O. Hotta et al., CLINICOPATHOLOGICAL STUDY OF IGA NEPHROPATHY IN PATIENTS WITH CONGENITALLY REDUCED NEPHRON MASS, Clinical nephrology, 44(6), 1995, pp. 362-366
In experimental animal models, a reduction in the number of functionin
g nephrons is considered to play a role in the progression of glomerul
ar injury, In human renal diseases, however, whether a superimposed re
duction in the number of nephrons causes the exacerbation of preexiste
nt glomerulopathy has not been elucidated. We herein report the result
s of a clinicopathological study of five patients with IgA nephropathy
(IgAN) which occurred in a reduced nephron mass status (four cases of
congenital solitary kidney and one case of bilateral hypoplastic kidn
eys), Four of the five patients had chronic renal failure (CRF) and ex
hibited a relatively rapid course to CRF as primary IgAN. Renal biopsy
revealed that all four of the patients with CRF had glomerular hypert
rophy and focal segmental glomerular sclerosis. In addition, two of th
em had a focal active lesion. In one pa tient with bilateral hypoplast
ic kidneys renal biopsies were performed twice in eight years. During
this period her creatinine clearance deteriorated from 60.0 ml/min to
20.7 ml/min. Her first renal biopsy showed mild mesangial proliferatio
n without sclerotic lesions, glomerular hypertrophy and mesangial IgA
deposition, while all of them were prominent in the second renal biops
y. These observations suggest that IgAN superimposed on a nephron loss
status may be frequently associated with a progressive course of dise
ase, and careful follow-up and early treatment should be considered in
such a condition.