• Childhood Kidney Diseases
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• v.9 no.2
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• pp.159-166
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• 2005

Purpose : Recently the merits of 6 weeks of initial prednisolone treatment for pediatric primary nephrotic syndrome have been reported, and the use of the 6 week regimen is increasing. We compared our experiences with the 6 week treatment versus the 4 week treatment for Korean patients. Methods : We conducted a retrospective analysis of 69 children who had primary nephrotic syndrome and who were followed up for at least 12 months in the 4 major medical centers in Daegu. The remission rate, the relapse rate, the frequency of relapse and complication of steroid treatment were compared between the 4 weeks and 6 weeks treatment group. Results : Of the 69 children, 42 were in the 4 week treatment group and 27 were in the 6 week group. The median age, blood pressure, serum total protein, serum albumin, cholesterol, creatinine, estimated creatinine clearance, 24 hour urine protein and 12 month cumulative dose did not differ between the two groups. Among the children who relapsed after steroid treatment, the relapse time was significantly later for the 6 week treatment group. The relapse rate after 1 year of treatment was 62$\%$ in the 4 week treatment group and 52$\%$ in the 6 week treatment group; however, there was no statistically significant difference between the two groups. The frequency of relapse at 12 months was $1.5{\pm}1.2$ times in the 4 week treatment group and $1.1{\pm}1.2$ times in the 6 week treatment group, and there was not different between the two groups. The most common side effects of steroid treatment were an increase of appetite and a cushingoid appearance, and there was no statistical difference between the two groups. Among the 27 children who had kidney biopsies performed, 21 suffered from minimal change nephrotic syndrome. Conclusion : The first relapse time after steroid treatment was significantly later in the 6 week steroid treatment group. The frequency of relapse and the 12 month cumulative dose of steroid were lower in the 6 week treatment group, but there was no statistical significance between the two groups. The side effects of steroid treatment did not differ between the two groups. We need to study the long term side effects and the advanced regimens of steroid treatment in the future.(J Korea Soc Pediatr Nephrol 2005;9:159-166)

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.167-174
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• 2005

Purpose : Relapses are a major problem in children with steroid responsive nephrotic syndrome(SRNS). This study has been performed to determine the predictive factors for relapse in children with SRNS. Methods : The study group consisted of 7,3 children with SRNS who had been admitted to the Department of Pediatrics, Kyungpook National University Hospital, over 6 years from 1996 to 2001. The medical records were reviewed retrospectively and analyzed to determine significant relationships between selected variables[age at onset, sex, laboratory data, the rapidity of response(days to remission), interval to first relapse] and the frequency of relapse. Results : The age($mean{\pm}SD$) of patients was $4.53{\pm}2.53$ years old. The male to female ratio was 52:21. In 95$\%$, 39 out of the 41 children had a renal biopsy, and the final diagnosis was minimal change nephrotic syndrome. There was no significant correlation between the frequency of relapse and the following variables age at onset, sex, and presence of hematuria. However, the rapidity of response correlated well with the frequency of relapse, especially during the first year after the onset of the disease(P=0.005). Conclusion : The rapidity of response is expected to be one of the predictive (actors for relapse in children with SRNS. (J Korean Soc Pediatr Nephrol 2005;9:167-174)

• 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.

• Radiation Oncology Journal
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• v.6 no.2
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• pp.183-194
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• 1988

