• Journal of Chest Surgery
• /
• 제55권2호
• /
• pp.158-167
• /
• 2022

Background: This study investigated mortality and morbidity in patients requiring postcardiotomy extracorporeal membrane oxygenation (ECMO) support after operations for congenital heart disease (CHD). Methods: CHD patients requiring postoperative ECMO support between May 2011 and May 2021 were retrospectively reviewed. Patients were divided into non-survivors and survivors to hospital discharge. Survival outcomes and associations of various factors with in-hospital death were analyzed. Results: Fifty patients required postoperative ECMO support. Patients' median age and weight at the time of ECMO insertion were 1.85 months (interquartile range [IQR], 0.23-14.5 months) and 3.84 kg (IQR, 3.08-7.88 kg), respectively. Twenty-nine patients (58%) were male. The median duration of ECMO support was 6 days (IQR, 3-12 days). Twenty-nine patients (58%) died on ECMO support or after ECMO weaning, and 21 (42%) survived to hospital discharge. Postoperative complications included renal failure (n=33, 66%), bleeding (n=11, 22%), and sepsis (n=15, 30%). Prolonged ECMO support (p=0.017), renal failure (p=0.005), continuous renal replacement therapy (CRRT) application (p=0.001), sepsis (p=0.012), bleeding (p=0.032), and high serum lactate (p=0.002) and total bilirubin (p=0.017) levels during ECMO support were associated with higher mortality risk in a univariate analysis. A multivariable analysis identified CRRT application (p=0.013) and a high serum total bilirubin level (p=0.001) as independent risk factors for death. Conclusion: Postcardiotomy ECMO should be considered as an important therapeutic modality for patients unresponsive to conventional management. ECMO implementation strategies and management in appropriate patients without severe complications, particularly renal failure and/or liver failure, are crucial for achieving positive outcomes.

• 대한자기공명의과학회:학술대회논문집
• /
• 대한자기공명의과학회 2003년도 제8차 학술대회 초록집
• /
• pp.99-99
• /
• 2003

To exhibit our clinical experience of diffusion-weighted (DW) MR imaging for various brain pathologies and to determine its role in characterizing brain pathologies in children. DW images in 177 children (M：F＝96：81, mean age, 4.7 years) with various brain pathologies were retrospectively collected over past 3 years. DW images (b value： 1000 s/mm) were reviewed along with corresponding apparent diffusion coefficient (ADC) maps. Brain pathologies included cystic or solid brain tumor (n = 55), cerebral infarct (n = 32), cerebritis with or without brain abscess (n = 21), metabolic or toxic brain disorder (n = 19), demyelinating disease (n = 16), hypoxic-ischemic encephalopathy (n = 16), intracerebral hemorrhage including traumatic brain lesion (n = 15), and posterior reversible leukoencephalopathy (n = 3). We reviewed whether DW images and ADCmaps contribute to further characterization of brain pathologies by defining a chronological age of lesions, the presence of cytotoxic edema in lesions, and the nature of cystic lesions.

• Clinical and Experimental Pediatrics
• /
• 제58권11호
• /
• pp.434-439
• /
• 2015

Purpose: Kawasaki disease involves acute febrile systemic vasculitis that can cause a variety of symptoms by affecting various organs. Here, we aimed to evaluate the prevalence, causes, and prognosis of sensorineural hearing loss (SNHL) occurring in children with Kawasaki disease. Methods: Patients who were diagnosed with Kawasaki disease and received inpatient treatment in the Pediatrics Department at one of three university hospitals in Daegu city from February 2012 to September 2012 were enrolled in the study. The clinical features, hematological results, echocardiography results, audiometry results, and aspirin and salicylic acid serum levels of the patients were evaluated. Results: Of the 59 children enrolled in the study, three showed mild bilateral SNHL on audiometry tests conducted after 48 hours of defervescence; these patients demonstrated normal patterns of recovery on follow-up tests 8 weeks later. Aspirin serum levels were significantly higher in the SNHL group after 48 hours of afebrile condition with high dose aspirin intake (P=0.034). However, no significant differences were found in other laboratory tests or for fever duration (P>0.05). Upon echocardiography, coronary artery abnormality was observed in 9 cases, but none of these patients showed hearing loss. Conclusion: The results indicate that SNHL in children with Kawasaki disease might occur during treatment of the acute phase; this SNHL usually involves mild bilateral hearing loss and recovers naturally. However, this study suggests that determination of the causes and clinical implications of hearing loss in Kawasaki disease requires long-term follow-up studies with more cases.

