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Cacchi-Ricci syndrome

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A congenital cystic disease of the pyramids with dilation of the terminal collecting ducts in the kidneys and multiple small cysts in the medulla, giving the kidneys a spongy appearance. Onset of detection from 3 weeks of age to 71 years, both sexes equally affected. Common features are pain in loin (38%), colic (28%), natural polyuria (28%), and occasional polydipsia. Uncomplicated form usually asymptomatic. Unknown aetiology. Frequently congenital and familial. Families with autosomal dominant inheritance reported.

Bibliography

  • R. Cacchi, V. Ricci:
    Sopra una rara e forse ancora non descritta affezione cistica della piramidi renali («rene a spugna»).
    Atti della Societa Italiana di Urologia, 1948, 21: 59. Sur une rare maladie kystique multiple des pyramides rénales, le "rein en éponge." Journal d'urologie medicale et chirurgicale, Paris, 1949, 55: 497-519.

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