• Clinical and Experimental Pediatrics
• /
• v.49 no.1
• /
• pp.29-33
• /
• 2006

Purpose : The aim of this study was to analyze the results and contributing factors of catch-up growth in very low birth weight infants(VLBWI) at the corrected age of 2 years. In addition, we looked for a relationship between the catch-up growth and the development by the bayley scales of infant development II(BSID II). Methods : A retrospective analysis by evaluating medical records was done for the 76 VLBWI whose follow-up was possible up to a corrected age of 2 years, out of the 114 who had been treated in the neonatal intensive care unit during the January of 2000 to December of 2001 at Samsung Cheil Hospital. Based on the Standard Korean Infant's Growth Curve, the catch-up growth group was defined over the 10th percentile of weight at 2 years as the corrected age and the failed catch-up growth group was defined under the 10th percentile. We investigated the clinical factors and courses of each group and compared the scores of the BSID II. Results : The catch-up growth group of the VLBWI was 51 infants and the failed catch-up growth group was 25 infants. In comparison with maternal clinical factors, use of antenatal steroids was significantly higher in the catch-up growth group. In comparisn with clinical factors and courses of VLBWI, two clinical factors were significantly different between the two groups : birth weight and intrauterine growth retardation(IUGR). Numbers of delayed development were increased in the failed catch-up growth group but statistically significant differences could not be observed. Conclusion : The catch-up growth of the VLBWI was affected by the use of antenatal steroids, birth weight and IUGR. No significant differences regarding neurodevelopmental outcome were observed between the catch-up and failed catch-up growth groups.

• Clinical and Experimental Pediatrics
• /
• v.55 no.11
• /
• pp.438-444
• /
• 2012

Mucolipidosis II (ML II) or inclusion cell disease (I-cell disease) is a rarely occurring autosomal recessive lysosomal enzyme-targeting disease. This disease is usually found to occur in individuals aged between 6 and 12 months, with a clinical phenotype resembling that of Hurler syndrome and radiological findings resembling those of dysostosis multiplex. However, we encountered a rare case of an infant with ML II who presented with prenatal skeletal dysplasia and typical clinical features of severe secondary hyperparathyroidism at birth. A female infant was born at $37^{+1}$ weeks of gestation with a birth weight of 1,690 g (<3rd percentile). Prenatal ultrasonographic findings revealed intrauterine growth retardation and skeletal dysplasia. At birth, the patient had characteristic features of ML II, and skeletal radiographs revealed dysostosis multiplex, similar to rickets. In addition, the patient had high levels of alkaline phosphatase and parathyroid hormone, consistent with severe secondary neonatal hyperparathyroidism. The activities of ${\beta}$-D-hexosaminidase and ${\alpha}$-N-acetylglucosaminidase were moderately decreased in the leukocytes but were 5- to 10-fold higher in the plasma. Examination of a placental biopsy specimen showed foamy vacuolar changes in trophoblasts and syncytiotrophoblasts. The diagnosis of ML II was confirmed via GNPTAB genetic testing, which revealed compound heterozygosity of c.3091C>T (p.Arg1031X) and c.3456_3459dupCAAC (p.Ile1154GlnfsX3), the latter being a novel mutation. The infant was treated with vitamin D supplements but expired because of asphyxia at the age of 2 months.

• Korea journal of population studies
• /
• v.35 no.1
• /
• pp.131-149
• /
• 2012

This study examined the effect of parental socioeconomic position on the association between birth outcomes and infant mortality in early and late 2000's. Linked Birth and Infant Death data sets from 2001 to 2003 (T1) and from 2006 to 2008 (T2), provided by the Korea National Statistical Office, were used for analysis. Birth outcomes were categorized into four groups: normal term, small-for-gestational age (SGA), large-for-gestational age (LGA) and intrauterine growth retardation (IUGR). Infant death was defined as the death of a live-born child under one year of age; indicators of parental socioeconomic position were limited to parental education and occupation. The results showed that T2 infant mortality hazard ratios of SGA and IUGR have increased compared to T1. Particularly, preterm and low birth weight babies with shorter gestational age and lighter birth weight than T1 have elevated in T2, possibly indicating that population quality might be continuously aggravated. Moreover, the effect of maternal age on infant mortality has disappeared, rather that of parents' socioeconomic position has increased during the periods, which entailed growing disparities in infant mortality by their social class. Further studies should be therefore done to estimate the effect of parental socioeconomic position on the relationship between birth outcomes and infant mortality in the near future.

