Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020639 (hypoproteinemia)
 1,134 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In order to examine the clinical characteristics and genetic background of secondary amyloidosis associated with rheumatoid arthritis, we analyzed clinical features and HLA typing of 85 patients in a multicenter study. Eighty-five patients with secondary amyloidosis associated RA were studied. The diagnosis of secondary amyloidosis were made on histological findings by biopsy or autopsy. The most common biopsy site was gastrointestinal tract (79.5%). Clinical symptom and the frequency at the time of diagnosis were; diarrhea (35 cases), abdominal pain (22 cases) and vomiting and nausea (16 cases). Abnormalities and the frequency in a laboratory test included proteinuria (49 cases), increased serum creatinine (32 cases), anemia (30 cases) and hematuria (15 cases). Twenty-eight patients were dead and 57 patients were alive at the time of the study. The average duration between diagnosis of amyloidosis and death was 19.4 +/- 18.5 (SD) months among the dead patients. The average duration after diagnosis of amyloidosis was 24.2 +/- 19.5 (SD) months in surviving patients. The causes of death were renal failure complicated with heart failure (6 patients), heart failure alone (3 patients) and renal failure alone (2 patients). Fifty-nine patients in the control group who were negative to amyloid deposition on biopsies at more than one site in the gastrointestinal tract, were clinically compared with patients in the amyloidosis group. No difference were noted in the age of RA occurrence and the stage between the two groups. As to the class, however, the number of patients with severe functional disorder (class 3 or severe) was larger in the amyloidosis group. There were no significant difference between the two groups in Lansbury's activity index. On hematology, biochemistry and urinalysis, the incidences of increased white blood cell count, anemia, increased platelet count, increased serum creatinine, hypoproteinemia, hypoalbuminemia, increased IgA, and increased urine and blood BMG were statistically significantly higher in the amyloidosis group than in the control group. HLA-A, -B, -C, and DR-locus antigens were compared in the 53 patients in the amyloidosis group and in the 59 subjects in the control group. There were no significant differences in frequency of HLA-A, and -B antigens between two groups. Frequency of CW7 antigen was significantly decreased in the amyloidosis group (13.2%) than in the control group (39.0%). Frequency of DR1 antigen was decreased in the amyloidosis group (3.8%) than in the control group (22.0%), although the difference was not significant. These findings suggest the possible involvement of genetic factors in the occurrence of amyloidosis. It is suggested that the occurrence of amyloidosis is suppressed by some genes which are linked with CW7 antigen.
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PMID:[Clinical characteristics and genetic background of secondary amyloidosis associated with rheumatoid arthritis in Japanese]. 871 35

To investigate a role of the Maillard reaction in the pathogenesis of diabetic nephropathy, we measured serum levels of 3-deoxyglucosone (3-DG), a potent protein cross-linking intermediate of the Maillard reaction, and tissue contents of advanced glycation end products (AGEs) in streptozotocin (STZ)-induced diabetic rats. We quantified serum 3-DG using gas chromatography/mass spectrometry, and measured AGE contents in tissues using a competitive enzyme-linked immunosorbent assay with a monoclonal anti-AGE antibody. The STZ-induced diabetic rats showed nephropathy with proteinuria, hypoproteinemia, hyperlipidemia and reduced creatinine clearance. Serum levels of 3-DG in the STZ-induced diabetic rats (mean +/- 3.46 +/- 0.23 mumol/l) were significantly (p < 0.01) higher than those in control rats (1.23 +/- 0.13 mumol/l). AGE contents in the kidney and the lens obtained from the STZ-induced diabetic rats (398 +/- 45 and 816 +/- 200 arbitrary units, respectively) were also significantly (p < 0.01) higher than those in the control rats (122 +/- 10 and 299 +/- 50 arbitrary units, respectively). The results indicate that increased levels of serum 3-DG and renal tissue. AGEs may be related to the occurrence of diabetic nephropathy.
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PMID:Serum levels of 3-deoxyglucosone and tissue contents of advanced glycation end products are increased in streptozotocin-induced diabetic rats with nephropathy. 893 85

