PURPOSE: The aim of this study was to explore patients' experience of participation in their hospital care. METHODS: Face-to-face interviews using a semi-structured interview guide were conducted with a total of 21 patients in a tertiary hospital in Seoul, South Korea. Collected data were analyzed using a qualitative content analysis. RESULTS: Two categories of patients' experience of participation in hospital care emerged: 1) Reactive participatory activity, 2) active participatory activity. Major participatory activities included ‘complying with medical instructions’, ‘listening’, ‘responding’ and ‘questioning’. Healthcare professionals' attentive attitudes and explanation, and availability of care equipment were facilitating factors affecting patient participation in their care, whereas limited accessibility to and poor communication with healthcare professionals, inconvenient patient room, and patient factors were barriers to their participation. CONCLUSION: The findings in this study reveal that patients tend to participate in their care in a reactive way and that healthcare professionals have a significant role in facilitating patients' participation. Furthermore, the care environment and patient factors need to be considered to promote patient participation in hospital care.
Delivery of Health Care ; Humans ; Korea ; Patient Participation ; Patients' Rooms ; Seoul ; Tertiary Care Centers

Purpose: This study aimed to develop a scale to measure hospital nurses’ silence behavior and examine its validity and reliability. Methods: A total of 52 preliminary items on hospital nurses’ silence behavior were selected using a content validity test by seven experts on 53 candidate items derived from a literature review and in-depth interviews with 14 nurses. A total of 405 hospital nurses participated in a psychometric testing. Data analysis comprised item analysis, exploratory and confirmatory factor analyses, and convergent and discriminant validity tests. Pearson’s correlation coefficient was used for assessing concurrent validity, and Cronbach’s alpha was used for the reliability test. Results: The final scale consisted of nine factors with 31 items, exhibiting acceptable model fit indices, convergent validity, and discriminant validity. The score of the entire scale was positively correlated with the ‘Organizational Silence Scale (OSS)-the issues on which nurses remain silent’ (r = .60, p < .001) and ‘OSS-the reasons why nurses remain silent’ (r = .68, p < .001). Cronbach’s α of the scale was .92, and α of each subscale ranged from .71 to .90. Conclusion The Hospital Nurses’ Silence Behavior Scale is a useful tool for assessing multifaceted silence behavior among nurses. It can provide basic data for developing better communication strategies among nurses and other hospital staff.

BACKGROUND: Rehabilitations in subacute phase are different from acute treatments regarding the characteristics and required resource consumption of the treatments. Lack of accuracy and validity of the Korean Diagnosis Related Group and Korean Out-Patient Group for the acute patients as the case-mix and payment tool for rehabilitation inpatients have been problematic issues. The objective of the study was to develop the Korean Rehabilitation Patient Group (KRPG) reflecting the characteristics of rehabilitation inpatients. METHODS: As a retrospective medical record survey regarding rehabilitation inpatients, 4,207 episodes were collected through 42 hospitals. Considering the opinions of clinical experts and the decision-tree analysis, the variables for the KRPG system demonstrating the characteristics of rehabilitation inpatients were derived, and the splitting standards of the relevant variables were also set. Using the derived variables, we have drawn the rehabilitation inpatient classification model reflecting the clinical situation of Korea. The performance evaluation was conducted on the KRPG system. RESULTS: The KRPG was targeted at the inpatients with brain or spinal cord injury. The etiologic disease, functional status (cognitive function, activity of daily living, muscle strength, spasticity, level and grade of spinal cord injury), and the patient's age were the variables in the rehabilitation patients. The algorithm of KRPG system after applying the derived variables and total 204 rehabilitation patient groups were developed. The KRPG explained 11.8% of variance in charge for rehabilitation inpatients. It also explained 13.8% of variance in length of stay for them. CONCLUSION: The KRPG version 1.0 reflecting the clinical characteristics of rehabilitation inpatients was classified as 204 groups.
Brain ; Classification ; Diagnosis ; Humans ; Inpatients ; Korea ; Length of Stay ; Medical Records ; Muscle Spasticity ; Muscle Strength ; Outpatients ; Rehabilitation* ; Retrospective Studies ; Spinal Cord ; Spinal Cord Injuries