• Journal of Preventive Medicine and Public Health
• /
• v.37 no.4
• /
• pp.300-305
• /
• 2004

Objectives : The exposure to particulate air pollution during the pregnancy has reported to result in adverse pregnancy outcome such as low birth weight, preterm birth, still birth, and intrauterine growth retardation (IUGR). We aim to assess whether prenatal exposure of particulate matter less than 10 (m in diameter ($PM_{10}$) is associated with preterm birth in Seoul, South Korea. Methods : We included 382,100 women who delivered a singleton at 25-42 weeks of gestation between 1998 and 2000. We calculated the average PM10 exposures for each trimester period and month of pregnancy, from the first to the ninth months, based on the birth date and gestational age. We used three different models to evaluate the effect of air pollution on preterm birth; the logistic regression model, the generalized additive logistic regression model, and the proportional hazard model. Results : The monthly analysis using logistic regression model suggested that the risks of preterm birth increase with PM10 exposure between the sixth and ninth months of pregnancy and the highest risk was observed in the seventh month (adjusted odds ratio=1.07, 95% CI=1.01-1.14). We also found the similar results using generalized additive model. In the proportional hazard model, the adjusted odds ratio for preterm births due to PM10 exposure of third trimester was 1.04 (95% CI=0.96-1.13) and PM10 exposure between the seventh month and ninth months of pregnancy was associated with the preterm births. Conclusions : We found that there were consistent results when we applied the three different models. These findings suggest that air pollution exposure during the third trimester pregnancy has an adverse effect on preterm birth in South Korea.

• Clinical and Experimental Pediatrics
• /
• v.53 no.3
• /
• pp.432-436
• /
• 2010

Transient neonatal diabetes mellitus (TNDM) has been associated with paternal uniparental isodisomy of chromosome 6, paternally inherited duplication of 6q24, or a methylation defect at a CpG island of the ZAC or HYMAI gene. We experienced a case of TNDM in which the patient presented with hyperglycemia, macroglossia, and intrauterine growth retardation, caused by a paternally derived HYMAI. An 18-day-old female infant was admitted to the hospital because of macroglossia and recurrent hyperglycemia. In addition to the macroglossia, she also presented with large fontanelles, micrognathia, and prominent eyes. Serum glucose levels were 200-00 mg/dL and they improved spontaneously 2 days after admission. To identify the presence of a maternal methylated allele, bisulfite-treated genomic DNA from peripheral blood was prepared and digested with BssHII after polymerase chain reaction (PCR) amplification with methylation-specific HYMAI primers. PCR and restriction fragment length polymorphism analysis showed that the patient had only the paternal origin of the HYMA1 gene. TNDM is associated with a methylation defect in chromosome 6, suggesting that an imprinted gene on chromosome 6 is responsible for this phenotype.

• Childhood Kidney Diseases
• /
• v.4 no.1
• /
• pp.77-83
• /
• 2000

Purpose: Pregnancy in transplanted mother is considered as a high-risk pregnancy, and significant incidences of prematurity and low-birthweight(LBW) infants have been reported. The objective of this study is to examine the outcome of pregnancy in transplanted mothers and to evaluate the current growth status in their children. Patients and Methods: We retrospectively reviewed 54 pregnancies in 40 kidney recipients until June 1999. Outcomes of pregnancy were reviewed and assessment of the current growth status in children was performed. Results: 54 pregnancies in 40 recipients were identified; 22 ended in termination of pregnancy because of unwanted pregnancy or therapeutic purposes. And of the other 32, 29 livebirths resulted in 28 recipients. The mean age of conception was $30.3{\pm}3.8$ years, with a mean interval from transplantation to conception of $35.9{\pm}23.2$ months. All patients were maintained on immunosuppressive regimens. Incidence of drug-treated hypertension(HTN) prior to pregancy was $52\%$, HTN during pregnancy, $48\%$; preeclampsia, $41\%$; urinary tract infection, $48\%$; oligohydramnios $4\%$; and no rejection during pregnancy and up to 3month post delivery. Of the 29 liveborn infants, prematurity(<37wk) occurred in $52\%$, LBW(<2500g) in $62\%$, VLBW(<1500g) in $7\%$ and $48\%$ born intrauterine growth retardation(IUGR). Mean gestational age was $36.3{\pm}3.0\;wk$; a mean birthweight, $2.23{\pm}0.6\;kg$; a mean birth-height, $45.1{\pm}3.6cm$. Current mean height standard deviation score (height SDS) was $0.29{\pm}0.91$ and mean weight SDS was $0.62{\pm}1.34$. Only one child($4\%$) under 1 year of age was below 10 percentile in height. Most of children had no medical problems except for 4 children; cleft palate(1), tuberous sclerosis(1), essential hematuria(1), and one child expired due to sepsis. Conclusion: This study showed similar incidence of premaure birth($57\%$) and low birth weight infants($62\%$), but lower incidence of spontaneous abortion($5.6\%$) was observed and compared to other studies. Postnatal growth in majority of children($96\%$) achieved catch-up growth before 1 year. Present study supports a more optimistic view of pregnancy in renal transplant mother and normal growth in their children.

