• Tuberculosis and Respiratory Diseases
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• v.43 no.6
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• pp.1035-1041
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• 1996

Klinefelter's syndrome is characterized by small testes, azoospermia, gynecomastia, and elevated levels of plasma gonadotropins in men with two or more X chromosomes. Previous investigators reponed that patients with Klinefelter's syndrome are predisposed to the development of a non-seminomatous germ cell tumor in the mediastinum. It is suggested that this linkage may be due to the hormonal imbalance in Klinefelter's syndrome and consequently, the formation of dysgenetic germ cell and/or abnomal migration of germ cell We report here a case of Klinefelter's syndrome in a 24-years-old man who was presented with anterior mediastinal mass. The clinical and laborarotory fmdings were compatible with Klinefelter's syndrome and he was found to have 47 XXY karyotype. Pathological findings for mediastinal mass revealed mixed germ cell tumor composed of mature cystic teraloma and endodermal sinus rumor. He was treated with cis platin containing chemotherapy and followed up in partial remission.

• Journal of Gastric Cancer
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• v.19 no.4
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• pp.408-416
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• 2019

Purpose: To study the efficacy of capecitabine or S-1 plus oxaliplatin (CAPOX or SOX) for treating thymidine phosphorylase (TP)- or dihydropyrimidine dehydrogenase (DPD)-positive advanced gastric cancer. Materials and Methods: Eighty-six patients with stage IIIC to IV gastric cancer were assessed for TP and DPD expression by immunohistochemistry. The association between CAPOX or SOX efficacy and TP/DPD expression was retrospectively analyzed. Results: There were no significant differences in the objective remission rate (ORR, 52.27% vs. 47.62%; P>0.05), disease control rate (72.73% vs. 73.81%, P>0.05), progression-free survival (hazard ratio [HR], 1.119; 95% confidence interval [CI], 0.739-1.741; P=0.586), and overall survival (OS; HR, 0.855; 95% CI, 0.481-1.511; P=0.588) between CAPOX and SOX. A higher number of stage IV patients showed TP positivity, while DPD-positive patients predominantly showed intestinal type of gastric cancer. In TP-positive patients, the ORRs associated with CAPOX and SOX treatments were 57.14% and 38.10%, respectively; OS was better with CAPOX than with SOX (HR, 0.447; 95% CI, 0.179-0.978; P=0.046). Among DPD-positive patients, the SOX treatment-associated ORR (60.87%) was significantly higher than the CAPOX treatment-associated ORR (43.48%). Furthermore, SOX treatment resulted in better OS than did CAPOX treatment (HR, 2.020; 95% CI, 1.019-4.837; P=0.049). Conclusions: No significant difference in clinical efficacy was found between CAPOX and SOX. TP-positive patients might respond better to CAPOX while DPD-positive patients may respond better to SOX. Our findings might serve as a guide for personalized chemotherapy for gastric cancer.

• Pediatric Infection and Vaccine
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• v.13 no.2
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• pp.137-146
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• 2006

Purpose : A regional lymphadenitis is the most frequent adverse reaction of BCG. In order to find out developmental factors and establish a strategy of management, we investigated the clinical courses of children with lymphadenitis following BCG on the aspect of BCG strains, suppurative rates according to the sizes of lymph node and the clinical difference with or without treatment. Methods : From January 1997 to June 2004, 52 children less than 24 month-age-old diagnosed as BCG lymphadenitis in Department of Pediatrics, St. Vincent's Hospital, The Catholic University of Korea were enrolled. The type of BCG strain, place of vaccination, location and size of lymphadenitis were assessed with medical records, retrospectively. Finally, we analysed the correlations between BCG strains or the sizes of lymph node and natural remission or suppuration. Results : The first detected mean age of BCG lymphadenitis was 5.5 month-age. The larger of the measurement was at the first visiting, the younger of age that was first presented. The most frequent location was the same sided axillary region of BCG injection. Among 52 subjects, 46 cases(88.5%) were vaccinated with intradermal Pastuer strain, and only 5 cases(9.6%) were done with percutaneous multipunctured Tokyo strain. Twenty eight cases(53.8%) were regressed naturally, otherwise 24 cases(46.2%) were suppurated. The larger those were sized, the higher freqeuncies those were suppurated on, significantly. Treatment with medications could not prevent the suppuration and could not shorten the healing periods. Conclusion : We predict that there are differences between the occurrent rate of BCG lymphadenitis and BCG strains or methods. Treatment with medication is not recommended owing to its ineffectiveness. Especially, in case of non-suppurative lymphadenitis should be onlyless influence on the tuberculin skin test, cause less adverse reactions, and is inexpensive. observed without treatment, because it could be regressed naturally. An ideal BCG makes a scar, We should make an effort to choose the best BCG strain that can fulfill such requirements.

