• Pediatric Infection and Vaccine
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• v.26 no.3
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• pp.179-187
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• 2019

Purpose: Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is a leading cause of periodic fever in children. This study describes the clinical characteristics of PFAPA syndrome in patients from a single center. Methods: Thirteen children diagnosed with PFAPA syndrome at Seoul National University Children's Hospital were included in this study. Retrospective medical chart reviews were performed. Results: Among the 13 patients, 8 (61.5%) were male. The median follow-up duration was 3.3 years (range, 10 months-8.3 years). The median age of periodic fever onset was 3 years (range, 1-6 years). All patients had at least 5 episodes of periodic fever and pharyngitis, managed with oral antibiotics, before diagnosis. The median occurrence of fever was every 3.9 weeks and lasted for 4.2 days. All patients had pharyngitis and 12 (92.3%) had cervical lymphadenitis. Blood tests were performed for 12 patients, and no patients had neutropenia. Both the C-reactive protein and erythrocyte sedimentation rate were elevated at medians of 4.5 mg/dL (range, 0.4-13.2 mg/dL) and 29 mm/hr (range, 16-49 mm/hr), respectively. Throat swab cultures and rapid streptococcal antigen tests were negative. Nine (69.2%) patients received oral prednisolone at a median dose of 0.8 mg/kg, and in 6 (66.7%) patients, fever resolved within a few hours. Three (23.1%) patients received tonsillectomy and adenoidectomy. Conclusions: PFAPA syndrome should be considered when a child presents with periodic fever along with aphthous stomatitis, pharyngitis, or cervical lymphadenitis. Glucocorticoid administration is effective for fever resolution and can reduce unnecessary use of antibiotics.

• Pediatric Infection and Vaccine
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• v.14 no.1
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• pp.43-46
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• 2007

Purpose : Neonatal bacterial meningitis has been known that its mortality and morbidity is high and its neurologic complications are common. This study was performed to review of the etiology of neonatal bacterial meningitis in Busan, Korea. Methods : We retrospectively analyzed 21 medical records of patients who were diagnosed neonatal bacterial meningitis in four university hospitals in Busan from January 1997 to December 2005. We analyzed age at onset of symptoms, symptoms at admission, causative organisms, and complications. Results : There were 11 male patients and 10 female patients. One premature neonate was included. 7 patients had symptoms at 2-3 weeks after birth and 6 patients at 1-2 weeks after birth. 18 cases had fever, 8 cases had seizure and some had poor feeding, lethargy and irritability. The most common organism causing neonatal bacterial meningitis was Group B streptococcus (GBS). 11 cases were due to GBS, 3 cases due to E. coli, 3 cases were due to Klebsiella pneumoniae, and 2 cases due to Streptococcus pneumoniae. one Listeria monocytogenes and one Enterobacter cloacae were included. 18 patients were alive and 3 patients died. Mental retardation, developmental delay and epilepsy occurred in a living patient. Conclusions : Although this was a retrospective study, we found that Group B streptococcus (GBS) was the most common organism causing neonatal bacterial meningitis and this data is nearly same as others in the world.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.5 no.1
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• pp.33-43
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• 2005

Purpose: X-linked adrenoleukodystrophy(ALD) is a rare disorder that shows a great deal of phenotypic variability. We subdivided chidhood X-linked ALD patients into several phenotypes by the age at onset, the sites of most severe clinical involvement and the rate of progression of neurologic symptoms. Methods: Thirteen patients who had been diagnosed as X-linked ALD and followed up for at least one year were enrolled from 1996 to 2003. Results: 1. Ten had childhood cerebral ALD, who showed first neurologic symptoms at 7.02 years and progressed rapidly: interval between first symptoms and vegetative state was 1.35 years, and interval from initial symptoms to death was 3.35 years. Treatment with Lorenzo's oil did not prevent neurologic progression. Two patients who underwent umbilical cord blood transplantation died. 2. Two had adolescent cerebral ALD. They had first symptoms at 11.5 years, and showed tendency to progress less rapidly than childhood cerebral form patients. 3. One "Addison only" patient who had adrenal insufficiency without nervous system involvement remained asymptomatic during Lorenzo's oil treatment. 4. All cerebral form patients except one showed the lesions in both parieto-ocipital white matter in brain magnetic resonance imaging. Conclusion: The cerebral ALD was the most common form in childhood and was asoociated with a serious prognosis.

