• Childhood Kidney Diseases
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• v.10 no.1
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• pp.27-32
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• 2006

Purpose : Conservative management of multicystic dysplastic kidney(MCDK) without nephrectomy has recently been advocated. The purpose of this study was to determine the clinical course of conservatively managed MCDK and to find out possible predictive factors for involution of MCDK by ultrasonography(US). Methods : A retrospective analysis was made on 45 patients(26 boys and 20 girls) in whom MCDK was detected and had been traced by US between Dec. 1993 and Aug. 2005 at Severance Hospital. Results : Median follow-up time was 30 months(range 2-102 months). All patients under-went radionuclide scans and voiding eystourethrograms. The serial follow-up US showed complete involution in 11(24%), partial involution in 19(41%), and no interval change or increased in cyst size in 13(28%) patients. Nephrectomy was done in 3 patients(7%) due to relapsing urinary tract infection(UTI) and severe abdominal distension. The mean age of complete involution of MCDK was 37 months(range 12-84 months). Episodes of UTI were present in 17 patients(37%) and additional genitourinary(GU) abnormalities were found in 22 patients(44%). Hypertension and renal insufficiency was complicated in one patient. No child developed malignant tumor. Univariate analysis showed that five variables were associated with complete involution of the MCDK; gender, site, UTI episode, additional GU abnormalities, and renal length on initial US. After adjusting using the Pearson model, the presence of additional GU abnormalities was exclusively associated with complete involution among the 5 variables(P=0.034). Conclusion : In our review of 46 cases of MCDK, non-surgical approach for patients with MCDK was advisable and we could predict poor prognosis when MCDK is associated with other GU anomalies.

• Childhood Kidney Diseases
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• v.7 no.2
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• pp.181-188
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• 2003

Purpose : Vesicoureteral reflux(VUR) is known to be the main cause of childhood hypertension and renal failure. Knowing its familial occurrence, we determined the incidence of VUR and renal scar in asymptomatic siblings of Korean patients with primary VUR Methods : A total of 50 siblings from 37 index patients were included. Voiding cystourethro-graphy(VCUG) and renal scintigraphy using $^{99m}Tc-DMSA$ were peformed in these siblings from June, 1994 to May, 2001, Index patients were classified into two groups according to the presence of VUR in their siblings, and the clinical factors of the index patients such as age, sex, grade of reflux and renal cortical defect were compared between the groups. Results : Among the 50 siblings, VUR were found in 8(16%) and renal cortical defects were detected in 8(16%) siblings respectively. The incidence of renal cortical defects was 87.5%(7 out of 8) in the VUR(+) siblings. There was a case of VUR(-) cortical defect in one sibling, presumed as a scar from an old VUR. There was no relationship among age, sex, grade of reflux and renal cortical defect of the index patient to the presence of VUR in siblings. Conclusion : This study confirmed a significant incidence of VUR(16%) and renal cortical defects(16%) in the asymptomatic siblings of patients with primary VUR in Korea. It Is resonable to recommend screening studies to the siblings of patients with VUR for the early detection and prevention of probable reflux nephropathy. (J K orean Soc Pediatr Nephrol 2003;7:181-188)

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.136-146
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• 2001

