RESUMEN
Purpose@#This study was conducted to update the previous evidence-based nursing clinical practice guidelines for pressure injury. @*Methods@#The guideline was revised in 22 steps based on the international standards. @*Results@#The updated nursing practice guideline for pressure injury consisted of four domains and 494 recommendations. The numbers of recommendations in each domain was: 31 hospital policy, 64 assessment, 386 prevention and management, and 13 education. The recommended grade was 10.3% for A, 13.8% for B, and 75.9% for C. Of these, the major revision was done in 40 recommendations (8.1%). A total of 55 recommendations (11.0%) were newly added. 25 recommendations had minor revisions such as changes or additions for some words, and only one recommendation was deleted. @*Conclusion@#The revised nursing practice guidelines for pressure injury are expected to serve as an evidence-based practice guideline for pressure injury in Korea. This guideline will provide healthcare providers, patients, and caregivers with information to help prevent and manage pressure injuries, leading to improving patient outcomes.
RESUMEN
Purpose@#Extravasation of diagnostic and therapeutic materials might occur when the intravascular solution leaks into the surrounding tissues. Injury associated with extravasation depends on various factors. It may range from mild skin reaction to severe necrosis. However, the incidence rate for extravasation is largely unknown because of the limited reporting in Korea. Therefore, this study was conducted to identify the incidence of extravasation and nurses’ attitude and knowledge of extravasation for providing high-quality nursing care. @*Methods@#Three acute care hospitals were surveyed to estimate the occurrence of extravasation. Knowledge and attitude toward extravasation were investigated from 793 nurses working in six hospitals. @*Results@#The incidence rate of extravasation was 0.5%. Extravasation commonly occurred in elderly patients aged 66 or older (59.9%) and internal medicine (48.2%), and it happened 13.73±20.68 days after hospitalization on average.It mostly occurred in the forearm site (52.9%) and was mainly caused by parenteral nutrition (33.6%). The mean scores of nurses’ knowledge and attitude were 14.63±2.86 and 28.91±36.00, respectively. There was a significant negative correlation between the subjects' knowledge and attitude (r=-.11, p=.002). @*Conclusion@#It is necessary to have a reporting system that can accurately monitor the occurrence of extravasation for patient safety management. In addition, it is necessary to develop a protocol that can be applied to clinical practice and a nurse education program.
RESUMEN
The interaction of mating pheromone and pheromone receptor from the B mating-type locus is the first step in the activation of the mushroom mating signal transduction pathway.The B mating-type locus of Lentinula edodes is composed of Bα and Bβ subloci, each of which contains genes for mating pheromone and pheromone receptor. Allelic variations in both subloci generate multiple B mating-types through which L. edodes maintains genetic diversity. In addition to the B mating-type locus, our genomic sequence analysis revealed the presence of a novel chromosomal locus 43.3 kb away from the B mating-type locus, containing genes for a pair of mating pheromones (LPHBN1 and PHBN2) and a pheromone receptor (RCBN). The new locus (Bα-N) was homologous to the Bα sublocus, but unlike the multiallelic Bα sublocus, it was highly conserved across the wild and cultivated strains. The interactions of RcbN with various mating pheromones from the B and Bα-N mating-type loci were investigated using yeast model that replaced endogenous yeast mating pheromone receptor STE2 with RCBN. The yeast mating signal transduction pathway was only activated in the presence of PHBN1 or PHBN2 in the RcbN producing yeast, indicating that RcbN interacts with self-pheromones (PHBN1 and PHBN2), not with pheromones from the B matingtype locus. The biological function of the Bα-N locus was suggested to control the expression of A mating-type genes, as evidenced by the increased expression of two A-genes HD1 and HD2 upon the treatment of synthetic PHBN1 and PHBN2 peptides to the monokaryotic strain of L. edodes.
