• Title/Summary/Keyword: Diagnosis Function

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Clinical Results of Medial Unicompartmental Knee Arthroplasty in Elderly Patients Older than 70 Years of Age (70세 이상의 고령 환자에서 시행한 내측 슬관절 단일구획치환술의 임상 결과)

  • Kim, Kyung Tae;Lee, Song;Kim, Jin Hak;Lee, Ho Young;Kim, Myung Jin
    • Journal of the Korean Orthopaedic Association
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    • v.56 no.1
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    • pp.34-41
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    • 2021
  • Purpose: To evaluate the long-term clinical results and survivorship of unicompartmental knee arthroplasty (UKA) in elderly patients older than 70 years by analyzing cases that have been implanted for >10 years ago. Materials and Methods: The long-term follow-up results were evaluated in 39 patients (46 cases) who underwent medial UKA from March 2002 to February 2004. The mean age of the patients at the time of surgery was 74.0 years, and the preoperative diagnosis was degenerative arthritis of the knee in all cases. Results: Of the 46 cases, reoperation occurred due to the complications in four cases. In 22 cases without 14 cases of death and six cases of follow-up loss, follow-up more than 10 years was possible. The mean Knee Society knee and function scores improved significantly from 53.0 and 52.5 points pre-operatively to 89.4 and 80.9 points at the last follow-up, respectively (p<0.001). The mean range of motion of the knee recovered to 132.5°, and the mean tibiofemoral angle changed to 5.9° of valgus at the last follow-up. Complications following the UKA occurred in four cases; the most prevalent complication was mobile bearing dislocation (n=2). One case of failure occurred due to aseptic loosening and degenerative arthritis of the lateral compartment, respectively. The cumulative survival rate of the implants was 95.0% at 10 years and 85.7% at 15 years. Of the 40 cases, excluding six cases of follow-up loss, 36 cases (90.0%) could be used without reoperation until death or at the last follow-up after surgery. Conclusion: These results showed the outstanding functions of the knee and satisfactory long-term survivorship after UKA. Therefore, UKA could be a useful method for the treatment of osteoarthritis of the knee in elderly patients older than 70 years of age.

A Study of Life Safety Index Model based on AHP and Utilization of Service (AHP 기반의 생활안전지수 모델 및 서비스 활용방안 연구)

  • Oh, Hye-Su;Lee, Dong-Hoon;Jeong, Jong-Woon;Jang, Jae-Min;Yang, Sang-Woon
    • Journal of the Society of Disaster Information
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    • v.17 no.4
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    • pp.864-881
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    • 2021
  • Purpose: This study aims is to provide a total care solution preventing disaster based on Big Data and AI technology and to service safety considered by individual situations and various risk characteristics. The purpose is to suggest a method that customized comprehensive index services to prevent and respond to safety accidents for calculating the living safety index that quantitatively represent individual safety levels in relation to daily life safety. Method: In this study, we use method of mixing AHP(Analysis Hierarchy Process) and Likert Scale that extracted from consensus formation model of the expert group. We organize evaluation items that can evaluate life safety prevention services into risk indicators, vulnerability indicators, and prevention indicators. And We made up AHP hierarchical structure according to the AHP decision methodology and proposed a method to calculate relative weights between evaluation criteria through pairwise comparison of each level item. In addition, in consideration of the expansion of life safety prevention services in the future, the Likert scale is used instead of the AHP pair comparison and the weights between individual services are calculated. Result: We obtain result that is weights for life safety prevention services and reflected them in the individual risk index calculated through the artificial intelligence prediction model of life safety prevention services, so the comprehensive index was calculated. Conclusion: In order to apply the implemented model, a test environment consisting of a life safety prevention service app and platform was built, and the efficacy of the function was evaluated based on the user scenario. Through this, the life safety index presented in this study was confirmed to support the golden time for diagnosis, response and prevention of safety risks by comprehensively indication the user's current safety level.

