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Juvenile Nephronophthisis. Part II A Histologic and Microangiographic Study
Author(s) -
IVEMARK BIÖRN I.,
LJUNGQVIST ARNE,
BARRY ALEXANDER
Publication year - 1960
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1960.tb07762.x
Subject(s) - medicine , emergency department , medical school , pediatrics , pathology , medical education , psychiatry
The renal changes in fatal cases of familial juvenile nephronophthisis have been described previously (Fanconi et al., 1951, Hackzell & Lundmark, 1958, and Hooft et al., 1959). The changes were regarded as being due to a diffuse renal contraction. No morphologic evidence of congenital defects in the kidney was given by any of these authors. I n the present paper, the renal pathology of two fatal cases of this disease will be described. The clinical features have been presented in the report by Broberger, Winberg & Zetterstrom in this issue (Cases 2 and 3). Evidence will be presented to show that there is a characteristic lesion of the descending and ascending limbs of the loop of Henle. This defect is associated with a cortical atrophy which is considered t o be secondary to the tubular lesions. I n view of the tendency of this disease to occur as a familial trait, it seems probable that the tubular defect has a congenital basis.