RESUMEN
Purpose@#Some patients with neonatal seizures show diffuse, symmetric diffusion-restricted lesions in the cerebral white matter. The aim of this study was to describe clinical and imaging findings of patients with neonatal seizures who had diffuse, symmetric diffusion-restricted lesions without any structural or metabolic etiology. @*Materials and Methods@#A total of 56 neonates aged less than 1 week underwent brain magnetic resonance imaging (MRI) for evaluation of seizures from November 2008 to February 2017. After excluding 43 patients, 13 patients showed diffuse white matter abnormality on diffusion-weighted imaging. Initial and follow-up clinical and MRI findings were analyzed retro-spectively. @*Results@#All 13 patients were born at full term. Among the ten patients who underwent a stool test for viruses, six were positive for rotavirus and one for astrovirus. MRI revealed diffuse, symmetric diffusion-restricted lesions distributed along the cerebral white matter, thalami, and midbrain variably. @*Conclusion@#Diffuse, symmetric diffusion-restricted lesions involving the cerebral white matter can be seen in patients with neonatal seizures without any structural or metabolic etiology. Rotavirus is commonly but not exclusively detected in these patients. Nevertheless, viral infection-associated encephalopathy should be considered for patients with characteristic clinical and MRI findings.
RESUMEN
Patients with neurological disorders are at high risk of developing osteoporosis, as they possess multiple risk factors leading to low bone mineral density. Such factors include inactivity, decreased exposure to sunlight, poor nutrition, and the use of medication or treatment that can cause lower bone mineral density such as antiepileptic drugs, ketogenic diet, and glucocorticoids. In this article, mechanisms involved in altered bone health in children with neurological disorders and management for patients with epilepsy, cerebral palsy, and Duchenne muscular dystrophy regarding bone health are reviewed.
RESUMEN
Purpose@#The aim of this study was to develop an evidence-based guideline for stoma management providing institutional policy, assessment, complications and follow-up care. Methods: The guideline adaptation manual consisting of 23 steps developed by the National Evidence-Based Healthcare Collaborating Agency was used for this study. It presents an overview of the process used to develop the guideline and lists specific recommendations from the guideline. @*Results@#It provides 55 recommendations that include the following 8 topics: 1) Organization and policy recommendations, 2) Preoperative nursing; Ostomy education, stoma site marking, 3) Ostomy formation, 4) Postpoperative nursing; education, assessment, high output stoma management, 5) Selection of ostomy products, 6) Colostomy irrigation, 7) Stomal and peristomal complications, 8) Follow-up care after discharge. @*Conclusion@#The guideline can be used to address stoma management in hospital settings. The intent of the guideline is to provide information that will assist healthcare providers to manage adult patients with ostomies, prevent or decrease complications, and improve patients’ outcomes.
RESUMEN
BACKGROUND@#AND PURPOSE: The US Food and Drug Administration approval for perampanel has only recently been expanded to patients as young as 4 years, and so there have been few real-life studies of the effects of perampanel in pediatric patients. The aim of this study was to determine the long-term efficacy, factors affecting treatment response, and tolerability of perampanel as an add-on therapy in pediatric patients aged 4 years or older with epilepsy.@*METHODS@#This multicenter retrospective observational study collected data from pediatric epilepsy centers of four Korean national universities. Changes in the seizure frequency from baseline, adverse events, and retention rates were obtained at 3, 6, and 12 months. Adverse events and discontinuation profiles were obtained to assess tolerability.@*RESULTS@#This study included 220 children and adolescents (117 males and 103 females) aged 4 to 20 years. The overall response rate was 43.6%, and the seizure-freedom rate was 17.7%. Factors affecting a good treatment response were the absence of intellectual disability, small number of concomitant antiepileptic drugs, and low baseline seizure frequency. Eighty-eight patients (40%) experienced adverse events, but they mostly were of mild severity and resolved after the dose reduction or discontinuation of perampanel. The retention rates at 3, 6, and 12 months were 85.0%, 71.8%, and 50.5%, respectively.@*CONCLUSIONS@#Adjunctive treatment with perampanel was efficacious and tolerated in pediatric patients aged 4 years or older with epilepsy. Early perampanel treatment may help to reduce the burden of their seizures and improve their quality of life.
