Abstract
Seventy-two members of 11 kindred with the nail-patella syndrome were studied. Twelve patients who had electron microscopic examinations of renal tissue had abnormalities of the glomerular basement membrane. An unaffected family member did not. This lesion consisted of irregular basement membrane thickening, epithelial foot process fusion and the presence of fibrillar collagen-like material within the substance of the membrane. Many patients also showed areas of increased lucency giving the membrane a "moth-eaten" appearance. The presence of the ultrastructural lesion bore no relation to the presence or absence of abnormalities by light or immunofluorescent microscopy and also no relation to demonstrable alterations of renal function. The determinants of clinically significant nephropathy are unknown but in most patients the prognosis is favorable.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 304-319 |
| Number of pages | 16 |
| Journal | The American journal of medicine |
| Volume | 54 |
| Issue number | 3 |
| DOIs | |
| State | Published - 1973 |
| Externally published | Yes |
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The nephropathy of the nail-patella syndrome. Clinicopathologic analysis of 11 kindred. / Bennett, William M.; Musgrave, James E.; Campbell, Robert A.; Elliot, Diane; Cox, Robert; Brooks, Robert E.; Lovrien, Everett W.; Beals, Rodney K.; Porter, George A.
In: The American journal of medicine, Vol. 54, No. 3, 1973, p. 304-319.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - The nephropathy of the nail-patella syndrome. Clinicopathologic analysis of 11 kindred
AU - Bennett, William M.
AU - Musgrave, James E.
AU - Campbell, Robert A.
AU - Elliot, Diane
AU - Cox, Robert
AU - Brooks, Robert E.
AU - Lovrien, Everett W.
AU - Beals, Rodney K.
AU - Porter, George A.
PY - 1973
Y1 - 1973
N2 - Seventy-two members of 11 kindred with the nail-patella syndrome were studied. Twelve patients who had electron microscopic examinations of renal tissue had abnormalities of the glomerular basement membrane. An unaffected family member did not. This lesion consisted of irregular basement membrane thickening, epithelial foot process fusion and the presence of fibrillar collagen-like material within the substance of the membrane. Many patients also showed areas of increased lucency giving the membrane a "moth-eaten" appearance. The presence of the ultrastructural lesion bore no relation to the presence or absence of abnormalities by light or immunofluorescent microscopy and also no relation to demonstrable alterations of renal function. The determinants of clinically significant nephropathy are unknown but in most patients the prognosis is favorable.
AB - Seventy-two members of 11 kindred with the nail-patella syndrome were studied. Twelve patients who had electron microscopic examinations of renal tissue had abnormalities of the glomerular basement membrane. An unaffected family member did not. This lesion consisted of irregular basement membrane thickening, epithelial foot process fusion and the presence of fibrillar collagen-like material within the substance of the membrane. Many patients also showed areas of increased lucency giving the membrane a "moth-eaten" appearance. The presence of the ultrastructural lesion bore no relation to the presence or absence of abnormalities by light or immunofluorescent microscopy and also no relation to demonstrable alterations of renal function. The determinants of clinically significant nephropathy are unknown but in most patients the prognosis is favorable.
UR - http://www.scopus.com/inward/record.url?scp=0015597714&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0015597714&partnerID=8YFLogxK
U2 - 10.1016/0002-9343(73)90025-9
DO - 10.1016/0002-9343(73)90025-9
M3 - Article
C2 - 4569963
AN - SCOPUS:0015597714
VL - 54
SP - 304
EP - 319
JO - American Journal of Medicine
JF - American Journal of Medicine
SN - 0002-9343
IS - 3
ER -