• Clinical and Experimental Pediatrics
• /
• v.50 no.6
• /
• pp.543-548
• /
• 2007

Purpose : The anthropometric data of newborns published by Lubchenco et al in the 1960's have been most commonly used in Korea as a standard of newborn growth. We hypothesized that Lubchenco's data have limitations for Korean premature infants born in the 2000's. We analyzed and compared the data of birth weight, length, and head circumference. Methods : The medical records of 1,159 premature infants of 26 to 35 weeks of gestational age born at Il-Sin Christian Hospital of Busan from January 2,000 to August 2,006 were reviewed. The anthropometric data from total 1,010 premature infants were analyzed after excluding the data from infants whose gestational age were estimated by other than ultrasonogram, and infants with major congenital anomalies or chromosomal anomaly, born from foreign parent, and extreme outliers. Results : In the birth weights by gestational age, our 90 percentile values were lower than Lubchenco's 90 percentile values for all gestational age studied, particularly for less than 30 weeks the 90 percentile curve was drawn at the area as that of Lubchenco' 75 percentile. And our 10 percentile values were higher than Lubchenco's 10 percentile values for all gestational age studied. In the birth length and head circumference by gestational age, our 90 percentile values were lower than Lubchenco's 90 percentile values for all gestational age studied, and the 90 percentile curve was drawn at the area as that of Lubchenco's 75 percentile. And our 10 percentile values were higher than Lubchenco's 10 percentile values for all gestational age studied. Conclusion : It is unreasonable to apply Lubchenco's data published before 4th decades to present Korean premature infants and have a risk to underestimate intrauterine growth retardation or small for gestational age and large for gestational age. Considering for the possibility of increasing the mortality and morbidity of premature infants due to delayed diagnosis and treatment by these underestimating, our anthropometric data of premature infant is expected to contribute to lower the mortality and morbidity of premature infants.

• Journal of Radiation Protection and Research
• /
• v.21 no.4
• /
• pp.273-284
• /
• 1996

Embryos and fetuses are more sensitive to various environmental agents than are adults or children. The biological effects such as intrauterine death and malformation are closely connected with prenatal exposure very various agents. The sensitivity of these embryonic/fetal effects depends on the stage of pregnancy. From the viewpoint of fetal development, embryonic and fetal stages can be divided into three stages : Preimplantation, organogenetic and fetal. Each stage corresponds to 0 to 4.5days, 4.5 to 13.5days, and 13.5days of gestation in mice, respectively. Many studies on the biologcal effects of mice irradiated by ${\gamma}-rays$ at various stages during organogenesis and fetal period have been performed. Based on these results, the dose-effect and dose-response relationships in malformations, intrauterine death, or retardation of the physical growth have been practically modeled by the ICRP(International Commission on Radiological Protection) and other international bodies for radiation protection. Many experimental studies on mice have made it clear that mice embryos in the preimplantation period have a higher sensitivity to radiation for lethal effects than the embryos/fetuses on other prenatal periods. However, no eratogenic effects of radiation at preimplantation stages of mice have been described in many textbooks. It has been believed that 'all or none action results' for radiation of mice during the preimplantation period were applied. The teratogenic and lethal effects during the preimplantation stage are one of the most important problems from the viewpoint of radiological protection, since the preimplantation stage is the period when the pregnancy itself is not noticed by a pregnant woman. There are many physical or chemical agents which affect embryos/fetuses in the environment. It is assumed that each agents indirectly effects a human. Then, a safety criterion on each agent is determined independently. The pregnant ICR mice on 2, 48, 72 or 96 hours post-conception (hpc), at which are preimplantation stage of embryos, were irradiated whole body Cesium-gamma radiation at doses of 0.1, 0.25, 0.5, 1.5, and 2.5 Gy with dose rate of 0.2 Gy/min. In the embryos from the fetuses from the mice irradiated at various period in preimplantation, embryonic/fetal mortalities, incidence of external gross malformation, fetal body weight and sex ratio were observed at day 18 of gestation. The sensitivity of embryonic mortalities in the mice irradiated at the stage of preimplantation were higher than those in the mice irradiated at the stage of organogenesis. And the more sensitive periods of preimplantation stage for embryonic death were 2 and 48 hpc, at which embryos were one cell and 4 to 7 cell stage, respectively. Many types of the external gross malformations such as exencephaly, cleft palate and anophthalmia were observed in the fetuses from the mice irradiated at 2, 72 and 96 hpc. However, no malformations were observed in the mice irradiated at 48 hpc, at which stage the embryos were about 6 cell stage precompacted embryos. So far, it is believed that the embryos on preimplantation stage are not susceptible to teratogens such as radiation and chemical agents. In this study, the sensitivity for external malformations in the fetuses from the mice irradiated at preimplantation were higher than those in the fetuses on stage of organogenesis.

