• The korean journal of orthodontics
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• v.36 no.4
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• pp.284-294
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• 2006

Objective: Activating mutations in the fibroblast growth factor receptor-2 (FGFR2) have been shown to cause syndromic craniosynostosis such as Apert and Crouzon syndromes. The purpose of this pilot study was to investigate the resultant phenotypes induced by the two distinctive bone-targeted gene constructs of FGFR2, Pro253Arg and Cys278Phe, corresponding to human Apert and Crouzon syndromes respectively. Methods: Wild type and a transgenic mouse model with normal FGFR2 were used as controls to examine the validity of the microinjection. Micro-CT and morphometric analysis on the skull revealed the following results. Results: Both Apert and Crouzon mutants of FGFR2 induced fusion of calvarial sutures and anteroposteriorly constricted facial dimension, with anterior crossbite present only in Apert mice. Apert mice differed from Crouzon mice and transgenic mice with normal FGFR2 in the anterior cranial base flexure and calvarial flexure angle which implies a possible difference in the pathogenesis of the two mutations. In contrast, the transgenic mice with normal FGFR2 displayed normal craniofacial phenotype. Conclusion: Apert and Crouzon mutations appear to lead to genotype-specific phenotypes, possibly causing the distinctive sites and sequence of synostosis in the calvaria and cranial base. The exact function of the altered FGFR2 at each suture needs further investigation.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.100-108
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• 2001

Purpose : We investigated the incidence and predisposing factors of pulmonary embolism in minimal change nephrotic syndrome(MCNS). Methods : Lung perfusion scan using 99mTC-MAA were done on 14 patients who were diagnosed to minimal change nephrotic syndrome. Group h: Five patients who had perfusion defects on scan, Group B; Nine patients who had no perfusion defect on scan. Between the two groups, the differences of platelet number, hematocrits, albumin, cholesterol, triglyceride, proteinuria were evaluated. Results : Five patients were found to have perfusion defect consistent with pulmonary embolism($35.7\%$). However, there were minimal or no respiratory symptoms and signs. In our laboratory studies, the mean proteinuria on admissions was $676{\pm}31\;mg/m2/hr$ in the group with pulmonary embolism, and $313{\pm}28\;mg/m2/hr$ in the group without pulmonary embolism. There were more severe proteinuria in group with pulmonary embolism(P<0.05). The mean platelet count at early stage of remission after steroid treatment was $746,600{\pm}280,000/mm3$ in the group with pulmonary embolism, $511,890{\pm}90,000/mm3$ in the group without pulmonary embolism. There were significant difference of platelet count between the two groups(P<0.01). In patients with pulmonary embolism, there were more higher and sustained increasement of platelet count. All cases of pulmonary embolism were treated with dipyridamole(5 mg/kg). In 4 cases the perfusion defects were improved in two weeks, however, one case showed persistent perfusion defect after 1 month. Conclusion : Our study suggested that pulmonry embolism might be one of tile major complications in childhood MCNS The occurrence rate was correlated with severity of proteinuria before treatment and sustained increasement of platelet counts in early remission state after steroid treatment. Therefore, the scintigraphic pulmonary perfusion study is mandatory in childhood MCNS, especially in the high risk patients, such as the patients with severe proteinuria and sustained increasement of platelet count. (J Korean Soc Pediatr Nephrol 2001;5 : 100-8)

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.7 no.1
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• pp.48-53
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• 2004

Purpose: Besides interferencence of esophageal motor function by the nasogastric tube, a decline of the positive gastro-esophageal pressure gradient caused by intermittent positive pressure ventilation seems to have a major role in the pathogenesis of gastroesophageal reflux (GER) in mechanically ventilated preterm infants. The aim of this study was to determine the incidence of GER and associated risk factors in mechanically ventilated preterm infants. Methods: Twenty four hour esophageal pH monitorings were performed using a antimony electrode on 11 mechanically ventilated preterm infants in Neonatal Intensive Care Unit in Pusan National University Hospital. We evaluated the following reflux parameters; reflux index, reflux episodes/hour, reflux episodes ${\geq}5min/hour$, duration of longest episode, and percent episodes ${\geq}5min$. Patients were considered to have significant GER if more than 2 among 5 parameters were satisfied. Results: The mean gestational age of the patients was 30.9 weeks, mean birth weight was 1,568 g, and mean age at the time of pH monitoring was 2.8 days. Significant GER was detected in 4 patients (36.4%). There was no relationship between the incidence of GER and gestational age, birth weight, postnatal age, or the ventilator settings. Conclusion: The incidence of GER in mechanically ventilated preterm infants was similar, compared with other previous studies. Associated risk factors of GER in these patients were not detected. Therefore, mechanical ventilation in preterm infants does not seem to be the high risk factor of GER.

