• Clinical and Experimental Pediatrics
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• v.48 no.2
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• pp.178-185
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• 2005

Purpose : The purpose of this study was to evaluate the outcome of children with juvenile myelomonocytic leukemia(JMML) treated with allogeneic hematopoietic stem cell transplantation(allo-HSCT). Methods : Eleven JMML patients aged 8-39 months underwent allo-HSCT. The sources of grafts were unrelated donors(n=7), HLA-matched siblings(n=3) and an HLA 1-antigen mismatched familial donor. All patients had received chemotherapy ${\pm}13$-cis-retinoic acid(CRA) before transplant, and CRA was used, posttransplant, in six patients. Results : Only three patients were in complete remission(CR) at the time of transplantation. Initial chimeric status revealed complete donor chimerism(CC) in five patients, mixed chimerism(MC) in five and autologous recovery(AR) in one. One patient with MC having persistent splenomegaly eventually turned to CC and CR after rapid tapering of cyclosporine, combined with daily use of CRA. An AR case relapsed shortly after transplant but was rescued with second, unrelated cord blood transplantation. Ultimately, six patients are alive, event-free, with a median follow-up of 15.5 months posttransplant. All three deaths occurred in patients who failed to achieve CC, leading to disease progression. Conclusion : We suggest that graft-versus-leukemia effect play an important role and CRA a possible role in posttransplant leukemic involution in JMML. In patients whose leukemic burden is still high with MC after transplant, early tapering of immunosuppressants and introduction of CRA might provide a chance of a cure for some patients.

• Childhood Kidney Diseases
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• v.4 no.2
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• pp.111-119
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• 2000

Purpose : Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP) is a common pediatric discase presenting most frequently with skin, gastrointestinal, joint and renal manifestations. The prognosis of HSP is mainly determined by the involvement of the kidney, but prognostic markers have not been established. We evaluated the patients who have HSP nephritis with nephrotic syndrome. Method : Clinical manifestations and laboratory findings were observed and analyzed in 34 cases with HSP which were manifested by nephrotic syndrome hospitalized at Kyung Hee university Hospital during the period from Jan. 1990 to Dec. 1998. Results : 1) Male to female ratio was 1.3:1, and mean age at onset was 8.3 year. 2) Mean duration from symptom onset to renal biopsy was 10.5 weeks. 3) Proportion of patients presenting with acute nephritis was 32.4$\%$, gross hematuria 17.6$\%$, microscopic hematuria 50$\%$. 4) The findings of renal biopsy were 20 cases of grade II, 11 cases of grade III, 2 cases of grade I, 1 case of grade IV according to classification by ISKDC. 5) Patients with grade I were recovered with no residual defect, but patients with grade IV shows active renal disease(states C). Conclusion : Among the 디le patients with Henoch-$Sch{\ddot{o}}nlein$ purpura accompanying nephrotic syndrome, more aggressive treatment might be needed in patients showing crescents formation on renal biopsy. A prospective study will be needed to explore the progression of this disease.

• The Korean Journal of Nuclear Medicine
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• v.30 no.4
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• pp.476-483
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• 1996

Following radiation therapy for brain tumors, patients often have clinical deterioration due to either radiation necrosis or recurrent tumor progression in the treatment field. The distinction between these entities is important but difficult clinically or even with CT or MRI. T1-201 has been known to accumulate in various tumors and be useful to grade, predict prognosis or detect recurrence of glioma. The aim of this study was to evaluate the usefulness of T1-201 SPECT in the differentiation of recurrent tumor from radiation necrosis. Of 67 patients who did T1-201 brain SPECT imaging with clinically suspected recurrent tumor or radiation necrosis, 20 patients underwent histopathological examination and constituted the study population. T1-201 uptake indices on T1-201 brain SPECT imaging rrere calculated and correlated with histopathological diagnosis. Of 20 patients, 15 were histopathologically confirmed as recurrent original tumor or malignant transformation of benign tumor and 5 were diagnosed as radiation necrosis. On T1-201 SPECT, 18 of 20 had T1-201 index above 2.5 which was regarded as positive indicator for the presence of tumor. Seventeen cases showed concordance, which consisted of 15 true positive and 2 true negative. Discordant 3 cases were all false positive. There was no case of false negative. The sensitivity, specificity, positive and negative predictive value of T1-201 SPECT were 100%, 40%, 83% and 100%. In conclusion, T1-201 brain SPECT is a sensitive diagnostic test in the detection of recurrent tumor following radiation therapy and is useful in the differentiation of recurrent tumor from radiation necrosis. Relatively low specificity should be evaluated further in larger number of patients in consideration of sampling error and referral bias for pathologic examination.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.27 no.5
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• pp.373-384
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• 2001