• Tuberculosis and Respiratory Diseases
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• v.45 no.1
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• pp.116-127
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• 1998

Background: The natural course of sarcoidosis is variable from spontaneous remission to significant morbidity or death. So the assessment of disease activity is important but no single parameter was generally accepted as a good marker. Recently several studies suggested that adhesion molecules, especially ICAM-1 can be a marker, but there are some controversies. And only few data are available about the relationship of ICAM-1 with clinical follow-up course. Methods: We measured the expression of adhesion molecules on BAL cells by flow cytometry and the level of soluble ICAM-1(sICAM-1) in serum and BALF at the time of diagnosis in 12 patients with active disease and 7 inactive sarcoidosis(5 male, 14 female, mean age: $39.4{\pm}10.7$ years, mean follow-up : $20{\pm}15$ months). Follow-up clinical course were compared with the changes in serum sICAMA-1 level and the adhesion molecule on BAL cells. Results: In the patients with active disease, the ICAM-1 on AM(RMFI: $3.68{\pm}1.71$) and sICAM-1 level in serum($582{\pm}193$ng/ml) and BAL fluid($47.8{\pm}16.5$ng/ml) were all higher than those of 7 inactive disease(RMFI: $1.89{\pm}0.75$, p=0.0298, serum: $294{\pm}117$ ng/ml, p=0.0049, BALF: $20.9{\pm}8.3$ ng/ml). In the active sarcoidosis, ICAM-1 on AM(RMFI : $1.51{\pm}0.84$) and serum sICAM-1 were decreased after the therapy($250{\pm}147$ ng/ml) but no significant change was noted in inactive disease. Also we found the initial ICAM-1 on AM and serum sICAM-1 had a significant correlation with the degree of improvement in PFT after the therapy. During the follow-up, the disease relapsed in 4 patients after the discontinuation of steroid and the serum sICAM-1 level went-up again at the time of relapse. Conclusion: Our data suggest that the serum sICAM-1 level and the ICAM-1 expression on AM can be a good marker of disease activity and also a predictor of outcome in sarcoidosis.

• The Korean Journal of Nuclear Medicine
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• v.33 no.4
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• pp.422-429
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• 1999

Purpose: The measurement of radiation absorbed dose is useful to predict the response after I-131 labeled metaiodobenzylguanidine (MIBG) therapy and determine therapy dose in patients with unresectable or malignant pheochromocytoma. We estimated the absorbed dose in tumor tissue after high dose I-131 MIBG in a patient with pheochromocytoma using a gamma camera and Medical Internal Radiation Dose (MIRD) formula. Materials and Methods: A 64-year old female patient with pheochromocytoma who had multiple metastases of mediastinum, right kidney and periaortic lymph nodes, received 74 GBq (200 mCi) of I-131 MIBG. We obtained anterior and posterior images at 0.5, 16, 24, 64 and 145 hours after treatment. Two standard sources of 37 and 74 MBq of I-131 were imaged simultaneously. Cummulated I-131 MIBG uptake in tumor tissue was calculated after the correction of background activity, attenuation, system sensitivity and count loss at a high count rate. Results: The calculated absorbed radiation dose was 32-63 Gy/ 74 GBq, which was lower than the known dose for tumor remission (150-200 Gy). follow-up studies at 1 month showed minimally reduced tumor size on computed tomography, and mildly reduced I-131 MIBG uptake. Conclusion: We estimated radiation absorbed dose after therapeutic I-131 MIBG using a gamma camera and MIRD formula, which can be peformed in a clinical nuclear medicine laboratory. Our results suggest that the measurement of radiation absorbed dose in I-131 MIBG therapy is feasible as a routine clinical practice that can guide further treatment plan. The accuracy of dose measurement and correlation with clinical outcome should be evaluated further.