Twenty five patients with histologically proven medulloblastoma received craniospinal radiotherapy (CSRT) at the Seoul National University Hospital from 1979 to 1984. The extent of tumor removal was biopsy only in 2 patients, partial in 18, and near total in 5. With orthogonal technique of CSRT, mainly 55Gy was delivered to the posterior fossa (PF), 40Gy to whole brain (WB), and 30Gy to whole spine (WS). And with AP; PA technique, 50Gy to PF, 45-50Gy to WB, and 36 Gy to WS. Complete remission was obtained in $84\%$ of patients. Among 21 CR's 10 failures were observed, thus total failure rate was $56\%$ (14/25). Of 14 faiure 13 had the primary failure, 11 failed in primary site alone, 1 failure was combined with ventricular seeding, and another 1 was combined with neck node metastasis. There was 1 isolated spinal failure. Actuarial overall survival rates at 3 and 5 years were $75\%$ and $54\%$, and disease-free survival rates were $58\%$ and $36\%$, respectively. Better 5 year disease-free survival was noted in patients with 55 Gy to the posterior fossa than those with 50Gy $(62\%\;vs\;17\%,\;p<0.05)$, in patients treated with orthogonal technique than those treated with AP:PA technique $(87\%\;vs\;12\%,\;p<0.05)$, and in patients with near total removal than those with partial or less removal of tumor $(56\%\;vs\;30\%,\;N.S.)$ Re-irradiation was not satisfactory No severe late sequelae was noted among the survivors. For the higher control of medulloblastoma, dose to posterior fossa should be at least 55Gy with orthogonal CSRT to small tumor burden. And dose reduction in the subarachnoidal spaces might be safe, but optimal dose to the subarchnoidal spaces should be determined by the thorough tumor staging before radiotherapy.

• Journal of Veterinary Clinics
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• v.20 no.2
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• pp.237-241
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• 2003

A case of juvenile onset generalized demodicosis of one year old, intact female Japanese Chin dog weighing 3.1kg was presented to the Small Animal Clinic of the College of Veterinary Medicine of Kyungpook National University. The diagnosis was made based on the physical examination, deep skin scrapings, hematology, serum chemistry, endocrinologic evaluation and bacterial culture. Numerous D. canis mites of various stages were observed in multiple skin scraping samples. CBC, serum chemistry, $T_3$, $T_4$ and free $T_4$ values were within normal range and Staphylococcus intermedius was isolated in bacterial culture of pustules. The dog was healthy other than skin lesions at the presentation. The three different treatment protocols were tried for the case. No clinical improvement was observed during 13 weeks of orally given daily basis ivermectin therapy at 600ug/kg and 2 weeks of spot-on weekly basis selamectin therapy at 30 mg/kg with cephalexin given orally twice a day at 25 mg/kg. However, a remarkable remission was seen by 0.1% amitraz dip on weekly basis with 4% chlorhexidine bath given twice a week. The secondary staphylococcal infection and accompanied pruritus was almost disappeared in two weeks and she was recovered clinically normal in 9 weeks of therapy. The therapy was continued for 4 more weeks to prevent relapse. The dog is clinically normal and being monitored for development of any abnormal dermatological signs for the time being.

• 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.

• Journal of muscle and joint health
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• v.1 no.1
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• pp.1-30
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• 1994

Rheumatoid arthritis is a chronic systematic disease with unpredictable course of exacerbation and remission, characterized by pain, joint deformity and reduced activity by joint limitation. The growing public awareness of the need for health management of chronic illnesses, provides impetus for nursing to demonstrate social effectiveness by active nursing intervention in this vast area in general, and with rheumatoid condition in particular. However, nursing interventions to date have not demonstrated its active participation in the management of chronic conditions. Nursing intervention for the patients having rheumatoid arthritis is one such area that needs to be studied intensively and to demonstrate their effectiveness empirically. The purposes of this study were two fold : first, to develop a comprehensive program that was intensified with therapeutic joint exercises in water, self-help group activities and utilization of self-efficacy promoting strategies : and secondly, to determine the effect of aquatic exercise program on pain, physiological changes and quality of life in the rheumatoid arthritis patients. Thirty five female subjects participating in this study were selected from outpatients in the Rheumatism Center, Hanyang University hospital. The period of data collection was from December, 1992 to March, 1993 in seoul and Taejeon. Subjects in Taejeon were assigned to the experimental group. The comprehensive aquatic exercise program for the experimental group was carried out three times a week for 6 weeks in regular swimming pool. Subjects in Seoul did not participated in the program, and treated as the control group. Data were analyzed with repeated measure MANCOVA, t-test, ANCOVA, percentage of change, Kruskal-Wallis 1-Way ANOVA using SPSS $PC^+$ program. Results were obtained as follows : 1) Scores on Korean pain scale, Numeric pain score, and RAI score of the experimental group were significantly lower than those of the control group (t=2.11, p=0.022 ; F=4.40, p=0.044 : t=3.10, p=0.002). 2) There was significant improvement in the physiological parameters (higher joint movement parameters, F=15.64, p=0.024 ; higher lean body mass, percentage of change=+12.2, lower body weight, t=1.01, p=0.026 : lower ESR, t=1.69, p=0.001) in the experimental group compaired with subjects In the control group. 3) There was significant improvement in the specific self-efficacy score through the comprehensive aquatic exercise program(t=2.73, p=0.011), but not in the general self- efficacy score(t=0.62, p=0.113). 4) The quality of life failed to show significant improvement in the experimental group as compaired with the control group (F=3.69, p=0.064). 5) In the experimental group, findings from additional analysis showed no significant difference in the specific self-efficacy between those who continued to aquatic exercise after completing 6 week program and those who stopped (X2=0.086, p=0.690). Therefore, adherence to aquatic exercise program for 6 weeks seem to be affected mainly by self-help group activities. An indepth study to delve into articulation of mechanisms affecting the effect of aquatic exercise program be recommended. A further study is necessary to determine the difference in the effect of group and individual aquatic exercise program, to assess factors affecting adherence to exercise for an extended length of time.

