Ventricular Tachycardia in Neonates: A Report of Two Cases and Review of the Literature

In the case of hemodynamically stable broad complex tachycardia, it is essential to evaluate the causes and to diagnose correctly in the selection of appropriate management and drugs. We report two neonates diagnosed with idiopathic ventricular tachycardia, which is extraordinarily rare. One presented with idiopathic fascicular ventricular tachycardia (right bundle branch block pattern with a superior axis), and the other presented with right ventricular outflow tract ventricular tachycardia (left bundle branch block pattern with an inferior axis). These two forms are representative of benign ventricular tachycardia. No features of cardiovascular shock were observed. While the recommended initial drug treatments are different, the conditions were well controlled by propranolol without the development of any adverse events. There was no recurrence of arrhythmia for several months in the outpatient clinic.

A Neonate Diagnosed with Wolff-Parkinson-White Syndrome Presenting with Cardiogenic Shock

We present the case of a healthy 28-day-old female full-term neonate who was admitted to the neonatal intensive care unit for severe metabolic acidosis, hypoglycemia, and an initial sinus rhythm. The first diagnostic hypothesis was hypovolemic shock, and fluid resuscitation was started immediately. During fluid therapy, cardiovascular collapse occurred with supraventricular tachycardia. The latter was successfully treated with adenosine and beta-blockers. After 8 days, electrocardiography showed ventricular pre-excitation, and Wolff-Parkinson-White syndrome was diagnosed. A novel variant of the MYL2 gene that is related to hypertrophic cardiomyopathy and conduction defect was found after discharge. Cardiogenic shock should be considered, despite being a rare cause of shock in neonates.

Oral Administration of Nonionic Water-Soluble Contrast Media to Treat Meconium Obstruction in Premature Infants: A Preliminary Study

Purpose@#Meconium obstruction of prematurity (MOP) predisposes premature infants to intestinal perforation and prolonged hospitalization if not diagnosed and treated promptly. A standard contrast enema is less effective to treat infants with distal ileal obstructions because the contrast may not reach the obstructed areas. In an effort to avoid risky surgery, we administered oral contrast media to seven clinically diagnosed patients with MOP whose obstructions were not relieved via conventional sonography-guided contrast enema. We retrospectively evaluated whether oral nonionic water-soluble contrast media relieves MOP. @*Methods@#Seven of 67 premature infants with MOP were administered oral contrast media from June 2015 to January 2019. Patients were followed-up radiographically for bowel distention and evacuation of contrast media after oral administration. We recorded radiographic improvements, meconium evacuation, time to first feeding after oral contrast media administration, maternal history, and neonatal clinical factors. @*Results@#We evaluated five male and two female infants. The median gestational ages and body weights at birth were 27+5 weeks and 890 g, respectively. Radiography in five infants revealed multiple distended intestinal loops without air-fluid interfaces. Two infants had gasless abdomens, in which only stomach gas was visible. Oral contrast media (median, 2.5 mL) were administered at a median age of 7 days; five infants (5/7, 71.4%) responded to this treatment. The remaining two infants, who had ileal stenosis and hypoganglionosis, were surgically managed. Five infants (5/7, 71.4%) had maternal risk factors, and two (28.6%) were small for gestational age. @*Conclusion@#Nonionic oral water-soluble contrast medium can serve as a valuable adjunct treatment in premature infants with meconium obstruction.

Ventricular Tachycardia in Neonates: A Report of Two Cases and Review of the Literature

In the case of hemodynamically stable broad complex tachycardia, it is essential to evaluate the causes and to diagnose correctly in the selection of appropriate management and drugs. We report two neonates diagnosed with idiopathic ventricular tachycardia, which is extraordinarily rare. One presented with idiopathic fascicular ventricular tachycardia (right bundle branch block pattern with a superior axis), and the other presented with right ventricular outflow tract ventricular tachycardia (left bundle branch block pattern with an inferior axis). These two forms are representative of benign ventricular tachycardia. No features of cardiovascular shock were observed. While the recommended initial drug treatments are different, the conditions were well controlled by propranolol without the development of any adverse events. There was no recurrence of arrhythmia for several months in the outpatient clinic.