• Neonatal Medicine
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• v.18 no.1
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• pp.14-22
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• 2011

Neonatal bleeding is a common problem encountered in nursery rooms or neonatal intensive care units, especially among premature infants. Furthermore, owing to recent remarkable improvement of neonatology, survival rates of preterm neonates have increased; hence, neonatal bleeding cannot be emphasized enough. Since the total blood volume of neonates is small, bleeding can be one of the causes of morbidities and mortalities. Therefore, rapid diagnosis and immediate therapy is urgently needed. The patient's medical history including a familial history of a bleeding disorder or of a previously affected infant who suffered from bleeding along with maternal and neonatal drugs can provide important diagnostic clues. Presence of bleeding with or without petechiae and ecchymoses in a healthy term or late preterm infant with thrombocytopenia but normal prothrombin time and activated partial thromboplastin time strongly suggests a congenital bleeding disorder. For a sick infant who is bleeding from multiple sites, an acquired disorder such as disseminated intravascular coagulation is suspected. Intracranial hemorrhage in term or late preterm infants without a history of birth trauma is highly suggestive of coagulation disorders. The purpose of this review is to summarize recent advances in diagnostic methods is as well as basic concepts of neonatal hemostatic disorders. First, an outline of background information will be presented followed by a discussion of primary and secondary hemostatic disorders as well as inherited and acquired disorders.

• Clinical and Experimental Pediatrics
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• v.51 no.9
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• pp.956-963
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• 2008

Purpose : Indomethacin is widely used for the prophylaxis and treatment of patent ductus arteriosus (PDA); however, it is associated with side effects such as renal failure, intraventricular hemorrhage, and gastrointestinal bleeding. Intravenous ibuprofen has been shown to be as effective as indomethacin in prompting PDA closure. If treatment with oral ibuprofen is as effective as indomethacin, it would have the advantages of greater availability, simpler administration, and lower cost. We conducted this study to compare the efficacy and side effects of indomethacin with those of oral ibuprofen, vis-$\grave{a}$-vis on the pharmacological closure of PDA. Methods : As a randomized double-blind study, 34 preterm infants with respiratory distress syndrome and hemodynamically significant PDA were treated with either intravenous indomethacin or oral ibuprofen. Echocardiography was performed by one cardiologist who was blind to the treatment that any given infant received. The rate of ductal closure, the need for additional drug treatment or surgical ligation, clinical outcome, and the side effects of drug treatment were compared. Results : Ductal closure occurred in 16 of 18 patients (88.9%) from the indomethacin group and in 14 of 16 patients (87.5%) from the ibuprofen group (P>0.05). Three patients in the indomethacin group and four in the ibuprofen group required a second drug treatment (P>0.05). Three patients (i.e., one patient in the indomethacin group and two in the ibuprofen group) underwent surgical ligation (P>0.05). Between the two groups, there was no significant difference vis-$\grave{a}$-vis in side effects or clinical outcome. Conclusion : Compared to indomethacin, oral ibuprofen has the advantages of simpler administration and lower cost, while being as effective; in addition, there are no differences between the two drug treatments with regards to side effects or clinical outcomes. Therefore, the widespread use of oral ibuprofen should be considered in treating PDA in preterm infants.

• Journal of Preventive Medicine and Public Health
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• v.22 no.3 s.27
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• pp.368-379
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• 1989

The relationship between mercury level of hair and mental retardation was investigated. The 297 subjects with mental retardation were drawn from two schools providing special educational services, one, consisted of children living in an orphan home, another, children with parents. The 117 centre] subjects were drawn from whom had got average or above average academic achivement in a regular elementary school. Hair sample were taken from the nape of the neck and the mercury analysis was carried out on an atomic absorption spectrophotometer(IL 551). There was no relationship between mercury contents and age, and there was a statistically significant difference in mercury contents between male and female in the mentally retarded children living with parents. Children in the retarded group had significantly higher mercury contents compared with control group except the female group with parents. Also, the mercury levels in the retarded group living in an orphan home were significantly higher than that of the retarded group with parents. The concomitant diseases were Down's syndrome, epilepsy, cerebral palsy and autism. There were statistically significant differences in hair mercury levels in the cases of accompanying Down's syndrome and cerebral palsy in male and Down's syndrome and autism in female compared with the control group of the same sex. The most accompanying handicap was speech disturbance(40.7%) and the others were crippled, emotional disturbance etc. The percentages of double handicap were 66.7% among 6 persons exceeding 6ppm of their hair mercury contents. 10.4% among $3{\sim}6$ ppm and 15.7% among the group of 3ppm or less. The findings of this study suggest that the more opportunities of exposure to mercury in mentally retarded children may have occurred, so it can not be excluded the possibility of mercury as a contributing factor to mental retardation. Therefore, the causal relationship between mercury levels and mental retardation should be established through the examinations about their living environments, dietary pattern, eating habit etc.