Cellular proliferation is an intricately regulated process mediated by the coordinated interactions of critical growth control genes. Two of these factors in mammalian cells are the p53 and mdm-2 genes. A protein product of the mem-2 oncogene has been recently shown to associate with the protein encoded by the tumor suppressor gene p53. The p53 tumor suppressor protein is stabilized in response to DNA damage and other stress signals and causes the cell to undergo growth arrest or apoptosis, thus preventing the establishment of mutations in future cellular generations. Mutation or loss of p53 is a very common event in tumor progression. It occurs in about 50% of all tumors analysed including of colon, lung, breast and liver. The cellular mdm-2 gene, which has potential transforming activity that can be activated by overexpression, is amplified in a significant percentage of human sarcoma and in other mammalian tumors. Proteins encoded by the mdm-2 gene are able to bind to the p53 protein and, when overexpressed, can inhibit p53's transcriptional activation function, thus mdm-2 can act as a negative regulator of p53 function. Experimental study was performed to observe the relationship between p53 gene mutation and mdm-2 protein expression and apply the results to the clinical activity. 36 golden syrian hamster each weighing $60{\sim}80g$ were used and painted with 0.5% DMBA by 3 times weekly on the right buccal cheek(experimental side) for 6, 8, 10, 12, 14 and 16 weeks. Left buccal cheek(control side) was treated with mineral oil as the same manner to the right side. The hamsters were sacrificed on the 6, 8, 10, 12, 14 & 16 weeks. Normal and tumor tissues from paraffin block were examined for histology and immunohistochemistry observation, and were completely dissected by microdissection and DNA from both tissue were isolated by proteins K/phenol/chloroform extraction. Segments of the hamster p53 exons 5, 6, 7 and 8 were amplified by PCR using the oligonucleotide primers, and then confirmational change was observed by SSCP respectively. The results were as follows : 1. Dysplasia at 6 weeks, carcinoma in situ at 8 weeks and invasive carcinoma from 10 weeks could be observed in experimental groups. 2. p53 mutations were detected in 10 of the 36(28%) and the exons 6(6 of the 10 : 60%) was the most hot spot area among the highy conserved region(exons 5, 6, 7 & 8). 3. Immunohistochemical study confirmed 22 of the 36(61%) of p53 expression involving 10 of p53 mutations. 4. mdm-2 expression of was showed in 3 of the 36(8%) involving 1 of the 22 of p53 expression and 2 of the 14 of p53 non-expression. From the above results, mutation of p53 gene or expression of p53 protein may have the influence of the DMBA induced carcinoma of hamster buccal pouch but the expression of mdm-2 protein may not have relationship with tumorigenesis.

• Radiation Oncology Journal
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• v.18 no.4
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• pp.293-299
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• 2000

Purpose :We conducted a prospective non-randomized clinical study to evaluate the efficacy and toxic of the preoperative concurrent chemoradiotherapy for locally advanced unresectable rectal cancer. Materials and Methods: Between January 1995 and June 1998, 37 conecutive patients with locally unresectable advanced rectal cancer were entered into the study. With 3- or 4- fields technique, a total of 45 Gy radiation was delivered on whole pelvis, followed by 5.4 Gy boost to the primary tumor in some cases. Chemotherapy was done at the first and fifth week of radiation with bolus i.v. 5-Fluorouracil (FU) 370$\~$450 mg/m$^{2}$, days 1$\~$5, plus Leucovorin 20 mg/m$^{2}$, days 1$\~$5. OF 37 patients, 6 patients did not receive all planned treatment course (refusal in 4, disease progression in 1, metastasis to lung in 1). Surgical resection was undergone 4$\~$6 weeks after preoperative concurrent chemoradiotherapy. Results :Complete resection rate with negative margins was 94$\%$ (29/31). Complete response was seen in 7 patients (23$\%$) clinically and 2 patients (6$\%$) pathologically. Down staging of tumor occured in 21 patients (68$\%$). Treatment related toxicity was minimal except grade III & IV leukopenia in 2 patients, respectively. Conclusion : Preoperative concurrent chemoradiotherapy in locally advanced rectal cancer was effective in inducing down staging and complete resection rate. Treatment related toxicity was minimal. Further follow up is on-going to determine long term survival following this treatment.