Purpose : One of the most difficult problems in the care of children with nephrotic syndrome remains the occurrence of relapses, despite initial response to steroids. Constantinescu reported that rapidity of initial response to steroid therapy could predict fewer relapses in the first year. So we evaluated the changes in serum lipid abnormalities in children with corticosensitive nephrotic syndrome before steroid treatment and the correlation between serum lipid levels and renal function, days to remission. Methods . We analyzed the Medical records of children who were managed by us between October 1994 and August 2000. In 33 patients with corticosensitive nephrotic syndrome, we evaluated the correlation between serum lipid levels and renal function [Creatinine clearance(Ccr)] and proteinuria before steroid treatment, and days to remission defined as the third day when the patient's urine becomes protein free. Results : There were 21 males and 12 females. Median age at presentation was 6.4 years (range: 1.8-17.3 years). Median days to remission were 15.4 days (range 4-42 days) on Prednisolone $60mg/m^2$ daily. The increased levels of triglyceride, total cholesterol, LDL cholesterol, apolipoprotein B, total cholesterol/HDL cholesterol, Lipoprotein(a) were observed. But the level of HDL cholesterol was not increased. Serum albumin was decreased a]id proteinuria was increased before steroid treatment. But Ccr was not decreased. There were negative correlation between serum albumin and total cholesterol (r = -0.5157, P<0.005), LDL cholesterol (r = -0.5543, P<0.005), total cholesterol/HDL cholesterol (r = -0.4506, P<0.01), lipoprotein(a) (r = -0.4570, P<0.025), apolipoprotein B (r = -0.5297, P<0.025), apolipoprotein B/apolipoprotein Al (r = -0.5851, P<0.01), apolipoprotein B/HDL cholesterol (r = -0.4961, P<0.05) before steroid treatment. There was no correlation between proteinuria and serum lipid profiles. Also Ccr and serum lipid profiles were not correlated. There was positive correlation between days to remission and HDL cholesterol (r = +0.4511, P<0.05), apolipoprotein B (r = +0.5190, P<0.05), apolipoprotein B/HDL cholesterol (r = +0.7169, P<0.005). Conclusions : This results reveal that HDL cholesterol, apolipoprotein B and apolipoprotein B/HDL cholesterol can be used as a predictive factor in corticosensitive nephrotic syndrome. We could not determine the significant level of these lipids for insufficient patients number, but these level may predict future relapses of corticosensitive nephrotic syndrome patients and thus may allow to better management and treatment protocols. More data and long term follow up studies should be needed. (J Korean Soc Pediatr Nephrol 2001;5 : 136-46)

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.78-86
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• 2001

Purpose: Rapidly progressive glomerulonephritis (RPGN) is characterized by the rapid increase in serum creatitnin and crescents formation involving more than $50\%$ of glomeruli. 10 patients who had been treated for RPGN were studied retrospectively for thier underlying diseases and clinical features Method: Cilinical review was performed on 10 children who were diagnosed with RPGN by clinical features and renal biopsy and followed up at department of pediatrics during tile last 10 years, from May 1990 to May 2000. Result: There were 6 males and 4 females between the ages of 2.1 and 14.3 years (mean $10.9{\pm}3.8$). 3 had Henoch-$Sch{\ddot{o}}nlein$ purpura nephritis; 2, idiopathic rapidly progressive glomerulonephritis; 2, lupus nephritis; 1, hemolytic uremic syndrome; 1, membranous glomerulonephritis and 1, microscopic polyangiitis. The most common chief complaints were gross hematuria and oliguria. Initial clinical features included proteinuria, edema, hypertension, nausea and arthralgia. Mean serum BUN was $74.2{\pm}39.1\;mg/dL$ mean serum creatinin, $3.2{\pm}1.8\;mg/dL$ and mean creatinin clearance, $26.5{\pm}13.2\;mL/min/1.73m^2$. Antineutrophil cytoplasmic antibody was positive only in microscopic polyangiitis. ANA and Anti-DNA antibody were positive in two lupus nephritis patients. Serum complements were decreased in 4 patients. All patients except Hemolytic uremic syndrome received steroid pulse therapy and immunosupressive agents. 3 patients were performed acute peritoneal dialysis and 2 patients were given plasmapheresis. At the last follow up, 1 patient was dead, 4 patients had elevated serum creatinin, 2 of these 4 patients were on chronic ambulatory peritoneal dialysis and 6 patients had normal renal function. Conclusion: Rapidly progressive glomerulonephritis is a medical emergency that requires very rapid diagnosis, classification, and therapy. Appropriate therapy selected on the basis of underlying disease mechanism can substantially improve renal survival. (J. Korean Soc Pediatr Nephrol 2001 ; 5 : 78-86)

• Clinical and Experimental Pediatrics
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• v.45 no.12
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• pp.1519-1527
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• 2002