A Study on the Changes in Functions of Ship Officer and Manpower Training by the Introduction of Maritime Autonomous Surface Ships (자율운항선박 도입에 따른 해기사 직능 변화와 인력양성에 관한연구)

  • Lim, Sung-Ju;Shin, Yong-John
    • Journal of Navigation and Port Research
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    • v.46 no.1
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    • pp.1-10
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    • 2022
  • This study aims to investigate changes in the demand for ship officers in response to changes in the shipping industry environment in which Maritime Autonomous Surface Ships (MASS) emerge according to the application of the fourth industrial revolution technology to ships, and it looks into changes in the skill of ship officer. It also analyzes and proposes a plan for nurturing ship officers accordingly. As a result of the degree of recognition and AHP analysis, this study suggests that a new training system is required because the current training and education system may cover the job competencies of emergency response, caution and danger navigation, general sailing, cargo handling, seaworthiness maintenance, emergency response, and ship maintenance and management, but tasks such as remote control, monitoring diagnosis, device management capability, and big data analysis require competency for unmanned and shore-based control. By evaluating the importance of change factors in the duties of ship officers in Maritime Autonomous Surface Ships, this study provides information on ship officer educational institutions' response strategies for nurturing ship officers and prioritization of resource allocation, etc. The importance of these factors was compared and evaluated to suggest changes in the duties of ship officers and methods of nurturing ship officers according to the introduction of Maritime Autonomous Surface Ships. It is expected that the findings of this study will be meaningful as it systematically derives the duties and competency factors of ship officers of Maritime Autonomous Surface Ships from a practical point of view and analyzed the perception level of each relevant expert to diagnose expert-level responses to the introduction of Maritime Autonomous Surface Ships.

The Location of Medical Facilities and Its Inhabitants' Efficient Utilization in Kwangju City (광주시(光州市) 의료시설(醫療施設)의 입지(立地)와 주민(住民)의 효율적(效率的) 이용(利用))

  • Jeon, Kyung-Sook
    • Journal of the Korean association of regional geographers
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    • v.3 no.2
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    • pp.163-193
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    • 1997
  • Medical services are a fundamental and essential service in all urban areas. The location and accessibility of medical service facilities and institutions are critical to the diagnosis, control and prevention of illness and disease. The purpose of this paper is to present the results of a study on the location of medical facilities in Kwangju and the utilization of these facilities by the inhabitants. The following information is a summary of the findings: (1) Korea, like many countries, is now witnessing an increase in the age of its population as a result of higher living standards and better medical services. Korea is also experiencing a rapid increase in health care costs. To ensure easy access to medical consultation, diagnosis and treatment by individuals, the hierarchical efficient location of medical facilities, low medical costs, equalized medical services, preventive medical care is important. (2) In Korea, the quality of medical services has improved significantly as evident by the increased number of medical facilities and medical personnel. However, there is still a need for not only quantitative improvements but also for a more equitable distribution of and location of medical services. (3) There are 503 medical facilities in Kwangju each with a need to service 2,556 people. This is below the national average of 1,498 inhabitants per facility. The higher locational quotient and satisfactory population per medical facility showed at the civic center. On the other hand, problem regions such as the traditional residential area in Buk-Gu, Moo-deung mountain area and the outer areas of west Kwangju still maintain rural characteristics. (4) In the study area there are 86 general medicine clinics which provide basic medical services. i. e. one clinic per every 14,949 residents. As a basic service, its higher locational quotient showed in the residential area. The lower population concentration per clinic was found in the civic center and in the former town center, Songjeong-dong. In recently build residential areas and in the civic center, the lack of general medicine clinics is not a serious medical services issue because of the surplus of medical specialists in Korea. People are inclined to seek a consultation with a specialist in specific fields rather than consult a general practitioner. As a result of this phenomenon, there are 81 internal medicine facilities. Of these, 32.1% provide services to people who are not referred by a primary care physician but who self-diagnose then choose a medical facility specializing in what they believe to be their health problem. Areas in the city, called dongs, without any internal facilities make up 50% of the total 101 dongs. (5) There are 78 surgical facilities within the area, and there is little difference at the locational appearance from internal medicine facilities. There are also 71 pediatric health clinics for people under 15 years of age in this area, represents one clinic per 5,063 people. On the quantitative aspect, this is a positive situation. Accessibility is the most important facility choice factor, so it should be evenly located in proportion to demander distribution. However, 61% of 102 dongs have no pediatric clinics because of the uneven location. (6) There are 43 obstetrical and gynecological clinics in Kwangju, and the number of residents being served per clinic is 15,063. These services need to be given regularly so it should increase the numbers. There are 37 ENT clinics in the study area with the lower concentration in Dong-gu (32.4%) making no locational differences by dong. There are 23 dermatology clinics with the largest concentration in Dong-Gu. There are 17 ophthalmic clinics concentrated in the residential area because of the primary function of this type of specialization. (7) The use of general medicine clinics, internal medicine clinics, pediatric clinics, ENT clinics by the inhabitants indicate a trend toward primary or routine medical services. Obstetrics and gynecology clinics are used on a regular basis. In choosing a general medicine clinic, internal medicine clinic, pediatric clinic, and a ENT clinic, accessibility is the key factor while choice of a general hospital, surgery clinic, or an obstetrics and gynecology clinic, thes faith and trust in the medical practitioner is the priority consideration. (8) I considered the efficient use of medical facilities in the aspect of locational and management and suggest the following: First, primary care facilities should be evenly distributed in every area. In Kwangju, the number of medical facilities is the lowest among the six largest cities in Korea. Moreover, they are concentrated in Dong-gu and in newly developed areas. The desired number of medical facilities should be within 30 minutes of each person's home. For regional development there is a need to develop a plan to balance, for example, taxes and funds supporting personnel, equipment and facilities. Secondly, medical services should be co-ordinated to ensure consistent, appropriate, quality services. Primary medical facilities should take charge of out-patient activities, and every effort should be made to standardize and equalize equipment and facility resources and to ensure ongoing development and training in the primary services field. A few specialty medical facilities and general hospitals should establish a priority service for incurable and terminally ill patients. (9) The management scheme for the inhabitants' efficient use of medical service is as follows: The first task is to efficiently manage medical facilities and related services. Higher quality of medical services can be accomplished within the rapidly changing medical environment. A network of social, administrative and medical organizations within an area should be established to promote information gathering and sharing strategies to better assist the community. Statistics and trends on the rate or occurrence of diseases, births, deaths, medical and environment conditions of the poor or estranged people should be maintained and monitored. The second task is to increase resources in the area of disease prevention and health promotion. Currently the focus is on the treatment and care of individuals with illness or disease. A strong emphasis should also be placed on promoting prevention of illness and injury within the community through not only public health offices but also via medical service facilities. Home medical care should be established and medical testing centers should be located as an ordinary service level. Also, reduced medical costs for the physically handicapped, cardiac patients, and mentally ill or handicapped patients should be considered.