• Tuberculosis and Respiratory Diseases
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• v.46 no.3
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• pp.317-326
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• 1999

Background: Neural control of airway function is through parasympathetic, sympathetic and non-adrenergic, non-cholinergic mechanisms. The autonomic nervous system controls the airway smooth muscle tone, mucociliary system, permeability and blood flow in the bronchial circulation and release of mediators from the mast cells and other inflammatory cells. The cardiovascular and respiratory autonomic efferent fibers have a common central origin, so altered cardiovascular autonomic reflexes could reflect the altered respiratory autonomic status. Therefore, we performed this study to assess the autonomic abnormality and determine the correlating factors of severity of autonomic neuropathy in patients with chronic obstructive pulmonary disease(COPD) using easily reproducible cardiovascular autonomic reflex function test. Method: The study included 20 patients with COPD and 20 healthy persons obtained on Health Promotion Center in Yeungnam university hospital. All the patients had history and clinical features of COPD as defined by the American Thoracic Society. Any patients with myocardial ischemia, cardiac arrythmia, hypertension, central or peripheral nervous system disease, diabetes mellitus, or any other diseases known to produce autonomic neuropathy, has excluded. The autonomic nervous system function tests included three tests evaluating the parasympathetic system and two tests evaluating the sympathetic system. And also all subjects were subjected to pulmonary function test and arterial blood gas analysis. Results: Autonomic dysfunction was more commonly associated with patients with COPD than healthy person The parasympathetic dysfunction was frequent in patient with COPD, but sympathetic dysfunction seemed preserved. The severity of parasympathetic dysfunction in patients with COPD was correlated with the degree of duration of disease, smoking, reductions in the value of $FEV_1$ and FVC, and arterial hypoxemia but no such correlation existed for age, type of COPD, $FEV_1$/FVC, or $PaCO_s$. Conclusion: There is high frequency of parasympathetic dysfunction associated with COPD and the parasympathetic abnormality in COPD is increased in proportion to severity of airway disease. In COPD, parasympathetic dysfunction probably does not the cause of disease, but it may be an effect of disease progression.

• Tuberculosis and Respiratory Diseases
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• v.45 no.4
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• pp.776-784
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• 1998

Background: The cyclin D1 gene is one of the most frequently amplified chromosomal regions(11q13) in human carcinomas. In laryngeal and head and neck carcinomas, its overexpression has been shown to be associated with advanced local invasion and presence of lymph node metastases. Cyclin D1 may therefore playa key role in cell growth regulation and tumorigenesis. Lung cancer is a worldwide problem and in many contries it is the most lethal malignancy. As relapse is frequent after resection of early stage non-small cell lung cancer, there is an urgent need to define prognostic factors. Purpose: This study was undertaken to evaluate the prognostic value of the cyclin D1, that is one the G1 cyclins which control cell cycle progression by allowing G1 to S phase transition, on the patients in radically resected non-small cell lung cancer. Method: Total 81 cases of formalin-fixed paraffin-embedded blocks from resected primary non-small cell lung cancer from January 1, 1983 to July 31, 1995 at Hanyang University Hospital were available for both clinical follow-up and immunohistochemical staining using monoclonal antibodies for cyclin D1. Results : The histologic classification of the tumor was based on WHO criteria, and the specimens included 45 squamous cell carcinomas, 25 adenocarcinomas and 11 large cell carcinomas. Cyclin D1 overexpression was noted in 26 cases of 81 cases tested (30.9%). Cyclin D1 expression was not significantly associated with cell types of the tumor, pathological staging and the size of the tumor. But cyclin D1 overexpression was significantly correlated with positive lymph node metastasis(p=0.035). The mean survival duration was $22.76{\pm}3.50$ months in cyclin D1 positive group and $45.38{\pm}5.64$ months in eyclin D1 negative group. There was a nearly significant difference in overall survival between cyclin D1 positive and negative groups(p=0.0515) in radically resected non-small cell lung cancer. Conclusion: Based on this study, cyelin D1 overexpression appears an important poor prognostic indicator in non-small cell lung cancer and may have diagnostic and prognostic importance in the treatment of resectable non-small cell lung cancer.

• Tuberculosis and Respiratory Diseases
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• v.49 no.2
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• pp.198-206
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• 2000

