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.

Clinical Efficacy of a Top-down Approach for Children with a First Febrile Urinary Tract Infection

PURPOSE: The aim of this study was to determine the clinical characteristics, frequency of renal abnormalities and benefits of a top-down approach in children with their first febrile urinary tract infection (UTI). METHODS: We reviewed 308 patients retrospectively who were admitted to Yeungnam University Hospital and were treated for their first febrile UTI from February 2006 to December 2013. We performed a comparative analysis of laboratory findings and results of imaging techniques including a Tc-99m dimercaptosuccinic acid (DMSA) renal scan. RESULTS: Among the patients, 69% (213/308) were males, and 90% (277/308) had their first UTI episode during infancy. A DMSA renal scan was performed on all patients, and showed positive findings in 60% (184/308) of cases. Laboratory indices of inflammation were significantly higher in the DMSA-positive group (P<0.05). There was a statistically significant difference in the age distribution between the two groups. In the DMSA-positive group, 165 patients underwent voiding cystourethrography (VCUG), and 58 (35%) cases demonstrated vesicoureteral reflux. In total, 110 patients in the DMSA-positive group, underwent repeat scanning at 6 months; 33 children (30%) demonstrated static scarring, but 77 (70%) had improved completely. The concordance of the ultrasonography (US) and VCUG was low. Older patients had more renal scarring. CONCLUSION: DMSA is a sensitive method for assessing the severity of inflammation and kidney injury. However, the ability of US to predict renal parenchymal damage was limited. A top-down approach in children with their first febrile UTI showed significant value.

Clinical Efficacy of a Top-down Approach for Children with a First Febrile Urinary Tract Infection

PURPOSE: The aim of this study was to determine the clinical characteristics, frequency of renal abnormalities and benefits of a top-down approach in children with their first febrile urinary tract infection (UTI). METHODS: We reviewed 308 patients retrospectively who were admitted to Yeungnam University Hospital and were treated for their first febrile UTI from February 2006 to December 2013. We performed a comparative analysis of laboratory findings and results of imaging techniques including a Tc-99m dimercaptosuccinic acid (DMSA) renal scan. RESULTS: Among the patients, 69% (213/308) were males, and 90% (277/308) had their first UTI episode during infancy. A DMSA renal scan was performed on all patients, and showed positive findings in 60% (184/308) of cases. Laboratory indices of inflammation were significantly higher in the DMSA-positive group (P<0.05). There was a statistically significant difference in the age distribution between the two groups. In the DMSA-positive group, 165 patients underwent voiding cystourethrography (VCUG), and 58 (35%) cases demonstrated vesicoureteral reflux. In total, 110 patients in the DMSA-positive group, underwent repeat scanning at 6 months; 33 children (30%) demonstrated static scarring, but 77 (70%) had improved completely. The concordance of the ultrasonography (US) and VCUG was low. Older patients had more renal scarring. CONCLUSION: DMSA is a sensitive method for assessing the severity of inflammation and kidney injury. However, the ability of US to predict renal parenchymal damage was limited. A top-down approach in children with their first febrile UTI showed significant value.

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.

Validation of Onen's Alternative Grading System for Congenital Hydronephrosis

PURPOSE: The purpose of this study was to compare the validity of Onen's alternative grading system (AGS) with that of the APDRP and SFU grading systems in patients with isolated and complicated congenital hydronephrosis. METHODS: We retrospectively reviewed the medical records of 153 patients (204 renal units) diagnosed with congenital hydronephrosis between January 2002 and December 2011. We classified patients into 2 groups; isolated or complicated hydronephrosis. All renal units were graded according to anterior-posterior diameter of renal pelvis (APDRP), Society for Fetus Urology (SFU) and Onen's grading systems. We analyzed the prognosis of hydronephrosis, according to each grading system, at 2 years of age. RESULTS: There were 152 renal units with isolated hydronephrosis and 52 renal units with complicated hydronephrosis. The isolated hydronephrosis group had a lower grade according to Onen's AGS, and showed more frequent spontaneous remission by 2 years of age. There was more frequent obstruction (P=0.000) and surgical treatment (P=0.000) of units with high-grade hydronephrosis according to Onen's AGS. In the complicated hydronephrosis group the frequencies of spontaneous remission (P=0.015) and renal dysfunction (P=0.013) were significantly higher than those in high-grade hydronephrosis, as indicated by Onen's AGS. There were no significant differences in clinical outcomes among the highest grade groups, according to the 3 systems, in either isolated or complicated hydronephrosis. CONCLUSION: Onen's AGS reflects the prognosis of hydronephrosis as well as other grading systems in those with isolated hydronephrosis. It was better predictor of renal dysfunction in those with complicated hydronephrosis. However, Onen's AGS was not superior to the other grading systems in terms of predicting prognosis, especially in high-grade hydronephrosis.