• Childhood Kidney Diseases
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• v.5 no.1
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• pp.43-50
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• 2001

Purpose : The urinary tract infection associated with vesicoureteral reflux(VUR) in children may result in serious complications such as renal scarring, hypertension, proteinuria and end stage renal disease. The purpose of this study was to evaluate the factors affecting renal scar such as age, gender, grade of VUR, and ACE gene polymorphism, and body growth in the patients with and those without renal scar associated with VUR Methods : During the period from January 1994 to July 2000, We had 93 children with urinary tract infection associated with VUR who were admitted to the Department of pediatrics of Chonbuk National University Hospital. The patients were divided into two groups according to follow up 99mTc-DMSA renal scan; patients with renal scar group and those with non-scar group. We analyzed and compared the factors associated with renal scarring between the two groups. Results : There were no significant difference in gender, causative organism, ACE gene polymorphism, height and weight at diagnosis between renal scar group and non-scar group. Fifty four patients were in renal scar group and forty seven of them had VUR. The age at diagnosis was significantly higher in renal scar group (2.48${\pm}$2.64yr) than in non renal scar group (1.26${\pm}$1.83yr). Especially, the infants who were less than 1 year of age with VUR developed relatively more renal scar compared with infants older than 1 tear of age. The incidence of renal scarring showed a direct correlation with the severity of VUR. Conclusion : The factors affecting renal scar formation were age at diagnosis, presence and grade of VUR, but the other factors such as gender, causative organism, ACE gene polymorphism were not associated with renal scarring. Therefore, further evaluation about uropathogenic E coli and foflow up study about body growth associated with severity of renal scar would be necessary. (J. Korean Soc Pediatr Nephrol 5 : 43- 50, 2001)

• Childhood Kidney Diseases
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• v.3 no.2
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• pp.196-202
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• 1999

Purpose : When the mature kidney fails to reach its norml location in the renal fossa, the condition is known as ectopic kidney. Presenting symtoms can be various and it generally depend on the associated anomaly. Beside urologic anomalies such as hydronephrosis and vesicoureteral reflux, various anomalous vascular net work, skeletal anomaly or genital anomaly can be observed in this condition. Methods : Sixteen children with ectopic kidney was studied retrospectively to analyse initial presentation, accompanied anomaly and prognosis. Results : 56% of the children were accompanied with other urologic anomalies such as true incontinence and vesicoureteral reflux that required surgical treatment. 31% of children were either diagnosed incidentally during evaluation of other non-urologic disease or during follow-up evaluation of abnormal antenatal renal sonogram. Conclusion : Ectopic kidney can be often misdiagnosed as tumorous condition or as a surgical condition depend on the abnormal location of the kidney. Careful evaluation using abdominal sonogram, DMSA, VCUG and abdominal CT scan should be performed in order to search for associated anomalous condition and for proper management.

• Childhood Kidney Diseases
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• v.8 no.2
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• pp.195-204
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• 2004

Purpose: Hypophosphatemic rickets is a hereditary disease, characterized by hypophosphatemia due to renal phosphate wasting, impaired renal production of 1,25-dihydroxyvitamin $D_3$, rachitic bone deformities and impaired growth. The purpose of this study is to provide clinical profiles of patients with hypophosphatemic rickets in our hospital. Methods: Between July 1983 and February 2004, 56 patients were diagnosed as having hypophosphatemic rickets. The medical records of these patients were reviewed retrospectively. Clinical manifestations, family histories, laboratory data, treatment outcomes were described. Results: Fifty six patients were enrolled in this study. The average age at symptom onset and diagnosis were 20 months and 5 years respectively. Fourteen patients had family histories. The main clinical manifestations were bow legs and short stature. There was a significant negative correlation between the ages and the height z-scores at the time of diagnosis(r=-0.47, P=0.005). Initial laboratory data showed normocalcemia, hypophosphatemia, elevated serum alkaline phosphatase, decreased tubular reabsorption of phosphate and a normal range of 1,25-dihydroxyvitamin $D_3$ Radiographic examinations of bone revealed fraying, widening and cupping of the metaphyseal ends. Treatment consisted of Joulie solution and vitamin D metabolites, and resulted in improved biochemical and radiographic findings. However, height z-scores remained essentially unchanged(P=0.224). Complications of treatment were frequently observed, including hyperparathyroidism, nephrocalcinosis, and hypercalciuria. Sixteen patients had corrective osteotomy and 4 of them underwent leg lengthening together. Conclusion: There was a gap of several years between the onset of symptoms and the diagnosis. Early treatment seems to be essential to growth. For the earlier treatment, the offsprings of affected parents should be followed up closely.

