• Pediatric Infection and Vaccine
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• 제30권3호
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• pp.129-138
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• 2023

목적: 선천면역장애 환자들은 감염에 취약할 뿐만 아니라, 면역이 정상인 사람들에 비해 암 발생률도 높은 것으로 알려져 있다. 본 연구는 단일 기관에서 추적 중인 선천면역장애 환자들에서의 암 발생을 조사하여 보고자 하였다. 방법: 1994년 11월부터 2023년 9월까지 삼성서울병원에서 선천면역장애 진단 하에 추적하는 환자를 대상으로 후향적으로 의무기록을 리뷰하였다. 선천면역장애 환자 중에서 암으로 진단된 환자를 확인하였다. 결과: 총 194명의 선천면역장애 환자 중, 7명(3.6%)의 환자에서 암이 진단되었다. 5명의 환자가 림프종으로 진단받았으며 그 중 4명의 환자는 Epstein-Barr 바이러스 연관 림프종이었다. 나머지 암은 위암과 다발 골수종이었다. 암 진단 당시 나이는 중앙값 18세 (범위, 1세–75세)였다. 암이 발생한 환자들의 면역결핍 질환은 X-linked lymphoproliferative disorder-1 (XLP-1) 3명, activated phosphoinositide 3-kinase delta disease (APDS) 2명, cytotoxic T-lymphocyte antigen 4 (CTLA-4) haplo-insufficiency 2명이었다. 개별 질환별로 분석하였을 때, XLP-1 환자의 75.0%, APDS 환자의 40.0%, CTLA-4 환자의 50.0%에서 암이 발생하였다. XLP-1 환자는 APDS 및 CTLA-4 haplo-insufficiency 환자에 비해 더 이른 나이에 암이 발생하였다 (중앙연령 5세, P<0.001). 한 명은 조혈모세포 이식 치료 중 사망하였다. 결론: 국내 단일 기관에서 진료받는 선천면역장애 환자들의 3.6%에서 암이 발생하였다. 선천면역장애 환자들을 진료하는 의료진들은 이들 환자에서 감염이나 염증 등의 문제외에도 암 발생의 가능성, 특히 Epstein-Barr 바이러스 감염과 연관된 암의 비중이 높은 것에 대한 인식을 갖는 것이 중요하다.

• Journal of Preventive Medicine and Public Health
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• 제20권1호
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• pp.165-203
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• 1987

This project was a service-cum-research effort with a quasi-experimental study design to examine the health benefits of an integrated Family Planning (FP)/Maternal & Child health (MCH) Service approach that provides crucial factors missing in the present on-going programs. The specific objectives were: 1) To test the effectiveness of trained nurse/midwives (MW) assigned as change agents in the Health Sub-Center (HSC) to bring about the changes in the eight FP/MCH indicators, namely; (i)FP/MCH contacts between field workers and their clients (ii) the use of effective FP methods, (iii) the inter-birth interval and/or open interval, (iv) prenatal care by medically qualified personnel, (v) medically supervised deliveries, (vi) the rate of induced abortion, (vii) maternal and infant morbidity, and (viii) preinatal & infant mortality. 2) To measure the integrative linkage (contacts) between MW & HSC workers and between HSC and clients. 3) To examine the organizational or administrative factors influencing integrative linkage between health workers. Study design; The above objectives called for quasi-experimental design setting up a study and control area with and without a midwife. An active intervention program (FP/MCH minimum 'package' program) was conducted for a 2 year period from June 1982-July 1984 in Seosan County and 'before and after' surveys were conducted to measure the change. Service input; This study was undertaken by the Soonchunhyang University in collaboration with WHO. After a baseline survery in 1981, trained nurses/midwives were introduced into two health sub-centers in a rural setting (Seosan county) for a 2 year period from 1982 to 1984. A major service input was the establishment of midwifery services in the existing health delivery system with emphasis on nurse/midwife's role as the link between health workers (nurse aids) and village health workers, and the referral of risk patients to the private physician (OBGY specialist). An evaluation survey was made in August 1984 to assess the effectiveness of this alternative integrated approach in the study areas in comparison with the control area which had normal government services. Method of evaluation; a. In this study, the primary objective was first to examine to what extent the FP/MCH package program brought about changes in the pre-determined eight indicators (outcome and impact measures) and the following relationship was first analyzed; b. Nevertheless, this project did not automatically accept the assumption that if two or more activities were integrated, the results would automatically be better than a non-integrated or categorical program. There is a need to assess the 'integration process' itself within the package program. The process of integration was measured in terms of interactive linkages, or the quantity & quality of contacts between workers & clients and among workers. Intergrative linkages were hypothesized to be influenced by organizational factors at the HSC clinic level including HSC