Neurodevelopmental Outcomes and Brain Volumetric Analysis of Low-Grade Intraventricular Hemorrhage

Purpose@#Extremely preterm infants are prone to brain injury and underdevelopment. Intraventricular hemorrhage (IVH) is the most common cause of brain injury and a significant risk factor for neurodevelopmental delay in preterm infants. Severe IVH is known to have a poor outcome; however, the outcomes of low-grade IVH remain controversial. This study aimed to evaluate neurodevelopmental outcomes and brain segmental volumes of preterm infants with low-grade IVH. @*Methods@#This retrospective cohort study included 109 extremely preterm infants who underwent term equivalent age-magnetic resonance imaging and neurodevelopmental evaluation at a corrected age of 18 to 24 months. We compared infants with and without low-grade IVH. @*Results@#Among the 109 extremely preterm infants, 25 had low-grade IVH and 84 had no IVH. There were no significant differences in the neurodevelopmental outcomes between the low-grade and no IVH groups. In multivariate analysis, low-grade IVH was associated with a smaller medullary volume (adjusted odds ratio, 0.575; 95% confidence interval, 0.346 to 0.957; P=0.034). @*Conclusion@#We found no significant differences in the neurodevelopmental outcomes of extremely preterm infants at a corrected age of 18 to 24 months between those with low-grade IVH and those without IVH. Low-grade IVH was associated with a smaller medullary volume.

Minimally Invasive Carpal Tunnel Release Using a Hook Knife through a Small Transverse Carpal Incision: Technique and Outcome

Background@#Compared to the classic open technique, limited exposure techniques have the advantages of less scar pain, more grip and pinch strength, and earlier return to daily life. We evaluated the effectiveness and safety of our novel method of minimally invasive carpal tunnel release using a hook knife through a small transverse carpal incision. @*Methods@#This study included 111 carpal tunnel decompressions in 78 patients who underwent carpal tunnel release from January 2017 to December 2018. We performed carpal tunnel release using a hook knife through a small transverse incision just proximal to the wrist crease under local infiltration of lidocaine with tourniquet inflation in the upper arm. All patients were tolerable during the procedure and discharged following the procedure on the same day. @*Results@#At an average of 29.4 months of follow-up (range, 12–51 months), all but one patient (99%) revealed complete or near complete symptomatic recovery. The average of the symptom severity score on the Boston questionnaire was 1.31 ± 0.30 and the average of the functional status score was 1.19 ± 0.26. The final mean Quick Disabilities of the Arm, Shoulder, and Hand questionnaire (QuickDASH) score was 8.66 (range, 2–39). There was no superficial palmar arch injury or nerve injury of the palmar cutaneous branch, recurrent motor branch, or median nerve itself as a complication of the procedure. No patient showed any wound infection or wound dehiscence. @*Conclusions@#Our carpal tunnel release using a hook knife through a small transverse carpal incision performed by an experienced surgeon is a safe and reliable method that is expected to have the benefits of simplicity and minimal invasiveness.

A Korean Child with Schaaf-Yang Syndrome Presented with Hearing Impairment: A Case Report

Schaaf-Yang syndrome (SYS) is a rare genomic imprinting disorder caused by truncating mutations in the paternally derived MAGE family member L2 (MAGEL2) allele. It is also responsible for Prader-Willi syndrome, characterized by neonatal hypotonia, developmental delay, intellectual disability, respiratory distress in early infancy, and arthrogryposis. More than 250 individuals with approximately 57 different molecular variants have been reported since 2013, but the phenotype-genotype association in SYS is not yet fully understood. Here, we describe the case of a Korean patient diagnosed with SYS harboring a mutation in the paternal allele of MAGEL2: c.2895G>A, resulting in a protein change of p.Trp965*. The patient’s phenotype included respiratory distress, arthrogryposis, hypotonia, and feeding difficulty in the early neonatal period. Mild renal dysfunction and hearing impairment were observed during infancy.