Purpose : Although influenza virus is one of the most important causes of acute respiratory tract infections(ARTIs) in children, virus isolation is not popular and there are only a few clinical studies on influenza in Korea. We evaluated the epidemiologic and clinical features of ARTIs by influenza virus in children. Methods : From February 1995 to August 2001, nasopharyngeal aspirations were obtained and cultured for the isolation of influenza virus in children admitted with ARTIs. The medical records of patients with influenza virus infection were reviewed retrospectively. Results : Respiratory viruses were isolated in 997(22.0%) out of 4,533 patients examined, and influenza virus was isolated in 164 cases(3.6%). Influenza virus was isolated year after year mainly from December to April of next year. The ratio of male and female was 1.9 : 1 with a median age of 15 months. The most common clinical diagnosis of influenza virus infection was pneumonia, and fever and cough developed in most patients. There was no difference between influenza A and B infection in clinical diagnoses and symptoms. All patients recovered without receiving antiviral treatment except for one patient diagnosed with pneumonia who had underlying disease of Down syndrome with ventricular septal defect. Conclusion : ARTIs caused by influenza virus developed every winter and spring during the period of study. Because fatal complication can develop in the high risk group, prevention, early diagnosis and proper management of influenza should be emphasized.

• Journal of Chest Surgery
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• v.36 no.1
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• pp.1-6
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• 2003

Currently, atrial septal defect repair has been considered low risk operation duo to the development of open heart surgery Not only the operation itself, but also the cosmetic aspect is now focused. Though many methods exist as minimally invasive cardiac surgery in atrial septal defect repair, some surgeons advocate that right anterolateral thoracotomy is better than the others in the cosmetic aspect and we compared right anterolateral thoracotomy with median sternotomy. Material and Method: From January 1999 to August 2002, 43 patient underwent atrial septal defect repair by one operator, including 15 patients through right anterolateral thoracotomy(group A) and 15 patients through median sternotomy(group B) in Hanyang university Hospital. The data were randomized and operation outcomes were analyzed between these two groups. Result: The mean weight of group A was 38.77$\pm$15.57kg and 38.21$\pm$21.82kg in group B. In group A, mean operation (OP) time was 197.6$\pm$61.40min, mean cardiopulmonary bypass(CPB) time was 48.66$\pm$13.02min and mean fibrillation time or aortic cross clamp(ACC) time was 30$\pm$11.64min. In group B, mean OP time was 212.33$\pm$31.95min, mean CPB time was 55$\pm$12.10min, and mean fibrillation or ACC time was 29.33$\pm$9.04min. There was no significant differences in these two groups. In group A, mean mechanical ventilation time was 3.78$\pm$0.78 hours, mean postoperative ICU stay was 1.2$\pm$0.47 days and mean postoperative hospital stay was 10.20 41.08 days. In group B, mean mechanical ventilation time was 5.95$\pm$3.73 hours, mean post operative ICU stay was 1.41$\pm$0.61 days, and mean postoperative hospital stay was 12.20$\pm$3.55 days. There was no any significant difference in two groups. Group A had significantly lower mean thoracic and pleuropericardial drainage than group B (175.33$\pm$90.54cc vs 352.33$\pm$239.43cc, p<0.05). Complication was seen in one case in group B, transient 2nd degree A-V block. Conclusion: Right anterolateral thoracotomy was better than median sternotomy not only in cosmetic aspect but also in postoperative thoracic and pleuropericardial drainage, using the same instrument(p.0.05). But, right anterolateral thoracotomy was more technically difficult due to narrow operative field and we should be careful of aortic cannulation.

• Childhood Kidney Diseases
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• v.8 no.1
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• pp.18-25
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• 2004

