Oligomeganephronia Workup

Updated: Oct 04, 2018
  • Author: Pascale H Lane, MD; Chief Editor: Craig B Langman, MD  more...
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Workup

Laboratory Studies

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  • Urinalysis: Proteinuria is often the first laboratory manifestation of oligomeganephronia and precedes decline in renal function by several years.

  • Electrolyte, BUN, and creatinine tests: Laboratory manifestations of renal failure are frequently present, including elevated BUN and creatinine levels, hyponatremia, and metabolic acidosis.

  • Calcium, phosphorus, alkaline phosphatase, and parathyroid hormone assessments: Advancing renal failure may result in secondary hyperparathyroidism.

  • Hemoglobin or hematocrit level: Advancing renal failure may result in anemia due to erythropoietin deficiency.

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Imaging Studies

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  • Renal ultrasonography (See the image below.)

    Renal sonogram of a newborn with spontaneous pneum 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.

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    • Small kidney size depicted on ultrasonogram usually establishes diagnosis of hypoplasia.

    • Follow-up of renal growth has not been demonstrated to be predictive of outcome.

  • Guidelines: The American College of Radiology has established guidelines regarding imaging studies in renal failure. [2]

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Other Tests

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  • If specific syndromes are suspected, the following may be useful:

    • Hearing tests

    • Ophthalmologic evaluation

    • Chromosomal studies

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Histologic Findings

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  • Exhaustive histologic examination of the kidney is the only way to establish an absolute diagnosis of oligomeganephronia.

  • The number of glomeruli is reduced, and the number of glomerular generations varies from 2-6 (normally ≥ 10).

  • Existing glomeruli and tubules are enlarged, and glomerular diameters of 300-400 nm are common.

  • As the disease progresses, segmental sclerosis and hyalinosis of glomeruli are present.

  • Tubular atrophy with interstitial fibrosis occurs.

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