• Title/Summary/Keyword: Pediatric approach

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Clinical characteristics and prognostic factors for survival in hemophagocytic lymphohistiocytosis (혈구탐식성 림프조직구증의 임상적 특징과 생존에 미치는 예후인자)

  • Kim, Khi Joo;Kim, Ki Hwan;Lee, Taek Jin;Chun, Jin-Kyong;Lyu, Chuhl Joo;Kim, Dong Soo
    • Clinical and Experimental Pediatrics
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    • v.51 no.3
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    • pp.299-306
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    • 2008
  • Purpose : Hemophagocytic lymphohistiocytosis (HLH) is a rare but fatal disorder characterized by fever, splenomegaly, pancytopenia, and hemophagocytosis in the bone marrow and other tissues. In this study, we investigated the clinical manifestations and prognostic factors in patients with HLH. Methods : We retrospectively analyzed the data from 29 patients who were diagnosed whit HLH in the Severance Children's Hospital from Jan. 1996 to Feb. 2007. Results : The median age at diagnosis was 3.8 years (range 0.1-12.2). The ratio of male to female patients was 1.1:1. The 5-year overall survival rate was 55.2% with a median follow-up duration of 32 months. In a multivariate analysis, the duration of fever before admission (survival vs. non-survival, 6.5 days vs. 14 days, P=0.010), the interval from the day of fever onset to the day of initiation of etoposide (survival vs. non-survival, 10 days vs. 35 days, P=0.002) and the presence of neurologic symptoms (survival vs. non-survival, 1 case vs. 7 cases, P=0.010) were independent, poor prognostic factors of HLH. EBV infection, gender, and the level of serum ferritin had no relations to the poor prognosis of the disease. Conclusion : This study showed that the presence of neurologic symptoms and a longer duration of fever were related to a poor prognosis. Therefore, if a patient develops neurologic symptoms and the duration of fever is prolonged, a prompt diagnostic approach and aggressive treatment for HLH are necessary.

Novel Gap Junction Molecules, Connexin 37, Enhances the Bystander Effect in HSVtk/GCV Gene Therapy (Herpes Simplex Virus thymidine Kinase/Ganciclovir 유전자 치료에서 새로운 간격결합분자 Connexin 37에 의한 방관자 효과의 증가)

  • Kim, Sun Young;Yi, Ho Keun;Lee, Jung Chang;Hwang, Dong Jin;Hwang, Pyoung Han;Lee, Dae Yeol;Cho, Soo Chul
    • Clinical and Experimental Pediatrics
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    • v.46 no.6
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    • pp.541-547
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    • 2003
  • Purpose : Gap junction intercellular communication(GJIC) is an important mechanism of the bystander effect in herpes simplex thymidine kinase/ganciclovir(HSVtk/GCV) gene therapy Therefore, we attempted to enhance the bystander effect in vitro by exogenous overexpressing connexin 37(Cx37) in cells to increase GJIC. Methods : NIH3T3 cells were transfected with the Cx37 and HSVtk gene or the HSVtk gene alone by the calcium phosphate method, and we detected their expression from these cells by RT-PCR. GCV-mediated cytotoxicity and the bystander effect of each transfectant was then assessed and compared. Results : Cells transfected with HSVtk became sensitive to low concentration of GCV. We found significantly increased cytotoxicity in HSVtk/GCV gene therapy after introduction of the HSVtk and Cx37 genes together compared with the cytotoxicity seen after introduction of the HSVtk gene in vitro. Co-expression of the HSVtk and Cx37 genes potentiates HSVtk/GCV gene therapy through the bystander effect. Conclusion : These results indicated that the increase of GJIC using Cx37 have potentiated the bystander effect of HSVtk/GCV therapy, and may be a new approach to improve response in suicidal cancer gene therapy.

Growth promoting effect of combined gonadotropin releasing hormone analogue and growth hormone therapy in early pubertal girls with predicted low adult heights (예측성인신장이 작은 조기사춘기 여아에서 성선자극호르몬 방출호르몬 효능약제와 성장호르몬 병합치료의 성장획득 효과)

