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Improved Acroparesthesia During Enzyme Replacement Therapy in a Patient Lately Diagnosed with Fabry Disease (진단이 지연된 Fabry 병 환자에서 효소대체요법을 통한 사지 말단 동통의 호전을 보인 1례)

  • Yang, Aram;Kim, Jinsup;Cho, Sung Yoon;Jin, Dong-Kyu
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.17 no.3
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    • pp.92-95
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    • 2017
  • Fabry disease (FD) is an X-linked lysosomal storage disorder caused by an ${\alpha}$-galactosidase A (GLA, MIM 300644) enzyme deficiency due to pathogenic variants in the ${\alpha}$-galactosidase A gene (GLA). The disease leads to accumulation of globotriaosylceramide (Gb3) and related glycophospholipids affecting nearly all major organ systems, with the primary sites damaged by Gb3 including renal glomeruli, myocardium, neurons of the dorsal ganglion and autonomic nervous system, and vascular endothelial and smooth muscle. Progressive deposition in these organ systems present with various clinical manifestations including acroparesthesia, renal failure and heart failure. Here, we report a Chinese male diagnosed with Fabry disease in his late $4^{th}$ decades showing improvement of acroparesthesia during enzyme replacement therapy (ERT). A 48-year-old Chinese man who presented with chronic recurrent severe burning pain in his fingers and toes since the age of 10, with worse involvement of the former visited to our clinic for further evaluation. His medical history included a transient ischemic attack aged 40 and diagnosed with stage 4-5 chronic kidney disease aged 47. In the family history, the patient's brother was found to be have Fabry disease 1 month before his visit. Except for his brother, all other members of the family are healthy. Based on his medical history and family history, he was strongly suspicious for Fabry disease. He was found to have a galactose-alpha-1,3-galactose level 4.96 (Reference range, 42.5-67.9) suggestive of Fabry disease. The followed sequencing of GLA coding region in our patient revealed hemizyosity for the mutation c.988C>T (Q330X) in Exon 7. Since ERT start, he showed significant improvement in his symptoms of burning sensation of fingers and toes. On the contrary, due to deteriorating kidney function even with ERT, he is considered for kidney transplantation. Despite of diagnostic delay until late 4th decades, ERT showed a potential improvement of acroparesthesia in our patient. However, late start of ERT can lead to poor outcome in multiorgan function. Therefore, early diagnosis with high index of suspicion followed by continuous ERT with regular monitoring have an impact on quality of life in Fabry disease.

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A Study on the Relationship between the Experience of Sanhujori, the Traditional Postpartal Care in Korea and Present Health Status of Chronic Arthritis Female Patient (만성관절염 여성 환자의 산후조리 경험과 건강상태와의 관계)

