• Clinical and Experimental Pediatrics
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• v.52 no.4
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• pp.464-470
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• 2009

Purpose : This study assessed the incidence and outcome of congenital anomalies of the kidney and urinary tract (CAKUT) detected by prenatal ultrasonography Methods : There were 906 cases of CAKUT detected by prenatal ultrasonography and postnatally confirmed at the Asan Medical Center from October 1989 to October 2007. We investigated the incidence and outcome of these cases by reviewing medical records retrospectively. Results : The order of incidence was hydronephrosis, multicystic dysplastic kidney (MCDK), duplex kidney, vesico-ureteral reflux (VUR), single kidney, hydroureteronephrosis, ectopic kidney, polycystic kidney, ureterocele, and posterior urethral valve (PUV). There were 520 cases (57.4%) of hydronephrosis, and 20% of these needed an operation due to significant obstruction. MCDK was associated with other CAKUT in 25.4% of all cases. Approximately 57.9% of duplex kidney cases needed surgical treatment due to ureterocele and VUR. VUR had a male: female ratio of 10:1. Two out of seven cases of autosomal recessive polycystic kidney had progressed to chronic renal failure. Patients with PUV were relatively uncommon, and one out of nine cases progressed to end-stage renal disease. Conclusion : CAKUTs detected by prenatal ultrasonography were composed of various anomalies, and almost all of them had a good outcome without any intervention. However, in some cases, recurrent urinary tract infection or renal failure occurred, especially in bilateral cases. For further management, a long-term multicenter study is needed to investigate the precise incidence and outcome of each anomaly in the general population.

• Clinical and Experimental Pediatrics
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• v.51 no.1
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• pp.42-46
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• 2008

Purpose : Apolipoprotein E (Apo E) plays a major role in lipoprotein metabolism and lipid transport. Many investigators have described that Apo E polymorphisms is one of the most important genetic determinants for cardiovascular disease. The purpose of this study was to evaluate the association between Apo E polymorphisms and serum lipid profiles in obese adolescent. Methods : We measured the serum concentrations of glucose, apolipoprotein (Apo) A1, Apo B, total cholesterol (TC), triglyceride (TG), HDL and LDL-cholesterol after overnight fasting in obese adolescent. Apo E polymorphisms were determined by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP). Results : 86 obese adolescents participated in this study. The body mass index (BMI) of participants were excess of 95 percentile by age and sex. Male to female ratio was 1.7 and mean age of study group was $16.2{\pm}1.8\;years$. Mean BMI was $27.4{\pm}2.5kg/m^2$. The frequency of ${\varepsilon}2$, ${\varepsilon}3$ and ${\varepsilon}4$ allele were 8.1%, 87.2% and 4.7% respectively. Study populations were classified into the following three genotypes 1) Apo E2 group (n=13, 15.1%) carrying either the ${\varepsilon}2/{\varepsilon}2$ or ${\varepsilon}2/{\varepsilon}3$ 2) Apo E3 group (n=65, 75.6%) carrying the most frequent ${\varepsilon}3/{\varepsilon}3$ 3) Apo E4 group (n=8, 9.3%) carrying either the ${\varepsilon}3/{\varepsilon}4$ or ${\varepsilon}4/{\varepsilon}4$. No differences were found among Apo E genotypes concerning age, sex, weight, height and BMI. Apo B and LDL-cholesterol concentrations were significantly higher in the Apo E4 group (P<0.05). No association were found between Apo E genotypes and glucose, Apo A1, TC, TG and HDL. Conclusions : We confirmed that serum concentrations Apo B and LDL-cholesterol were influenced by Apo E genotypes. Apo E polymorphisms seems to influence some alteration of lipid metabolism associated with obesity in adolescent.

• Clinical and Experimental Pediatrics
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• v.51 no.1
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• pp.67-72
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• 2008

Purpose : In this study, we retrospectively analyzed the clinical outcomes of patients who underwent allogeneic hematopoietic stem cell transplantation (HSCT) grafted from HLA-matched parents. Methods : Seven children with acute leukemia (4 acute lymphoblastic leukemia, 3 acute myeloid leukemia) in first complete remission received allogeneic HSCT from their respective parents at the St. Marys Hospital between April, 1999 and October, 2005. The median age of patients at transplantation was 5 years (range, 1-11 years; 2 male, 5 female) and the median age of donors was 35 years (range, 30-41 years; 5 male, 2 female). We investigated the clinical outcomes such as engraftment, acute and chronic graft-versus-host disease (GVHD), transplant-related morbidity and mortality, relapse and survival. Results : Median time from transplantation to last follow-up was 69.5 months (range, 18.8-96.5 months). All patients were successfully engrafted, with a median time of 11 days (range, 10-16 days) and 26 days (range, 13-39 days) for neutrophil and platelet recovery, respectively. Grade II acute GVHD occurred in 3, and grade III acute GVHD in 1 of 7 recipients. Extensive chronic GVHD developed in 2, and limited chronic GVHD in 1 of 7 recipients. Death from transplant-related complications occurred in 1, and relapse occurred in 1 of 7 recipients. Estimated 5-year overall survival was $83{\pm}15%$. Conclusion : The clinical outcomes of recipients who underwent HSCT from HLA-matched parents were comparable to those of patients who received HSCT grafted from HLA-matched sibling donors in childhood leukemia. HLA typing of parents, as well as siblings will increase the likelihood of finding an HLA-matched family donor for patients who need HSCT.

