• Childhood Kidney Diseases
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• v.13 no.1
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• pp.84-91
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• 2009

We experienced a female neonate with congenital nephrotic syndrome (CNS) associated with congenital diaphragmatic hernia (CDH). Because of the rare combination of two conditions, we report this case with literature review. CDH was found immediately after birth and emergency operation was done for hernia repair. But on the next day, generalized edema and oliguria(0.59 mL/kg/hour) was found and her blood chemistry showed hypoalbuminemia (1.6 g/dL), increased BUN (27.7 mg/dL) and serum creatinine( 1.8 mg/dL) along with heavy proteinuria (4+). We started albumin infusion with a bolus of intravenous furosemide. We suspected the neonate had congenital nephrotic syndrome and her 24hr urine protein was 1,816 mg/day. In spite of immunosuppressive therapy, the nephrotic syndrome and renal failure progressed. We started peritoneal dialysis on the day of life 22 but it was not satisfactory. She was complicated by intracranial hemorrhage and multi-organ failure and expired at 34 days of age. Kidney necropsy was performed which showed diffuse mesangial sclerosis (DMS). Her chromosome study revealed 46, XX and her gene study revealed a heterozygous missense mutation, Arg366His, in Wilms tumor suppressor gene (WT1). This case deserves attention on account of the 4th case of CNS with CDH revealing the Arg366His mutation in the WT1 gene and G the 1st case of early onset renal failure without male pseudohermaphroditism and Wilms tumor with CNS, CDH and the Arg366His mutation in the WT1 gene. So, this report gives support to the hypothesis that Arg366His mutation in the WT1 gene can result in CNS and CDH.

• Childhood Kidney Diseases
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• v.6 no.1
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• pp.102-108
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• 2002

We report two cases of hemolytic uremic syndrome (HUS) associated with Escherichia coli O114. Two cases were similar and showed the same clinical courses. After prodrome of diarrhea and vomiting lasting 1-2 days, azotemia persisted for about 10 days, and during that period, the patients were on peritoneal dialysis. They recovered without any sequelae after about 15 days. Direct multiplex PCR of stool culture revealed eae and stx2 gene and the result of ELISA done on the colony positive of one gene confirmed Escherichia coli O114. This is the first report of HUS associated with Escherichia coli O114. We recommend, Shiga toxin producing bacterial Infection must be considered and efforts should be made to scrutinize the organism in all diarrhea-prodrome HUS patients.(J Korean Soc Pediatr Nephrol 2002 ;6 : 102-8)

• Journal of Korean Academy of Fundamentals of Nursing
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• v.5 no.2
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• pp.313-323
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• 1998

This study was a descriptive research on the level of self-care available to continous ambulatory peritoneal dialysis patients (hereinafter referred to as 'CAPD patient') related to the specific area of infection management. The method employed for the collection of data was a modified instrument of the self-care survey essentially based on Young Sook Choi's instrument. The relevant data was collected from september 1, 1996 to september 30, 1996. The subjects were provided with an open-ended question regarding the reasons behind why they did not seek self-care. The answers provided about self-care compliance were analyzed by SPSS for frequency, percentage, mean, t-test, ANOVA. Reasons for non-compliance were analyzed by content analysis. The results of the study were as follows : 1. The percentage of patients engaging in self-care were according to the following self performed tasks : preparation of dialysis : 30.58 points Dialysate exchange procedures : 49.40 points - Two bag type : 50.50 points - Spike type : 48.80 points - Neo type : 48.90 points Catheter exit site care : 25.13 points More specifically, in relation to the preparation of dialysis as referred to above, those patients engaging in self care was relatively high with respect to the cleaning of the dialysis before use and for the preservation of peritosol. However, in dialysate exchange procedures, data revealed that those patients engaging in self-care are relatively low with respect to putting on a mask during the performance of peritosol exchange. Similary in peritosol exchange procedure and catheter exit care, low levels of self-care performance were found in the area of putting on a mask during the peritosol exchange procedures and catheter line testing procedures, respectively. 2. In general characteristics, there appeared to be no distinction in self-care compliance among CAPD patients. 3. The main reasons for non-compliance were based in the following factor : intellectual, attitude, enviromental surrounding and physical. As a result of the foregoing finding, nurses should provide adequate assistance to promote self-care compliance by CAPD patients by checking the preparation of dialysis, dialysate exchange procedure, and catheter exit site care which recieved low point in this research.

