A Rare Case of Oligodendroglioma in Sacrococcygeal Mature Teratoma Diagnosed in Preterm Infant

Sacrococcygeal teratoma is the most common congenital tumor in neonates, and is reported in approximately 1/35,000 to 1/40,000 live births. Oligodendroglioma is a rare central nervous system tumor that is usually found in the cerebral hemisphere of young and middle aged adults. When associated with a teratoma, it is mainly identified in ovarian teratoma in adolescents and adults. We describe a rare case of a preterm infant with oligodendroglioma in a mature sacrococcygeal teratoma. The male neonate was born at a gestational age of 30 weeks with a protruding mass in the sacrococcygeal region. Pelvic magnetic resonance imaging showed a sacrococcygeal teratoma of approximately 11 cm comprising fat components and skeletal structure, that extended from the anterior part of the sacrum to the abdominal cavity. Radical resection was performed at 36 days of age. Macroscopically, the resected intra-abdominal mass had the characteristics of a cystic lesion, and the intrapelvic mass was a predominantly solid mixed cystic-solid lesion. Histologically, this solid lesion in the intrapelvic mass was composed of mature glial tissue, which comprised as a proliferation of monotonous cells with small and round nuclei, surrounded by a perinuclear halo (“fried egg” appearance). Additionally, these cells were immunohistochemically positive for glial fibrillary acidic protein. These findings confirmed the diagnosis of oligodendroglioma in sacrococcygeal mature teratoma. After the treatment, no recurrence was observed during the follow-up period, and no additional intervention was required. However, the patient is undergoing treatment for voiding dysfunction caused by a neurogenic bladder.

Perinatal Prognostic Factors for Congenital Diaphragmatic Hernia: A Korean Single-Center Study

Purpose@#This study aimed to identify prognostic factors based on treatment outcomes for congenital diaphragmatic hernia (CDH) at a single-center and to identify factors that may improve these outcomes. @*Methods@#Thirty-five neonates diagnosed with CDH between January 2011 and December 2021 were retrospectively analyzed. Pre- and postnatal factors were correlated and analyzed with postnatal clinical outcomes to determine the prognostic factors. Highest oxygenation index (OI) within 24 hours of birth was also calculated. Treatment strategy and outcome analysis of published literatures were also performed. @*Results@#Overall survival rate of this cohort was 60%. Four patients were unable to undergo anesthesia and/or surgery. Three patients who commenced extracorporeal membrane oxygenation (ECMO) post-surgery were non-survivors. Compared to the survivor group, the non-survivor group had a significantly higher occurrence of pneumothorax on the first day, need for high-frequency ventilator and inhaled nitric oxide use, and high OI within the first 24 hours. The non-survivor group showed an early trend towards the surgery timing and a greater number of patch closures. Area under the receiver operating characteristic curve was 0.878 with a sensitivity of 76.2% and specificity of 92.9% at an OI cutoff value of 7.75. @*Conclusion@#OI within 24 hours is a valuable predictor of survival. It is expected that the application of ECMO based on OI monitoring may help improve the opportunity for surgical repair, as well as the prognosis of CDH patients.

Implementing the Golden Hour Protocol to Improve the Clinical Outcomes in Preterm Infants

Purpose@#Since premature infants are sensitive to the changes in blood glucose levels and body temperature, maintaining these parameters is important to avoid the risk of infections. The authors implemented the Golden Hour protocol (GHP) that aims to close the final incubator within one hour of birth by implementing early treatment steps for premature infants after birth, such as maintaining body temperature, securing airway, and rapidly administering glucose fluid and prophylactic antibiotics by securing breathing and rapid blood vessels. This study investigated the effect of GHP application on the short- and long-term clinical outcomes. @*Methods@#We retrospectively analyzed the medical records between 2017 and 2018 before GHP application and between 2019 and 2020 after GHP application in preterm infants aged 24 weeks or older and those aged less than 33 weeks who were admitted to the neonatal intensive care unit. @*Results@#Overall, 117 GHP patients and 81 patients without GHP were compared and analyzed. Peripheral vascularization time and prophylactic antibiotic administration time were shortened in the GHP-treated group (P=0.007 and P=0.008). In the short-term results, the GHP-treated group showed reduced hypothermia upon arrival at the neonatal intensive care unit (P=0.002), and the blood glucose level at 1 hour of hospitalization was higher (P=0.012). Furthermore, the incidence of neonatal necrotizing enteritis decreased (P=0.043). As a long-term result, the incidence of BPD was reduced (P=0.004). @*Conclusion@#We confirmed that applying GHP improved short- and long-term clinical outcomes in premature infants aged <33 weeks age of gestation, and we expect to improve the treatment quality by actively using it for postnatal treatment.

