• Childhood Kidney Diseases
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• v.3 no.1
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• pp.80-87
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• 1999

Purposes : Renal involvement is a potentially serious complication of systemic lupus erythematosus (SLE). There have been only few studies of lupus nephritis in pediatric age. In this study, the clinical manifestations, pathologic findings, response to treatment, and clinical course of lupus nephritis in children were analyzed. And the results will provide basic data for future nation-wide prospective multi-center study. Methods . The medical records of 46 children clinically and pathologically diagnosed to have lupus nephritis at Seoul National University Children's Hospital during 1986 to 1997 were analyzed retrospectively. Results : 1) The median age of diagnosis of lupus nephritis was 12.8 years ($2\;years\~\;15year$ 8months), and the sex ratio was 1:2.5. 2) FANA($85.7\%$), anti-ds-DNA antibody ($78.0\%$), and malar rash ($60.8\%$) were the most common findings among the classification criteria by ARA Decreased C3 was detected in $88.9\%$ of patients. 3) Hematuria ($87.0\%$) was the most common renal symptom, and WHO class IV lupus nephritis was identified in 41 cases by renal biopsy. 4) In most of patients, the disease activity was controlled relatively well with a single or combined therapy of prednisolone, azathioprine, or cyclophosphamide. The response revealed no difference according to the mode of treatment. 5) Infection, especially of Varicella-Zoster virus and candida, was the most common complication during the disease course. Conclusion : The renal involvement was noted in $87.0\%$ of childhood SLE, and $89.1\%$ of renal lesions was WHO class IV lupus nephritis known to associated with poor long-term prognosis. So, aggressive treatment using immunosuppressants in the early disease course may be helpful to increase long-term prognosis of lupus nephritis. A prospective multi-center study is necessary to analyze the therapeutic efficacy of various treatment modalities.

• Childhood Kidney Diseases
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• v.3 no.1
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• pp.88-94
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• 1999

Purpose : Malformation of urinary tract is among the most common of all congenital anomalies and can progress to irreversible renal damage before diagnosis due to difficulty of early diagnosis. Present study was undertaken to determine the clinical characteristics of urinary tract anomaly and to find out the most appropriate diagnostic and therapeutic measures for children with these anomalies. Methods : During the past 10 years from 1987 to 1998, review of medical records revealed 65 children with congenital anomaly of urinary tract and the following results were obtained. Results : The most common anomalies were ureteropelvic junction obstruction occuring in 26 cases ($36\%$), followed by ureteral duplication in 11 cases, renal agenesis in 10 cases and ureterovesical function obstruction in 7 cases. Complex anomaly of urinary tract was found in 8 cases and anomaly of other systems such as congenital heart disease was detected in 11 cases. The most frequent age group at the time of diagnosis was below 1 year of age constituting 39 cases ($60\%$) and male preponderance was noted as male to female ratio being 2.25:1. Presenting symptoms were urinary tract infection in 25 cases, followed by hematuria, abdominal mass, abdominal pain and voiding difficulty, etc, and in 11 cases, the anomaly was picked up by routine prenatal ultrasonography. Azotemia was noted in 9 cases and the underlying anomaly was obstructive uropathy in 4 out of these 9 cases. Surgical correction was undertaken in 38 cases (most frequently in cases of obstructive uropathy) and in 2 out off cases with obstructive uropathy in whom surgical correction was done, azotemia disappeared during follow up period of 1-5years. No new cases of deteriorating renal function appeared during follow-up period. Conclusion : In spite of high incidence of congenital malformation of urinary tract, early diagnosis is still hampered by nonspecific symptoms and signs. Therefore, in patients with symptoms such as urinary tract infection, abdominal pain and voiding problems, etc, it Is advisable to take various diagnostic tests promptly to pick up any urinary tract anomaly and to apply proper therapy in order to avoid progression to irreversible renal damage. In this regard, prenatal ultrasonography should be utilized more widely as a routine procedure to detect any urinary tract anomalies before birth.

