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)

Eighty-two patients, 56 male and 26 female, biopsied since 1972 had IgA nephropathy. At the time of kidney biopsy, 24 patients were children and 58 were adults. In both groups the clinical course was documented in sufficient detail to allow prediction of disease outcome. Twenty-six (45%) of the adult patients had chronic renal insufficiency either at first evaluation or subsequently. Fourteen eventually required chronic hemodialysis. Hypertension as the initial sign of disease was seen more frequently in patients with chronic renal insufficiency. Adult males were more likely to have chronic renal insufficiency. The life table method was used to predict age at initiation of dialysis and kidney survival from date of onset of clinically apparent disease. Thirty-five percent of the male patients were predicted to require dialysis by age 40. Kidney death was predicted at 10 years from onset for 33% of male and 22% of all patients biopsied as adults. While all patients with progressive disease had over 2.0 g/24 h urinary protein excretion at least once, many individuals with serum creatinine concentration below 1.5 mg/dL showed marked fluctuation in degree of proteinuria, often exceeding 2.0 g/24 h. Thus, in some cases, degree of proteinuria was not a reliable predictor of outcome.
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PMID:Iga nephropathy: presentation, clinical course, and prognosis in children and adults. 647 50

Forty seven patients with IgA glomerulonephritis (GN) were studied. The males predominated among them--the ratio males-female--2,9:1. The first clinical manifestations were macroscopic hematuria (in 2/3 of the cases) or only urine changes--low-degree proteinuria and microscopic erythrocyturia. As early as the establishment--38,2 per cent of the patients had elevated arterial pressure, and it reached, 59,5 per cent during the follow-up period. Anemia was rare. Manifestations of nephrotic syndrome were absent. ESR was normal in 1/3 of the employees or slightly accelerated. Chronic renal insufficiency developed 6,31 per cent of the patient all of them males. Terminal renal insufficiency developed 8 patients. The elevated arterial pressure, proteinuria over 2g/24 h, accelerated ESR, high serum levels of IgA, the longer duration of the disease and higher age of the patients to a certain extent--were unfavourable prognostic criteria. The basic immune deviations of IgA GN are the high serum levels of IgA (in 53,1% of the patients) and to a lower extent of IgG (in 17% of the patients) as well as the reduced serum levels of C3 (in 31,9% of the patients). CIC are not a frequent finding. The percentage of ERF is lower than in the healthy subjects. The treatment with a combination of azathioprine (acenocoumarol, indomethacin or levamisole) has no effect on the clinical manifestations and evolution.
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PMID:[Clinical, immunological and therapeutic research in mesangial immunoglobulin-A glomerulonephritis]. 653 70

A patient is described, admitted to the clinic on the occasion of proteinuria, edemas and arterial hypertension. The clinical and paraclinical data formed the picture of a renal involvement of mainly glomerular type with proteinuria to 5 gr%0, nonselective type, edemas, hypertension and data of chronic renal insufficiency I degree (serum creatinine to 3.8%, blood urea to 112 mg%). What impresses are the extrarenal manifestations as paresthesia of the lower limbs, with a sense of numbness to acute burning pain, a rich psychonervous symptomatics. The skin efflorescence, followed up and observed at the clinic of dermatology and the puncture biopsy material from the kidney revealed specific signs of Fabry syndrome.
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PMID:[Case of Fabry's disease]. 681 15

The investigate whether the blood rheological disturbances occurring in hemodialyzed patients are related to the erythrocyte lipid composition, cholesterol and phospholipid content was studied of the erythrocyte membrane. The study included 40 patients with chronic renal insufficiency. 34 of them had hypertension and proteinuria. The cholesterol/phospholipid ratio was significantly higher in the patients after hemodialysis (mean 2.48; SD 0.14) compared with the patients before hemodialysis (mean 2.08; SD 0.19). There was a significant increase of cholesterol in subgroup with hypertension and proteinuria. A significant correlation was found between membrane cholesterol/phospholipid ratio and serum levels of LDL-cholesterol.
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PMID:[Increased cholesterol content in erythrocyte membranes of patients with chronic renal failure treated with hemodialysis]. 747 40

