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.

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.

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.

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.

Purpose: We aimed to assess the characteristics of patients with concurrent tinnitus and hyperacusis, determine the best audiological criteria for predicting hyperacusis, and confirm whether objective evidence of changes in the brain exists. Materials and Methods: The medical records of patients with tinnitus who visited the hospital between March 2020 and December 2021 were reviewed. Data on accompanying hyperacusis, audiological profiles, and questionnaires including the Tinnitus Handicap Inventory (THI), Beck Depression Inventory, and numerical rating scale were analyzed. Resting-state quantitative electroencephalography (qEEG) using power spectral density (PSD) and event-related spectral perturbation (ERSP) were performed to objectively quantify changes in the brain. Results: A total of 194 patients were analyzed. Among them, 51 (26.3%) reported combined subjective hyperacusis with tinnitus.However, the proportions widely varied from 7.4% to 68.4% based on three audiological criteria for assessment. A higher score on the THI questionnaire was independently associated with the co-occurrence of tinnitus and hyperacusis. Fair agreement was observed between subjective hyperacusis and the audiological criterion based on a loudness discomfort level (LDL) of ≤90 dB at two or more frequencies for the diagnosis of hyperacusis. An increased beta-PSD and decreased levels of gamma-PSD, all-ERSP, and delta-ERSP were observed in patients with hyperacusis (p<0.05). Conclusion Patients with co-occurring tinnitus and hyperacusis had more severe tinnitus distress. An LDL of ≤90 dB at two or more frequencies may be applicable to predict accompanying hyperacusis in subjects with tinnitus, and qEEG also provides more objective information.

Purpose: We aimed to assess the characteristics of patients with concurrent tinnitus and hyperacusis, determine the best audiological criteria for predicting hyperacusis, and confirm whether objective evidence of changes in the brain exists. Materials and Methods: The medical records of patients with tinnitus who visited the hospital between March 2020 and December 2021 were reviewed. Data on accompanying hyperacusis, audiological profiles, and questionnaires including the Tinnitus Handicap Inventory (THI), Beck Depression Inventory, and numerical rating scale were analyzed. Resting-state quantitative electroencephalography (qEEG) using power spectral density (PSD) and event-related spectral perturbation (ERSP) were performed to objectively quantify changes in the brain. Results: A total of 194 patients were analyzed. Among them, 51 (26.3%) reported combined subjective hyperacusis with tinnitus. However, the proportions widely varied from 7.4% to 68.4% based on three audiological criteria for assessment. A higher score on the THI questionnaire was independently associated with the co-occurrence of tinnitus and hyperacusis. Fair agreement was observed between subjective hyperacusis and the audiological criterion based on a loudness discomfort level (LDL) of ≤90 dB at two or more frequencies for the diagnosis of hyperacusis. An increased beta-PSD and decreased levels of gamma-PSD, all-ERSP, and delta-ERSP were observed in patients with hyperacusis (p<0.05). Conclusion Patients with co-occurring tinnitus and hyperacusis had more severe tinnitus distress. An LDL of ≤90 dB at two or more frequencies may be applicable to predict accompanying hyperacusis in subjects with tinnitus, and qEEG also provides more objective information.

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.

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.

Purpose: This study aimed to identify the effects of a direct breastfeeding program for premature infants in neonatal intensive care units (NICUs). Methods: This quasi-experimental study was conducted during August 2016 to April 2017. Sixty mothers of premature infants were assigned to the experimental (n = 31) or control groups (n = 29). The program was comprised of breastfeeding education and direct breastfeeding support. The experimental and control groups were provided with education and counseling on breastfeeding at the time of admission and discharge. In the experimental group, the mothers initiated oral feeding with direct breastfeeding and engaged in breastfeeding at least seven times during the NICU stay. The collected data were analyzed by the χ2 -test and repeated measures ANOVA using an SPSS program. Results: The experimental group showed a higher direct breastfeeding practice rate (χ2 = 19.29, p < .001), breastfeeding continuation rate (χ2 = 3.76, p < .001), and self-efficacy (F = 25.37, p < .001) than the control group except for maternal attachment. Conclusion The direct breastfeeding program in the NICU has significant effects on the practice and continuation rate of breastfeeding and breastfeeding self-efficacy. Therefore, this program can be applied in the NICU settings where direct breastfeeding is limited.

Background/Aims: Understanding leukemic stem cell (LSC) is important for acute myeloid leukemia (AML) treatment. However, association of LSC with patient prognosis and genetic information in AML patients is unclear. Methods: Here we investigated the associations between genetic information and the various LSC phenotypes, namely multipotent progenitor (MPP)-like, lymphoid primed multipotent progenitor (LMPP)-like and granulocyte-macrophage progenitors (GMP)-like LSC in 52 AML patients. Results: In secondary AML patients, MPP-like LSC was significantly higher than de novo AML (p = 0.0037). The proportion of MPP-like LSC was especially high in post-myeloproliferative neoplasm AML (p = 0.0485). There was no correlation between age and LSC phenotype. Mutations of KRAS and NRAS were observed in MPP-like LSC dominant patients, TP53 and ASXL1 mutations in LMPP-like LSC dominant patients, and CEBPA, DNMT3A and IDH1 mutations in GMP-like LSC dominant patients. Furthermore, KRAS mutation was significantly associated with MPP-like LSC expression (p = 0.0540), and TP53 mutation with LMPP-like LSC expression (p = 0.0276). When the patients were separated according to the combined risk including next generation sequencing data, the poorer the prognosis, the higher the LMPP-like LSC expression (p = 0.0052). This suggests that the dominant phenotype of LSC is one of the important factors in predicting the prognosis and treatment of AML. Conclusions LSC phenotype in AML is closely associated with the recurrent mutations which has prognostic implication. Further research to confirm the meaning of LSC phenotype in the context of genetic aberration is warranted.