• Clinical and Experimental Pediatrics
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• v.48 no.1
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• pp.63-67
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• 2005

Purpose : Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP) is a systemic vasculitis, characterized by cutaneous palpable purpura, gastrointestinal(GI) symptoms, arthritis and renal involvement. In general, the prognosis is determined by GI complication as well as the severity of nephritis. In this study, we analyzed the statistical relationship between the GI symptom and other clinical findings for assessing the prognosis, and evaluated abdominal ultrasonographic findings for early diagnosis of this disease with atypical clinical presentation and early detection of serious GI complications. Methods : One hundred seventy seven patients with HSP in the Department of Pediatrics, Wonkwang University Hospital from January 1994 to June 2004, were enrolled. We retrospectively analyzed charts about clinical and abdominal ultrasonographic findings, and classified our patients into two groups(GI-Sx(-), GI-Sx(+)) for statistical analysis. Results : The ratio of female to male is 1.5 : 1. The peak age incidence was five to eight years in 95 cases(53%). The GI symptoms appeared in 117 cases(66%), which include abdominal pain 115 (98 %), tenderness 45(38%), nausea and vomiting 35(30%), bloody stool 10(8.5%), diarrhea four(3.4%), rebound tenderness four(3.4%), and also intussusception and appendicitis were complicated in five and two cases respectively. GI-Sx(+) group had an increased risk of renal involvement and relapse than the GI-Sx(-) group. But there were no relationships about sex and age incidence, or other clinical and laboratory findings between two groups. Ultrasonographic findings in 98 patients with GI symptoms included small bowel thickening in 70 cases(71%) in which duodenum, jejunum and ileum were involved in 71%, 45.7%, 40% respectively, small bowel dilatation in 41 cases(42%), lymph node swelling in 46 cases(47%), and ascites in 25 cases(25.5%). Conclusion : GI symptoms in patients with HSP suggested increased risk of renal involvement and relapse. Abdominal ultrasonography could be helpful in the early diagnosis on atypical clinical presentation and early detection of serious GI complication in these patients.

• Clinical and Experimental Pediatrics
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• v.49 no.5
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• pp.500-506
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• 2006

Purpose : We evaluated an outbreak of Serratia marcescens infections in 24 neonates in a neonatal intensive care unit(NICU). Methods : From January to August, 2004 a nosocomial outbreak of S. marcescens occurred in our NICU. We describe the clinical characteristics of the outbreak and analyse the risk factors for infections with S. marcescens. After the outbreak stopped, 7 isolates from blood were typed using rapid amplified polymorphic DNA analysis(RAPD). Results : S. marcescens was isolated from 24 neonates, 19 infected and 5 colonized. Seven out of nineteen neonates had bacteremia, 4 had ventilator associated pneumonia, 4 had purulent conjunctivitis, 2 had UTI, 1 had meningitis and 1 had a wound infection. Three neonates died due to S. marcescens infection, 2 of 3 had ventilator associated pneumonia, 1 had meningitis complicated with abscess. The mortality rate of S. marcescens infection was 15.8%. Factors associated with S. marcescens infections were previous antibiotic therapy, indwelling catheter and use of ventilators. The isolated strains were resistant to most antibiotics, but frequently sensitive to imipenem, bactrim and amikacin. RAPD typing results show that at least 3 epidemic strains were related with this outbreak. But one genotype was predominant type in this outbreak. The control measures were instituted and the outbreak stopped within 2 months. Conclusion : S. marcescens can cause rapidly spreading outbreaks associated with fatal infections in neonates. If S. marcescens is isolated from clinical specimens, meticulous infection control measures and epidemiologic investigations should be done at an early stage of the outbreak.

