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Renal manifestations in tuberous sclerosis complex (결절성 경화증 환자에서의 신장 발현)

  • Jeong, Il Cheon;Kim, Ji Tae;Hwang, You Sik;Kim, Jung A;Lee, Jae Seung
    • Clinical and Experimental Pediatrics
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    • v.50 no.2
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    • pp.178-181
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    • 2007
  • Purpose : The renal manifestations of tuberous sclerosis complex (TSC) are remarkably diverse, including polycystic kidney disease, simple renal cysts, renal cell carcinomas, and angiomyolipomas. All of these occur in children as well as adults in TSC. Angiomyolipomas, which can cause spontaneous life-threatening hemorrhages, are by far the most prevalent and the greatest source of morbidity. Here, we will address our experience, adding to the literature on pediatric patients with TSC requiring evaluation and treatment for renal manifestations. Methods : A retrospective analysis was made on 19 patients in whom TSC was diagnosed between May 2001 and Oct. 2005 at Severance Hospital. All patients had clinical diagnoses of TSC as defined by the 1998 tuberous sclerosis complex consensus conference. Results : The patients consisted of 13 boys and 6 girls with a mean age of 7.3 years (range 1 to 22). The renal disease associated with TSC included angiomyolipoma in nine patients (47.4 percent), renal simple cyst in one (5.3 percent), hydronephrosis in one (5.3 percent) patient. Eight patients (42.1 percent) presented with normal kidney contours at abdominal ultrasonography. One patient underwent renal replacement therapy due to chronic renal insufficiency after nephrectomy. Hemorrhage from angiomyolipoma was not detected. Conclusion : In our review of 19 cases of TSC, renal manifestations are reported in 57.9 percent of patients. Asymptomatic angiomyolipoma associated with TSC grow gradually, although severe hemorrhages are rare. So patients with TSC should be followed up with serial computerized tomography or abdominal ultrasonography. And also, renal function should be monitored conservatively.

Evaluation of Health Impact of Heat Waves using Bio-Climatic impact Assessment System (BioCAS) at Building scale over the Seoul City Area (생명기후분석시스템(BioCAS)을 이용한 폭염 건강위험의 검증 - 서울시 건물규모를 중심으로 -)

  • Kim, Kyu Rang;Lee, Ji-Sun;Yi, Chaeyeon;Kim, Baek-Jo;Janicke, Britta;Holtmann, Achim;Scherer, Dieter
    • Journal of Environmental Impact Assessment
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    • v.25 no.6
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    • pp.514-524
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    • 2016
  • The Bio-Climatic impact Assessment System, BioCAS was utilized to produce analysis maps of daily maximum perceived temperature ($PT_{max}$) and excess mortality ($r_{EM}$) over the entire Seoul area on a heat wave event. The spatial resolution was 25 m and the Aug. 5, 2012 was the selected heat event date. The analyzed results were evaluated by comparing with observed health impact data - mortality and morbidity - during heat waves in 2004-2013 and 2006-2011,respectively. They were aggregated for 25 districts in Seoul. Spatial resolution of the comparison was equalized to district to match the lower data resolution of mortality and morbidity. Spatial maximum, minimum, average, and total of $PT_{max}$ and $r_{EM}$ were generated and correlated to the health impact data of mortality and morbidity. Correlation results show that the spatial averages of $PT_{max}$ and $r_{EM}$ were not able to explain the observed health impact. Instead, spatial minimum and maximum of $PT_{max}$ were correlated with mortality (r=0.53) and morbidity (r=0.42),respectively. Spatial maximum of $PT_{max}$, determined by building density, affected increasing morbidity at daytime by heat-related diseases such as sunstroke, whereas spatial minimum, determined by vegetation, affected decreasing mortality at nighttime by reducing heat stress. On the other hand, spatial maximum of $r_{EM}$ was correlated with morbidity (r=0.52) but not with mortality. It may have been affected by the limit of district-level irregularity such as difference in base-line heat vulnerability due to the age structure of the population. Areal distribution of the heat impact by local building and vegetation, such as spatial maximum and minimum, was more important than spatial mean. Such high resolution analyses are able to produce quantitative results in health impact and can also be used for economic analyses of localized urban development.