• Childhood Kidney Diseases
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• v.2 no.2
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• pp.110-117
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• 1998

Purpose : Henoch-$Sch{\ddot{o}}nlein$ purpura nephritis(HSPN) accompanied by nephrotic syndrome(NS) is known to have a poor prognosis and effective treatment is still controversial, even though both corticosteroids and immunosuppresant have been used for therapy. Cyclosporine A(CsA) is a well known immunosuppresant and widely used in renal transplantation and glomerular diseases especially steroid resistant. The aims of this study was to evaluate the therapeutic effect of CsA and to compare CsA with previously reported our data of rifampin(RFP) and azathioprine(AZA) in children with HSPN accompanied by NS. Methods : 37 HSPN patients with NS confirmed by renal biopsy were selected. Of these, 17 patients were treated with CsA(5 mg/kg/day) fur 6-8 months, 7 children were treated with RFP(10-20 mg/kg/day) for 9-12 months and 13 patients were treated with AZA(2 mg/kg/day) fur 8 months. Along with these regimens, low dose oral prednisolone(0.5-1 mg/kg, qod) was also used. Sequential renal biopsy was done in all patients 1 month after termination of treatment. Results : Complete remission rate of nephrotic syndrome was $58.8\%$ in CsA, $57.1\%$ in RFP and $38.4\%$ in AZA group after 17, 22, 11 months of mean follow-up period. Overall remission rate including partial remission was $88.2\%$ in CsA, $85.7\%$ in RFP and $84.6\%$ in AZA group. Disappearance rate of hematuria was $58.8\%$ in CsA, $57.1\%$ in RFP and $46.2\%$ in AZA group. Improvement of grade of clinical status was observed in 17 out of 17 CsA, 7 out of 7 RFP and 10 out of 13 AZA group. Improvement of pathologic class on sequencial renal biopsy was shown in 5 CsA($29.4\%$), none RFP($0\%$) and 2 AZA group($12.4\%$). Improvement on histologic immune-deposition was seen in 15 CsA($88.2\%$), 6 RFP($85.9\%$) and 4 AZA group($30.8\%$). Conclusion : In conclusion, Both CsA and RFP treated groups showed better result in complete remission rate of nephrotic syndrome and significant improvement of histologic immune-deposition compared with AZA treated group(p=0.004). So, we recommend CsA and REP rather than AZA for immunosuppresant treatment in HSPN with nephrotic syndrome.

• Childhood Kidney Diseases
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• v.2 no.2
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• pp.125-132
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• 1998

Purpose : To study whether a low protein diet increase the efficacy of antihypertensive therapy on the progression of renal failure, we conducted an experimental study using 5/6 nephrectomized rats(n=63). Methods : At 7 days after surgery, rats were randomly assigned to three groups according to receiving antihypertensive drug: no antihypertensive drug (U), enalapril (E), and nicardipine (N), respectively and fed a low protein diet (6$\%$ protein). Proteinuria, mesangial matrix expansion score and glomerular volume were assessed at 4, 12 and 16 weeks after renal ablation. Results : Group U rats on a low protein diet developed progressive hypertension ($140{\pm}8,\;162{\pm}5,\;171{\pm}5\;and\;184{\pm}11\;mmHg$ at 4, 8, 12 and 16 weeks) which were controlled by E and N. Group U rats on a low protein diet developed proteinuria ($74{\pm}15\;mg/day$ at 16 weeks) which were decreased by E ($42{\pm}12 mg/day$) or N ($48{\pm}8 mg/day$) (p<0.05). Mesangial matrix expansion score and glomerular volume were not different between groups U, E and N on a low protein diet regardless of the antihypertensive drugs administered. Conclusion : A low protein diet did not affect blood pressure. Enalapril and nicardipine-treated rats on a low protein diet did not have different mesangial matrix expansion and glomerular volumes from rats on a low protein diet at 12 weeks and 16 weeks, in spite of the better controlling of systemic hypertension and lessening of proteinuria. Thus, combined treatment with a low protein diet and antihypertensive drugs didn't appear to show any addition,11 effects to attenuate glomerular injury.

• The Korean Journal of Nuclear Medicine Technology
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• v.13 no.1
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• pp.93-97
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• 2009