• Journal of Chest Surgery
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• v.37 no.9
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• pp.787-792
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• 2004

Even though there well developments in various treatment techniques for acute limb ischemia, this disease is both life threatening and limb threatening. We investigated early and mid-term results of operation for acute limb ischemia with symptoms, the combined diseases, location of occlusion, complication in our patients. Material and Method: A retrospective review was conducted in 54 patients (43 men, 11 women, mean age 67.2 years) presenting with acute limb ischemia due to arterial thrombosis or embolism between Jan. 1996 and Dec. 2003, initially underwent thromboembolectomy. Result: In 33 patients (61.1%) the timeinterval from the onset of symptom to admission was within 24 hours. Causes of acute limb ischemia were embolic occlusion (27.8%), native arterial thrombosis (66.7%), and bypass graft thrombosis (5.6%). The distribution of arterial occlusion location was at 8 aortoiliac (14.8%) and 43 distal to femoral (79.6%) and brachial (5.6%). Clinical categories were grade I in 64.8%, IIa in 24.1%, IIb in 7.4%, and III in 3.7%, All the patients were received embolectomy. Underlying diseases were heart disease (72.2%), hypertension (33.3%), cerebrovascular accident (16.7%) and diabetes (18.5%). History of smoking was noted in 96,3% of the cases. Mortality rate was 5.6％ and overall amputation rate was 9.3% (5/54). The 1-year limb salvage rate was 93.62%. Postoperative complications were 1 wound infection, 1 G1 bleeding, 3 acute renal failure, and 1 compartment syndromes. The functional outcomes of the salvaged limb according to the recommended scale for gauging changes in clinical status, revised version in 1997 were +3 in 68.5%, +2 in 9.3%, +1 in 7.4%, -1 in 5.6%, -2 in 3.7%, and -3 in 5.6%. Conclusion: This study revealed 5.6% mortality and the amputation rate was 9.3%. We have retrospectively shown good results from early diagnosis & early operation. To improve outcome, early diagnosis and understand the underlying diseases, prompt treatment and operation would be appreciated.

• Tuberculosis and Respiratory Diseases
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• v.44 no.5
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• pp.1105-1113
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• 1997

Background : Prone position improves oxygenation in some patients with ARDS. According to some authors, prone position can also improve the deteriorated hemodynamics induced by PEEP. But these respiratory and hemodynamic effects of prone position has not yet been fully established. Methods : Twentythree consequtive patients with ARDS(M : F= 11 : 12, $62.1{\pm}20.8yrs$) were the subjects for this study. ABGA, static compliance of the respiratory system, mean arterial pressure and pulse rate were obtained in supine position and at 5min, 0.5h and 2h of prone position. Positive respiratory response was defined as 20mmHg or more increase in $PaO_2/FIO_2$ within 2h of prone position. Early of late respiratory responses were defined if the positive response was observed within of after 3 day of ARDS onset, respectively. Positive hemodynamic response was defined as 10mmHg or more increase in mean arterial pressure at 5min of prone position. Results : Fifteen patients (65%) showed positive respiratory response. In the respiratory responders, $PaO_2$ was $69.8{\pm}17.6mmHg$ in supine position, $83.2{\pm}22.6mmHg$ in prone position 0.5h, $96.8{\pm}22.7mmHg$ in prone position 2h(p<0.001), and $PaO_2/FIO_2$ was $108{\pm}41mmHg$, $137{\pm}57mmHg$, $158{\pm}50mmHg$, respectively(p=0.001). Age, sex, cause of ARDS, supine $PaO_2$ and $PaO_2/FIO_2$ were not different between the respiratory responders and the nonresponders. The respiratory responders, however, showed higher mean arterial pressure than the nonresponders($91.1{\pm}13.1mmHg$ vs. $76.0{\pm}18.7mmHg$, p=0.035), and tendency of higher survival rate(9/15 vs. 2/8, p=0.074). Static compliance of the respiratory system was decreased in prone position 0.5h($28.4{\pm}7.9ml/cm$ $H_2O$ vs. $23.8{\pm}7.6ml/cm$ $H_2O$, p=0.007). The overall rate of early response(n=23) and late response(n=11) were similar(14/23 vs. 7/11, p>0.05). But patient without early response showed late response only in 25%(1/4), while patient with early response showed late response in 85.7%(6/7)(p=0.072). Five patients(22%) showed positive hemodynamic response, two of them being respiratory nonresponders. There were no differences in the baseline mean arterial pressure or the level of PEEP applied in supine between the hemodynamic responders and the hemodynamic nonresponders. Conclusions : Prone position either improved oxygenation or increased arterial pressure in significant proportion of patients with ARDS. And the respiratory response to prone position was thought to be determined in the early stage of ARDS.