Neurodevelopmental Outcomes of Very-Low-Birth-Weight Infants without Severe Brain Lesions and Impact of Postnatal Steroid Use: A Single-Center Korean Study

Purpose@#We used the Bayley Scales of Infant and Toddler Development (BSID)-III to analyze the incidence and risk factors of developmental delay in very-low-birth-weight infants without severe brain lesions. We further examined the correlation between the cumulative dexamethasone dose and developmental assessment results. @*Methods@#We retrospectively analyzed data of preterm infants (birth weight <1,500 g) admitted to our neonatal intensive care unit between January 2014 to December 2020. The BSID-III scores obtained between the corrected ages of 12 and 24 months and after 24 months were analyzed. Developmental delay was defined as a composite score of <85 for the cognition, language, and motor domains. Univariate and multivariate analyses of developmental delay risk factors and developmental changes from the first to second BSID-III were performed. Correlations between the accumulated dexamethasone dose used for bronchopulmonary dysplasia (BPD) and the first and second test scores were analyzed. @*Results@#Seventy-one and thirty-six infants completed the first and second tests, respectively. In both tests, developmental delay was most commonly observed in the language domain (26.8%, 47.2%). In multivariate analysis, mild BPD was identified as a developmental delay risk factor (P<0.05), whereas prenatal steroid use reduced the developmental delay risk (P<0.05). All domain scores were lower in the second test than in the first test. The cognition and language domain scores in the second test decreased with increasing cumulative dexamethasone doses. @*Conclusion@#Very-low-birth-weight infants typically experience language delay, which can persist as they age.

Congenital Syphilis in Neonate: A Single Center Study for 10 Years

Purpose@#Syphilis infections are becoming more prevalent in the Republic of Korea, and inadequately treated syphilis can lead to congenital syphilis (CS) in newborns. This study aimed to analyze the clinical manifestations of syphilis in mothers and newborns and to make suggestions to improve disease prognosis. @*Methods@#This single-center study was performed between August 2009 and August 2019 and included 29 newborns with CS. We retrospectively evaluated the clinical features, rapid plasma reagin (RPR) card test, fluorescent treponemal antibody absorption test (FTA-ABS), morbidity, and treatment regimen of all the syphilis-affected mothers and their newborns. @*Results@#At the time of delivery, mean maternal age was 29.0±6.1 years old, and newborn gestational age was 38.0 weeks. In cases when syphilis was confirmed during the second and third trimesters of pregnancy, the newborn with CS had morbidity (p=0.004). The mean RPR titer was related to morbidity (p= 0.036). Positive results of FTA-ABS IgM (p<0.001) and pleocytosis in the cerebrospinal fluid (CSF) (p= 0.020) also increase morbidity. The most common symptoms were desquamation and skin rash, followed by hepatomegaly, neurodevelopmental disability, and bone abnormalities. The highest number of CS cases per 1,000 live births in this hospital was in 2014. @*Conclusion@#CS is a preventable and treatable disease if physicians detect symptoms and provide appropriate treatment through RPR examinations during every trimester. General practitioners should be widely trained on various aspects including early detection, formal treatment, and regular follow-up. Additionally, medical services should be provided for the entire childbearing population regardless of the socioeconomic status.

Congenital Syphilis in Neonate: A Single Center Study for 10 Years

Purpose@#Syphilis infections are becoming more prevalent in the Republic of Korea, and inadequately treated syphilis can lead to congenital syphilis (CS) in newborns. This study aimed to analyze the clinical manifestations of syphilis in mothers and newborns and to make suggestions to improve disease prognosis. @*Methods@#This single-center study was performed between August 2009 and August 2019 and included 29 newborns with CS. We retrospectively evaluated the clinical features, rapid plasma reagin (RPR) card test, fluorescent treponemal antibody absorption test (FTA-ABS), morbidity, and treatment regimen of all the syphilis-affected mothers and their newborns. @*Results@#At the time of delivery, mean maternal age was 29.0±6.1 years old, and newborn gestational age was 38.0 weeks. In cases when syphilis was confirmed during the second and third trimesters of pregnancy, the newborn with CS had morbidity (p=0.004). The mean RPR titer was related to morbidity (p= 0.036). Positive results of FTA-ABS IgM (p<0.001) and pleocytosis in the cerebrospinal fluid (CSF) (p= 0.020) also increase morbidity. The most common symptoms were desquamation and skin rash, followed by hepatomegaly, neurodevelopmental disability, and bone abnormalities. The highest number of CS cases per 1,000 live births in this hospital was in 2014. @*Conclusion@#CS is a preventable and treatable disease if physicians detect symptoms and provide appropriate treatment through RPR examinations during every trimester. General practitioners should be widely trained on various aspects including early detection, formal treatment, and regular follow-up. Additionally, medical services should be provided for the entire childbearing population regardless of the socioeconomic status.