goals, sltrurture, authority, leadership style, resources, and personal characteristics of HSC staff. The extent or degree of integration, as measured by the intensity of integrative linkages, was in turn presumed to influence programme performance. Thus as indicated diagrammatically below, organizational factors constituted the independent variables, integration as the intervening variable and programme performance with respect to family planning and health services as the dependent variable: Concerning organizational factors, however, due to the limited number of HSCs (2 in the study area and 3 in the control area), they were studied by participatory observation of an anthropologist who was independent of the project. In this observation, we examined whether the assumed integration process actually occurred or not. If not, what were the constraints in producing an effective integration process. Summary of Findings; A) Program effects and impact 1. Effects on FP use: During this 2 year action period, FP acceptance increased from 58% in 1981 to 78% in 1984 in both the study and control areas. This increase in both areas was mainly due to the new family planning campaign driven by the Government for the same study period. Therefore, there was no increment of FP acceptance rate due to additional input of MW to the on-going FP program. But in the study area, quality aspects of FP were somewhat improved, having a better continuation rate of IUDs & pills and more use of effective Contraceptive methods in comparison with the control area. 2. Effects of use of MCH services: Between the study and control areas, however, there was a significant difference in maternal and child health care. For example, the coverage of prenatal care was increased from 53% for 1981 birth cohort to 75% for 1984 birth cohort in the study area. In the control area, the same increased from 41% (1981) to 65% (1984). It is noteworthy that almost two thirds of the recent birth cohort received prenatal care even in the control area, indicating that there is a growing demand of MCH care as the size of family norm becomes smaller 3. There has been a substantive increase in delivery care by medical professions in the study area, with an annual increase rate of 10% due to midwives input in the study areas. The project had about two times greater effect on postnatal care (68% vs. 33%) at delivery care(45.2% vs. 26.1%). 4. The study area had better reproductive efficiency (wanted pregancies with FP practice & healthy live births survived by one year old) than the control area, especially among women under 30 (14.1% vs. 9.6%). The proportion of women who preferred the 1st trimester for their first prenatal care rose significantly in the study area as compared to the control area (24% vs 13%). B) Effects on Interactive Linkage 1. This project made a contribution in making several useful steps in the direction of service integration, namely; i) The health workers have become familiar with procedures on how to work together with each other (especially with a midwife) in carrying out their work in FP/MCH and, ii) The health workers have gotten a feeling of the usefulness of family health records (statistical integration) in identifying targets in their own work and their usefulness in caring for family health. 2. On the other hand, because of a lack of required organizational factors, complete linkage was not obtained as the project intended. i) In regards to the government health worker's activities in terms of home visiting there was not much difference between the study & control areas though the MW did more home visiting than Government health workers. ii) In assessing the service performance of MW & health workers, the midwives balanced their workload between 40% FP, 40% MCH & 20% other activities (mainly immunization). However, $85{\sim}90%$ of the services provided by the health workers were other than FP/MCH, mainly for immunizations such as the encephalitis campaign. In the control area, a similar pattern was observed. Over 75% of their service was other than FP/MCH. Therefore, the pattern shows the health workers are a long way from becoming multipurpose workers even though the government is pushing in this direction. 