To assess long-term hematologic and renal effects associated with a solitary kidney, 16 healthy cats undergoing uninephrectomy for kidney donation between May 1987 and January 1991 were evaluated by use of physical examination, CBC, serum biochemical analysis, urinalysis, and urine protein:creatinine ratio. Results of preoperative CBC, serum biochemical analysis, and urinalysis were within reference limits in all donors. Median age at surgery and at follow-up evaluation was 34 and 72 months, respectively. Mean (+/- SEM) interval between follow-up and uninephrectomy was 39.3 +/- 14.6 months. Postuninephrectomy hematocrit and RBC indices were within reference limits in 15 donors. One cat with chronic renal insufficiency had normocytic, normochromic, nonregenerative anemia. In 15 clinically normal donor cats, mean (+/- SEM) serum creatinine concentrations pre- and post-uninephrectomy were 1.36 +/- 0.20 and 1.71 +/- 0.33 mg/dl, respectively (P = 0.0002); however, the clinical relevance of this statistical difference in serum creatinine is uncertain, because all values were within reference limits. In addition, urine-concentrating ability was maintained in 14 donors, with urine specific gravity > or = 1.040. Two donors, including the cat with chronic renal insufficiency, produced dilute urine (specific gravity < or = 1.020) and had substantial proteinuria, with urine protein:creatinine ratios of 2.16 and 3.62, respectively. Mean urine protein:creatinine ratio in donor cats was not significantly different from that in an age- and sex-matched comparison group. Renal and erythropoetic function was clinically preserved in the group of donor cats within 2 to 5 years after uninephrectomy.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Long-term renal and hematologic effects of uninephrectomy in healthy feline kidney donors. 755 43

We describe a patient with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) who was found to have renal involvement with particular renal pathological findings. So far, 17 other cases, most of them from Japan, of POEMS syndrome with renal involvement, have been published. Clinical features are variable: acute renal failure with anasarca or moderate chronic renal insufficiency with mild proteinuria. This latter presentation often passes unnoticed. There is no severe hypertension, no microangiopathic hemolytic anemia. Renal biopsy shows prominent glomerular changes which are unusual and distinct from membranoproliferative glomerulonephritis (MPGN) and from glomerular thrombotic microangiopathy (TMA). Mesangial proliferation and thickening of the capillary wall with double contour on light microscopy suggest an MPGN. By immunofluorescent microscopy, no immunoglobulins or complement deposits were found. The occurrence of mesangiolytic lesions has led to the term of "mesangiolytic glomerulonephritis". The presence, on electron microscopy, of lucent subendothelial spaces could indicate TMA. But there are neither thrombi nor arteriolar changes. We are inclined to consider that the microangiopathic lesions are due to chronic injury of glomerular endothelial cells, exacerbated at outbreaks of the disease. Increased production of IL 6 could support the efficacy of corticosteroid therapy, particularly in acute clinical situations.
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PMID:Renal involvement in POEMS syndrome. 763 52

Fabry's disease is a rare hereditary disease transmitted as an X-linked recessive trait with the primary metabolic defect of an enzyme alpha-galactosidase A, resulting in deposition of glycolipids (ceramide trihexoside) in various tissues, including the kidneys. Two sibling cases of Chinese adult male patients in a family with Fabry's disease were completely evaluated including the clinical, pathologic and biochemical studies. Both of the patients had the similar clinical manifestations such as telangiectases, proteinuria, acral pains, corneal opacities, tortuous renal vessels and recurrent fever. Chronic renal insufficiency was noted in Case 1, whereas Case 2 had normal renal function. Microscopic hematuria was noted in Case 1. In renal biopsy, LM showed foamy vacuolation of the glomerular visceral epithelial cells and EM showed widespread myelin bodies (Zebra bodies) in kidney tissues, most numerous in visceral epithelia in both cases. Those findings are diagnostic for Fabry's disease. The plasma activity of alpha-galactosidase of Case 1 was 0.8 and that of Case 2 was 1.0 (normal reference range: 8.5-18.5 nmol/hr/min). The plasma activity of alpha-galactosidase A of Case 1 was 0.4 and that of Case 2 was 0.8 (normal reference range: 7.9-16.9 nmol/hr/min). All the enzyme activities in both cases were much lower than those of normal subjects. In addition to clinical presentations, pathologic study and biochemical study with assays of plasma or serum activities of alpha-galactosidase and alpha-galactosidase A are important steps in the diagnosis of Fabry's disease.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Fabry's disease: clinical, pathologic and biochemical manifestations in two Chinese males. 783 62

