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)

The wide utilization of renal biopsy and the introduction of electron microscopic and immunohistologic methods has allowed better definition of the clinico-pathological conditions associated with the nephrotic syndrome (NS). Two major categories of facts can be differentiated. In the first one, diffuse lesions of glomeruli, either secondary to specific diseases, or apparently primary diseases such as membranous or membrano-proliferative glomerulonephropathy (GN) are responsible for the increased permeability of the glomerular capillaries. In most of these, there is evidence that immunological mechanisms play a role in the injury of the glomerular capillary. Any of the following clinical symptoms are suggestive of this category of NS: an acute nephritic onset, a moderate NS, macroscopic hematuria, marked hypertension and/or renal insufficiency, poorly selective proteinuria and decreased plasma C3 levels. Patients affected with any of these glomerulopathies usually do not respond to steroids. In the second one, usually referred to as the idiopathic nephrotic syndrome (INS) the mechanism of glomerular capillary alteration is unknown and the nephrotic syndrome is more marked. Minimal change NS (MCNS) accounts for the great majority of INS and is characterized in most cases by a selective proteinuria, the absence of hematuria, a good response to steroids and a good prognosis. However, in some instances, renal biopsy reveals either diffuse mesangial proliferation (DMP) or focal glomerular sclerosis (which may be superimposed on MCNS or on DMP). In both instances, hematuria may be present and 50--75% of patients do not respond to steroids and have a poor prognosis. There is still considerable controversy about the exact relationship between these 3 patterns. We believe that they are not distinct entities but represent variants of the same disease. In addition to these 2 major categories of NS, there are, in infancy, 2 conditions associated with a NS of poor prognosis: congenital NS of Finnish type and infantile mesangial sclerosis. Since steroid-sensitive nephrosis is by far the commonest cause of NS especially in young children up to 8 years, a renal biopsy should be performed only in 2 instances: (a) when the clinical symptoms suggest diffuse glomerular lesions, and (b) when steroid resistance has been demonstrated.
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PMID:Clinicopathologic correlations in the nephrotic syndrome. 39 6

The nephrotic syndrome is characterized by gross proteinuria, hypoproteinemia, hyperlipemia, and edema. The authors review the diagnostic features and management protocol of this syndrome in children.
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PMID:Nephrotic syndrome in childhood: diagnosis and management. 41 35

A 27-year-old man, a known heroin addict with previously diagnosed nephrotic syndrome, had a percutaneous renal biopsy for recurrent proteinuria. The biopsy revealed a glomerular lesion compatible with focal sclerosing hephropathy. The renal interstitium also demonstrated numerous active granulomas characterized by the presence of both multilamellated basophilic material and areas of unstained fibrillar material. These findings represent a previously undescribed complication of parenteral heroin abuse--renal granuloma formation.
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PMID:Granulomatous interstitial nephritis: a complication of heroin abuse. 42 94

Nineteen patients, 20 to 47 years old with heroin addiction and renal disease, were studied. Patients with pre-existing systemic disease that can cause renal pathology were excluded. The mean duration of the addiction was 3.8 +/- 8 years. Most of the patients had the nephrotic syndrome. Renal biopsy revealed focal histological changes in 10 patients (5 with focal sclerosis) and diffuse changes in 9. Immunofluorescence studies done in 17 patients revealed diffuse deposition of IgG and beta 1C in 11 patients and focal deposits in 6 (mostly IgM). They were followed for a period ranging from 7 to 67 months. Most patients experienced progressive deterioration of renal function except in 4 cases in whom there was cessation of the drug addiction. These 4 displayed a rise in glomerular filtration rate (from 87 +/- 16 to 100 +/- 11 ml/min), marked reduction in proteinuria (from 6.5 +/- 2.6 to 0.4 +/- 0.1 g/24 hr) and an increase in serum albumin (from 3.2 +/- 0.5 to 4.0 +/- 0.1 g/dl). In summary, the fact that the renal abnormalities of our patients could not be explained by other diseases and the marked improvement upon cessation of the addiction favors the notion that heroin addiction "per se" may be the cause of the nephropathy. However, the heroin nephropathy was not manifested by a specific histological or immunofluorescence pattern.
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PMID:Heroin associated nephropathy: clinical and histological studies in 19 patients. 42 56

