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Unilateral renal cystic disease: a case presentation
P F Middlebrook1, E Nizalik, J F Schillinger
1Department of Pathology, Children's Hospital of Eastern Ontario, Ottawa, Canada.
The Journal of Urology
|October 1, 1992
Summary
A unilateral renal cystic process in an infant led to nephrectomy for hypertension. Pathology suggested autosomal dominant polycystic kidney disease or a rare unilateral cystic condition.
Area of Science:
- Pediatric Nephrology
- Medical Genetics
- Surgical Pathology
Background:
- Unilateral renal cystic diseases are rare in infants.
- Hypertension can be a presenting symptom of renal abnormalities in children.
Observation:
- An infant presented with a unilateral renal cystic mass.
- The infant developed hypertension, necessitating a nephrectomy.
- Family and genetic investigations were negative for known hereditary kidney diseases.
Findings:
- Pathological examination of the resected kidney revealed cystic changes.
- The findings were most consistent with either autosomal dominant polycystic kidney disease (ADPKD) or a distinct entity of unilateral renal cystic disease.
- Absence of contralateral kidney involvement or other systemic anomalies was noted.
Implications:
- This case highlights the importance of considering cystic renal pathology in infants presenting with hypertension.
- It underscores the diagnostic challenge in differentiating sporadic unilateral cystic disease from early-onset ADPKD.
- Further research into the genetic underpinnings of unilateral renal cystic disease is warranted.