• Asian Pacific Journal of Cancer Prevention
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• v.16 no.15
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• pp.6627-6632
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• 2015

Background: We conducted a study exploring the clinical safety and efficacy of decitabine in patients with acute myeloid leukemia (AML) and myelodysplastic syndromes (MDS), combined with a complex karyotype. Materials and Methods: From April 2009 to September 2013, a total of 35 patients with AML/MDS combined with a complex karyotype diagnosed in the First Affiliated Hospital of Soochow University were included for retrospective analysis. All patients were treated with decitabine alone ($20mg/m^2$ daily for 5 days) or combination AAG chemotherapy (Acla 20mg qod*4d, Ara-C $10mg/m^2$ q12h*7d, G-CSF $300{\mu}g$ qd, the dose of G-CSF adjusted to the amount in blood routinely). Results: In 35 patients, 15 exhibited a complete response (CR), and 6 a partial response (PR), the overall response rate (CR+PR) being 60% (21 of 35). Median disease-free survival was 18 months and overall survival was 14 months. In the 15 MDS patients with a complex karyotype, the CR rate was 53.3% (8 of 15); in 20 AML patients with complex karyotype, the overall response rate was 65% (13 of 20). The response rate of decitabine alone (22 cases) was 56.5% (13 of 22), while in the combination chemotherapy group (13 cases), the effective rate was 61.5% (8 of 13)(P>0.05). There are 15 patients with chromosome 7 aberration, after treatment with decitabine, 7 CR, 3 PR, overall response rate was 66.7% (10 of 15). Of 18 patients with 3 to 5 kinds of chromosomal abnormalities, 66.7% demonstrated a response; of 17 with more than 5 chromosomal abnormalities, 52.9% had a response. In the total of 35 patients, with one course (23 patients) and ${\geq}$two courses (12 patients), the overall response rate was 40.9% and 92.3% (P<0.05). Grade III to IV hematological toxicity was observed in 27 cases (75%). Grade III to IV infections were clinically documented in 7 (20%). Grades I to II non-hematological toxicity were infections (18 patients), haematuria (2 patients), and bleeding (3 patients). With follow-up until September 2013, 7 patients were surviving, 18 had died and 10 were lost to follow-up. In the 6 cases who underwent allogeneic hematopoietic stem cell transplantation (HSCT) all were still relapse-free survivors. Conclusions: Decitabine alone or combination with AAG can improve outcome of AML/MDS with a complex karyotype, there being no significant difference decitabine in inducing remission rates in patients with different karyotype. Increasing the number of courses can improve efficiency. This approach with fewer treatment side effects in patients with a better tolerance should be employed in order to create an improved subsequent chance for HSCT.

• 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.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.