RESUMEN
PURPOSE: Epilepsy is the most common neurological disorder in childhood. Hospital nurses, who are the first to recognize seizures in epilepsy patients in the ward environment, possess expertise related to epilepsy and play a central role in epilepsy management. The purpose of this study was to develop an algorithm-based education program and to improve nurses' knowledge and self-efficacy related to providing nursing care to children with epilepsy. METHODS: The education program consisted of lectures on the definition, cause, classification, diagnosis, treatment, and nursing of epilepsy based on a booklet, as well as practice using an algorithm for nursing interventions when a child experiences a seizure. Twenty-seven nurses working at pediatric neurological wards and a pediatric emergency room participated in the education program. The data were analyzed using descriptive statistics and the paired t-test. RESULTS: Nurses' knowledge and self-efficacy showed a statistically significant improvement after participation in the education program on nursing care for children with epilepsy. CONCLUSION: The application of this education program for hospital setting is expected to improve nurses' capability to care for children with epilepsy, thereby contributing to a higher quality of nursing.
Asunto(s)
Niño , Humanos , Clasificación , Diagnóstico , Educación , Educación en Enfermería , Servicio de Urgencia en Hospital , Epilepsia , Clase , Enfermedades del Sistema Nervioso , Atención de Enfermería , Enfermería , Folletos , ConvulsionesRESUMEN
Epilepsy is one of the most common chronic neurological disorder affecting 6–7 per 1000 worldwide. Nearly one-third of patients with newly diagnosed epilepsy continue to have recurrent seizures despite adequate trial of more than two anti-seizure drugs : drug-resistant epilepsy (DRE). Children with DRE often experience cognitive and psychosocial co-morbidities requiring more urgent and aggressive treatment than adults. Epilepsy surgery can result in seizure-freedom in approximately two-third of children with improvement in cognitive development and quality of life. Understanding fundamental differences in etiology, co-morbidity, and neural plasticity between children and adults is critical for appropriate selection of surgical candidates, appropriate presurgical evaluation and surgical approach, and improved overall outcome.
Asunto(s)
Adulto , Niño , Humanos , Epilepsia Refractaria , Epilepsia , Enfermedades del Sistema Nervioso , Plásticos , Calidad de Vida , ConvulsionesRESUMEN
PURPOSE: This study was conducted to update the existing evidence-based nursing clinical practice guideline in management of pressure injury in South Korea. METHODS: The update process underwent the 22 steps according to the update method based on the international standards. RESULTS: The updated nursing practice guideline for pressure injury consists of 4 domains and 436 recommendations. The numbers of recommendations in each domain were 25 for hospital policies, 51 for assessment, 350 for prevention and management, and 10 for education. There were 2.5% of A, 13.3% of B, 84.2% of C in terms of grading of recommendations. Among these, the major revision was done in 32 recommendations (7.4%). A total of 299 recommendations (68.6%) were added newly. Minor revisions, such as change or addition of some words, were also made in 25 recommendations(5.7%). No change was made in 80 recommendations (18.3%) compared to the previous ones. CONCLUSION: The nursing practice guideline for pressure injury has been updated. This updated guideline can be used as educational materials for both healthcare workers and patients with pressure injury.