A Neonate Diagnosed with Wolff-Parkinson-White Syndrome Presenting with Cardiogenic Shock

We present the case of a healthy 28-day-old female full-term neonate who was admitted to the neonatal intensive care unit for severe metabolic acidosis, hypoglycemia, and an initial sinus rhythm. The first diagnostic hypothesis was hypovolemic shock, and fluid resuscitation was started immediately. During fluid therapy, cardiovascular collapse occurred with supraventricular tachycardia. The latter was successfully treated with adenosine and beta-blockers. After 8 days, electrocardiography showed ventricular pre-excitation, and Wolff-Parkinson-White syndrome was diagnosed. A novel variant of the MYL2 gene that is related to hypertrophic cardiomyopathy and conduction defect was found after discharge. Cardiogenic shock should be considered, despite being a rare cause of shock in neonates.

Oral Administration of Nonionic Water-Soluble Contrast Media to Treat Meconium Obstruction in Premature Infants: A Preliminary Study

Purpose@#Meconium obstruction of prematurity (MOP) predisposes premature infants to intestinal perforation and prolonged hospitalization if not diagnosed and treated promptly. A standard contrast enema is less effective to treat infants with distal ileal obstructions because the contrast may not reach the obstructed areas. In an effort to avoid risky surgery, we administered oral contrast media to seven clinically diagnosed patients with MOP whose obstructions were not relieved via conventional sonography-guided contrast enema. We retrospectively evaluated whether oral nonionic water-soluble contrast media relieves MOP. @*Methods@#Seven of 67 premature infants with MOP were administered oral contrast media from June 2015 to January 2019. Patients were followed-up radiographically for bowel distention and evacuation of contrast media after oral administration. We recorded radiographic improvements, meconium evacuation, time to first feeding after oral contrast media administration, maternal history, and neonatal clinical factors. @*Results@#We evaluated five male and two female infants. The median gestational ages and body weights at birth were 27+5 weeks and 890 g, respectively. Radiography in five infants revealed multiple distended intestinal loops without air-fluid interfaces. Two infants had gasless abdomens, in which only stomach gas was visible. Oral contrast media (median, 2.5 mL) were administered at a median age of 7 days; five infants (5/7, 71.4%) responded to this treatment. The remaining two infants, who had ileal stenosis and hypoganglionosis, were surgically managed. Five infants (5/7, 71.4%) had maternal risk factors, and two (28.6%) were small for gestational age. @*Conclusion@#Nonionic oral water-soluble contrast medium can serve as a valuable adjunct treatment in premature infants with meconium obstruction.

MRI Findings to Predict Neurodevelopmental Outcomes in Preterm Infants Near Term-Equivalent Age

Purpose@#Preterm infants are at high risk for adverse neurodevelopmental outcomes.Magnetic resonance imaging (MRI) has been proposed as a means of predicting neurodevelopmental outcomes in this population. It is controversial whether diffuse excessive high signal intensity (DEHSI) represents damage to the white matter or delayed myelination in preterm infants. This study investigated MRI findings for predicting the severity of neurodevelopmental outcomes and assessing whether preterm infants with DEHSI near term-equivalent age have abnormal neurodevelopmental outcomes. @*Materials and Methods@#Preterm infants (n = 64, gestational age at birth < 35 weeks) undergoing brain MRI near term-equivalent age and subsequent neurodevelopmental outcomes were evaluated between 18 and 24 months of age. The associations of MRI findings and the risk of severe cognitive delay, severe psychomotor delay, cerebral palsy (CP), and neurosensory impairment were analyzed.The associations of DEHSI with risks of severe cognitive delay, severe psychomotor delay, CP, and neurosensory impairment (hearing or visual impairment) were analyzed.Outcome data were evaluated by logistic regression and the Fisher’s exact test. @*Results@#There were significant associations between abnormal white matter findings and delayed mental development, delayed psychomotor development, neurosensory impairment, and presence of CP. The presence of DEHSI was not correlated with delayed neurodevelopmental outcomes or presence of CP. In multivariate logistic regression analyses, cystic encephalomalacia, punctate lesion, loss of white matter volume and ventricular dilation were significantly associated with CP. @*Conclusion@#Abnormal MRI findings near term-equivalent age in preterm infants predict adverse neurodevelopmental outcomes. No significant association between DEHSI and adverse neurodevelopmental outcomes was demonstrated.

