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renopulmonary cystic hamartomata

Friday 13 January 2006

Definition: Developmental tissular malformation of the lung and kidney. It represents a spectrum of abnormal morphogenesis affecting both kidney and lung.

Synopsis

- bilateral renal multilocular cysts
- hamartomatous pulmonary cysts
- markedly hyperplastic renomegaly
- renal medullary dysplasia
- bilateral pulmonary cystic hamartomas

Associations

- cellular mesoblastic nephroma

Differential diagnosis

- Weinberg-Zumwalt syndrome [1977]

  • multifocal cystic hamartomata of the lung associated marked parenchymal overgrowth of the kidney

References

- Graham JM Jr, Boyle W, Troxell J, Cullity GJ, Sprague PL, Beckwith JB. Cystic hamartomata of lung and kidney: a spectrum of developmental abnormalities. Am J Med Genet. 1987 May;27(1):45-59. PMID: 3605206