β Scribed by Bernstein, Jay
No coin nor oath required. For personal study only.
The recent paper by Odent et al. [1998] contains an attribution to me of a statement that cysts in dominant polycystic kidney disease (PKD) develop exclusively in tubules. This is neither true nor was said by me. The paper they cite [Bernstein, 1993] was written in part to establish just the opposite. Glomerulocystic kidneys in young infants are a common expression of early onset D-PKD. Tubules are often also cystic. I know of no way to differentiate D-PKD glomerulocystic kidneys from sporadic glomerulocystic kidneys on morphologic or clinical grounds. A positive family history points the clinician in the right direction, which may then be verified by linkage analysis. Syndromal glomerulocystic disease, on the other hand, may have distinctive renal morphologic features in addition to those defining the primary syndrome. The glomerular cysts in oro-facialdigital syndrome type I (OFD1) are predominantly inner cortical, and the tubular cysts do not get as large as those in D-PKD.
Glomerular cysts in D-PKD are not unique to babies, but glomerular localization is easier to identify in babies with small cysts than in adults with large cysts.
The larger the cyst, the less often a random section traverses the glomerular tuft. In my own studies, glomerular cysts have been identified in 40% of adult D-PKD kidneys examined for their presence [Bernstein, 1993]. That is not surprising, since Heggo Β¨and Natvig [1966] and Baert [1978] showed by microdissection that cysts arose in virtually all portions of the nephron.
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