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Adult or Infantile Polycystic Disease?-Reply

Walter F. Piering, MD; Jacob Lemann Jr, MD; Lee A. Hebert, MD
Arch Intern Med. 1978;138(3):497. doi:10.1001/archinte.1978.03630270101039.
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In Reply.—  Dr Pollack doubts the diagnosis of infantile polycystic kidney disease (IPCD) because our patient failed to show characteristic "streaking" on her intravenous urogram. This finding, however, is seen in only some of the patients with classical IPCD, and in those patients, the finding disappears as they grow older. Furthermore, among those patients with IPCD and prolonged survival, the appearance of the kidneys by intravenous urography or by gross examination is very similar to the classic adult form (dominantly inherited) of polycystic kidney disease (PCD) (Medicine 50:277-318, 1971).Dr Pollack suggests that our patient's disease may be an example of the infantile appearance of the adult form of PCD. This seems unlikely, however, since her parents had normal intravenous urograms in their fifth and sixth decades of life, and there was no evidence of PCD in any other member of her kindred. Although our patient could represent a spontaneous


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