  • Yoo, Eun-Kwang;Lee, Sun-Hyae;Kim, Myoung-Hee
    • Women's Health Nursing
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    • v.4 no.2
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    • pp.217-230
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    • 1998
  • The purpose of this descriptive correlational study was to define the relationship between the experience of Sanhuujori, Korean traditional non-professional postpartal care after delivery and abortion and present health status of chronic arthritis female patient who visited to outpatient clinic of rheumatic internal medicine at a hospital located in Seoul, Korea. A convenience sample of 64 women who orally agreed to be a participant and data were collected form October 1996 to May, 1997 for sis months by way of interview with semistructured questionnaire. The data were analyzed by the SPSS pc program using t-test, ANOVA and Scheffe test as a post hoc and Pearson Correlation Coefficient. The results of the study were as follows ; Mean age of participants was 53.2 years and mean number of children was 3.1. Mean frequency of abortion was 2.1 times per woman. Seventy four percentage of respondents did not have Sanhujori after abortion. The mean period of Sanhujori after delivery was 17.7, 15.2, 13.8 days from the first child to third child and shorter than that of general woman such as 20.0, 19.0, 17.3 days in the previous study. On the subjective evaluation of whether the women did Sanhujori well or not, the rate of 'did Sanhujori wrongly' was the highest rank in each child where as general woman 'did Sanhujori well' at the first child, 'moderate' at the second and third child and 'did Sanhujori wrongly' at the 4th and fifth child. The health status implies both subjective health status women perceived and the rate of complaints of physical symptom distress women are experiencing presently. The respondents of 82.5% perceived them as unhealthy or sick and 68.9% of women complained more than two symptoms. Mean number of physical symptom distress women complained was 2.33. The main sites of physical symptom distress were upper & lower extremities 69.1% including knee and hand, whole body 19.1%, neck 3.7%, waist & shoulders 2.7% respectively. The characteristics of the symptoms were mostly pain 60%, swelling 19.8%, rigidity & deformity 7.9% respectively, sensation of heat 6.8% and weakness 1.7%. Women perceived the etiology of the chronic arthritis as stress 25.8%, 'did Sanhujori wrongly' & overwork 23.4% respectively, genetic 12.9%, malnutrition, 4.8%, and aging process 3.2%. There were significant positive correlation between subjective health status and the period of Sanhujori after delivery of the second child(r=-0.22) and negative correlation with the number of child at the level of 5% of significance statistically(r=0.27). There were significant negative correlation between the rate of complaints of physical symptom distress and the subjective evaluation whether she did Sanhujori well or not at the level of 5% of significance statistically(r=-0.23). And the rate of complaints of physical symptom distress in the group of women who experienced abortion was significantly higher than that of women who did not experience it at the level of 5% significance statistically(t=2.00) In conclusion, this finding reconfirmed the possible relationship between health status of chronic arthritis female patient and the experience of Sanhujori after delivery & abortion. It provides a challenge to the professional care givers to research further on the effects of Sanhujori on the health status, health recovery after abortion or delivery from the various aspects through the crosssectional and longitudinal research for the refinement of the reality of not only as cultural phenomenon but as conceptual model for the appropriateness of intervention and quality of care for desirable health outcomes.

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Treatment and Follow-up of Human Papillomavirus Infected Women in a Municipality in Southern Brazil

  • Ruggeri, Joao Batista;Agnolo, Catia Millene Dell;Gravena, Angela Andreia Franca;Demitto, Marcela de Oliveira;Lopes, Tiara Cristina Romeiro;Delatorre, Silvana;Carvalho, Maria Dalva de Barros;Consolaro, Marcia Edilaine Lopes;Pelloso, Sandra Marisa
    • Asian Pacific Journal of Cancer Prevention
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    • v.16 no.15
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    • pp.6521-6526
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    • 2015
  • Background: This study aimed toanalyze the risk behavior for cervical cancer (CC) and the human papillomavirus (HPV) prevalence and resolution among women who received care through the private healthcare network of a municipality in southern Brazil. Materials and Methods: This descriptive and retrospective study was conducted with 25 women aged 20 to 59 years who received care through the private healthcare network and were treated at a specialty clinic in the period from January to December 2012 in a municipality in Northwest Parana, Southern Brazil. Data from medical records with cytological and HPV results were used. Following treatment, these women were followed-up and reassessed after 6 months. Data were statistically analyzed using the t-test and chi-squared test at a 5% significance level. Results: The mean age of the studied women was $27.8{\pm}7.75$ years old, and the majority were married, with paid employment and were non-smokers. The mean age at menarche was $13.0{\pm}0.50$ years old, and the mean age at first intercourse was $17.5{\pm}1.78$ years, with only 8.0% (2) initiating sexual activity at an age ${\leq}15$ years old. The majority had 1 to 2 children (60.0%), while 88.0% reported having had one sexual partner in their lifetime, and all the women were sexually active. A total of 68.0% used a hormonal contraceptive method. All the women had leukorrhea and pain and were infected by a single HPV type. Regarding the lesion grade, 80.0% showed high risk and 20.0% low risk. The most prevalent high-risk HPV strain was 16. Conclusions: These findings provide relevant information on HPV risk factors and infection, as well as the treatment and 6-month follow-up results, in economically and socially advantaged women with no traditional risk factors, corroborating previous reports that different risk factors may be described in different populations. Thus, this study reinforces the fact that even women without the traditional risk factors should undergo HPVmonitoring and assessment to determine the persistence of infection, promoting early diagnosis of the lesions presented and appropriate treatment to thus prevent the occurrence of CC.

