No abstract available.
Ascites* ; Pancreatitis, Chronic* ; Pleural Effusion*

No abstract available.
Stevens-Johnson Syndrome*

Cranial fasciitis is a rare fibroblastic tumor which shows a predilection for the scalp and skull of youngchil-dren. Histologically, cranial fasciitis is identical to nodular fasciitis which is typically found in theextremities of adults, though is unique in that it may present as an osteolytic lesion of the skull. We report acase of cranial fasciitis in a 20-month-old girl, describing the ultrasonography, CT and MRI findings.
Adult ; Child* ; Fasciitis* ; Female ; Fibroblasts ; Humans ; Infant ; Magnetic Resonance Imaging ; Scalp ; Skull ; Ultrasonography

PURPOSE: To determine (1) the relationship between the cortical defects seen on 99 mTc-DMSA renal scans and age, and (2) the presence and degree of vesicoureteral reflux, and then to depict the risk factors for cortical defects in children with acute urinary tract infection (UTI). Furthermore, to assess the diagnostic value of VCUG in predicting a defect on 99 mTc-DMSA renal scans. MATERIALS AND METHODS: We studied 134 kidneys in 67 children aged 15 days-10 years (M:F=39:28) in whom symptomatic UTI was present. In all these children, both DMSA renal scans and voiding cystourethrography (VCUG) were performed. Scanning took place within 7 days of diagnosis and VCUG was performed after one month of diagnosis. Scintigraphic findings were graded according to the extent and number of cortical defects. We evaluated the relationships between the cortical defects seen on DMSA scans and age, and the grade of vesicoureteral reflux. The diagnostic value of VCUG in predicting cortical defects was analysed. Results: The prevalence of cortical defects was greater in patients older than two years (38/54, 70%) than in those aged less than two (38/80, 48%). The frequency of cortical defects was related to vesicoureteral reflux (p<0.05) and grade of reflux (p<0.05). As this latter increased, the extent of cortical defects also increased (p<0.05), and DMSA scans revealed the presence of these in 76 of the 134 kidneys (57%) with acute UTI. In 30 of these 76 (39.5%), VCUG demonstrated the presence of vesicoureteral reflex. On the other hand, vesi-coureteral reflex was found in 36 of the 134 kidneys (27%), and in 30 of these 36 (83%), cortical defects were noted. The sensitivity of VCUG in predicting cortical defect was 39.5%, while specificity was 89.7%. The positive predictive value for defects was 83.3%, and the negative predictive value was 53.1%. The relative risk of cortical defect in the presence of vesicoureteral reflux was 1.78. CONCLUSION: Renal cortical defects are significantly related to age and grade of vesicoureteral reflux. Risk factors for developing cortical defects were older age (> or =2yrs) at the time of acute UTI, and high grade of vesicoureteral reflux. The specificity of VCUG in predicting cortical defects is relatively high but the sensitivitiy is low, and a significant proportion of cortical defects therefore occurred in the absence of vesicoureteral reflux.
Child* ; Diagnosis ; Hand ; Humans ; Kidney ; Prevalence ; Radionuclide Imaging* ; Reflex ; Risk Factors* ; Sensitivity and Specificity ; Succimer* ; Urinary Tract Infections* ; Urinary Tract* ; Vesico-Ureteral Reflux

Cystic masses of the adrenal gland are unusual in the fetus and most are secondary to hemorrhage. Cysticneuroblastoma is extremely rare, and in contrast to solid neuroblastoma, follows a benign course, is diagnosedearlier, and rarely presents with metastatic lesions(1-4). We report one case of cystic neuroblastoma diagnosedprenatally by ultrasound(US) and magnetic resonance(MR) imaging, and include a review of the literature.
Adrenal Glands ; Fetus ; Hemorrhage ; Neuroblastoma*