Clinical variables and laboratory and histologic findings were evaluated in 53 children with IgA nephropathy, of whom 44 were followed for a mean period of 6.2 years (range 1.2-14). At the end of the follow-up 8 patients (18.2%) had had no urinary anomalies for at least 1 year (stage A disease), 28 (63.6%) had microscopic hematuria with proteinuria < 1 g/m2/day (stage B), 5 (11.4%) had proteinuria > 1 g/m2/day (stage C), and 3 (6.8%) had chronic renal insufficiency (stage D). None of the patients in apparent remission presented with elevated serum IgA levels at disease onset. Gross or microscopic hematuria at onset correlated with stage A/B disease at the end of follow-up (p < .05) whereas the presence of proteinuria or nephritic syndrome at onset correlated with stage C/D disease after follow-up (p > .05). Presenting features of gross or microscopic hematuria without or with proteinuria (< 0.5 g/m2/day) correlated (p < .001) with minimal glomerular abnormalities at biopsy, whereas patients with nephritic syndrome had more severe histologic pictures. The presence of proliferative glomerulonephritis with crescents correlated (p < .0001) with poor outcome. The results demonstrate that the prognosis of IgA nephropathy in childhood must be viewed with caution and that outcome correlates with mode of onset and severity of the renal pathology.
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PMID:Clinical features and prognosis in childhood IgA nephropathy. 785 18

We described a patient with a POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal grammapathy and Skin changes) who was found to have renal involvement with peculiar renal pathological findings. Hitherto, 17 other cases, most of them from Japan, of POEMS syndrome with renal involvement, have been published. Clinical features are variable: acute renal failure with anasarca or moderate chronic renal insufficiency with mild proteinuria. This latter presentation often passes unnoticed. There is no severe HTA, no microangiopathic hemolytic anemia. Renal biopsy shows prominent glomerular changes which are unusual and distinct from membranoproliferative glomerulonephritis (MPGN) and from glomerular thrombotic microangiopathy (TMA). Mesangial proliferation and thickening of the capillary wall with double contour evoke by light microscopy a MPGN. By immunofluorescent microscopy, no immunoglobulins or complement deposits are found. The finding of mesangiolytic lesions has led to the term of "mesangiolytic glomerulonephritis". The presence, on electron microscopy, of lucent subendothelial space could evoke TMA. But there is neither thrombi, nor arteriolar changes. We are inclined to presume that microangiopathic lesions are due to chronic injury of glomerular endothelial cells, exacerbated at outbreaks of the disease. An increased production of IL-6 could support the efficacity of corticosteroid therapy, particularly in acute clinical situations.
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PMID:[Renal involvement in POEMS syndrome]. 786 28

Several factors promote the progression of renal disease, including glomerular hypertension and hypertrophy, molecular factors such as cytokines and growth hormones, proteinuria, acidosis, and hyperlipidemia. Regardless of the underlying etiology, many patients with chronic renal insufficiency will ultimately require kidney replacement therapy. Your goal is to delay the progression of renal failure, mainly through aggressive control of blood pressure. Other possible interventions include protein restriction, bicarbonate therapy, and lipid-lowering drugs.
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PMID:Chronic renal disease: new therapies to delay kidney replacement. 803 27


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