3. Villagers were much more likely to visit the health sub-center clinic in the study area than in the control area (58% vs.31%) and for more combined care (45% vs.23%). C) Organization factors (admistrative integrative issues) 1. When MW (new workers with higher qualification) were introduced to HSC, it was noted that there were conflicts between the existing HSC workers (Nurse aids with less qualification than MW) and the MW for the beginning period of the project. The cause of the conflict was studied by an anthropologist and it was pointed out that these functional integration problems stemmed from the structural inadequacies of the health subcenter organization as indicated below; i) There is still no general consensus about the objectives and goals of the project between the project staff and the existing health workers. ii) There is no formal linkage between the responsibility of each member's job in the health sub-center. iii) There is still little chance for midwives to play a catalytic role or to establish communicative networks between workers in order to link various knowledge and skills to provide better FP/MCH services in the health sub-center. 2. Based on the above findings the project recommended to the County Chief (who has power to control the administrative staff and the technical staff in his county) the following ; i) In order to solve the conflicts between the individual roles and functions in performing health care activities, there must be goals agreed upon by both. ii) The health sub·center must function as an autonomous organization to undertake the integration health project. In order to do that, it is necessary to support administrative considerations, and to establish a communication system for supervision and to control of the health sub-centers. iii) The administrative organization, tentatively, must be organized to bind the health worker's midwive's and director's jobs by an organic relationship in order to achieve the integrative system under the leadership of health sub-center director. After submitting this observation report, there has been better understanding from frequent meetings & communication between HW/MW in FP/MCH work as the program developed. Lessons learned from the Seosan Project (on issues of FP/MCH integration in Korea); 1) A majority or about 80% of the couples are now practicing FP. As indicated by the study, there is a growing demand from clients for the health system to provide more MCH services than FP in order to maintain the achieved small size of family through FP practice. It is fortunate to see that the government is now formulating a MCH policy for the year 2,000 and revising MCH laws and regulations to emphasize more MCH care for achieving a small size family through family planning practice. 2) Goal consensus in FP/MCH shouBd be made among the health workers It administrators, especially to emphasize the need of care of 'wanted' child. But there is a long way to go to realize the 'real' integration of FP into MCH in Korea, unless there is a structural integration FP/MCH because a categorical FP is still first priority to reduce the rate of population growth for economic reasons but not yet for health/welfare reasons in practice. 3) There should be more financial allocation: (i) a midwife should be made available to help to promote the MCH program and coordinate services, (in) there should be a health sub·center director who can provide leadership training for managing the integrated program. There is a need for 'organizational support', if the decision of integration is made to obtain benefit from both FP & MCH. In other words, costs should be paid equally to both FP/MCH. The integration slogan itself, without the commitment of paying such costs, is powerless to advocate it. 4) Need of management training for middle level health personnel is more acute as the Government has already constructed 90 MCH centers attached to the County Health Center but without adequate manpower, facilities, and guidelines for integrating the work of both FP and MCH. 5) The local government still considers these MCH centers only as delivery centers to take care only of those visiting maternity cases. The MCH center should be a center for the managment of all pregnancies occurring in the community and the promotion of FP with a systematic and effective linkage of resources available in the county such as i.e. Village Health Worker, Community Health Practitioner, Health Sub-center Physicians & Health workers, Doctors and Midwives in MCH center, OBGY Specialists in clinics & hospitals as practiced by the Seosan project at primary health care level.