Case Series of Isolated Deep Gray Matter Injuries in Preterm Infants

Hypoxic-ischemic encephalopathy in neonates is an important cause of brain damage that leads to severe neurological sequelae or death. Brain injury patterns on magnetic resonance imaging (MRI) scans are used to predict neurodevelopmental outcome severity. This case series describes the clinical manifestations and neurologic outcomes of four preterm infants with isolated deep gray matter injuries. Basal ganglia and thalamic lesions were noted without white matter and cerebral cortex lesion on brain MRI. All patients were preterm infants born at less than 33 weeks’ gestation and required resuscitation in the delivery room. All had seizures during the neonatal period requiring anti-seizure medications. Severe neurologic disability was identified in three patients using neurodevelopmental assessment tools. Another patient has not been evaluated with assessment tools yet as he was 2 months’ corrected age, but he was supported by home ventilation via a tracheostomy due to insufficient self-respiration. This case series demonstrates that isolated deep gray matter injuries in preterm infants could predict severe neurodevelopmental outcomes.

An infant with subglottic cysts presenting as abruptly-progressed stridor and respiratory distress 2 months after extubation

Subglottic cysts are rare but can cause significant airway obstruction that potentially threatens infants’ lives. It is critically important to suspect subglottic cysts in infants with upper-airway obstructive symptoms because it can be treated effectively with only marsupialization without tracheostomy or complicated surgery. Here, we report an infant case of subglottic cyst, who showed prominent stridor and respiratory distress that abruptly progressed 2 months after extubation. The patient was born prematurely and had a history of endotracheal intubation in for a total of 16 days. He was stable with minimal stridor without respiratory distress until he was successfully discharged from the neonatal intensive care unit. However, he showed abrupt progress in stridor and respiratory difficulty about 2 weeks after discharge. Laryngoscopic exam revealed multiple subglottic cysts obstructing the trachea, and all of them were successfully removed by marsupialization. No recurrence of subglottic cysts was observed for 12 months thereafter. Subglottic cysts should be primarily suspected in infants with stridor, especially with a history of prematurity and intubation. Increased awareness of this fatal but curable condition is needed for timely and proper management.

Disseminated Postnatal Cytomegalovirus Infection in a Preterm Neonate: Autopsy Case Report

Treatment guidelines for postnatal cytomegalovirus (pCMV) infection in preterm have not been established yet. Neutropenia, thrombocytopenia, hepatitis, colitis, and sepsis-like disease are among the clinical manifestations, which range from moderate to serious. We present a case of autopsy diagnosed as pCMV infection in a premature infant delivered at gestational age of 24 weeks and 5 days. On the 7th and 14th days of birth, urinary CMV polymerase chain reaction samples were negative, ruling out congenital CMV infection. However, autopsy examination revealed that the patient had disseminated pCMV infection. CMV inclusion bodies were found in the majority of tissues, including the lung, liver, pancreas, breast, kidney, and adrenal gland, but not the placenta. The thymus exhibited significant cortical atrophy and T-cell immunodeficiency, possibly induced by dexamethasone treatment for bronchopulmonary dysplasia or by pCMV infection itself. If dexamethasone treatment is extended or high doses are considered, it may be beneficial to test the CMV infection status to prevent aggravation of infection. This case demonstrates that, despite the low prevalence, pCMV infection should be considered a differential diagnosis in preterm if other conditions or etiology cannot justify clinical deterioration.

Cognitive and Behavioral Outcomes of School-aged Children Born Extremely Preterm: a Korean Single-center Study with Long-term Follow-up

