As the disease progresses, segmental sclerosis and hyalinosis of glomeruli are present. Tubular atrophy with interstitial fibrosis occurs. Medical care is supportive, including fluid and electrolyte balance, nutritional support, and management of the manifestations of chronic renal failure. Treatment with angiotensin-converting enzyme inhibitors may be of benefit in slowing progression. Once diagnosis of renal hypoplasia is suspected or established by renal ultrasound, refer patients to a pediatric nephrologist for ongoing management of chronic renal failure.
Dietary recommendations depend on the degree of renal functional impairment.
Most patients with oligomeganephronia have renal salt-wasting and require no restriction of dietary salt or water. As renal failure advances, potassium and phosphorus balance may become problematic and require dietary restrictions. In general, no restriction of normal activity is required. Advise patients with oligomeganephronia to maintain an active, healthy lifestyle. In addition, angiotensin-converting enzyme inhibitors may be of benefit in slowing progression of renal failure, even if the patient has healthy blood pressure.