Resistance to thyroid hormone syndrome (RTH) is a genetic disease caused by the mutation of either the thyroid hormone receptor-β (THRB) gene or the thyroid hormone receptor-α (THRA) gene. RTH caused by THRB mutations (RTH-β) is characterized by the target tissue's response to thyroid hormone, high levels of triiodothyronine and/or thyroxine, and inappropriate secretion of thyroid-stimulating hormone (TSH). THRA mutation is characterized by hypothyroidism that affects gastrointestinal, neurological, skeletal, and myocardial functions. Most patients do not require treatment, and some patients may benefit from medication therapy. These syndromes are characterized by decreased tissue sensitivity to thyroid hormones, generating various clinical manifestations. Thus, clinical changes of resistance to thyroid hormones must be recognized and differentiated, and an approach to the practice of personalized medicine through an interdisciplinary approach is needed.
Lee, So Hee;Chung, Un Sun;Hwang, Sun Yung;Jeong, Jae Hoon;Kim, Eun Ji;Woo, Jeong Min;Jo, Hyun Young
Journal of the Korean Academy of Child and Adolescent Psychiatry
/
v.26
no.4
/
pp.258-265
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2015
Objectives : Children with attention-deficit hyperactivity disorder (ADHD) have problems in social interactions. We compared the effect of 10-session social skill training (SST) among two groups, children with pure ADHD, and those with ADHD with comorbidity. Methods : Consecutive 10-session SST was conducted for 34 children from 2006 to 2012. There were 22 children with pure ADHD (male 20, female 2), and 12 children suffering from ADHD with comorbidity (male 11, female 1). All children took medication as prescribed by their doctors before the start of SST. The Child Behavior Checklist (CBCL), the Korean Personality Inventory for Children (K-PIC), the Conner's Rating Scale, the ADHD Rating Scale, and the Home Situation Questionnaire were completed by mothers before and after the SST. All children completed the Child Depression Inventory, the Stat-Trait Anxiety Inventory for Children, the Self-Concept Scale and the ADHD Diagnostic System before and after the SST. Results : Only children with pure ADHD showed improvement in anxiety and self-concept in scales rated by children. In the CBCL rated by parents, the pure ADHD group and the ADHD with comorbidity showed improvement in both externalizing and internalizing subscales. In the K-PIC rated by parents, the pure ADHD group showed improvement in most outcomes and ADHD with comorbidity showed positive change in verbal development. Conclusion : These results suggest that SST has significant positive effects on both the pure ADHD and ADHD with comorbidity group. Further research is needed in order to target diverse comorbidity groups with ADHD to improve the effectiveness of the SST.
Journal of the Korean Academy of Child and Adolescent Psychiatry
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v.9
no.2
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pp.154-164
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1998
Medication is widely accepted as an effective method to reduce the problem of attention deficit, hyperactivity, impulsivity, resistance and violence of ADHD children. However, it does not provide us with the solution on the conflicting routinized behavioral patterns to gain a high level of self-control and acceptable behavior. As a way of replacing medication, this study applies the social skills training program for ADHD children and measures the level of improvement of social skills and the change of the behavioral patterns. The experiment is carried out on 16 children ranged from 6 to 13 years of age for 10 weeks. The patients are divided into three groups:a pure ADHD group, an ADHD group with conduct disorder, an ADHD group with mental retardation and other symptoms. The change of symptoms and the change of social skills are measured by the Child Behavior Checklist(CBCL), the ADD-H Comprehensive Teacher’s Rating Scale(ACTeRS) and the Social Skills Rating Scale(SSRS), and finally Mastson Evaluation of Social Skills for Youth(MESSY). Wilcoxon signed ranks test is used to evaluate the effect of the treatment, and Kruskal-Wallis test is also used to measure the change after the treatment in each of the three groups. In the ADHD group with conduct disorder, the examination of the effect of the treatment shows a significant reduction of violence in the area of behavior(p<.05), and a significant difference of activity and social skills in the area of social competent(p<.001). In the ADHD group with mental retardation and other symptoms, a significant rise of social skills is found in the area of social skills evaluation (p<.05). However, there is no significant difference of effect by the treatment among the three groups. In addition, the current examination shows that the social skills training program does not make a statistically significant contribution to the social skills of the ADHD children. On the other hand, the training helps some children, when it is suitable for the characteristics and accompanying symptoms of the children:it reduces the level of violence in the ADHD group with conduct disorder, and it raises the social skills in the ADHD group with mental retardation. In other words, the social skills training program will reduce the conduct disorder and helps peer relation for ADHD children.