Clinical and Immunologic Features of Miliary Tuberculosis with Positive Bone Marrow Study (골수생검 양성인 속립성 결핵의 고찰)

  • Song, Kwang-Seon;Yong, Suk-Joong;Shin, Kye-Chul;Lee, Won-Yeon;Ryu, Jeong-Seon
    • Tuberculosis and Respiratory Diseases
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    • v.43 no.1
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    • pp.22-29
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    • 1996
  • Background: Abnormalities of the peripheral blood are frequent and varied in patients with miliary tuberculosis. Anemia, leukopenia, thrombocytopenia, pancytopenia, monocytosis, basophilia, eosinophilia and leukemoid reactions have been reported. These abnormalities are more frequent in patients with positive bone marrow study. In this report, we evaluated clinical, hematological and immunological features in patients with miliary tuberculosis in order to know whether difference is existed between "bone marrow biopsy positive group(pathologically proven to miliary tuberculosis)" and "negative group". Method: Clinical evaluation, serum ADA, sIL-2R, and T-lymphocyte subsets were measured in 40 patients with miliary tuberculosis who received bone marrow biopsy. Results: 1) The average age of patients was 39 year-old. There were 23 male and 17 female patients. Associated extrapulmonary tuberculosis are 9 CNS tuberculosis, 6 joint tuberculosis, and 2 tuberculous pleurisy. 2) Sixteen of the 40 patients were positive bone marrow biopsy(60%). 3) Sixteen of the 40 patients(60%) had anemia(11 positive patients: 13 negative patients). Leukopenia occurred in 12 per cent(4:1). Thrombocytopenia was noted in 10%(3:1). 4) The mean value of serum ADA was 83 U/L(90 U/L: 70.6 U/L, p=0.23). 5) The mean activity of Soluble IL-2 receptor was 4,643 pmol/L($6840{\pm}7446\;pmol/L$: $1,897{\pm}1,663\;pmol/L$, p=0.06). 6) In the T lymphocyte subsets, the percent of T-lymphocytes was 64%(62%:73%, p=0.2). In some patients(9), $T_4$ and $T_8$ ratio in BAL fluid($1.97{\pm}1.2$) was higher than that in the peripheral blood($1.16{\pm}0.5$). Conclusion: Bone marrow examination are diagnostic in 60% of cases of miliary tuberculosis. Percents of the total T lymphocyte and helper T cell in BAL are more elevated than in peripheral blood. There was no significant difference in peripheral blood abnormalities and marker of T lymphocyte activation between the bone marrow biopsy positive and negative group.

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Clinical Outcomes After Arthroscopic Double-Row Rotator Cuff Repair and Evaluation of Cuff Integrity by CT Arthrography (관절경적 2열 고정 회전근개 복원술 후의 임상 결과 및 CT 관절조영술을 이용한 건의 치유 평가)

  • Jo, Chris H.;Kim, Je-Kyoon;Yoon, Kang-Sup;Lee, Ji-Ho;Kang, Seung-Baek;Lee, Jae-Hyup;Han, Hyuk-Soo;Rhee, Seung-Whan
    • Clinics in Shoulder and Elbow
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    • v.12 no.2
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    • pp.199-206
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    • 2009
  • Purpose: Our goal for this study was to prospectively evaluate the functional & structural outcomes, by means of CT arthroscopy, of arthroscopic double-row fixation for treating rotator cuff tear. We also attempted to determine the variants that affect the functional & structural outcomes. Materials and Methods: Twenty seven consecutive patients underwent arthroscopic rotator cuff repair with double-row fixation. The average age at the time of the operation was fifty six years. The preoperative and postoperative examinations consisted of determining the Constant score, the score for the visual analogue scale for pain, the UCLA score, the American Shoulder and Elbow Surgeons (ASES) score, as well as a full physical examination of the shoulder. Preoperative MR arthrography was used to evaluate the integrity and atrophy of the rotator cuff. We measured the intraoperative tear size in the sagittal and coronal planes. Postoperative CT arthrography was used at one year postoperatively to evaluate the integrity and atrophy of the repaired tendons and muscles. Results: Preoperative MR arthrography revealed an average 29.22 mm tear size in the sagittal plane and an average 22.72 mm tear size in the coronal plane. Twelve cases of supraspinatus muscle atrophy and two cases of infraspinatus atrophy were observed on the preoperative MR arthrography. The average clinical outcome scores all significantly improved at the time of follow-up. At a mean of one year postoperatively, CT arthrography revealed 48.1% of the shoulders had healed, 11.1% showed incomplete healing and 40.7% showed retear of the repaired tendon. Conclusion: Arthroscopic double-row repair can result in improved clinical outcomes and good patient satisfaction. However, the problems about how to enhance healing of the repaired tendon still remain.