• 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.46 no.8
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• pp.769-776
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• 2003

Purpose : The survival rate of very low birth weight infants(VLBWI) has improved by virtue of specialized neonatal care. This study was performed to analyze the changes in incidence, survival rate and morbidity of VLBWI who admitted to Chonnam National University Hospital from 1996 to 2001. Methods : We enrolled 565 VLBWI, and compared the incidence and the survival rate according to the birth weight or gestational weeks between period I(1996 to 1998) and period II(1999 to 2001). The mortality rate according to the postnatal age, cause of death, morbidity and days of hospital stay were also compared. Morbidity is categorized into 'short term' which is curable until discharge, and 'long term' causing any types of sequelae after discharge. Results : Incidence of VLBWI significantly increased in period II over period I(6.0% vs. 11.0%, P< 0.001). The survival rate also increased in period II(71.8% vs. 80.1%, P<0.05), especially in 1,000 to 1,249 gm of birth weight(P<0.001) and in 28 to 30 weeks of gestation(P<0.001). The most common cause of death was respiratory distress syndrome in period I; however it was sepsis in period II. Although overall and short term morbidity rate increased, long term morbidity and days of hospital stay didn't increase in period II. Conclusion : Although the incidence of VLBWI significantly increased and the survival improved in period II compared to period I, especially in 1,000 to 1,249 gm of birth weight and 28 to 30 weeks of gestation, 'long term' morbidity rate and hospital days didn't increase.

• Clinical and Experimental Pediatrics
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• v.51 no.11
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• pp.1205-1210
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• 2008

Purpose : The development of the corpus callosum occupies the entire period of cerebral formation. The myelination pattern on magnetic resonance imaging (MRI) is very useful to evaluate neurologic development and to predict neurologic outcome in high risk infants. The thickness of the corpus callosum is believed to depend on the myelination process. It is possible to calculate the length and thickness of the corpus callosum on MRI. Thus, we can quantitatively evaluate the development of the corpus callosum. We investigated the clinical significance of measuring various portions of the corpus callosum in neonate with neurologic disorders such as hypoxic brain damage and seizure disorder. Methods : Forty-two neonates were evaluated by brain MRI. We measured the size of the genu, body, transitional zone, splenium, and length of the corpus callosum. Each measurement was divided by the total length of the corpus callosum to obtain its corrected size. The ratio of corpus callosal length and the anteroposterior diameter of the brain was also measured. Results : There was no statistical significance in the sample size of each part of the corpus callosum. However, the corrected size or the ratio of body of the corpus callosum correlated with periventricular leukomalacia and hypoxic ischemic encephalopathy. Conclusion : The abnormal size of the corpus callosum showed a good correlation with periventricular leukomalacia and hypoxic ischemic encephalopathy in neonates. We can predict clinical neurological problems by estimation of the corpus callosum in the neonatal period.

• Neonatal Medicine
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• v.16 no.2
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• pp.205-212
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• 2009

Purpose: The purpose of this study was to evaluate the differences according to the hospitals of antenatal care in premature infants. Methods: We retrospectively reviewed the medical records of premature infants with gestational ages <37 weeks and very low birth weights who were admitted immediately after birth to the neonatal intensive care unit (NICU) at the Dongguk University Ilsan Hospital between March 2007 and February 2009. The hospitals of antenatal care were divided into two levels (primary antenatal care hospital: hospitals with less than a level 2 NICU, secondary antenatal care hospital: hospitals with a level 3 NICU) based on the level of NICU in hospitals. In addition, total infants were divided into two groups (Immediate group: infants born within 24 hours of maternal admission, Delayed group: infants born after 24 hours of maternal admission). The differences between maternal and neonatal variables in each groups were studied. Results: Neonates in secondary antenatal care hospitals comprised 11.0% of the study neonates (10 of 91). We compared with two groups (primary antenatal care hospital and secondary antenatal care hospital), but there were no differences in all subjects. However, the 1 minute Apgar score ($\leq3$) was lower in the immediate group than the delayed group. Conclusion: Shorter duration of maternal admission to delivery was associated with a lower 1 minute Apgar score of neonates. These findings suggest that if maintenance of pregnancy is difficult when high-risk gravidas are transferred, clinicians must prepare for emergencies of neonates.