ABSTRACT
The Korean Neonatal Network (KNN), which is celebrating its 10th anniversary, currently has more than 100 staff and researchers from >70 neonatal intensive care units nationwide in Korea. More than 85% to 90% of the population of very low birth weight infants in Korea are registered yearly, and more than half are followed up for up to 3 years at the KNN. A total of >19,000 cases have been accumulated through a strict data quality management process of the KNN. Based on this, >100 research projects have been proposed, >70 peer-reviewed papers have been published in leading international journals, and the cornerstone of the quality improvement project has been launched. In addition, we recently expanded the number of subjects for registration to very premature infants <32 weeks of age and continue to provide essential evidence data for establishing national health policies, such as linking with government policy projects of the Ministry of Health and Welfare for preterm infants. In such manner, the KNN is confident to be successfully and actively moving toward reaching the ultimate goal of standardizing neonatal intensive care and management of preterm infants in Korea by providing essential data for national health policy establishment along with quality improvement through evidence-based interactive data.
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Background@#In Korea, there have been no reports comparing the prevalence of major congenital anomalies with other countries and no reports on surgical treatment and longterm mortality. We investigated the prevalence of 67 major congenital anomalies in Korea and compared the prevalence with that of the European network of population-based registries for the epidemiological surveillance of congenital anomalies (EUROCAT). We also investigated the mortality and age at death, the proportion of preterm births, and the surgical rate for the 67 major congenital anomalies. @*Methods@#Korean National Health Insurance claim data were obtained for neonates born in 2013–2014 and admitted within one-year-old. Sixty-seven major congenital anomalies were defined by medical diagnoses classified by International Classification of Diseases-10 codes according to the EUROCAT definition version 2014. Mortality and surgery were defined if any death or surgery claim code was confirmed until 2020. Poisson distribution was used to calculate the 95% confidence interval of the congenital anomaly prevalence. @*Results@#The total prevalence of the 67 major anomalies was 433.5/10,000 livebirths. When compared with the prevalence of each major anomaly in EUROCAT, the prevalence of spina bifida, atrial septal defect (ASD), congenital megacolon, hip dislocation and/or dysplasia and skeletal dysplasia were more than five times higher in Korea. In contrast, the prevalence of aortic atresia/interrupted aortic arch and gastroschisis was less than one-fifth in Korea. The proportion of preterm births was 15.7%; however, more than 40% of infants with anencephaly, annular pancreas and gastroschisis were preterm infants. Additionally, 29.2% of the major anomalies were admitted to the neonatal intensive care units at birth, and 25.6% received surgical operation. The mortality rate was 1.7%, and 78.2% of the deaths occurred within the first year of life. However, in neonates with tricuspid valve atresia and stenosis, duodenal atresia or stenosis, and diaphragmatic hernia, more than half died within their first month of life. ASD and ventricular septal defect were the most common anomalies, and trisomy 18 and hypoplastic left heart syndrome were the most fatal anomalies. All infants with aortic atresia/interrupted aortic arch and conjoined twins received surgery. @*Conclusion@#The proportion of surgeries, preterm births and mortality was high in infants with major congenital anomalies. The establishment of a national registry of congenital anomalies and systematic support by national medical policies are needed for infants with major congenital anomalies in Korea.
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Background@#Though antenatal magnesium sulfate (MgSO4 ) is widely used for fetal neuroprotection, suspicions about the long-term neuroprotection of antenatal MgSO4 have been raised. @*Methods@#We investigated short- and long-term outcomes of antenatal MgSO4 use for 468 infants weighing < 1,500 g with a gestational age of 24–31 weeks. @*Results@#Short-term morbidities and the risk of developmental delay, hearing loss, and cerebral palsy at a corrected age of 18–24 months and 3 years of age did not decrease in the MgSO4 group (infants who were exposed to MgSO4 for any purpose) or neuroprotection group (infants who were exposed to MgSO4 for fetal neuroprotection) compared with the control group (infants who were not exposed to MgSO4 ). The z-scores of weight, height, and head circumference did not increase in the MgSO4 group or neuroprotection group compared with the control group. @*Conclusion@#Antenatal MgSO4 including MgSO4 for neuroprotection did not have beneficial effects on long-term neurodevelopmental and growth outcomes.
