Pediatric management challenges of hyperglycemic hyperosmolar state: case series of Korean adolescents with type 2 diabetes

The hyperglycemic hyperosmolar state (HHS) is considered the most fatal complication of type 2 diabetes mellitus (DM). The number of case reports describing pediatric HHS has increased recently in parallel with obesity and the prevalence of type 2 DM in pediatric patients. In this study, we investigated the patient characteristics and outcomes of HHS in 9 adolescents with obesity and type 2 DM. Almost all patients exhibited mixed clinical features of HHS and diabetic ketoacidosis (DKA), including characteristics such as hyperosmolality and ketoacidosis. These features made definitive diagnosis difficult; 5 out of 9 patients were initially diagnosed with DKA and were treated accordingly. Patients who were initially diagnosed with HHS received a more vigorous and appropriate fluid replacement than other patients did. No patients died, although 3 exhibited complications, such as arrhythmia, acute kidney injury requiring renal replacement therapy, rhabdomyolysis, and acute pancreatitis. Hyperosmolality with consequent severe dehydration is considered a significant factor contributing to the outcomes of patients with HHS. Therefore, early recognition of hyperosmolality is crucial for an appropriate diagnosis and adequate fluid rehydration to restore perfusion in the early period of treatment to improve patient outcomes for this rare but serious emerging condition in pediatric patients.

The first case of novel variants of the FSHR mutation causing primary amenorrhea in 2 siblings in Korea

Follicle-stimulating hormone receptor (FSHR) mutation is a rare cause of amenorrhea. We report the first case of FSHR mutations in Korea. Two female siblings, aged 16 (patient 1) and 19 (patient 2) years, were referred to the pediatric endocrinology clinic because of primary amenorrhea despite normal breast budding. Gonadotropin-releasing hormone stimulation test showed markedly elevated luteinizing hormone and follicle-stimulating hormone with a relatively low level of estrogen, suggesting hypergonadotropic hypogonadism. Pelvic magnetic resonance imaging revealed a bicornuate uterus in patient 1 and uterine hypoplasia with thinning of the endometrium in patient 2. The progesterone challenge test revealed no withdrawal of bleeding. After two months of administration of combined oral contraceptives, menarche was initiated at regular intervals. To determine the genetic cause of amenorrhea in these patients, whole exome sequencing (WES) was performed, which revealed a compound heterozygous FSHR mutation, c.1364T>G (p.Val455Gly) on exon 10, and c.374T>G (p.Leu125Arg) on exon 4; both of which were novel mutations and were confirmed by Sanger sequencing. The patients maintained regular menstruation and improved bone mineral density while taking combined oral contraceptives, calcium, and vitamin D. Therefore, FSHR mutations can be the cause of amenorrhea in Koreans, and WES facilitates diagnosing the rare cause of amenorrhea.

Development of Parent Guidelines for Parent-Performed Developmental Screening Tests

Objectives@#Most developmental screening tests have been built as parent-performed questionnaires. However, they often do not guide parents on how to answer the questionnaire. This study aimed to develop easily applicable parent guidelines. @*Methods@#We implemented the Delphi procedure with 20 panelists. The development of the initial questionnaire was based on the results of two surveys of parents and experts provided by a policy research report that investigated the item adequacy of the Korean Developmental Screening Test. Round one included 33 items comprising all possible measurements in six categories that were identified as difficult to understand or confusing. Round two merged and modified some items and included 32 items. We defined consensus as a median agreement value of one or less and convergence and stability values of 0.5 or less. The subjective usefulness of the parent guidelines was examined based on their previous test experiences. @*Results@#Consensus was reached after the second round, reflecting the items with the highest level of accuracy in each category. Of the 167 parents who participated in the survey, 113 (67.7%) affirmed the usefulness of the guidelines, while 10 (6.0%) answered that they were not useful. Items that recommended a different scoring strategy in answering the questionnaire from their previous measurements were found to be more useful by the parents. @*Conclusion@#The parent guidelines, composed of five bullet points, drew on the consensus of the experts. Further studies are required to assess whether these guidelines improve the accuracy of screening tests in clinical settings.