• Tuberculosis and Respiratory Diseases
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• v.54 no.1
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• pp.33-44
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• 2003

Background : Mycobacterium avium complex(MAC) is the most common respiratory pathogen in nontuberculous mycobacterial pulmonary disease. This study described the clinical characteristics of the patients with pulmonary disease caused by MAC. Materials and Methods : The clinical characteristics of 24 patients with pulmonary disease caused by the MAC, who fulfilled the 1997 American Thoracic Society diagnostic criteria for nontuberculous mycobacterial pulmonary disease, were retrospectively analyzed. Results : Fourteen patients(58%) were male and the median age at diagnosis was 61 years(range 46-75). Of the 24 patients, 16(67%) had a M. intracellulare infection, 7(29%) had a M. avium infection and one patient was not identified. Coughing (92%) and sputum (88%) were most frequently observed symptoms. The sputum smear for acid-fast bacilli was positive in 17(71%) patients. Fourteen(58%) patients had the upper lobe cavitary form and 10(42%) patients had the nodular bronchiectatic form. In a comparison between the patients with the upper lobe cavitary form and those with the nodular bronchiectatic form, significant differences were found according to sex(male 86% vs. 20%, p=0.003), smoking history(79% vs. 10%, p=0.008), the presence of an underlying disease(64% vs. 20%, p=0.036), the pulmonary function(% forced vital capacity, median 71% vs. 88%, p=0.022; % forced expiratory volume in one second, median 69% vs. 89%, p=0.051) and bilateral disease at chest radiography(29% vs. 90%, p=0.005). The time from the onset of symptoms to diagnosis was longer in those with the nodular bronchiectatic form(median 72 months, range 8-132) than those with the upper lobe cavitary form(median 22 months, range 6-60) Conclusions : MAC pulmonary disease occurs in two distinct populations with two distinct clinical presentations. For a correct diagnosis of MAC pulmonary disease, knowledge of the diverse clinical and radiological findings is essential.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.10 no.2
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• pp.145-157
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• 1999