Comparison of Effectiveness about Image Quality and Scan Time According to Reconstruction Method in Bone SPECT (영상 재구성 방법에 따른 Bone SPECT 영상의 질과 검사시간에 대한 실효성 비교)

  • Kim, Woo-Hyun;Jung, Woo-Young;Lee, Ju-Young;Ryu, Jae-Kwang
    • The Korean Journal of Nuclear Medicine Technology
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    • v.13 no.1
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    • pp.9-14
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    • 2009
  • Purpose: Nowadays in the nuclear medicine, many studies and efforts are being made to reduce the scan time, as well as the waiting time to be needed to execute exams after injection of radionuclide medicines. Several methods are being used in clinic, such as developing new radionuclide compounds that enable to be absorbed into target organs more quickly and reducing acquisition scan time by increase the number of Gamma Camera detectors to examine. Each medical equipment manufacturer has improved the imaging process techniques to reduce scan time. In this paper, we tried to analyze the difference of image quality between FBP, 3D OSEM reconstruction methods that commercialized and being clinically applied, and Astonish reconstruction method (A kind of Iterative fast reconstruction method of Philips), also difference of image quality on scan time. Material and Methods: We investigated in 32 patients that examined the Bone SPECT from June to July 2008 at department of nuclear medicine, ASAN Medical Center in Seoul. 40sec/frame and 20sec/frame images were acquired that using Philips‘ PRECEDENCE 16 Gamma Camera and then reconstructed those images by using the Astonish (Philips’ Reconstruction Method), 3D OSEM and FBP methods. The blinded test was performed to the clinical interpreting physicians with all images analyzed by each reconstruction method for qualitative analysis. And we analyzed target to non target ratio by draws lesions as the center of disease for quantitative analysis. At this time, each image was analyzed with same location and size of ROI. Results: In a qualitative analysis, there was no significant difference by acquisition time changes in image quality. In a quantitative analysis, the images reconstructed Astonish method showed good quality due to better sharpness and distinguish sharply between lesions and peripheral lesions. After measuring each mean value and standard deviation value of target to non target ratio with 40 sec/frame and 20sec/frame images, those values are Astonish (40 sec-$13.91{\pm}5.62$ : 20 sec-$13.88{\pm}5.92$), 3D OSEM (40 sec-$10.60{\pm}3.55$ : 20 sec-$10.55{\pm}3.64$), FBP (40 sec-$8.30{\pm}4.44$ : 20 sec-$8.19{\pm}4.20$). We analyzed target to non target ratio from 20 sec and 40 sec images. And we analyzed the result, In Astonish (t=0.16, p=0.872), 3D OSEM (t=0.51, p=0.610), FBP (t=0.73, p=0.469) methods, there was no significant difference statistically by acquisition time change in image quality. But FBP indicates no statistical differences while some images indicate difference between 40 sec/frame and 20 sec/frame images by various factors. Conclusions: In the circumstance, try to find a solution to reduce nuclear medicine scan time, the development of nuclear medicine equipment hardware has decreased while software has marched forward at a relentless. Due to development of computer hardware, the image reconstruction time was reduced and the expanded capacity to restore enables iterative methods that couldn't be performed before due to technical limits. As imaging process technique developed, it reduced scan time and we could observe that image quality keep similar level. While keeping exam quality and reducing scan time can induce the reduction of patient's pain and sensory waiting time, also accessibility of nuclear medicine exam will be improved and it provide better service to patients and clinical physician who order exams. Consequently, those things make the image of department of nuclear medicine be improved. Concurrent Imaging - A new function that setting up each image acquisition parameter and enables to acquire images simultaneously with various parameters to once examine.

