Crossed renal ectopia is a congenital malformation in which both kidneys lie on the same side of the spine, usually side by side longitudinally. More often on the right side. Fusion of the two renal units is eight times more common than nonfusion. Although crossed renal ectopia is uncommon, this unusual entity must be considered in an infant when cystic mass in the abdomen or pelvis paticularly if no kidney can be found on the opposite side. In many cases of crossed fused ectopia with multicystic dysplastic kidney(MCDK), the diagnosis can be strongly suspected from the sonogram, and no other studies may be necessary. However, both intravenous urography and isotope renography is useful to assess the function of the crossed kidney. Crossed renal ectopia and MCDKs are associated with a greater incidence of ureteropelvic junction obstruction and reflux. So, screening voiding cystourethrography should be performed. Very few studies of MCDK in the setting of crossed fused ectopia have been reported. We have experienced a 3-year-old boy with crossed fused renal ectopia with multicystic dysplasia.
Abdomen ; Humans ; Incidence ; Infant ; Kidney ; Mass Screening ; Multicystic Dysplastic Kidney ; Pelvis ; Preschool Child ; Radioisotope Renography ; Spine ; Urography

BACKGROUND: The purpose of the study was to investigate prospectively the relation of lipoprotein(a)[Lp(a)],apoproteins and serum lipid parameters to restenosis after percutaneous transluminal coronary angioplasyt(PTCA). METHODS: One hundred and forty-five patients who received successful PTCA were enrolled and their serum levels of lipids, apoproteins and Lp(a) were measured before PTCA. After 6 months of follow-up, the patients were reevaluated for the development of restenosis by coronary angiography, treadmill test or thallium scan. RESULTS: A total 137 patients could be followed. Restenosis occurred in 71 patients(52%). Clinical parameters(e.g. age, sex, hypertension, diabetes, smoking) and angiographic parameters(e.g. lesion site, type and degree of stenosis) were not significantly different between the group without restenosis and the group with restenosis. Lipid parameters and apoproteins were not associated with restenosis. Lp(a) and Lp(a) phenotype analysis showed no significant difference between the two gruops. CONCLUSION: Serum lipid parameters, apoproteins and Lp(a) concentration are not associated with restenosis after PTCA and cannot be used as predictios of restenosis.
Angioplasty, Balloon, Coronary* ; Apoproteins ; Coronary Angiography ; Exercise Test ; Follow-Up Studies ; Humans ; Hypertension ; Lipoprotein(a)* ; Phenotype* ; Prospective Studies ; Thallium

BACKGROUND: Many studies evaluating the performance of in vitro diagnostic kits have been criticized for the lack of reliability. To attain reliability those evaluation studies should be preceded by sample size calculation ensuring statistical power. This study was intended to develop a web-based system to estimate the sample size, which was often neglected because it would require expert knowledge in statistics. METHODS: For sample size calculation, we extracted essential parameters from the performance studies on the 3rd generation anti-hepatitis C virus (HCV) kits reported in the literature. We developed a system with PHP web-script language and MySQL. The statistical models used in this system were as follows; one sample without power consideration (model 1), one sample with power consideration (model 2), and two samples with power consideration (model 3). RESULTS: Among the articles published between 1989 and 2005, 13 articles that evaluated the performance of anti-HCV kits were identified by searching with Medical Subject Headings (MeSH). The diagnostic sensitivity was 83-100% with a median of 145 samples (range; 12-1,091) and the specificity was 97-100% with a median of 1,025 samples (range; 33-4,381). The estimated sample size would be 280 in the model 1, 817 in the model 2, and 1,510 in the model 3, when we set 2% prevalence of HCV infection, 95% sensitivity of a conventional kit, 97% sensitivity of a new kit , 95% significance level (two-sided test), 2% allowable error, and 80% power. CONCLUSIONS: Our study indicates that an insufficient sample size is still a problem in performance evaluation. Our system should be helpful in increasing the reliability of performance evaluation by providing an appropriate sample size.
Medical Subject Headings ; Models, Statistical ; Prevalence ; Sample Size* ; Sensitivity and Specificity

Loeffler's endocarditis is generally caused by hypereosinophilic syndrome. It is a restrictive cardiomyopathy characterized with eosinophilia and eosionophilic penetration leading to the fibrous thickening of endocardium of both ventricles, apical obliteration and heart failure. We report a case of a 23-year-old male with Loeffler's endocarditis caused by Churg-Strauss syndrome. The echocardiogram showed that biventricular failure with large thrombus in left ventricle. His symptoms and typical echocardiographic findings markedly improved within 2 months after treatment for Churg-Strauss syndrome.
Cardiomyopathy, Restrictive ; Churg-Strauss Syndrome ; Endocardium ; Eosinophilia ; Heart Failure ; Heart Ventricles ; Humans ; Hypereosinophilic Syndrome ; Male ; Thrombosis ; Young Adult