Patients with nephrotic syndrome have low blood levels of 25 hydroxyvitamin D (25-OH-D) most probably because of losses in urine, and a vitamin D-deficient state may ensue. The biological consequences of this phenomenon on target organs of vitamin D are not known. This study evaluates one of these target organs, the bone. Because renal failure is associated with bone disease, we studied six patients with nephrotic syndrome and normal renal function. The glomerular filtration rate was 113+/-2.1 (SE) ml/min; serum albumin, 2.3+/-27 g/dl; and proteinuria ranged between 3.5 and 14.7 g/24 h. Blood levels of 25-OH-D, total and ionized calcium and carboxy-terminal fragment of immunoreactive parathyroid hormone were measured, and morphometric analysis of bone histology was made in iliac crest biopsies obtained after double tetracycline labeling. Blood 25-OH-D was low in all patients (3.2-5.1 ng/ml; normal, 21.8+/-2.3 ng/ml). Blood levels of both total (8.1+/-0.12 mg/dl) and ionized (3.8+/-0.21 mg/dl) calcium were lower than normal and three patients had true hypocalcemia. Blood immuno-reactive parathyroid hormone levels were elevated in all. Volumetric density of osteoid was significantly increased in three out of six patients and the fraction of mineralizing osteoid seams was decreased in all. Evidence for an increase in active lacunae (bone-osteoclast interface) occurred in three out of six patients and in inactive (Howship's lacunae) bone resorption in six out of six. The data indicate that the loss of 25-OH-D in urine of patients with nephrotic syndrome and normal renal function may result in a decrease of blood levels of ionized calcium, secondary hyperparathyroidism and enhanced bone resorption. In addition, the vitamin D-deficient state causes osteomalacia as evidenced by defective mineralization and increased osteoid volume.
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PMID:Osteomalacia and hyperparathyroid bone disease in patients with nephrotic syndrome. 42 68

Thionamide drugs are widely used in the management of hyperthyroidism and are infrequently associated with adverse reactions. We report the development of the nephrotic syndrome during methimazole (Tapazole) therapy in a young man with Graves' disease. His proteinuria remitted promptly with discontinuance of the drug, and renal histologic features bore a striking resemblance to the toxic nephrosis induced in animals by the aminonucleoside of puromycin. In view of the histologic similarities, we propose that methimazole acted as a direct glomerular toxin, inducing the nephrotic syndrome in this patient.
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PMID:Nephrotic syndrome associated with methimazole therapy. 43 80

In order to assess to what extent glomerular or tubular function is involved in the renal handling of amylase and the lysozyme to creatine clearance ratios (CAm/CCr and CLys/CCr) were evaluated in 22 healthy volunteers and in 71 patients with different renal diseases. In normal controls, the mean CAm/CCr was 2.55 +/-1.54 SD, with an upper normal limit of 5.56. A normal ratio was found in patients with glomerulonephritis, with or without a nephrotic syndrome, and in patients with pyelonephritis. A significantly elevated ratio (P less than 0.001) was instead found in patients with uremia and in patients with uremia and in patients with either chronic or acute tubular damage. The CLus/CCr ratio was elevated in all the groups, except in patients with glomerulonephritis and minimal proteinuria. These results show that in humans, as in animals, the amylase filtered load undergoes partial tubular reabsorption. In renal diseases, an increase of the CAm/CCr is caused by either a marked reduction of functioning nephrons or a severe tubular damage, while the glomerular permeability does not seem to be involved. Some other mechanism is probably involved in the elevation of the CAm/CCr during acute pancreatitis.
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PMID:Amylase to creatine clearance ratio in renal diseases. 44 31

Patients with a pure mesangial proliferative glomerulonephritis may present with either hematuria, asymptomatic proteinuria, or nephrotic syndrome. For patients with hematuria, the clinical course is self-limited, and spontaneous resolution of recurrent episodes of macroscopic hematuria occurred in many of these patients. For patients with asymptomatic proteinuria, a spontaneous decrease in proteinuria occurred and none of these patients developed a progressive course. The clinical course of patients with nephrotic syndrome was similar to that usually observed in children and adults with pure minimal change lesions. A complete remission of the nephrotic syndrome occurred in most of these patients during treatment with prednisone and cyclophosphamide. Overall, the prognosis for the patients reported in this series was quite good.
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PMID:The clinical course of mesangial proliferative glomerulonephritis. 44 64

Plasma and urinary antithrombin III (AT-III) was measured in 15 cases of nephrotic syndrome. Plasma AT-III correlated well with serum albumin, but poorly with proteinuria, whereas urinary AT-III correlated well to proteinuria. The plasma AT-III level had a mean similar to 25 healthy controls, but the range was significantly wider. A case with nephrotic syndrome and left renal vein thrombosis is reported. The urinary output of AT-III rose and the plasma level fell with the activity of the disease. Although AT-III and albumin have similar molecule weight, their renal clearance was found to be different. It is suggested that urinary loss of AT-III plays a role in the hypercoagulable state sometimes found in the nephrotic syndrome.
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PMID:Antithrombin III and the nephrotic syndrome. 47 63

Two cases of secondary amyloidosis are presented. Both cases presented as nephrotic syndrome and were confirmed by biopsy dying two years later of the appearance of proteinuria. Incidence and characteristics of amyloidosis in infancy is commented.
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PMID:[Two cases of secondary amyloidosis (author's transl)]. 48 45


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