PURPOSE: The purpose of this study was to investigate the needs for pediatric palliative care (PPC) among parents of children with complex chronic conditions (CCCs) and to investigate differences in the needs for PPC according to their general characteristics. METHODS: A cross-sectional survey was conducted between March 28 and May 18, 2018. Parents (N=96) who had a child under 18 years with a CCC were recruited. Data were analyzed using descriptive statistics, independent t-test, and one-way ANOVA. RESULTS: The overall average need for PPC was 3.58±0.33 out of 4.00. In terms of care for the subjects' children, the highest need was physical care, followed by psychosocial and spiritual care. In the sub-dimensions, preservation of physical function received the highest score. Of the items, the highest need was for seizure control. In terms of care for the subjects themselves, the highest need was for psychosocial care, followed by bereavement and spiritual care. In the sub-dimensions, communication received the highest score. Of the items, the highest need was for smooth communication with medical staff. Differences in needs for PPC according to participants' general characteristics were not statistically significant. CONCLUSION: Medical staff should provide PPC according to the priorities of parents' perceived needs.
Bereavement ; Child* ; Chronic Disease ; Cross-Sectional Studies ; Humans ; Medical Staff ; Needs Assessment ; Palliative Care* ; Parents* ; Seizures

Purpose: The rising prevalence of smartphone overdependence among adolescents and its detrimental impact on mental health have become a growing concern. This study aimed to investigate the association between smartphone overdependence and the mental health of Korean adolescents. Methods: Participants were drawn from the 16th Korea Youth Risk Behavior Web-based Survey conducted in 2020. The dependent variable as smartphone overdependence, while the main exposure of interest was mental health, encompassing generalized anxiety disorder (GAD), perceived stress, sources of perceived stress, perceived loneliness, and perceived depressive symptoms. The study employed the Rao-Scott chi-square test and multiple logistic regression using IBM SPSS version 26.0. Results: The participants comprised 54,948 adolescents aged 13 to 18 years. Among them, 25.1% (n=13,775) were categorized as smartphone overdependence group. Specifically, 20.3% of adolescents who reported GAD ≥10 and 22.5% of those who reported experiencing high levels of perceived loneliness were identified as smartphone overdependent. The GAD increased a risk of smartphone overdependence by 2.61 times (95% confidence interval [95% CI]: 2.46–2.77). Perceived loneliness was associated with 1.98-fold (95% CI: 1.87–2.09) increased risk of smartphone overdependence. Additionally, conflict with peers was found to increase the risk of smartphone overdependence by 4.63-fold (95% CI: 3.89–5.52), followed by conflict with parents (odds ratio [OR]: 4.52, 95% CI: 3.84–5.31), and family environment (OR: 4.52, 95% CI: 3.75–5.46). Conclusion The findings underscore a significant association between smartphone overdependence and mental health in Korean adolescents. Healthcare services to improve their emotional coping and interpersonal skills are necessary.

OBJECTIVE: The aim of this study is to describe the general characteristics and potential susceptibility genes of a large cohort of Korean rheumatoid arthritis (RA) patients. METHODS: After giving consent, the patients were invited to undergo a structured interview and clinical examination that were performed by rheumatologists and a specially trained research nurse. When appropriate, the information obtained by interview was supplemented by information from the patient's medical record. We reviewed the genetic studies for the subjects in the Hanyang RA cohort to investigate the genetic characteristics of Korean RA patients. RESULTS: The mean age of the Hanyang RA cohort was 51.6+/-12.4 years, and 88.1% were women. The unemployment rate was 19.6%, and 52.2% of the patients had limitation of everyday life or their work life. The mean age at the time of disease onset was 41.0+/-12.9 years and the duration from disease onset to initiation of treatment was 23.6+/-57.1 months. The smoking rate of the Hanyang RA cohort was 16.8%, and 30.8% of the patients were exposed to passive smoking. Total joint arthroplastys were performed in 158 (10.3%) patients, and the most common operation site was the knee. On the review of the genetic studies for the Hanyang RA cohort, the representative susceptibility genes for the development of RA were HLA-DRB1, PADI4, STAT4 and TRAF1-C5. CONCLUSION: This data of Korean RA patients can be used as the preliminary data for important studies. Establishment of a large prospective, multicenter cohort is imperative to determine the characteristics of Korean RA, and the Hanyang RA cohort is expected to play a lead role for this.
Arthritis, Rheumatoid ; Cohort Studies ; Female ; HLA-DRB1 Chains ; Humans ; Joints ; Knee ; Medical Records ; Smoke ; Smoking ; Tobacco Smoke Pollution ; Unemployment

17α-hydroxylase deficiency is a rare cause of congenital adrenal hyperplasia and is characterized by primary amenorrhea, delayed puberty and hypertension. Although 17α-hydroxylase deficiency mimics mineralocorticoid-induced hypertension, impaired sexual development can aid in the differential diagnosis of this disease. A 32-year-old woman, who had a history of testicular feminization syndrome, presented with hypertension. Her aldosterone level was elevated whereas plasma renin activity was reduced, and her computed tomography scan showed a left adrenal adenoma, which was thought to be an aldosterone producing adenoma. A left adrenalectomy was performed to treat hypertension; however, the condition did not improve. The hormonal tests revealed high levels of plasma progesterone, mineralocorticoid and adrenocorticotropic hormone, and low levels of 17a hydroxyprogesterone, cortisol and sex hormones. The patient was diagnosed with 17α-hydroxylase deficiency and commenced on prednisolone, which controlled hypertension. Here, we report a case of 17α-hydroxylase deficiency mimicking hyperaldosteronism via aldosterone-producing adrenal adenoma.
Adenoma* ; Adrenal Hyperplasia, Congenital ; Adrenalectomy ; Adrenocortical Adenoma ; Adrenocorticotropic Hormone ; Adult ; Aldosterone ; Amenorrhea ; Androgen-Insensitivity Syndrome ; Diagnosis, Differential ; Female ; Gonadal Steroid Hormones ; Humans ; Hydrocortisone ; Hyperaldosteronism* ; Hypertension ; Male ; Plasma ; Prednisolone ; Progesterone ; Puberty, Delayed ; Renin ; Sexual Development

