Search

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.

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.

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.

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.

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

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.

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 (

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

Purpose: We aimed to evaluate the association between immunoglobulin G (IgG) at birth and late-onset sepsis (LoS) in preterm infants. Methods: Medical records of very-low-birth-weight infants, born at gestational age <28 weeks, between 2013 and 2016, were retrospectively reviewed. Subjects were divided into two groups based on the occurrence of LoS (LoS vs. non-LoS), and IgG levels at 1 day, and at 2 weeks and 4 weeks after birth were investigated. IgG levels, other perinatal factors, and clinical factors were compared in the two groups. The relationship between IgG levels and mortality among infants in the LoS group was also analyzed. Results: A total of 105 infants were analyzed after exclusion of cases with early onset sepsis or death at <72 hours of life. Gestational age in the LoS group was lower than in the non-LoS group (25.0±1.8 vs. 26.3±1.4 weeks, P=0.004). IgG levels at birth were similar between the two groups (236.4±96.4 vs. 282.0±104.7 mg/dL, P=0.078). Multivariate analysis showed that IgG at birth was not an independent risk factor for LoS. In the LoS group, IgG levels at birth were comparable between survivors and cases involving mortality. Conclusion: IgG levels at birth were not associated with the occurrence of LoS in extremely preterm infants. [ABSTRACT FROM AUTHOR]

Purpose: The prevalence of antibiotics resistant bacterial infection among preterm infants has been increased due to indeliberate use of prophylactic broad spectrum antibiotics. The objective of this study was to assess the effectiveness of restricted usage of prophylactic antibiotics by comparing the incidence of culture proven early onset sepsis (EOS). Methods: This was a retrospective cohort study for extremely low birth weight infants who were born in Seoul National University Children's Hospital during 2009-2014. The groups were divided into two periods, from 2009 to 2011 (period I) and from 2012 to 2014 (period II) based on the implementation on quality improvement activity since 2012. The indication of prophylactic antibiotics were; 1) umbilical vein catheter (UVC) insertion for resuscitation in delivery room, 2) prolonged preterm premature rupture of membrane >18 hours, 3) maternal fever during labor or sustained septic amniotic fluid. The incidence of EOS and the rate of empirical antibiotics usage were compared between two periods. Results: A total of 245 infants were admitted to the neonatal intensive care unit during the study period. Baseline demographics and clinical characteristics were similar between two periods except UVC insertion rate. The rate of empirical antibiotics usage significantly decreased in period II (71.1% for period I vs. 56.4% for period 2, P=0.022). Incidence of EOS was not different between two periods whether prophylactic antibiotics use or not. Conclusion: Quality improvement for reducing prophylactic antibiotics use may be effective to reduce a use of antibiotics without increasing EOS. [ABSTRACT FROM AUTHOR]

Purpose: We aimed to evaluate the effect of admission hypothermia on neonatal outcomes in very low birth weight infants (VLBWIs). Methods: Medical records of 153 preterm infants, with birth weights <1,500 g and gestational ages <32 weeks, were retrospectively reviewed. The clinical characteristics and neonatal outcomes in infants who experienced moderate hypothermia during the first hour of life (Group I) were compared to those in infants with mild hypothermia or normothermia (Group II). Results: Fifty of 153 infants experienced moderate hypothermia after birth. Group I had lower birth weight than Group II (867.8±304.4 g vs. 1,140.3±247.5 g, P<0.001), and were younger than Group II (27.6±2.6 weeks vs. 29.1±1.9 weeks, P<0.001). Adjusted proportion of moderate to severe bronchopulmonary dysplasia (BPD) and persistent pulmonary hypertension of newborn (PPHN) were higher in Group I than in Group II (56% vs. 21.8%, P=0.005), (9.1% vs. 1.5%, P=0.019). Multiple logistic regression analysis that did not control for PPHN (model II) showed that gestational age (Odds ratio [OR] 0.93, P=0.001), moderate hypothermia (OR 4.07, P=0.013), and surgical patent ductus arteriosus (OR 4.96, P=0.023) were associated with moderate to severe BPD. Association of moderate hypothermia with moderate to severe BPD was invalid when further multiple logistic regression analysis adjusting for PPHN (model I), which had a strong association with moderate to severe BPD (OR=15.46, P=0.039), was performed. Conclusion: Moderate hypothermia after birth in VLBWIs was associated with PPHN and moderate to severe BPD. The association between moderate hypothermia and moderate to severe BPD might be mediated by PPHN. [ABSTRACT FROM AUTHOR]