Inversion, one of the balanced rearrangements, usually does not lead to phenotypic abnormalities; all genetic information exists in the proper amount, merely in a different order or in an abnormal location. However, offspring of an inversion carrier is at risk of chromosomal imbalance because an inversion loop can be formed during crossing-over of the paternal and the maternal chromosomes in meiosis. We report a 38-year-old woman with inversion and balanced translocation and her fetus with unusual rearrangement causing chromosomal imbalance. We performed conventional cytogenetic analysis, MLPA, and subtelomeric FISH in the cells of the embryo. The results showed that the distal portion of chromosome 13q was added to the terminal portion of chromosome 9p during crossing-over. Therefore, the final karyotype of the fetus was 46,XY,rec(9)t(9;13)(p22;q32)inv(9)(p12q13)mat, confirmed using molecular-cytogenetic analyzing tools.
Adult ; Cytogenetic Analysis ; Embryonic Structures ; Female ; Fetus ; Humans ; Karyotype ; Meiosis ; Pregnancy

Exogenous nucleic acids induce an innate immune response in mammalian host cells through activation of the retinoic acid-inducible gene I (RIG-I). We evaluated RIG-I protein for RNA binding and ATPase stimulation with RNA ligands to investigate the correlation with the extent of immune response through RIG-I activation in cells. RIG-I protein favored blunt-ended, double-stranded RNA (dsRNA) ligands over sticky-ended dsRNA. Moreover, the presence of the 5'-triphosphate (5'-ppp) moiety in dsRNA further enhanced binding affinity to RIG-I. Two structural motifs in RNA, blunt ends in dsRNA and 5'-ppp, stimulated the ATP hydrolysis activity of RIG-I. These structural motifs also strongly induced IFN expression as an innate immune response in cells. Therefore, we suggest that IFN induction through RIG-I activation is mainly determined by structural motifs in dsRNA that increase its affinity for RIG-I protein and stimulate ATPase activity in RIG-I.
Adenosine Triphosphatases ; Adenosine Triphosphate* ; Hydrolysis* ; Immunity, Innate ; Interferon Type I* ; Ligands ; Nucleic Acids ; RNA ; RNA, Double-Stranded

Purpose: Osteogenesis imperfecta (OI) is a rare bone fragility disorder caused by defects in type 1 collagen biosynthesis. This study investigated the genotype-phenotype correlations and the efficacy of pamidronate therapy in patients with OI in a single academic center. Methods: This study included 24 patients with OI. A clinical scoring system was used to evaluate disorder severity. COL1A1 and COL1A2 genes were analyzed in 13 patients using Sanger sequencing. Genotype-phenotype correlations and the efficacy of pamidronate therapy were analyzed through a retrospective medical chart review. Results: Of the 24 patients, 18 (75%) were classified as type I (12 with type Ia and 6 with type Ib), 2 as type III (8.4%), and 4 as type IV (16.7%). Type Ia patients showed relatively higher lumbar bone mineral density (BMD) standard deviation scores (SDS) and lower clinical scores than those with other types. Seven patients with qualitative mutations had lower lumbar BMD-SDS (P=0.015) and higher clinical scores (P=0.008) than 6 patients with quantitative mutations. The annual fracture frequency and lumbar BMD-SDS improved in patients with qualitative mutations after pamidronate treatment. Conclusion This study demonstrated that OI patients with qualitative mutations in COL1A1/2 had a more severe phenotype than those with quantitative mutations. Patients with qualitative mutations showed a significant reduction in fracture frequency and an increase in lumbar BMD-SDS after pamidronate treatment. Clinical score and genotype might be helpful for predicting phenotype and response to pamidronate therapy in OI patients.

Background and Objectives: The increased expression for the Fms-related tyrosine kinase-4 (FLT-4, known as VEGFR-3) is relevant to dysfunctional natural killer (NK) cells in acute myeloid leukemia (AML). MAZ51 (M), a VEGFR-3 inhibiting chemical, was effectively restored the function of NK cells via the high expression of interferon-gamma (IFN-γ) in NK cells, as shown in our previous study. Although tremendous amount of clinical data using peptides are currently available in real clinic, peptides targeting FLT-4 in modulating immune cells such as NK cells are not fully elucidated. Methods: and Results: In present study, we developed peptides targeting FLT-4 (P), which is inhibiting an affinity for AML-NK expressing FLT-4 in vitro and in vivo. Bone marrow (BM) and peripheral blood (PB) mononuclear cells (MNCs) from AML patients were treated with combinational cocktails of the three agents including P, M, ara-C (A) and FLT-4 expression and IFN-γ release were examined. In an AML mouse model, IFN-γ expression were examined in T and NK cells from mouse BM, spleen, and liver to address relevance between peptides and immune cell activation. We found that AML-NK cells both in human and mouse samples showed a gradual increase the IFN-γ levels compared to the controls. There was a trend toward a reduction in leukemic blasts in the BM, spleen, and liver from the AML mice, when we compared the effects of combinational treatments. Conclusions Our results suggest that the function of AML-NK cells was synergistically activated by P in combination with M or A.