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AN EPIDEMIOLOGIC STUDY ON THE PEDIATRIC PATIENTS IN DEPARTMENT-OF PEDIATRIC DENTISTRY, CHOSUN UNIVERSITY DENTAL HOSPITAL FOR LAST 10 YEARS(1990-1999) (최근 10년간 조선대학교 치과병원 소아치과에 내원한 신환에 관한 역학적 연구(1990-1999))

  • Ryu, Hyun-Seop;Kim, Hyo-Suck;Lee, Chang-Seop;Lee, Sang-Ho
    • Journal of the korean academy of Pediatric Dentistry
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    • v.28 no.3
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    • pp.345-354
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    • 2001
  • The purpose of this study was to make public oral health plan more effective and the improvement of the hospital management system for better clinical dental practice. Distribution and trends were examined in all patients who had been examined and diagnosed at Department of Pedodontics, Dental Hospital, Chosun University over 10 year-period from 1990 to 1999 Results were as follows, 1. The number of patients per year was increasing trend after 1996 and higher visiting rate in male(55.9%) than in female(44.1%). 2. Age distribution had shown $3\sim4$ year-old cup being the largest (23.4%) and each percentage of $5\sim6,\;7\sim8,\;0\sim2,\;9\sim10,\;11\sim12,\;13\sim14,\;above\sim15$ year-old group was 19.9%, 17.7%, 14.6%, 12.6%, 8.0%, 3.3%. 0.5%. 3. Geographic distribution showed a majority of patients in Kwang-Ju (83.0%). Group in the suburbs of Kwang-Ju(Jang-Sung group) was 5.4%. 4. Dental caries showed the highest percentage(40.5%) in chief complaints and percentage of oral exam, orthodontic problem, oral pain, trauma, supernumerary teeth, swelling was 13.9% 12.6%, 8.8%, 7.4%, 5.5%, 4.9%. 5. In all patients with traumatic problem, crown fracture showed the highest percentage(41.4%) and percentage of subluxation was 19.4%. 6. Majority(78.7%) of patients were not refered, and percentage of patients refered from local clinic was 20.5%. 7. Patients who had periodic check-up comprised 19.6%, and percentage of after 3, 6, 9, 12, 18, 24month was 36.7%, 22.2%, 13.5%, 11.3%, 5.4%, 11.0%.

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Short-term Follow up Study of Calcific Tendinitis Using Ultrasonography Guided Injection (석회화 건염에 대한 외래 초음파 유도 주사 치료요법의 단기추시결과)

  • Sim, Jung-Hyun;Kwon, Jae-Bum;Park, Chang-Min;Choi, Chang-Hyuk
    • The Journal of Korean Orthopaedic Ultrasound Society
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    • v.4 no.2
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    • pp.77-83
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    • 2011
  • Purpose: We compared with USG-guided steroid injection group and blind steroid injection group for the treatment of calcific tendinitis to evaluate the effectiveness of the treatment modalities. Materials and Methods: We reviewed two groups of calcific tendinitis treated with steroid injection and follow up upto 6 month after injection. Group I was blind injection for 88 patient with average age of 53years old and female gender in 77%. Group II was USG- guided injection for 102 patients with average age of 54years old and female gender in 85%. We compared follow up results according to age, gender, size of tendon involvement, initial VAS score, and phase of the calcific tendinitis between two groups. Results: At 6 month's follow up period, USG-guided injected group was more prevalent compared to blind injection group with 92%(94/102) and 72%(64/88) (P<0.05). In Group I, 77%(49/64) patients have improved symptom. And in Group II, 92%(86/94) patients have improved symptom in formative or resorptive phase. Despite of symptom improvement, calcium deposit is remained 47%(30/64) in Group I, and 20%(19/94) in Group II. Conclusion: Conservative treatment of Shoulder calcific tendinitis patients through USG-guided injection is more effective than Blind injection in pain relief & calcium decrease. The patients USG-guided injected group was more prevalent in follow up at outpatient clinic, throughout more accurate injection with improved confidence.

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Factors Contributing to Treatment Outcome of Functional Constipation in Children (소아 기능성 변비의 치료에 영향을 미치는 인자)

