• Asian Pacific Journal of Cancer Prevention
• /
• 제15권21호
• /
• pp.9495-9498
• /
• 2014

Background: The difference in prognosis of adult and childhood acute lymphoblastic leukemia (ALL) can be attributed largely to variation in cytogenetic abnormalities with age groups. Cytogenetic analysis in acute leukemia is now routinely used to assist patient management, particularly in terms of diagnosis, disease monitoring, prognosis and risk stratification. Knowing about cytogenetic profile at the time of diagnosis is important in order to take critical decisions in management of the patients. Aim and Objectives: To determine the frequency of cytogenetic abnormalities in Pakistani adult patients with ALL in order to have insights regarding behavior of the disease. Materials and Methods: A retrospective analysis of all the cases of ALL (${\geq}15$years old) diagnosed at Aga Khan University from January 2006 to June 2014 was performed. Phenotype (B/T lineage) was confirmed in all cases by flow cytometry. Cytogenetic analysis was made for all cases using the trypsin-Giemsa banding technique. Karyotypes were interpreted using the International System for Human Cytogenetic Nomenclature (ISCN) criteria. Results: A total of 166 patients were diagnosed as ALL during the study period, of which 151 samples successfully yielded metaphase chromosomes. The male to female ratio was 3.4:1. The majority (n=120, 72.3%) had a B-cell phenotype. A normal karyotype was present in 51% (n=77) of the cases whereas 49% (n=74) had an abnormal karyotype. Of the abnormal cases, 10% showed Philadelphia chromosome; t(9;22)(q34;q11.2). Other poor prognostic cytogenetic subgroups were t(4;11)(q21;q23), hypodiploidy (35-45 chromosomes) and complex karyotype. Hyperdiploidy (47-57 chromosomes) occurred in 6.6%; all of whom were younger than 30 years. Conclusions: This study showed a relatively low prevalence of Philadelphia chromosome in Pakistani adults with ALL with an increase in frequency with age (p=0.003). The cumulative prevalence of Philadelphianegative poor cytogenetic aberrations in different age groups was not significant (p=0.6).

• Childhood Kidney Diseases
• /
• 제3권2호
• /
• pp.145-152
• /
• 1999

목적 : 최근 학동기 소아의 집단 신체검사에 소변검사가 포함된 후 소아 신장질환에 대한 관심이 높아지고 있으나 우리나라에서 소아에서의 신증후군을 포함한 신질환의 유병율은 잘 알려져 있지않으며 과거에 비해 변화가 있는지도 확실하지 않다. 저자들은 지난 13년동안 대전시와 인근 지역으로부터 입원한 모든 신증후군 환아의 연도별 발생수와 입원환아에 대한 비율 등을 알아보고 임상 및 신조직병리학적 소견에 따라 원인질환을 분석하므로써 단일 병원에서 조사된 결과가 지금까지 알려진 소아 신증후군의 연구결과와 얼마나 근접하는지 알아보고 신증후군의 유병률의 변화를 짐작하는데 도움을 받고자 본 연구를 시행하였다. 방법 : 1986년 5월부터 1998년 12월까지 13년동안 입원한 신증후군 환아 96명을 대상으로 입원 및 외래기록지를 후향적으로 분석하였다. 소아 신증후군의 발생빈도는 대전시내 3개의 종합병원의 도움을 얻었다. 결과 : 연도별 환아수는 1987년 8명, 1993년 8명, 1998년 6명으로 평균 환아수는 $7.4{\pm}2.1$명이었으며, 총입원환아에 대한 비율은 1987년 0.40%, 1993년 0.27%, 1998년 0.13%으로 평균 $0.30{\pm}0.11%$를 보였다. 15세 이하 소아 10만명당 발생빈도는 1988턴 5.6명, 1993년 5.5명 및 1998년 4.8명이었다. 초발시 연령은 평균 $6.8{\pm}3.5$세로 $1{\sim}4$세 26명(27.1%), $5{\sim}7$세 29명(30.2%), $8{\sim}10$세 25명(26.0%), 11세 이상 16명(16.7%)이었다. 일차성 신증후군의 평균연령는 $6.4{\pm}3.3$세, 이차성 신증후군의 평균연령은 $11.6{\pm}3.4$세이었다. 남아가 75명으로 남녀비는 3.6:1을 보였다. 임상병리학적 분류상 일차성 신증후군이 89명(92.7%), 이차성 신증후군이 7명(7.3%)이었으며 일차성 신증후군에서 minimal change nephrotic syndrome이 71명으로 79.8%, focal segmental glomerulosclerosis 11.2%, mesangial proliferation 4.5%, membranoprolifrative glomeulonephritis 3.4%, membranous nephropathy 1명 1.1%이었으며 2차성 신증후군은 $Henoch-Sch\"{o}nlein$ nephritis가 3례로 가장 많았다. 미세변화 신증후군 71명 중 비재발군이 16명으로 22.5%, 비빈발 재발군 49.3%, 빈발 재발군 18.3%, 스테로이드 의존군 9.9%를 보였다. 결론 : 대전시에서의 신증후군 환아의 발생빈도는 15세이하 소아 10만명당 약 5명으로 추정되었으며 10여 년전과 비교하여 큰 변화를 보이지는 않았다. 또한 저자들의 임상병리학적 연구결과가 다른 문헌에서 보고된 소아 신증후군의 연구결과와 큰 차이를 보이지 않음을 알 수 있었다.