Infantile cortical hyperostosis, or Caffey's disease, usually presents with typical radiological features of soft tissue swelling and cortical thickening of the underlying bone. The disease can be fatal when it presents antenatally, especially before a gestational age of 35 weeks. This fatal, premature form of the disease is known to occur in various ethnic groups around the globe, and approximately 30 cases have been reported in English literature. This paper is unique in that it is the first paper to report a lethal form of prenatal-type infantile cortical hyperostosis diagnosed in South Korea. Born at gestational age of 27 weeks and 4 days, the patient had typical features of polyhydramnios, anasarca, hyperostosis of multiple bones, micrognathia, pulmonary hypoplasia, and hepatomegaly. The patient was hypotonic, and due to pulmonary hypoplasia and persistent pulmonary hypertension, had to be supported with high frequency ventilation throughout the entire hospital course. Due to the disease entity itself, as well as prolonged parenteral nutrition, liver failure progressed, and the patient expired on day 38 when uncontrolled septic shock was superimposed. The chromosome karyotype of the patient was normal, 46, XX, and COL1A1 gene mutation was not detected.
Edema ; Ethnic Groups ; Gestational Age ; Hepatomegaly ; High-Frequency Ventilation ; Humans ; Hyperostosis ; Hyperostosis, Cortical, Congenital ; Hypertension, Pulmonary ; Infant, Newborn ; Infant, Premature ; Karyotype ; Korea ; Liver Failure ; Micrognathism ; Parenteral Nutrition ; Polyhydramnios ; Shock, Septic

Purpose: To investigate the risk factors associated with hypospadias in very low birth weight (VLBW) infants (VLBWIs). Methods: We retrospectively analyzed 729 infants born at ≥24 weeks of gestational age and weighing <1,500 g from January 2012 to December 2022. We assessed the prevalence of hypospadias by birth weight percentiles and also compared the demographics and placental histopathology of the infants with hypospadias (n=52) and those without hypospadias (n=677). Results: Of the 729 patients analysed, hypospadias was recorded in 26 (20.3%), 14 (26.9%), and 12 (2.5%) infants in the <3rd, ≥3rd–<10th, and ≥10th–<90th percentiles, respectively. Of all of the patients with hypospadias, 50% had birth weights <3rd percentile (p<0.001). The hypospadias group demonstrated a longer mean gestational age (30.1 weeks vs. 27.9 weeks, p<0.001), lower incidence of maternal pregnancy-induced hypertension (48.1% vs. 17.3%, p<0.001), lower incidence of premature rupture of membrane (11.5% vs. 27.1%, p=0.013), lower acute chorioamniotic maternal response (9.1% vs. 35.2%, p<0.001), and higher maternal underperfusion (95.5% vs. 71.9%, p<0.001). Conclusion The frequency of hypospadias was found to be the highest among VBLWIs <3rd percentile, who were severely small for their gestational age (SGA). Additionally, the incidence increased with a decreasing birth weight. Physical examination is necessary at birth for VLBWIs classified as SGA. Moreover, the data on the incidence of hypospadias among VLBWIs in neonatal intensive care units can assist in tracking counseling from the prenatal to the postnatal period for patients born <3rd percentile.

Purpose: The advances in the treatment of retinoblastoma have enabled salvaging the globe in advanced stages with intra-arterial chemotherapy (IAC). We developed a strategy of alternate application of systemic intravenous chemotherapy (IVC) and IAC (referred to as alternate systemic IVC and IAC; ASIAC) to reduce central nervous metastases during IAC and examined its efficacy and safety in eye globe salvage in this study. Materials and Methods: Between January 2010 and February 2021, 43 eyes of 40 patients received ASIAC treatment for retinoblastoma at the Yonsei Cancer Center, Yonsei University Health System. Their medical records were reviewed retrospectively to evaluate the eye salvage rate (ESR), defined from diagnosis to enucleation. High-risk retinoblastoma was defined as group D or E by the International Classification of Retinoblastoma. Results: The study enrolled 38 and five cases of high-risk and low-risk retinoblastoma, respectively. In total, 178 IAC and 410 IVC courses were administered, with a median of 4 (interquartile range [IQR], 3.0 to 5.0) IAC and 9 (IQR, 6.0 to 11) IVC courses per eye, respectively. The 5-year ESR was 60.4%±8.7% for the whole cohort, 100% for low-risk retinoblastoma, and 53.6%±9.8% for high-risk retinoblastoma. Among those diagnosed since 2015, the 5-year ESR for high-risk retinoblastoma was 63.5%±14.0%. Fifteen eyes underwent enucleation; no viable tumor was found in three enucleated eyes. There were no deaths in this cohort. Conclusion Primary IAC-IVC (i.e., ASIAC) for patients with retinoblastoma was tolerable and effective in salvaging the eye and maintaining survival.