  • Kim, Hyung-Seok;Hong, You-Rha;We, Ju-Hee;Park, Jae-Hong
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.13 no.1
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    • pp.36-43
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    • 2010
  • Purpose: The aim of this study was to evaluate the clinical features and factors contributing to treatment outcome for chronic functional constipation in children. Methods: We analyzed the medical records of patients with constipation, who visited the inpatient or outpatient clinic of the Department of Pediatrics of Pusan National University Hospital, between January 1998 and December 2007. The clinical features, outcomes, and factors affecting the treatment response according to the main drug (lactulose vs. PEG 4000) were analyzed retrospectively. Results: Two hundred forty children (142 males and 98 females) were enrolled in this study. The mean age was 51.2${\pm}$37.9 months. The duration of symptoms was 32.6${\pm}$33.7 months. The accompanying symptoms were as follows: encopresis, 91 (30.4%); abdominal pain, 76 (31.6%); and blood-tinged stool, 37 (15.4%). The treatment response was achieved earlier in females (p<0.001), patients with accompanying symptoms (p<0.05), and patients treated with PEG 4000 (p=0.001). The duration of symptoms (p<0.05) and stool frequency before treatment (p<0.05) were related to a delayed treatment response. Relapse occurred in 7 children, all of whom were treated successfully later. Conclusion: Factors contributing to treatment response are female gender, accompanying symptoms, duration of symptoms, and stool frequency before treatment. PEG 4000 is superior to lactulose in response time and taken into consideration as a primary drug for the treatment of functional constipation of children. Early treatment and sufficient treatment time may also be important factors to achieve an early response and prevent relapse.

A Study on the Dental Service Statifation of Cityizens in Deajeon (대전시 시민의 치과의료서비스에 관한 만족도 조사연구)

  • Sung, Bo-Kyun
    • Journal of Korean society of Dental Hygiene
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    • v.8 no.4
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    • pp.19-30
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    • 2008
  • This study reached the following conclusions as a result of carrying out the questionnaire survey of self-descriptions for the satisfaction after the citizens of Daejon uses the dental clinics, in order to identify the factors of satisfaction to the medical services of such dental clinics to be utilized in the patient management by dental hygienists, provide the basic data to provide the medical services desired by patients. 1. 43.9% men responded to the facilities and 56.1% women to the atmosphere for the standards of selection of dental clinics by general characteristic, and the college graduates or more to the kindness (38.2%), high-school graduates (43.2%) and middle-school graduates (25.9%) or less to the close distance for the level of educational attainment (p=0.009), which was meant to have a statistical significance. 2. The execution of reservation system for the dental clinics showed 54.7%, the reserved time was observed upon the execution of such reservation system, the dental clinics where they practice such system were 40.6%, and the confirmation methods was done through the telephone with 62.5%. 3. The experience of fear upon the dental treatment showed 74.6%. The type of fear showed the machine sound (48.7%) for men and cry of others for women (70.8%) at the highest. 70% of those under 30 at the age responded to the sharp instruments at the highest. 83.3% of Yousung-gu showed the highest by responding to the cry of others for the residential areas. The statistically significant difference was shown in both the age and residential area (p=0.000). 4. Women showed higher in the distribution of gender for the sterilization of instruments for the external satisfaction of dental clinics(p=0.000) and those under 30 at the age showed the highest with 2.98${\pm}$0.95(p=0.001). Seo-gu (3.48${\pm}$0.77) was the highest for the residential area (p=0.000), and there was statistically significant differences in the gender, age and residential area. 5. Men showed higher satisfaction than women in the clean state and the statistically significant differences were shown (p=0.000) at the age as the high satisfaction was shown for those under 30 at the age (2.35${\pm}$0.79), those having the income not less than 10 million won and not more than 20 million won (2.43${\pm}$0.78), and Seo-gu (2.63 ${\pm}$0.69) for the residential area. 6. For the internal satisfaction of dental clinic by users for the medical services in the dental clinics, 61.1% women responded to no in the ability of solving the inconvenience in the service process, and showed low ability of solving the inconvenience from 30 at the age (26.2%) and by responding to Dong-gu (22.1%) for the residential area, showing statically significant differences(p=0.000). For the re-use of dental clinics, 46.6% men (p=0.043) for the gender, 24.3% under 30 at the age and 22.9% of Dong-gu for the residential area responded to the re-use, showing statistically significant differences for the gender and residential area (p=0.000). 7. The dissatisfaction showed a high rate of 69.5% for the satisfaction to the medical services of dental clinics. 46.2% men responded to the pain and women to the feeling of foreign substance for the reason of dissatisfaction while those under 30 at the age showed 55.6% for others, those between 50 and 59 41.7% for the feeling of foreign substance. 86.3% carried out the education for cautions after the treatments and most people turned out that they do not carry out the continuous health management of mouth as 20.5% responded to that they carry out such health management.