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Diffuse Panbronchiolitis : Clinical Significance of High-resolution CT and Radioaerosol Scan Manifestations (미만성 범세기관지염에서 흉부 고해상도 전산화 단층촬영의 임상적의의 및 폐환기주사 소견)

  • Song, So Hyang;Kim, Hui Jung;Kim, Young Kyoon;Moon, Hwa Sik;Song, Jeong Sup;Park, Sung Hak;Kim, Hak Hee;Chung, Soo Kyo
    • Tuberculosis and Respiratory Diseases
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    • v.44 no.1
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    • pp.124-135
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    • 1997
  • Background : Diffuse panbronchiolitis(DPB) is a disease characterized clinically by chronic cough, expectoration and dyspnea; and histologically by chronic inflammation localized mainly in the region of the respiratory bronchiole. It is prevalent in Japanese, but is known to be rare in Americans and Europians. Only a few cases in Chinese, Italians, North Americans and Koreans have been reported. It is diagnosed by characteristic clinical, radiological and pathologic features. High-resolution CT(HRCT) is known to be valuable in the study of the disease process and response to therapy in DPB. To our knowledge, there has been no correlation of its appearance on HRCT with the severity of the disease process, and radioaerosol scan(RAS) of the lung has not previously been used for the diagnosis of DPB. Method : During recent two years we have found 12 cases of DPB in Kangnam St. Mary's Hospital, Catholic University Medical College. We analysed the clinical characteristics, compared HRCT classifications with clinical stages of DPB, and determined characteristic RAS manifestations of DPB. Results : 1. The ages ranged from 31 to 83 years old(mean 54.5 years old), and male female ratio was 4:8. 75%(9/12) of patients had paranasal sinusitis, and only one patient was a smoker. 2. The patients were assigned to one of three clinical stages of DPB on the basis of clinical findings, sputum bacterology and arterial blood gas analysis. of 12 cases, 5 were in the first stage, 4 were in the second stage, and 3 were in the third stage. In most of the patients, pulmonary function tests showed marked obstructive and slight restrictive impairments. Sputum culture yielded P.aeruginosa in 3 cases of our 12 cases, K.pneumoniae in 2 cases, H.influenzae in 2 cases, and S.aureus in 2 cases. 3. Of 12 patients, none had stage I characteristics as classified on HRCT scans, 4 had slage II findings, 5 had stage III findings, and 3 had stage IV characteristics. 4. We peformed RAS in 7 of 12 patients With DPB. In 71.4% (5/7) of the patients, RAS showed mottled aerosol deposits characteristically in the transitional and intermediary airways with peripheral airspace defects, which contrasted sharply with central aerosol deposition of COPD. 5. There were significant correlations between HRCT stages and clinical stages(r= 0.614, P < 0.05), between HRCT types and Pa02(r= -0.614, P < 0.05), and between HRCT types and ESR(r= 0.618, P < 0.01). Conclusion : The HRCT classifications correspond well to the clinical stage. Therfore in the examination of patients with DPB, HRCT is useful in the evaluation of both the location and severity of the lesions. Also, RAS apears to be a convenient, noninvasive and useful diagnostic method of DPB.

