Effect of Short-term Exposure of Different Concentrations of Hyperoxia on Fetal Alveolar Type II Cell Death

The Korean Society of Neonatology (KAMJE) 2013 Neonatal medicine Vol.20 No.2

Macrophage Activation Syndrome Presented in a Case of Neonatal Lupus

Kang, Chang Min,Choi, Jinwha,Lee, JungHwa The Korean Society of Neonatology 2021 Neonatal medicine Vol.28 No.3

Macrophage activation syndrome (MAS) is a potentially life-threatening complication in many autoimmune diseases. Early recognition and intervention are essential for a favorable outcome. Neonatal lupus, an acquired autoimmune disease in neonates caused by the transplacental passage of maternal autoantibodies, is rare and usually self-limited. Herein, we report a case of MAS in a patient with neonatal lupus, which improved with intravenous immunoglobulin.

Cerebral Oxygenation during Apnea in Preterm Infants: Effects of Accompanying Peripheral Oxygen Desaturation

Choi, Seo Hee,Lee, Juyoung,Nam, Soo Kyung,Jun, Yong Hoon The Korean Society of Neonatology 2021 Neonatal medicine Vol.28 No.1

Purpose: Premature infants have immature respiratory control and cerebral autoregulation. We aimed to investigate changes in cerebral oxygenation during apnea with and without peripheral oxygen desaturation in premature infants. Methods: This prospective observational study was conducted at Inha University Hospital. Near-infrared spectroscopy (NIRS)-monitored regional cerebral oxygen saturation (rScO₂) and pulse oximeter-monitored peripheral oxygen saturation (SpO₂) were assessed during the first week of life in 16 stable, spontaneously breathing preterm infants. Apneic episodes that lasted for ≥20 seconds or were accompanied by desaturation or bradycardia were included for analysis. The average rScO₂ value during the 5-minute prior to apnea (baseline), the lowest rScO₂ value following apnea (nadir), the time to recover to baseline (recovery time), the area under the curve (AUC), and the overshoot above the baseline after recovery were analyzed. Results: The median gestational age and birth weight of the infants were 29.2 weeks (interquartile range [IQR], 28.5 to 30.5) and 1,130 g (IQR, 985 to 1,245), respectively. A total of 73 apneic episodes were recorded at a median postnatal age of 2 days (IQR, 1 to 4). The rScO₂ decreased significantly following apneic episodes regardless accompanied desaturation. There were no differences in baseline, nadir, or overshoot rScO₂ between the two groups. However, the rScO₂ AUC for apnea with desaturation was significantly higher than that for apnea without desaturation. Conclusion: Cerebral oxygenation can significantly decrease during apnea, especially when accompanied by reduced SpO₂. These results add the evidence for the clinical utility of NIRS in monitoring premature infants.

Autosomal Recessive Malignant Infantile Osteopetrosis Associated with a TCIRG1 Mutation: A Case Report of a Neonate Presenting with Hypocalcemia in South Korea

Oh, Yun Kyo,Choi, Koung Eun,Shin, Youn-Jeong,Kim, Eun Ryoung,Kim, Ji Yeon,Kim, Min Sun,Cho, Sung Yoon,Jin, Dong Kyu The Korean Society of Neonatology 2021 Neonatal medicine Vol.28 No.3

Osteopetrosis refers to a group of genetic skeletal disorders characterized by osteosclerosis and fragile bones. Osteopetrosis can be classified into autosomal dominant, autosomal recessive, or X-linked forms, which might differ in clinical characteristics and disease severity. Autosomal recessive osteopetrosis, also known as malignant osteopetrosis, has an earlier onset, more serious clinical symptoms, and is usually fatal. We encountered a 1-day-old girl who was born full-term via vaginal delivery, which was complicated by meconium-stained amniotic fluid, cephalo-pelvic disproportion, and nuchal cord. Routine neonatal care was provided, in addition to blood tests and chest radiography to screen for sepsis, as well as skull radiography to rule out head injuries. Initial blood tests revealed hypocalcemia, which persisted on follow-up tests the next day. Radiographic examinations revealed diffusely increased bone density and a "space alien" appearance of the skull. Based on radiographic and laboratory findings, the infantile form of osteopetrosis was suspected and genetic testing for identification of the responsible gene. Eventually, a heterozygous mutation of the T cell immune regulator 1, ATPase H+ transporting V0 subunit a3 (TCIRG1) gene (c.292C>T) was identified, making this the first reported case of neonatal-onset malignant osteopetrosis with TCIRG1 mutation in South Korea. Early-onset hypocalcemia is common and usually results from prematurity, fetal growth restriction, maternal diabetes, perinatal asphyxia, and physiologic hypoparathyroidism. However, if hypocalcemia persists, we recommend considering 'infantile of osteopetrosis' as a rare cause of neonatal hypocalcemia and performing radiographic examinations to establish the diagnosis.

