Medical Care
Medical care in patients with oligomeganephronia 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. [5, 6, 7]
Consultation
Once diagnosis of renal hypoplasia is suspected or established based on renal ultrasonography findings, refer patients to a pediatric nephrologist for ongoing management of chronic renal failure.
Diet and Activity
Diet
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 level balance may become problematic and require dietary restrictions.
Activity
In general, no restriction of normal activity is required. Advise patients with oligomeganephronia to maintain an active, healthy lifestyle.
Long-Term Monitoring
Long-term follow-up care
Oligomeganephronia is a chronic progressive renal disease that leads invariably to renal failure.
Assessment of renal function, biochemical status, and growth is necessary to manage this condition and to provide optimal care.
Fuke et al reported a case of a 23-year-old man with oligomeganephronia that did not progress to end-stage renal failure. Clinical features included renal insufficiency, persistent proteinuria, and bilateral small kidneys. Renal pathology revealed greatly enlarged glomeruli (mean diameter, 325 μm, which was approximately 2 times larger than normal). [8]
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Renal sonogram of a newborn with spontaneous pneumothorax, preauricular pits, and branchial cysts. The right kidney was absent, and the left kidney was hyperechoic and hypoplastic. The left kidney's length measured 1.8 cm; kidneys in newborns are normally 4.5 cm.