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Background@#The aim of this study was to capture multifaceted clinical characteristics of congenital cytomegalovirus (CMV) infection from diagnosis to treatment using a multidisciplinary approach including obstetrics, pediatrics, pathology, and otorhinolaryngology-head and neck surgery. @*Methods@#This is a retrospective study including 30 consecutive cases of congenital CMV infection that were diagnosed at a single tertiary hospital located in Seoul, Korea from January 2009 to December 2020. Congenital CMV infection was defined as a positive result by polymerase chain reaction from urine, saliva or cerebrospinal fluid or positive CMV IgM from neonatal blood sampled within 3 weeks after birth. All cases were analyzed with respect to whole clinical characteristics from diagnosis to treatment of congenital CMV by a multidisciplinary approach including prenatal sonographic findings, maternal immune status regarding CMV infection, detailed placental pathology, neonatal clinical manifestation, auditory brainstem response test, and antiviral treatment (ganciclovir or valganciclovir). Long-term outcomes including developmental delay and hearing loss were also investigated. @*Results@#The total number of births during the study period in our institution was 19,385, with the prevalence of congenital infection estimated to be 0.15%. Among 30 cases of congenital CMV, the median gestational age at delivery was 32.2 weeks [range, 22.6–40.0] and 66.7% of these infants were delivered preterm at less than 37 weeks. Suspected fetal growth restriction was the most common prenatal ultrasound finding (50%) followed by ventriculomegaly (17.9%) and abnormal placenta (17.9%), defined as thick placenta with calcification. No abnormal findings on ultrasound examination were observed in one-third of births. Maternal CMV serology tests were conducted in only 8 cases, and one case each of positive and equivocal IgM were found. The most common placental pathologic findings were chronic villitis (66.7%) and calcification (63.0%), whereas viral inclusions were identified in only 22.2%. The most common neonatal manifestations were jaundice (58.6%) followed by elevation of aspartate aminotransferase (55.2%) and thrombocytopenia (51.7%). After excluding cases for which long-term outcomes were unavailable due to death (n = 4) or subsequent follow up loss (n = 3), developmental delay was confirmed in 43.5% of infants (10/23), and hearing loss was confirmed in 42.9% (9/21) during the follow-up period. In our cohort, 56.7% (17/30) of neonates were treated for congenital CMV with ganciclovir or valganciclovir. @*Conclusion@#Our data show that prenatal findings including maternal serologic tests and ultrasound have limited ability to detect congenital CMV in Korea. Given that CMV is associated with high rates of developmental delay and hearing loss in infants, there is an urgent need to develop specific strategies for the definite diagnosis of congenital CMV infection during the perinatal period by a multidisciplinary approach to decrease the risks of neurologic impairment and hearing loss through early antiviral treatment.