Asunto(s)
Humanos , Atención a la Salud , Educación , Enfermería Basada en la Evidencia , Corea (Geográfico) , Métodos , Enfermería , Úlcera por PresiónRESUMEN
Epilepsy is one of the most common chronic neurological disorder affecting 6–7 per 1000 worldwide. Nearly one-third of patients with newly diagnosed epilepsy continue to have recurrent seizures despite adequate trial of more than two anti-seizure drugs : drug-resistant epilepsy (DRE). Children with DRE often experience cognitive and psychosocial co-morbidities requiring more urgent and aggressive treatment than adults. Epilepsy surgery can result in seizure-freedom in approximately two-third of children with improvement in cognitive development and quality of life. Understanding fundamental differences in etiology, co-morbidity, and neural plasticity between children and adults is critical for appropriate selection of surgical candidates, appropriate presurgical evaluation and surgical approach, and improved overall outcome.
Asunto(s)
Adulto , Niño , Humanos , Epilepsia Refractaria , Epilepsia , Enfermedades del Sistema Nervioso , Plásticos , Calidad de Vida , ConvulsionesRESUMEN
In the originally published version of this article, complete anonymity was not achieved.
RESUMEN
Combined oxidative phosphorylation deficiency-17 (COXPD-17) is very rare and is caused by homozygous or compound heterozygous mutations in the ELAC2 gene on chromosome 17p12. The ELAC2 gene functions as a mitochondrial tRNA processing gene, and only 4 different pathogenic mutations have been reported in ELAC2-associated mitochondrial dysfunction involving oxidative phosphorylation. Affected patients show various clinical symptoms and prognosis, depending on the genotype. We report a novel mutation in the ELAC2 gene (c.95C>G [p.Pro32Arg], het), in an infant with COXPD-17 who presented with encephalopathy including central apnea and intractable epilepsy, and growth and developmental retardation. During hospitalization, consistently elevated serum lactic acid levels were noted, indicative of mitochondrial dysfunction. The patient suddenly died of shock of unknown cause at 5 months of age. This is the first case report of COXPD-17 in Korea and was diagnosed based on clinical characteristics and genetic analysis.
Asunto(s)
Humanos , Lactante , Encefalopatías , Epilepsia Refractaria , Genotipo , Crecimiento y Desarrollo , Hospitalización , Hiperlactatemia , Corea (Geográfico) , Ácido Láctico , Fosforilación Oxidativa , Pronóstico , ARN de Transferencia , Choque , Apnea Central del SueñoRESUMEN
BACKGROUND: Megalencephalic leukoencephalopathy with subcortical cysts (MLC) is a rare inherited disorder characterized by infantile-onset macrocephaly, slow neurologic deterioration, and seizures. Mutations in the causative gene, MLC1, are found in approximately 75% of patients and are inherited in an autosomal recessive manner. We analyzed MLC1 mutations in five unrelated Korean patients with MLC. METHODS: Direct Sanger sequencing was used to identify MLC1 mutations. A founder effect of the p.Ala275Asp variant was demonstrated by haplotype analysis using single-nucleotide polymorphic (SNP) markers. Multiple ligation-dependent probe amplification (MLPA) and comparative genomic hybridization plus SNP array were used to detect exonic deletions or uniparental disomy (UPD). RESULTS: The most prevalent pathogenic variant was c.824C>A (p.Ala275Asp) found in 7/10 (70%) alleles. Two pathogenic frameshift variants were found: c.135delC (p.Cys46Alafs*12) and c.337_353delinsG (p.Ile113Glyfs*4). Haplotype analysis suggested that the Korean patients with MLC harbored a founder mutation in p.Ala275Asp. The p.(Ile113Glyfs*4) was identified in a homozygous state, and a family study revealed that only the mother was heterozygous for this variant. Further analysis of MLPA and SNP arrays for this patient demonstrated loss of heterozygosity of chromosome 22 without any deletion, indicating UPD. The maternal origin of both chromosomes 22 was demonstrated by haplotype analysis. CONCLUSIONS: This study is the first to describe the mutational spectrum of Korean patients with MLC, demonstrating a founder effect of the p.Ala275Asp variant. This study also broadens our understanding of the mutational spectrum of MLC1 by demonstrating a homozygous p.(Ile113Glyfs*4) variant resulting from UPD of chromosome 22.