Cystic fibrosis in a female adolescent carrying c.263T>G (p.Leu88X) and c.2977G>T (p.Asp993Tyr) mutation

Cystic fibrosis (CF) is a chronic progressive autosomal recessive disease and caused by cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations. CFTR gene encodes an ion channel involved in salt and water transport in multiple organs including lung, pancreas and reproductive tract. To date, more than 2,000 mutations of CFTR gene have been identified. Because the age of onset and the severity of symptoms may vary greatly, a diagnosis of CF can be challenging. Although CF is common in Caucasians, it is extremely rare in Koreans. A few cases of CF have been reported in Korean children since 1988. Here, we report a 13-year-old girl carrying c.263T>G (p.Leu88X) and c.2977G>T (p.Asp993Tyr). Furthermore, we reviewed 22 Korean cystic fibrosis cases reported in the literature.

Risk Factors for Delayed Hyperthyrotropinemia in Late Preterm Infants

PURPOSE: This study aimed to investigate the prevalence of delayed thyroid dysfunction based on iodine disinfectant use and to analyze associated risk factors.METHODS: A retrospective study was conducted on late preterm infants admitted to the neonatal intensive care unit between January 2010 and June 2018, who underwent neonatal thyroid screening (NTS) and ≥2 thyroid function tests (TFTs). NTS was performed 3 days after birth, with at least two TFTs 1 week and 2 to 4 weeks after birth. To distinguish between normal and dysfunctional thyroid levels, we reviewed TFT results at 2 to 4 weeks and examined possible risk factors for the development of thyroid dysfunction.RESULTS: Of 295 late preterm infants, 262 were enrolled with a mean gestational age and birth weight of 34.8±0.7 weeks and 2,170±454 g, respectively. A total of 7.6% developed hyperthyrotropinemia at the age of 24.3±14.6 days (range, 12 to 69). The incidence of hyperthyrotropinemia during iodine use was approximately 12.6%, while that during discontinuation was 2.4% (P=0.002). Multivariate analysis revealed that small for gestational age (SGA), iodine disinfectant use, and abnormal NTS results were significant risk factors for delayed hyperthyrotropinemia (adjusted odds ratio [AOR]: 4.27, P=0.008; AOR: 8.24, P=0.003; and AOR: 7.80, P=0.002, respectively).CONCLUSION: Delayed hyperthyrotropinemia was prevalent in late preterm infants exposed to topical iodine and those identified as being SGA. Secondary TFTs should be considered 2 to 4 weeks after birth for this population at risk.

Haemophilus parainfluenzae Infective Endocarditis Diagnosed by Direct 16S rRNA Sequencing of Vegetation

The HACEK group of microorganisms is responsible for approximately 3-6% of endocarditis cases and is a major cause of culture-negative endocarditis. Here, we report a case of Haemophilus parainfluenzae infective endocarditis that was diagnosed by direct PCR sequencing of 16S rRNA from resected vegetation. A healthy 26-yr-old man was admitted to the emergency room (ER) on March 27, 2011 because of intermittent high fever. The patient was prescribed cefpodoxime for 5 days at the ER. Six and 11 sets of blood cultures were performed at the ER and in a general ward, respectively, using BACTEC Plus Aerobic/F (Becton-Dickinson, USA) and Lytic Anaerobic/F Plus (BD) together. Echocardiography revealed a large vegetation at the posterior mitral valve leaflet. After performing mitral valvoplasty on hospital day (HD) 11, the vegetation tissue was cultured in thioglycolate broth, blood agar, Brucella agar, and MacConkey agar for 7 days, but no organism was grown. Direct PCR sequencing of 16S rRNA of the tissue revealed the presence of H. parainfluenzae. In the 17 sets of blood cultures, bacterial growth was detected in only 2 aerobic bottles of 5 sets taken at HD 9 after 10-day and 14-day incubation. The organism was identified as H. parainfluenzae by using the VITEK NHI card (bioMerieux, France). Direct PCR sequencing of vegetation could be useful in diagnosing bacterial pathogens in infective endocarditis patients, especially in culture-negative cases.