Arthroscopic Treatment using Bioabsorbable Knotless Anchor for Anterior Instability of Shoulder (관절경하 생체흡수형 Knotless Anchor를 이용한 견관절 전방 불안정성의 치료)

  • Lee, Yong-Jae;Lee, Tong-Joo;Lim, Kwang-Yul;Kim, Myung-Ku
    • Journal of the Korean Arthroscopy Society
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    • v.8 no.2
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    • pp.103-108
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    • 2004
  • Purpose: This study reported the outcomes following the use of bioabsorbable knotless anchor in patients with anterior instability of shoulder. Methods: We studied fifteen cases with traumatic anterior shoulder instability underwent arthroscopic Bankart repair with bioabsorbable knotless suture anchor between January 2003 and June 2003. Among fifteen patients, fourteen were male and one was female, with a mean patient age of 24 years (range 16-42). The mean follow-up was 14 months (range 12-18 months). We compared with operation time of twenty cases of arthroscopic Bankart repair by the suture anchor technique between January 2002 and October 2002. Results: Neither recurrent dislocation nor subluxation was happened in postoperative follow-up. Mean score for functional evaluation by Rowe et al. was 89.4 and that for patient subjective satisfaction was 87,5. At last follow-up period, average shoulder range of motion for flexion and external rotation was 171$^{\circ}$ and 54$^{\circ}$ respectively. All patients were satisfied except three who had an apprehension at the follow up. During Bankart repair, it took an average of 25.5 minutes for one knot with the use of suture anchor technique whereas an average of 16.5 minutes for one knot with the use of bioabsorbable knotless anchor. Significantly, we saved operation time with the use of bioabsorbable knotless anchor (P<0.05).Conclusion: Repairing the Bankart lesion with the use of knotless anchor technique has the advantage of obtaining good capsular tensioning and saving operation time. And it is considered to be very successful in treating shoulder instability without recurrent dislocation or subluxation.

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The Evaluation of Clinical Usefulness on Application of Myocardial Extract in Quantitative Perfusion SPECT (QPS 프로그램에서 Myocardial extract 적용에 따른 임상적 유용성 평가)

  • Yun, Jong-Jun;Lim, Yeong-Hyeon;Lee, Mu-Seok;Song, Hyeon-Seok;Jeong, Ji-Uk;Park, Se-Yun;Kim, Jae-Hwan;Kim, Jeong-Uk
    • The Korean Journal of Nuclear Medicine Technology
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    • v.15 no.2
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    • pp.88-93
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    • 2011
  • Purpose: As to analytical method of data, the AutoQUANT software in which it is used quantitative rating of the myocardial perfusion SPECT are reported that there is a difference. Therefore the measured value error of the mutual program is expected to be generated even if the quantitative analysis is made data of the same patient. The purpose of this study is to offer the comparative analysis of myocardial extract method in Quantitative Perfusion SPECT. Materials and methods: We analyzed the 51 patients who were examined by Tc-99m MIBI gated myocardial SPECT in nuclear medicine department of Pusan National University Hospital from June to December 2010(34 men, 17 women, mean age $66.5{\pm}9.9$). We acquired the extracted image in myocardial extract protocol. QPS program that uses the AutoQUANT software measured TID(Transient Ischemic Dilation), ESD(Extent of Stress Defect), SSS(Summed Stress Score). Then analyzed the results. Results: The correlation of appyling myocardial extract is TID(r=0.98), ESD(r=0.99), SSS(r=0.99). In the 95% confidence limit, there was no satistically significant difference(TID p=0.78, ESD p=0.31, SSS p=0.19). After blinding test with a physician for making a qualitative analysis, there was no difference. Conclusion: Quantitative indices in QPS program showed good correlation and the results showed no statistically signigicant difference. The variance between method was small. therefore, the functional parameters by each method can be used interchangeably. Also, we expect patient's satisfaction.