Association between vitamin D level at birth and respiratory morbidities in very-low-birth-weight infants / 소아과

PURPOSE: This study aimed to evaluate vitamin D status at birth in very-low-birth-weight infants (VLBWIs: <1,500 g) and to determine the association between vitamin D level and respiratory morbidity. METHODS: A retrospective study was conducted at Soonchunhyang University Bucheon Hospital between November 2013 and November 2017. We collected blood samples and data on respiratory morbidity from 230 VLBWIs on the first day of life. Patients who were transferred to other hospitals (n=19), died before 36 weeks of gestational age (n=18), or whose blood samples were not collected immediately after birth (n=5) were excluded. Finally, 188 patients were enrolled. VLBWIs with different vitamin D levels were compared with respect to demographic features, maternal diseases, respiratory morbidities, and other neonatal diseases. RESULTS: The mean serum vitamin D level, as measured by 25-hydroxyvitamin D (25(OH)D), was 13.4±9.3 ng/mL. The incidence of vitamin D deficiency (<20 ng/mL) was 79.8%, and 44.1% of preterm infants had severe vitamin D deficiency (<10 ng/mL). Logistic analysis shows that a low serum 25(OH)D level (<20 ng/mL) was a risk factor for respiratory distress syndrome (odds ratio [OR], 4.32; P=0.010) and bronchopulmonary dysplasia (OR, 4.11; P=0.035). CONCLUSION: The results showed that 79.8% of preterm infants in this study had vitamin D deficiency at birth. Low vitamin D status was associated with respiratory morbidity, but the exact mechanism was unknown. Additional studies on the association between vitamin D level and neonatal morbidity are required.

Measles outbreaks in the Kyeongin area of the Republic of Korea, 2013-2014: A single-center experience in a country of measles elimination

OBJECTIVE@#To identify the source of infection and determine the clinical features and laboratory finding of measles infection.@*METHODS@#In 27 measles patients, except for 3 adult patients, the rest of 24 pediatric measles cases were analyzed with regard to age, sex, immunization status, transmission routes and molecular genotyping of measles virus. Eighteen measles patients who admitted in isolation ward were set apart to investigate clinical findings and its correlation with laboratory characteristics. Retrospective analysis of cases was conducted in this study.@*RESULTS@#Of the 24 pediatric patients, 23 (95.8%) had not received any measles-containing vaccine (MCV). Sixteen of the patients (66.7%) were aged <12 months. The suspicious index case of a girl aged 34 months was not vaccinated with MCV1 and got measles after a trip to Philippines, and molecular genotype was revealed as B3. Measles outbreaks in the community such as a restaurant were followed by this one imported case. According to analysis of 18 patients admitted in isolation ward, the median level of C-reactive protein (CRP) was 0.38 mg/dL and that of lactate dehydrogenase (LDH) was 1200 IU/L. All of the 18 patients had LDH levels above the normal range. Age correlated with CRP (ρ = 0.528, P = 0.024) and LDH (ρ = 0.501, P = 0.034). The duration of fever was correlated with the duration of fever before rash (ρ = 0.898, P < 0.01). The duration of hospitalization was correlated with CRP (ρ = 0.586, P = 0.011). The white blood cell counts were correlated with the levels of LDH (ρ = 0.505, P = 0.033), aspartate aminotransferase (ρ = 0.507, P = 0.032), and alanine aminotransferase (ρ = 0.481, P = 0.043).@*CONCLUSIONS@#Early weaning of maternally derived measles antibodies therefore vaccination of MCV1 at a young age from 9 months to 12 months should be considered in situations of early exposure. Furthermore, there is a call for consideration of scheduling an earlier age for the first dose of MMR vaccine in Europe. It is necessary for Korea to investigate the duration of the presence and quantitative analysis of maternal measles antibodies in infants and to reconsider the timing of MCV1.