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Background@#Herein, we aimed to evaluate the maternal mortality ratio and perinatal mortality rate for different perinatal medical care service areas (PMCSAs), which were established by considering their geographical accessibility to maternal-fetal intensive care units (MFICUs) and neonatal intensive care units (NICUs), and to compare the PMCSAs according to their accessibility to these perinatal care services. @*Methods@#Based on the 70 hospital service areas (HSAs) across the country confirmed through the Dartmouth Atlas methodology analysis and gathering of expert opinions, the PMCSAs were designated by merging HSAs without MFICUs and NICUs to the nearest HSA that contained MFICUs and NICUs, based on which MFICU and NICU could be reached within the shortest amount of time from population-weighted centroids in HSAs. PMCSAs where 30% or more of the population could not access MFICUs and NICUs within 60 minutes were identified using the service module ArcGIS and were defined as having access vulnerability. @*Results@#Thirty-three of 70 HSAs in the country did not contain MFICUs and NICUs, and 39 PMCSAs were finally derived by merging 70 HSAs. Ten of 39 PMCSAs (25.6%) were classified as having access vulnerability to MFICUs and NICUs. The national maternal mortality ratio was 9.42, with the highest ratio seen in the region of Wonju (25.86) and the lowest in Goyang (2.79). The national perinatal mortality rate was 2.86, with the highest and lowest rates observed in the Gunsan (4.04) and Sejong (1.99) regions, respectively. The perinatal mortality rates for areas vulnerable and invulnerable to maternal and neonatal healthcare accessibility were 2.97 and 2.92, respectively, but there was no statistically significant difference in this rate (P = 0.789). The maternal mortality ratio for areas vulnerable and invulnerable to maternal and neonatal healthcare accessibility were 14.28 and 9.48, respectively; this ratio was significantly higher in areas vulnerable to accessibility (P = 0.022). @*Conclusion@#Of the PMCSAs across the country, 25.6% (10/39) were deemed to be vulnerable to MFICU and NICU accessibility. There was no difference in the perinatal mortality rate between the vulnerable and invulnerable areas, but the maternal mortality ratio in vulnerable areas was significantly higher than that in invulnerable areas.
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Background@#We aimed to determine the current survival rate and short-term outcomes of very-low-birth-weight infants (VLBWIs) in Korea, as well as whether the survival rate and short-term outcomes have improved over time since 2013, which was when the Korean Neonatal Network (KNN) was launched. @*Methods@#This study used data from the annual reports of the KNN from 2013 to 2020. A total of 16,351 VLBWIs born at gestational age (GA) ≥ 22 weeks between January 1, 2013, and December 31, 2020, and who were registered in the KNN were enrolled. Serial outcomes were analyzed according to era (2013–14, 2015–16, 2017–18, and 2019–20). @*Results@#More mothers delivered by cesarean section, had diabetes or hypertension during their pregnancy, and received antenatal steroids when analyzed by era. Fewer infants were intubated at birth and had air leaks when analyzed by era. The overall survival rate of VLBWIs between 2013 and 2020 was 87%. The rate of respiratory distress syndrome was 77% and that of bronchopulmonary dysplasia was 32% between 2013 and 2020. The rates of intraventricular hemorrhage (grade ≥ 3), periventricular leukomalacia, and sepsis decreased over time. The survival rate of infants with a GA of 26 weeks has improved serially according to era. @*Conclusion@#Since the launch of the KNN in 2013, the survival rates of infants with GA 26 weeks and short-term outcomes have improved, which implies a quality improvement in antenatal and delivery room care. Further studies on the long-term neurodevelopmental outcomes of these KNN registrants are warranted.
ABSTRACT
Neonatal diabetes mellitus (NDM) is a rare disease that occurs at less than 6 months of age and is presumably caused by a mutation in the gene that affects pancreatic beta-cell function. Approximately 80% of NDM cases reveal a known genetic mutation, and mutations in potassium inwardly rectifying channel subfamily J member 11 (KCNJ11) and ABCC8 affecting the pancreatic beta-cell adenosine triphosphate-sensitive potassium channel may be treated with oral sulfonylurea. Early recognition of mutations in KCNJ11 and ABCC8 is important because early administration of sulfonylurea can not only control blood glucose levels but also improve neurodevelopmental outcomes. In the present study, we report a case of NDM that initially presented as diabetic ketoacidosis at the age of 1 month, accompanied by seizures during hospitalization. After confirmation of the KCNJ11 gene mutation (c.989A>C), we started administering oral sulfonylurea (glimepiride) at the age of 2 months. After gradually increasing the dosage of glimepiride, insulin was discontinued at the age of 3 months. To date, the infant’s blood glucose levels have been well controlled without significant hypoglycemic events. No further episodes of seizures have occurred, and his developmental status is favorable.
