99mTc-MAG3 Scintigraphic Scan is sensitive at depicting focal parenchymal abnormalities and can be used for the measurement of overall renal function. We experienced a 12-year-old girl presenting with fever and flank pain. On the ultrasonogram and post-voiding delayed image of 99mTc-MAG3 scintigraphic scan, severe right cortical atrophy and hydronephrosis with vesicoureteral reflux were detected. We could demonstrate the reflux nephropathy by these two diagnostic work-up without conventional voiding cystourethrography.
Atrophy ; Child ; Female ; Fever ; Flank Pain ; Humans ; Hydronephrosis ; Technetium Tc 99m Mertiatide ; Ultrasonography ; Vesico-Ureteral Reflux

PURPOSE: Vesicoureteral reflux(VUR) is known to be the main cause of childhood hypertension and renal failure. Knowing its familial occurrence, we determined the incidence of VUR and renal scar in asymptomatic siblings of Korean patients with primary VUR. METHODS: A total of 50 siblings from 37 index patients were included. Voiding cystourethrography(VCUG) and renal scintigraphy using 99mTc-DMSA were performed in these siblings from June, 1994 to May, 2001. Index patients were classified into two groups according to the presence of VUR in their siblings, and the clinical factors of the index patients such as age, sex, grade of reflux and renal cortical defect were compared between the groups. RESULTS: Among the 50 siblings, VUR were found in 8(16%) and renal cortical defects were detected in 8(16%) siblings respectively. The incidence of renal cortical defects was 87.5%(7 out of 8) in the VUR(+) siblings. There was a case of VUR(-) cortical defect in one sibling, presumed as a scar from an old VUR. There was no relationship among age, sex, grade of reflux and renal cortical defect of the index patient to the presence of VUR in siblings. CONCLUSION: This study confirmed a significant incidence of VUR(16%) and renal cortical defects(16%) in the asymptomatic siblings of patients with primary VUR in Korea. It is resonable to recommend screening studies to the siblings of patients with VUR for the early detection and prevention of probable reflux nephropathy.
Cicatrix* ; Humans ; Hypertension ; Incidence* ; Korea ; Mass Screening ; Radionuclide Imaging ; Renal Insufficiency ; Siblings* ; Technetium Tc 99m Dimercaptosuccinic Acid ; Vesico-Ureteral Reflux*

PURPOSE: Vesicoureteral reflux(VUR) is known to be the main cause of childhood hypertension and renal failure. Knowing its familial occurrence, we determined the incidence of VUR and renal scar in asymptomatic siblings of Korean patients with primary VUR. METHODS: A total of 50 siblings from 37 index patients were included. Voiding cystourethrography(VCUG) and renal scintigraphy using 99mTc-DMSA were performed in these siblings from June, 1994 to May, 2001. Index patients were classified into two groups according to the presence of VUR in their siblings, and the clinical factors of the index patients such as age, sex, grade of reflux and renal cortical defect were compared between the groups. RESULTS: Among the 50 siblings, VUR were found in 8(16%) and renal cortical defects were detected in 8(16%) siblings respectively. The incidence of renal cortical defects was 87.5%(7 out of 8) in the VUR(+) siblings. There was a case of VUR(-) cortical defect in one sibling, presumed as a scar from an old VUR. There was no relationship among age, sex, grade of reflux and renal cortical defect of the index patient to the presence of VUR in siblings. CONCLUSION: This study confirmed a significant incidence of VUR(16%) and renal cortical defects(16%) in the asymptomatic siblings of patients with primary VUR in Korea. It is resonable to recommend screening studies to the siblings of patients with VUR for the early detection and prevention of probable reflux nephropathy.
Cicatrix* ; Humans ; Hypertension ; Incidence* ; Korea ; Mass Screening ; Radionuclide Imaging ; Renal Insufficiency ; Siblings* ; Technetium Tc 99m Dimercaptosuccinic Acid ; Vesico-Ureteral Reflux*

Background: Niemann-Pick disease type C (NPC) is caused by the mutation of NPC genes, which leads to the abnormal accumulation of unesterified cholesterol and glycolipids in lysosomes. This autosomal recessive disease is characterized by liver dysfunction, hepatosplenomegaly, and progressive neurodegeneration. Recently, the application of induced neural stem cells (iNSCs), converted from fibroblasts using specific transcription factors, to repair degenerated lesions has been considered a novel therapy. Objectives: The therapeutic effects on NPC by human iNSCs generated by our research group have not yet been studied in vivo; in this study, we investigate those effects. Methods: We used an NPC mouse model to efficiently evaluate the therapeutic effect of iNSCs, because neurodegeneration progress is rapid in NPC. In addition, application of human iNSCs from NPC patient-derived fibroblasts in an NPC model in vivo can give insight into the clinical usefulness of iNSC treatment. The iNSCs, generated from NPC patientderived fibroblasts using the SOX2 and HMGA2 reprogramming factors, were transplanted by intracerebral injection into NPC mice. Results: Transplantation of iNSCs showed positive results in survival and body weight change in vivo. Additionally, iNSC-treated mice showed improved learning and memory in behavior test results. Furthermore, through magnetic resonance imaging and histopathological assessments, we observed delayed neurodegeneration in NPC mouse brains. Conclusions iNSCs converted from patient-derived fibroblasts can become another choice of treatment for neurodegenerative diseases such as NPC.