• Clinical and Experimental Pediatrics
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• v.49 no.3
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• pp.287-291
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• 2006

Purpose : Aneurysms of sinus valsalva are rare anormalies thought to be primarily congenital in origin, progressing into death by acute heart failure in cases of rupture. Surgical correction is the only method of treatment. With these clinical implications, we reviewed the clinical characteristics and surgical results of patients with ruptured sinus of valsalva aneurysm. Methods : Between January 1991 and February 2004, 17 patients with ruptured sinus of valsalva aneurysm were retrospectively reviewed for their clinical symptoms, physical findings, past history, coexistent cardiac anormalies, surgical results, and mid-term prognosis. Results : The 17 patients included 13 men and four women, with a mean age of 30 years(10-59 years). Preoperatively accompanying cardiac anormalies were ventricular septal defect(VSD, eight cases of doubly committed juxta-arterial VSD) and aortic insufficiency(11 cases). During operations, patterns of fistulous tracts were found to be right colonary sinus-right ventricle in 13 patients, right coronary sinus-right atrium in one, noncoronary sinus - right ventricle in two, noncoronary sinus - right atrium in one, and VSD was noticed in 14 patients(all were doubly committed juxta-arterial in type). The defects were closed with a patch in 13 patients, without a patch in four, with concommitant aortic valve replacement in four and with aortic valvuloplasty in two. There were no mortalities during operations or the mid-term follow-up periods($40{\pm}49$ months). Conclusion : Because, at least in Orientals, VSD(especially doubly committed juxta-arterial) was accompanied in large numbers of patients with aneurysms of sinus valsalva, preoperative evaluations of this congenital heart disease should be made very careful. And we may need to revise the algorithm of treatment policy in small sized doubly committed juxta-arterial VSD.

• Clinical and Experimental Pediatrics
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• v.45 no.8
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• pp.961-966
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• 2002

Purpose : We compared the underlying or associated diseases according to the frequency of platelet transfusions in neonates with thrombocytopenia to know the factors predicting which patients will require multiple platelet transfusions. We also compared mortality. Methods : A retrospective study was performed in 72 neonates who received the platelet transfusions in neonatal intensive care unit(NICU) between August 1996 and July 2001. Group I received one platelet transfusion and group II received two or more. We compared the frequency of underlying or assodiated diseases such as sepsis/disseminated intravascular coagulopathy(DIC), respiratory distress syndrome(RDS), intraventricular hemorrhage(IVH), patent ductus arteriosus (PDA), necrotizing enterocolitis(NEC), liver or renal disease, and mortality between two groups. Results : Of the 72 patients, 29(40.2%) received one and 43(59.7%) received two or more transfusions; 16(22.2%) received four or more. There were no statistically significant differences in gestational age, birth weight, sex, and maternal history between two groups. C-section rate was higher in group II(20.7% vs. 55.8%, P<0.05) and the incidence of PDA was higher in group I (55.2% vs. 30.2%, P<0.05). Otherwise, there were no statistically significant differences in the incidence of sepsis/DIC, RDS, IVH, RDS, CLD, NEC, liver or renal disease, pulmonary hemorrhage and hypoxic ischemic encephalopathy, and mortality between group I and group II. Conclusion : There was no significant difference in clinical morbidity and mortality according to the frequency of platelet transfusion in neonates with thrombocytopenia. Further study is needed to know the predicting factor for multiple platelet transfusions in neonates with thrombocytopenia.

• Clinical and Experimental Pediatrics
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• v.45 no.9
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• pp.1090-1096
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• 2002

Purpose : We evaluated the clinical manifestations, bronchoscopic findings and therapeutic effects of flexible fiberoptic bronchoscopy in atelectasis of children. Methods : Sixty six children who received bronchoscopy due to persistent atelectasis, acute severe atelectasis and incidental atelectasis on plain chest radiography were studied retrospectively. Results : The most common causative underlying disease was pneumonia(60.4%). Other underlying conditions were pulmonary tuberculosis, chronic lung disease, postoperative state, bronchial asthma and chest trauma. The most common abnormal findings were inflammatory changes such as bronchial stenosis(n=15), mucosal edema and large amount of secretion(n=14), granulation tissue( n=3) and mucus plug(n=3) although 39.4% showed normal airways. Other findings were congenital airway anomalies, endobronchial tuberculosis, extrinsic compression and obstruction by blood clot. In 32 children with pneumonia-associated atelectasis, 43.7% revealed normal airways, and the most common abnormal findings were also inflammatory changes. Eighteen out of 39 patients who received therapeutic intervention such as suctioning of secretion, bronchial washing and intrabronchial administration of N-acetylcysteine($Mucomyst^{(R)}$) had complete or partial resolution of their atelectasis. In 32 patients with pneumonia-associated atelectasis, 56.5% showed improvement by therapeutic intervention. Conclusion : In this study, atelectasis was mainly associated with inflammatory airway diseases such as pneumonia. The most common abnormal bronchoscopic findings were inflammatory changes such as mucosal edema and large amounts of secretion and bronchial stenosis, although about 40% revealed normal airway. Flexible bronchoscopy is helpful for either diagnosis or treatment, especially in pneumonia-associated atelectasis.

