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Nephropathy in patients with cyanotic congenital heart disease
H Akita1, S Matsuoka, Y Kuroda
1Department of Pediatrics, School of Medicine, University of Tokushima, Japan.
Insights
Older children with cyanotic congenital heart disease (CCHD) experience kidney problems. This study found both tubular and glomerular dysfunction, with urinary N-acetyl-beta-D-glucosaminidase (NAG) indicating early tubular issues.
Area of Science:
- Pediatric Nephrology
- Cardiology
- Renal Medicine
Background:
- Cyanotic congenital heart disease (CCHD) is associated with nephropathy in older pediatric patients.
- Renal tubular abnormalities in CCHD patients are understudied, with focus often on glomerular dysfunction.
Purpose of the Study:
- To evaluate renal function, including tubular and glomerular parameters, in pediatric patients with CCHD.
- To identify potential early biomarkers for renal dysfunction in this population.
Main Methods:
- Assessed renal function in 16 CCHD patients (aged 3-28 years).
- Measured urinary microalbumin, N-acetyl-beta-D-glucosaminidase (NAG), blood urea nitrogen, creatinine, and beta 2-microglobulin.
- Compared serum uric acid levels to controls and analyzed correlations with age.
Main Results:
- Older CCHD patients (15-28 years) showed proteinuria and low creatinine clearance.
- Elevated urinary NAG in 14/16 patients, suggesting widespread tubular dysfunction.
- Increased urinary microalbumin in 7/16 patients, indicating glomerular involvement.
- All CCHD patients had higher serum uric acid than controls; levels correlated with age.
Conclusions:
- Both glomerular and tubular renal dysfunction are present in pediatric CCHD patients.
- Urinary NAG may serve as an early marker for tubular dysfunction.
- Urinary microalbumin is useful for assessing glomerular dysfunction in CCHD.
Abstract:
Older pediatric patients with cyanotic congenital heart disease (CCHD) often develop nephropathy. Although felt to be secondary to glomerular dysfunction, there have been only a few papers examining renal tubular abnormalities in such patients. We therefore evaluated renal function in 16 patients with CCHD aged 3 to 28 years. The six oldest patients (aged 15 to 28 years), had documented proteinuria and low creatinine clearance levels. The urinary concentration of microalbumin was increased when compared to control values in 7 of the 16 patients; six of the patients had proteinuria while one did not. Urinary levels of N-acetyl-beta-D-glucosaminidase (NAG) were elevated in 14 patients including those without proteinuria. Concentrations of blood urea nitrogen and creatinine as well as serum and urinary concentrations of beta 2-microglobulin levels were elevated in certain patients. The serum concentration of uric acid was notably greater in all CCHD patients relative to controls. Furthermore, serum uric acid concentrations and urinary microalbumin levels correlated with patient age. In conclusion, renal tubular dysfunction as well as glomerular dysfunction occur in patients with CCHD. Urinary NAG may be useful as an early marker for the early detection of tubular dysfunction, while urinary microalbumin levels are useful in assessing glomerular dysfunction in these patients.