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A Study on the Expressed Desire at Discharge of Patients to Use Home Nursing and Affecting Factors of the Desire (퇴원환자의 가정간호 이용의사와 관련 요인)

  • Lee, Ji-Hyun;Lee, Young-Eun;Lee, Myung-Hwa;Sohn, Sue-Kyung
    • The Korean Journal of Rehabilitation Nursing
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    • v.2 no.2
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    • pp.257-270
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    • 1999
  • The purpose of this study is to investigate factors related to the intent of using home nursing of chronic disease patients who got out of a university hospital. For the purpose, the study selected 153 patients who were hospitalized and left K university hospital with diagnoses of cancer, hypertension, diabetes and cerebral vascular accident and ordered to be discharged and performed interviews with them and surveys on their medical records to obtain the following results. For this study a direct-interview survey and medical record review was conducted from June 28 to Aug. 30, 1998. The frequency and mean values were computed to find the characteristics of the study subjects, and $X^2$-test, t-test, factor analysis and multiple logistic regession analysis were applied for the analysis of the data. The following results were obtained. 1) When characteristics of the subjects were examined, men and women occupied for 58.8% and 41.2%, respectively. The subjects were 41.3 years old in aver age and had the monthly aver age earning of 0.99 million won or below, which was the most out of the total subjects at 34.6%. Among the total, 87.6% resided in cities and 12.4 in counties. The most left the hospital with diagnosis of cancer at 51.6%, followed by hyper tension at 24.2%, diabetes at 13.7% and cerebral vascular accident at 7.2%. 2) 93.5% of the selected patients had the intent of using home nursing and 6.5%, didn't. Among those patients having the intent, 85.6% had the intent of paying for home nursing and 14.4%, didn't. The subjects expected that the nursing would be paid 9,143 won in aver age and 47.7% of them preferred national authorities as the main servers. 86.3% of the subjects thought that home nursing business had the main advantage of making it possible to learn nursing methods at home and thereby contributing to improving the ability of patients and their facilities to solve health problems. 3) Relations between the intent of use and characteristics of the subjects such as demography-related social, home environment, disease and physical function characteristics did not show statistically significant differences among one another. Compared to those who had no intent of using home nursing, the group having the intent had more cases of male patients, the age of 39 or below, residence in cities, 5 family member s or more, no existence of home nursing servers, leaving the hospital from a non-hospitalized building, disease development for five months or below, hospitalization for ten days or more, non-hospitalization with in the recent one month, two times or over of hospitalization, leaving the hospital with no demand of special treatment, operation underwent, poor results of treatment, leaving the hospital with demand of rehabilitation services, physical disablement and high evaluation point of daily life. 4) Among those patients having the intent of using home nursing, 47.6% demanded technical nursing and 55.9%, supportive nursing. As technical nursing,' inject into a blood vessel ' and 'treat pustule and teach basic prevention methods occupied for 57.4%, respectively, topping the list. Among demands of supportive nursing, 'observe patients 'status and refer them to hospitals or community resources as available, if necessary' was the most with percent age point of 59.5. Regarding the intent of paying for home nursing, 39.2% of those patients wishing to use the nursing responded paying for technical services and 20.2, supportive services. In detail, 70.0% wanted to pay for a service stated as 'inject into a blood vessel', highest among the former services and 30.7%, a service referred to as 'teaching exercises needed to make the body of patients move', highest among the latter. When this was analyzed in terms of a relation between the need(the need for home nursing) and the demand(the intent of paying for home nursing), The rate of the need to the demand was found two or three times higher in technical nursing(0.82) than in supportive nursing(0.35). In aspects of tech ical nursing, muscle injection(1.26, the 1st rank) was highest in the rate while among aspects of supportive nursing, a service referred to as 'teach exercises needed for making patients move their bodies normally'(0.58, the 1st rank). 5) factors I(satisfaction with hospital services), II(recognition of disease state), III(economy) and IV(period of disease) occupied for 34.4, 13.8, 11.9 and 9.2 percents, respectively among factors related to the intent by the subjects of using home nursing, totaled 59.3%. In conclusion, most of chronic disease patients have the intent of using hospital-based home nursing and satisfaction with hospital services is a factor affecting the intent most. Thus a post-management system is needed to continue providing health management to those patients after they leave the hospital. Further, supportive services should be provided in order that those who are satisfied with hospital services return to their community and live their in dependent lives. Based on these results, the researcher would make the following recommendation. 1) Because home nursing becomes more and more needed due to a sharp increase in chronic disease patients and elderly people, related rules and regulations should be made and implemented. 2) Hospital nurses specializing in home nursing should be cultivated.