Purpose: Recognize circulation work system and fixing work system's merits and demerits that is enforced in operation of PET/CT center in sudden increase recently. Wish to estimate connectivity degree of individual exposure dose and PET/CT working that is managed periodically through this and look for operation efficiency of PET/CT center. Materials and Methods: (1) Find interrelationship of length of service to be individual exposure dose and PET/CT through TLD interpretation. Specially, evaluate on the basis of data of 2.5 years until 2 quarters 2006~2008 year that show patient increase rapidly the latest. (2) Recognize what countermeasure is evaluating problems happened at circulation work system and fixing work system. Results: Patient examination's number was 14,674 items until 2 quarters 2006~2008 year, and the $^{18}F$-FDG average injection amount was 461.5 MBq. 2 people of 10 radiotechnologist did fixing work PET area and GAMMA area each, and 8 people did circulation work of 3 times for 2.5 years. Average exposure dose that PET area and Gamma area's circulation men in service receive was 1.32 mSv, and PET area men in services came out average 0.825 mSv high than Gamma area men in services. Nurse's exposure dose is 0.28 mSv, and next 2 reason is conjectured. One is contact with patient that medicate $^{18}F$-FDG injection, and another is consultation about patient's next time schedule after examination end. Although exposure dose's amount is not much, is expected to consider continuation work possibility by exposure dose in case is a nurse with pregnancy possibility. Also, $^{131}I$-isotope therapy area's radiotechnologist that use capsule appeared by 0.12 mSv and a nuclear medicine doctor appeared by exposure dose that is less of 0.11 mSv. Conclusions: In case do PET/CT center circulation work after a long time, connoted danger that most men in service is consecutiveness deficiency of business and individual exposure dose increase at early 1 month. Specially, way for individual exposure dose's decrease should be considered. Also, need to evaluate abhorrent work form for efficient work system introduction, and enforce circulation and fixing work suggestion suitable shift working. Finally, must make normalized business guide and so on to prevent circulation work people's business efficiency decline.

• Journal of the Korean Society of Radiology
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• v.11 no.7
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• pp.627-635
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• 2017

Brachytherapy is generally performed in conjunction with external radiation therapy, and the treatment course is very complicated, which can lead to radiation accidents. In order to solve this problem, we designed the process map by applying the failure mode and effects analysis (FMEA) method to the Brachytherapy and scored the risk priority number (RPN) for each treatment course based on this process map. The process map consisted of five steps, Patient consulting", "Brachytherapy simulation", "CT simulation", "Brachytherapy treatment planning" and "Treatment". In order to calculate the RPN, doctor, medical physicist, dose planners, therapist, and nurse participated in the study and evaluated occurrence, severity, and lack of detectability at each detail step. Overall, the process map is preceded by a patient identification procedure at each treatment stage, which can be mistaken for another patient, and a different treatment plan may be established to cause a radiation accident. As a result of evaluating the RPN for the detailed steps based on the process map, overall "Patient consulting" and "Brachytherapy treatment planning" step were evaluated as high risk. The nurses showed a tendency to be different from each other, and the nurses had a risk of 55 points or more for all the procedures except "Treatment", and the "Brachytherapy simulation" step was the highest with 88.8 points. Since the treatment stage differs somewhat for each medical institution performing radiotherapy, it is thought that the risk management should be performed intensively by preparing the process map for each institution and calculating the risk RPN.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.8 no.2
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• pp.194-201
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• 2005

Purpose: Gall bladder (GB) stones in children are rarely observed, and so reports of them have been quite limited in Korea. Therefore, we tried to assess the epidemiology and clinical features of children with GB stone diagnosed at Severance Hospital. Methods: A clinical study was performed on 18 patients below 15 years of age diagnosed with GB stone by abdominal ultrasonography or CT scan at Severance hospital from January 1999 to May 2005. Results: Concerning patients' profile, their sex ratio of male to female in total 18 cases was 2 : 1 with the mean age of $6{\pm}4.3years$. 15 patients (83.3%) were asymptomatic. Inducing factors were found in 14 children (77.8%) including ceftriaxone therapy in 6 cases (33.3%), infection in 4 cases (22.2%), spherocytosis in 3 cases (16.7%), Down syndrome and abdominal operation in 1 case (5.6%) respectively. Single stone was found in 13 cases (76.5%) and multiple in 4 cases (23.5%). 15 cases (88.2%) had the stones less than 5 mm in size, and 2 cases (11.8%) between 5 and 10 mm. 17 patients received conservative treatment, and one patient had cholecystectomy due to Meckel diverticulum. Conclusion: In this report, Pediatric GB stones are predominant in male children, without typical symptoms, having inducing factors, single and small. Recently the diagnosis of them has been increased because of the development of imaging study. But the reports for them were still rare in Korea. Therefore the study for them is more necessary to find it's clinical characteristics.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.6 no.2
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• pp.129-139
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• 2003

