The gene encoding solute carrier family 9 member 6 (SLC9A6) on Xq26.3 is associated with Christianson syndrome (CS) mimicking Angelman syndrome. In CS, developmental and epileptic encephalopathy (DEE) appears in about 20%, and DEE with spike-and-wave activation in sleep (SWAS) is reported only in several cases. A 10-year-old boy with DEE showed multidrug resistant focal seizures from 6 months of age. He had progressive microcephaly, regression, global developmental delay without speech, hyperkinesia, and truncal ataxia; he had a long thin face, esotropia, and happy demeanor. Brain magnetic resonance imaging demonstrated cerebellar atrophy. Electroencephalogram at 7.5 years of age showed nearly continuous diffuse paroxysms in slow wave sleep. The seizures were responsive to corticosteroids for a while. Trio whole exome sequencing exhibited a likely pathogenic variant of SLC9A6 in the proband and his asymptomatic mother: c.1194dup (p.Leu399AlafsTer12).This is a rare case report of CS with DEE-SWAS in a Korean patient.

Purpose: : This study aims to explore nursing activities after the decision to discontinue life-sustaining treatment, awareness of a good death, and perception of life-sustaining treatment decisions among nurses in intensive care units (ICUs) at tertiary general hospitals. Methods: : Participants were 173 nurses working in two tertiary general hospitals. The data were collected using structured questionnaires and analyzed using an independent t-test, paired t-test, one-way ANOVA, Scheffé’s test, and Pearson’s correlation coefficient. Results: : Participants were 173 nurses working in two tertiary hospitals. The nursing activity increase was the greatest in the spiritual domain, and the physical domain was where the activities decreased the most. There were significant associations between Awareness of good death (Clinical) and Perception of life-sustaining treatment decision(r=.26, p＜.001), Awareness of good death (Closure) and Perception of life-sustaining treatment decision(r=.36, p＜.001), and Awareness of good death (Personal control) and Perception of life-sustaining treatment decision(r=.49, p＜.001). Conclusion : Based on the results, systematic education programs and job training are required to improve the awareness regarding good death and perception of life-sustaining treatment decision for nurses in ICUs where discontinuing lifesustaining treatment decisions are made.

Background: Lichen planus-like keratosis (LPLK), also known as benign lichenoid keratosis, usually presents as a single, brown to red, scaly, flat-topped plaque. Several large-scale studies have reported LPLK in Caucasians; however, no study has reported LPLK in Koreans. Objective: To investigate the clinicopathological features of LPLK in Koreans. Methods: We retrospectively investigated 60 patients with LPLK at Yeungnam University Hospital between 2001 and 2019. Diagnosis of LPLK was confirmed by histopathological examination in all patients. Results: Most patients (96.6%) had a single lesion. The most common location was the face (63.3%), followed by the trunk (16.7%), and upper extremities (11.7%). Mean patient age was 62.7 years, and the female:male ratio was 1.3:1. The initial clinical impression involved seborrheic keratosis in 56.7% of cases and LPLK in only 16.7% of cases. Solar elastosis (58.3%) and red blood cell extravasation (55%) were also commonly observed in this study population. Histopathological findings revealed focal parakeratosis (35%), dermal eosinophils (30%), and plasma cells (10%). Conclusion LPLK may commonly be misdiagnosed, because it is often clinically indistinguishable from seborrheic keratosis and other epithelial neoplasms. Skin biopsy findings and clinicopathological correlation are therefore necessary for differential diagnosis. Several histopathological features, such as parakeratosis as well as dermal eosinophil and plasma cell infiltration, can distinguish LPLK from lichen planus.