• Pediatric Infection and Vaccine
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• v.15 no.2
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• pp.206-211
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• 2008

Streptococcus pneumoniae is a common cause of acute otitis media, sinusitis, pneumonia, and the invasive bacterial infections in children. Rarely, S. pneumoniae is an uncommon cause of hemolytic-uremic syndrome (HUS). We report a 33 month-old girl who presented with pneumonia, and subsequently developed HUS. Her pulmonary infection was complicated by necrotizing pneumonia and acute respiratory distress syndrome. Cultures from blood and pleural fluid grew S. pneumoniae, serotype 19A. She was treated with antibiotics, dialysis and mechanical ventilatory support. She was discharged with normal renal function after 2 months of management. She remained healthy without renal complications at the 5 year follow-up visit.

• Neonatal Medicine
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• v.17 no.2
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• pp.254-261
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• 2010

Little is known about neonatal mitochondrial disease, though mitochondrial metabolic disorders may often present in the neonatal period because of the high energy requirement of neonate. In newborn period, common presentations are not specific and the disease course may be rapid and fatal. In this study, we report three cases of neonatal mitochondrial disease. The first case was strongly suspected because of sudden seizure and mental change with severe lactic acidosis, and multiorgan failure. Plasma lactate/pyruvate (L/P) ratio was increased to 55.6 with marked lactic aciduria and increased plasma alanin up to 2,237 nmol/mL. In the second patient, a peritoneal dialysis was performed for acute adrenal and renal failure, but metabolic acidosis persisted. Plasma L/P ratio was increased to 23.9, and MRC I (mitochondrial respiratory chain defect) was diagnosed through the enzymatic analysis of the muscles. The third case showed repetitive episode of lactic acidosis during the first two months of life, hypotonia, failure to thrive and feeding difficulties. We found markedly increased cerebrospinal fluid L/P ratio up to 57 though plasma L/P ratio(19.4) was borderline with increased plasma lactate. The lactate peak was prominent in brain magnetic resonance spectroscopy (MRS). MRC II was confirmed through muscle biopsy. Plasma lactate level and lactate peak of brain MRS were normalized after conservative treatment.

• Applied Chemistry for Engineering
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• v.17 no.3
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• pp.303-309
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• 2006

In this study, functional anion-exchange membranes have been prepared and characterized to improve the permeation fluxes of the anion and urea for peritoneum dialysis. They were prepared by UV and radiation graft polymerization methods. The separation-membrane prepared by UV graft polymerization showed the highest grafting degree when HEMA and VBTAC were mixed by 1:2 ratio. However, the grafting degree decreased slightly at compositions above the 1:2 ratio because of the disruption of UV penetration caused by build-up of homopolymer. In the case of photo-initiator, the grafting degree increased up to 0.2 wt%, above which it decreased to a small extent. For the two membranes prepared by radiation graft polymerization, the VBTAC/HEMA membrane showed 96% grafting degree for 6 h reaction time and the GMA membrane showed over 100% grafting degree for 2 h reaction time. Anion-exchange membranes were prepared with 113% grafting degree and with DEA and TEA exchange groups. The DEA membrane showed the conversion degree of 70% in 4 h reaction time while the TEA membrane showed 30% in 2 h reaction time. The prepared anion-exchange membranes were permeable to only anions and urea, but not cations.

• Journal of the Korean Society of Food Science and Nutrition
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• v.31 no.5
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• pp.847-885
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• 2002

The purpose of this study was to develop a web-based internet program for nutritional assessment and diet Prescription by renal diseases. Renal diseases were classified by nephrotic syndrome, renal failure, hemodialysis and peritoneal dialysis. The system consisted of five parts according to their functions and contents. The first part is to assess the general health status such as body weight, obesity index, basal metabolic rate and total energy requirement by the input of age, sex, height, weight and degree of activity. The second part was designed to investigate dietary history of patient, that is, to find out his inappropriate dietary habit and give him some suggestions for appropriate dietary behavior by investigating his dietary history. This part also offers the diet and nutrition management by personal status with renal disease, and the information for food selection, snacks, convenience foods, dine-out, behavioral modification, cooking methods, food exchange lists and terms. The third part is evaluating their energy and nutrients intake by comparing with recommended dietary allowance for Koreans or standardized data for patient with renal disease. In this part, it is also analyzing energy and nutrients of food consumed by food group and meals, and evaluating the status of nutrient intake. The fort]1 one, a major part of the system, is implementing the diet and menu planning by using food exchange lists. This Part Provides the patient with menus lists and I day menu suitable to his weight, activity and the status of renal disease. The fifth part is providing information on energy and nutrients of foods and drinks, and top 20 foods classified by nutrients. These results are finally displayed as tabular forms and graphical forms on the computer screen.