Purpose: We compared neurodevelopmental outcomes according to prenatal and postnatal growth patterns in preterm infants and evaluated the proper catch-up growth timing and risk factors that affect poor neurodevelopmental outcomes. Methods: We retrospectively reviewed the electronic medical records of preterm infants born at <32 weeks of gestational age or with <1,500 g of birth weight, who were tested with the Bayley scales of infant and toddler development, third edition (Bayley-III), at 8 and/or 18 months of corrected age in the outpatient clinic. Study populations were divided into four groups according to catch-up growth patterns, which were evaluated about <10th or ≥10th percentile at birth and at 8 or 18 months. Results: In this study, 107 preterm infants were enrolled and 149 results of Bayley-III were analyzed. Infants whose lengths were within <10th percentile at birth had lower cognitive score than those whose lengths were ≥10th percentile at birth (P=0.007). The catch-up growth of length affected cognitive score (P<0.001), and the catch-up growth of head circumference affected cognitive (P<0.001) and motor scores (P= 0.024). The catch-up growth of head circumference by 8 months than that by 18 months was more correlated to cognitive (R2=0.300 vs. 0.266, respectively) and motor development (R2=0.257 vs. 0.210, respectively) at 18 months. Conclusion: Appropriate body length at birth in preterm infants was significantly associated with optimal cognitive development. Catch-up growth of body length was related to cognitive development, while catch-up growth of head circumference was related to both cognitive and motor development. Earlier catch-up growth of head circumference was more critical for neurodevelopment than weight and length. [ABSTRACT FROM AUTHOR]

Purpose: This study aimed to investigate the influence of routine probiotic supplementation on causes of neonatal morbidity and mortality, such as necrotizing enterocolitis (NEC) and late onset sepsis. Methods: All neonates born at <32 weeks of gestation and weighing <1,500 g admitted to the neonatal intensive care unit during the study period were included. The study period was divided into the pre-probiotic period, between January 2009 and February 2011, and the probiotic period, between November 2012 and December 2014. The probiotic given was a mixture of Lactobacillus plantarum, L. rhamnosus, Bifidobacterium lactis and B. longum, administered at the time of the first feeding over 2 mL once daily. Results: A total of 358 infants were screened for enrollment, with 149 infants included in the pre-probiotic group (mean birth weight 937 g, mean gestational age 27.9 wk), and 158 in the probiotic group (1,040 g, 28.6 wk). Probiotics had no statistically significant impact on NEC and late onset sepsis. However, three cases of probiotic related sepsis occurred after the infants were routinely administered probiotics in our unit. Conclusion: Routine probiotic supplementation did not reduce the incidence of NEC in very low birth weight (VLBW) infants. However, severe sepsis was caused by strains in the probiotic administered to patients. Therefore, routine prophylactic use of probiotic in VLBW infants should be performed cautiously. [ABSTRACT FROM AUTHOR]

Probiotics are living micro-organisms that beneficially affect the composition of the host intestinal microflora. In very preterm infants, probiotics reportedly help reduce necrotizing enterocolitis (NEC), invasive fugal colonization and sepsis, and enable the establishment of complete enteral feeding at an earlier stage. However, emerging evidence has indicated the risk of potential side effects of probiotic use, such as gut organism translocation, including probiotic organisms, in infants that are more premature. In the present report, we describe a case of Lactobacillus bacteremia in a very preterm infant with short bowel syndrome. Lactobacillus sepsis developed during the therapeutic use of this organism for diarrhea and diarrhea-related malabsorption. The organism isolated from the blood sample was found to be of the same strain as that administered, by using molecular techniques. The findings of the present case suggest that probiotics should be carefully used, particularly in very preterm infants with altered intestinal permeability such as short bowel syndrome. [ABSTRACT FROM AUTHOR]

Purpose: We aimed to evaluate the effects of two different macrolide prophylaxis protocols (prenatal and postnatal) for Ureaplasma on the development of bronchopulmonary dysplasia (BPD). Methods: We retrospectively reviewed the medical charts of 121 preterm infants whose birth weights were <1,250 g or gestational ages were <30 postmenstrual weeks. The demographic and clinical characteristics, including the presence of BPD, were compared between a prophylactic group, who received macrolide as prophylaxis prenatally and postnatally according to risk level, and a confirmed treatment group, who received macrolide prenatally and postnatally after detection of Ureaplasma infection. Results: Seventy-four (61.2%) of 121 preterm infants were included in the prenatal prophylaxis group. No significant differences in demographic characteristics were observed between the prenatal prophylaxis and prenatal confirmed treatment group. The detection rate of Ureaplasma and the frequency of postnatal therapeutic treatment with macrolide were lower in the prenatal prophylaxis group than in the prenatal confirmed treatment group (16.2% vs. 40.4%, P=0.003; 8.1% vs. 48.9%, P< 0.001, respectively). Although no significant differences in the incidence of moderate to severe BPD, the rate of severe BPD was lower in the prenatal prophylaxis group than in prenatal confirmed treatment group (18.9% vs. 40.4%, P=0.010). No significant differences in the incidences of BPD of any level of severity were observed between the postnatal prophylaxis and confirmed treatment groups. Conclusion: Administration of prenatal prophylaxis with macrolide decreased the detection rate of Ureaplasma after birth and was associated with the decrease in the incidence of severe BPD in preterm infants. [ABSTRACT FROM AUTHOR]