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Asymptomatic Primary Hematuria in Children (소아의 무증상성 일차성 혈뇨에 관한 고찰)

  • Lee, Jung-Mi;Park, Woo-Saeng;Ko, Cheol-Woo;Koo, Ja-Hoon;Kwak, Jung-Sik
    • Childhood Kidney Diseases
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    • v.4 no.1
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    • pp.25-32
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    • 2000
  • Purpose: This retrospective study of 126 children with symptomless primary hematuria was undertaken to determine the distribution of various histologic types by renal biopsy, clinical outcome according to the biopsy findings and also to find out feasibility of performing renal biopsy in these children. Patients and Methods : Study population consisted of 126 children with symptom-less primary hematuria who have been admitted to the pediatric department of Kyung-poot National University Hospital for the past 11 years from 1987 to 1998 and renal biopsy was performed percutaneously. Hematuric children with duration of less than 6 months, evidences of systemic illness such as SLE or Henoch-Schonlein purpura, urinary tract infection, and idiopathic hypercalciuria were excluded from the study. Results : Mean age of presentation was 9.2${\pm}$3.3 years (range ; 1.5-15.3 years) and male preponderance was noted with male to female ratio of 2:1. IgA nephropathy was the most common biopsy finding occuring in 60 children ($47.6\%$), followed by MsPGN in 13 ($10.3\%$), MPGN in 5 ($3.9\%$), TGBM in 6 ($4.7\%$), Alport syndrome in 2 ($1.6\%$), FSGS in 1 ($0.8\%$), and in 39 children ($30.9\%$), 'normal' glomeruli were noted. Recurrent gross hematuria was more common than persistent microscopic hematuria (84 versus 42), and especially in IgA nephropathy, recurrent gross hematuria was the most prevalent pattern of hematuria. In 58 out of 126 cases ($46.0\%$), hematuria was isolated without accompa-nying proteinuria and this was especially true In cases of MsPGN and 'normal' glomer-uli by biopsy finding. Normalization of urinalysis (disappearance of hematuria) in IgA nephropathy, MsPGN and 'normal' glomuli group were similar and it was $14\%,\;27\%\;and\;21\%$ respectively during 1-2 years of follow-up period, and $37.1\%,\;40\%\;and\;35\%$ respectively during 3-4 years of follow-up periods. However, abnormal urinalysis persi-sted in the majority of children with MPGN, TGBM. Alport syndrome and FSGS. Renal function deteriorated progressively in 6 cases (3 with IgA nephropathy, 2 with Alport syndrome and 1 with TGBM). Conclusion : In summary, present study demonstrates that in 126 children with symptomless primary hematuria, IgA nephropathy was the most common biopsy findings followed by MsPGN, MPGN, TGBM, Alport syndrome and FSGS, and 'normal glomeruli' was also seen in 39 cases ($30.9\%$). Renal histology could not be predictable on the clinical findings, so that to establish appropriate long-term planning for these children, we would recommend to obtain precise histologic diagnosis by renal biopsy.

