Purpose: Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is a congenital anomaly that can cause frequent digestive and nutritional problems, even after repair. The most common complication is anastomotic stricture, for which reoperation or balloon dilatation is performed. This study aimed to evaluate the postoperative complications of EA and the role of endoscopic balloon dilatation (EBD) in cases of anastomotic stricture. Methods: We retrospectively analyzed patients diagnosed with EA with or without TEF between January 2000 and February 2021. Patients’ baseline characteristics, associated anomalies, and postoperative complications were reviewed. Results: Among 26 patients, 14 (53.8%) were male, 12 (46.2%) had coexisting anomalies, and the median follow-up was 6.1 years (range, 1.2–15.7 years). In univariate analysis, prematurity, low birth weight, and long-gap EA were associated with postoperative complications in 12 (46.2%) patients. Among the 10 (38.5%) patients with anastomotic stricture, nine (90.0%) required EBD. Regarding the first EBD, it was performed at a median of 3.3 months (range, 1.2–7.6 months) post-repair, while the average patient weight was 4.6 kg. The mean diameter ranged from 3.3 to 9.1 mm without major complications. In univariate analysis, long-gap EA alone was significantly associated with EBD. Conclusion Approximately half of the patients experienced complications after EA repair.In particular, patients with a long-gap EA had a significantly increased risk of complications, such as anastomotic strictures. EBD can be safely used, even in infants.

Purpose: The purpose of this study was to identify the influence of health literacy and insight on medication adherence in mental illness.
Methods: A cross-sectional, descriptive correlational study design was used. Participants were 135 adults diagnosed with mental disorders and living in D city. Collected data were analyzed using descriptive statistics, t-test, ANOVA, Pearson’s correlation, and hierarchical multiple regression with SPSS 26.0.
Results: On multiple regression analysis, clinical insight (β=.32, p<.001) and reading response (β=.23, p=008) were statistically significant factors for medication adherence and showed a total explanatory power of 20%.
Conclusion In order to enhance medication adherence, strategies and programs are needed to improve the insight and health literacy among patients with severe mental illness.

The Say–Barber–Biesecker–Young–Simpson variant of Ohdo syndrome (SBBYSS) (Online Mendelian Inheritance in Man #603736) is a rare autosomal dominant disorder and clinically features blepharophimosis with ptosis, a mask-like facial appearance, cryptorchidism, congenital heart defect, long thumbs/great toes, and thyroid dysfunction. The etiology of SBBYSS has been shown to be due to heterozygous KAT6B gene mutation. Here we report a case of a neonate with SBBYSS identified a novel mutation in KAT6B gene. The patient showed typical dysmorphic facies, cryptorchidism with micropenis, overriding fingers, and long thumbs and toes at birth. He had also hypothyroidism, large atrial septal defect, and sensorineural hearing loss. The next generation sequencing identified a heterozygous novel variant, c.5206C>T (p.Gln1736Ter) in KAT6B gene. At the 9 months of age, he underwent patch closure for atrial septal defect. Until the 12-month follow-up, he was under-developed.

Purpose: The purpose of this study was to identify the influence of health literacy and insight on medication adherence in mental illness.
Methods: A cross-sectional, descriptive correlational study design was used. Participants were 135 adults diagnosed with mental disorders and living in D city. Collected data were analyzed using descriptive statistics, t-test, ANOVA, Pearson’s correlation, and hierarchical multiple regression with SPSS 26.0.
Results: On multiple regression analysis, clinical insight (β=.32, p<.001) and reading response (β=.23, p=008) were statistically significant factors for medication adherence and showed a total explanatory power of 20%.
Conclusion In order to enhance medication adherence, strategies and programs are needed to improve the insight and health literacy among patients with severe mental illness.

We performed a point seroprevalence survey of measles among healthcare workers (HCWs) at two Korean teaching hospitals in 2019. A total of 2,830 HCWs underwent an antibody test.The overall seropositivity of measles was 93.1%. The seroprevalence of measles was lowest in HCWs aged 20 - 24 years (81.2%), followed by those aged 25 - 29 years (90.1%). The rates of anti-measles IgG positivity were significantly different between the two hospitals (97.0% vs.89.4%, P <0.001). These results suggest that the seropositivity of measles in HCWs may differ depending on the hospital's vaccination policy.

Short stature homeobox-containing gene (SHOX) is a well-known causative gene for the short stature in Turner syndrome. The clinical manifestation of SHOX gene related disorders varies from SHOX haploinsufficiency, presenting with idiopathic short stature, disproportionate short stature, or Leri-Weill dyschondrosteosis (LWD) to recessive form of extreme dwarfism and limb deformity in Langer mesomelic dysplasia. LWD is usually diagnosed upon suspicion based on short stature and skeletal abnormalities, and it is rarely accompanied with respiratory failure in the neonatal period. Here, we report the case of a newborn infant with LWD presenting with severe micrognathia that caused respiratory distress, which was diagnosed using microarray testing. Even when the manifestation of Madelung deformity is not yet apparent, LWD should be considered as one of underlying diseases related to congenital micrognathia.

Hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome is a rare autosomal recessive urea cycle disorder. HHH is caused by a deficiency of the mitochondrial ornithine transporter protein, which is encoded by the solute carrier family 25, member 15 (SLC25A15) gene. Recently, government supported Korean newborn screening has been expanded to include a tandem mass spectrometry (MS/MS) measurement of ornithine level. We report a case of a neonate with HHH syndrome showing a normal MS/MS measurement of ornithine level. A female newborn was admitted to neonatal intensive unit due to familial history of HHH syndrome. Her parents were consanguineous Parkistani couple. The subject's older sister was diagnosed with HHH syndrome at age of 30 months based on altered mental status and liver dysfunction. Even though the subject displayed normal ammonia and ornithine levels based on MS/MS analysis, a molecular test confirmed the diagnosis of HHH syndrome. At 1 month of age, amino acid analysis of blood and urine showed high levels of ornithine and homocitrulline. After 11 months of follow up, she showed normal growth and development, whereas affected sister showed progressive cognitive impairment despite no further hyperammonemia after protein restriction and standard therapy. Our report is in agreement with a previous Canadian study, which showed that neonatal samples from HHH syndrome patients demonstrate normal ornithine levels despite having known mutations. Considering the delayed rise of ornithine in affected patients, genetic testing, and repetitive metabolic testing is needed to prevent patient loss in high risk patients.

OBJECTIVES: The aim of this study was to evaluate the effect of a school-based social skills training program on peer relationships in children and adolescents and to assess the plan for effective school-based mental health services. METHODS: The Child and Adolescent Mental Health Promotion Team of Bugok National Hospital conducted 7-sessioned school-based social skills training for elementary and middle school students (n=90). Changes in peer relationships were evaluated before and after application of the program using a name generator question. RESULTS: The social skills training program increased peer relations, indicating significant changes in social network indices. CONCLUSION: The social skills training program positively influenced peer relationships. The school-based social skills training program can be expected to have positive effects on school-based mental health services. Future investigation is needed to validate the long term effects of this program.
Adolescent ; Child ; Education ; Humans ; Mental Health ; Mental Health Services ; Social Skills

OBJECTIVES: The purposes of this study are to evaluate the effect of a school-based social skills training program on the emotional regulation of children and adolescents and to assess the plan for effective school-based mental health services. METHODS: The Child and Adolescent Mental health promotion team of Bugok National Hospital conducted school-based social skills training (N=90, 7 sessions) for elementary and middle school students. Evaluations were conducted before and after the application of the program using a prosocial behavior questionnaire, a cohesiveness questionnaire, the Korean version of the 20-item Toronto Alexithymia Scale, a self-esteem scale, and the Novaco anger scale, in order to identify any changes. RESULTS: The social skills training program increased the prosocial behavior and cohesiveness of the children and adolescents and decreased their alexithymic tendency and degree of anger, but did not significantly change their self-esteem. CONCLUSION: The social skills training program positively influences the emotional and behavioral levels of children and adolescents. The emotional regulation program based on a social skills training program is expected to have positive results in school-based mental health services. Future investigations are needed to validate the long term effects of this program.
Adolescent ; Affective Symptoms ; Anger ; Child ; Education* ; Humans ; Mental Health ; Mental Health Services ; Social Skills*

BACKGROUND: Anemia is a major risk factor that contributes to mortality in patients with chronic kidney disease. There is controversy over the optimal hemoglobin (Hb) target in these patients. This study investigated the association between Hb level and mortality in a cohort of hemodialysis (HD) patients in Korea. METHODS: This study was a multicenter prospective observational study of maintenance HD patients that was performed for 5 years in western Seoul, Korea. Three hundred and sixty-two participants were enrolled. Laboratory values and mortality were accessed every 6 months. Repeated measures of laboratory values in each interval were averaged to obtain one semiannual mean value. The Hb values were divided into six groups: (1) Hb<9 g/dL; (2) 9 g/dL< or =Hb<10 g/dL; (3) 10 g/dL< or =Hb<11 g/dL; (4) 11 g/dL< or =Hb<12 g/dL; (5) 12 g/dL< or =Hb<13 g/dL; and (6) Hb> or =13 g/dL. We analyzed the odds ratio for all-cause mortality, based on the Hb group, and adjusted for demographics and various laboratory values. Statistics were performed with SAS, version 9.1 software (SAS Institute Inc., Cary, NC, USA). RESULTS: Mortality odds ratios relative to the reference group (10-11 g/dL) in the fully adjusted model were 3.61 for<9 g/dL; 3.17 for 9-10 g/dL*; 4.65 for 11-12 g/dL*; 5.50 for 12-13 g/dL*; and 2.05 for> or =13 g/dL (* indicates P<0.05). CONCLUSION: In this study, a Hb level of 10-11 g/dL was associated with the lowest mortality among the groups with Hb level<13 g/dL. Larger interventional trials are warranted to determine the optimal Hb target for Korean HD patients.
Anemia* ; Cohort Studies* ; Demography ; Humans ; Korea ; Mortality ; Observational Study ; Odds Ratio ; Prospective Studies ; Renal Dialysis* ; Renal Insufficiency, Chronic ; Risk Factors ; Seoul