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Background@#In accordance with the guidelines for the expectant management of women exposed to previable preterm premature rupture of membrane, we compared neonatal outcomes according to the latent period from membrane rupture to delivery among extremely preterm infants exposed to maternal preterm premature rupture of membrane using the Korean Neonatal Network database. @*Methods@#Of the 3,305 extremely preterm infants born at 23–27 weeks' gestation between 2014 and 2017 who were registered in the Korean Neonatal Network, 1,464 infants were born to pregnant women who were exposed to preterm premature rupture of membrane. The short latency group was defined as infants born with a latent period between membrane rupture and delivery < 7 days (n = 450), whereas the prolonged latency group was defined as infants born with a latent period of ≥ 7 days (n = 434). Using well-established risk factors for adverse short-term outcomes, multivariate logistic regression analysis was performed to assess a prolonged latent period in preterm premature rupture of membrane as an independent risk factor for neonatal outcomes in extremely preterm infants exposed to preterm premature rupture of membrane. @*Results@#The mean gestational age at membrane rupture in the prolonged latency group was significantly lower than that in the short latency group (22.7 ± 2.5 vs. 25.4 ± 1.3 weeks, P < 0.001). Nevertheless, the mean gestational age at delivery and birth weight were not significantly different between the two groups. The incidence of oligohydramnios and histologic chorioamnionitis in the prolonged latency group was significantly higher than that in the short latency group (38.7 [155/401] vs. 26.1 [105/403], 69.8 [270/384] vs. 61.0 [242/397], respectively, P < 0.05). The survival rate in the prolonged latency group did not differ from that in the short latency group (71.2 [309/434] vs. 73.3 [330/450], P = 0.478). Although the prolonged latency group was not associated with mortality during hospitalization in the multivariate logistic regression analysis, the prolonged latency group's early pulmonary hypertension and bronchopulmonary dysplasia rates were increased by 1.8 and 1.5 times, respectively. @*Conclusion@#A prolonged latent period of 7 days or more does not affect the survival rate but increases the risk of bronchopulmonary dysplasia occurrence among extremely preterm infants who are exposed to maternal preterm premature rupture of membrane.
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Background@#Although the overall quality of high-risk neonatal care has improved recently, there is still concern about a difference in the quality of care when comparing off-hour births and regular-hour births. Moreover, there are no data in Korea regarding the impact of time of birth on mortality and morbidities in preterm infants. @*Methods@#A total of 3,220 infants weighing < 1,000 g and born at 23–34 weeks in 2013–2017 were analyzed based on the Korean Neonatal Network data. Mortality and major morbidities were analyzed using logistic regression according to time of birth during off-hours (nighttime, weekend, and holiday) and regular hours. The institutes were sub-grouped into hospital group I and hospital group II based on the neonatal intensive care unit (NICU) care level defined by the mortality rates of < 50% and ≥ 50%, respectively, in infants born at 23–24 weeks' gestation. @*Results@#The number of births during regular hours and off-hours was similar. In the total population and hospital group I, off-hour births were not associated with increased neonatal mortality and morbidities. However, in hospital group II, increased early mortality was found in the off-hour births when compared to regular-hour births. @*Conclusion@#Efforts to improve the overall quality of NICU are required to lower the early mortality rate in off-hour births. Also, other sensitive indexes for the evaluation of quality of NICU care should be further studied.