• Asian Pacific Journal of Cancer Prevention
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• v.17 no.9
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• pp.4517-4525
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• 2016

Background: Sperm DNA damage is underlying aetiology of poor implantation and pregnancy rates but also affects health of offspring and may also result in denovo mutations in germ line and post fertilization. This may result in complex diseases, polygenic disorders and childhood cancers. Childhood cancer like retinoblastoma (RB) is more prevalent in developing countries and the incidence of RB has increased more than three fold in India in the last decade. Recent studies have documented increased incidence of cancers in children born to fathers who consume alcohol in excess and tobacco or who were conceived by assisted conception. The aetiology of childhood cancer and increased disease burden in these children is lin ked to oxidative stress (OS) and oxidative DNA damage( ODD) in sperm of their fathers. Though several antioxidants are in use to combat oxidative stress, the effect of majority of these formulations on DNA is not known. Yoga and meditation cause significant decline in OS and ODD and aid in regulating OS levels such that reactive oxygen speues meditated signal transduction, gene expression and several other physiological functions are not disrupted. Thus, this study aimed to analyze sperm ODD as a possible etiological factor in childhood cancer and role of simple life style interventions like yoga and meditation in significantly decreasing seminal oxidative stress and oxidative DNA damage and thereby decreasing incidence of childhood cancers. Materials and Methods: A total of 131 fathers of children with RB (non-familial sporadic heritable) and 50 controls (fathers of healthy children) were recruited at a tertiary center in India. Sperm parameters as per WHO 2010 guidelines and reactive oxygen species (ROS), DNA fragmentation index (DFI), 8-hydroxy-2'-deoxy guanosine (8-OHdG) and telomere length were estimated at day 0, and after 3 and 6 months of intervention. We also examined the compliance with yoga and meditation practice and smoking status at each follow-up. Results: The seminal mean ROS levels (p<0.05), sperm DFI (p<0.001), 8-OHdG (p<0.01) levels were significantly higher in fathers of children with RB, as compared to controls and the relative mean telomere length in the sperm was shorter. Levels of ROS were significantly reduced in tobacco users (p<0.05) as well as in alcoholics (p<0.05) after intervention. DFI reduced significantly (p<0.05) after 6 months of yoga and meditation practice in all groups. The levels of oxidative DNA damage marker 8-OHdG were reduced significantly after 3 months (p<0.05) and 6 months (p<0.05) of practice. Conclusions: Our results suggest that OS and ODD DNA may contribute to the development of childhood cancer. This may be due to accumulation of oxidized mutagenic base 8OHdG, and elevated MDA levels which results in MDA dimers which are also mutagenic, aberrant methylation pattern, altered gene expression which affect cell proliferation and survival through activation of transcription factors. Increased mt DNA mutations and aberrant repair of mt and nuclear DNA due to highly truncatred DNA repair mechanisms all contribute to sperm genome hypermutability and persistant oxidative DNA damage. Oxidative stress is also associated with genome wide hypomethylation, telomere shortening and mitochondrial dysfunction leading to genome hypermutability and instability. To the best of our knowledge, this is the first study to report decline in OS and ODD and improvement in sperm DNA integrity following adoption of meditation and yoga based life style modification.This may reduce disease burden in next generation and reduce incidence of childhood cancers.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.169-177
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• 2002