Measles outbreaks in the Kyeongin area of the Republic of Korea, 2013–2014: A single-center experience in a country of measles elimination

Objective To identify the source of infection and determine the clinical features and laboratory finding of measles infection. Methods In 27 measles patients, except for 3 adult patients, the rest of 24 pediatric measles cases were analyzed with regard to age, sex, immunization status, transmission routes and molecular genotyping of measles virus. Eighteen measles patients who admitted in isolation ward were set apart to investigate clinical findings and its correlation with laboratory characteristics. Retrospective analysis of cases was conducted in this study. Results Of the 24 pediatric patients, 23 (95.8%) had not received any measles-containing vaccine (MCV). Sixteen of the patients (66.7%) were aged <12 months. The suspicious index case of a girl aged 34 months was not vaccinated with MCV1 and got measles after a trip to Philippines, and molecular genotype was revealed as B3. Measles outbreaks in the community such as a restaurant were followed by this one imported case. According to analysis of 18 patients admitted in isolation ward, the median level of C-reactive protein (CRP) was 0.38 mg/dL and that of lactate dehydrogenase (LDH) was 1200 IU/L. All of the 18 patients had LDH levels above the normal range. Age correlated with CRP (ρ = 0.528, P = 0.024) and LDH (ρ = 0.501, P = 0.034). The duration of fever was correlated with the duration of fever before rash (ρ = 0.898, P < 0.01). The duration of hospitalization was correlated with CRP (ρ = 0.586, P = 0.011). The white blood cell counts were correlated with the levels of LDH (ρ = 0.505, P = 0.033), aspartate aminotransferase (ρ = 0.507, P = 0.032), and alanine aminotransferase (ρ = 0.481, P = 0.043). Conclusions Early weaning of maternally derived measles antibodies therefore vaccination of MCV1 at a young age from 9 months to 12 months should be considered in situations of early exposure. Furthermore, there is a call for consideration of scheduling an earlier age for the first dose of MMR vaccine in Europe. It is necessary for Korea to investigate the duration of the presence and quantitative analysis of maternal measles antibodies in infants and to reconsider the timing of MCV1.

Immature Gastric Teratoma in a Newborn: A Case Report

A teratoma is the most common germ cell tumor in children; however, a gastric teratoma is very rare. An immature gastric teratoma has malignant potential; therefore, it should be removed surgically and followed up routinely to assess for recurrence by performing imaging studies and estimating serum alpha-fetoprotein (AFP) level. We describe the case of a 2-day-old male neonate with abdominal distension and a palpable mass. He underwent surgical resection of a tumor that was diagnosed as an immature gastric teratoma.

Deaths in the Neonatal Intensive Care Unit between 2002 and 2014

PURPOSE: To report the causes and patterns of death among infants admitted to our neonatal intensive care unit (NICU) over a 13-year period. In addition, we analyzed trends regarding the type of end-of-life care provided. METHODS: All of the neonates who died at the Soonchunhyang University Bucheon Hospital between January 1, 2002, and December 31, 2014, were identified. The causes and circumstances of death were extracted from individual medical records. Trends in mortality were compared between two time periods: 2002 to 2007 and 2008 to 2014. RESULTS: Of the 5,223 admissions to our NICU, 97 neonates died. The overall mortality rate was 1.9%. The most common cause of death was sepsis (15%). At a lower gestational age, infants died of extreme prematurity and complications of prematurity. Among term infants, the principal cause of death shifted to hypoxic ischemic encephalopathy and asphyxia. A total of 63 infants (64.9%) received maximal intensive care, and 34 infants (35%) had redirection of intensive care. During this period, the proportion of death after redirection of care increased from 30.6% to 39.6%. Infants decided to forgo life-sustaining care before death had significantly lower gestational ages and lower birth weights (30.5 vs. 27.1 weeks, P=0.005; 1,528 vs. 1,063 g, P=0.025). CONCLUSION: Infection remained an important cause of death for neonate, particularly for preterm infants. The proportion of infants who had redirectoin of care before death was increased, suggesting that quality-of-life should be considered an important factor in the decision-making process for the infant, parents, and medical staff.

Primary Adrenal Insufficiency in a Newborn With Adrenal Hypoplasia Congenita Caused by a Mutation of the DAX1 Gene

Adrenal hypoplasia congenita (AHC) is a rare inherited disorder of the adrenal gland caused by deletion or mutation of the dosage-sensitive sex-reversal AHC critical region on the X chromosome, gene 1 (DAX1) gene. The DAX1 gene is expressed in the adrenal cortex, the pituitary gland, the hypothalamus, the testis, and the ovary. Most affected infants present with failure to thrive, salt wasting, and hypoglycemic seizure in early life. Immediate mineralocorticoid and glucocorticoid replacement is essential. Most boys with AHC present with hypogonadotropic hypogonadism, resulting in failure to enter puberty and the need for testosterone treatment. However, a recent study revealed that the onset of puberty in boys with AHC can be variable, ranging from arrested or absent to precocious. We describe a case involving a newborn who presented with primary adrenal insufficiency due to a mutation of the DAX1 gene and was finally diagnosed with AHC.