Background@#School-aged children born very preterm have been suggested to have worse cognitive and behavioral outcomes than children born full-term. Executive function (EF) is a higher level of cognitive function related to academic achievement. The present study aimed to evaluate the cognitive (including EF) and behavioral outcomes of Korean children born extremely preterm (EP) and to analyze any biological or socioeconomic risk factors for poor cognitive outcomes in this population. @*Methods@#A total of 71 infants weighing < 1,000 g at birth or born before 30 weeks of gestation (EP group) who were admitted to the neonatal intensive care unit from 2008 to 2009 were included in this study and compared with 40 term-birth controls. The Korean Wechsler Intelligence Scale for Children-Fourth Edition, Advanced Test of Attention (ATA), Stroop test, Children's Color Trails Test (CCTT), and Wisconsin Card Sorting Test (WCST) were used.Additionally, the Korean Child Behavior Checklist (K-CBCL) and Korean ADHD Rating Scale (K-ARS) were completed. Perinatal and demographic data were collected and analyzed. @*Results@#The mean full-scale intelligence quotient (FSIQ) score in the EP group was significantly lower than that of the term control group (89.1 ± 18.3 vs. 107.1 ± 12.7; P < 0.001).In the EP group, 26 (37%) children had an FSIQ score below 85, compared to only one child (3%) in the control group. Furthermore, the EP group showed significantly worse EF test results (ATA, Stroop test, CCTT, WCST). Except for the higher social immaturity subscore in the EP group, the K-CBCL and K-ARS scores were not different between the two groups. EP children who received laser treatment for retinopathy of prematurity (ROP) had an 8.8-fold increased risk of a low FSIQ score, and a 1-point increase in the discharge weight Z-score decreased the risk of a low FSIQ score by approximately half in this EP cohort. @*Conclusion@#This is the first Korean study to investigate the cognitive and behavioral outcomes of school-aged children born EP. In the study cohort, EP children exhibited significantly lower FSIQ scores and EF than their full-term peers, and 37% of them had cognitive problems. Nonetheless, except for social immaturity, the behavioral problems werenot different in EP children. Severe ROP and low discharge weight Z-score were identified as independent risk factors for low FSIQ score after adjusting for birth weight.

Effectiveness and Biocompatibility of Decellularized Nerve Graft Using an In Vivo Rat Sciatic Nerve Model

BACKGROUND@#Decellularized nerve allografting is one of promising treatment options for nerve defect. As an effort to develop more efficient nerve graft, recently we have developed a new decellularization method for nerve allograft. The aim of this study was to evaluate the effectiveness and biocompatibility of nerve graft decellularized by our newly developed method. @*METHODS@#Forty-eight inbred male Lewis rats were divided into two groups, Group I (autograft group, n = 25), Group II (decellularized isograft group, n = 23). Decellularized nerve grafts were prepared with our newly developed methods using amphoteric detergent and nuclease treatment. Serum cytokine level measurements at 0, 2, and 4 weeks and histologic evaluation for inflammatory cell infiltration at 6 and 16 weeks after nerve graft. @*RESULTS@#There was no significant difference in mean maximum isometric tetanic force and weight of tibialis anterior muscle or ankle angle at toe-off phase between two groups at 6 and 16 weeks survival time points (p > 0.05). There was no inflammatory cell infiltration in either group and histomorphometric assessments of 6- and 16-week specimens of the isograft group did not differ from those in the autograft group with regard to number of fascicle, cross sectional area, fascicle area ratio, and number of regenerated nerve cells. @*CONCLUSION@#Based on inflammatory reaction, axonal regeneration, and functional outcomes, our newly developed decellularized nerve grafts were fairly biocompatible and had comparable effectiveness to autografts for nerve regeneration, which suggested it would be suitable for nerve reconstruction as an alternative to autograft.

Disseminated Postnatal Cytomegalovirus Infection in a Preterm Neonate: Autopsy Case Report

Treatment guidelines for postnatal cytomegalovirus (pCMV) infection in preterm have not been established yet. Neutropenia, thrombocytopenia, hepatitis, colitis, and sepsis-like disease are among the clinical manifestations, which range from moderate to serious. We present a case of autopsy diagnosed as pCMV infection in a premature infant delivered at gestational age of 24 weeks and 5 days. On the 7th and 14th days of birth, urinary CMV polymerase chain reaction samples were negative, ruling out congenital CMV infection. However, autopsy examination revealed that the patient had disseminated pCMV infection. CMV inclusion bodies were found in the majority of tissues, including the lung, liver, pancreas, breast, kidney, and adrenal gland, but not the placenta. The thymus exhibited significant cortical atrophy and T-cell immunodeficiency, possibly induced by dexamethasone treatment for bronchopulmonary dysplasia or by pCMV infection itself. If dexamethasone treatment is extended or high doses are considered, it may be beneficial to test the CMV infection status to prevent aggravation of infection. This case demonstrates that, despite the low prevalence, pCMV infection should be considered a differential diagnosis in preterm if other conditions or etiology cannot justify clinical deterioration.