Cholineacetyltrasnferase (ChAT) is a key enzyme that facilitates synthesis of acetylcholine affecting the memory, learning, awakening and sleep process of the cerebrum. The object of this study was to test the hypothesis that the ChAT-gene 2384G>A (rs3810950) polymorphism is associated with Alzheimer's disease (AD) susceptibility, and galantamine response. To elucidate a genetic predisposition of AD, we studied ChAT-gene 2384G>A (rs3810950) polymorphism in 52 AD patients in 93 normal controls. We also examined the association of this polymorphism and galantamine therapeutic response in 52 AD patients who received a 24-week galantamine treatment. There were no significant differences in the genotype or allele frequency of the ChAT polymorphism between the AD and control groups. However, we found that the allele-carrier distributions, allele frequency for the ChAT polymorphism differed significantly between responders and non-responders. The frequency of A-allele carriers (GA+AA) was higher in responders than in non-responders (chi-square=4.282, df=1, p=0.039), as was the A-allele frequency (chi-square=5.216, df=1, p=0.022). These results suggest that the ChAT-gene 2384G>A (rs3810950) polymorphism is associated with galantamine therapeutic response.
Acetylcholine ; Alzheimer Disease ; Cerebrum ; Galantamine ; Gene Frequency ; Genetic Predisposition to Disease ; Genotype ; Humans ; Learning ; Memory

Insulinomas are the most common type of islet cell tumor. Generally, these tumors are benign (90%), intrapancreatic, solitary, and small. Moreover, hyperinsulinemia in infants and children can result in permanent damage to the central nervous system. Thus, early diagnosis and treatment are important. The principal challenge during anesthesia is the avoidance of hypoglycemia, which may occur during tumor manipulation, though hyperglycemia may follow after successful surgical removal of the tumor. Because symptoms of hypoglycemia (systemic hypertension, tachycardia, diaphoresis) may be masked during anesthesia, it is important to monitor blood glucose levels frequently during the perioperative period. Here we report up on the case of a 3 year old female with insulinoma. We monitored blood glucose levels intermittently (about every 15 minutes) using a glucometer (Medisense Optium, Abbott, USA). No hypoglycemic episode occurred during anesthesia. We report this clinical experience and review anesthetic choices and the management of this patient.
Adenoma, Islet Cell ; Anesthesia ; Blood Glucose ; Central Nervous System ; Child ; Child, Preschool ; Early Diagnosis ; Female ; Humans ; Hyperglycemia ; Hyperinsulinism ; Hypertension ; Hypoglycemia ; Infant ; Insulinoma* ; Isoflurane ; Masks ; Pancreas ; Perioperative Period ; Tachycardia

OBJECTIVES: Serotonin transporter (5-HTT) is a key synaptic regulator of serotonergic neurotransmission and a major site of action of serotonin selective reuptake inhibitors (SSRIs) such as fluoxetine or paroxetine. Two PCR-fomatted polymorphisms at this locus have been described, the first of which is a repeat sequence polymorphism located in the promoter region (5-HTT gene-linked polymorphic region, 5-HTTLPR), and the second is a variable number tandem repeat located in intron2 (STin2). 5-HTTLPR insertion/deletion polymorphism with long (l) and short (s) forms affects the transcriptional efficiency of 5-HTT transporter expression. We examined the pharmacodynamic characteristic of 5-HTT gene polymorphism in the patients with major depression, which was expressed in the peripheral platelet. METHODS: 5-HTT gene polymophisms and pharmacodynamic characteristics of 5-HTT in the platelet was measured in 41 patients with major depression defined by DSM IV criteria and 35 healthy normal volunteers. 5-HTT gene polymophisms were analyzed with the primers flanking the regulatory region and the second intron from genomic DNA. Pharmacodynamic characteristics of 5-HTT in the platelet was measured with [3H]-serotonin uptake study. The uptake of [3H]-serotonin was quantified with Vmax and Km value. RESULTS: We found that the Vmax value of 5-HTT in peripheral platelet was higher in the patients with s/s genotype (2.17 pmol/10(4) platelets/min, 1.53-3.90 pmol/10(4) platelets/min) than with s/l (1.73 pmol/10(4) platelets/min, 0.83-3.40 pmol/10(4) platelets/min) or l/l (1.0(4) pmol/10(4) platelets/min, 0.88-1.31 pmol/10(4) platelets/min) genotype of 5-HTTLPR. Normal subjects with s/s genotype also had significantly higher Vmax value than those with s/l or l/l genotype. However, STin2 genotype showed no significant association with Vmax or Km in both groups. CONCLUSIONS: These results suggest that allelic variation of 5-HTT gene affects the phenotypic expression of 5-HTT in human platelet, and may be linked with phenotypic heterogeneity in the antidepressant responsiveness in depressed patients. This is another different finding based on ethnic variation with respect to pharmacodynamic characteristics of 5-HTT gene polymorphism.
Blood Platelets* ; Depression ; DNA ; Fluoxetine ; Genotype ; Healthy Volunteers ; Humans ; Introns ; Paroxetine ; Population Characteristics ; Promoter Regions, Genetic ; Regulatory Sequences, Nucleic Acid ; Serotonin Plasma Membrane Transport Proteins* ; Serotonin* ; Synaptic Transmission ; Tandem Repeat Sequences