Background : Pulmonary complications following bonemarrow transplantation (BMT) are common and associated with a high mortality rate. We investigated the yield, safety, and impact of fiberoptic bronchoscopy (FOB) for diagnosis of postBMT pneumoniae. Methods : From May 1997 to April 2000, 56 FOBs were performed in 52 post BMT patients for clinical pneumoniae. BMT patients with respiratory symptoms and/or pulmonary infiltrates had a thoracic HRCT(high resolution computed tomography) and bronchoscopic examination including BAL (bronchoalveolar lavage), TBLB (transbronchial lung biopsy), PSB (protected specimen brush). Results : The characteristics of the subjects were as follows : 37 males, 15 females, mean age of 31.3 years(l7-45), 35 sibling donor allogenic BMTs, 15 nonrelated donor allogenic BMTs, and 2 autologous BMTs. Fiftynine percent of FOBs (33 FOBs, 31 patients) were diagnostic. Isolated pathogens included the following : 12 cytomegalovirus (CMV) (21.4 %), 7 pneumocystis carinii (PC) (12.5 %), 11 CMV with PC (19.6 %), 2 Mycobacaterium tuberculosis (3.6%), and 1 streptococcus (1.8%). Most of the radiographic findings were diffuse interstitial lesions. CMV pneumoniae had mainly diffuse interstitial nodular lesion, and PC pneumoniae had diffuse, interstitial ground glass opacity(GGO). When CMV was accompanied by PC, a combined pattern of nodular and GGO was present. Of the 56 cases (23.2%), 13 died of CMV pneumoniae (n=2), PCP (n=2),mixed infection with CMV and PC (n=3), underlying GVHD (n=1), underlying leukemia progression (n=1), or respiratory failure of unknown origin (n=4). There was no major complication by bronchoscopy. Only 3 cases developed minor bleeding and 1 episode temporary hypoxemia. Conclusion : Based on our findings, CMV and PC are the major causes of postBMT pneumoniae. In addition, BAL can be considered a safe and accurate procedure for the evaluation of pulmonary complications after BMT.

• Journal of the korean academy of Pediatric Dentistry
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• v.31 no.4
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• pp.587-597
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• 2004

Over the past 20 years, great strides have been made in research regarding the mechanisms involved in the progression of carious lesions, but new equipment and research tools need to be developed to continue these advancements in caries research. Various methods have been applied to reduce the incidence of carious lesions, which have led to a significant decrease in the number of occlusal caries, but a concurrent increase in the proportion of proximal carious lesions. New diagnostic equipment has been developed to detect early stage carious lesions, and these have demonstrated excellent laboratory results and show promise in clinical applications. The research presented here examines the efficacy of the newly developed $DIFOTI^{TM}$ system in detecting proximal carious lesions compared to traditional intraoral exam and bitewing radiography, possible problems or deficiencies of using the system in clinic, possible improvements that can be made to the system, and the efficacy of detecting early, reversible carious lesions that can be remineralized by preventative fluoride applications. The subject pool consisted of 23 grammer school age patients just prior to entering the mixed dentition phase. Each patient was given a thorough oral examination, radiographic examination consisting of bitewing radiographs of the posterior teeth, and $DIFOTI^{TM}$ examination of the anterior and posterior teeth. Each examination was carried out two times by two examiners, and the data were statistically analyzed. The results are as follows: 1. The mean alpha value of reliability test of the visual oral examination was as follows; occlusal surface was 0.8470. mesial surface was 0.6430, distal surface was 0.5727. lingual surface was 0.2807 and distal surface was 0.2339. When the examination was limited to posterior teeth, the mean alpha value was as follows; occlusal surface was 0.8577, distal surface was 0.8211, lingual surface was 0.7728, buccal surface was 0.7152 and mesial surface was 0.6782. 2. The alpha value of reliability test of the radiographic analysis of carious lesions of the occlusal, mesial, and distal surfaces was 0.8500. 3. The alpha value of reliability test of the $DIFOTI^{TM}$ diagnostic analysis of carious lesions of the occlusal, buccal, lingual, mesial, and distal surfaces was determined to be 0.7917. 4. The $DIFOTI^{TM}$ diagnostic system was found to be the most accurate means of detecting occlusal, buccal, and lingual surface carious lesions (p<0.05), while mesial and distal proximal carious lesions were most accurately assessed using bitewing radiography (p<0.05).

• Journal of The Korean Ophthalmological Society
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• v.59 no.12
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• pp.1152-1159
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• 2018

Purpose: We prospectively investigated clinical changes and long-term outcomes after administration of the drugs recommended by the Age-Related Eye Disease Study-2 to patients with intermediate age-related macular degeneration (AMD). Methods: This prospective multicenter study enrolled 79 eyes of 55 patients taking lutein and zeaxanthin. The primary endpoint was contrast sensitivity; this was checked every 12 months for a total of 36 months after treatment commenced. The secondary endpoints were visual acuity, central macular thickness, and drusen volume; the latter two parameters were assessed using spectral domain optical coherence tomography. Results: The mean patient age was $72.46{\pm}7.16years$. Contrast sensitivity gradually improved at both three and six cycles per degree. The corrected visual acuity was $0.13{\pm}0.14logMAR$ and did not change significantly over the 36 months. Neither the central macular thickness nor drusen volume changed significantly. Conclusions: Contrast sensitivity markedly improved after treatment, improving vision and patient satisfaction. Visual acuity, central retinal thickness, and drusen volume did not deteriorate. Therefore, progression of AMD and visual function deterioration were halted.