This study was performed by the children and adolescents who were abused or neglected physically, emotionally that were selected in child & adolescents psychiatric ward. We investigated the number of these case in admitted children & adolescents, and also observed characteristics of symptoms, developmental history, characteristics of abuse style, characteristics of abusers, family dynamics and psychopathology. We hypothesized that all kinds of abuse will influnced to emotional, behavioral problems, developmental courses on victims, interactive effects on family dynamics and psychopathology. That subjects were 22 persons of victims who be determined by clinical observation and clinical note. The results of the study were as follows：1) Demographic characteristics of victims：ratio of sex was 1：6.3(male：female), mean age was $11.1{\pm}2.5$. According to birth order, lst was 12(54.5%), 2nd was 5(23%), 3rd was 2(9%) and only child was 3(13.5%). 2) Characteristics of family：According to socioeconomic status, middle to high class was 3(13.5%), middle one was 9(41.% ), middle to low one was 9(41%), low one was 1(0.5%). according to number of family, under the 3 person was 3(13.5%), 4-5 was 17(77.5%), 6-7 was 2(9%). according to marital status of parents, divorce or seperation were 5(23%), remarriage 2(9%), severe marital discord was 19(86.5%). In father, antisocial behavior was 7(32%), alcohol dependence was 10(45.5%). In mother, alcohol abuse was 5(23%), depression was 17(77.3%), history of psychiatric management was 6(27%). 3) Characteristics of abuse：Physical abuse was 18(81.8%), physical and emotional abuse and neglect were 4(18.2%). according to onset of abuse, before 3 years was 15(54.5%), 3-6 years was 5(27.5%), schooler was 1(15%). Only father offender was 2(19%), only mother offender was 8(35.4%), both offender was 8(35.4%), accompaning with spouse abuse was 7(27%), and accompaning with other sibling abuse was 4(18.2%). 4) General characteristics and developmental history of victims：Unwanted baby was 12(54.5%), developmental delay before abuse was9(41%), comorbid developmental disorder was 15(68%). there were 6(27.5%) who didn‘t show definite sign of developmental delay before abuse. 5) Main diagnosis and comorbid diagnosis：According to main diagnosis, conduct disorder 6(27.3%), borderline child 5(23%), depression4(18%), attention deficit hyperactivity disorder(ADHD) 4(18%), pervasive developmental disorder not otherwise specified 2(9%), selective mutism 1(5%). According to comorbid diagnosis, ADHD, borderline intelligence, mental retardation, learning disorder, developmental language disorder, oppositional defiant disorder, chronic tic disorder, functional enuresis and encoporesis, anxiety disorder, dissociative disorder, personality disorder due to medical condition. 5) Course of treatment：A mean duration of admission was $2.4{\pm}1.5$ months. 11(15%) showed improvement of symtoms, however 11(50%) was not changed of symtoms.

• Childhood Kidney Diseases
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• v.3 no.2
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• pp.123-129
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• 1999

Purpose: Acute poststreptococcal glomerulonephritis(APSGN) is a renal disease which is characterized by glomerular proliferation and inflammatory changes due to immune reaction. Although the 95% of patients with APSGN seems to recover fully and present as benign course, the remaining patients show poor prognosis. Therefore comparative retrograde study between APSGN with and without nephrotic syndrome was done to find out the any prognostic indicator to predict the outcome in patients with APSGN. Methods: We had retrospectively analyzed seventy-one patients who were diagnosed as APSGN clinically from Mar.1989 to Feb.1999 in Yonsei university medical center. Sixty-four of the patients was APSGN without nephrotic syndrome(Group A) and seven patients were in APSGN with nephrotic syndrome(Group B). Results: Patients who were diagnosed as APSGN with nephrotic syndrome were seven(9.9%) out of seventy-one. In the comparative study, sex ratio was 1:1 in group A and 1.9: 1 in group B, onset mean age was $8.9{\pm}2.6$ in group A and $8.8{\pm}2.6$ in group B. Following clinical profiles were compared but there were no significant difference between these two groups: WBC count($9413{\pm}2964\;vs\;9368{\pm}2650(/mm^3)$), hemoglobin($10.6{\pm}1.2\;vs\;10.0{\pm}0.9(gm/dL)$), ASO($746.1{\pm}640.7\;vs\;614.9{\pm}475.9(IU/ml)$), $C_3(20.1{\pm}17.0\;vs\;16.9{\pm}13.1(mg/dL)$), $C_4(22.8{\pm}9.5\;vs\;22.6{\pm}6.9(mg/dL)$), BUN($25.8{\pm}26.1\;vs\;28.1{\pm}14.5(mg/dL)$), creatinin($0.8{\pm}0.3\;vs\;0.8{\pm}0.3(mg/dL)$), $C_{cr}(80.6{\pm}28.8{\pm}62.4{\pm}31.4(ml/min/1.73\;m^2$)), the duration of edma, gross hematuria, and hypertension. However, we found that there were a significant difference in the duration of proteinuria($1.95{\pm}2.27\;vs\;13.3{\pm}21.1(months)$)(P<0.05), decreased $C_3$ duration($1.9{\pm}2.9\;vs\;7.3{\pm}5.0(weeks)$)(P<0.05) and especially it was proloned according to the amount of early urine protein excretion. Conclusion: Our study showed markedly prolonged duration of proteinuria and decreased $C_3$ duration in patients with APSGN with nephrotic syndrome. We were not able to find the definite prognostic factor that will guide the outcome of patients with APSGN accompaning nephrotic syndrome, but above findings seemed to represent as a relative indication of the outcome of the disease. All patients recovered completely and we did not experience any cases that progressed into the renal failure.