Purpose : The aim of the present study was to ascertain whether the increase of carotid intima-media thickeness(cIMT) as one of premature pathologic changes of atherosclerosis, was present in obese children compared to normal weight children. Methods : The obese group consisted of 21 obese/overweight(body mass index(BMI) above 85 percentile of age, sex standards) children and the control group of 11 normal weight children. None of the children had any chronic illnesses or previous medication history. We investigated the age, sex, height, weight, and systolic/diastolic blood pressure. We measured cIMT by ultrasonogram. In 19 of the obese group, we tested the serum glucose level, liver transaminase level, and cholesterol level etc. Results : The increase of cIMT in obese group did not achieved statistical significance(obese group vs. control group; 0.42 vs. 0.40 mm, P=0.0592). In addition, cIMT showed no significant correlation with any physical/laboratory variables including BMI(P=0.0585). Conclusion : To our knowledge, this is the first study to measure the cIMT in Korean children. Though the results approached statistical significance, we could not prove an increase of cIMT in obese children or an association between cIMT and BMI, due to the study's small sample size. In the future, larger and more extensive trials are needed.
Journal of the korean academy of Pediatric Dentistry
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v.25
no.4
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pp.710-716
/
1998
It is one of difficulties to control children who show definitely negative behavior in dental clinic. In such a case, the pharmacologic management has been used to provide quality care, minimize the extremes of disruptive behavior, promote a positive psychologic response to treatment and patient welfare and safety. Deep sedation can be defined as a controlled, pharmacologically-induced state of depressed consciousness from which the patient is not easily aroused which may be accompanied by a partial loss of protective reflexes. In this retrospective report, the sedation records of 200 pediatric dental patients of ASA Class I & II who were not successfully treated under conscious sedation were used for analysis. Most frequently used regimen of deep sedation was the co-medication of midazolam(0.3mg/kg), enflurane(1.0-2.0 vol%) and 50-70% $N_2O_2$. The average age and weight of the patients was 4.6 yr (S.D: 2.72) and 18.7kg(S.D: 6.35) respectively. The average operative time was 52 minutes and midazolam (0.1-0.2cc) was additionally administered intranasally to prolong the operative time as needed. The episodes of untoward side effects were reported during and/or after the procedure in 58 patients. Serious adverse reactions such as cyanosis or laryngospasm were even reported in 7 patients but without mortality. Deep sedation is a very effective way of completing the dental treatments for those who failed to respond well to the conscious sedation. This technique has many practical advantages over general anesthesia case but the demands for the rigid monitoring criteria limit its use in general practice setting. The continuous efforts to improve the safety of the medication and the technique are required for the benefits of the patients and parent.
Since the initial International Society of Heart Lung Transplantation registry was published in 1982, the number of pediatric heart transplantations has increased markedly, reaching a steady state of 500-550 transplantation annually and occupying up to 10% of total heart transplantations. Heart transplantation is considered an established therapeutic option for patients with end-stage heart disease. The long-term outcomes of pediatric heart transplantations were comparable to those of adults. Issues affecting long-term outcomes include acute cellular rejection, antibody-mediated rejection, cardiac allograft vasculopathy, infection, prolonged renal dysfunction, and malignancies such as posttransplant lymphoproliferative disorder. This article focuses on medical issues before pediatric heart transplantation, according to the Korean Network of Organ Sharing registry and as well as major problems such as graft rejection and cardiac allograft vasculopathy. To reduce graft failure rate and improve long-term outcomes, meticulous monitoring for rejection and medication compliance are also important, especially in adolescents.
Very few studies regarding the effects of preoperative nursing intervention on children's welfare after surgery have been conducted. The purpose of this study was 1) to evaluate the effects of preoperative nursing instructions on children's physical recovery and emotional state after surgery and 2) to analyse the relationship between the effects of preoperative nursing instructions and the age and general anxiety of child. The study was conducted flow: August 15th to October 30th at Severance Hospital, Seoul, Korea. A total of 41 Pediatric surgical Patients, between the age of 4 to 14, participated in the study. Twenty patients, randomly selected, were visited by the experimenter in the evening before, surgery and received specific preoperative nursing instructions. These instructions were designed to alleviate emotional stress and were adopted to the age of child. The postoperative recovery of these patients were then compared with a randomly selected control group of 21 patients who received only the routine preoperative care by the staff in the hospital Dependent variable were vomiting, pain medication, ability to void, elevation of temperature, infection, the between surgery and hospital discharge and postoperative emotional stress which was measured by the vital signs. Because of the limitation of the sample size it was difficult to obtain valid statistical results. However, the analysis of the raw data indicates that: 1) the preoperative nursing instructions appears to promote physical recovery and it seems especially effective in preventing elevation of temperature and shortening the length of the hospital stay, 2) the preoperative nursing instruction also seems to be effective in relieving the child's emotional stress(situational anxiety) after surgery, 3) the patient's general anxiety level preparatively seems to be a predictor of postoperative problems, 4) the preoperative nursing instructions were an effective means of promoting physical recovery in every age group as long as the child understood the instructions. The above data would seem to indicate that all preoperative pediatric patients four years of age or older, if they can understand, should have preoperative nursing instruction in order to alleviate postoperative stress and enhance physical recovery. The level of general anxiety preparatively deserves special attention since the amount of nursing instruction needed seems to vary with tile level of anxiety.