Cognitive and Behavioral Outcomes of School-aged Children Born Extremely Preterm: a Korean Single-center Study with Long-term Follow-up

Background@#School-aged children born very preterm have been suggested to have worse cognitive and behavioral outcomes than children born full-term. Executive function (EF) is a higher level of cognitive function related to academic achievement. The present study aimed to evaluate the cognitive (including EF) and behavioral outcomes of Korean children born extremely preterm (EP) and to analyze any biological or socioeconomic risk factors for poor cognitive outcomes in this population. @*Methods@#A total of 71 infants weighing < 1,000 g at birth or born before 30 weeks of gestation (EP group) who were admitted to the neonatal intensive care unit from 2008 to 2009 were included in this study and compared with 40 term-birth controls. The Korean Wechsler Intelligence Scale for Children-Fourth Edition, Advanced Test of Attention (ATA), Stroop test, Children's Color Trails Test (CCTT), and Wisconsin Card Sorting Test (WCST) were used.Additionally, the Korean Child Behavior Checklist (K-CBCL) and Korean ADHD Rating Scale (K-ARS) were completed. Perinatal and demographic data were collected and analyzed. @*Results@#The mean full-scale intelligence quotient (FSIQ) score in the EP group was significantly lower than that of the term control group (89.1 ± 18.3 vs. 107.1 ± 12.7; P < 0.001).In the EP group, 26 (37%) children had an FSIQ score below 85, compared to only one child (3%) in the control group. Furthermore, the EP group showed significantly worse EF test results (ATA, Stroop test, CCTT, WCST). Except for the higher social immaturity subscore in the EP group, the K-CBCL and K-ARS scores were not different between the two groups. EP children who received laser treatment for retinopathy of prematurity (ROP) had an 8.8-fold increased risk of a low FSIQ score, and a 1-point increase in the discharge weight Z-score decreased the risk of a low FSIQ score by approximately half in this EP cohort. @*Conclusion@#This is the first Korean study to investigate the cognitive and behavioral outcomes of school-aged children born EP. In the study cohort, EP children exhibited significantly lower FSIQ scores and EF than their full-term peers, and 37% of them had cognitive problems. Nonetheless, except for social immaturity, the behavioral problems werenot different in EP children. Severe ROP and low discharge weight Z-score were identified as independent risk factors for low FSIQ score after adjusting for birth weight.

Effectiveness and Biocompatibility of Decellularized Nerve Graft Using an In Vivo Rat Sciatic Nerve Model

BACKGROUND@#Decellularized nerve allografting is one of promising treatment options for nerve defect. As an effort to develop more efficient nerve graft, recently we have developed a new decellularization method for nerve allograft. The aim of this study was to evaluate the effectiveness and biocompatibility of nerve graft decellularized by our newly developed method. @*METHODS@#Forty-eight inbred male Lewis rats were divided into two groups, Group I (autograft group, n = 25), Group II (decellularized isograft group, n = 23). Decellularized nerve grafts were prepared with our newly developed methods using amphoteric detergent and nuclease treatment. Serum cytokine level measurements at 0, 2, and 4 weeks and histologic evaluation for inflammatory cell infiltration at 6 and 16 weeks after nerve graft. @*RESULTS@#There was no significant difference in mean maximum isometric tetanic force and weight of tibialis anterior muscle or ankle angle at toe-off phase between two groups at 6 and 16 weeks survival time points (p > 0.05). There was no inflammatory cell infiltration in either group and histomorphometric assessments of 6- and 16-week specimens of the isograft group did not differ from those in the autograft group with regard to number of fascicle, cross sectional area, fascicle area ratio, and number of regenerated nerve cells. @*CONCLUSION@#Based on inflammatory reaction, axonal regeneration, and functional outcomes, our newly developed decellularized nerve grafts were fairly biocompatible and had comparable effectiveness to autografts for nerve regeneration, which suggested it would be suitable for nerve reconstruction as an alternative to autograft.

Risk of Down syndrome in duodenal atresia and atrioventricular septal defect: Is there an ethnic difference?