• Radiation Oncology Journal
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• v.22 no.1
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• pp.17-24
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• 2004

Purpose: To evaluate the treatment outcomes after postmastectomy radiotherapy (PMRT) and chemotherapy in patients with breast cancer. Materials and Methods: The PMRT were retrospectively analyzed in 83 patients with stage II-III female breast cancer treated between 1989 and 1995. The median age was 46 years (range, 23-77): Seventy-seven patients had modified radical mastectomies, 5 radical mastectomies and 1 simple mastectomy. Three patients ($4\%$) had pathologically negative axillae, and the remaining 80 ($96\%$) had positive axillae. Eleven, 23, 44 and 5 patients had pathological stages IIA, IIB, IIIA, and IIIB, retrospectively. Eighty ($96\%$) patients were treated with hockey-stick fields. The median dose of PMRT was 50.4 ey, in 1.8 Gy fractions. Adjuvant systemic chemotherapy was given to 74 patients ($89\%$). CMF-based or doxorubicin-containing regimens were given to 54 patients ($55\%$). The median follow-up time was 82 months (range, 8-171) after the mastectomy. Results: The 5 and 10-year overall survival rates for all patients were 65 and $49\%$, respectively. The univariate and multivariate analyses of the factors affecting the overall survival revealed the stage to be the most significant prognostic factor (p=0.002), followed by the combination of chemotherapy. Thirteen patients $16\%$ developed a LRF, at an interval of 4-84 months after radiotherapy, with a median of 20 months. The only significant prognostic factor affecting LRF was the combination of chemotherapy, in both the univariate and multivariate analyses. With respect to the sequence of chemoradiation, the sequence had no saatistical significance (p=0.90). According to the time interval from mastectomy to the onset of radiotherapy, the LRFR of the patients group treated by RT within or after 6 month postmastectomy 6 months were 14 vs. $27\%$ respectively (p=0.24). One third of the pa41en1s (26/83) developed distant metastasis, in 2-92 months, after radiotherapy, with a median of 21 months. The most commonly involved site was bone in 13 cases. The pathological staging was the only significant prognostic factor in both the univariate and multivariate analyses that affected distant failure. Radiological finding of radiation pneumonitis on a simple chest x-ray was shown in $20\%$ (17/83), with a time interval ranging from 2 to 7 months post-radiotherapy, with a median of 3 months. The stable lung fibrosis settled in 11 patients ($65\%$). Conclusion: It was concluded through this analysis that the combination of PMRT with in chemotherapy resulted in better overall survival and local control than PMRT alone in patients needing PMRT.

• Tuberculosis and Respiratory Diseases
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• v.39 no.6
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• pp.453-473
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• 1992