• 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.

• Childhood Kidney Diseases
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• v.12 no.1
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• pp.30-37
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• 2008

Purpose: The present study is an investigation of the progression and prognosis of acute intrinsic renal failure in neonates and children with a diagnosis of acute renal failure or other diseases on admission. Methods: This research is based on a retrospective analysis conducted on 59 patients(male: female=2.2:1) diagnosed with acute intrinsic renal failure between January 2000 and June 2006 at Busan Paik Hospital. The clinical diagnostic criteria of acute renal failure used was serum creatinine <1.2 mg/dL, oliguria with urine output$\leq$0.5 mL/kg/hr and anuria with urine output <50 mL per day. Results: Among those placed under investigation, 7 patients were neonates, 10 patients were 2 months-2 years old, 12 patients were 3-6 years old, 21 patients were 7-12 years old and 9 patients were 13-16 years old. It took 3.1${\pm}$2.8 days on average until the diagnosis was made. The urine output distribution was 21 persons for the oliguria group, and 36 persons for the non-oliguria group, and 2 persons for the anuria group. For the underlying causes, 30 persons were classified in the primary renal disease group, 14 persons in the infection group, 9 persons in the malignancy group, and 6 persons were categorized in another group. As for age distribution, the infected group was predominantly neonates, whereas the dominant age ranges for the primary renal disease and infection categories were 2 months to 2 years old. Also, the primary renal disease was dominant among older children, aged 3 and up. No difference was detected according to seasonal prevalence. However, there was a high morbidity rate among hemolytic uremic syndrome diagnosed in the summer. Peritoneal dialysis was used to treat 4 patients. It took 10.0${\pm}$6.7 days until the patients improved. 18 patients died. The non-oliguria group's mortality rate was lower than other groups. There was a high mortality rate in the neonates and malignancy group. Conclusion: Acute renal failure in childhood seems to take a better clinical course than in adulthood when there is an early diagnosis and proper treatment of underlying diseases.

• Clinical and Experimental Pediatrics
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• v.46 no.9
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• pp.883-888
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• 2003

Purpose : The etiology of hemolytic anemia can be classified as either cellular or extracellular defects of red blood cells. The aim of this study was to investigate the clinical and laboratory findings of hemolytic anemia concerning its etiological classification. Methods : Clinical and laboratory findings of the patients with hemolytic anemia treated from January 1987 to May 2002 at Severance Hospital were analyzed retrospectively. They were divided into two groups based on the types of red cell defects(group I : erythrocytic defect, group II : extraerythrocytic defect). Results : Twenty one cases were included in group I, thirty four cases in group II, and three cases were unclassified. In group I, nineteen cases(90.5%) were diagnosed as hereditary spherocytosis and were proved to have red cell membrane disorders while two cases(9.5%) were shown to have red cell enzyme deficiencies. In group II, thirteen cases(38.2%) were noted as autoimmune hemolytic anemia, eleven cases(32.4%) as traumatic or microangiopathic hemolytic anemia, four cases(11.8%) as drug induced hemolytic anemia, two cases(5.9%) were related with systemic lupus erythematosus and one case(2.9%) with malignancy. Hemoglobin at the time of diagnosis(7.5 g/dL vs. 6.2 g/dL, P<0.05) and the incidence of splenomegaly(85.7% vs. 18.2%, P<0.05) were higher in group I though blood urea nitrogen(9.0/0.4 mg/dL vs. 27.8/1.6 mg/dL, P<0.05) was higher in group II. Conclusion : Comparing the clinical features of pediatric hemolytic anemia, we concluded as following : In cases associated with extraerythrocytic defect, blood tests revealed significant initial lower hematocrit with higher level of BUN and Cr while cases with erythrocytic defect, splenomegaly were more common noted.