Nephropathia epidemica (NE) is a hemorrhagic fever with renal syndrome (HFRS) normally taking a benign clinical course. The etiologic agent, Puumala hantavirus is genetically closely related to Sin Nombre virus, which causes a frequently lethal febrile syndrome with pulmonary involvement (hantavirus pulmonary syndrome, HPS). HPS is characterized by acute respiratory distress, non-cardiogenic pulmonary edema and severe and hypotension, but usually no significant renal involvement. Pulmonary involvement and respiratory symptoms also occur in NE. To understand the mechanisms of pulmonary involvement in NE, we studied the clinical records and chest X-rays of 125 hospital-treated acutely ill NE patients. Twenty-eight percent of the patients had disease-related changes in their chest radiographs. Pleural effusion and atelectasis were the most common X-ray findings, whereas frank pulmonary edema was rare. The patients with pathologic X-ray findings had a more marked hypoproteinemia (lowest measured serum protein concentration 54 +/- 1 g/l) than those with normal X-ray (62.1 +/- 0.9 g/l, p < 0.001) and leukocytosis (highest measured blood leukocyte count 14.1 +/- 0.9 x 10(9)/l vs. 10.6 +/- 0.6 x 10(9)/l, p < 0.001) and more severe renal insufficiency (highest measured serum creatinine 590 +/- 60 mumol/l vs. 356 +/- 29 mumol/l, p < 0.05). Hypoproteinemia best predicted the occurrence of abnormal chest X-ray findings in NE. This suggests, that capillary leakage and inflammation may play a role in the pathogenesis of NE lung involvement, similarly as in HPS. Differently from HPS, the fluid volume overload associated with renal insufficiency seemed to contribute strongly to the chest X-ray changes in NE.
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PMID:Pulmonary involvement in nephropathia epidemica: radiological findings and their clinical correlations. 898 52

The human nephrotic syndrome (NS) is accompanied by important alterations of mineral and bone metabolism. The purpose of the present study was to examine bone metabolism in rats with experimental NS and normal creatinine clearance, and to evaluate the reversibility of this alteration. NS was induced by three injections of puromycin aminonucleoside (PAN) on days 0, 21, and 35 (10, 5, and 5 mg/100 g body weight, respectively). The biochemical markers of bone formation (osteocalcin and alkaline phosphatase) and bone resorption (hydroxyproline and pyridinoline), bone mineral content (BMC), and bone mineral density (BMD), determined by dual-energy x-ray absorptiometry (DEXA), were studied on days 0, 7, 14, 28, 42, 56, 84, and 112. Proteinuria was present throughout the study. Hypoproteinemia was seen on days 7, 28, 42, and 56, returning to control values on days 84 and 112. In serum, osteocalcin (OC) concentration increased (p < 0.001), and alkaline phosphatase (ALP) decreased (p = 0.002). In urine, hydroxyproline increased (p < 0.001), but urinary pyridinoline was not different from the control group throughout the study. Increased serum parathyroid hormone concentration and decreased levels of 25-hydroxy and 1,25-dihydroxyvitamin D were found from day 7. During the intense proteinuria, bone resorption predominates and decreased BMC and BMD ensues in PAN-nephrotic rats. PAN-nephrotic rats showed low BMC and BMD compared to control group (p < 0.001). At the end of the study, when proteinuria persisted but total serum protein returned to control values, the biochemical bone markers, BMC, and BMD returned to normal. In conclusion, PAN-nephrotic rats had reversible bone alterations that were related to the magnitude of proteinuria and the concentration of total serum protein.
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PMID:Biochemical bone markers, bone mineral content, and bone mineral density in rats with experimental nephrotic syndrome. 915 58

We investigated the hemodynamic, renal, and hormonal effects of cyclosporin A (CyA) treatment (6 mg/kg per day) for 4 weeks in 12 patients with nephrotic syndrome (8 women: 4 men, aged 36-66 years, 3 cases of focal glomerular sclerosis: 9 cases of membranous nephropathy). To evaluate the effects of CyA on the diurnal variation of blood pressure (BP), 24-h non-invasive BP monitoring was performed using model ABPM-630 (Nihon Colin, Tokyo, Japan) before and during CyA treatment. As indices of hemodynamics, intra-arterial pressure was monitored and cardiac output was measured by the dye-dilution technique using a cuvette at 0 and 4 weeks after treatment. CyA ameliorated urinary protein excretion and hypoproteinemia from 3.5 +/- 0.9 to 2.2 +/- 0.7 g/day, and serum protein concentration from 4.9 +/- 0.2 to 5.5 +/- 0.2 g/dl after 4 weeks' treatment. Endogenous creatinine clearance, 24-h urinary sodium excretion, and plasma renin activity decreased significantly at 1 week. CyA treatment raised casual BP from 122 +/- 4/75 +/- 2 to 140 +/- 5/87 +/- 3 mmHg after 1 week and to 146 +/- 4/90 +/- 2 mmHg after 4 weeks. Before treatment 24-h ambulatory BP monitoring showed BP reduction at night (116 +/- 5/68 +/- 3 mmHg) compared to the daytime (124 +/- 5/75 +/- 2 mmHg). The diurnal variation of BP disappeared during CyA treatment; mean daytime and nighttime pressures were 135 +/- 4/81 +/- 2, 132 +/- 5/80 +/- 3 mmHg at 1 week and 139 +/- 5/83 +/- 3, 131 +/- 6/80 +/- 3 mmHg at 4 weeks, respectively. On hemodynamic study; a 4-week treatment with CyA increased mean arterial pressure from 91 +/- 3 to 104 +/- 3 mmHg, total peripheral resistance index from 2.1 +/- 0.1 to 2.5 +/- 0.1 x 10(3) dyne.sec.cm-5.m2, and unchanged heart rate and cardiac index. Serum Mg concentration decreased from 2.1 +/- 0.1 to 1.7 +/- 0.1 mg/dl. These results suggest that CyA-induced hypertension is characterized by the loss of nocturnal decline in blood pressure, which is accompanied by volume retention after 1 week and systemic vasoconstriction after 4 weeks.
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PMID:[Effects of cyclosporin A on the diurnal variation of blood pressure in patients with nephrotic syndrome]. 919 62