ABSTRACT
Neonatal diabetes mellitus (NDM) is a rare disease that occurs at less than 6 months of age and is presumably caused by a mutation in the gene that affects pancreatic beta-cell function. Approximately 80% of NDM cases reveal a known genetic mutation, and mutations in potassium inwardly rectifying channel subfamily J member 11 (KCNJ11) and ABCC8 affecting the pancreatic beta-cell adenosine triphosphate-sensitive potassium channel may be treated with oral sulfonylurea. Early recognition of mutations in KCNJ11 and ABCC8 is important because early administration of sulfonylurea can not only control blood glucose levels but also improve neurodevelopmental outcomes. In the present study, we report a case of NDM that initially presented as diabetic ketoacidosis at the age of 1 month, accompanied by seizures during hospitalization. After confirmation of the KCNJ11 gene mutation (c.989A>C), we started administering oral sulfonylurea (glimepiride) at the age of 2 months. After gradually increasing the dosage of glimepiride, insulin was discontinued at the age of 3 months. To date, the infant’s blood glucose levels have been well controlled without significant hypoglycemic events. No further episodes of seizures have occurred, and his developmental status is favorable.
ABSTRACT
Background@#In accordance with the guidelines for the expectant management of women exposed to previable preterm premature rupture of membrane, we compared neonatal outcomes according to the latent period from membrane rupture to delivery among extremely preterm infants exposed to maternal preterm premature rupture of membrane using the Korean Neonatal Network database. @*Methods@#Of the 3,305 extremely preterm infants born at 23–27 weeks' gestation between 2014 and 2017 who were registered in the Korean Neonatal Network, 1,464 infants were born to pregnant women who were exposed to preterm premature rupture of membrane. The short latency group was defined as infants born with a latent period between membrane rupture and delivery < 7 days (n = 450), whereas the prolonged latency group was defined as infants born with a latent period of ≥ 7 days (n = 434). Using well-established risk factors for adverse short-term outcomes, multivariate logistic regression analysis was performed to assess a prolonged latent period in preterm premature rupture of membrane as an independent risk factor for neonatal outcomes in extremely preterm infants exposed to preterm premature rupture of membrane. @*Results@#The mean gestational age at membrane rupture in the prolonged latency group was significantly lower than that in the short latency group (22.7 ± 2.5 vs. 25.4 ± 1.3 weeks, P < 0.001). Nevertheless, the mean gestational age at delivery and birth weight were not significantly different between the two groups. The incidence of oligohydramnios and histologic chorioamnionitis in the prolonged latency group was significantly higher than that in the short latency group (38.7 [155/401] vs. 26.1 [105/403], 69.8 [270/384] vs. 61.0 [242/397], respectively, P < 0.05). The survival rate in the prolonged latency group did not differ from that in the short latency group (71.2 [309/434] vs. 73.3 [330/450], P = 0.478). Although the prolonged latency group was not associated with mortality during hospitalization in the multivariate logistic regression analysis, the prolonged latency group's early pulmonary hypertension and bronchopulmonary dysplasia rates were increased by 1.8 and 1.5 times, respectively. @*Conclusion@#A prolonged latent period of 7 days or more does not affect the survival rate but increases the risk of bronchopulmonary dysplasia occurrence among extremely preterm infants who are exposed to maternal preterm premature rupture of membrane.
ABSTRACT
Background@#Although the overall quality of high-risk neonatal care has improved recently, there is still concern about a difference in the quality of care when comparing off-hour births and regular-hour births. Moreover, there are no data in Korea regarding the impact of time of birth on mortality and morbidities in preterm infants. @*Methods@#A total of 3,220 infants weighing < 1,000 g and born at 23–34 weeks in 2013–2017 were analyzed based on the Korean Neonatal Network data. Mortality and major morbidities were analyzed using logistic regression according to time of birth during off-hours (nighttime, weekend, and holiday) and regular hours. The institutes were sub-grouped into hospital group I and hospital group II based on the neonatal intensive care unit (NICU) care level defined by the mortality rates of < 50% and ≥ 50%, respectively, in infants born at 23–24 weeks' gestation. @*Results@#The number of births during regular hours and off-hours was similar. In the total population and hospital group I, off-hour births were not associated with increased neonatal mortality and morbidities. However, in hospital group II, increased early mortality was found in the off-hour births when compared to regular-hour births. @*Conclusion@#Efforts to improve the overall quality of NICU are required to lower the early mortality rate in off-hour births. Also, other sensitive indexes for the evaluation of quality of NICU care should be further studied.
