Comparison of 99mTc-DMSA Renal Scan and Power Doppler Ultrasonography for the Detection of Acute Pyelonephritis and Vesicoureteral Reflux

PURPOSE: Urinary tract infection (UTI) is one of the common infectious diseases in children. Several imaging modalities can be used to confirm the presence of acute pyelonephritis (APN). Among them the 99mTcdimercaptosuccinic acid renal scan (DMSA scan) is used as a gold standard for diagnosis. Ultrasonography technology is evolving. Therefore, in this study, we investigated the sensitivity and specificity of Power Doppler ultrasonography (PDU) compared to the results from the previous study. METHODS: There were 260 patients included in this study, aged between 1 and 12 months old. The patients were admitted to the Yeungnam University Medical Center between January 2008 and December 2015. All patients underwent both DMSA scan and PDU within 5days of admission. Voiding cystourethrography (VCUG) was performed in 195 patients with abnormal DMSA scan or PDU. RESULTS: The diagnostic sensitivity of APN using PDU was 45.5% and specificity was 85.5% in 260 patients following detection of a defect on DMSA scan that was defined as APN. The diagnostic sensitivity and specificity of PDU for VUR were 65.5 % and 60.1%, respectively. The diagnostic sensitivity and specificity of DMSA scan for VUR were 95.7% and 14.1%, respectively. CONCLUSION: PDU has a high specificity but low sensitivity, so there are limitations in using it to replace a DMSA scan for the diagnosis of APN in children. DMSA scan and PDU have different sensitivity and specificity in diagnosis of VUR, respectively. Therefore, we suggest that the sensitivity and specificity of each test can be helpful in diagnosing APN and VUR when used in conjunction.

Long-term Prognosis of thin Glomerular Basement Membrane Nephropathy in Children: A Retrospective Single Center Study

PURPOSE: Thin glomerular basement membrane nephropathy (TBMN) is, along with the IgA nephropathy, the most common cause of asymptomatic hematuria in Korean children. TBMN is usually a benign renal disease not requiring treatment and is associated with a good prognosis, but some cases hematuria is indicative of a state of progressive renal insufficiency. We aimed to retrospectively evaluate clinical manifestations and renal prognosis of patients with TBMN. METHODS: Among the 428 renal biopsies performed on children at Yeungnam University Hospital between January 2000 and February 2017, 167 patients were diagnosed as having TBMN. We retrospectively investigated 167 pediatric patients and identified 59 children with follow-up duration >3 years. RESULTS: Among 59 patients, there were 33 boys and 26 girls. Mean age of onset of hematuria was 7.18±2.64 years, and mean time from onset of disease until a renal biopsy was performed was 2.48±2.10 years. There were no clinical features or laboratory findings among studied children to indicate decreased renal function during follow-up; however, one case progressed to chronic kidney disease (CKD) due to an unknown cause. There were seven patients among these related a positive family history of hematuria or renal insufficiency. CONCLUSION: Although almost all patients had normal renal functions during follow-up, there were one patient who progressed to CKD and seven patients with family history of hematuria or renal insufficiency. Moreover, four among the 428 patients over 17 years underwent repeat renal biopsies, which showed results different from their earlier biopsies.Thus, large-scales studies may be required to determine long-term prognosis of TBMN in children, and further evaluation for Alport syndrome in TBMN cases is essential.

Long-term Prognosis of thin Glomerular Basement Membrane Nephropathy in Children: A Retrospective Single Center Study

PURPOSE: Thin glomerular basement membrane nephropathy (TBMN) is, along with the IgA nephropathy, the most common cause of asymptomatic hematuria in Korean children. TBMN is usually a benign renal disease not requiring treatment and is associated with a good prognosis, but some cases hematuria is indicative of a state of progressive renal insufficiency. We aimed to retrospectively evaluate clinical manifestations and renal prognosis of patients with TBMN. METHODS: Among the 428 renal biopsies performed on children at Yeungnam University Hospital between January 2000 and February 2017, 167 patients were diagnosed as having TBMN. We retrospectively investigated 167 pediatric patients and identified 59 children with follow-up duration >3 years. RESULTS: Among 59 patients, there were 33 boys and 26 girls. Mean age of onset of hematuria was 7.18±2.64 years, and mean time from onset of disease until a renal biopsy was performed was 2.48±2.10 years. There were no clinical features or laboratory findings among studied children to indicate decreased renal function during follow-up; however, one case progressed to chronic kidney disease (CKD) due to an unknown cause. There were seven patients among these related a positive family history of hematuria or renal insufficiency. CONCLUSION: Although almost all patients had normal renal functions during follow-up, there were one patient who progressed to CKD and seven patients with family history of hematuria or renal insufficiency. Moreover, four among the 428 patients over 17 years underwent repeat renal biopsies, which showed results different from their earlier biopsies.Thus, large-scales studies may be required to determine long-term prognosis of TBMN in children, and further evaluation for Alport syndrome in TBMN cases is essential.

Two Cases of Toxic Epidermal Necrolysis Associated with Deflazacort Therapy in Nephrotic Syndrome: Successfully Treated with Cyclosporine A

Toxic epidermal necrolysis (TEN) is a rare, acute, serious, and potentially fatal skin disease, in which cell death causes the epidermis to separate from the dermis. It is thought to be a hypersensitivity complex that affects the skin and mucous membranes, and is caused by certain medications, infections, genetic factors, underlying immunologic disease, or more rarely, cancers. We report two cases of TEN associated with deflazacort (DFZ), a derivative of prednisolone, used in the first episode of nephrotic syndrome (NS). The skin eruption appeared on the 4(th) and 5(th) weeks after DFZ administration, while NS was in remission. The widespread lesions were managed by intensive supportive treatment, discontinuation of DFZ, and oral administration of cyclosporine. Both patients showed a rapid improvement in symptoms of TEN without any complications or relapse of NS.