  • Hong, Eun-Jeong;Han, Heon-Seok
    • Clinical and Experimental Pediatrics
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    • v.50 no.7
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    • pp.678-685
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    • 2007
  • Purpose : Recent reports pointed out that gonadotropin releasing hormone analogue (GnRHa) therapy alone is not so promising for improving adult height in precocious puberty. So, that we studied the growth promoting effect of combined therapy with GnRHa and growth hormone (GH) in early pubertal girls. Methods : Twenty three early pubertal girls ($9.73{\pm}1.59yr$) with predicted adult heights (PAH) below-2 standard deviation score (SDS) were included. They were divided into two groups as follows; Group I before menarche (n=19) and Group II after menarche (n=4). After combined therapy, various growth parameters were compared between two groups and between the before and after therapy. Results : Between the two groups before therapy, chronologic age (CA), growth velocity (GV), body mass index (BMI), target height (TH), PAH and serum insulin-like growth factor binding protein-3 were not different, but BA, height and difference between bone age (BA) and CA were significantly higher and insulin-like growth factor-1 (IGF-1) was marginally higher in group II. After therapy, BA still remained higher in group II, but other parameters were not different. In both groups, after therapy, the difference between BA and CA, the ratio of BA over CA, and GV were significantly decreased, but PAH, height SDS and BMI were significantly increased. Regarding IGF-1 level, a significant increase was noted in group I, but not in group II. Conclusion : With combined therapy of GnRHa and GH, PAH in early pubertal girls might be improved significantly and even approach TH. Among them, those who were before menarche might have greater potential for the height gain than those after menarche in view of IGF-1 changes during therapy.

Clinical Outcome of Multicystic Dysplastic Kidney in 46 Children (다낭성 이형성 신 환아 46명의 예후와 관련한 임상적 고찰)

  • Jeong Il-Cheon;Hwang You-Sik;Ahn Sun-Young;Han Sang-Won;Lee Jae-Seung
    • Childhood Kidney Diseases
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    • v.10 no.1
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    • pp.27-32
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    • 2006
  • Purpose : Conservative management of multicystic dysplastic kidney(MCDK) without nephrectomy has recently been advocated. The purpose of this study was to determine the clinical course of conservatively managed MCDK and to find out possible predictive factors for involution of MCDK by ultrasonography(US). Methods : A retrospective analysis was made on 45 patients(26 boys and 20 girls) in whom MCDK was detected and had been traced by US between Dec. 1993 and Aug. 2005 at Severance Hospital. Results : Median follow-up time was 30 months(range 2-102 months). All patients under-went radionuclide scans and voiding eystourethrograms. The serial follow-up US showed complete involution in 11(24%), partial involution in 19(41%), and no interval change or increased in cyst size in 13(28%) patients. Nephrectomy was done in 3 patients(7%) due to relapsing urinary tract infection(UTI) and severe abdominal distension. The mean age of complete involution of MCDK was 37 months(range 12-84 months). Episodes of UTI were present in 17 patients(37%) and additional genitourinary(GU) abnormalities were found in 22 patients(44%). Hypertension and renal insufficiency was complicated in one patient. No child developed malignant tumor. Univariate analysis showed that five variables were associated with complete involution of the MCDK; gender, site, UTI episode, additional GU abnormalities, and renal length on initial US. After adjusting using the Pearson model, the presence of additional GU abnormalities was exclusively associated with complete involution among the 5 variables(P=0.034). Conclusion : In our review of 46 cases of MCDK, non-surgical approach for patients with MCDK was advisable and we could predict poor prognosis when MCDK is associated with other GU anomalies.

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A Study on improvement of curriculum in Nursing (간호학 교과과정 개선을 위한 조사 연구)