Background: National survey was performed to estimate the incidence of sarcoidosis in Korea. The clinical data of confirmed cases were analysed for the practice of primary care physicians and pulmonary specialists. Methods: The period of study was from January 1991 to December 1992. Data were retrospectively collected by correspondence with physicians in departments of internal medicine, dermatology, ophthalmology and neurology of the hospitals having more than 100 beds using returning postcards. In confirmed and suspicious cases of sardoidosis, case record chart for clinical and laboratory findings were obtained in detail. Results: 1) Postcards were sent to 523 departments in 213 hospitals. Internal medicine composed 41%, dermatology 20%, ophthalmology 20% and neurology 19%. 2) Postcards were returned from 241 departments (replying rates was 48%). 3) There were 113 confirmed cases from 50 departments and 10 cases. The cases were composed from internal medicine (81%), dermatology (13%), ophthalmology (3%) and neurology (3%). 78 confirmed cases were analysed, which were composed from department of internal medicine (92%), dermatology (5%), and neurology (3%). 4) The time span for analysed cases was 1980 to 1992. one case was analysed in 1980 and the number gradually increased to 18 cases in 1991. 5) The majority of patients (84.4%) were in the age group of 20 to 49 years. 6) The ratio of male to female was 1 : 1.5. 7) The most common chief complains were respiratory symptoms, dermatologic symptoms, generalized discomforts, visual changes, arthralgia, abdominal pains, and swallowing difficulties in order. 16% of the patients were asymptomatic. 8) Mean duration between symptom onset and diagnosis was 2 months. 9) The most common symptoms were respiratory, general, dermatologic, ophthalmologic, neurologic and cardiac origin in order. 10) Hemoglobin, hematocrits and platelet were in normal range. 58% of the patients had lymphopenia measuring less than 30% of white cell count. The ratio of CD4 to CD8 lymphocytes was $1.73{\pm}1.16$ with range of 0.43 to 4.62. ESR was elevated in 43% of the cases. 11) Blood chemistry was normal in most cases. Serum angiotensin converting enzyme (S-ACE) was $66.8{\pm}58.6\;U/L$ with the range of 8.79 to 265 U /L. Proteinuria of more than 150 mg was found in 42. 9% of the patients. 12) Serum IgG was elevated in 43.5%, IgA in 45.5%, IgM in 59.1% and IgE in 46.7%. The levels of complement C3 and C4 were in the normal range. Anti-nuclear antibody was detected in 11% of the cases. Kweim test was performed in 3 cases, and in all cases the result was positive. 13) FVC was decreased in 17.3%, FEV1 in 11.5%, FEV1/FVC in 10%, TLC in 15.2%, and DLco in 64.7%. 14) PaO2 was decreased below 90 mmHg in 48.6% and PaCO2 was increased above 45 mmHg in 5.7%. 15) The percentage of macrophages in BAL fluid was $51.4{\pm}19.2%$, lymphocytes $44.4{\pm}21.1%$, and the ratio of CD4 to CD8 lymphocytes was $3.41{\pm}2.07$. 16) There was no difference in laboratory findings between male and female. 17) Hilar enlargement on chest PA was present in 87.9% (bilaterally in 78.8% and unilaterally in 9.1%). 18) According to Siltzbach's classification, stage 0 was 5%, stage 158.3%, stage 228.3%, and stage 38.3%. 19) Hilart enlargement on chest CT was present in 92.6% (bilaterally 76.4% and unilaterally in 16.2%). 20) HRCT was done in 16 cases. The most common findings were nodules, interlobular thickening, focal patchy infiltrations in order. Two cases was normal finding. 21) Other radiologic examinations showed bone change in one case and splenomegaly in two cases. 22) Gallium scan was done in 12 cases. Radioactivity was increased in hilar and mediastinal lymph nodes in 8 cases and in parenchyme in 2 cases. 23) The pathologic diagnosis was commonly performed by transbrochial lung biopsy (TBLB, 47.3%), skin and mediastinal lymph nodes biopsy (34.5%), peripheral lymph nodes biopsy (23.6%), open lung biopsy (18.2%) and bronchial biopsy in order. 24) The most common findings in pathology were non·caseating granuloma (100%), multi-nucleated giant cell (47.3%), hyalinized acellular scar (34.5%), reticulin fibrin network (20%), inclusion body (10.9%), necrosis (9.1%), and lymphangitic distribution of granuloma (1.8%) in order. Conclusion: Clinical, laboratory, radiologic and pathologic findings were summarized. This collected data will assist in finding a test for detection and staging of sarcoidosis in Korea in near future.