Congenital Long QT Syndrome Type 8 Characterized by Fetal Onset of Bradycardia and 2:1 Atrioventricular Block

Joo, Donghoon,Lee, Hyoung Doo,Kim, Taehong,Ko, Hoon,Byun, Joung-Hee The Korean Society of Neonatology 2021 Neonatal medicine Vol.28 No.1

An important, albeit rare, cause of fetal bradycardia is long QT syndrome (LQTS). Congenital LQTS is an ion channelopathy caused by mutations in genes encoding cardiac ion channel proteins. Fetal onset of LQTS imposes high risk of life-threatening tachyarrhythmias and sudden cardiac death. Here, we report the case of a female newborn with fetal onset of bradycardia and a 2:1 atrioventricular (AV) block. After birth, a 12-lead electrocardiogram (ECG) revealed bradycardia with QT prolongation of a corrected QT (QTc) interval of 680 ms and pseudo 2:1 AV block. Genetic testing identified a heterozygous Gly402Ser (c.1204G>A) mutation in CACNA1C, confirming the diagnosis of LQTS type 8 (LQT8). The patient received propranolol at a daily dose of 2 mg/kg. Mexiletine was subsequently administered owing to the sustained prolongation of the QT interval and pseudo 2:1 AV block. One week after mexiletine inception, the ECG still showed QT interval prolongation (QTc, 632 ms), but no AV block was observed. There were no life-threatening tachyarrhythmias in a follow-up period of 13 months.

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

Ha, Ji Eun,Lee, Sun Hyang,Park, Ga Young,Shin, Young-Lim,Kim, Sung Shin,Jang, Mi-Ae The Korean Society of Neonatology 2021 Neonatal medicine Vol.28 No.2

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.

Targeting Risk Factors for the Control of Central Line-Associated Bloodstream Infection in the Neonatal Intensive Care Unit: A Single Tertiary Center Experience

Jeong, Jiyoon,Kwun, Yoojin,Kim, Min-ju,Choi, Sang-Ho,Jung, Euiseok,Lee, Byong Sop,Kim, Ki-Soo,Kim, Ellen Ai-Rhan The Korean Society of Neonatology 2021 Neonatal medicine Vol.28 No.3

Purpose: The aim of this study was to estimate the effect of targeting risk factors for the control of central line-associated bloodstream infection (CLABSI) among high-risk infants in a tertiary neonatal intensive care unit (NICU). Methods: Infants admitted to the NICU and diagnosed with CLABSI from January to December 2013 were eligible for inclusion to the study. The CLABSI group (n=47) was matched in a 1:2 ratio to the control group (n=94) based on gestational age, birth weight, and Score for Neonatal Acute Physiology-II. Risk factors for CLABSI were identified using the Cox proportional hazard model, and analysis of the effect of these risk factors targeting infection control was performed. Results: The risk factors associated with CLABSI were prolonged central line dwell days (adjusted hazard ratio [HR], 1.028; 95% confidence interval [CI], 1.011 to 1.045; P=0.001), use of a silicone catheter (adjusted HR, 5.895; 95% CI, 1.893 to 18.355; P=0.002), surgical treatment (adjusted HR, 3.793; 95% CI, 1.467 to 9.805; P=0.006), and less probiotic supplementation (adjusted HR, 0.254; 95% CI, 0.068 to 0.949; P=0.042). By targeting these risk factors with a quality improvement initiative, the mean CLABSI incidence rate per 1,000 catheter-days decreased from 6.6 to 3.1 (P=0.004). Conclusion: Targeting risk factors for infection control significantly reduced the rate of CLABSI among high-risk infants in the NICU.

Transient Neonatal Diabetes Mellitus Managed with Continuous Subcutaneous Insulin Infusion (CSII) and Continuous Glucose Monitoring

Kim, Min Soo,Kim, Sung Eun,Lee, Na Yeong,Kim, Seul Ki,Kim, Shin Hee,Cho, Won Kyoung,Cho, Kyoung Soon,Jung, Min Ho,Suh, Byung-Kyu,Ahn, Moon Bae The Korean Society of Neonatology 2021 Neonatal medicine Vol.28 No.1

Neonatal diabetes mellitus can be categorized as transient, permanent, or syndromic, and approximately half of the cases are transient. We present a case involving a term newborn who showed overt progression of transient neonatal diabetes mellitus, with complete remission within 6 months. On the second day of life, the patient presented with tachypnea, hyperglycemia, and decreased serum levels of C-peptide and insulin. Continuous subcutaneous infusion of insulin and continuous glucose monitoring were well tolerated. The patient showed a normal growth pattern, with no hyperglycemic or hypoglycemic episodes at 6 months of age. As it is rare and often asymptomatic, hyperglycemia may be attributed to various factors, including intrauterine environment, perinatal stress, and diverse genetic background. Therefore, consistent blood glucose monitoring and prompt early insulin therapy are crucial for any term newborns with persistent hyperglycemia, to prevent further diabetic complications. Moreover, continuous subcutaneous insulin infusion and the utilization of continuous glucose monitoring devices are the most effective and practical management strategies.

Intussusception and Jejunal Atresia Caused by an Ectopic Pancreas in a Newborn

Kim, Han-Sol,Lee, Sun-Young,Han, Song-Hee,Nam, So-Hyun,Jo, Chae-Ku,Kim, Myo-Jing The Korean Society of Neonatology 2021 Neonatal medicine Vol.28 No.2

Ectopic pancreas is defined as an abnormally located pancreatic tissue not sufficiently connected with the normal pancreas, which rarely occurs in neonates. To our knowledge, only a few cases of ectopic pancreas have been reported in newborns in South Korea. We report a case of ectopic pancreas as the cause of intussusception and jejunal atresia in a newborn. This clinical association is extremely rare, and this is the first report in South Korea.

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

Hong, Hyun Sook,Kim, Sung Shin,Park, Ga Young The Korean Society of Neonatology 2021 Neonatal medicine Vol.28 No.1

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.