• Clinical and Experimental Pediatrics
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• v.50 no.1
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• pp.28-32
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• 2007

Purpose : Human angiotensin converting enzyme (ACE) gene shows an insertion/deletion polymorphism in 16 intron, and three genotypes are determined by whether a 287 bp fragment of the DNA is present or not; II, ID and DD genotype. DD genotype has been suggested as a risk factor of chronic nephrotic disease such as IgA nephropathy and diabetic nephropathy, various cardiovascular diseases and several other diseases. ACE activity increases in acute hepatitis, chronic persistent hepatitis, chronic active hepatitis and cirrhosis. On the other hand, patients with fatty livers have normal ACE activity. This study was designed to find out the relation between polymorphsims of the ACE genes and neonatal hyperbilirubinemia in Koreans. Methods : The genomic DNA was isolated from 110 full-term Korean neonates who had hyperbilirubinemia with no obvious causes (serum bilirubin$${\geq_-}12mg/dL$$) and 164 neonates of a control population (serum bilirubin <12 mg/dL). We performed polymerase chain reaction (PCR) to see the allele of the ACE gene. Electrophoresis was done in the PCR products in 1.5 percent agarose gel, and then DNA patterns were directly visualized under ethidium bromide staining. Results : ACE genotypes in the hyperbilirubinemia group are as follows; 26.36 percent for II, 53.64 percent for ID, 20.00 percent for DD, 0.532 for I allele and 0.468 for D allele. These distributions were not significantly different from those in the control group; 24.39 percent for II, 51.83 percent for DI, 23.78 percent for DD, 0.503 for I allele and 0.497 for D allele. Conclusion : In this study, ACE gene polymorphism was detected in the neonatal hyperbilirubinemia and control group. The most frequent genotype was ID. Our results indicate that the ACE gene polymorphism is not associated with the prevalence of neonatal hyperbilirubinemia in Koreans.

• Clinical and Experimental Pediatrics
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• v.50 no.1
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• pp.65-73
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• 2007

Purpose : Short stature is an important complication that impairs the quality of life in survivors of childhood brain tumors. We studied their final adult height (FAH) to evaluate risk factors for short stature. Methods : We reviewed the medical data of 95 survivors of childhood brain tumors (64 males and 31 females) who had been followed up from 1982 to 2006, reached FAH, and had a more than five year-disease-free survival. Results : Final adult height standard deviation score (FAHTSDS: $mean{\pm}SD$) of the patients was lower than those of general population ($-1.15{\pm}1.72$), HTSDS at diagnosis ($-0.13{\pm}1.57$), and target HTSDS ($-0.49{\pm}0.69$). FAHTSDS of craniopharyngioma patients did not decrease ($0.57{\pm}1.17$), but those of germ cell tumor and medulloblastoma patients were significantly reduced ($-1.20{\pm}1.45$, $-2.70{\pm}1.46$; P<0.05). The patients treated with craniospinal radiation or chemotherapy had lower FAHTSDS ($-1.93{\pm}1.58$, $-2.27{\pm}1.44$; P<0.01). In the spinal irradiation group, the younger the age at diagnosis was, the more the loss of FAH (r=0.442, P<0.01). Growth hormone replacement (GHR) didn't improve FAHTSDS, but starting GHR under 12 years was an independent factor for improving FAH once treatment methods were taken into account (P=0.01). Conclusion : The younger age at diagnosis, spinal radiation and chemotherapy were all important risk factors of height loss, and height gain was expected in patients who received GHR under the age of 12 years. Therefore, regular check-ups of growth and early intervention with growth hormones are needed for high risk groups to improve FAH.