Exploring Spatial Variations and Factors associated with Walking Practice in Korea: An Empirical Study based on Geographically Weighted Regression

Purpose@#Walking practice is a representative indicator of the level of physical activity of local residents. Although the world health organization addressed reduction in prevalence of insufficient physical activity as a global target, the rate of walking practice in Korea has not improved and there are large regional disparities. Therefore, this study aimed to explore the spatial variations of walking practice and its associated factors in Korea. @*Methods@#A secondary analysis was conducted using Community Health Outcome and Health Determinants Database 1.3 from Korea Centers for Disease Control and Prevention. A total of 229 districts was included in the analysis. We compared the ordinary least squares (OLS) and the geographically weighted regression (GWR) to explore the associated factors of walking practice. MGWR 2.2.1 software was used to explore the spatial distribution of walking practice and modeling the GWR. @*Results@#Walking practice had spatial variations across the country. The results showed that the GWR model had better accommodation of spatial autocorrelation than the OLS model. The GWR results indicated that different predictors of walking practice across regions of Korea. @*Conclusion@#The findings of this study may provide insight to nursing researchers, health professionals, and policy makers in planning health programs to promote walking practices in their respective communities.

A Preliminary Study for the Curriculum Development of Community Care Coordinators: Educational Needs Analysis

Purpose@#A care coordinator is an emerging nursing professional role in South Korea. The purpose of this study was to identify educational needs and priorities for care coordinators among nurses. @*Methods@#An online survey was conducted on 661 current or retired nurses from January 30 to February 28, 2021. A total of 17 essential competencies for care coordinators, recognized based on literature review, were used to analyze the educational needs. The data were analyzed using descriptive statistics, a paired t-test, and one-way analysis of variance with SPSS 25.0. The educational needs analysis was conducted by using a paired t-test, the Borich Needs Assessment Model, and the Locus for Focus Model. @*Results@#Five contents were identified as the first priorities for educational needs: ‘Health program planning and evaluation’, ‘Care planning’, ‘Coordinating community-based services’, ‘Case management’, and ‘Transitional care’. The second priorities for educational needs included 'Population health management' and'Welfare resource linkages via communicating with social workers’. @*Conclusion@#The priority items derived from this study offer underpinning insights for the development of care coordination training program.

From diagnosis to treatment of mucopolysaccharidosis type VI: A case report with a novel variant, c.1157C>T (p.Ser386Phe), in ARSB gene

Mucopolysaccharidosis type VI (MPS VI) is an autosomal recessive lysosomal disorder caused by the deficiency of arylsulfatase B due to mutations in the ARSB gene. Here, we report the case of a Korean female with a novel variant of MPS VI.A Korean female aged 5 years and 8 months, who is the only child of a healthy non-consanguineous Korean couple, presented at our hospital for severe short stature. She had a medical history of umbilical hernia and recurrent otitis media. Her symptoms included snoring and mouth breathing. Subtle dysmorphic features, including mild coarse face, joint contracture, hepatomegaly, and limited range of joint motion, were identified. Radiography revealed deformities, suggesting skeletal dysplasia. Growth hormone (GH) provocation tests revealed complete GH deficiency. Targeted exome sequencing revealed compound heterozygous mutations in the ARSB genes c.512G>A (p.Gly171Asp; a pathogenic variant inherited from her father) and c.1157C>T (p.Ser386Phe; a novel variant inherited from her mother in familial genetic testing). Quantitative tests revealed increased urine glycosaminoglycan (GAG) levels and decreased enzyme activity of arylsulfatase B. While on enzyme replacement therapy and GH therapy, her height increased drastically; her coarse face, joint contracture, snoring, and obstructive sleep apnea improved; urine GAG decreased; and left ventricular mass index was remarkably decreased. We report a novel variant—c.1157C>T (p.Ser386Phe)—of the ARSB gene in a patient with MPS VI; these findings will expand our knowledge of its clinical spectrum and molecular mechanisms.