Purpose: Hypertension accelerates the progression of chronic renal disease, whether it results from, or causes, the renal disease. Therefore, the control of hypertension is one of the important factors that retard the rate of renal deterioration. We compared the effects of different antihypertensive agents on renal function and glomerular morphology In subtotal nephrectomized rats. Materials and methods: After induction of chronic renal failure with 5/6 nephrectomy, the rats were divided into three groups; control group (Group C), enalapril group (Group E), and nicardipine group (Group N). Systolic blood pressure was measured by tail cuff method every 4 weeks until 12 weeks after nephrectomy. At 12 weeks after nephrectomy, all rats were placed in metabolic cages for 24 hour urine collections to measure urinary protein and creatinine excretion. After urine collection and blood sampling for serum creatinine, all rats were sacrificed. The renal tissue was processed for morphometric study with light microscope and electron microscope. Results: 1. The blood pressure of Group C increased progressively, but both enalapril and nicardipine prevented the development of hypertension, and the two drugs were equally effective in maintaining normal blood pressure throughout the study. 2. Twenty-four hour urinary protein excretion was lower in Group E compared to Group C and Group N 3. Mesangial expansion score in both treated groups were significantly lower than the control group. Mean glomerular volume in Group E was significantly reduced compared to Group C and Group N. There was no significant difference in mean glomerular volume between Group C and Group N. 4. There was no significant difference in podocyte structural changes, estimated by filtration slit length density, among control, enalapril and nicardipine treated groups. Conclusion: Control of hypertension with enalapril or nicardipine afforded considerable protection from mesangial expansion in the rat remnant kidney model. But protein excretion and glomerular growth were significantly reduced in Group E compared to Group N. There was no significant difference in podocyte structural changes among the 3 groups.

• Childhood Kidney Diseases
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• v.3 no.1
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• pp.72-79
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• 1999

Renal scarring associated with vesico-ureteral reflux(VUR) is one of the major causes of end stage renal failure and renal hypertension in children. Urinary ${\beta}_2$-microglobulin(MG) has been suggested as a potential marker for presence of renal tubular damage. This study was designed to evaluate the significance of random urine ${\beta}_2$-MG as a predictor of presence of vesico-ureteral reflux in children with urinary tract infection(UTI). 57 children with urinary tract infection were studied. Patients were devided into two groups; 35($78.9\%$) children have UTI without VUR and 12($21.1\%$) children have UTI and VUR. Beta2-MG and creatinine in random urine sample was measured to decide the excretion ratio(${\beta}_2$-MG/creatinine). Among the 57 children with UTI, 44 children were confirmed by urine culture study and 13 children suspected by compatible clinical feature. Random urine ${\beta}_2$-MG of VUR group ($2.2{\pm}5.91$ mg/L) were significantly higher than that of simple UTI group($0.19{\pm}0.16mg/L$)(P=0.03). The ${\beta}_2$-MG/creatinine ratio of VUR group($32.41{\pm}25.7$) were significantly higher than that of simple UTI group($3.93{\pm}3.44$)(P=0.007). In conclusion, random urine ${\beta}_2$-MG and excretion ratio deserved early predictor of presence of VUR in children with UTI. And this method was more simple and inexpensive than the method of measuring ${\beta}_2$-MG with 24 hour urine collection, so might be a useful screening test for VUR in children with UTI.

• Childhood Kidney Diseases
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• v.11 no.1
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• pp.41-50
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• 2007

Purpose : Dialysis in children with chronic renal failure presents with many difficulies. The purpose of this study is to find an improved method in chronic dialysis in infants and children less than 2 years of age by analyzing the experience with 10 cases. Methods : A retrospective review of the medical records of 10 patients(6 boys and 4 girls) was conducted. The patients had chronic renal failure and underwent chronic dialysis at Samsung medical center from March 1999 to February 2007. Results : At Initiation of dialysis, the median age was 3 months old(22 days-20 months), the median body weight was 3.75 kg(2.2-10.3 kg), and the median serum creatinine level was 4.3 mg/dL(2.0-11.4 mg/dL). The median duration of dialysis was 29.5 months(3-62 months). Dysplastic kidney disease was the most common underlying renal disease. Two patients were treated with hemodialysis, 4 patients with peritoneal dialysis, and 4 patients eventually switched dialysis modality. Nine of the 10 patients took erythropoietin and anti-hypertensive drugs. At the end of the follow up period, 1 patient received kidney transplantation, 2 patients died due to sepsis, and 5 patients were treated with peritoneal dialysis. Two patients were lost to follow up. The most common complication of dialysis was infection. Achieving vascular access and maintaining proper catheter function were the most important factors in treating patients with hemodialysis. The growth status of patients was aggravated after 6 month of dialysis but improved after 1 year of dialysis. Patients showed better growth on peritoneal dialysis than hemodialysis. Conclusion : Chronic dialysis can be performed successfully in infants and children under 2 years of age. Vascular access was the main limitation of hemodialysis, and infection was the common problem in both hemodialysis and peritoneal dialysis. To improve the patients survival rate and quality of life, major efforts should be directed toward the prevention of infection and preservation of catheter function. (J Korean Soc Pediatr Nephrol 2007;11:41-50)