Cheshmi, Behzad;Jafari, Zahra;Naseri, Mohammad Ali;Davari, Heidar Ali
Maxillofacial Plastic and Reconstructive Surgery
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v.42
/
pp.26.1-26.6
/
2020
Background: Orofacial clefts (OFCs) comprise a wide range of malformations, including cleft lip, cleft palate, and cleft lip with cleft palate, which can vary in terms of etiology, severity, and disease burden. Objective(s): This study aimed to evaluate the correlation between various risk factors and orofacial cleft disorder spectrum in newborns. Study design: A total of 323 cases and 400 controls were enrolled in this study and evaluated in terms of the maternal history of abortion or miscarriage, child's sex, maternal and paternal age, maternal history of systemic disease, history of medication therapy during pregnancy, birth order, consanguineous marriage, and complications during pregnancy. Results: Analysis of the results suggested that consanguineous marriage, a maternal history of abortion/miscarriage, and complications during pregnancy could potentially increase the risk of OFCs in children (P < 0.05). However, the analyses revealed that the other variables could not potentially increase the risk of OFCs (P > 0.05). Conclusion(s): Multiple cofactors may simultaneously contribute to the formation of such abnormalities; therefore, a comprehensive, multidisciplinary care program is necessary to ensure a successful pregnancy period and the birth of a healthy newborn.
Purpose: Attention deficit hyperactivity disorder (ADHD) is a common disorder in school-aged children. Patients with restless legs syndrome (RLS) often present with ADHD symptoms and vice versa. This study was the first to attempt to identify the prevalence of RLS and sleep problems in children with ADHD in Korea. Methods: Patients diagnosed with ADHD were asked to complete a sleep questionnaire. The sleep questionnaire included items to help identify the presence of four typical symptoms that are used as diagnostic criteria for RLS. Results: A total of 56 patients, including 51 boys and 5 girls (mean age, 10.7 years old) participated. Of these, 24 complained of pain, discomfort, or an unpleasant sensation in the legs. Based on the RLS diagnostic criteria, 2 patients were diagnosed with definite RLS and 4 with probable RLS. There were no significant differences in age, medication dosage, or neuropsychological test scores between the patients with and without RLS symptoms. Conclusion: Approximately 42.9% of patients with ADHD presented with RLS symptoms and 7.1% of these were diagnosed with RLS. Patients with ADHD also experienced various other sleep disorders. Thus, appropriate assessment and treatment for sleep disorders in patients with ADHD is essential.
Myocarditis was previously attributed to an epidemic viral infection. Additional harmful reagents, in addition to viruses, play a role in its etiology. Coronavirus disease 2019 (COVID-19) vaccine-induced myocarditis has recently been described, drawing attention to vaccine-induced myocarditis in children and adolescents. Its pathology is based on a series of complex immune responses, including initial innate immune responses in response to viral entry, adaptive immune responses leading to the development of antigen-specific antibodies, and autoimmune responses to cellular injury caused by cardiomyocyte rupture that releases antigens. Chronic inflammation and fibrosis in the myocardium eventually result in cardiac failure. Recent advancements in molecular biology have remarkably increased our understanding of myocarditis. In particular, microRNAs (miRNAs) are a hot topic in terms of the role of new biomarkers and the pathophysiology of myocarditis. Myocarditis has been linked with microRNA-221/222 (miR-221/222), miR-155, miR-10a*, and miR-590. Despite the lack of clinical trials of miRNA intervention in myocarditis yet, multiple clinical trials of miRNAs in other cardiac diseases have been aggressively conducted to help pave the way for future research, which is bolstered by the success of recently U.S. Food and Drug Administration-approved small-RNA medications. This review presents basic information and recent research that focuses on myocarditis and related miRNAs as a potential novel biomarker and the therapeutics.
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