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A Survey on Treatment of Breast Cancer Patients with Korean Medicine: Preliminary Research for Clinical Practice Guidelines (한의표준임상진료지침 개발을 위한 유방암 보완치료 실태조사)

  • Kim, Nam-Hoon;Kang, Na-Hoon;Yoo, Eun-Sil;Park, Nam-Chun;Lee, Jin-Wook;Park, Kyoung-Sun;Lee, Jin-Moo;Lee, Chang-Hoon;Jang, Jun-Bock;Jang, Bo-Hyoung;Hwang, Deok-Sang
    • The Journal of Korean Obstetrics and Gynecology
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    • v.31 no.4
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    • pp.165-178
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    • 2018
  • Objectives: To determine the current status of the treatment of breast cancer patients by Korean Medicine doctors (KMDs) and to examine the need for clinical practice guidelines (CPGs) for the supportive care of these patients. Methods: This cross-sectional study used a self-administered questionnaire. Participants were asked about their experience in treating breast cancer patients; the number of breast cancer patients they currently treat; the main complaints expressed by, diagnoses of, and treatments used for such patients; and their opinions about Korean Medicine (KM) as the basis for providing supportive care for breast cancer. Results: The data for this study were collected from 322 respondents. 84 of whom reported having ever treated patients with breast cancer. Most breast cancer patients who visited the KM clinic were classified as stage I or II, and their major complaints were fatigue, general weakness and musculoskeletal pain. The major diagnostic strategies were syndrome differentiation and pulse diagnosis. The major treatments administered were herbal medicine, acupuncture, and moxibustion. KMDs cited a need for medical information, such as CPGs, as their most important concern with regard to the treatment of breast cancer patients. Conclusions: This survey determined the prevalence of the use of KM for Korean breast cancer patients. Our results underscore the need for clinical practice guidelines for using of KM as the basis of supportive care for breast cancer and for informing clinicians and patients about this approach.

Is a Fast-track Critical Pathway Possible in Gastric Cancer Surgery? (위암 수술 후 조기회복을 위한 임상진료 지침서의 활용이 가능한가?)

  • Yi, Jeong-Min;Hur, Hoon;Kim, Sung-Keun;Song, Kyo-Young;Chin, Hyung-Min;Kim, Wook;Park, Cho-Hyun;Park, Seung-Man;Lim, Keun-Woo;Jeon, Hae-Myung
    • Journal of Gastric Cancer
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    • v.9 no.1
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    • pp.18-25
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    • 2009
  • Purpose: The postoperative hospital stay after gastric cancer surgery is usually 8 to 14 days. The main reason for a prolonged hospital stay may be the 3 to 4 day period of postoperative starvation. The aim of this study is to investigate the feasibility of a critical pathway for early recovery after gastric cancer surgery, and this pathway included early postoperative feeding. Materials and Methods: One hundred three consecutive patients who underwent gastric resection and reconstruction for gastric cancer from October 2007 to June 2008 at St. Mary's Hospital were prospectively enrolled in a fast-track critical pathway. The pathway included minimal preoperative procedures, optimal pain relief, proper fluid administration, early mobilization and early enteral nutrition at postoperative 1 day. The exclusion criteria were determined preoperatively, intraoperatively and postoperatively. Results: Of the 103 patients, 19 patients were excluded for preoperative (5), intraoperative (7) and postoperative (7) reasons. Eighty-four patients (81.6%) were included in the fast-track critical pathway. Sixty-eight (88.6%) of 84 patients were discharged at the planned 8 day after surgery during the initial period of the study, and the postoperative hospital stay was shortened up to 6 days during the more recent period. Postoperative complications occurred in 22 patients (26.2%), and these were gastrointestinal track-related complications in 6 cases (3 cases of ileus, 1 case of stasis and 2 cases of leakage) and infection-related complications in 8 cases. There was no statistical difference in the incidence of morbidity according to the clinic-operative features that included age, gender, stomach resection and lymphadenectomy. Conclusion: The fast-track critical pathway with using the available exclusion criteria was a valid option for patients who underwent gastric cancer surgery.

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