• Clinical and Experimental Pediatrics
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• v.48 no.11
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• pp.1232-1238
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• 2005

Purpose : Though more than 15 years have passed after introduction of Lorenzo's oil therapy in X-linked adrenoleukodystrophy(ALD), its efficacy is still fully not known. In patients who already have neurologic symptoms, most reports indicate that the neurologic disability continues to increase. We evaluated its efficacy in X-linked ALD patients. Methods : Four patients who were treated with Lorenzo's oil for at least 12 months were enrolled from 1996 to 2003. During treatment, changes of neurologic symptoms, brain magnetic resonance imaging(MRI) findings, and serum saturated very long-chain fatty acid(C26:0) were assessed. Results : Two patients with childhood cerebral ALD had progression of neurologic symptoms and MRI lesions during treatment. One asymptomatic patient developed childhood cerebral ALD after six year treatment of Lorenzo's oil. One "Addison only" patient remained neurologically intact after three years. During Lorenzo's oil therapy, serum C26:0 levels which had increased at diagnosis decreased in all four patients. Conclusion : Treatment with Lorenzo's oil did not prevent disease progression in childhood cerebral ALD patients who were already symptomatic. Long term follow up will be needed in asymptomatic ALD including "Addison only" to reveal the efficacy of Lorenzo's oil.

• Clinical and Experimental Pediatrics
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• v.49 no.6
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• pp.643-647
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• 2006

Purpose : Recently, incidences of chronic cough with allergic disease have been increased. The identification of specific allergens for chronically coughing children is significant for diagnosis, proper treatment and prevention. The aim of this study was to identify significant contributors for sensitization in chronically coughing children. Methods : A total of 106 children, aged 1-6 years, visiting for chronic cough(>3 weeks), were tested for inhalant multiple allergosorbent assay(MAST), eosinophil count and total IgE. Parents completed a questionnaire on past history of atopy(infantile eczema) and bronchiolitis, family history of allergy, breastfeeding, smoking, pets and floor coverings at home. Results : Significant contributors for sensitization were : age(4-6 years), infantile eczema, positive family history of allergy and smoking by family. Conclusion : We were able to identify significant contributors for sensitization in chronically coughing children.

• Clinical and Experimental Pediatrics
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• v.46 no.7
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• pp.661-667
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• 2003

Purpose : This study was performed to find the chief clinical problems associated with the ages of adult patients of tetralogy of Fallot(TOF) who had undergone total correction. Methods : Of the 30 patients who were registered at the Grown-Up Congenital Heart Disease (GUCH) Clinic of Samsung Medical Center for TOF, a retrospective investigation was carried out on 28 patients who underwent total correction. Results : Mean age at retrospective study was 30.8(range : 16-53) years old. Age at total correction was 15.8(range : 2-49) years old. Problems after corrective surgery were assessed. They were arrhythmia, pulmonary valve regurgitation, left pulmonary artery stenosis, residual ventricular septal defect, mitral valve regurgitation, tricuspid valve regurgitation, right ventricle outflow tract obstruction, aortic valve regurgitation, infective endocarditis and protein losing enteropathy. After repair of TOF, such arrhythmias as atrial arrhythmia and AV conduction disturbances were observed in some patients. Cardiomegaly was found significantly in the subjects with arrhythmia(P<0.05), and arrhythmia was less observed in patients who underwent surgery at a young age. Eight patients required a reoperation; the main indications were residual ventricular septal defect, right ventricle outflow tract obstruction and peripheral pulmonary artery stenosis. Conclusion : The majority of the patients seemed to live normal lives after Tetralogy of Fallot repair. However, as residual anatomic and functional abnormalities exist postoperatively, continued careful follow-up is needed to detect and correct structural and functional abnormalities.