Purpose : Alport syndrome is clinically characterized by hereditary progressive nephritis causing ESRD with irregular thickening of the GBM and sensory neural hearing loss. The mutations of type IV collagen gene(COL4A5) located on the long arm of X chromosome is considered responsible for most of the structural abnormalities in the GBM of Alport patients. Since no definite clinical prognostic predictor has been reported in the disease yet, we designed this study to evaluate the significance of genetic polymorphism of the angiotensin converting enzyme in children with Alport syndrome as a prognostic factor for disease progression. Methods : ACE I/D genotype were examined by PCR amplification of the genomic DNA in 12 patients with Alport syndrome and 12 of their family members. Alport patients were divided into two groups; the conservative group, those who had preserved renal function for more than 10 years of age, the early CRF group, those who had progressed to CRF within 10 years of age. Results : The mean age of onset was $3.45{\pm}2.4$ years in the conservative group, $4.4{\pm}1.2$ years in the early CRF group. Sex ratios were 5:3 and 2:1 in each group. Among 12 cases of patients, 4 cases were in early CRF group and their mean duration of onset to CRF was 4.5 yews(8.9 years of age). Eight patients(67%) were in the conservative group and they had normal renal function for more than 10 years of age(mean duration of renal preservation was 10.6 years). The incidence of II type ACE gene were in 25.0%(3 cases), ID type in 41.7%(5 cases), DD type in 33.3%(4 cases). There was no significant difference between Alport patient and normal control(II type 44.3%, ID type 40.9%, DD type 14.8%). The incidence of DD type of early CRF group were higher than that of the conservative group(75% vs 12.5%)(p<0.05). There was no difference in ACE gene polymorphism between normal Alport family members and control group. Conclusion : Even though there was no significant difference of ACE polymorphism between Alport patients and the normal control group, the incidence of DD type is significantly increased in early CRF group which means DD type of ACE polymorphism has a possibility of being a predictor for early progression to CRF in Alport patients.

• Journal of dental hygiene science
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• v.4 no.1
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• pp.21-25
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• 2004

In modern times, children's trauma is increasing every year because of car accidents and life environment changes. There is a limit to prevent traumatic damage for oral cavity organization. The fundamental data of trauma treatment and prevention will be presented through the survey and analysis of traumatic teeth damage. I examined 113 patients from Oct. 4th, 2000 to Feb. 27th, 2004 at Dept. of Children's Dental Clinic, Kangnung National University. The results are as follows. (1) The trauma frequency of male subjects is higher than that of female at a rate of 2.05:1. The average age is 5.27 for men and 5.27 for women. The highest percentage of trauma patients is among 2 year old children. It is 21.2%. (2) A patient survey was taken at a trauma treatment hospital. On the first day 34.4% of the patients had come to receive treatment of their first set of teeth. However, after a week, 38.8% of the patients had received treatment on their permanent teeth. (3) As a result of falling, 59% of patients needing treatment on their first set of teeth. 55.1% of patients is permanent teeth. As a result of bump against physical solid, 26.6% of patients is the first set of teeth and 26.5% of patients is permanent teeth. (4) Teeth damage happened at home. 42.1% were male. 35.1% were female. According to trauma, 59.4% of teeth damage happened at home. 28.6% of permanent teeth damage happened at school or kindergarten. (5) According to trauma, the number of teeth damaged was in the first set of teeth are as follows: 56.3%, one-31.3%, three or four-6.3% each. For permanent teeth: two-46.9%, one-28.6%, four over-16.3% and three-8.2%. Over four teeth is larger number for permanent teeth. (6) 56% of first set of teeth patients and 43.4% of permanent teeth patients were male. 56.8% of first set of teeth patients and 43.2% of permanent teeth were female. Trauma happened to both male and female frequently in the first set of teeth. (7) Most of the tooth damage which was in the first set of teeth and permanent teeth was done to the upper jaw. 75% of patients are the first set of teeth. 63.8% of patients are permanent teeth. Trauma is very high in the two mid teeth of the upper jaw. (8) According to trauma survey, 30.2% is from impulse. 28.0% is from crown fracture, 14.7% is from depression. 8.9% is from concussion. 7.1% is from full dislocation of a joint. 2.2% of patients are extrusion. 1.8% is from displacement. According to teeth damage trauma, 35.8% is pulse in the first set of teeth. The breaking of the crown of a tooth happened a lot in permanent teeth. (9) According to data, 43.2% of teeth damage in the first set of teeth goes without treatment. In permanent teeth, it is 38.9%. After treatment, 22.0% of first set of teeth treatment requires a dental pulp treatment. In permanent teeth, which is used for temporary acid etching resin restoration.