Purpose: This study aimed to evaluate the prognosis of necrotizing enterocolitis (NEC) according to the extent of involvement, among very low birth weight infants. Furthermore, the predictive factors for extent of involvement were evaluated. Methods: Medical records of all newborns with surgically treated NEC admitted to the neonatal intensive care unit of Seoul National University Children's Hospital between 2005 and 2013 were reviewed. Infants were grouped according to the extent of involvement of NEC: isolated segment involvement (ISI, n=31) and multi-segment involvement (MSI, n=17). We evaluated the clinical characteristics, outcomes, and pre-operative factors according to symptoms, laboratory and radiologic findings. Results: The incidence of small for gestational age was significantly higher in the MSI than ISI group (12.9% vs. 41.2%, P=0.036). The length of resected bowel was significantly longer (1.7 cm vs. 8 cm, P=0.010), and the incidence of short bowel syndrome (SBS) (0% vs. 23.1%, P=0.023) and mortality (3.2% vs. 23.5%, P=0.047) were significantly higher in the MSI than ISI group. However, there was no significant difference between the two groups in terms of high-output stoma, time of full enteral feeding, extrauterine growth retardation, changes of z-score of body weight between admission and discharge and reoperation. Portal vein gas detected by ultrasonography was the only statistically significant predictive factor of extent of involvement (odds ratio= 13.237, P=0.029). Conclusion: SBS and mortality were higher in MSI NEC compared to ISI NEC. However, there was no difference in the time of full enteral feeding and growth between the two groups. Portal vein gas detected by ultrasonography maybe a predictive factor of extent of NEC. [ABSTRACT FROM AUTHOR]

Many infants with congenital hypothyroidism have few or no clinical manifestations at birth. In preterm infants, discovering related signs and symptoms of congenital hypothyroidism is even more difficult, despite the higher incidence of transient thyroid function abnormalities. Therefore, these patients need to be closely monitored during the early neonatal period. We report a case of a very low birth weight infant presenting with meconium obstruction concurrent with congenital hypothyroidism in the early neonatal period. Unless diagnosed and treated appropriately, meconium obstruction of prematurity may lead to feeding intolerance, intestinal perforation, necrotizing enterocolitis, and sepsis. It is important for medical caregivers to understand that meconium obstruction can be an early sign of congenital hypothyroidism in preterm infants with abdominal distension. [ABSTRACT FROM AUTHOR]

Purpose: We aimed to evaluate the clinical outcomes of preterm infants with bronchopulmonary dysplasia after tracheostomy. Methods: Medical records of preterm infants with bronchopulmonary dysplasia (BPD) requiring tracheostomy who were admitted to the neonatal intensive care unit of Seoul National University Hospital between January 1999 and December 2013 were reviewed. Data on the age at tracheostomy, indication for the tracheostomy, and the clinical outcomes were collected. Results: Of all admitted preterm infants, 24 (1.0%) were treated with tracheostomy, and the median age at tracheostomy was 185 days. Fifteen patients (62.5%) were weaned from mechanical ventilation after tracheostomy. Of these, 56.5% patients were weaned from positive pressure ventilation (PPV) within 24 months, and 81.3% within 60 months. The median time from the tracheostomy to off-PPV was 15.9 months. Decannulation was achieved in 8 patients (33.3%); of these, 41.5% were decannulated within 24 months, and 69.9% within 60 months. The median time from the tracheostomy to decannulation was 48.8 months. In patients with airway disease, the time from the tracheostomy to off-PPV and decannulation was found to be extended. However, the presence of airway disease was not significantly associated with the duration of mechanical ventilation, or with the time to decannulation. The 5-year survival rate was 80.2% in preterm infants with BPD requiring tracheostomy. Conclusion: Infants with bronchopulmonary dysplasia occasionally require tracheostomy for prolonged mechanical ventilation. In cases with airway disease, the time from the tracheostomy to off-PPV and decannulation tended to increase, although this increase couldn't reach any statistical significance. [ABSTRACT FROM AUTHOR]