Purpose: The aim of the study was to evaluate the long-term outcome of glycogen storage disease (GSD) type 1 with particular reference to hepatic adenoma and hepatocellular carcinoma, and to analyze risk factors affecting the development of hepatic adenoma in GSD type 1. Methods: Forty-three GSD type 1 patients (31 males and 12 females, mean age $13.9{\pm}6.4$ years) were analyzed retrospectively. Hepatic adenoma was detected on abdominal USG and diagnosed on histologic examination. Clinical profiles were compared between patients with hepatic adenoma (n=16) and age-matched controls without hepatic adenoma (n=16). Results: 1) Of 43 GSD type 1 patients, 16 (37.2%) had hepatic adeoma. Hepatic adenoma was detected at the age of mean $14.2{\pm}4.1$ years (range: 7.9~25.7 years). Fourteen (87.5%) adenomas were multiple at detection. 2) Comparison of the clinical profiles between adenoma group and non-adenoma group revealed that age at first introduction of uncooked cornstarch treatment was significantly late in adenoma group compared with non-adenoma group ($9.1{\pm}5.2$ years vs. $3.0{\pm}1.8$ years, p=0.003). Portocaval shunt surgery was performed in 11 (68.8%) patients in adenoma group and 3 (18.8%) in non-adenoma group (p=0.004). Hepatic adenoma developed mean $5.8{\pm}4.2$ years after shunt operation. 3) One patient was diagnosed as hepatocellular carcinoma at the age of 25.7 years. Conclusion: Hepatic adenoma is an important late complication of GSD type 1 with the risk of malignant transformation. Early introduction of cornstarch therapy with strict metabolic control is needed to prevent the development of hepatic adenoma in GSD type 1.

• Pediatric Infection and Vaccine
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• v.21 no.2
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• pp.129-138
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• 2014

Purpose: Streptococcus pyogenes is an important cause of invasive diseases in children. We aimed to describe the clinical characteristics of invasive infections due to S. pyogenes in children in Korea. Methods: A retrospective study of children under 18 years of age with invasive infections due to S. pyogenes at Seoul National University Children's Hospital between March 1992 and December 2012, and Seoul National University Bundang Hospital between March 2003 and December 2012 was conducted. Demographic factors, clinical characteristics, laboratory findings, treatment, mortality and morbidity of all patients were reviewed. Results: A total of 30 among 36 cases identified as invasive disease due to S. pyogenes were available for review. There was a predominance for male subjects (male:female=2.75:1). The median age was 50 months (range 12 days to 15 years) and 53.3% were under 5 years of age. Skin and soft tissue infections (9/30, 30.0%), bacteremia without identified focus (4/30, 13.3%) and bone and joint infections (6/30, 20.0%) were the most frequent clinical presentations. Streptococcal toxic shock syndrome (3/30, 10.0%) pulmonary, abdomen and central nervous system infections (2/30, 6.7%) were also seen. There was a peak in number of patients in year 2012 (9/30, 30.0%). There were no cases of mortality. Erythromycin and clindamycin resistance rates were low by 3.8% and 7.5%, respectively. Conclusion: We studied the clinical presentations of invasive infections due to S. pyogenes during the past 20 years in Korean children. The findings of this study help us understand the characteristics of the disease, enhancing early recognition and prompting adequate antibiotic therapy which is important in reducing morbidity and mortality.

• Journal of Preventive Medicine and Public Health
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• v.20 no.2 s.22
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• pp.312-321
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• 1987

To investigate the maternal and child factors associated with early detection of cerebral palsy, 74 mothers of cerebral palsy children who were born since January 1, 1980 and being treated at Taegu Rehabilitation Center for the Handicapped, Rehabilitiation Center of Taegu University, St. Paul Children's House and Pusan Welfare Association of Cerebral Palsy Children were interviewed from February to April 1987. There is no association between age of child when parents noticed the child's abnormality and educational level of father but it tend to be detected earlier when education level of mother is college or above compared with high school or under. There is a trend of earlier detection of child's abnormality although statistically not significant in case father is professional or managerial worker, monthly income of father is over \610,000, child is first-born, age of the parents is 34 years or under, child is a boy, and child has periodic well-baby check-up. The child's abnormality is detected earlier when mothers had 7 prenatal visits or more compared with those who had 6 visits or less (p<0.05). Parents noticed the child's abnormality first in 85.1% of the cases whereas doctors detected it first in 2.7% and this percentage was not different whether the child had periodic well-baby check-up or not. The first physician's diagnosis of the children was cerebral palsy in 36.5% and the rest was normal, need for observation, uncertain, etc. Parents took the child to doctor for diagnosis 2-3 months after they noticed the child's abnormality and after the child was diagnosed as cerebral palsy parents either took no therapeutic measure or brought the child to physiotherapy or acupuncture or gave herb medicine before they started specific rehabilitative therapy. For early detection of the cerebral palsy children, teaching of evaluation method for child development should be reinforced both in medical school and clinical training course and should train the specialist for diagnosis and treatment of crippling conditions. Also, public education is needed for the importance of early detection of crippling conditions and currently available methods for diagnosis and treatment.