• 대한유전성대사질환학회지
• /
• 제5권1호
• /
• pp.33-43
• /
• 2005

목적 : X 염색체 연관 부신백질이영양증은 과산화소체의 지방산 대사 장애로 인하여 포화 장쇄 지방산이 신경계의 수초와 부신 피질에 축적되어 여러 가지 신경학적 증상과 부신 기능 저하를 나타내는 드문 유전 질환이다. 증상의 발현 시기와 임상 경과에 따라 소아 대뇌형, 성인부신척수신경병형 등 여러 가지 형태로 나누어 지며 예후도 달라진다. 대상 및 방법 : 1996년 1월부터 2003년 6월까지 광주기독병원 소아과와 전남대병원 소아과에서 부신백질이영양증으로 진단받은 6명과 타병원에서 진단받고 골수 이식을 위해 내원한 7명, 총 13명의 남아들을 최소 1년 이상 추적 관찰하여 임상 경과를 분석하였다. 결과 : 1. 첫 신경학적 증상은 행동 장애, 청력 저하, 보행 장애, 시력 저하 및 사시 순이었고, 10명에서 ALD 가족력이 있었다. 2. 신경학적 증상의 발현시기, 증상의 진행 정도에 따라 분류하였을 때, 소아 대뇌형이 10명, 청소년 대뇌형이 2명, 신경학적 증상과 MRI 병변은 없이 부신 기능의 저하만 있는 "Addison-only"가 1명이었다. 1) 소아 대뇌형의 경우 8명이 사망하였는데 첫 증상 발현 시기는 평균 7.02세, 자연 경과로 첫 증상으로부터 식물 인간 상태에 이르는 기간은 1.35년, 사망에 이르는 기간은 3.35년으로 빠르게 진행하였다. Lorenzo oil을 투여하였던 2명에서 증상은 진행하였고, 제대혈 이식을 시행했던 2례는 질환의 진행과 이식편 대 숙주반응에 의해 사망하였다. 2) 청소년 대뇌형의 첫 증상 발현시기는 11.5세로, 2명 모두 현재 19세, 26세로 생존해 있으나, 1례는 식물 인간 상태이다. 3) "Addison only" 1례는 Lorenzo oil과 steroid를 투여하면서 3년째 증상 없이 지내고 있다. 3. 뇌 MRI에서 양측 두정엽과 후두엽의 백질을 침범한 형태가 대부분(11명)이었고, 청소년대뇌형 1명에서만 내포, 대뇌각과 뇌교를 침범하였다. 4. 진단 당시 부신 기능 저하가 67%에서 있었으며, ACTH만 상승된 형태였다. 결론 : 소아기에 발현한 부신백질이영양증의 경우, 두정엽과 후두엽 백질을 침범한 소아 대뇌형이 대부분이었고, 첫 증상 이후 빠르게 진행하여 사망에 이르는 치명적인 경과를 보였다. 따라서 가족력이 있는 경우, 많은 구성원에서 장쇄지방산 검사를 시행하여 조기에 무증상 환자를 진단하고 적극적으로 대처하는 것이 중요하리라 생각된다.

• Clinical and Experimental Pediatrics
• /
• 제56권3호
• /
• pp.130-134
• /
• 2013

Purpose: Anthracyclines have been utilized in the treatment of children with acute lymphoblastic leukemia (ALL). Recent studies have shown that anthracyclines may induce toxicity in the vascular endothelium. This study was performed using brachial artery reactivity (BAR) to evaluate vascular endothelial function in ALL patients who were treated with anthracycline chemotherapy. Methods: We included 21 children with ALL who received anthracycline chemotherapy and 20 healthy children. The cumulative dose of anthracyclines in the ALL patients was $142.5{\pm}18.2/m^2$. The last anthracycline dose was administered to the patients 2 to 85 months prior to their examination using BAR. The diameter of the brachial artery was measured in both groups using echocardiography, and BAR was calculated as the percentage change in the arterial diameter after release of the cuff relative to the baseline vessel diameter. Results: In the anthracycline-treated group, BAR was observed to be $3.4%{\pm}3.9%$, which was significantly lower than that observed in the control group ($12.1%{\pm}8.0%$, P<0.05). The time elapsed after the last anthracycline treatment and the age at the time of treatment did not affect the change in BAR (P =0.06 and P =0.13, respectively). Conclusion: These results provided evidence that treatment of ALL patients with anthracycline results in endothelial dysfunction. A larger cohort study and a longer follow-up period will be required to clarify the relationship between endothelial dysfunction resulting from anthracycline treatment for childhood ALL and occurrence of cardiovascular diseases later in life.