Global health curricula in Korean nursing schools: Focusing on the changes since 2015 / 한국간호교육학회지

Purpose@#This study aimed to understand the current status of global health curricula and characteristics in nursing schools, focusing on the changes since 2015. @*Methods@#Data were collected from the websites of 202 nursing schools nationwide in Korea. Global health curricula were analyzed using a structured framework developed by the authors. @*Results@#Among 202 nursing schools, 173 (85.6%) schools offer global health-related courses. Of these, 72 (35.6%) schools offer a ‘Multiculturalism’ course, and 42 (20.8%) schools offer a ‘Global Nursing’ course. Fifty-nine schools (29.2%) offer both courses. Compared to the study findings in 2015, the number of global health-related courses and the percentage of global health-related courses designated as a requirement dramatically increased. An additional analysis of five syllabi of global-health related courses found several differences in the courses’ aims, contents and evaluation methods. @*Conclusions@#Due to social and political changes, nursing schools are more likely to offer global health curricula. However, there is still a lack of consensus on the core contents and approaches of such curricula, necessitating systematic discussions about the core contents and effective learning methods to increase nursing student competency in global health nursing.

Response to growth hormone according to provocation test results in idiopathic short stature and idiopathic growth hormone deficiency

Purpose@#To investigate growth response in children with either idiopathic short stature (ISS) or growth hormone (GH) deficiency (GHD). @*Methods@#The data of prepubertal GHD or ISS children treated using recombinant human GH were obtained from the LG Growth Study database. GHD children were further divided into partial and complete GHD groups. Growth response and factors predicting growth response after 1 and 2 years of GH treatment were investigated. @*Results@#This study included 692 children (98 with ISS, 443 partial GHD, and 151 complete GHD). After 1 year, changes in height standard deviation score (ΔHt-SDS) were 0.78, 0.83, and 0.96 in ISS, partial GHD, and complete GHD, respectively. Height velocity (HV) was 8.72, 9.04, and 9.52 cm/yr in ISS, partial GHD, and complete GHD, respectively. ΔHt-SDS and HV did not differ among the 3 groups. Higher initial body mass index standard deviation score (BMI-SDS) and midparental height standard deviation score (MPH-SDS) were predictors for better growth response after 1 year in ISS and the partial GHD group, respectively. In the complete GHD group, higher Ht-SDS and BMI-SDS predicted better growth response after 1 year. After 2 years of GH treatment, higher BMI-SDS and MPH-SDS predicted a better growth outcome in the partial GHD group, and higher MPH-SDS was a predictor of good growth response in complete GHD. @*Conclusion@#Clinical characteristics and growth response did not differ among groups. Predictors of growth response differed among the 3 groups, and even in the same group, a higher GH dose would be required when poor response is predicted.

Job Analysis of Nurse Care Coordinators for Chronic Illness Management in Primary Care Settings: Using Developing a Curriculum Process

Purpose@#This study aimed to conduct a job analysis of nurse carecoordinators and to identify the frequency, importance and difficulty of each task of their job. @*Methods@#A committee for developing a curriculum (DACUM) was formed and members of the committee defined nurse care coordinators’ jobs and enumerated the duties, tasks and task elements by applying the DACUM technique. Then nurse care coordinators enrolled in the pilot project evaluated the frequency, importance and difficulty of each task. @*Results@#From the job descriptions of nurse care coordinators, we identified 12 duties and 42 tasks. Each task comprised 1~5 task elements. Among tasks, ‘assess the patient’s general health status’ was carried out most frequently. Nurse care coordinators perceived that ‘check vital signs’ and ‘strengthen patient competence to promote health behaviors’ were more important than all other tasks. The most difficult task was ‘develop professionalism as a nurse care coordinator’. @*Conclusion@#The nurse care coordinators' roles developed in this study will serve as the key guidelines for human resource management of care coordinators. Further, job specifications for nurse care coordinators need to be developed, which is necessary for designing education and training programs. We also need to integrate primary health care as an essential component in nursing education.

Economic Evaluation of Hospital-based Home Care Services for the Breast Cancer Surgery Patients

Purpose@#This study conducted an economic evaluation of hospital-based home care services for the patients who had undergone breast cancer surgery. @*Methods@#A total of 12,483 patients over 18 years of age who had received breast cancer surgery in 26 tertiary hospitals in 2018 were analyzed with the claim data from the Health Insurance Review & Assessment Service using cost-minimization analysis and societal perspectives. @*Results@#There were 156 patients who utilized hospital-based home care services within 30 days after breast cancer surgery, and they received 2.17 (SD=1.17) hospital-based home care service on average. The average total cost was 5,250,028 KRW (SD=1,905,428) for the group receiving continuous hospital-based home care and 6,113,402 KRW (SD=2,033,739) for the group not receiving continuous hospital-based home care (p＜.001). The results of the economic evaluation of continuous hospital-based home care services in patients who had undergone breast cancer surgery indicated a total benefit of 953,691,000 KRW, a total cost of 819,004,000 KRW, and a benefit-cost ratio of 1.16 in 2018. @*Conclusion@#Continuous hospital-based home care was considered economically feasible as the total costs for the group receiving continuous hospital-based home care were lower than those of the group not receiving continuous hospital-based home care. Therefore, policy modification and financial incentives are recommended to increase the utilization of hospital-based home care services for patients who had undergone breast cancer surgery.

