A57-year-old womanwho had had renal amyloidosis with nephrotic syndrome for five years was found to have the Fanconi syndrome and monoclonal lambda light-chain proteinuria. The amyloidosis of this patient was of primary type on the basis of the permanganate-resistent amyloid, the presence of monoclonal light-chain proteinuria, and the absence of clinical and histologic evidence of multiple myeloma.To best of our knowledge, this is the first case report in which primary amyloidosis with monoclonal lambda light-chain proteinuria was accompanied by adult Fanconi syndrome.
The effect of the methylprednisolone (MP) pulse therapy on renal function was examined in 15 patients with renal or collagen disease. Three nephrotic patients who had reduced renal function and active renal disease with progressive deterioration of renal function prior to the use of MP developed transient renal failure following an MP pulse therapy. The renal failure in each case was reversed by discontinuation of MP and/or by forced diuresis using albumin and furosemide. We examined the correlations between the individual changes in serum creatinine (Scr), body weight (BW) and urine volume (UV) before and after the pulse therapy and other laboratory data such as Scr, total serum protein and albumin. There were significant correlations between a change in Scr on the one hand and changes in BW and UV, Scr and serum albumin on the other. These findings mean that the effect of the MP pulse therapy on renal function depends on the clinical state of the patient and that renal deterioration after the pulse therapy may be more marked in patients who are more nephrotic and more impaired in renal function and suggest that increasing sodium and water retention during an MP therapy and the associated renal interstitial edema, proposed as one of the mechanisms of acute renal failure occurring in patients with minimal-change nephrotic syndrome, may be responsible for the MP-induced transient renal failure.
A patient with acute lymphoblastic leukemia, who developed acute renal failure, was imaged by computed tomography (CT). Persistent wedge-shaped areas of low attenuation in the kidneys were shown by sequential scans but no areas of infarction were found at autopsy. This case suggests that a wedge-shaped low-density lesion on CT may occur not only in infarcted areas, but also in areas where patchy vasoconstriction occurs.
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