In 1989-1995 229 patients with acute renal failure were treated by hemodialysis. A total of 1470 procedures have been performed, 8.8 +/- 1.5, on the average. 61 patients (26.6%) died. There is no significant relation between duration of oliguria, maximum BUN, creatinine level and lethality. The latter is associated with the patient's age, acute respiratory failure, sepsis, coma, hyperbilirubinemia and hypoproteinemia. Lethality was higher at failure of two and more organs and poor prognosis defined by a simplified acute physiology score (SAPS). 10 patients (5.9%) were discharged with increased creatinine level. Conventional hemodialysis is recommended as a basic technique of dialysis for patients with mono-organ failure and relatively good prognosis according to SAPS scale.
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PMID:[The course and outcome of acute kidney failure in patients treated by hemodialysis]. 929 76

From a clinical aspect, there are still a number of unsolved problems in patients with end-stage renal disease, for instance intractable malnutrition. The present study was undertaken to establish the therapeutic effect of recombinant human growth hormone (r-hGH) on the nutritional state and clarify the usefulness of the insulin-like growth factor-I (IGF-I) insulin-like growth factor binding protein-1 (IGFBP-1) ratio as nutritional indices for prediction of the clinical response in uremic patients on hemodialysis. Thirty hemodialysis patients (13 females and 17 males, mean age 56.7 +/- 15.2 years) were studied who were suffering from malnutrition and could not be treated by any of the usual nutritional therapies; they were subjected to 0.5 IU/kg/week of r-hGH subcutaneously after hemodialysis for 2 weeks. Blood samples were collected for measurement of the following plasma biochemical and hematological indices: serum IGF-I, IGFBP-1, growth hormone, total protein (TP), prealbumin, transferrin, albumin, serum urea nitrogen (SUN), creatinine, hematocrit, and amino acids. Immediately after r-hGH administration, SUN, essential amino acid and nonessential amino acid changed from 67.7 +/- 12.3 to 56.5 +/- 10.5 mg/dl (p < 0.05), from 798 +/- 84 to 1,115 +/- 208 microM/l (p < 0.05 vs. baseline), and from 2,185 +/- 221 to 2,814 +/- 621 microM/l (p < 0.05), respectively. Serum IGF-I increased markedly from 193 +/- 49 to 321 +/- 81 ng/ml, whereas serum IGFBP-1 decreased from 139 +/- 13 to 81 +/- 19 micrograms/l (p < 0.05). Four weeks after r-hGH administration, serum TP and albumin increased from 5.5 +/- 0.2 to 6.0 +/- 0.3 g/dl (p < 0.05) and from 3.2 +/- 0.2 to 3.6 +/- 0.3 g/dl (p < 0.05), respectively. Serum IGF-I/IGFBP-1 ratio was significantly higher in patients with an increase of 0.5 g/dl or more in serum albumin than in other patients with poor response and the control group before r-hGH administration. Patients with marked improvement in serum albumin showed an IGF-I/IGFBP-1 ratio of 2 or less. On the other hand, patients without favorable improvement in serum albumin had a higher ratio of 4 or more. We conclude that r-hGH and IGF-I improve the malnutritional state by alleviating hypoproteinemia and abnormality of serum amino acid profile in uremic patients on hemodialysis. In addition, the serum IGF-I/IGFBP-1 ratio is useful not only as a nutritional parameter but also as a predicting index of responsiveness to r-hGH.
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PMID:IGF-I/IGFBP-1 as an index for discrimination between responder and nonresponder to recombinant human growth hormone in malnourished uremic patients on hemodialysis. 938 Feb 35