Purpose@#Duodenal atresia (DA) and atrioventricular septal defect (AVSD) are well known ultrasonographic findings associated with Down syndrome. The risk of Down syndrome in fetuses with these anomalies has been reported as 30% to 40%. However, on the basis of our clinical experience, the risk of Down syndrome of DA may be lower in Korean population. To clarify this issue, we compared the risk of Down syndrome between cases with DA and AVSD. @*Materials and Methods@#The study population consisted of neonates who were confirmed as DA or AVSD by postnatal diagnosis. Postnatal diagnosis was made by surgery, postnatal echocardiography, or autopsy. Medical record was reviewed retrospectively. @*Results@#A total of 213 neonates with DA or AVSD were included: 67 cases with DA and 146 cases with AVSD. The risk of Down syndrome was 4.5% (3/67) in DA vs. 29.5% (43/146) in AVSD. When confining analysis to those whose karyotyping were not performed during antenatal period, the risk of Down syndrome were 7.9% (3/38) in DA and 35.4% (35/99) in AVSD. @*Conclusion@#The risk of Down syndrome in cases with DA was much lower in Korean population than previously reported risk in the literature. The significance of some antenatal sonographic markers for Down syndrome may be different according to ethnicity.

A Premature Baby with Severe Oligohydramnios and Hypotension: a Case Report of Renal Tubular Dysgenesis

Renal tubular dysgenesis (RTD) is a rare fatal disorder in which there is poor development of proximal tubules, leading to oligohydramnios and the Potter sequences. RTD occurs secondary to renin-angiotensin system (RAS) blockade during the early stages of fetal development or due to autosomal recessive mutation of genes in the RAS pathway. A boy born at 33+1 weeks due to cord prolapse was found to be anuric and hypotensive. Pregnancy was complicated by severe oligohydramnios from gestational age 28+4 weeks. Abdominal sonography revealed diffuse globular enlargement of both kidneys with increased cortical parenchymal echogenicity. Infantogram showed a narrow thoracic cage and skull X-ray showed large fontanelles and wide sutures suggestive of ossification delay. Basal plasma renin activity was markedly elevated and angiotensin-converting enzyme was undetectable. Despite adequate use of medications, peritoneal dialysis, and respiratory support, he did not recover and expired on the 23rd day of life. At first, autosomal recessive polycystic kidney disease was suspected, but severe oligohydramnios along with refractory hypotension, anuria, skull ossification delay and high renin levels made RTD suspicious. ACE gene analysis revealed compound heterozygous pathogenic variations of c.1454.dupC in exon 9 and c.2141dupA in exon 14, confirming RTD. Based on our findings, we propose that, although rare, RTD should be suspected in patients with severe oligohydramnios and refractory hypotension.

Comparison of Acute Abdominal Surgical Outcomes of Extremely-Low-Birth-Weight Neonates according to Differential Diagnosis

BACKGROUND: Improvements in perinatal intensive care have improved survival of extremely-low-birth-weight (ELBW) neonates, although the risk of acute abdomen has increased. The differential diagnosis resulting in abdominal surgery can be categorized into necrotizing enterocolitis (NEC), spontaneous intestinal perforation (SIP), meconium-related ileus (MRI), and meconium non-related ileus (MNRI). The purpose of this study was to review our experience with abdominal surgery for ELBW neonates, and to evaluate characteristics and prognosis according to the differential diagnosis. METHODS: Medical records of ELBW neonates treated between 2003 and 2015 were retrospectively reviewed. RESULTS: Of 805 ELBW neonates, 65 (8.1%) received abdominal surgery. The numbers of cases by disease category were 29 for NEC, 18 for SIP, 13 for MRI, and 5 for MNRI. Ostoma formation was performed in 61 (93.8%) patients; primary anastomosis without ostoma was performed in 4 (6.2%). All patients without ostoma formation experienced re-perforation of the anastomosis site. Seven patients had 30-day postoperative mortality (6 had NEC). Long-term survival of the surgical and non-surgical groups was not statistically different. NEC was a poor prognostic factor for survival outcome (P = 0.033). CONCLUSION: Abdominal surgery for ELBW neonates is feasible. Ostoma formation can lead to reduced complications compared to primary anastomosis.