• Clinical and Experimental Pediatrics
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• v.46 no.5
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• pp.484-489
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• 2003

Purpose : To understand morphologic and hemodynamic variations in patients with pulmonary atresia with intact ventricular septum(PA/IVS), and to decide the best treatment modalities, we measured right ventricular volume, inflow, and outflow valvular annulus size in these patients and compared them with those of normal newborns. Methods : Eight patients with PA/IVS diagnosed by echocardiography from January to December 2001 were enrolled in this study. Among the total eight patients, five were male and three were female. The mean age of patients was 6.9 days(1-34 days), and the mean body weight was 3,343 gm (2,970-4,000 gm). Ten fullterm newborn infants with sepsis or hyperbilirubinemia without heart disease were enrolled as a control group. Echocardiographic and Doppler studies using Acuson Aspen (7Mh probe) were recorded on super-VHS videotape and later on, with review mode. We measured volumes of right and left ventricles, aortic, pulmonic, mitral and tricuspid valvular annulus sizes using an installed program, and then these parameters were compared with those of the control group. Results : Mean Z-value of tricuspid valvular annulus in PA/IVS was $-3.69{\pm}2.80$(-8.4--0.45), and tricuspid/mitral valvular annulus size ratio $0.68{\pm}0.15$(0.43--0.84). The more the tricuspid/mitral valvular annulus size ratio, the more Z-value of tricuspid valvular annulus(P=0.003, r=0.885). Those patients who underwent pulmonary valvuloplasty(balloon or surgical) had a tendency toward larger volume of the right ventricle, more Z-value of pulmonic and tricuspid valvular annulus, and more tricuspid/mitral valvular annulus size ratio than those patients who underwent a shunt operation. Conclusion : Compared to a measurement of the volume of the right ventricle, measurements of tricuspid/mitral valvular annulus size ratio and Z-value of tricuspid valvular annulus may be easier and better parameters to decide the treatment method and to predict prognosis in PA/IVS patients.

• Clinical and Experimental Pediatrics
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• v.46 no.5
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• pp.454-458
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• 2003

Purpose : Rotavirus is the main cause of infantile diarrheal disease worldwide. The purpose of this study is to assess the difference between clinical manifestations and feeding or delivery methods in healthy full-term neonates and those with nosocomially-acquired rotaviral infection. Methods : The study was conducted on 348 babies who were delivered in Dongguk University Kyongju Hospital from Jan 1 to June 30 in 2001. Stool specimens were collected from clinically symptomatic neonates. We studied the rate of positive stool rotazyme ELISA tests and positive symptoms. We compared the frequency of clinical manifestations according to the feeding methods [breast feeding(BF) or formula feeding(FF)] and the delivery methods[normal vaginal delivery(NVD) or Cesarean section(C/sec)]. Results : Rotavirus was detected in 152 of 348 babies. The rate of positive rotazyme ELISA test was 43.7% in healthy full-term symptomatic neonates. The most common symptom of rotaviral enteritis was mild fever; the others were watery diarrhea, abdominal distension, vomiting, delayed weight gain, irritability, poor oral intake and dehydration. Compared with FF neonates, BF neonates have lower frequency of symptoms, especially watery diarrhea, delayed weight gain and poor oral intake. The symptomatic frequency was higher in FF and NVD neonates than BF and C/sec. Conclusion : The symptoms of rotaviral enteritis were less frequent in BF or C/sec delivered neonates. BF appeared to alleviate the rotaviral enteritis but further studies are needed. The cause of the lower frequency of symptoms in C/sec delivered neonates was unknown.