No abstract available.
Hernia, Umbilical*

No abstract available.
Angioedema*

No abstract available.
Female ; Meigs Syndrome*

PURPOSE: The American Society for Apheresis provides clinical guidelines for therapeutic apheresis in adults, but there are no guidelines for children. This study aimed to analyze the effect of therapeutic plasma exchange (TPE) in pediatric patients with various kidney diseases in Korea. METHODS: We retrospectively reviewed the data of 16 children (up to 18 years of age) who were admitted to Severance Children's Hospital with refractory kidney disease. All patients received TPE between 1994 and 2016. Clinical and laboratory characteristics such as age, weight, sex, change in blood urea nitrogen (BUN), and creatinine level before and after TPE, and complications after TPE were analyzed. RESULTS: The mean age and weight of the 16 patients at the time of TPE was 11.3±4.0 years and 34.6±17.5 kg, respectively. The BUN level was 35.4 mg/dL before TPE and significantly decreased to 21.5 mg/dL (P=0.025) at 1 week and 20.5 mg/dL (P=0.01) at 1 month after TPE. The creatinine level significantly decreased from 1.20 mg/dL before TPE to 0.90 mg/dL (P=0.02) at 1 week after TPE. Four complications (hypovolemia, anemia, hypocalcemia, and thrombocytopenia) were reported, but were not fatal. CONCLUSION: Our findings suggest that TPE is an effective therapeutic modality in children with refractory kidney disease and can be indicated for the treatment of various kidney diseases.
Adult ; Anemia ; Blood Component Removal ; Blood Urea Nitrogen ; Child ; Creatinine ; Humans ; Hypocalcemia ; Kidney Diseases* ; Kidney* ; Korea* ; Pediatrics ; Plasma Exchange* ; Plasma* ; Retrospective Studies

PURPOSE: The American Society for Apheresis provides clinical guidelines for therapeutic apheresis in adults, but there are no guidelines for children. This study aimed to analyze the effect of therapeutic plasma exchange (TPE) in pediatric patients with various kidney diseases in Korea. METHODS: We retrospectively reviewed the data of 16 children (up to 18 years of age) who were admitted to Severance Children's Hospital with refractory kidney disease. All patients received TPE between 1994 and 2016. Clinical and laboratory characteristics such as age, weight, sex, change in blood urea nitrogen (BUN), and creatinine level before and after TPE, and complications after TPE were analyzed. RESULTS: The mean age and weight of the 16 patients at the time of TPE was 11.3±4.0 years and 34.6±17.5 kg, respectively. The BUN level was 35.4 mg/dL before TPE and significantly decreased to 21.5 mg/dL (P=0.025) at 1 week and 20.5 mg/dL (P=0.01) at 1 month after TPE. The creatinine level significantly decreased from 1.20 mg/dL before TPE to 0.90 mg/dL (P=0.02) at 1 week after TPE. Four complications (hypovolemia, anemia, hypocalcemia, and thrombocytopenia) were reported, but were not fatal. CONCLUSION: Our findings suggest that TPE is an effective therapeutic modality in children with refractory kidney disease and can be indicated for the treatment of various kidney diseases.
Adult ; Anemia ; Blood Component Removal ; Blood Urea Nitrogen ; Child ; Creatinine ; Humans ; Hypocalcemia ; Kidney Diseases* ; Kidney* ; Korea* ; Pediatrics ; Plasma Exchange* ; Plasma* ; Retrospective Studies