Effects of Early Phosphorus Intake on Respiratory Distress in Extremely Low-Birth-Weight Infants

PURPOSE: This study was aimed to investigate the effect of early phosphorus intake on respiratory distress in extremely low-birth-weight infants (ELBWIs) with a high incidence of hypophosphatemia. METHODS: We performed a retrospective study to target 164 ELBWIs admitted to the neonatal intensive care unit in Seoul National University Children's Hospital. Birth characteristics, nutritional intake, and electrolyte levels during the first week were investigated as predictors that would affect the clinical outcomes. The correlations among invasive ventilation at postnatal age of 2 weeks, moderate-to-severe bronchopulmonary dysplasia (BPD), and phosphorous intake were analyzed. RESULTS: Hypophosphatemia (phosphorus level <4 mg/dL) was observed in 72.0% of the subjects. The rates of invasive ventilation (P=0.001) and moderate-to-severe BPD (P=0.005) were significantly lower in the high phosphorus intake group (≥0.7 mM/kg/day) than in the low phosphorus intake group (<0.7 mM/kg/day). Phosphorus intake during the first week was a significant factor affecting invasive ventilation at 2 weeks of age (adjusted odds ratio [OR], 8.212; 95% confidence interval [CI], 2.256 to 28.896; P=0.001) and moderate-to-severe BPD (adjusted OR, 3.402; 95% CI, 1.274 to 9.084; P=0.015). CONCLUSION: Early insufficient phosphorus intake confers a significantly higher risk with invasive ventilation at 2 weeks of age and moderate-to-severe BPD. Therefore, early sufficient phosphorus supply may improve respiratory outcomes in ELBWIs.

A newborn girl with harlequin ichthyosis genetically confirmed by ABCA12 analysis

Harlequin ichthyosis (HI, OMIM #242500) is one of the most severe skin diseases among the autosomal recessive congenital ichthyoses, with high morbidity and mortality, particularly in newborns. Clinically, it is characterized by a typical appearance of generalized, thick, yellowish, hyperkeratotic plates with deep erythematous fissures on the skin. Herein, we present the case of a newborn girl with HI that was genetically confirmed by targeted gene panel analysis. The premature baby was encased in an opaque white membrane with erosion covering the skin of the entire body except the lips, with her hands and feet restricted by the membrane. Humidification, emollient, and retinoic acid treatment were started; the thick ichthyosis gradually peeled off and the underlying skin was only covered with thin scales. Targeted gene panel analysis using next-generation sequencing and validation with Sanger sequencing and quantitative polymerase chain reaction analyses confirmed compound heterozygous mutations of the ABCA12 gene (p.N1380S and a partial gene deletion encompassing exon 9). The parents were carriers for each of the identified mutations. Early recognition of the genetic etiology of congenital ichthyosis can, thus, facilitate genetic counseling for patients and their families.

Antenatal Corticosteroids and Clinical Outcomes of Preterm Singleton Neonates with Intrauterine Growth Restriction

PURPOSE: We assessed the influence of antenatal corticosteroid (ACS) on the inhospital outcomes of intrauterine growth restriction (IUGR) infants. METHODS: A retrospective study was conducted with singletons born at 23⁺⁰ to 33⁺⁶ weeks of gestation at Seoul National University Hospital from 2007 to 2014. We compared clinical outcomes between infants who received ACS 2 to 7 days before birth (complete ACS), at 7 days (incomplete ACS), and those who did not receive ACS in IUGR and AGA infants. Multivariate logistic regression using Firth's penalized likelihood was performed. RESULTS: 304 neonates with 91 IUGR neonates were eligible. Among AGA neonates, mortality (adjusted odds ratio [aOR], 0.13; 95% confidence interval [CI], 0.02 to 0.78), hypotension within 7 postnatal days (aOR, 0.20; 95% CI, 0.06 to 0.64), and severe bronchopulmonary dysplasia (BPD) or death (aOR, 0.24; 95% CI, 0.07 to 0.77) were lower in complete ACS group after adjusting for pregnancy induced hypertension and uncontrolled preterm labor. Mortality (aOR, 0.18; 95% CI, 0.04 to 0.78), hypotension (aOR, 0.26; 95% CI, 0.09 to 0.70), and severe BPD or death (aOR, 0.33; 95% CI, 0.12 to 0.92) were also lower in the incomplete ACS group. Among IUGR infants, after adjusting for birth weight and 5-minute Apgar score, inhaled nitric oxide use within 14 postnatal days was lower in both complete ACS (aOR, 0.07; 95% CI, 0.01 to 0.67) and incomplete ACS (aOR, 0.04; 95% CI, 0.01 to 0.37) groups. CONCLUSION: ACS was not effective in reducing morbidities in IUGR preterm infants.