The therapeutic effect of probucol on hypercholesterolemia in cyclosporine A (CyA)-treated renal transplant patients was prospectively studied. Twelve posttransplantation patients aged 34.2+/-2.5 years with serum total cholesterol (t-CHL) of 250 mg/dL or greater, whose serum creatinine was 2.9 mg/dL or less, and who had no diabetes mellitus or hypoproteinemia, were treated with probucol, 250 mg twice daily for 3 months. Seventeen age-matched (36.8+/-1.6 years) normal volunteers served as control. Blood was drawn after at least a 12-hour fast to measure lipids in serum and lipoprotein fractions, apoproteins (apo), lipoprotein fractions, lethicin cholesterol acyl transferase (LCAT), free fatty acids (FFA), and CHL-ester. Serum t-CHL, triglycerides (TG), and phospholipids (PL) in posttransplantation patients before treatment were significantly higher compared with normal control subjects. Very-low-density lipoprotein (VLDL) and low-density lipoprotein (LDL) fractions in these patients were significantly expanded. The pretreatment levels of serum apo AII, B, CII, and CIII were significantly increased compared with those of normal controls. After treatment with probucol, serum t-CHL, LDL-CHL, high-density lipoprotein (HDL)-CHL, PL, LDL-PL, and apo AI were significantly decreased, and CHL-ester significantly increased compared with the pretreatment levels. These data suggest that although probucol causes a decrease in HDL-CHL, it may act anti-atherogenically by modulating HDL metabolism and stimulating reverse transfer of CHL from peripheral tissue.
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PMID:Effect of probucol on serum lipoprotein and apoprotein profiles in renal transplant patients. 946 11

Here we report a case of microscopic polyangitis (MPA) with anti-neutrophil cytoplasmic antibodies against myeloperoxidase (MPO-ANCA) accompanied by perceptive dominant hearing loss as the initial sign of the disease. A 52-year-old woman was admitted to Tokyo Metropolitan Ohtsuka Hospital in April 1996 because of bilateral progressive hearing loss. On admission, she had presented bilateral perceptive hearing loss, fever, myalgia, and weight loss. Laboratory data showed accelerated erythrocyte sedimentation rate, proteinuria, microscopic hematuria, leukocytosis, hypoproteinemia, and elevated creatinine level. Serum level of MPO-ANCA was elevated. Chest X-ray and computed tomography showed mild pleural effusion and interstitial shadow in bilateral lung fields. The biopsy findings detected that focal glomerulonephritis, and necrotizing angitis of small artery in kidney. The diagnosis of MPA was made with clinical course, laboratory findings, radiographic findings, and biopsy finding. Treatment was initiated with 45 mg of prednisolone, followed by marked improvement of hearing loss and decreased titer of serum MPO-ANCA. There are only seven reports showing hearing loss as the initial sign of the disease occurred in polyarteritis nodosa and MPA. The present case suggests the possibility that hearing loss may be the symptom of MPO-ANCA associated vasculitis.
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PMID:[A case of microscopic polyangitis accompanied by hearing loss as the initial sign of the disease]. 949 68

A 22-year-old woman was admitted to our hospital for evaluation of fever, renal dysfunction, and a 3-month-history of macrohematuria. Laboratory evaluation revealed proteinuria (1.8 g/day), hypoproteinemia, microcytic microchromic anemia, renal failure (blood urea nitrogen 30.3 mg/dl, serum creatinine 4.0 mg/dl), and positive serum antiglomerular basement membrane (anti-GBM) antibody. Renal biopsy revealed cellular crescents in all 8 glomeruli and partial rupture of the GBM. The interstitium showed severe inflammatory cell infiltration. Immunofluorescent examination revealed linear deposits of IgG and C3 along the GBM. Pulmonary biopsy revealed linear deposits of IgG along the alveolar basement membrane in the immunofluorescent examination. A diagnosis of Goodpasture's syndrome was made because all of the diagnostic criteria were fulfilled. After admission, the patient's renal function deteriorated rapidly. Hemodialysis was started, and the patient was treated with methylprednisolone pulse therapy and oral prednisolone with double filtration plasma pheresis (DFPP). However, her renal function did not improve. On the 30th hospital day, she showed hemoptysis, and a chest X-ray and CT revealed massive bilateral pulmonary hemorrhage. Despite treatment with pulsed methylprednisolone, oral prednisolone (80 mg/day), and DFPP, the pulmonary hemorrhage improved only transiently, worsening again 5 days later. Cyclophosphamide pulse therapy was administered. After this treatment, the patient's pulmonary manifestations and pulmonary hemorrhage improved. At the present time she is on maintenance dialysis therapy without pulmonary manifestations. These findings suggest that cyclophosphamide pulse therapy is effective against Goodpasture's syndrome with massive pulmonary hemorrhage showing resistance to other conventional therapy.
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PMID:[A case of Goodpasture's syndrome with massive pulmonary hemorrhage ameliorated by cyclophosphamide pulse therapy]. 1050 44


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