Concept Analysis of Parents’ Treatment Adherence for an Epileptic Child or Adolescent

Purpose@#This concept analysis was conducted to clarify ‘parents’ treatment adherence for an epileptic child or adolescent’. @*Methods@#The analysis used a hybrid model comprising three phases: theoretical phase, fieldwork phase, and integration phase. In the theoretical phase, fifty studies were reviewed. Interviews with four parents of epileptic children or adolescents were conducted during the fieldwork phase. In the integration phase, the results derived from prior phases were synthesized and clarified. All phases were performed cyclically. @*Results@#The concept, ‘parents’ treatment adherence for an epileptic child or adolescent’ was defined as parents’ voluntary and goal-directed behavior towards the epilepsy treatment for their children: a collaborative decision-making process with health-care providers, establishing a support system, adaptability to the treatment plans, and appraisals of the child’s health condition. @*Conclusion@#This achievement is thought to contribute to improving the accuracy and validity of the concept measurement. It has implications for additional research on how the concept ‘treatment adherence’ differs in diverse health problems and other population groups than parents of children and adolescents with epilepsy.

A Systematic Review of Studies Using Video-recording to Capture Interactions between Staff and Persons with Dementia in Long-term Care Facilities

PURPOSE: The use of video-recording offers important advantages in observing and assessing the relationship between specific behaviors in health care settings. The purpose of this systematic review is to investigate and synthesize the methodological characteristics of studies using video-technology for measuring interactions with the older persons with dementia and staff in long-term care facilities.METHODS: We searched Medline, Embase and CINHAL databases for published articles in English using a video-recording method for both staff and the residents with dementia. Quantitative research design studies (e.g., descriptive or experimental studies) were included.RESULTS: Among 5,605 searched papers, a total of 20 studies were selected for this review. Situations of video-recording were providing personal care (n=12), mealtime (n=6), and conversation (n=3). Concepts measured by video-recording were classified into two groups: 1) Staff [care practice by staff (n=13) and communication by staff (n=11)] and 2) residents [communication by resident (n=4), activities of daily life function of resident (n=8), and behavioral and psychological symptoms of dementia (n=10)].CONCLUSION: This review demonstrates that video technologies are actively used to evaluate the relationship between quality of care and health outcomes of the elderly with dementia in many international nursing studies. This study provides the foundation for a future research using video-recording technologies to examine the interactions and relationships between staff and the residents in Korean long-term care settings.

Trajectories of Self-rated Health among One-person Households: A Latent Class Growth Analysis

PURPOSE: The aim of this study is to explore different types of self-rated health trajectories among one-person households in Korea.METHODS: We used five time-point data derived from Korea Health Panel (2011~2015). A latent growth curve modeling was used to assess the overall feature of self-rated health trajectory in one-person households, and a latent class growth modeling was used to determine the number and shape of trajectories. We then applied multinomial logistic regression on each class to explore the predicting variables.RESULTS: We found that the overall slope of self-rated health in one-person households decreases. In addition, latent class analysis demonstrated three classes: 1) High-Decreasing class (i.e., high intercept, significantly decreasing slope), 2) Moderate-Decreasing class (i.e., average intercept, significantly decreasing slope), and 3) Low-Stable class (i.e., low intercept, flat and nonsignificant slope). The multinomial logistic regression analysis showed that the predictors of each class were different. Especially, one-person households with poor health condition early were at greater risk of being Low-Stable class compared with High-Decreasing class group.CONCLUSION: The findings of this study demonstrate that more attentions to one-person households are needed to promote their health status. Policymakers may develop different health and welfare programs depending on different characteristics of one-person household trajectory groups in Korea.

