Renal medullary cystic disease or familial juvenile nephronophthisis: A renal tubular disease

1970 ◽  
Vol 48 (2) ◽  
pp. 174-184 ◽  
Author(s):  
Nils Giselson ◽  
Dick Heinegard ◽  
Carl-Gottfrid Holmberg ◽  
Lars-Göran Lindberg ◽  
Eric Lindstedt ◽  
...  
Keyword(s):  
Cystic Disease ◽  
Medullary Cystic Disease ◽  
Familial Juvenile Nephronophthisis ◽  
Renal Tubular ◽  
Renal Tubular Disease ◽  
Juvenile Nephronophthisis ◽  
Renal Medullary Cystic Disease

Familial Uremic Medullary Cystic Disease or Nephronophthisis?

PEDIATRICS ◽  
1971 ◽  
Vol 47 (2) ◽  
pp. 477-477
Author(s):  
R. N. Srivastava
Keyword(s):  
Age Of Onset ◽  
Renal Medulla ◽  
Familial Occurrence ◽  
Cystic Disease ◽  
Medullary Cystic Disease ◽  
Familial Juvenile Nephronophthisis ◽  
Cystic Changes ◽  
Juvenile Nephronophthisis

Drs. Alexander and Campbell (Pediatrics, 45: 1024) rightly mention the similarity between Medullary Cystic disease and familial juvenile nephronophthisis. It is now clear that the separation of these disorders on the basis of familial occurrence, age of onset, and presence of medullary cysts is not possible. I, however, disagree with these authors' recommendation to refer to these conditions as "Familial Uremic Medullary Cystic disease." The disorder is not always familial and the cystic changes are not confined to renal medulla.

FAMILIAL UREMIC MEDULLARY CYSTIC DISEASE

PEDIATRICS ◽  
1970 ◽  
Vol 45 (6) ◽  
pp. 1024-1028
Author(s):  
Fred Alexander ◽  
Sara Campbell
Keyword(s):  
Clinical Features ◽  
Cystic Disease ◽  
Medullary Cystic Disease ◽  
Familial Juvenile Nephronophthisis ◽  
Concentrate Urine ◽  
Juvenile Nephronophthisis

The typical clinical features of uremic medullary cystic disease—anemia, polyuria, polydypsia, and uremia—were observed in three young siblings (the typical setting of familial juvenile nephronophthisis). Renal juxtemedullary cysts were found at autopsy in one sibling, confirming the concept that these two diseases are truly rehated. The mother of the siblings studied in this paper demonstrated an inability to concentrate urine.

Familial juvenile nephronophthisis and medullary cystic disease

1968 ◽  
Vol 73 (1) ◽  
pp. 77-83 ◽  
Author(s):  
Frank A. Pedreira ◽  
Ellen L. Marmer ◽  
William H. Bergstrom
Keyword(s):  
Cystic Disease ◽  
Medullary Cystic Disease ◽  
Familial Juvenile Nephronophthisis ◽  
Juvenile Nephronophthisis

Renal Medullary Cystic Disease

1988 ◽  
Vol 29 (5) ◽  
pp. 527-529 ◽  
Author(s):  
A. Olsen ◽  
J. Hansen Højhus ◽  
G. Steffensen
Keyword(s):  
Renal Failure ◽  
Renal Disease ◽  
Diagnostic Tools ◽  
Adult Onset ◽  
Cystic Disease ◽  
Terminal Renal Failure ◽  
Medullary Cystic Disease ◽  
Corticomedullary Junction ◽  
Autosomal Inheritance ◽  
Renal Medullary Cystic Disease

Medullary cystic disease (MCD) is an uncommon renal disease with adult onset and autosomal inheritance, eventually progressing to terminal renal failure. It may be difficult to identify because of insufficient diagnostic tools. At urography, the same ring- shaped accumulation of contrast medium at the corticomedullary junction was observed in two patients (mother and son) suffering from MCD. To our knowledge this observation has not been reported before.

Renal medullary cystic disease

1988 ◽  
Vol 29 (5) ◽  
pp. 527-529
Author(s):  
A. Olsen ◽  
J. Hansen Højhus ◽  
G. Steffensen
Keyword(s):  
Cystic Disease ◽  
Medullary Cystic Disease ◽  
Renal Medullary Cystic Disease
Sign in / Sign up

Export Citation Format

Share Document