• Tuberculosis and Respiratory Diseases
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• 제46권5호
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• pp.674-684
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• 1999

연구배경 : 특발생 폐섬유화증에서 폐암의 발생이 높다고 알려져 왔으나 실제로는 보고자마다 차이가 많고 가장 많은 조직형에 대해서도 논란이 많다. 또한 동반된 폐섬유증에 의해 폐암의 발견시기나 치료방침, 예후 등이 다를 것이 예상되나 아직 이에 대한 보고는 없는 실정이다. 본 연구는 특발성 폐섬유화증이 동반된 폐암 환자에서 위험인자, 폐암의 발생부위 및 조직학적 분포, 특히 폐암과 폐섬유화 부위의 일치도 등을 분석하여 특발성 폐섬유화증이 폐암 발생에 미친 영향을 알아보고, 특발성 폐섬유화증이 폐암의 치료에 미친 영향을 알아보고자 시행되었다. 방 법 : 서울중앙병원을 방문하였던 특발성 폐섬유화증 환자중 폐암이 동반되었던 63명($66.8{\pm}:7.8$세, 남 : 녀= 61 : 2)을 대상으로 폐암의 위치, 조직형, 폐암 진단시 폐기능 검사소견, 폐암의 치료형태, 폐암 진단 후 생존기간 등을 후향적으로 조사하였다. 또한 대조군으로 본원을 내원한 2,660명의 대조폐암환자와 조직형의 차이를 분석하였다. 결 과 : 특발성 폐섬유화증환자들 중 22.4%(63/281명)에서 폐암이 동반되었다(IPF-CA). 45명(71.4%)의 환자에서는 폐암이 동시에 발견되었고 18명(28.6%)에서는 특발성 폐섬유화증 진단 후 25.2(3.7-42.9)개월이 지나 폐암이 발견되어, 폐섬유증의 경과 도중에 폐암의 발생율은 6.7%이었다. 폐 말초부에 위치한 폐암은 35명(55.5%) 뿐 이었고, 28명(44.4%)에서는 중심부 폐암이었으며, 폐하엽 보다는 상엽에 33명(51.6%)으로 더 많았다. 전체 특발성 폐섬유화증군에 비하여 특발성 폐섬유화증에 동반된 폐암군에서 연령 ($66.8{\pm}:7.84$ vs. $63.4{\pm}:11.1$세, p=0.007), 남성 비율 (96.8 vs. 67.6%, p<0.001), 흡연력 (88.9 vs. 67.2%, p<0.001)이 의미있게 높았다. 폐섬유화 병변과 일치한 부위에 폐암이 발생한 경우는 23명(35.9%) 뿐 이었고, 40명(62.5%)은 폐섬유화 병변과 다른 부위에서 폐암이 발생하였다. 특발성 폐섬유화증에 동반된 폐암의 조직형은 편평상피암 22명(34.9%), 선암 19명(30.2%), 소세포암 12명(19.0%), 대세포암 4명(6.3%), 기타가 6명(9.5%)이었다. 2,660명의 대조폐암환자군의 조직형은 편평상피암 44.6%, 선암 34.2%, 소세포암 14.7%, 대세포암 1.5%, 기타 5.0%으로 조직형의 분포에 있어 두 군간에 유의한 차이는 없었다. 특발성 폐섬유화증의 경과 도중에 폐암이 발생한 군과 특발성 폐섬유화증과 폐암이 동시에 발견된 군간에 인적사항이나, 폐암의 위치, 조직형 및 병기 등에 유의한 차이를 관찰 할 수 없었으나 중증 섬유화증(방사선학적 소견상 폐침범 정도가 50% 이상)은 특발성 폐섬유화증 경과 도중에 폐암이 발견된 군에서 특발성 폐섬유화증과 폐암 동시 발견군에 비하여 심한 양상을 나타내었다(33.3 % vs. 61.1%, p=0.043). 폐섬유화와 일치한 부위의 폐암군은 방사선학적으로 폐섬유화가 더 광범위하게 진행된 경향을 보였고 중앙값생존기간이 폐섬유화와 다른 부위의 폐암군에 비하여 낮았다(4개월 vs. 7개월, p=0.04). 비소세포암군에서 병기 IIIa 이하인 19명중 33.3%(폐엽절제술 6명, 전폐절제술 1명)에서만 수술이 시행되었다. 10명은 수술 후 잔여 폐기량과 활동능력을 고려하여 수술을 시행하지 못하였고 2명은 수술을 거부하였다. 결 론 : 특발성 폐섬유화증에서 폐암 발생은 폐섬유화에 의한 영향보다는 연령, 흡연 및 성별(남성) 등의 위험인자와 더 연관되는 것으로 사료된다.