• Journal of Genetic Medicine
• /
• 제19권1호
• /
• pp.22-26
• /
• 2022

Cerebrotendinous xanthomatosis (CTX) is a rare genetic disease caused by a deficiency of enzymes for the synthesis of bile acid, resulting in the accumulation of cholestanol with reduced chenodeoxycholic acid (CDCA) production and causing various symptoms such as chronic diarrhea in infancy, juvenile cataracts in childhood, tendon xanthomas in adolescence and young adulthood, and progressive neurologic dysfunction in adulthood. Because oral CDCA replacement therapy can effectively prevent disease progression, early diagnosis and treatment are critical in CTX. This study reports the case of CTX in a 10-year-old male who presented with Achilles tendon xanthoma and mild intellectual disability. Biochemical testing showed normal cholesterol and sitosterol levels but elevated cholestanol levels. Genetic testing showed compound heterozygous variants of CYP27A1, c.379C>T (p.Arg127Trp), and c.1214G>A (p.Arg405Gln), which confirmed the diagnosis of CTX. The patient had neither cataracts nor other focal neurologic deficits and showed no abnormalities on brain imaging. The patient received oral CDCA replacement therapy without any adverse effects; thereafter, the cholestanol level decreased and no disease progression was noted. The diagnostic possibility of CTX should be considered in patients with tendon xanthoma and normolipidemic conditions to prevent neurological deterioration.

• 대한영상의학회지
• /
• 제81권4호
• /
• pp.756-769
• /
• 2020

소아의 다양한 응급질환 중 급성 폐질환 또는 급성 기도질환은 영상의학과 의사가 자주 대하게 되는 임상 상황이며, 일차적으로 시행되는 영상검사는 흉부 방사선사진이다. 따라서 다양한 임상 상황에서의 감별진단과 영상 소견을 숙지하는 것이 중요하다. 본 종설에서는 급성폐질환의 다양한 원인과 폐렴을 알아보고, 폐렴과 감별해야 하는 급성 폐질환을 생각해보았다. 급성 기도질환으로는 크룹, 급성 후두염, 기관연화증, 천식, 감염 후 폐쇄세기관지염, 그리고 이물 흡인을 검토하였다. 이렇게 소아에서 고려해야 할 질환들의 영상 소견을 검토하여 진단과 치료에 도움을 줄 수 있길 바란다.

• 대한유전성대사질환학회지
• /
• 제14권1호
• /
• pp.42-47
• /
• 2014

Gaucher disease is a multisystemic disorder arising from a deficient activity of the lysosomal enzyme glucocerebrosidase, which leads to accumulation of glycosylceraide and other glycolipids in the regiculoendothelial system. The characteristics of Gaucher disease are anemia, thrombocytopenia, hepatosplenomegaly, and skeletal disease. Enzyme replacement therapy (ERT) has been proven to prevent progressive manifestations of Gaucher disease and effective in improving anemia, thrombocytopenia, bone markers and biomarkers. However, some patient needs still remain unmet because of the inaccessibility of certain sites including brain, bone and various organs. ERT could not Improve the irreversible lesion such as liver fibrosis, hepatopulmonary syndrome, and necrosis or infarction of bone and other organs. Adult patients with Gaucher disease should be screened for longterm complication such as bone disease, pulmonary hypertension, gallstone, and cancer, especially in patients with splenectomy. Parkinsonism and polyneuropathy was also reported among patients with type 1 Gaucher disease, but ERT does not improve neurological function. We need to review the benefits and unmet needs of ERT in Gaucher disease.

• Clinical and Experimental Pediatrics
• /
• 제46권2호
• /
• pp.207-209
• /
• 2003

가와사끼병은 주로 심혈관계를 침범하는 전신적인 염증 질환이다. 8세 이상의 나이가 많은 소아에서 가와사끼병은 발생률이 낮고 비정형적인 다양한 임상양상을 보이기 때문에 진단이 늦어질 수 있다. 저자들은 10세 남아에서 급성 신부전과 근염을 비롯하여 여러 가지 비정형적인 증상을 보임으로써 진단이 늦어진 가와사끼병을 경험하였기에 보고하는 바이다.