OBJECTIVES: Stress is considered a causal factor in many diseases, allergic disease being one of them. The prevalence of allergic disease is increasing in Korea, but the relationship between allergic symptoms and stress is not empirically well known. We aimed to evaluate the relationship between allergy-related symptoms and stress in children and adolescents. METHODS: We investigated 698 children and adolescents living in Gwangyang Bay, Korea, using a multi-stage cluster sampling method. Using the International Study of Asthma and Allergies in Childhood and the Psychosocial Well-being Index, these subjects were surveyed on allergy-related symptoms and psychosocial stressors in their lives, respectively. We used a multivariate logistic analysis for odds ratios for the complaint rate of allergic symptoms, after adjusting for age, gender, household income, body mass index, and residence. RESULTS: After adjustments, lifetime rhinitis (odds ratio [OR], 1.024), rhinoconjunctivitis (OR, 1.090), diagnosis of itchy eczema (OR, 1.040), treatment of itchy eczema (OR, 1.049), 12-month allergic conjunctivitis (OR, 1.026), diagnosis of allergic conjunctivitis (OR, 1.031), and treatment of allergic conjunctivitis (OR, 1.034) were found to be significantly associated with stress. CONCLUSIONS: Our results support the notion that there is a relationship between stress and allergic symptoms in children and adolescents. Further research into any causal relationship between stress and allergies, as well as preventative public health plans for decreasing stress in children and adolescents are needed.
Adolescent ; Child ; Cluster Analysis ; Conjunctivitis, Allergic/diagnosis/psychology/therapy ; Cross-Sectional Studies ; Eczema/diagnosis/psychology/therapy ; Female ; Humans ; Hypersensitivity/diagnosis/epidemiology/*psychology ; Interviews as Topic ; Logistic Models ; Male ; Questionnaires ; Republic of Korea ; Rhinitis/diagnosis/psychology ; *Stress, Psychological

Since 2015, the Health Insurance Review and Assessment Service (HIRA) has performed annual qualitative assessments of asthma management provided by all medical institutions that care for asthma patients in Korea. According to the third report of qualitative assessment of asthma management in 2017, the assessment appears to have contributed to improving the quality of asthma care provided by medical institutions, especially primary clinics. However, there is still a gap between the ideal goals of asthma management and actual health care policies/regulations in real clinical settings, which leads to the state of standstill with respect to the quality of asthma management despite considerable efforts such as the qualitative assessment of asthma management by national agencies such as the HIRA. At this point, a harmonized approach is needed to raise the level of asthma management among several components including medical policies, efforts of academic associations such as education and distribution of the guideline for management, and reliable financial support by the government.
Asthma* ; Delivery of Health Care ; Education ; Financial Support ; Humans ; Insurance, Health* ; Korea* ; Physicians, Primary Care

Torsion of the fallopian tube is an uncommon cause for acute low abdominal pain in female that is difficult to diagnose. Because it has no pathognomonic clinical symptoms or laboratory findings, a high index of suspicion is important when there is history of pelvic pathologic conditions or surgery. The early diagnosis and surgical treatment of the disease are mandatory to preserve oviduct.
Abdominal Pain ; Adolescent* ; Animals ; Early Diagnosis ; Fallopian Tubes* ; Female ; Humans ; Oviducts

Gynandroblastoma is an extremely rare sex cord-stromal tumor with both female (granulosa cell tumor) and male (Sertoli-Leydig cell tumor) elements. Juvenile granulosa cell tumors are also very rare and are so named because they usually occur in children and adolescents. A 71-year-old woman with right upper quadrant abdominal pain visited our hospital. Pelvic computed tomography showed a large multilocular cystic mass, suspected to be of ovarian origin. We performed a total abdominal hysterectomy (total abdominal hysterectomy was performed) with bilateral salpingo-oophorectomy. A 13-cm multilocular cystic mass with serous fluid was observed in her right ovary. Upon microscopic examination, the solid component of the mass showed both Sertoli-Leydig cell and juvenile granulosa cell differentiation, which we diagnosed as gynandroblastoma. Gynandroblastoma with a juvenile granulosa cell tumor component is extremely rare and, until now, only six cases have been reported in the English literature. We report the first gynandroblastoma with a juvenile granulosa cell tumor component diagnosed in an elderly patient, along with a literature review.
Abdominal Pain ; Adolescent ; Aged ; Child ; Female ; Granulosa Cell Tumor ; Granulosa Cells ; Humans ; Hysterectomy ; Male ; Ovary ; Postmenopause ; Sex Cord-Gonadal Stromal Tumors