the high incidence of sepsis in the PCVC group, every attempt should be made to minimize the length of TPN therapy and encourage early enteral feeding. We also recommend the use of PCVC carefully in patients requiring prolonged nutritional support.

• Journal of Korean Society of Environmental Engineers
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• v.34 no.2
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• pp.109-118
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• 2012

This study achieves results from 22 maternity breast milk samples in total to demonstrate exposure level and risk assessment of PBDEs in Seoul area. PBDEs were detected in all the breast milk samples of the present study, indicating that general population in these Seoul area are widely exposed to these chemicals. Residue levels of total PBDEs (sum PBDEs from tri- to hepta-BDE) ranged of 0.84~13.1 ng/g lipid with an arithmetic mean and median of 3.56 ng/g lipid and 2.6 ng/g lipid, respectively. Global comparison shows that the levels of total PBDEs were relatively similar to those of China, Taiwan and European country (Sweden), and somewhat higher than those in some Asian (Vietnam, Philippines, and Indonesia). In the present study, however, the levels of total PBDEs in human milk are still one to two orders of magnitude lower than those in North America. Contribution rate of each congener appeared to be predominant with BDE-47, followed by BDE-153, BDE-100, BDE-99, BDE-154 and BDE-183. The sum of BDE-47 and BDE-153 accounted for more than 65% of total PBDEs in most samples. From the result of the human risk assessment of infants for total PBDEs and BDE-47 by breast milk feeding, we could find out that the average daily doses and hazard index (95th percentile) were 16.5 ng/kg bw/day and $1.2{\times}10^{-1}$ each other when nursing for 6 months after infants born. However, it was concluded that the infant health hardly had adverse seriously effects under this research condition.

• Clinical and Experimental Pediatrics
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• v.52 no.11
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• pp.1216-1220
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• 2009

Purpose:To analyze and compare various cases in which peritoneal drainage was used as the primary treatment method in preterm infants with intestinal perforation. Methods:Among the preterm infants of less than 28 weeks of gestation who were admitted to the neonatal intensive care unit (NICU) at the Gangnam Severance Hospital from April 2006 to April 2009, 7 who had developed intestinal perforation were studied retrospectively. We investigated the clinical characteristics, secondary operation performances, morbidities, complications, and mortalities. Results:Among the 7 infants, 5 survived. Of the 5 cases, 3 received laparotomy, of which 2 were confirmed as having necrotizing enterocolitis. Of the 2 infants who died, 1 had received laparotomy before 48 h of peritoneal drainage, while the other had not received any subsequent treatment. Of the 7 children, 4 had patent ductus arteriosus (PDA), of which 3 had received indomethacin injection. Five infants had begun enteral feeding before they developed intestinal perforation. Of the 5 infants who survived, 4 were diagnosed with cholestasis. Of the 7 infants, 4 developed periventricular leukomalacia (PVL) and 3 developed rickets. Conclusion:Although the use of peritoneal drainage as the primary management of intestinal perforation in preterm infants is controversial, we suggest that it can be used for treating extreme premature neonates. Further randomized controlled study will be required to determine the feasibility of using this method.

• Clinical and Experimental Pediatrics
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• v.49 no.9
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• pp.959-965
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• 2006

Purpose : The purpose of this study was to investigate the effect of prophylactic indomethacin on reduction of patent ductus arteriosus(PDA) and intraventricular hemorrhage(IVH) in extremely low birth weight infants(ELBWI). Methods : Retrospective review of 84 ELBWI who were admitted to our neonatal intensive care unit from June 2004 to April 2006 was performed. Patients were divided into prophylactic group(n=28) and control group(n=56), where prophylactic indomethacin were given within 6 hours after birth. Clinical outcomes were compared between these groups. Results : There were no significant differences in gestational age, birth weight, incidence of hemodynamically significant PDA and severe IVH, and mortality between prophylactic group and control group. However, there were more frequent indications for therapeutic indomethacin, higher incidence of intestinal perforation, and longer time to achieve full enteral feeding in prophylactic group than control group. The incidence of other adverse events attributed to indomethacin prophylaxis did not differ between two groups. Conclusions : Prophylactic indomethacin may not prevent hemodynamically significant PDA and severe IVH in ELBWI. On the contrary, it may be associated with increased risk of adverse events. Further efforts should be investigated to decrease PDA and severe IVH in ELBWI.