• 부모자녀건강학회지
• /
• 제6권1호
• /
• pp.18-30
• /
• 2003

Congenital heart disease is the most frequently shown congenital disease among children, most of them can be corrected with operation. However, Patients and their parents need nursing intervention when they face this risky incident of operation. Therefore, parents' experiences are absolutely needed to plan nursing intervention to give practical help to the patients and their parents. The purpose of this study is to provide basic resources to develop a feasible intervention program for the parents by understanding the experiences related to the informed consent of cardiac surgery of their child. From January to June 2003, 10 parents of children patients with congenital heart disease were interviewed who filled out Operation Agreement before the primary operation after they are diagnosed as congenital heart disease in pediatric chest surgery of A hospital in Seoul. They were asked to give opinion regarding pre-operation needs and the interviewed information was analyzed. The results of this study are as follows : Firstly, they were asked what they felt before they were told about the operation of their children from the doctor before filling out the informed consent of operation. They felt 1) vague, 2) confusion of choose, 3) risky, 4) resented, 5) uneasy, 6) guilty, and 7) the burden of operation. Secondly, they were asked what they felt after they filled out the informed consent of operation and the doctor gave them detailed information on the operation of their children. They felt 1) confused, 2) responsible, 3) rejected, 4) angry, 5) plain, 6) to have hope, 7) trying to trust medical people, 8) that consolation is needed, and 9) conditional reduction of the burden of operation. Thirdly, followings are the categories of congenital parents' demand before operation based on the analysis of experiences related to the preparation of the informed consent of operation. 1) Information Demand (1) Anticipatory information (2) Concrete and precise information (3) Individual information 2) Support (1) Empathy (2) Parental supporting (3) Support of parents in the same situation 3) Education and consulting (1) Children-oriented Education (2) Consultation Considering the results of this study, parents of the child with congenital heart disease seem to have various emotional experiences related to filling out the informed consent and they need concrete and practical helps before cardiac surgery. This study proposes that systematic nursing intervention is needed according to the needs of the parents who have the child with congenital heart disease before operation in the field of child health nursing.

• 한국학교보건학회지
• /
• 제37권1호
• /
• pp.12-21
• /
• 2024

Purpose: This study aimed to provide basic data for future infectious disease prevention educational programs targeting preschoolers by verifying the effects of the 2023 Home-visiting Infectious Disease Prevention Class Project for Preschoolers. Methods: The study is a descriptive survey that analyzed educational satisfaction of kindergarten teachers and the activity reports of visiting teachers. The effectiveness of the education on the children's side was evaluated with a quasi-experimental approach using pre- and post-test design. Data collection was conducted from September 6 to November 10, 2023. The data from 96 kindergarten teachers, 100 activity reports written by visiting teachers, 56 children in the experimental group, and 50 children in the control group were analyzed. Results: As a result of analyzing the satisfaction survey results of the kindergarten teachers and the activity reports submitted by the visiting teachers, all questions received a very positive evaluation with 4 or more out of 5 points. In addition, the children in the experimental group showed statistically significant positive changes both in hand-washing accuracy and their awareness of immunity. Conclusion: The Home-visiting Infectious Disease Prevention Class Project for Preschoolers is of great educational significance and is expected to be a useful educational intervention that can improve infection prevention behaviors and show practical preventive effects in preschoolers so that they can be prepared for various infectious diseases in the future.

• Child Health Nursing Research
• /
• 제2권1호
• /
• pp.69-92
• /
• 1996

The purpose of study was : 1) to analyze the trend of research on the family with chronically ill children in Korea, 2) to suggest direction for future study on the family with chronically ill children, and contributing to the use of intervention in family nursing practice. Research studies on the family with chronically ill children were selected from the Korean Nusre, the Korean Nurses' Academic Society Journal, and from dissertations, which were conducted between 1975 and 1995. The total numbers of the studies were 35. These studies were analyzed for 1)time of publication or presentation, 2)research design, 3)characteristics of subjects, 4) type of chronic disease, 5)main concepts, 6)measurement tool, 7) the sis for a degree or nondegree, 8) result of correlational studies. The findings of the analysis were as follows : 1) The numbers of studies on the family with chronically ill children have increas rapidly the early 1990's. In research design, the numbers of survey research studies were the highest. Especially, the most frequently research design was the correlational survey. There were 19 correlational studies(25.7%) during the early 1990's. 2) The subjects in 16 studies(45.7%) were mother of chronically ill children and, in 8 studies (22.9% ) were their parents. 3) In most types of chronic diseases, there were 14 hematooncologic disease(32.6%) and 14 hadicapped children (32.6% ). 4) Frequently used research concepts were stress, degree of coping or way of coping, social support, parents' support, family functioning, intensity of family and family adaptation. 5) Acceding to the results of correlational studies, the more family stress was higher the more degree of coping, family functioning, intensity of family and degree of family adaption was lower. The more degree of social support was higher the more stress was lower and degree of coping, family functioning and intensity of family was higher. The more family functioning was higher the more intensity of family and family adaptation was higher. 6) 24 researches on the family with chronically ill children were done for a thesis for a degree and 11 were nondegree research studies. The following suggestions are made based on the above findings : 1) The pattern of these studies related to the family with chronically ill children in domain of Nursing need to be compared with trend in other domains. 2) More replicated research on the family with chronically ill children is needed to develop family nursing intervention and prove the effect of that and more qualitative research on the family with chronically ill children is needed to comprehensive indepth the family with chronically ill children. 3) Further research on the family with chronically ill children is needed to verify subjects and type of chronic disease, develop applicable measurement tools in Korea and identify relation between other concepts. 4) Family nursing researchers should make an effort to apply research result in various clinical settings and community settings, and try to carry out not only team research with clinical nurse but also other multidisciplinary researcher related to the family.