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Peak Expiratory Flow(PEF) Measured by Peak Flow Meter and Correlation Between PEF and Other Ventilatory Parameters in Healthy Children (정상 소아에서 최고호기유량계(peak flow meter)로 측정한 최고호기유량(PEF)와 기타 환기기능검사와의 상관관계)

  • Oak, Chul-Ho;Sohn, Kai-Hag;Park, Ki-Ryong;Cho, Hyun-Myung;Jang, Tae-Won;Jung, Maan-Hong
    • Tuberculosis and Respiratory Diseases
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    • v.51 no.3
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    • pp.248-259
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    • 2001
  • Background : In diagnosis or monitor of the airway obstruction in bronchial asthma, the measurement of $FEV_1$ in the standard method because of its reproducibility and accuracy. But the measurement of peak expiratory flow(PEF) by peak flow meter is much simpler and easier than that of $FEV_1$ especially in children. Yet there have been still no data of the predicted normal values of PEF measured by peak flow meter in Korean children. This study was conducted to provide equations to predict the normal value of PEF and correlation between PEF and $FEV_1$ in healthy children. Method : PEF was measured by MiniWright peak flow meter, and the forced expiratory volume and the maximum expiratory flow volume curves were measured by Microspiro HI 501(Chest Co.) in 346 healthy children(age:5-16 years, 194 boys and 152 girls) without any respiratory symptoms during 2 weeks before the study. The regression equations for various ventilatory parameters according to age and/or height, and the regression equations of $FEV_1$ by PEF were derived. Results : 1. The regression equation for PEF(L/min) was: $12.6{\times}$age(year)+$3.4{\times}$height(cm)-263($R^2=0.85$) in boys, and $6{\times}$age(year)+$3.9{\times}$height(cm)-293($R^2=0.82$) in girls. 2. The value of FEFmax(L/sec) derived from the maximum expiratory flow volume curves was multiplied by 60 to compare with PEF(L/min), and PEF was faster by 125 L/min in boys and 118 L/min in girls, respectively. 3. The regression equation for $FEV_1$(ml) by PEF(L/min) was:$7{\times}$PEF-550($R^2=0.82$) in boys, and $5.8{\times}$PEF-146 ($R^2=0.81$) in girls, respectively. Conclusion : This study provides regression equations predicting the normal values of PEF by age and/or height in children. And the equations for $FEV_1$, a gold standard of ventilatory function, was predicted by PEF. So, in taking care of children with airway obstruction, PEF measured by the peak flow meter can provide useful information.

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Clinical Characteristics of Arthrogryposis, Renal Tubular Dysfunction, Cholestasis(ARC) Syndrome in Korea (ARC(Arthrogryposis, Renal Tubular Dysfunction, Cholestasis) 증후군의 발병양상에 관한 연구)