• Clinical and Experimental Pediatrics
• /
• 제55권3호
• /
• pp.100-106
• /
• 2012

Purpose: The survival rate for childhood acute lymphoblastic leukemia (ALL) has improved significantly. However, overall prognosis for the 20 to 25% of patients who relapse is poor, and allogeneic hematopoietic stem cell transplantation (HSCT) offers the best chance for cure. In this study, we identified significant prognostic variables by analyzing the outcomes of allogeneic HSCT in ALL patients in second complete remission (CR). Methods: Fifty-three ALL patients (42 men, 79%) who received HSCT in second CR from August 1991 to February 2009 were included (26 sibling donor HSCTs, 49%; 42 bone marrow transplantations, 79%). Study endpoints included cumulative incidence of acute and chronic graft-versus-host disease (GVHD), relapse, 1-year transplant-related mortality (TRM), disease-free survival (DFS), and overall survival (OS). Results: Cumulative incidences of acute GVHD (grade 2 or above) and chronic GVHD were 45.3% and 28.5%, respectively. The estimated 5-year DFS and OS for the cohort was $45.2{\pm}6.8%$ and $48.3{\pm}7%$, respectively. Only donor type, i.e., sibling versus unrelated, showed significant correlation with DFS in multivariate analysis ($p$=0.010). The rates of relapse and 1 year TRM were $28.9{\pm}6.4%$ and $26.4{\pm}6.1%$, respectively, and unrelated donor HSCT ($p$=0.002) and HLA mismatch ($p$=0.022) were significantly correlated with increased TRM in univariate analysis. Conclusion: In this single institution study spanning more than 17 years, sibling donor HSCT was the only factor predicting a favorable result in multivariate analysis, possibly due to increased TRM resulting from unrelated donor HSCT.

• 한국보육지원학회지
• /
• 제8권2호
• /
• pp.81-99
• /
• 2012

본 연구는 보육교사의 발달에 적합한 실제(DAP)에 대한 신념 및 교사효능감이 갈등해결태도에 미치는 영향을 살펴보고자 하였다. 이를 위하여 인천지역 어린이집에 근무하는 보육교사 128명을 대상으로 설문조사를 실시하였다. 수집된 자료는 상관분석, 다변량 분산분석, 로지스틱 회귀분석을 이용하여 분석되었다. 연구결과는 다음과 같다. 첫째, 사회인구학적 변인 가운데 연령에 따른 차이만 유의미하게 나타났는데, 20대 교사가 30대 이상 교사보다 DAP신념, 협력형 갈등해결 수준이 높고 회피형 갈등해결 수준은 낮은 것으로 나타났다. 둘째, 보육교사의 DAP신념과 교사효능감은 협력형 갈등해결과 정적 상관이 있는 반면에, 회피형 및 경쟁형 갈등해결 수준과는 부적 상관이 있는 것으로 나타났다. 마지막으로 보육교사의 DAP신념 및 교사효능감은 사회인구학적 변인을 통제한 이후에도 갈등해결태도에 영향을 미쳐, DAP신념과 교사효능감 수준이 높을수록 협력형 갈등해결을 선호하는 것으로 나타났다. 본 연구의 결과는 유아의 사회성 발달을 위한 적절한 교육이 이루어지기 위해 교사의 신념 및 교수전략에 대한 교사교육의 자료로 활용될 수 있을 것이다.

• Asian Pacific Journal of Cancer Prevention
• /
• 제13권9호
• /
• pp.4281-4284
• /
• 2012