• Clinical and Experimental Pediatrics
• /
• v.52 no.4
• /
• pp.481-487
• /
• 2009

Purpose : This study investigated the clinical course and prognostic factor of very low birth weight infants (VLBWI) with hemodynamically significant congenital heart defects (CHDs). Methods : Medical records of 1,098 VLBWI with birth weight <1,500 g who had been admitted to the neonatal intensive care unit of Samsung Medical Center from October 1994 to December 2007 were reviewed retrospectively. The data for these patients with hemodynamically significant CHD (n=33) were compared with those without CHD (n=1,065). Results : The incidence of CHD was 3.0% (33 patients) 7 patients (21%) had CHD combined with the congenital abnormalities or chromosomal disorders. The most common CHD was a ventricular septal defect. The incidence of intrauterine growth retardation was higher in patients with CHD than in patients without CHD (34% vs. 20%), but there were no significant differences in gestational age, birth weight, respiratory distress syndrome, bronchopulmonary dysplasia, necrotizing enterocolitis, severe intraventricular hemorrhage (${\geq}$Gr III), and periventricular leukomalacia. Cardiac surgery was performed on 13 patients (39%). Nine patients received staged operations, and 10 patients received early intervention. The overall mortality in patients who had CHD was higher than in the patients who did not have CHD (27% vs. 16%). In patients with CHD, congenital abnormalities or chromosomal disorders were more important factors for increased mortality (86% vs. 11%) than the degree of complexity of CHD (19% vs. 42%). Conclusion : The most important prognostic factors of VLBWI with CHD are the associated congenital abnormalities or chromosomal disorders.

• Neonatal Medicine
• /
• v.18 no.2
• /
• pp.211-220
• /
• 2011

Purpose: Late-onset hypotension in preterm infants is not a rare condition. Late circulatory collapse due to adrenal insufficiency (AI) is one of the major causes of late-onset hypotension. We assessed the incidence and causes of late-onset hypotension. We also compared the clinical findings according to the presence of AI. Methods: In total, 244 preterm infants with a gestational age ${\leq}$32 weeks and who were admitted to the neonatal intensive care unit (NICU) of Seoul National University Boramae Hospital and Seoul National University Hospital from January 2009 to April 2011 were included. Clinical findings were analyzed retrospectively. Results: Forty-four infants (18%) suffered from late-onset hypotension. Hydrocortisone was administered to 30 infants (68.2%) and AI occurred in 16 infants (36.4%). Cesarean section, sepsis before hypotension, and gastrointestinal surgery were independently associated with late-onset hypotension. Intrauterine growth retardation (IUGR) was less frequent in the hydrocortisonetreated group than in infants not treated with hydrocortisone. The AI group had fewer IUGR infants, and the duration of hospitalization was shorter in the AI group than in infants who were not administered hydrocortisone. Blood pressure tended to normalize more quickly in the AI group, however, the difference was not significant. Conclusion: AI was a major cause of late-onset hypotension, and the use of hydrocortisone shortened the length of hospitalization.