Asunto(s)
Humanos , Alelos , Cromosomas Humanos Par 22 , Hibridación Genómica Comparativa , Exones , Efecto Fundador , Haplotipos , Leucoencefalopatías , Pérdida de Heterocigocidad , Megalencefalia , Madres , Convulsiones , Disomía UniparentalRESUMEN
Headache is one of the most common neurological symptoms reported in childhood and adolescence, leading to high levels of school absences, learning problems, and poor psychiatric conditions. Pediatric headache has been associated with several neurological and psychiatric comorbidities; in particular, depression, anxiety disorders, sleep disorders, and epilepsy have been associated with pediatric migraine. The goal of this article is to review the comorbidities associated with pediatric headache, thereby enabling pediatric neurologists to identify shared triggers and develop synergistic management strategies that address headache and its comorbidities.
Asunto(s)
Adolescente , Niño , Humanos , Trastornos de Ansiedad , Comorbilidad , Depresión , Epilepsia , Cefalea , Aprendizaje , Trastornos Migrañosos , Trastornos del Sueño-VigiliaRESUMEN
Myasthenia gravis (MG) is a chronic autoimmune disease of neuromuscular blockade, characterized by muscle weakness and fatigue, and is associated with the production of autoantibodies against the skeletal muscle acetylcholine receptor, muscle-specific kinase (MuSK), low-density lipoprotein receptor-related protein 4 (LRP4), and other muscle endplate proteins. In addition, MG may be classified according the location of the affected muscles (ocular vs. generalized), patient age at symptom onset, and thymic pathology. Subgroup classification based on serum antibodies and clinical features include early-onset, late-onset, thymoma-associated, MuSK and LRP4 antibody-negative, and ocular forms of MG, and can help with therapeutic decisions and prognosis. Pyridostigmine is the chosen symptomatic treatment. For patients who do not adequately respond to symptomatic therapy, corticosteroids, other immunomodulating agents, and thymectomy are the first-line immunosuppressive treatments. The treatment of MG is highly individualized and depends on the age of the patient, the type and severity of the disease, and the pace of progression.
Asunto(s)
Adulto , Niño , Humanos , Acetilcolina , Corticoesteroides , Anticuerpos , Autoanticuerpos , Enfermedades Autoinmunes , Clasificación , Fatiga , Lipoproteínas , Debilidad Muscular , Músculo Esquelético , Músculos , Miastenia Gravis , Bloqueo Neuromuscular , Patología , Fosfotransferasas , Pronóstico , Bromuro de Piridostigmina , TimectomíaRESUMEN
Myasthenia gravis (MG) is a chronic autoimmune disease of neuromuscular blockade, characterized by muscle weakness and fatigue, and is associated with the production of autoantibodies against the skeletal muscle acetylcholine receptor, muscle-specific kinase (MuSK), low-density lipoprotein receptor-related protein 4 (LRP4), and other muscle endplate proteins. In addition, MG may be classified according the location of the affected muscles (ocular vs. generalized), patient age at symptom onset, and thymic pathology. Subgroup classification based on serum antibodies and clinical features include early-onset, late-onset, thymoma-associated, MuSK and LRP4 antibody-negative, and ocular forms of MG, and can help with therapeutic decisions and prognosis. Pyridostigmine is the chosen symptomatic treatment. For patients who do not adequately respond to symptomatic therapy, corticosteroids, other immunomodulating agents, and thymectomy are the first-line immunosuppressive treatments. The treatment of MG is highly individualized and depends on the age of the patient, the type and severity of the disease, and the pace of progression.