• Radiation Oncology Journal
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• v.20 no.1
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• pp.17-23
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• 2002

Purpose : To clarify the clinical benefit derived from the combined modality therapy (CMT) consisting of chemotherapy (CT) and involved field radiotherapy (RT) for stage I and II angiocentric lymphomas of the head and neck. Materials and Methods : Of 143 patients with angiocentric lymphoma of the head and neck treated at our hospital between 1976 and 1995, 104 patients (RT group) received involved field RT alone with a median dose of 50.4 Gy (range : 20~70 Gy), while 39 patients (CMT group) received a median 3 cycles (range : 1~6 cycles) of CT before involved field RT. The response rate, patterns of failure, complications, and survival data of the RT group were compared with those of the CMT group. Results : Despite a higher response rate, local failure was the most common pattern of failure in patients of both groups. The patterns of failure, including the systemic relapse rate were not influenced by the addition of combination CT. Although both modalities were well tolerated by the majority of patients, aberrant immunologic disorders or medical illnesses, such as a hemophagocytic syndrome, sepsis, intractable hemorrhage, or the evolution of second primary malignancies were more frequently observed in patients of the CMT group. The prognosis of patients in the RT group was relatively poor, with a 5-year overall actuarial survival rate of 38% and disease-free survival rate of 32%, respectively. However, their clinical outcome was not altered by the addition of systemic CT. Achieving complete remission was the most important prognostic factor by univariate and multivariate analyses, but treatment modality was not found to be a prognostic variable influencing survival. Conclusions : Involved field RT alone for angiocentric lymphoma of the head and neck was insufficient to achieve an improved survival rate, but the addition of CT to involved field RT failed to demonstrate any therapeutic advantage over involved field RT alone.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.161-169
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• 2009

Purpose : This study was performed to report the diagnosis and treatment of nephrotic syndrome manifesting in the first year of life. Methods : We retrospectively reviewed the clinical data with chart review in 7 patients who were diagnosed as nephrotic syndrome manifesting in the first year of life from 1996 to 2007. Results : Three patients had congenital nephrotic syndrome, the other 4 patients had infantile nephrotic syndrome. Their ages ranged from birth to 11 months and male to female ratio was 1 to 6. Renal biopsies were done in 6 patients. One patient had Finnish type congenital nephrotic syndrome, 2 patients had diffuse mesangial sclerosis, 2 patients had focal segmental glomerulosclerosis and 1 patient had minimal change disease. Genetic analyses of NPHS2, PLCE1, and WT1 were done in 4 patients and 2 of them had WT1 mutation. Among 3 patients with congenital nephrotic syndrome, 1 patient was diagnosed as congenital nephrotic syndrome of Finnish type and the other 2 patients were diagnosed as Denys-Drash syndrome. All of the patients with congenital nephrotic syndrome died due to sepsis. Among 4 patients with infantile nephrotic syndrome, 2 patients died and 1 had remission, another patient progressed to end stage renal disease. Conclusion : Most of nephrotic syndrome manifesting in the first year was hereditary renal disease. Patients with nephrotic syndrome manifesting in the 3 month of life had poorer prognosis and needed more aggressive management including early dialysis and renal transplantation might be considered compared with infantile nephrotic syndrome. Further genotype-phenotype correlation studies are needed.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.15 no.2
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• pp.143-151
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• 2004