BACKGROUND: The automated border detection(ABD) echocardiography has the ability of recognizing the endocardial-blood interface, and therefore, on-line estimation of the left ventricular(LV) volume every cardiac cycle. Compared with the off-line conventional 2-dimensional echocardiographic method that requires tracing the endocardial border manually, the ABDd system can be a convenient and objective method in the estimation of the LV volume and the ejection fraction(EF). The purpose of this study is to compare the LV volume and EF between the on-line ABD system and the convertional off-line echocardiographic method. METHODS: In two weeks, 83 adult patients older than 16 years of age were referred to our echocardiographic laboratory. Among these 83 patients, 64 patients who had a normal sinus rhythm were included to our study. Using the Hewlett-Packard SONOS 1500, a 2.5 MHz transducer was placed dat the cardiac apex. Patients with an apical 4 chamber view of the LV in which at least 75% of the endocardium was clearly seen were selected for study. On that view, the ABD system was turned on, and the reansmit power and the time-gain compensation controls were adjusted in order to approximate the automated border to the visually apparent endocardial surface. The LV end-diastolic volume(LVESV) and LV end-systolic volume(LVESV) were calculated by the method of disc. LVEDV, LVESV, and EF were displayed every cardiac cycle. Also the off-line estimation of the LV volume was performed by the method of disc, after manually tracing the endocardial border on the apical 4 chamber view. RESULTS: 44 patients(69%) of 64 patients had > or =75% of the LV endocardium visualized. LVEDV, LVESV, and Ef with the ABD system were highly correlated with those with the off-line, manually traced method(r=0.95, 0.8, respectively), but LVEDV and EF with toe ABD system were significantly less than those with the latter(p<0.01). The limits of agreement between tow methods(off-line, manually traced method-ABD system) were somewhat wide. Those of LVEDV, LVESV and EF were +22~-10ml(mean 6ml), +15~-14ml(mean 0.1ml), and +19~-12%(mean 3.8%), respectively. CONCLUSION: LVEDV,LVESV, and EF measurements by the ABD system and the off-line manually traced methods thve a strong correlation, The ABD system should habe clinical applications in setting, in which measurements of LV volume and Ef are important, But, the comparison with a more reliable method is necessary.
Adult ; Compensation and Redress ; Echocardiography* ; Endocardium ; Humans ; Toes ; Transducers

Purpose: To compare the Doppler sonographic findings of the left renal vein (LRV) of children diagnosed with nutcracker syndrome with and without orthostatic proteinuria. Methods: Fifty and 53 consecutive children with and without orthostatic proteinuria, respectively, underwent renal Doppler ultrasonography examinations. The peak velocity (PV) was measured at the hilar portion of the LRV and between the aorta and superior mesenteric artery. Renal Doppler ultrasonography findings and clinical data including urine protein-to-creatinine ratio (UPCR) were compared according to the presence or absence of orthostatic proteinuria. Results: Between the two groups, no significant differences were observed in terms of age or sex. The PV ratio between the aortomesenteric and hilar portions was 7.79±2.65 and 6.32±3.01 in children with and without orthostatic proteinuria, respectively (P=0.009). No significant differences were observed between the two groups in terms of the UPCR in the first morning urine sample. However, the UPCR in the afternoon urine sample was significantly higher in children with orthostatic proteinuria than in those without orthostatic proteinuria (0.49±0.46 vs. 0.11±0.04 mg/mg, P<0.001). Furthermore, the PV ratio between the aortomesenteric and hilar portions revealed a positive correlation with the ratio of UPCR of the afternoon and that of first morning urine samples (R=0.21, P=0.034). Conclusions This study suggests that there can be a significant correlation of the PV ratio between the aortomesenteric and hilar portion of the LRV with orthostatic proteinuria in pediatric patients with nutcracker syndrome.