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Objective@#To evaluate the effects of maternal body mass index (BMI) and plurality on maternal and umbilical cord serum magnesium levels after antenatal magnesium sulfate treatment. @*Methods@#This was a retrospective cohort analysis of 135 women treated with antenatal magnesium sulfate at less than 32 weeks of gestation between January 2012 and June 2018. Subjects were stratified into groups according to maternal BMI (group I [18.5–22.9 kg/m²], group II [23.0–24.9 kg/m²], and group III [≥25.0 kg/m²]) and plurality (singleton and twin). Univariable and multivariable analyses were performed to compare the umbilical cord serum magnesium levels between the groups. @*Results@#Maternal serum magnesium levels were not significantly different between the maternal BMI groups and singleton and twin pregnancies. Umbilical cord serum magnesium levels were significantly different among the maternal BMI groups (3.3±1.2 mg/dL in group I, 3.3±1.2 mg/dL in group II, and 4.0±1.4 mg/dL in group III, P=0.003). The trend of increase in magnesium levels was statistically significant (P=0.001, Jonckheere-Terpstra test). Umbilical cord serum magnesium levels were not significantly different according to plurality. However, in the multivariable analysis, maternal BMI and plurality were not significantly associated with umbilical cord serum magnesium levels after adjusting for indication and total dose of magnesium sulfate treatment, gestational age at delivery, mode of delivery, neonatal sex, and birth weight. @*Conclusion@#Maternal BMI and plurality were not significantly associated with maternal or umbilical cord serum magnesium levels after exposure to antenatal magnesium sulfate treatment.
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Purpose@#To compare respiratory outcomes between less invasive surfactant admi nistration (LISA) and the intubation-surfactant-extubation (INSURE) technique in premature infants with respiratory distress syndrome (RDS). @*Methods@#We performed a retrospective medical chart review for 75 premature in fants who were born at a gestational age (GA) of ≤34 weeks (between January 2017 and December 2019) and developed RDS after birth. Data on the demographic and outcome variables, including respiratory outcomes, were collected and compared between the infants who received LISA and those who received INSURE as a rescue therapy for RDS. @*Results@#No signifcant differences in GA, birth weight, and other demographic characteristics were found between the LISA and INSURE groups (GA: 28.7 weeks vs.28.8 weeks, P=0.449; birth weight: 1,236 g vs. 1,124 g, P=0.714). At the delivery room, although the infants showed no significant difference in positive pressure ventilation rate after birth, the LISA group showed a higher rate of continuous positive airway pressure application than the INSURE group. The infants in the LISA group presented a higher risk of requiring multiple doses of surfactant for RDS than the infants in the INSURE group (57% vs. 17.5%, P=0.001). However, the duration of invasive and/ or noninvasive respiratory support and incidence of bronchopulmonary dysplasia showed no signifciant difference between the two groups. @*Conclusion@#In the present study, no significant differences in the incidence of inhospital respiratory outcomes such as bronchopulmonary dysplasia were found between the LISA and INSURE groups. These results suggest that LISA can be an alternative therapeutic option for treating RDS to avoid intubation and mechanica ventilation in premature infants.
