Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033687 (proteinuria)
 24,015 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report five cases of crescentic IgA nephropathy. All are males, 16-60 years of age. One case each came to medical attention with uremia, nephrotic syndrome, and gross hematuria; two cases presented with microhematuria and proteinuria on routine urinalysis. All had hypertension, azotemia (serum creatinine 1.6-9.4 mg/dl), proteinuria (greater than 6 g/24 hr in four cases), hypoalbuminemia (less than 3 g/dl), and hematuria (gross in two cases). All progressed to end-stage renal failure renal failure ending in dialysis (three cases) or death from unrelated causes (two cases). Prednisone, 60 mg/day for 1 month in two patients (with two 1-g doses of iv methylprednisolone in 1 case) did not improve the serum creatinine level, but one patient subsequently experienced a less rapid fall in renal function. A crescentic glomerulonephritis was present in all biopsies (crescents in 31-80% of glomeruli; mean, 50%). The size and stage of the crescents were variable. Numerous glomeruli had focal or diffuse sclerosis. In all cases, there was a 3 or 4+ deposition of IgA. Low-intensity staining for IgG and IgM was noted in four and three patients, respectively. On electron microscopy, dense granular mesangial deposits were noted in all cases and in four patients capillary subepithelial deposits were also observed. This form of IgA nephropathy is not common, but some studies indicate that it may occur in about 5% of patients with IgA nephropathy.
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PMID:Crescentic IgA nephropathy. 639 83

Two hundred and two children with primary nephrotic syndrome were studied. Clinical features at onset and evolution were studied and correlated with microscopic findings and response to treatment. Histologic type and steroid-response were correlated with urinary protein selectivity in a group of them. Mean follow-up (by the same group of nephrologists) was 6.6 years; 22% were followed for over 8 years. The presence of hematuria, hypertension and renal failure at onset and during the course of the disease were evaluated. 122 patients were steroid-responders, 64 of them had frequent relapses. After a mean follow-up of 6.3 years, 81% were on remission; 14% had transient proteinuria, and 5% developed persistent proteinuria with late resistance to steroid therapy. 80 children were steroid-resistant. Their mean follow-up period was 5.5 years (1.0-10.5 years). 65 of them were biopsied: 12 had minimal glomerular change; 27 focal and segmental sclerosis. 9 membrano-proliferative glomerulonephritis, 4 focal and global sclerosis, 4 membranous nephropathy, 4 diffuse mesangial proliferation, 2 diffuse mesangial sclerosis and 2 were not classifiable. Hematuria had a prognostic value only when it was permanent or macroscopic. Urinary protein selectivity was measured in 43 patients, and C3 in 81; hypocomplementemia was found only in the 9 children with membranoproliferative glomerulonephritis. 8 children had tubular dysfunction: 7 had focal and segmental sclerosis, and 1 diffuse mesangial sclerosis; glycosuria and aminoaciduria were present in all 8, but only 4 of them had deficient urinary acidification. Secondary infections appeared in 42 patients; 19 were steroid-resistant and 23 steroid-responders, 17 of them showed frequent-relapses, 3 had localized thromboembolism. 23 children went into chronic renal failure and 17 of them died, 13 in end-stage renal failure and 4 because of complications. Their first biopsies showed minimal change in 1, focal and segmental sclerosis in 8, membranoproliferative glomerulonephritis in 3, diffuse mesangial proliferation in 1, diffuse sclerosis in 2, and 2 were not classifiable.
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PMID:[Primary nephrotic syndrome. Follow-up of 202 pediatric patients]. 727 77

Loss of renal functional reserve, that is, absence of the glomerular vasodilatory response to amino-acid infusion, has been interpreted as equivalent to glomerular hyperperfusion/hypertension, and therefore proposed as a marker of high risk for progressive glomerular sclerosis. To substantiate the validity of this hypothesis we evaluated the renal response to glycine and the extent of glomerular damage 10-12 weeks after induction of anti-glomerular basement membrane glomerulonephritis with or without superimposed clip hypertension. Untreated rats and rats chronically treated with quinapril, a converting-enzyme inhibitor, were studied. In untreated groups, loss of renal functional reserve was demonstrated since GFR, single-nephron GFR (SNGFR) and plasma flow (SNPF) did not increase during glycine infusion. The absence of renal reserve was associated with glomerular hyperfusion/hypertension, and development of proteinuria and glomerulosclerosis. Quinapril reduced proteinuria and diffuse sclerosis in anti-glomerular basement membrane GN, and decreased blood pressure and segmental glomerulosclerosis in antiglomerular basement membrane GN with superimposed clip hypertension. Both treated groups demonstrated a restoration of renal functional reserve, as depicted by increases in GFR, SNGFR, and SNPF after glycine, despite persistence of glomerular hyperperfusion/hypertension. These data demonstrate that renal functional reserve testing, although it does not detect glomerular hyperperfusion/hypertension, can provide information on the progression of glomerular damage.
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PMID:Renal functional reserve in experimental chronic glomerulonephritis. 781 49