The Bayley-III Adaptive Behavior and Social-Emotional Scales as Important Predictors of Later School-Age Outcomes of Children Born Preterm

PURPOSE: We aim to assess the Bayley Scales of Infant and Toddler Development, third edition (Bayley-III), Adaptive Behavior (AB) and Social-Emotional (SE) scales at 18 to 24 months of corrected age (CA) to examine their associations with school-age cognitive and behavioral outcomes in children born preterm. METHODS: Eighty-eight infants born with a very low birth weight ( < 1,500 g) or a gestational age of less than 32 weeks who were admitted to the neonatal intensive care unit from 2008 to 2009 were included. Of the 88 children who completed school-age tests at 6 to 8 years of age, 37 were assessed using the Bayley-III, including the AB and SE scales, at 18 to 24 months of CA. Correlation, cross-tabulation, and receiver operating characteristic analyses were performed to assess the longitudinal associations. RESULTS: A significant association was observed between communication scores on the Bayley-III AB scale at 18 to 24 months of CA and the Korean version of the Wechsler Intelligence Scale for Children (K-WISC) full-scale intelligence quotient (FSIQ) at school age (r=0.531). The total behavior problem scores of the Korean version of the Child Behavior Checklist (K-CBCL) at school age were significantly negatively related to the Bayley-III SE and AB scales but not to the cognitive, language, or motor scales. CONCLUSION: Our findings encourage AB and SE assessments during the toddler stage and have important implications for the early identification of children in need of intervention and the establishment of guidelines for follow-up with high-risk infants.

Respiratory Severity Score as a Predictive Factor for the Mortality of Congenital Diaphragmatic Hernia

PURPOSE: Congenital diaphragmatic hernia (CDH) is rare but potentially fatal. The overall outcome is highly variable. This study aimed to identify a simple and dynamic parameter that helps predict the mortality of CDH patients in real time, without invasive tests. METHODS: We conducted a retrospective chart review of 59 CDH cases. Maternal and fetal information included the gestational age at diagnosis, site of defect, presence of liver herniation, and lung-to-head ratio (LHR) at 20 to 29 weeks of gestational age. Information regarding postnatal treatment, including the number of days until surgery, the need for inhaled nitric oxide (iNO), the need for extracorporeal membrane oxygenation (ECMO), and survival, was collected. The highest respiratory severity score (RSS) within 24 hours after birth was also calculated. RESULTS: Statistical analysis showed that a younger gestational age at the initial diagnosis (P < 0.001), a lower LHR (P=0.001), and the presence of liver herniation (P=0.003) were prenatal risk factors for CDH mortality. The RSS and use of iNO and ECMO were significant factors affecting survival. In the multivariate analysis, the only remaining significant risk factor was the highest preoperative RSS within 24 hours after birth (P=0.002). The area under the receiver operating characteristic curve was 0.9375, with a sensitivity of 91.67% and specificity of 83.87% at the RSS cut-off value of 5.2. The positive and negative predictive values were 82.14% and 92.86%, respectively. CONCLUSION: Using the RSS as a prognostic predictor with simple calculations will help clinicians plan CDH management.

Improved Regional Disparities in Neonatal Care by Government-led Policies in Korea

BACKGROUND: Although the number of high-risk neonates has increased in Korea, hospitals were reluctant to open or maintain neonatal intensive care unit (NICU) due to the low medical cost. Consequently, there were regional disparities in facilities, equipment, and neonatal health outcomes. For these reasons, the Korean government began to invest in neonatal care during the last decade. We identified the status of NICUs in Korea and assessed changes after the government-driven policies. METHODS: We surveyed 87 of 89 hospitals that operated NICUs in 2015. The questionnaire assessed the number of NICU beds, admission and mortality rates of very low birthweight infants (VLBWIs), personnel status, equipment and facilities, and available multidisciplinary approach. Current data was compared with the previous studies and changes in the status and function of the nationwide NICU from 2009 and 2011. RESULTS: During the last 7 years, there was an increase of 462 NICU beds, which met the required number estimated by the number of births and covered about 90% of regional VLBWI births. Status of facilities and equipment improved in all regions in Korea but there were still regional differences in multidisciplinary approach and human resources. The difference in odds ratios for mortality of VLBWI between regions decreased compared to 2009. CONCLUSION: There was improvement in regional disparities of neonatal care and mortality of premature babies with the government investment in Korea. Further supports are required for human resources and referral system.