Background: Osteosarcoma is the most common bone cancer in children, responsible for a high rate of amputation and death. This is the first long-term, population-based, epidemiologic and survival study in Thailand. Objective: To study the incidence and survival rates of pediatric osteosarcoma in Khon Kaen. Method: Childhood osteosarcoma cases (0-19 years) diagnosed between 1985-2010 were reviewed. The data were retrieved from the population-based data set of the Khon Kaen Cancer Registry and medical records from Srinagarind Hospital, Faculty of Medicine, Khon Kaen University. All cases were censored until the end of April 2012. The age-standardized incidence rate (ASR) was calculated using the standard method. Survival experience was analyzed using the standard survival function (STATA 9.0) and presented with a Kaplan-Meier curve. Results: 58 cases were enrolled. The overall ASR was 14.1 per million. Males and females were equally affected. The peak incidence was for 15-19 year-olds in both sexes (ASR=10.4 per million in males and 8.5 in females). The 5-year overall survival rate was 27.6% (95% CI: 15.8-40.8%). The median survival time was 1.6 years (95% CI: 1.2-2.1). In a subgroup analysis, the patients who received only chemotherapy survived longer (5-year survival 45.7%, median survival time 4.1 years, p=0.12). Conclusion: The incidence rate for childhood osteosarcoma was slightly less than those reported for Western countries. The survival rate was also lower than reports from developed countries. Further evaluation of the treatment protocol and risk factor stratification is needed.

• 한국보육지원학회지
• /
• 제9권6호
• /
• pp.223-241
• /
• 2013

이 연구는 영유아교사의 교사효능감과 스트레스 대처방식이 직무만족도에 미치는 영향을 살펴보기 위해 실시되었다. 영유아교사 189명을 대상으로 조사를 하여 얻은 주요 결과는 다음과 같다. 첫째, 직무만족도 가운데 직무특성에 대한 만족도가 가장 높았고, 훈육에 대한 교사 효능감이 가장 높았으며, 스트레스를 받을 경우 직접적 대처방식을 가장 많이 사용하였다. 둘째, 교사효능감의 하위요인 가운데 긍정적인 학습환경을 조성하는 능력은 영유아교사직의 직무특성, 원장 및 동료와의 관계에 정적인 영향을, 의사결정참여 능력은 원장 및 동료와의 관계, 근무환경, 복리 및 보수에 정적인 영향을 미쳤다. 셋째, 스트레스 대처방식의 하위요인 가운데 직접적인 대처 방식이 직무만족도의 모든 하위요인에 영향을 미치는 것으로 나타났으며 정신적, 심리적, 파괴적인 대처 방식은 각각 직무특성, 원장 및 동료와의 관계, 복리 및 보수에 영향을 미쳤다. 이와 같은 결과는 영유아교사의 직무만족도를 향상시키는 방법을 모색하는데 기초자료로 사용될 것이다.

• 가정과삶의질연구
• /
• 제19권4호
• /
• pp.185-199
• /
• 2001

The purpose of this study was to examine the dynamics of psychological and sexual violence in dating among high school students. Subjects consisted of 1,205 male and female adolescents from all parts of the country. 1,092 respondents were included in the final analysis(90.6%). For psychological violence, 13.3%(n=145) reported to have such experiences with a dating partner at least once or more. The most frequently used forms of violence were shouting, insulting language and behaviors, and blaming for a physical appearance. There were no sex differences in frequency of psychological violence between males and females, as an aggressor or a victim. Responses of victims were mostly negotiation and communication, and trying to forget it. For sexual violence, 27.8%(n=303) was found to have such experiences in a dating scence at least once or more. Hugging and kissing, close contact of parts of body, and gazing at parts of body were most frequently used forms. Responses of victims were pretending not to notice it and responding in the same way. There were significantly more male aggressors of sexual violence than females, but no sex difference in the number of victims. Those who reported to have experiences of violence were compared to the counterparts who had no experience in attitudes toward violence between dating partners and married couples, experiences of violence in childhood, school violence, and observations of violence between parents. Those with experience of dating violence reported to have significantly more experiences of violence in childhood and school, and parental violence. Based on the findings of this study, suggestion were made in regard to the need for development of dating violence prevention program for adolescents, and for welfare programs for adolescents in community.

• 아동학회지
• /
• 제33권6호
• /
• pp.187-205
• /
• 2012

In this study, 150 previous studies of young children's challenging behavior, conducted over the past 10 years, from 2003 to 2012, were selected. These studies were in relation to young children both with and without disabilities, and they were thus used for the purposes of comparative analysis. The major findings are as follows. First, research on challenging behavior not only of young children with disabilities but also of those without disabilities has been an increasing trend from 2008. While most studies of those without disabilities were based on observations by parents or teachers using a variety of tools, studies regarding those with disabilities mainly used single-subject research methods using an operational definition. Second, the bulk of the studies of young children without disabilities were focused on interaction between various children's internal variables and a variety of parent-related variables related to the occurrence of challenging behavior. In comparison, studies of young children with disabilities were focused excessively on direct intervention after the occurrence of problems. Regarding those without disabilities, the interaction between children's temperament, internal variables, and their mothers' parenting behavior, external variables were the main areas of interest for these researchers. There is clearly a growing need for more active research aimed at the prevention of challenging behavior. Challenging behavior in early childhood has an influence not only on each child's present and future academic achievement levels and their quality of life but also on the quality of instruction in classroom available to all children.