Related Experiment Video
Updated: Oct 28, 2026
Implementing Patch Clamp and Live Fluorescence Microscopy to Monitor Functional Properties of Freshly Isolated PKD Epithelium
Published on: September 1, 2015
Factors relating to urinary protein excretion in children with autosomal dominant polycystic kidney disease
Insights
Proteinuria and microalbuminuria are early indicators of kidney disease severity in children with autosomal dominant polycystic kidney disease (ADPKD). These findings suggest they may signal a more aggressive disease course in pediatric ADPKD patients.
Area of Science:
- Pediatric Nephrology
- Genetics
- Renal Disease Research
Background:
- Autosomal dominant polycystic kidney disease (ADPKD) is a genetic disorder characterized by cyst formation in the kidneys.
- In adults, proteinuria is associated with poorer renal outcomes and higher blood pressure.
- The early markers and progression of ADPKD in children require further investigation.
Purpose of the Study:
- To investigate the presence and significance of proteinuria and microalbuminuria in children with ADPKD.
- To explore the relationship between urinary protein excretion and indicators of renal disease severity in pediatric ADPKD.
- To compare urinary protein excretion in ADPKD children versus non-ADPKD children.
Main Methods:
- Analysis of urinary protein and albumin excretion rates in 189 children from 81 ADPKD families.
- Comparison of excretion rates between ADPKD patients and non-ADPKD controls.
- Correlation analysis between proteinuria, albuminuria, and clinical factors like renal cyst burden and blood pressure.
Main Results:
- ADPKD children exhibited significantly higher urine protein and albumin excretion rates compared to non-ADPKD children.
- Children with severe renal cystic disease showed greater protein excretion than those with moderate disease.
- Thirty percent of ADPKD children had albuminuria, and 23% had overt proteinuria; 30% had significant microalbuminuria.
- A correlation was observed between urinary protein excretion and diastolic blood pressure in children diagnosed after one year of age.
Conclusions:
- Proteinuria and albuminuria are prevalent in children with ADPKD, appearing early in the disease course.
- These urinary abnormalities may serve as potential biomarkers for more severe renal disease in pediatric ADPKD.
- Early detection of proteinuria and albuminuria could aid in monitoring disease progression and guiding interventions in children with ADPKD.
Abstract:
Adults with autosomal dominant polycystic kidney disease (ADPKD) who have overt proteinuria (>300 mg/d) have higher mean arterial pressures, lower creatinine clearances, larger renal volumes, and a more aggressive course of renal disease than ADPKD patients without proteinuria. This study examines the relationship between proteinuria and microalbuminuria and similar factors in ADPKD children. A total of 189 children from 81 ADPKD families was included in the analysis. The ADPKD children (n = 103) had significantly greater urine protein excretion rates than the non-ADPKD children (n = 86) (3.9+/-0.3 versus 2.8+/-0.2 mg/m2 per h, P < 0.001). Children with severe renal cystic disease (> 10 cysts; n = 54) had greater protein excretion than those with moderate disease (< or = 10 cysts; n = 49) (4.4+/-0.5 versus 3.3+/-0.2 mg/m2 per h, P < 0.05). The ADPKD children had significantly greater albumin excretion rates than the non-ADPKD children (32+/-6 versus 10+/-2 mg/m2 per 24 h, P < 0.001), and a higher percentage of ADPKD children had significant microalbuminuria (>15 mg/m2 per 24 h in boys and >23 mg/m2 per 24 h in girls) than their unaffected siblings (30% versus 10%, P < 0.05). Thirty percent of ADPKD children had albuminuria and 23% had overt proteinuria. For all ADPKD children, there was no correlation between proteinuria and hypertension. However, there was a significant correlation between urinary protein excretion and diastolic BP among children diagnosed after the first year of life (r = 0.23, P < 0.05). Therefore, proteinuria and albuminuria occur early in the course of ADPKD and may be markers of more severe renal disease.
Related Concept Videos
01:17Factors Affecting Renal Clearance: Renal Impairment
One condition associated with renal failure is uremia. Uremia is characterized by impaired glomerular filtration and fluid accumulation in the body. This condition hinders the renal clearance of drugs, resulting in drug accumulation and potential...
01:26Nephrotic Syndrome II : Assessment and Medical Management
01:25Chronic Kidney Disease I: Introduction
01:31Factors Affecting Renal Clearance: Drug's Physicochemical Properties and Plasma Levels
One important factor is the drug's molecular size. The kidneys readily excrete smaller molecules below 300 Daltons (Da). On the other hand, molecules weighing between 300 and 500 Da are excreted through both urine and bile. Larger molecules above 500 Da tend to be excreted less...
01:10Nephrons
01:28Urinary Tract Calculi I: Introduction