Objectives : Child and adolescent onset obsessive compulsive disorder(C-OCD) is known to be associated with poor drug response, high comorbid rate and strong genetic tendencies. Till now studies for C-OCD have been very rare in Korea. We conducted this study to investigate the informations about clinical features, familial psychiatric loading, treatment profiles and course of server C-OCD from the retrospective analysis of inpatient data of Seoul National University Children's Hospital. Method : Retrospective chart review and data analysis was performed. Twenty(male 16 : female 4) patients with final C-OCD diagnosis by DSM-IV at discharge from 1994 to 2002 were found and their medical chart, psychological data, family interview data and nursing reports were collected and analyzed. Results : 1) The sex ratio of C-OCD was male dominant(4:1). 2) Phenomenological, most common obsession was pathologic doubt, contamination fear, followed by aggressive obsession, need for symmetry, sexual obsession, most common compulsion was checking and washing, followed by breathing, movement, symmetry, repetitive asking, hoarding, mental compulsion. 3) Most common comorbid diagnosis was depression. Other axis-I diagnosis associated OCD were anxiety disorder, tic disorder, conduct and oppositional defiant disorder and psychosis. 4) Regarding psychiatric familial loadings, 17 patients(85%) had relatives with psychiatric disorders, OC-spectrum disorders(OCD or OCPD) were found in 9 patients(45%). 5) The majority of patients(75%) have received SSRI and antipsychotics treatment. The response rate above 'moderate improved" by CGI was 75%. 6) During follow-up period in outpatient clinic, five patients(25%) showed continuous complete remission, 10 patients (50%) did residual symptoms with chronic course. Conclusion : This seems to be the first systemic investigation of severe pediatric OCD patients in Korea. The children & adolescents with severe OCD in inpatient-setting showed the high comorbid rate, familial psychiatric loading, and combined pharmacotherapy with antipsychotics, As for symptoms, high rate of aggressive-sexual obsession and atypical compulsions like breathing and moving was reported in this study. Severe pediatric OCD patients, however, responded well to the combined SSRI and antipsychotics regimen.

• Childhood Kidney Diseases
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• v.2 no.2
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• pp.118-124
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• 1998

Purpose : There is no specific treatment guidelines for Henoch-$Sch{\ddot{o}}nlein$(HS) nephritis. Therefore we performed this study to observe the effect of long term steroid therapy combined with azathioprine Methods : Treatment protocols; 1) Steroid pulse therapy: methylprednisolon 30 mg/kg/dose, maximum 1 gm, intravenolisly 6 times for alternate day. 2) Oral steroid was given 2 mg/kg/day for 1 month, 1 mg/kg/day for following 1 month and alternate day oral steroid combined with azathioprine 2 mg/kg/day for 2 years. Results : Time period from HSP to onset of HS nephritis was between 2 weeks to 5 months with mean $7.4{\pm}7.4$ weeks. Clinical remission were seen in 4 cases out of 5 ($80\%$). Mean time period with disappearance of proteinuria and microscopic hematuria were $5{\pm}2.4$ month and $13.3{\pm}2.9$ month respectively. On pathologic findings by ISKDC, 3 cases were grade IIIb, 2 cases were grade IV in first kidney biopsies and showed pathologic improvement in follow up tidneybiopsiesafterlyearstreatment. Conclusion : As there is no definitive treatment for HS nephritis so far, our study of long term oral steroid therapy with azathioprine was effective in clinical and histologic aspect. Therefore further study in HS nephritis with in a large group will be needed in the future.

• Clinical and Experimental Pediatrics
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• v.50 no.2
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• pp.170-177
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• 2007

Purpose : Clinicopathological features were investigated to clarify the outcome and prognostic indicators for patients with IgA nephropathy in Korean children. Methods : We reviewed the outcomes of 61 patients in whom IgA nephropathy was diagnosed before the age of 15 years from 1991 to 2005 and followed-up at least for one year. All patients were confirmed by renal biopsy. Results : After mean follow-up of 5.2 years from onset, 24 patients of 61 (39.3%) were in clinical remission at the last examination. Thirty patients (49.2%) had hematuria or mild proteinuria (<$1g/m^2/d$), five (8.2%) had severe proteinuria (${\geq}1g/m^2/d$), and two (3.3%) had chronic renal failure. By univariate analysis, initial presentation at onset and Haas classification were less concordant with outcome. Hypertension during follow-up, rather than hypertension at presentation, was significantly correlated with outcomes (P<0.01). Sixty percent of patients who had more than 20% of glomerular sclerosis or crescent progressed to severe proteinuria or chronic renal failure, as compared with 7.1% of those who did not (P<0.01). Conclusion : Prognosis of childhood IgA nephropathy had a relatively benign course during a mean follow-up of 5.2 years. Persistent hypertension during follow-up and more than 20% of glomerular sclerosis or crescent were strong predictors of a progressive course of IgA nephropathy. A new histologic classification according to characteristics of childhood IgA nephropathy must be established to assess prognosis. Further efforts should be made to understand the prognosis of IgA nephropathy through long-term follow-up.