A Delphi Study for Developing a Person-centered Dementia Care Online Education Program in Long-term Care Facilities

PURPOSE: There has been a growing recognition that person-centered care enhances the quality of life of nursing home residents with dementia. This study was conducted to develop a person-centered dementia care online education program for direct care staff in long-term care facilities. METHODS: Delphi method with expert group was used to validate contents. We developed 61 draft items based on literature review. Twenty experts participated in consecutive three round surveys including 5-point Likert scale questions and open-ended questions. Based on experts' opinions, the content validity ratio for content validity and the coefficient of variation for stability were calculated. RESULTS: Three-round Delphi surveys and additional feedback from the expert panel established a consensus of core contents: 1) dementia (7 categories), 2) person-centered care (6 categories), 3) communication (8 categories), and 4) behavioral and psychological symptoms of dementia (6 categories). Specific sub-categories in each category were differentiated according to the job qualifications (65 sub-categories for registered nurses, 64 sub-categories for nursing aids, and 41 sub-categories for personal care workers). CONCLUSION: This delphi study identified person-centered dementia education curricula, in which the person-centered approach should be a key policy priority in Korean long-term care system. Now it is urgently needed to develop education programs utilizing online platforms that enable efficient and continuous learning for long-term care staff, which can contribute to behavior changes in the person-centered dementia care approach and improvement of care quality in long-term care facilities.

Evaluation and management of amenorrhea related to congenital sex hormonal disorders

Primary amenorrhea is a symptom with a substantial list of underlying etiologies which presents in adolescence, although some conditions are diagnosed in childhood. Primary amenorrhea is defined as not having menarche until 15 years of age (or 13 years with secondary sex characteristics). Various etiologies of primary amenorrhea include outflow tract obstructions, gonadal dysgenesis, abnormalities of the central nervous system, various endocrine diseases, chronic illnesses, psychologic problems, and constitutional delay of puberty. The management of primary amenorrhea may vary considerably depending on the patient and the specific diagnosis. In this article, the various causes, evaluation, and management of primary amenorrhea are reviewed with special emphasis on congenital sex hormonal disorders.

Comparison of effectiveness of growth hormone therapy according to disease-causing genes in children with Noonan syndrome / 소아과

PURPOSE: To analyze the growth response to growth hormone (GH) therapy in prepubertal patients with Noonan syndrome (NS) harboring different genetic mutations. METHODS: Twenty-three patients with prepubertal NS treated at Pusan National University Children’s Hospital between March 2009 and July 2017 were enrolled. According to the disease-causing genes identified, the patients with NS were divided into 4 groups. Three groups were positive for mutations of the PTPN11, RAF1, and SOS1 genes. The five genes undetected (FGU) group was negative for PTPN11, RAF1, SOS1, KRAS, and BRAF gene mutations. The influence of genotype was retrospectively analyzed by comparing the growth parameters after GH therapy. RESULTS: The mean chronological age at the start of GH treatment was 5.85±2.67 years. At the beginning of the GH treatment, the height standard deviation score (SDS), growth velocity (GV), and lower levels of insulin-like growth factor-1 (IGF)-1 levels were not statistically different among the groups. All the 23 NS patients had significantly increased height SDS and serum IGF-1 level during the 3 years of treatment. GV was highest during the first year of treatment. During the 3 years of GH therapy, the PTPN11, RAF1, and SOS1 groups showed less improvement in height SDS, IGF-1 SDS, and GV, and less increase in bone age-to-chronological age ratio than the FGU group. CONCLUSION: The 3-year GH therapy in the 23 prepubertal patients with NS was effective in improving height SDS, GV, and serum IGF-1 levels. The FGU group showed a better response to recombinant human GH therapy than the PTPN11, RAF1, and SOS1 groups.

