Purpose: Bronchopulmonary dysplasia (BPD) is one of the most fatal respiratory morbidities in preterm infants, causing adverse respiratory and neurodevelopmental outcomes. Despite advances in neonatal ventilator care, the incidence of BPD has been static or even increased. The purpose of this study was to evaluate the incidence of BPD in a single center over 10 years. Methods: Preterm infants with gestational age (GA) <30 weeks who were admitted to Inje University Busan Paik Hospital from January 2009 to December 2018 and survived 28 days or more were enrolled. The incidence of BPD according to year and GA and the risk factors of BPD were evaluated. Results: Among 629 infants, 521 infants who survived 28 days or more were enrolled (BPD group, n=252; non-BPD group, n=269). The incidence of BPD was 48.4%, with moderate to severe BPD accounting for 13.9%. In preterm infants with GA ≤25, 26 to 27, and 28 to 29 weeks, the incidences of BPD were 57.5%, 51.5%, and 14.6%, respectively, with moderate to severe BPD accounting for 23.8%, 10.5%, and 3.7%, respectively. The incidence of BPD decreased from 68% in 2009 to 34.3% in 2014. Subsequently, it increased. Surfactant re-dosing and patent ductus arteriosus were more frequent in the BPD group than in the non-BPD group. Conclusion: BPD did not decrease over the previous 10 years despite advances in neonatal care.

Purpose: We compared perinatal characteristics, clinical outcomes, and treatment between late preterm infants born at 34 weeks and 35 to 36 weeks of gestation. Methods: We reviewed the medical records of 254 neonates (gestational age 34⁺⁰ to 36⁺⁶ weeks) who were born at Inje University Sanggye Paik Hospital between July 1, 2013 and June 31, 2018. Late preterm infants were categorized into two groups: Group 1 (born at 34 weeks, n=88) and Group 2 (born at 35 to 36 weeks, n=162). We compared the clinical outcomes, treatment, and readmission within 12 months after birth between two groups. Results: Group 1 showed higher frequencies of antenatal steroid administration, premature membrane rupture, maternal antibiotic use, and histologic chorioamnionitis. Group 1 also had significantly more medical problems such as respiratory distress, feeding intolerance, gavage feeding, neonatal jaundice, apnea or bradycardia, and hypocalcemia. Treatment during hospital stay including respiratory support, nutritional support, and antibiotics use over 24 hours was also significantly higher in Group 1. In addition, delayed discharge was more frequent in Group 1. Conclusion: Late preterm infants born at 34 weeks gestation had significantly higher morbidity, required more aggressive management, and more often had delayed discharge compared to those in late preterm infants born at 35 to 36 weeks' gestation.

Collagen type IV alpha 1 (COL4A1) plays an important role in construction of the basement membranes of all human tissues, especially vessels. Mutations in COL4A1 lead to various multisystemic dysfunctions, including hereditary porencephaly, hemorrhagic stroke, hemiplegia, cerebral small vessel disease, and nephropathy. In this study, we describe a neonatal case featuring a novel de novo COL4A1 mutation, manifesting as fetal intraventricular hemorrhage and porencephaly. This patient is one of the youngest to have been diagnosed with the most severe phenotype. Our experience may assist clinicians in the diagnosis and management of this extremely rare genetic condition.

Iatrogenic esophageal perforation is a rare condition in children, but occasionally occurs in premature infants due to repeated intubation or nasogastric tube insertion. Esophageal perforation is common in pneumothorax and interstitial emphysema, but rarely occurs in the absence of pneumothorax. Although complications, such as mediastinitis, after esophageal perforation are rare, they can be fatal. Therefore, rapid and accurate diagnosis and appropriate treatment are important. The authors report a case of huge retrocardiac pneumomediastinum and subcutaneous emphysema suspected to be caused by esophageal perforation after repeated intubation.

Hypophosphatasia is a rare disease characterized by defective bone mineralization due to deficiency of tissue-nonspecific alkaline phosphatase. The patient was an 8-day-old male infant who presented with seizure since that day. Other symptoms included respiratory failure, requiring the use of a mechanical ventilator. Physical examination revealed a large bulging anterior fontanelle, soft skull bone, and radial deviation of both wrists. Laboratory examination showed normal serum calcium, low parathyroid hormone, normal 25-hydroxy vitamin D, and severely low alkaline phosphatase levels. Skeletal X-ray revealed dysplasia of the skull and cupping of the epiphysis of the limbs. Two heterozygous mutations (c.1052A>G, c.1559delT) of the ALPL gene were identified by Sanger sequencing. Thus, we report a case of confirmed lethal perinatal hypophosphatasia in Korea.

Central diabetes insipidus (CDI) is extremely rare in neonates, especially in extremely-low-birth-weight infants, and most cases are secondary to conditions, such as ischemic or hemorrhagic brain damage. Here, we report a case of CDI in a 530-g infant born at 23⁺³ weeks of gestation, with suspected ectopic posterior pituitary gland. Hypernatremia was noticed at 33⁺⁶ weeks of postmenstrual age, and it persisted with increased volumes of diluted urine, despite adequate sodium intake. Serum and urine osmolality returned to the normal range after administration of a desmopressin injection. The bright spot of the posterior pituitary was absent, and brain magnetic resonance imaging suggested an ectopic posterior pituitary gland. At the time of writing this manuscript, the patient was on oral desmopressin medication without complications at the corrected age of 8 months. Through this report, we emphasize that although CDI is extremely rare in premature infants, it should be suspected when hypernatremia and polyuria that are unexplained by other causes are noted.

Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular neoplasm that often develops a coagulopathy known as Kasabach-Merritt phenomenon (KMP). Visceral involvement denotes a poor prognosis. We report a case of visceral KHE with KMP successfully treated with corticosteroids and vincristine. The infant had been born vaginally at 34 weeks 6 days' gestation, weighing 2,360 g. He was admitted for the management of respiratory failure. Blood tests showed anemia and thrombocytopenia 1 hour after delivery. Additional blood tests revealed a prothrombin time of 12.1 seconds, activated partial thromboplastin time of 60.6 seconds, fibrinogen levels of 72.4 mg/dL, and D-dimer levels >3,200 ng/mL. Despite supportive measures and daily transfusions, the clinical condition and coagulopathy gradually worsened. Renal ultrasonography performed to find the origin of the coagulopathy revealed an echogenic mass measuring >3 cm in the abdominal cavity. A magnetic resonance imaging scan showed an ill-marginated, infiltrative mass like lesion in the right anteromedial and posteromedial perirenal space that was hypointense with mild enhancement on T1- and T2-weighted images. Large vascular tumors adherent to other visceral organs were noted during exploratory laparotomy but could not be resected. Treatment with methylprednisolone was ineffective. Vincristine was administered weekly from the 17th hospital day, and the coagulation profile showed gradual improvement after its initiation. Intravenous methylprednisolone was switched to oral prednisolone on the 57th hospital day. He was discharged on the 73rd hospital day and continued vincristine treatment every 2 weeks and oral prednisolone administration as an outpatient treatment for 8 weeks. He remained symptom-free at the 39-month follow-up.