• Clinical and Experimental Pediatrics
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• v.49 no.7
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• pp.769-776
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• 2006

Purpose : Pulse wave velocity(PWV) and ankle brachial index(ABI) are not only noninvasive methods used to assess arterial stiffness in adults, but also useful, simple ways to estimate the severity of hypertension, end stage renal disease and atherosclerosis in adults. But there are few studies on PWV and ABI in adolescents and children. In this study, hypertensive adolescents were compared to normal individuals to find out the usefulness of PWV as the index of progress in the disease. Methods : 413 teenagers were divided into three groups. The first group consisted of adolescents that only had hypertension(Group 1 : 23 teenagers). The second group consisted of the adolescents who had hypertension with obesity(Group 2 : 17 teenagers), and the last group was the normal subjects(Group 3 : 328 teenagers). Weight, height and body mass index were measured. Simultaneous measurements of systolic, diastolic and average blood pressure were obtained from the four extremities. PWV, ABI, ejection time and preejection period were also measured. Results : Right brachial-ankle PWV was significantly higher in both group 1 and group 2 compared with group 3, and significantly higher in group 2 compared with group 1. Likewise, left brachial-ankle PWV also showed significantly higher values in both group 1 and group 2 compared with group 3, and also higher values in group 2 compared with group 1. Positive correlations were found between the systolic, diastolic, average blood pressure and PWV. There were also positive correlations between the blood pressure and weight, body mass index. Conclusion : Higher PWV was demonstrated in adolescents with essential hypertension compared to normal subjects. Follow-up study is needed to evaluate the progress.

• Clinical and Experimental Pediatrics
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• v.46 no.1
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• pp.11-16
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• 2003

Purpose : Iron deficiency is still the most common nutrient deficient disorder despite the improvement in general health and nutrition. This study is designed to evaluate the dietary history of infants and young children with iron deficiency anemia(IDA) and the effects of nutritional counseling. Methods : This study was conducted on 120 children from 6 to 36 months of age with IDA. Their parents completed a questionnaire and took counsel for nutrition. IDA was defined as Hb <11.0 g/dL, ferritin <10 ng.mL or transferrin saturation <15%, or Hb increase >1 g/dL after iron preparation. The questionnaire consisted of their feeding patterns, weaning time and kinds of food. Results : In the 120 infants and young children aged from 6 to 36 months, the parents of 82 cases was counseled about nutrition. Fifty six infants among 82 cases have started weaning and the main foods of weaning were rice and/or rice gruel. Nutritional problems in weaning were that some children over one year of age were using a bottle, and parents restricted weaning food at will because of allergic disease or chronic disease. Most parents were satisfied with the nutritional counseling given from a clinical dietitian and showed good compliance. Conclusion : Many infants and young children with IDA were provided with non iron-fortified foods and made an inadequate wean. Most parents were satisfied with the nutritional counseling and showed good compliance. The need of dietary counseling was required for prevention and treatment of iron deficiency anemia because of inadequate weaning.

• Clinical and Experimental Pediatrics
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• v.53 no.2
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• pp.195-202
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• 2010

Purpose : Despite recent advances in pulmonary hypertension management and surgery, appropriate guidelines remain to be developed for operability in congenital heart disease with pulmonary artery hypertension (PAH). Our aim was to evaluate clinical outcomes of patients with severe PAH who underwent surgical closure of left-to-right shunt lesions (LRSL) on the basis of pulmonary reactivity. Methods : We retrospectively reviewed 21 patients who underwent surgical closure of LRSL with severe PAH (${\geq}8$ Wood unit) from January 1995 to April 2009. The median age at operation was 26 years. Atrial septal defect, ventricular septal defect (VSD), VSD and patent ductus arteriosus (PDA), and PDA was present in 11, 4, 4, and 2 patients, respectively. Results : Operability was based on vasoreactivity of PAH. Of the 21 patients, 5 showed response to pulmonary vasodilator therapy and 8 showed vasoreactivity after balloon occlusion of defects. The remaining 8 patients were considered operable because of significant left-to-right shunt (Qp/Qs ${\geq}1.5$). Five patients underwent total closure of defects and 16 were left with small residual shunts. The median follow-up duration was 32 months. There was no significant postoperative mortality or morbidity. Systolic pulmonary artery pressure (PAP) decreased in all but 2 patients. All patients except 1 showed improvement of New York Heart Association functional class. Conclusion : Closure of LRSL in patients with severe PAH on the basis of pulmonary vasoreactivity seems reasonable. PAP and clinical symptoms improved in most patients. Further research is needed for the evaluation of long-term results.