Genetic overgrowth syndrome: A single center's experience

PURPOSE: Overgrowth syndromes are conditions that involve generalized or localized areas of excess growth. In this study, the clinical, molecular, and genetic characteristics of Korean patients with overgrowth syndrome were analyzed. MATERIALS AND METHODS: We recruited 13 patients who presented with overgrowth syndrome. All patients fulfilled inclusion criteria of overgrowth syndrome. Analysis of the clinical and molecular investigations of patients with overgrowth syndrome was performed retrospectively. RESULTS: Among the 13 patients with overgrowth syndrome, 9 patients (69.2%) were found to have molecular and genetic causes. Among the seven patients with Sotos syndrome (SS), two had a 5q35microdeletion that was confirmed by fluorescent in situ hybridization. In two patients with SS, intragenic mutations including a novel mutation, c.5993T>A (p.M1998L), were found by Sanger sequencing. One patient had one copy deletion of NDS1 gene which was confirmed by multiplex ligation-dependent probe amplification. Among five patients with Beckwith-Wiedemann syndrome, three had aberrant imprinting control regions; 2 hypermethylation of the differentially methylated region of H19, 1 hypomethylation of the differentially methylated region of Kv. In one patient displaying overlapping clinical features of SS, a de novo heterozygous deletion in the chromosomal region 7q22.1-22.3 was found by single nucleotide polymorphism-based microarray. CONCLUSION: Considering high detection rate of molecular and genetic abnormalities in this study, rigorous investigations of overgrowth syndrome may be an important tool for the early diagnosis and genetic counseling. A detailed molecular analysis of the rearranged regions may supply the clues for the identification of genes involved in growth regulation.

Interventions Using Technologies for Older Adults in Long-term Care Facilities: A Systematic Review

PURPOSE: Although innovative interventions using technologies have been introduced in long-term care settings, available evidence is still anecdotal. The purpose of this study is to investigate and synthesize the outcomes of interventions using technologies delivered to nursing home residents. METHODS: Published clinical trials were identified through PubMed, CINHAL, Cochrane and PsycINFO databases and manually hand-searching. Eligible studies were articles published between 1997–2016 in English or Korean with a randomized controlled trial or quasi-experimental design in which interventions using technologies were delivered to nursing home residents. RESULTS: A total of 20 studies were selected for this review. Types of interventions using technologies were classified into the electronic documentation technology (n=1), the clinical decision support system (n=1), the safety technology (n=1), the health and wellness technology (n=10), and the social connectedness technology (n=7). Overall resident outcomes indicated that interventions using technologies improved behavioral symptoms and psycho-social outcomes, but mixed results were shown in the aspects of physical function, cognitive function, social relationship and quality of service. CONCLUSION: This review demonstrates that incorporating technologies into nursing home care have positive effects on residents' psycho-social outcomes and behavioral symptoms. To disseminate the effectiveness of interventions using technologies, further research is needed to determine what mechanisms underlying such relationships exist.

Gonadal Function in Female Childhood Cancer Survivors / 임상소아혈액종양

During the last three decades childhood cancer survivorship study has extensively developed and now we have much extent of knowledge about survivor's long-term mortality and chronic illness. One of the many challenges that females survivors face includes reproductive health, which involves reproductive and sexual health changes that could later significantly reduce one's quality of life. Alkylating chemotherapy, irradiation of the CNS and/or ovaries, and pelvic and genitourinary surgery can adversely affect the reproductive organs. Increased awareness of the unique needs of female cancer survivors, timely evaluation of gonadal function, and active intervention for fertility preservation before, during, and after therapy is warranted.

Effects of Emotional Labor and Workplace Violence on Physical and Mental Health Outcomes among Female Workers: The 4th Korean Working Conditions Survey / 한국직업건강간호학회지

PURPOSE: We investigated the effects of emotional labor and workplace violence on various physical and mental health outcomes among female workers. METHODS: We obtained data from 24,760 female workers who participated in the 4th Korean Working Conditions Survey (2014). Hierarchical logistic regression analysis was used to analyze the relationships. RESULTS: Female emotional workers were more exposed to workplace violence than were female non-emotional workers. Verbal abuse was the most common type of workplace violence. Logistic regression analyses revealed that (1) emotional labor was significantly associated with higher odds of having musculoskeletal or abdominal pain (physical health outcomes), along with overall fatigue (a mental health outcome), and (2) workplace violence experiences were significantly associated with higher odds of musculoskeletal pain, headache/eye strain, abdominal pain, depression/anxiety disorder, overall fatigue, and insomnia/sleep disturbance, after controlling for covariates. CONCLUSION: This study demonstrated that both emotional labor and workplace violence have negative effects on physical and mental health. However, workplace violence experience has a stronger negative impact on health outcomes than does emotional labor alone. A management system to eradicate workplace violence and programs aimed at managing emotional labor are urgently needed at the organizational level.