PURPOSE: To evaluate, using magnetic resonance (MR) imaging, the clinal significance of the corpus callosum by measuring the size of various portions of the corpus callosum in children with cerebral palsy, and in paired controls. MATERIALS AND METHODS: Fifty-two children [30 boys and 22 girls aged between six and 96 (median, 19) months in whom cerebal palsy was clinically diagnosed underwent MR imaging. There were 23 term patients and 29 preterm, and the control group was selected by age and sex matching. Clinal subtypes of cerebal palsy were classified as hemiplegia(n =14), spastic diplegia(n=22), or spastic quadriplegia(n=16), and according to the severity of motor palsy, the condition was also classified as mild(n=26), moderate(n=13), or severe(n=13). In addition to the length and height of the corpus callosum, the thickness of its genu, body, transitional zone and splenium, as seen on midsagittal T1-weighted MR images, were also measured. Differences in the measured values of the two groups were statistically analysed and differences in the size of the corpus callosum according to the clinical severity and subtypes of cerebral palsy, and gestational age, were also assessed. RESULTS: Except for height, the measured values of the corpus callosum in patients with cerebral palsy were significantly less than those of the control group (p < 0.05). Its size decreased according to the severity of motor palsy. Compared with term patients, the corpus callosum in preterm patients was considerably smaller (p< 0.05). CONCLUSION: There was statistically significant correlation between the severity of motor palsy and the size of the corpus callosum. Quantitative evaluation of the corpus callosum might be a good indicator of neurologic prognosis, and a sensitive marker for assessing the extent of brain injury.
Brain Injuries ; Cerebral Palsy* ; Child ; Corpus Callosum* ; Evaluation Studies as Topic ; Female ; Gestational Age ; Humans ; Magnetic Resonance Imaging ; Muscle Spasticity ; Paralysis ; Prognosis

Two external quality assessment (EQA) trials of conventional newborn screening tests for phenylketonuria, galactosemia, congenital adrenal hyperplasia, maple syrup urine disease, homocystinuria, and congenital hypothyroidism, as well as newborn screening tests using tandem mass spectrometry, were performed in 2015. A total of 44 specimens in the form of dried blood spots were distributed to 16 laboratories and the response rate of these laboratories was 100%. The mean, standard deviation, coefficient of variation, median, and cut-offs were evaluated for each analyte in the newborn screening tests. Two EQA trials for the analyses of methylmalonic acid, vanillylmandelic acid, catecholamines, metanephrines, organic acids, and amino acids were also performed. A well-designed EQA program and continuous education would improve the performance of biochemical genetics tests.
Adrenal Hyperplasia, Congenital ; Amino Acids ; Catecholamines ; Congenital Hypothyroidism ; Education ; Galactosemias ; Homocystinuria ; Humans ; Infant, Newborn ; Korea* ; Maple Syrup Urine Disease ; Mass Screening ; Methylmalonic Acid ; Molecular Biology* ; Phenylketonurias ; Tandem Mass Spectrometry ; Vanilmandelic Acid

Two trials of external quality assessment (EQA) of conventional newborn screening tests for phenylketonuria, galactosaemia, congenital adrenal hyperplasia, maple syrup urine disease, homocystinuria, and congenital hypothyroidism, as well as newborn screening tests were performed using tandem mass spectrometry in 2014. A total of 39 specimens in the form of dried blood spots were distributed to 16 laboratories and the response rate of these laboratories was 100%. Screening tests for phenylketonuria and congenital hypothyroidism did not meet the accepted performance criteria in some laboratories. The mean, standard deviation, coefficient of variation, median, and cut-offs were evaluated for each analyte in the newborn screening tests. Two trials of EQA for the analyses of methylmalonic acid, vanillylmandelic acid, catecholamines, metanephrines, organic acids, and amino acids were also performed. A well-designed EQA program and continuous education would improve the performance of biochemical genetic testing.
Adrenal Hyperplasia, Congenital ; Amino Acids ; Catecholamines ; Congenital Hypothyroidism ; Education ; Homocystinuria ; Humans ; Infant, Newborn ; Korea ; Maple Syrup Urine Disease ; Mass Screening ; Methylmalonic Acid ; Molecular Biology* ; Phenylketonurias ; Tandem Mass Spectrometry ; Vanilmandelic Acid

Four trials of external quality assessment in diagnostic hematology were performed in 2002 with about 400 participating laboratories in Korea. We performed quality assessment for white blood cell count, hemoglobin, red blood cell count, platelet count, reticulocyte count, white cell differential count, and red blood cell morphology test. The response rate was more than 90%. The performance of quality assessment appeared to be gradually improved year by year.
Equidae* ; Erythrocyte Count ; Erythrocytes ; Hematology* ; Korea* ; Leukocyte Count ; Platelet Count ; Reticulocyte Count

Four trials of external quality assessment in diagnostic hematology were performed in 2004 with about 440 participating laboratories in Korea. We performed quality assessment for white blood cell count, hemoglobin, red blood cell count, platelet count, white cell differential count, red blood cell morphology and coagulation test. The response rate was more than 96%. The coefficients of variation in hemoglobin and RBC number was stable but variable in platelet number and WBC number according to measuring cell counts. Blood coagulation study was performed twice. Test results show wide variation according to measuring machine and reagents.
Blood Coagulation ; Cell Count ; Equidae* ; Erythrocyte Count ; Erythrocytes ; Hematology* ; Indicators and Reagents ; Korea* ; Leukocyte Count ; Platelet Count