PURPOSE: Podocytes are important architectures that maintain the crucial roles of glomerular filtration barrier functions. Despite this structural importance, however, the mechanisms of the changes in podocytes that can be an important pathogenesis of minimal change nephrotic syndrome (MCNS) are not clear yet. The aim of this study was to investigate whether apoptosis is induced by interleukin (IL)-13 in cultured human podocytes. METHODS: Human podocytes were treated with different IL-13 doses and apoptotic cells were analyzed using terminal deoxynucleotidyl transferase dUTP nick-end labeling (TUNEL assay) and fluorescence-activated cell sorting (FACS). RESULTS: The IL-13 increased the number of TUNEL-positive cells in a dose-dependent manner at 6 and 18 hours (P<0.05 and P<0.05, respectively). The apoptosis rate was appeared to be increased slightly in the IL-13-stimulated podocytes (8.63%, 13.02%, and 14.46%; 3, 10 and 30 ng/mL, respectively) than in the control cells (7.66%) at 12 hours by FACS assay. CONCLUSION: Our study revealed that IL-13 expression may increase podocyte apoptosis. Blocking the IL-13 signal pathway can potentially play an important role in regulating the apoptosis of podocytes.
Apoptosis ; DNA Nucleotidylexotransferase ; Flow Cytometry ; Glomerular Filtration Barrier ; Humans ; Interleukin-13 ; Interleukins ; Nephrosis, Lipoid ; Podocytes ; Signal Transduction

Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease that affects multiple organs. More than half of the patients with SLE have kidney involvement, and up to 10% of patients with lupus nephritis develop end-stage renal disease (ESRD). Central nervous system (CNS) involvement in SLE occurs in 21% to 95% of patients. Severe neurological manifestations such as seizures, cerebrovascular disease, meningitis, and cerebrovascular accidents can develop in childhood-onset SLE, but cerebral infections, such as brain abscess and hemorrhage, are seldom reported in lupus nephritis, even in adults. Here, we report a rare case of childhood-onset SLE with ESRD, a cerebral abscess, and hemorrhage. A 9-year-old girl diagnosed with lupus nephritis was administered high-dose steroids and immunosuppressant therapy to treat acute kidney injury (AKI) and massive proteinuria. The AKI deteriorated, and after 3 months, she developed ESRD. She received hemodialysis three times a week along with daily peritoneal dialysis to control edema. She developed seizures, and imaging showed a brain abscess. This was complicated by spontaneous cerebral hemorrhage, and she became unstable. She died shortly after the hemorrhage was discovered. In conclusion, CNS complications should always be considered in clinical practice because they increase mortality, especially in those with risk factors for infection.

BACKGROUND: In the patients with thoracic injury, we suspect simultaneous cardiac contusion or concussion. We analyzed the patients with possible cardiac injury by electrocardiography, serum creatine kinase (CK), creatine kinase isoenzyme fraction (CK-MB) screening, followed by two dimentional echocardiogram (2-DE) to access the severity of injury. MATERIAL AND METHOD: From January 1997 to April 1998, 15-month retrospective study of suspicious myocardial injury was undertaken in including 24 patients admitted for suspected cardiac injury. All patients with history or signs of blunt chest injury were checked serially and the serial CK, CK-MB fraction, electrocardiography (EKG) analysis screening were followed by 2-DE. RESULT: The age range was between 20-40 years and were predominant male patients in(M:F=3:1). Most common causes of injury were traffic accidents, 15 patients(62.5%). Associated injuries involved multiple rib fractures, sternal fracture and such. EKG findings on the cardiac concussion were within normal limits, EKG findings on the cardiac contusion were nonspecific ST and T wave abnormality. In cardiac contusion patients, CK-MB fraction did not increase significantly on admission but on 2nd, 3rd, 4th hospital days, it increased significantly (p=0.0080, 0.0130, 0.0130). The average admission days were 9.22 in concussion and 26.18 in contusion patients(p=0.0075). Most common complication was the adult respiratory distress syndrome(7 cases), 5 out of the patients with ARDS were mechanically ventilated. There were no deaths. CONCLUSION: We believe the serial checks of CK-MB, EKG and subsquent two-dementional echocardiographic sector scanning are presently the most sensitive indicators available for structural and functional cardiac injury.
Accidents, Traffic ; Adult ; Commotio Cordis* ; Contusions* ; Creatine Kinase ; Echocardiography ; Electrocardiography ; Heart Injuries ; Humans ; Male ; Mass Screening ; Retrospective Studies ; Rib Fractures ; Thoracic Injuries