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Purpose@#We aimed to compare two different sedation protocols for brain magnetic resonance imaging (MRI) in preterm infants. One protocol used chloral hydrate (CH) with monitoring conducted by non-anesthesiologists, and the other used a continuous infusion of propofol (PF) with monitoring by anesthesiologists. @*Methods@#A total of 250 preterm infants born between January 2011 and December 2015 who received brain MRI during hospitalization in our neonatal intensive care unit (NICU) were included in this retrospective study. In period 1, sedation for brain MRI was done using a single dose or multiple doses of CH with monitoring conducted by NICU medical staff. In period 2, an anesthesiologist prescribed a continuous infusion of PF and titrated the dosage for minimal and adequate sedation. Data on the adverse events, including desaturation and bradycardia, were collected and compared between periods 1 and 2. @*Results@#Despite similar gestational ages of the patients in periods 1 and 2, the infants in period 1 showed a higher risk of developing bradycardia after sedation compared to those in period 2 (30.2% vs. 14.8%; an adjusted odds ratio of 2.35; 95% confidence interval of 1.12 to 4.91). Infants who had an adverse event after sedation had a lower gestational age and corrected age at the time of MRI (26.8 weeks vs. 27.9 weeks, P=0.004; 37.3 weeks vs. 38.3 weeks, P=0.023). The duration of MRI was significantly longer in infants that had an adverse event than those that did not (70.9 minutes vs.64.3 minutes). After adjusting for various clinical factors, lower gestational age, lower corrected age at the time of MRI, and period 1 increased the risk of developing adverse events after sedation for MRI. @*Conclusion@#The use of a continuous PF infusion with dose titration and monitoring by an anesthesiologist is safe and feasible as a sedation protocol for brain MRI in prematurely born infants.
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Communicating bronchopulmonary foregut malformation (CBPFM) is a communication between the respiratory and gastrointestinal tracts that can be difficult to differentiate from pulmonary sequestration or H-type tracheoesophageal fistula (TEF) because of the similarities in clinical features. A female neonate born at full term had been experiencing respiratory difficulty during feeding from the third day of life. The esophagography performed to rule out H-type TEF revealed that the esophageal bronchus directly communicated with the left lower lobe (LLL) of the lung. Lobectomy of the LLL, fistulectomy of the esophagobronchial fistula, and primary repair of the esophagus were performed. Finally, CBPFM type III with pulmonary sequestration was confirmed on the basis of the postoperative histopathological finding. We report the first newborn case of CBPFM type III with pulmonary sequestration in Korea.
Subject(s)
Female , Humans , Infant, Newborn , Bronchi , Bronchial Fistula , Bronchopulmonary Sequestration , Esophagus , Fistula , Gastrointestinal Tract , Korea , Lung , Tracheoesophageal FistulaABSTRACT
Infantile cortical hyperostosis, or Caffey's disease, usually presents with typical radiological features of soft tissue swelling and cortical thickening of the underlying bone. The disease can be fatal when it presents antenatally, especially before a gestational age of 35 weeks. This fatal, premature form of the disease is known to occur in various ethnic groups around the globe, and approximately 30 cases have been reported in English literature. This paper is unique in that it is the first paper to report a lethal form of prenatal-type infantile cortical hyperostosis diagnosed in South Korea. Born at gestational age of 27 weeks and 4 days, the patient had typical features of polyhydramnios, anasarca, hyperostosis of multiple bones, micrognathia, pulmonary hypoplasia, and hepatomegaly. The patient was hypotonic, and due to pulmonary hypoplasia and persistent pulmonary hypertension, had to be supported with high frequency ventilation throughout the entire hospital course. Due to the disease entity itself, as well as prolonged parenteral nutrition, liver failure progressed, and the patient expired on day 38 when uncontrolled septic shock was superimposed. The chromosome karyotype of the patient was normal, 46, XX, and COL1A1 gene mutation was not detected.