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Treatment of Intracranial Meningioma with Linac Based Radiosurgery (뇌수막종에서 선형가속기를 이용한 방사선수술 효과)

  • Kay Chul Seung;Yoon Sei Chul;Chung Su Mi;Ryu Mi Ryung;Kim Yeon Sil;Suh Tae Suk;Choi Kyuho;Son Byung Chul;Kim Moon Chan
    • Radiation Oncology Journal
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    • v.19 no.2
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    • pp.81-86
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    • 2001
  • Prupose : To evaluate the role of linac based radiosurgery (RS) in the treatment of meningiomas, we retrospectively analyzed the results of clinical and follow up CT/MRI studies. Methods and Materials : From the 1988 July to 1998 April, twenty patients of meningioma had been treated with 6 MV linear accelerator based radiosurgery. Of the 20 patients, four $(20\%)$ were male and 16 $(80\%)$ were female. Mean age was 51 years old ($22\~78$ years old). Majority of intracranial location of tumor for RS were parasagittal and sphenoid wing area. RS was done for primary treatment in 6 $(30\%)$, postoperative residual lesions in 11 $(55\%)$ and regrowth after surgery in 3 $(15\%)$. Mean tumor volume was $5.72\;cm^3\;(0.78\~15.1\;cm^3)$ and secondary collimator size was 2.04 cm $(1\~3\;cm)$. The periphery of tumor margin was prescribed with the mean dose of 19.6 Gy $(9\~30\;Gy)$ which was $40\~90\%$ of the tumor center dose. The follow up duration ranged from 2.5 to 109 months (median 53 months). Annual CT/MRI scan was checked. Results : By the follow up imaging studies, the tumor volume was reduced in 5 cases $(25\%)$, arrested growth in 14 cases $(70\%)$, and increased size in 1 case $(15\%)$. Among these responsive and stable 19 patients by imaging studies, there showed loss of contrast enhancement after CT/MRI in four patients. In clinical response, nine $(45\%)$ patients were considered improved condition, 10 $(50\%)$ patients were stable and one $(5\%)$ was worsened to be operated. This partly resulted in necrosis after surgery. Conclusion : The overall control rate of meningiomas with linac based RS was $95\%$ by both imaging follow-up and clinical evaluation. With this results, linac based RS is considered safe and effective treatment method for meningioma.

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Clinical Evaluation of Coronary Artery Fistula (관상동맥루의 임상적 고찰)

  • Lee Sak;Park Han-Ki;Lim Sang-Hyun;Hong You-Sun;Chang Byung-Chul;Kang Meyun-Shick;Cho Bum-Koo;Park Young-Hwan
    • Journal of Chest Surgery
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    • v.38 no.10 s.255
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    • pp.699-704
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    • 2005
  • Background: Coronary artery fistula is rare congenital anomaly, which account for $0.27\~0.40\%$ of all congenital heart diseases. We report the clinical observations of 45 patients with coronary artery fistula. Material and Method: We reviewed all patients presented with or without symptoms of coronary artery fistula between 1987 and 2004. Age ranged from 1 to 83 years. Twenty-six patients were female. The patients were divided into 2 groups according to the presenting symptoms. Twelve patients were in group A (asymptomatic) and 33 patients in group B (symptomatic). The most common clinical presentation in group B was angina (18) followed by dyspnea (7), atypical chest pain (5), syncope (1), fatigue (1), and palpitation (1). Twenty-five patients were associated with other cardiac diseases, which were atrial septal defect (4), coronary artery occlusive disease (6), hypertension (12), and valvular heart disease (2). Result: Patients were followed-up for a mean period of $64.8\pm62.7$ months. There was no complication related to coronary artery fistula during the follow-up period in both group. There was no mortality related to coronary artery fistula. Conclusion: In symptomatic patients, early surgical treatment is recommended considering the low perioperative morbidity. In asymptomatic patients receiving medical treatment, close follow up may be necessary.