  • 김애실
    • Journal of Korean Academy of Nursing
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    • v.4 no.2
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    • pp.1-16
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    • 1974
  • This Study involved the development of a survey form and the collection of data in an effort-to provide information which can be used in the improvement of nursing curricula. The data examined were the kinds courses currently being taught in the curricula of nursing education institutions throughout Korea, credits required for course completion, and year in-which courses are taken. For the purposes of this study, curricula were classified into college, nursing school and vocational school categories. Courses were directed into the 3 major categories of general education courses, supporting science courses and professional education course, and further subdirector as. follows: 1) General education (following the classification of Philip H. phoenix): a) Symbolics, b) Empirics, c) Aesthetics. 4) Synthetics, e) Ethics, f) Synoptic. 2) Supporting science: a) physical science, b) biological science, c) social science, d) behavioral science, e) Health science, f) Educations 3) Professional Education; a) basic courses, b) courses in each of the respective fields of nursing. Ⅰ. General Education aimed at developing the individual as a person and as a member of society is relatively strong in college curricula compared with the other two. a) Courses included in the category of symbolics included Korean language, English, German. Chines. Mathematics. Statics: Economics and Computer most college curricula included 20 credits. of courses in this sub-category, while nursing schools required 12 credits and vocational school 10 units. English ordinarily receives particularly heavy emphasis. b) Research methodology, Domestic affair and women & courtney was included under the category of empirics in the college curricula, nursing and vocational school do not offer this at all. c) Courses classified under aesthetics were physical education, drill, music, recreation and fine arts. Most college curricula had 4 credits in these areas, nursing school provided for 2 credits, and most vocational schools offered 10 units. d) Synoptic included leadership, interpersonal relationship, and communications, Most schools did not offer courses of this nature. e) The category of ethics included citizenship. 2 credits are provided in college curricula, while vocational schools require 4 units. Nursing schools do not offer these courses. f) Courses included under synoptic were Korean history, cultural history, philosophy, Logics, and religion. Most college curricular 5 credits in these areas, nursing schools 4 credits. and vocational schools 2 units. g) Only physical education was given every Year in college curricula and only English was given in nursing schools and vocational schools in every of the curriculum. Most of the other courses were given during the first year of the curriculum. Ⅱ. Supporting science courses are fundamental to the practice and application of nursing theory. a) Physical science course include physics, chemistry and natural science. most colleges and nursing schools provided for 2 credits of physical science courses in their curricula, while most vocational schools did not offer t me. b) Courses included under biological science were anatomy, physiologic, biology and biochemistry. Most college curricula provided for 15 credits of biological science, nursing schools for the most part provided for 11 credits, and most vocational schools provided for 8 units. c) Courses included under social science were sociology and anthropology. Most colleges provided for 1 credit in courses of this category, which most nursing schools provided for 2 creates Most vocational school did not provide courses of this type. d) Courses included under behavioral science were general and clinical psychology, developmental psychology. mental hygiene and guidance. Most schools did not provide for these courses. e) Courses included under health science included pharmacy and pharmacology, microbiology, pathology, nutrition and dietetics, parasitology, and Chinese medicine. Most college curricula provided for 11 credits, while most nursing schools provide for 12 credits, most part provided 20 units of medical courses. f) Courses included under education included educational psychology, principles of education, philosophy of education, history of education, social education, educational evaluation, educational curricula, class management, guidance techniques and school & community. Host college softer 3 credits in courses in this category, while nursing schools provide 8 credits and vocational schools provide for 6 units, 50% of the colleges prepare these students to qualify as regular teachers of the second level, while 91% of the nursing schools and 60% of the vocational schools prepare their of the vocational schools prepare their students to qualify as school nurse. g) The majority of colleges start supporting science courses in the first year and complete them by the second year. Nursing schools and vocational schools usually complete them in the first year. Ⅲ. Professional Education courses are designed to develop professional nursing knowledge, attitudes and skills in the students. a) Basic courses include social nursing, nursing ethics, history of nursing professional control, nursing administration, social medicine, social welfare, introductory nursing, advanced nursing, medical regulations, efficient nursing, nursing english and basic nursing, College curricula devoted 13 credits to these subjects, nursing schools 14 credits, and vocational schools 26 units indicating a severe difference in the scope of education provided. b) There was noticeable tendency for the colleges to take a unified approach to the branches of nursing. 60% of the schools had courses in public health nursing, 80% in pediatric nursing, 60% in obstetric nursing, 90% in psychiatric nursing and 80% in medical-surgical nursing. The greatest number of schools provided 48 crudites in all of these fields combined. in most of the nursing schools, 52 credits were provided for courses divided according to disease. in the vocational schools, unified courses are provided in public health nursing, child nursing, maternal nursing, psychiatric nursing and adult nursing. In addition, one unit is provided for one hour a week of practice. The total number of units provided in the greatest number of vocational schools is thus Ⅲ units double the number provided in nursing schools and colleges. c) In th leges, the second year is devoted mainly to basic nursing courses, while the third and fourth years are used for advanced nursing courses. In nursing schools and vocational schools, the first year deals primarily with basic nursing and the second and third years are used to cover advanced nursing courses. The study yielded the following conclusions. 1. Instructional goals should be established for each courses in line with the idea of nursing, and curriculum improvements should be made accordingly. 2. Course that fall under the synthetics category should be strengthened and ways should be sought to develop the ability to cooperate with those who work for human welfare and health. 3. The ability to solve problems on the basis of scientific principles and knowledge and understanding of man society should be fostered through a strengthening of courses dealing with physical sciences, social sciences and behavioral sciences and redistribution of courses emphasizing biological and health sciences. 4. There should be more balanced curricula with less emphasis on courses in the major There is a need to establish courses necessary for the individual nurse by doing away with courses centered around specific diseases and combining them in unified courses. In addition it is possible to develop skill in dealing with people by using the social setting in comprehensive training. The most efficient ratio of the study experience should be studied to provide more effective, interesting education Elective course should be initiated to insure a man flexible, responsive educational program. 5. The curriculum stipulated in the education law should be examined.

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