Asunto(s)
Adulto , Niño , Humanos , Acetilcolina , Corticoesteroides , Anticuerpos , Autoanticuerpos , Enfermedades Autoinmunes , Clasificación , Fatiga , Lipoproteínas , Debilidad Muscular , Músculo Esquelético , Músculos , Miastenia Gravis , Bloqueo Neuromuscular , Patología , Fosfotransferasas , Pronóstico , Bromuro de Piridostigmina , TimectomíaRESUMEN
PURPOSE: This study aimed to describe the clinical characteristics and outcomes of children with acute combined central and peripheral nervous system demyelination (CCPD); and compare with the children of isolated acute central or peripheral nervous system demyelination. METHODS: A retrospective chart review of 145 children with acute demyelinating disease between 2010 and 2015 was undertaken in children with younger than 18 years old. Among these, 96 fulfilled criteria (clinical features and positive neuroimaging or electromyography/nerve conduction studies) for either acute central (group A, n=60, 62.5%) or peripheral (group B, n=30, 31.3%) nervous system demyelination, or a CCPD (group C, n=6, 6.3%). RESULTS: Significant differences among the groups (A vs B vs C) were evident for occurrence of disease between 2013-2015 (45.0% vs 43.3% vs 83.3%; P=0.024), admission to intensive care unit (8.3% vs 26.7% vs 50.0%; P=0.027), length of hospitalization (median, 9.7 vs 12.3 vs 48.3 days; P<0.001), treatment with steroids (88.3% vs 10.0 vs 100.0%; P=0.003), immunoglobulins (13.3% vs 100.0% vs 100.0%; P=0.002) and plasmapheresis (0.0% vs 3.3% vs 50.0%; P=0.037) and severe disability at discharge (3.3% vs 16.7% vs 33.3%; P=0.012). Children of group C showed good response to simultaneous use of immunoglobulin and high-dose corticosteroids and earlier try of plasmapheresis, however, two patients had moderate degree of neurological disability. CONCLUSION: Systemic studies using neuroimaing and electromyography/nerve conduction studies in all patients with demyelinating disease will be necessary to verify the combined or isolated disease, because CCPD might have the poorer outcome than isolated disease.
Asunto(s)
Niño , Humanos , Corticoesteroides , Enfermedades Desmielinizantes , Encefalomielitis Aguda Diseminada , Síndrome de Guillain-Barré , Hospitalización , Inmunoglobulinas , Unidades de Cuidados Intensivos , Síndrome de Miller Fisher , Mielitis Transversa , Sistema Nervioso , Neuroimagen , Neuritis Óptica , Sistema Nervioso Periférico , Plasmaféresis , Estudios Retrospectivos , EsteroidesRESUMEN
PURPOSE: Venous angioma (VA) is the most common congenital abnormality of the intracranial vasculature. This study aimed to investigate the relationship between VA and epilepsy and to identify the characteristics of children with VA and epilepsy. METHODS: The records of all patients aged less than 18 years who underwent brain magnetic resonance imaging (MRI) at Pusan National University Hospital were retrospectively reviewed. Patients with isolated VA and patients with normal MRI were compared in terms of the prevalence of epilepsy. RESULTS: In total, 2,385 pediatric patients who underwent brain MRI were enrolled. Isolated VA was identified in 26 patients (VA group). Among the patients with normal MRI findings, 225 age- and sex-matched patients to the VA-group were assigned to the control group. Nine patients in the VA group (9 of 26, 34.6%) and 27 patients in the control group (26 of 225, 11.5%; P<0.001) had epilepsy. In the VA group, 20 patients (76.9%) had the VA in the cerebral hemispheres, and 6 patients (23.1%) had the VA in the brainstem and cerebellum. The latter showed a higher prevalence of epilepsy (5 of 6, 83.3%) than the former (4 of 20, 20.0%; P=0.004). Among the nine patients who had epilepsy with VA, patients whose VA involved the brainstem and cerebellum showed a significantly higher frequency of abnormal Electroencephalographic findings than patients whose VA involved the cerebral hemispheres (P=0.016). CONCLUSION: VA, especially in the brainstem and cerebellum, might be associated with epilepsy.