Treatment Outcome of Postoperative Radiotherapy in Extrahepatic Bile Duct Cancer (간외담관암의 수술 후 방사선치료 성적)

  • Choi, Young-Min;Cho, Heung-Lae
    • Radiation Oncology Journal
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    • v.21 no.1
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    • pp.27-34
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    • 2003
  • Purpose : To evaluate the efficacy of radiotherapy, and the factors affecting survival in patients of extrahepatic bile duct cancer, by analyzing the results of postoperative radiotherapy Materials and Methods : Between October 1991 and July 2001, 21 patients with extrahepatic bile duct cancer, who received radiotherapy after a radical resection, were retrospectively reviewed. The patients' ages ranged from 39 to 75 years, with a median of 61 years, and a male to female ratio of 16 to 5. The numbers of patients with proximal and distal bile duct cancer were 14 and 7, respectively. From the postoperative pathological examination, 19 of the patients were found to have microscopic residues, and 7 to be lymph node positive. Patients with AJCC stages I, II and III were 10, 10 and 1, respectively. The total radiation dose administered was 4,500$\~$6,300 cGy, with a median dose of 5,040 cGy. The follow up period was 20$\~$81 months, with a median of 57.5 months. Results : The overall and disease free survival rates at 3 and 5 years were 41.0 and 29.3$\%$, and 41.6 and 29.7$\%$, respectively. The influences of age, sex, tumor location, differentiation, microscopic residue, neural invasion, 7 and N stage, the stage itself, the dose of radiation and chemotherapy, on survival were evaluated. The T stage and the stage itself were found to be significant from a univariate analysis (p<0.05), but the degree of significance was limited by the small number of patients. A recurrence occurred in 12 patients (57.1$\%$), 5 in locoregional sites, 4 in distant sites and 3 in a combination of locoregional and distant sites, and the sites of distant metastasis were the liver, 6, and the bone, 1 Grade 2 or 3 acute leucopenias occurred in 2 patients and grade 2 chronic peptic ulcers occurred in 4, who were all recovered by conservative management. Conclusion : Postoperative radiotherapy is feasigbls in extrah데atic bile duct cancer, with tolerable toxicity, but prospective studies, with a large patient enrollment, are needed for the evaluation of the effects of postoperative radiotherapy and the related prognostic factors.

Antibody Responses in Hematopoietic Cell Transplantation Recipients after Vaccination Against Haemophilus Influenzae Type b and Streptococcus pneumoniae (소아 조혈모세포 이식 환자에서 b형 헤모필루스 인플루엔자와 폐렴구균 백신 접종 후 항체 반응에 관한 연구)

  • Kim, Yae-Jean;Hwang, Ji-Young;Choi, Soo-Han;Kong, Eunhye;Kim, Yanghyun;Park, Ki-Sup;Yoo, Keon-Hee;Sung, Ki-Woong;Koo, Hong Hoe;Kim, Kyung-Hyo
    • Pediatric Infection and Vaccine
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    • v.21 no.2
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    • pp.81-95
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    • 2014
  • Purpose: Hematopoietic cell transplantation (HCT) recipients are vulnerable to invasive infection by Haemophilus influenzae type b (Hib) and Streptococcus pneumoniae (Sp). This study was performed to evaluate immune responses after Hib and Sp vaccination in Korean pediatric HCT recipients. Methods: Patients were prospectively enrolled at Samsung Medical Center during 2009-2011. ELISA tests to detect anti-PRP IgG antibody and antibodies to Sp serotypes 4, 6B, 9V, 14, 18C, 19F, and 23F were performed at the Center for Vaccine Evaluation and Study, Ewha Medical Research Institute. Results: Ten patients (two allogeneic, eight autologous recipients) with median age 5.4 years (range 2.7-12.2 years) were enrolled. Before Hib vaccination, 60% of patients' anti-PRP IgG titers were below $0.15{\mu}g/mL$. After vaccination, 100% of patients' anti-PRP IgG titers increased above $0.15{\mu}g/mL$ (cut-off value for detection) and $1.0{\mu}g/mL$ (cut-off value for seroprotection). For pneumococcus, in 2-5 year-old patients, pre-vaccination geometric mean concentrations (GMCs) of IgG for six serotypes (4, 6B, 9V, 14, 18C, and 23F) were below $0.35{\mu}g/mL$ and at 5 months post-vaccination GMCs of IgG for all seven serotypes increased to above $0.35{\mu}g/mL$. In patients older than 5 years, pre-vaccination GMCs of IgG for four serotypes (4, 9V, 14, and 23F) were below $0.35{\mu}g/mL$ and at 3 months post-vaccination GMCs of IgG for all seven serotypes increased to above $0.35{\mu}g/mL$. Conclusion: Most HCT recipients had low or no protective antibodies to Hib and Sp before vaccination, but showed good immune responses to protective levels after vaccination.