Subject(s)
Humans , Infant, Newborn , Edema , Ethnicity , Gestational Age , Hepatomegaly , High-Frequency Ventilation , Hyperostosis , Hyperostosis, Cortical, Congenital , Hypertension, Pulmonary , Infant, Premature , Karyotype , Korea , Liver Failure , Micrognathism , Parenteral Nutrition , Polyhydramnios , Shock, SepticABSTRACT
BACKGROUND: This study was performed to determine survival and morbidity rates in very low birth weight infants (VLBWIs) in the Korean Neonatal Network (KNN), and to compare neonatal outcomes with those in other countries. METHODS: Data were collected for 8,269 VLBWIs with gestational age (GA) ≥ 22 weeks who were born between January 1, 2013 and December 31, 2016, and admitted to the neonatal intensive care units of the KNN. RESULTS: The survival rate of all VLBWIs and of infants with GA 22–23, 24–25, 26–27, 28–29, 30–32, and > 32 weeks were 86% (total), 33%, 65%, 84%, 94%, 97%, and 98%, respectively. The bronchopulmonary dysplasia (BPD) rates of all VLBWIs and of infants with GA 22–23, 24–25, 26–27, 28–29, 30–32, and > 32 weeks were 30% (total), 88%, 64%, 47%, 26%, 14%, and 5%, respectively. The intraventricular hemorrhage rates (≥ grade III) of all VLBWIs and of infants with GA 22–23, 24–25, 26–27, 28–29, 30–32, and > 32 weeks were 10% (total), 45%, 27%, 12%, 5%, 2%, and 1%, respectively. In an international comparison, the survival rate of VLBWIs with GA 24–27 weeks in KNN was lower, and the BPD rate of VLBWIs in the KNN was higher than that of the neonatal networks of other countries. CONCLUSION: Despite overall improvements in neonatal outcomes, the survival and morbidity rates of more immature infants with GA 22–27 weeks need further improvement. Therefore, it would be necessary to develop more optimal treatment strategies and perform more active quality improvement to further improve neonatal outcomes of VLBWIs in Korea.
Subject(s)
Humans , Infant , Infant, Newborn , Bronchopulmonary Dysplasia , Gestational Age , Hemorrhage , Infant Mortality , Infant, Premature , Infant, Very Low Birth Weight , Intensive Care Units, Neonatal , Korea , Quality Improvement , Survival RateABSTRACT
BACKGROUND: To investigate the incidence of surgical intervention in very low birth weight (VLBW) infants and the impact of surgery on neurodevelopmental outcomes at corrected ages (CAs) of 18–24 months, using data from the Korean Neonatal Network (KNN). METHODS: Data from 7,885 VLBW infants who were born and registered with the KNN between 2013 to 2016 were analyzed in this study. The incidences of various surgical interventions and related morbidities were analyzed. Long-term neurodevelopmental outcomes at CAs of 18–24 months were compared between infants (born during 2013 to 2015, n = 3,777) with and without surgery. RESULTS: A total of 1,509 out of 7,885 (19.1%) infants received surgical interventions during neonatal intensive care unit (NICU) hospitalization. Surgical ligation of patent ductus arteriosus (n = 840) was most frequently performed, followed by laser therapy for retinopathy of prematurity and laparotomy due to intestinal perforation. Infants who underwent surgery had higher mortality rates and greater neurodevelopmental impairment than infants who did not undergo surgery (P value < 0.01, both). On multivariate analysis, single or multiple surgeries increased the risk of neurodevelopmental impairment compared to no surgery with adjusted odds ratios (ORs) of 1.6 with 95% confidence interval (CI) of 1.1–2.6 and 2.3 with 95% CI of 1.1–4.9. CONCLUSION: Approximately one fifth of VLBW infants underwent one or more surgical interventions during NICU hospitalization. The impact of surgical intervention on long-term neurodevelopmental outcomes was sustained over a follow-up of CA 18–24 months. Infants with multiple surgeries had an increased risk of neurodevelopmental impairment compared to infants with single surgeries or no surgeries after adjustment for possible confounders.
Subject(s)
Humans , Infant , Infant, Newborn , Cohort Studies , Ductus Arteriosus, Patent , Follow-Up Studies , Hospitalization , Incidence , Infant, Very Low Birth Weight , Intensive Care, Neonatal , Intestinal Perforation , Korea , Laparotomy , Laser Therapy , Ligation , Mortality , Multivariate Analysis , Odds Ratio , Retinopathy of PrematurityABSTRACT
An important error in the result of Table 3 was confirmed in the article.