  • Lee Soon Min;Kim Ji Hong;Lee Jae Seung;Han Suk Joo
    • Childhood Kidney Diseases
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    • v.9 no.2
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    • pp.222-230
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    • 2005
  • Purpose : ARC syndrome refers to an association of arthrogryposis, renal tubular dysfunction, and cholestasis. The VPS33B gene was recently identified as the causative gene. So far, 41 cases of ARC syndrome have been reported worldwide, and it has rarely been reported in Korea. This study was conducted to report the clinical findings of seven ARC syndrome cases in Korean children, focusing especially on renal tubular dysfunction. Methods : The hospital records of 7 cases diagnosed as ARC syndrome at Severance Hospital between Mar. 1995 and Aug. 2005 were reviewed and analyzed. Results : Of the 7 cases, 4 were boys and 3 were girls. Six patients(85$\%$) were born with normal birth weight at term, and one was born at preterm. All cases presented with cholestasis and severe jaundice. According to the type of arthrogryposis described by Brown et al, type 3 and 4 were found in 2 patients and type,6, 7, and the undistributed type in one patient respectively. Other associated clinical findings were as follows : failure to thrive in 6(85$\%$), lax skin in 5(71$\%$), and gray platelet syndrome in 4(57$\%$). Urine analysis revealed 6 cases(85$\%$) with proteinuria, 3(43$\%$) with hematuria, 5(71$\%$) with glycosuria, 2(29$\%$) with phosphaturia and 2(29$\%$) with calciuria. Serum electrolytes showed 4 cases(57$\%$) with hyponatremia, 3(43$\%$) with hypokalemia, and 1(14$\%$) with creatinine elevation. Renal tubular dysfunctions were diagnosed as renal tubular acidosis in 6 cases(85$\%$), nephrogenic diabetes insipidus in 2(29$\%$), and Fanconi syndrome in 2(29$\%$). During the follow-up period, 2(29$\%$) had no treatment, 5(85$\%$) had continuous supplementation to correct the electrolyte imbalance and acidosis, and 1(14$\%$) had dialysis. Only one patient had a family history of ARE syndrome in a sibling. Four cases(57$\%$) were diagnosed at the mean age of 8.2 months, and one case was lost during follow-up. Ages of the survived 2 cases were 13 and 25 months, respectively. Conclusion : The rare disease of ARC syndrome Is associated with severe renal dysfunction. However, this study revealed that the renal manifestation of ARC syndrome in Korean children is relatively mild and survival rate is higher than that of previous studies. Contrary to previous reports, this study showed that familial cases are rare and sporadic occurence is possible in Korea. Thus, the diagnosis of this syndrome requires a careful evaluation of the venal function in cases of congenital arthrogryposis, and a mandatory genetic counseling of affected family for prevention of familial occurance. (J Korean Soc Pediatr Nephrol 2005;9:222-230)

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Analysis of Childhood Rapidly Progressive Glomerulonephritis (소아 급속 진행성 사구체신염의 임상적 고찰)

  • Uhm Ji Hyun;Kim Mi Jin;Lee Young-Mock;Kim Ji Hong;Lee Jae Seung;Kim Pyung-Kil;Hong Soon Won;Jeung Hyeun Joo
    • Childhood Kidney Diseases
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    • v.5 no.2
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    • pp.78-86
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    • 2001
  • Purpose: Rapidly progressive glomerulonephritis (RPGN) is characterized by the rapid increase in serum creatitnin and crescents formation involving more than $50\%$ of glomeruli. 10 patients who had been treated for RPGN were studied retrospectively for thier underlying diseases and clinical features Method: Cilinical review was performed on 10 children who were diagnosed with RPGN by clinical features and renal biopsy and followed up at department of pediatrics during tile last 10 years, from May 1990 to May 2000. Result: There were 6 males and 4 females between the ages of 2.1 and 14.3 years (mean $10.9{\pm}3.8$). 3 had Henoch-$Sch{\ddot{o}}nlein$ purpura nephritis; 2, idiopathic rapidly progressive glomerulonephritis; 2, lupus nephritis; 1, hemolytic uremic syndrome; 1, membranous glomerulonephritis and 1, microscopic polyangiitis. The most common chief complaints were gross hematuria and oliguria. Initial clinical features included proteinuria, edema, hypertension, nausea and arthralgia. Mean serum BUN was $74.2{\pm}39.1\;mg/dL$ mean serum creatinin, $3.2{\pm}1.8\;mg/dL$ and mean creatinin clearance, $26.5{\pm}13.2\;mL/min/1.73m^2$. Antineutrophil cytoplasmic antibody was positive only in microscopic polyangiitis. ANA and Anti-DNA antibody were positive in two lupus nephritis patients. Serum complements were decreased in 4 patients. All patients except Hemolytic uremic syndrome received steroid pulse therapy and immunosupressive agents. 3 patients were performed acute peritoneal dialysis and 2 patients were given plasmapheresis. At the last follow up, 1 patient was dead, 4 patients had elevated serum creatinin, 2 of these 4 patients were on chronic ambulatory peritoneal dialysis and 6 patients had normal renal function. Conclusion: Rapidly progressive glomerulonephritis is a medical emergency that requires very rapid diagnosis, classification, and therapy. Appropriate therapy selected on the basis of underlying disease mechanism can substantially improve renal survival. (J. Korean Soc Pediatr Nephrol 2001 ; 5 : 78-86)

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