• Clinical and Experimental Pediatrics
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• v.50 no.11
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• pp.1110-1115
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• 2007

Purpose : Idiopathic central diabetes insipidus (CDI) is defined in CDI patients without definite etiology. Some patients initially diagnosed as idiopathic CDI progressed to organic causes. We reviewed clinical, endocrinological, and radiological courses of 20 patients who was initially diagnosed as idiopathic CDI, to assess the predicting factors for progression to brain tumors. Methods : We reviewed the medical data and followed up their clinical courses in 20 CDI patients who had no definite organic etiology, such as malformation, tumor, at the time of diagnosis. Results : Our study included 15 males and 5 females. Mean age of CDI diagnosis was $7.8{\pm}3.6$ (2.1-14.7) years. Mean follow-up duration was $8.6{\pm}5.1$ (1.5-18) years. Six (30%) patients were diagnosed as brain tumor during follow-up. Ten (50%) of 20 patients had growth hormone deficiency. Multiple pituitary hormone deficiencies were found more frequently in brain tumor patients than idiopathic patients (60% vs 7%, P=0.037). Pituitary stalk thickening (PST) and loss of posterior pituitary signal were observed in 9 patients (47%), respectively. The newly development of PST was observed in patients diagnosed as brain tumor. Conclusion : About 30% of idiopathic CDI patients progress to organic disease such as germ cell tumor or histiocytosis. If there are multiple anterior pituitary hormone deficiency or newly development of PST, more close and careful follow-up is needed.

• Clinical and Experimental Pediatrics
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• v.50 no.11
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• pp.1104-1109
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• 2007

Purpose : Since 1998, school urinary screening tests have been performed on Korean school children. We could detect and treat so many asymptomatic chronic renal disease in early stage. We investigated the efficacy of school urinary screening tests from children with membranoproliferative glomerulonephritis (MPGN) type I. Methods : We analyzed the characteristics and prognosis of 18 patients with MPGN type I who admitted after 1996 and received steroid therapy with or without cyclosporine. These patients were divided into two groups. Group A (asymptomatic patients detected by school urinary screening tests) consisted of 7 patients; Group S (symptomatic patients) consisted 11 patients. Results : Mean follow-up duration was 6.3 years (from 2 to 11 years). Urinary protein excretion was 1.1 g/day in group A and 6.6 g/day in group S. 24 hour creatinine clearance (mL/min/$1.73m^2$) was 134.3 in group A and 82.3 in group S. No patients in group A had renal insufficiency, but three patients in group S had renal insufficiency and one patient required peritoneal dialysis. Conclusion : Early detection by school urinary screening tests improves prognosis of MPGN type I.

• Clinical and Experimental Pediatrics
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• v.46 no.2
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• pp.178-182
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• 2003

Purpose : Brain tumors are the second most common tumor in childhood, and medulloblastomas comprise 15-25% of brain tumors. The well known prognostic factors are age at diagnosis, stage of disease, and extent of surgical excision. In this study, we analysed the prognostic factors in patients who received chemotherapy after excision. Methods : We reviewed the medical records of 61 patients who received chemotherapy among the 94 patients who were diagnosed and treated between Jan 1985 and Sep 2001 in the Department of Pediatrics and Neurosurgery at Severance Hospital. Results : Among the total survival rate of patients who underwent chemotherapy, the 3-yr progression-free survival rate was $66.5{\pm}6.3%$ and the 15-yr progression-free survival rate was $60.3{\pm}6.7%$. The progression-free survival rate for patients with age at diagnosis over 3 yrs old and under 3 yrs old, was $64.5{\pm}7.7%$ and $48.2{\pm}12.9%$ respectively and there was no statistically significant difference. The survival rate of the high vs low risk group by staging was $72.7{\pm}10.5%$ and $54.6{\pm}8.3%$ respectively, and there was no significant difference. The survival rate of patients with total removal vs subtotal removal was $65.8{\pm}11.8%$ and $56.8{\pm}8.2%$ respectively, showing no statistical difference. Conclusion : The reason there is no difference in survival rate according to the traditional prognostic factors is that chemotherapy has improved not only the total survival rate but also the survival rate in patients with poor traditional prognostic factors. So, sufficient removal of tumor followed by proper chemotherapy and radiotherapy is an important factor which influences the survival rate of medulloblastoma patients.

• Journal of Korean Academy of Nursing
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• v.3 no.3
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• pp.97-110
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• 1973