Characteristics And Risk Factors Of Newborn Infants Who Visited Emergency Department

PURPOSE: This study aimed to improve emergency care for future neonatal patients. METHODS: We conducted a clinical analysis of neonatal patients who visited the emergency department of Soonchunhyang University Bucheon Hospital from January 2004 to December 2012. We reviewed the medical records of 972 neonates less than 28 days of age. RESULTS: The male:female ratio was 1.2:1. In terms of patient inflow, the peak month was September (10.3%), the peak day of the week was Saturday (205%), and the peak time of the day was 18:00-23:59 (39.0%). The most common symptom was fever (17.7%), and final outcomes were as follows: approved discharge (50.1%), admittance to the hospital (37.6%), transfer to another hospital (3.2%), discharge against medical advice (3.0%) and death (0.1%). There was no difference in the number of visits for irritability based on sex, age of the child, maternal age, parity, gestation, birth weight, type of birth, or season. However, the proportion of discharges was significantly higher in the group comprising the irritable infants (77.4%) than in the group comprising non-irritable infants (50.4%) (P<0.001). CONCLUSION: A newborn's transition from intrauterine to extrauterine life involve numerous physiologic and biochemical changes. Some visits are due to non-serious diseases, and reflect insufficient knowledge and information of the parents; this highlights the pressing need for parents' education.

Effect of Prophylactic Palivizumab on Admission Due to Respiratory Syncytial Virus Infection in Former Very Low Birth Weight Infants with Bronchopulmonary Dysplasia

The aim of this study was to observe the effects of prophylactic palivizumab on hospitalization secondary to respiratory syncytial virus (RSV) infection (RSVhospitalization) in former very low birth weight infants (VLBWI) with bronchopulmonary dysplasia (BPD). This study also sought to identify the risk factors of RSVhospitalizationin this particular infant population. A prospective observational study was conducted between September 2007 and April 2008 in seven Korean hospitals. Children with a history of very low birth weight, a diagnosis of BPD and who were <2 yr old at the onset of the RSV season were included in this study. Palivizumab injections were administered monthly for a maximum of five months during the RSV season. RSVhospitalization rates were reviewed, and RSVhospitalization rates between subgroups were categorized by gestational age, birth weight, and duration of ventilator care. A total of 90 subjects completed the follow-up interviews. The mean gestational age at birth was 26.1+/-1.7 weeks, and the mean birth weight was 889.4+/-222.2 g. The incidence of RSVhospitalization in the study population was 8.9% (8/90), and the mean hospital stay was 11.0+/-5.5 days, including one death. There were no statistically significant differences in the patients' demographic characteristics or risk factors for RSV hospitalization. When subgroup analyses were conducted, there were still no statistically significant differences. The administration of palivizumab prophylaxis during the entire RSV season is important in VLBWI with BPD, regardless of their gestational age and birth weight, or previous ventilator dependency.

Postnatal weight gain in the first two weeks as a predicting factor of severe retinopathy of prematurity requiring treatment / 소아과

PURPOSE: This study aimed to investigate the relative weight gain at 2-week intervals up to 6 weeks after birth to predict retinopathy of prematurity (ROP) requiring treatment among very low birth weight infants. METHODS: A total of 211 preterm infants with birth weights <1,500 g and gestational age <32 weeks were retrospectively reviewed. The main outcome was the development of ROP requiring treatment. Body weight measurements were recorded daily. Relative weight gains (g/kg/day) were calculated at the second, fourth, and sixth week after birth. RESULTS: Of the 211 infants, 89 developed ROP, of which 41 spontaneously regressed and 48 with early treatment of ROP type I required laser treatment. The relative weight gain at 2, 4, and 6 weeks postnatal age was significantly lower in infants with ROP requiring treatment than in infants without ROP or those with spontaneous regression (P<0.001, P=0.005, and P=0.004, respectively). On logistic regression, poor relative weight gain in the first 2 weeks was found to be related to ROP requiring treatment (adjusted odds ratio, 0.809; 95% confidence interval, 0.695-0.941; P=0.006). Relative weight gain at 2 weeks postnatal age was significantly lower in infants with ROP requiring treatment compared to that in ROP requiring no treatment (P=0.012). CONCLUSION: Poor postnatal weight gain in the first 2 weeks of life is an important and independent risk factor for ROP requiring treatment. Postnatal weight gain can predict the development of severe ROP requiring treatment.