BACKGROUND AND OBJECTIVES: The factors related to the progression of mild aortic stenosis (AS) remain unknown. We wanted to evaluate the long-term outcomes and predictors of echocardiographic progression in patients with mild AS. SUBJECTS AND METHODS: We prospectively included 103 consecutive asymptomatic patients (62.1+/-11.9 years, 31 males) with mild AS. Mild AS was defined as aortic valve (AV) thickening accompanied by a peak aortic jet velocity (AV Vmax) > or =2.0 and <3.0 m/sec, and rapid progression of AS was defined as an average annual increase in the AV Vmax > or =0.2 m/sec, and cardiac events were defined as cardiac death or AV replacement. RESULTS: During a median echocardiographic follow-up time of 6.0 years, the average change in the AV Vmax was 0.08+/-0.10 m/sec per year. The rate of progression was significantly associated with age, moderate-to-severe AV calcification and the baseline AV Vmax, but not with the serum cholesterol level. The baseline AV Vmax (2.6+/-0.3 m/sec vs. 2.2+/-0.3 m/sec, respectively, p<0.001) and the incidence of moderate-to-severe AV calcification (92.9% vs. 36.5%, respectively, p<0.001) were significantly higher in the rapid progression group than in the slow progression group. The 7-year cardiac event-free survival rate was lower in the rapid progression group than in the slow pro-gression group (87.5+/-8.3% vs. 100%, respectively). CONCLUSION: The progression of AS was slower than expected and it was related to age, the baseline AV Vmax and AV calcification. Because of the marked individual variability in progression, the patients showing rapid progression of AS need closer follow-up.
Aortic Valve ; Aortic Valve Stenosis ; Cholesterol ; Death ; Disease-Free Survival ; Follow-Up Studies ; Humans ; Incidence ; Prognosis ; Prospective Studies

PURPOSE: To investigate the changes of voiding methods according to the time after discharge and to clarify the reason of changing voiding method. MATERIALS AND METHODS: One hundred and seventeen patients with spinal cord injury were enrolled. We classified these patients into five groups such as normal voiding, reflex voiding, intermittent catheterization, Foley catheter, and suprapubic cystostomy group. We interviewed the patients for necessary information including current voiding method and reason of changing voiding method. RESULTS: 50% of intermittent catheterization patients changed to reflex voiding method. 66% of these patients changed the voiding method without consult with their physician. Higher percentage of the paraplegic patients with intermittent catheterization changed to reflex voiding than the quadriplegic patients. Moreover, female patients indwelt the catheters due to absence of external collecting device than males. CONCLUSION: It is necessary to follow up the voiding methods of patients with spinal cord injury more closely to avoid the following inadvertent complications.
Catheterization ; Catheters ; Cystostomy ; Female ; Follow-Up Studies ; Humans ; Male ; Reflex ; Spinal Cord Injuries* ; Spinal Cord* ; Urinary Bladder ; Urinary Catheterization

PURPOSE: To establish the proper approach and management of an urachal anomaly in children. MATERIALS AND METHODS: We retrospectively reviewed the medical records and imaging studies of 33 children (21 boys, 12 girls) treated for an urachal anomaly over a ten-year period. RESULTS: Twenty-four patients were equally diagnosed with either an urachal sinus or urachal cyst; the other nine patients were confirmed to have a patent urachus. Umbilical discharge (14 patients) and umbilical granuloma (9 patients) were the most common presentations. The 12 patients with an urachal sinus underwent ultrasonography (USG) (10; diagnostic), 2 fistulography (all; diagnostic). Those with an urachal cyst underwent either USG (6/12; diagnostic), computed tomography (CT) (3; diagnostic), fistulography (2; diagnostic), or magnetic resonance imaging (MRI) (1; diagnostic). One subject was affected by acute appendicitis, which was confirmed by CT. Of the 9 children with patent urachus, 7 underwent USG (all; diagnostic); exploration without further imaging studies was performed on the remaining 2 subjects. Surgical excision was performed in 30 patients. Omphalomesenteric duct or Meckel's diverticulum were incidental findings, which were simultaneously repaired. Conservative treatment was successful in only 3 patients. CONCLUSIONS: Urachal anomalies in children mainly manifest as umbilical discharges and umbilical granuloma, but may present non-specific symptoms in some cases. USG is a useful method for diagnosis, but other imaging modalities can be useful to establish the differential diagnosis. A limited number of children with urachal anomalies, mainly presenting with an umbilical discharge, can be managed conservatively. However, complete surgical excision of the lesion, with the possible associated anomalies, should be the basic scheme for children with urachal anomalies.
Appendicitis ; Child* ; Diagnosis ; Diagnosis, Differential ; Granuloma ; Humans ; Incidental Findings ; Magnetic Resonance Imaging ; Meckel Diverticulum ; Medical Records ; Retrospective Studies ; Ultrasonography ; Urachal Cyst ; Urachus ; Vitelline Duct