The goal of modern nursing is to provide comprehensive nursing care to patients. If comprehensive nursing care to children (within the hospital setting) is to be provided, consideration of the stage of growth and development of the child is especially important. From clinical observation, it appeared that nurses often disregarded individual requirements of children in giving nursing care. Therefore, the purpose of this study is to show that comprehensive nursing care which is based on an understanding of the growth and development of the child contributes to both the child and the mother's adaptability to the child's hospitalization. Method: Sixty children, three to three year of age, hospitalized at the Yonsei University Pediatric Ward ware studied. From April 1, 1973 to May 5, 1973, children admitted to the hospital were assigned to either an experimental or a compare groups. There were 30 children in each group. The sex and age of the children in each group was similar. In both groups were more male than female children. In the experimental group, each mother stayed with hot child continuously during his hospitalization. In the compare groups, the mother or some other member of the family stayed with the child. Each day on the child's admission the investigator visited the ward from 1-2 P.M. to 9-10 P.M., in order to provide comprehensive care for the experimental -group. The assistance given the nurses by the investigator was in the form of conferences regarding care and in giving direct care to the child and his mother. The compare group of children received nursing care as usually provided by the hospital. The instruments used to obtain the data for analysis were as follows: 1. The fear and anxiety reaction of the child was recorded by observation of the investigator for four areas: 1) separation from parent and relatives 2) reaction to Doctor and Nurse with white gowns 3) reaction to nursing care 4) reaction to injection and tests, etc. 2. Regression in area of eating, sleeping, and elimination were recorded by the investigator by questioning the mother and by observation. 3. Adaptability to the hospitalization was recorded by direct questioning of the children for areas of emotional and social adjustment. For children older than 3 years of age or children not seriously ill, using the simple I. Q. test this was possible for only 35 of the total 60 children. Result: 1. 55 percents of the total 60 children had been prepared by their parents for hospitalization. The children who had received prior preparation accepted hospitalization more readily than those who had received no preparation. (χ²=4.6 Ρ<0.05) 2. On admission 31.7 percent of the children expressed verbal fear of their discase or treatment. 25 percent felt that the disease was due to their mistake. 3. There was a significant difference in the reaction of the child to separation from the parent or relatives between the two groups. The experimental groups showed less anxiety due to separation than the compare group. (χ²=4.34 Ρ<0.05) In both groups there was less anxiety due to separation among school age (6-12 years) children than among preschool age (3-5 years) children. (χ²=9.22 Ρ<0.05) 4. More than half of the children in both groups reacted with fear and avoidance to doctor and/or nurses wearing white gowns. (χ²=0.06 Ρ<0.05) 5. The experimental group reacted more favorably to nursing in general than the compare group. (χ²=4.8 Ρ<0.05) 6. There was no difference in the fear and refused reaction to special tests and/or such as X-rays and injections, etc. between the groups. (χ²=3.77 Ρ<0.05) 7. More children in the compare group showed regressive tendencies in eating, sleeping, and elimination habits than in experimental groups. (χ²=2.3 Ρ<0.05 χ²=3.88 Ρ<0.05 χ²=4.9 Ρ<0.05) 8. There was a significant difference in the adaptability to hospitalization between the two groups. The experimental groups adapted more readily. (χ²=2.02 Ρ<0.05) 9. For children who had higher I.Q. s the adaptability to hospitalization was better regardless of the group. (χ²=5.03 Ρ<0.05) However, because of the small number of cases (60), this finding cannot be extrapolated without further verification. The date demonstrates that there was a greater adaptability to hospitalization by the child when comprehensive nursing care was given. By planning care and applying knowledge of growth and development to meet, nurses are in a position to prevent some of the psychological trauma associated with hospitalization.

• Childhood Kidney Diseases
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• v.5 no.1
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• pp.36-42
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• 2001

Purpose : Vesicoureteral reflux is the most commonly inherited disease detected in children with urinary tract infection. The incidence of vesicoureteral reflux among siblings of children with known vesicoureteral reflux is 8$\%$ to 45$\%$ according to different authors. Family screening of a patient with vesicoureteral reflux is important in order to prevent reflux nephropathy. The purpose of this study is to determine the incidence of vesicoureteral reflux in asymptomatic family of children with vesicoureteral reflux and the factors which influence the family history. Methods : The study group consisted of 27 families of patients with vesicoureteral reflux. The total number in the group were 79 persons. BUN, Cr, urineanalysis, voidingcystourethrography(VCUG) and 99mTc -dimercaptosuccinic acid(DMSA) renal scan were performed oil tile siblings. As for tile parents the same tests were performed except the VCUG. Results : The abnormality was detected in 7 of 27 families(25.9$\%$). Vesicoureteral reflux was detected in 5 of 20 siblings and renal scar ns detected in 3 of 32 parents. In children with vesicoureteral reflux, renal scar was detected in 24 of 32 children. Between the group with the abnormality in its family(Group A) and the group without the abnormality in its family(Group B), There was no difference of creatinine clearance between two groups. More renal scars were detected in group A according to the DMSA(A:100$\%$, B:75$\%$. t-test P<0.05). There was no difference of grade of VCUG between two groups. There was no difference between one site and both sites in two groups. In tile case of tile siblings with vesicoureteral reflux, there was high incidence of renal scar in a patient with vesicoureteral reflux according to the DMSA. Conclusion : It is important to screen vesicoureteral reflux and renal scar in case of urinary tract infection to prevent reflux nephropathy. This study implies that it is necessary to screen the family of a patient with vesicoureteral reflux especially with renal scar. (J, Korean Soc Pediatr Nephrol 5 : 36- 42, 2001)