Food Protein-Induced Enterocolitis Syndrome in a Neonate with Peripheral Epimerase Deficiency Galactosemia

Food protein-induced enterocolitis syndrome (FPIES) is a severe infantile form of non-immunoglobulin E-mediated gastrointestinal food hypersensitivity that manifests as profuse, repetitive vomiting, often with diarrhea, which leads to acute dehydration and lethargy and failure to thrive if chronic. Symptoms such as dehydration and lethargy are also observed in sepsis, viral infection, and food poisoning. It is difficult to differentiate FPIES from sepsis-like illness. The diagnosis is based on clinical criteria and/or an oral food challenge. FPIES developed in the patient with peripheral epimerase deficiency galactosemia after the use of soy formula. The change in feeding to soy formula is not required of a patient with peripheral epimerase deficiency galactosemia. Early intake of soy formula in our patient was harmful. Therefore, we think the changing the formula should be taken carefully. Another important point is the diagnosis. Late diagnosis and misdiagnosis are common, and inappropriate treatment or invasive treatment can occur.

Metameric Arteriovenous Malformation in the Spinal Cord of a Neonate

We present a case of metameric arteriovenous malformation (AVM) in a neonate. The neonate appeared normal, except for a dimple and blue discoloration in the lower thoracic spine. Spinal ultrasonography revealed cutaneomeningiospinalangiomatosis in the thoracic spine. Spinal magnetic resonance imaging revealed a definite metameric AVM at the T10-T11 level and a cutaneous hemangioma at T11. The neonate was conclusively diagnosed with spinal metameric AVM at the T10-T11 level. The neonate was asymptomatic with no, abnormal physical or neurological complications, including urinary incontinence, fecal incontinence, or muscle weakness.

Optimal Hemoglobin Level in Preterm Infants

Anemia is a common condition in preterm infants. Packed red blood cell (RBC) transfusion is used for the management of anemia; however, evidence on the benefits of this treatment is limited. RBC transfusion in preterm infants is associated with necrotizing enterocolitis, severe intraventricular hemorrhage, retinopathy of prematurity, transfer of infectious agents, and negative neurodevelopmental outcome. Whether the benefits outweigh the risks of RBC transfusion is controversial, and guidelines on transfusion that are based on expert opinions are unclear. Clinical signs such as apnea, tachycardia, frequent desaturation, poor weight gain, and increasing oxygen requirement and postnatal age are the factors are included in the decision to perform RBC transfusion. To date, the optimal timing and threshold hemoglobin level for administering treatment have not been established. The purpose of this review is to summarize the current transfusion guidelines.

Novel Compound Heterozygote Mutations of the SLC25A13 Gene in an Infant with Neonatal-onset Type II Citrullinemia Detected by Newborn Mass Screening

Citrin deficiency caused by the SLC25A13 gene mutations is associated with both neonatal-onset type II citrullinemia (CTLN2), also known as neonatal intrahepatic cholestasis caused by citrin deficiency and adult-onset CTLN2. Neonatal-onset CTLN2 is an autosomal recessive disorder characterized by poor growth, intrahepatic cholestasis, and increased serum citrulline. A 16-days old infant with hyperammonemia was referred for evaluation of increased plasma citrulline diagnosed using tandem mass spectrometry. Blood amino acid analysis showed significant elevation of citrulline. Mild elevation in serum galactose levels had been found. DNA analysis of the SLC25A13 gene in this patient showed two novel compound heterozygous mutations, c.221C>T in exon4 and c.1645C in exon16 (p.[Ser74Phe]+[Gln549X]). We suggest that infants with a high serum citrulline level on a tandem mass screening test are candidates for gene analysis and blood amino acid analysis for neonatal-onset CTLN2.