Fatma El-Husseiny Moustafa1, Riham Eid2, Nashwa Hamdy3. 1. Pathology Department, Faculty of Medicine, Mansoura University, Mansoura, Egypt. 2. Pediatric Nephrology Unit, Departement of Pediatrics, Faculty of Medicine, Mansoura University, Mansoura, 35516, Egypt. rihameid@mans.edu.eg. 3. Pediatric Nephrology Unit, Departement of Pediatrics, Faculty of Medicine, Mansoura University, Mansoura, 35516, Egypt.
Abstract
BACKGROUND: Hematuria is a common problem in pediatric practice and necessitates exhausting studies to detect etiology and establish proper management and counselling. SUBJECTS AND METHODS: We reviewed the clinical and pathological findings in 95 children presented between 2013 and 2019 with gross or microscopic hematuria with or without proteinuria in whom non-glomerular causes were excluded. In addition, a reference range for normal glomerular basement membrane thickness (GBMT) is introduced based on the assessment of biopsies of 20 steroid-resistant nephrotic syndrome cases aged 3-15 years, and with minimal change pathology. RESULTS: Fifty-seven males and 38 females with a mean age of 7.72 ± 3.27 years were included. Recurrent gross hematuria was the most frequent presentation (42%) while accidentally discovered hematuria was reported in 34.7% of patients. Alport syndrome (AS) was the most frequent diagnosis (66.3%) followed by thin basement membrane disease (TBMD) (29.5%) and Immunoglobulin A nephropathy (IgAN) (4%). A reference for normal GBMT was 360.3 ± 87.5 nm which is comparable to published references. Males with AS had a higher incidence of progression to renal failure and requirement for dialysis (p = 0.006 and 0.03, respectively) compared to females. Persistent proteinuria and lower glomerular filtration rate at diagnosis were associated with poor outcomes (p < 0.001 each). CONCLUSION: Alport Syndrome is the most common cause of glomerular hematuria in Egyptian children. Persistence of proteinuria and lower GFR at initial presentation were the main predictors of poor outcomes. The establishment of GBMT reference range for each locality is mandatory for the proper diagnosis of such cases.
BACKGROUND:Hematuria is a common problem in pediatric practice and necessitates exhausting studies to detect etiology and establish proper management and counselling. SUBJECTS AND METHODS: We reviewed the clinical and pathological findings in 95 children presented between 2013 and 2019 with gross or microscopic hematuria with or without proteinuria in whom non-glomerular causes were excluded. In addition, a reference range for normal glomerular basement membrane thickness (GBMT) is introduced based on the assessment of biopsies of 20 steroid-resistant nephrotic syndrome cases aged 3-15 years, and with minimal change pathology. RESULTS: Fifty-seven males and 38 females with a mean age of 7.72 ± 3.27 years were included. Recurrent gross hematuria was the most frequent presentation (42%) while accidentally discovered hematuria was reported in 34.7% of patients. Alport syndrome (AS) was the most frequent diagnosis (66.3%) followed by thin basement membrane disease (TBMD) (29.5%) and Immunoglobulin A nephropathy (IgAN) (4%). A reference for normal GBMT was 360.3 ± 87.5 nm which is comparable to published references. Males with AS had a higher incidence of progression to renal failure and requirement for dialysis (p = 0.006 and 0.03, respectively) compared to females. Persistent proteinuria and lower glomerular filtration rate at diagnosis were associated with poor outcomes (p < 0.001 each). CONCLUSION:Alport Syndrome is the most common cause of glomerular hematuria in Egyptian children. Persistence of proteinuria and lower GFR at initial presentation were the main predictors of poor outcomes. The establishment of GBMT reference range for each locality is mandatory for the proper diagnosis of such cases.
Authors: D F Barker; C J Pruchno; X Jiang; C L Atkin; E M Stone; J C Denison; P R Fain; M C Gregory Journal: Am J Hum Genet Date: 1996-06 Impact factor: 11.025