Pneumocystis pneumonia (PCP) is a rare disease in healthy people but a potentiallyfatal opportunistic infection by Pneumocystis jirovecii in immunocompromisedpatients with organ transplantation. We present three cases of PCP after kidneytransplantation in pediatric patients. First case was a 4-year-old boy diagnosedwith Denys-Drash syndrome and received living-donor kidney transplantationfrom his mother at age of 1. Second case was a 19-year-old male, with polycystickidney disease, who received kidney transplantation from his mother at the age of18. Third case was a 19-year-old female with chronic kidney disease of unknownetiology, who received kidney transplantation from her father at age of 15. Thesethree patients who were on immunosuppressive therapy and completed ofroutine PCP prophylaxis for 6 months had presented with cough and dyspneamore than 1 year after transplantation. Chest x-ray all showed diffuse haziness ofboth lung fields, and bronchoalveolar lavage from bronchoscopy revealed Pneumocystisjirovecii infection. All patients showed clinical resolution with intravenoustrimethoprim-sulfamethoxazole (TMP-SMX) therapy for at least 3 weeks and hadcontinued secondary prophylaxis for another 6–12 months. This report suggeststhat clinicians should have suspicion for the possibilities of opportunistic infectionsuch as PCP after kidney transplantation in children.

A previously healthy, 3-year-old boy presented to the emergency department with an afebrile focal motor seizure. He was found crying and having a seizure 30 minutes earlier. During this seizure, he was jerking his head and right extremities. Subsequent magnetic resonance imaging showed acute infarction in the bilateral frontal lobes, chiefly in the left. After hospitalization, conventional angiography demonstrated bilateral stenosis of the distal internal carotid arteries with development of lenticulostriate collaterals, which confirmed the diagnosis of moyamoya disease. It is vital to recognize focal motor seizures and situations related to hyperventilation in children with a seizure, which imply a structural lesion and a provoked cerebral ischemia in preexisting moyamoya disease, respectively.
Angiography ; Brain Ischemia ; Carotid Artery, Internal ; Child ; Child, Preschool ; Constriction, Pathologic ; Crying ; Diagnosis ; Emergencies ; Emergency Service, Hospital ; Extremities ; Frontal Lobe ; Head ; Hospitalization ; Humans ; Hyperventilation ; Infarction ; Ischemia ; Magnetic Resonance Imaging ; Male ; Moyamoya Disease ; Seizures ; Stroke ; Vasoconstriction

Purpose: Deficiency of 21-hydroxylase (21-OHD) is an autosomal recessively inherited disorder that is characterized by adrenal insufficiency and androgen excess. This study was performed to investigate the clinical utility of prenatal diagnosis of 21-OHD using molecular genetic testing in families at risk. Methods: This study included 27 pregnant women who had previously borne a child with 21-OHD. Fetal tissues were obtained using chorionic villus sampling (CVS) or amniocentesis. After the genomic DNA was isolated, Sanger sequencing of CYP21A2 and multiplex ligation-dependent probe amplification were performed. The clinical and endocrinological findings were reviewed retrospectively. Results: A total of 39 prenatal genetic tests was performed on 27 pregnant women and their fetal tissues. The mean gestational age at the time of testing was 11.7 weeks for CVS and 17.5 weeks for amniocentesis. Eleven fetuses (28.2%) were diagnosed with 21-OHD. Among them, 10 fetuses (90.9%) harbored the same mutation as siblings who were previously diagnosed with 21-OHD. Among these, 4 fetuses (3 males and 1 female) identified as affected were born alive. All 4 patients have been treated with hydrocortisone, 9α-fludrocortisone, and sodium chloride since a mean of 3.7 days of life. The male patients did not show hyponatremia and dehydration, although they harbored pathogenic variants associated with the salt-wasting type of 21-OHD. Conclusion This study demonstrated the diagnostic efficacy and clinical consequences of diagnosis by prenatal genetic testing in families at risk for 21-OHD. All patients identified as affected were treated with hydrocortisone and 9α-fludrocortisone early after birth, which can prevent a life-threatening adrenal crisis.