particularly important in dogs;
∙
may be from abnormal structure or abnormal function;
Abnormal structure: most familial renal diseases in this category are glomerulopathies, with diffuse
involvement of glomeruli and secondary tubular and interstitial changes; an
abnormal collagen type IV protein results in inadequate cross-linking of
collagen in the glomerular basement membrane (GBM) and thus progressive
wear and tear caused by filtration pressure; the age of onset of clinical signs in
familial glomerulopathies can vary from a few weeks to several years; known
as Alport syndrome in humans.
-
Samoyed hereditary glomerulopathy
and
Familial glomerulonephritis of Doberman Pinshers
:
result from a defective GBM.
-
Familial renal fibrosis in Norwegian Elkhounds
: primarily a tubulointerstitial nephropathy,
beginning with periglomerular and interstitial fibrosis and ending with more or less diffuse
fibrosis of renal cortex and medulla.
Abnormal function: familial renal diseases in this category are principally tubular diseases that
consist mostly of biochemical defects in renal tubular cells, with subtle to no
characterizing gross or microscopic lesions.
-
Fanconi Syndrome in Basenji dogs
: a proximal tubular disorder similar to the syndrome of
the same name in humans; affected dogs have impaired renal tubular reabsorption of glucose,
phosphate, sodium, potassium, uric acid, and amino acids; failure of the proximal tubules to
reabsorb bicarbonates also leads to acidosis; microscopic renal lesions are nonspecific and
include interstitial fibrosis and tubular atrophy.
-
Primary renal glucosuria in Norwegian Elkhound
: a singular transport abnormality without
other defects.
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