muramidase and Kidney-Failure--Chronic

Lysozyme amyloidosis (ALys) is a form of hereditary systemic non-neuropathic amyloidosis, which is inherited in an autosomal dominant fashion. Lysozyme, which is the amyloidogenic precursor protein in ALys, is a ubiquitous bacteriolytic enzyme synthesized by hepatocytes, polymorphs and macrophages. The aim of this study is to describe the phenotype and outcome of patients with ALys including the role of solid organ transplantation.. Retrospective evaluation of patients with ALys.. UK National Amyloidosis Centre.. All 16 patients with ALys followed at the centre.. A family history of amyloidosis was present in every affected individual. Although the phenotype was broadly similar amongst those from the same kindred, there were marked phenotypic differences between kindreds who possessed the same amyloidogenic mutation. Symptomatic gastrointestinal (GI) amyloid was prevalent, and macroscopically visible amyloidotic lesions were present in nine of 10 patients who underwent GI endoscopy. All symptomatic ALys individuals had hepatic amyloid. Four patients received orthotopic liver transplants (OLT), three for spontaneous hepatic rupture and one case, who had extensive hepatic amyloid and a strong family history of hepatic rupture, pre-emptively. All of the liver grafts were functioning at censor 1.7, 5.8, 9.0 and 11.0 years after OLT. Five patients had progressive amyloidotic renal dysfunction culminating in end-stage renal failure, three of whom underwent renal transplantation (RTx). There was no evidence of renal allograft dysfunction at censor 6.6, 1.8 and 0.8 years after RTx.. Lysozyme amyloidosis is a disease of the GI tract, liver and kidneys, which has a slow natural history. There was a clear family history in all cases within this cohort, demonstrating a high clinical penetrance in the presence of an amyloidogenic lysozyme mutation. There is currently no amyloid-specific therapy for the condition which is managed symptomatically. OLT and RTx appear to be successful treatments for patients with liver rupture or end-stage renal disease, respectively, with excellent outcomes in terms of medium-term graft function and patient survival.

Topics: Adult; Aged; Amyloidosis, Familial; Child; Female; Gastrointestinal Diseases; Humans; Kidney Failure, Chronic; Kidney Transplantation; Liver Diseases; Liver Transplantation; Lymphatic Diseases; Male; Middle Aged; Muramidase; Mutation; Peptic Ulcer Hemorrhage; Phenotype; Purpura; Radionuclide Imaging; Retrospective Studies; Rupture, Spontaneous; Serum Amyloid P-Component; Sjogren's Syndrome; Survival Analysis; United Kingdom

It is a general opinion that the renal transplant patients may show the decrease of immunity which manifests by their increased susceptibility to bacterial, viral and fungal infections. One of the important non-specific defensive factors of the organism participating in the protection against infections is lysozyme. It is the purpose of the present paper to estimate the lysozyme activity in the un- and diluted serum in the renal transplant patients and to determine the possible mechanism of the observed deviations. 32 renal transplant patients (24 men and 8 women) aged of 16 up to 50 were investigated, being in the period of 1 until 4 years after transplantation. In the investigation the kind of immunosuppressive treatment used was considered which served the purpose of dividing the total group of patients into two subgroups: one treated with cyclosporine (12 patients) and the other treated with azathioprine (20 patients). The control group consisted of 20 healthy persons, matching by age, sex and living place the group investigated. In both groups investigated and control, were determined: lysozyme activity in the serum of the undiluted and diluted blood, total leukocyte number, the absolute number of peripheral blood neutrophils as well as their adherence to fibres was evaluated. In the renal transplant patients compared with the control group and in the separated subgroups, no statistically significant differences were found concerning leukocyte count, absolute neutrophil count and adherence of those cells.(ABSTRACT TRUNCATED AT 250 WORDS)

Topics: Adolescent; Adult; Azathioprine; Cyclosporine; Drug Synergism; Drug Therapy, Combination; Female; Graft Enhancement, Immunologic; Hemodilution; Humans; Kidney Failure, Chronic; Kidney Transplantation; Male; Middle Aged; Muramidase; Postoperative Care; Prednisone

The elevated serum lysozyme activity in 13 chronic renal failure patients (n = 26) dropped significantly during their first three months of CAPD and subsequently returned to initial levels. When compared with peritoneal mass transfer of lysozyme and serum creatinine levels, a distinct correlation was observed between these and the fluctuations in serum lysozyme activity recorded up to three years of CAPD (r = 0.319, P less than 0.05 and r = 0.425, P less than 0.025, respectively). A notable drop in the mass transfer of this low molecular weight protein took place after the first hour of dialysis. We concluded that long-term CAPD does not affect serum lysozyme activity and that passive loss across the peritoneal membrane could account for the lysozyme found in the effluent fluid.

Topics: Adolescent; Adult; Aged; Biological Transport; Dialysis Solutions; Female; Humans; Kidney Failure, Chronic; Male; Middle Aged; Muramidase; Peritoneal Dialysis, Continuous Ambulatory; Peritoneum

The diagnostical relevance of the low-molecular proteins ribonuclease, beta 2-microglobulin and lysozyme in serum and urine to detect a reduced glomerular filtration rate was examined in 52 patients with chronic renal diseases. The radioisotope clearance using 99mTc-DTPA was the base reference; the reference values of the low-molecular proteins were estimated in a control group. Ribonuclease was increased above the upper borderline value, if the glomerular filtration rate was lower than 1.24 ml s-1. Creatinine, beta 2-microglobulin and lysozyme remain yet in part in the normal range. The estimation of the ribonuclease in serum is suitable to detect an impaired glomerular filtration rate if the creatinine value is still not increased. Thereby, the diagnostics in renal diseases may be improved in the creatinine-blind area.

Topics: Adult; beta 2-Microglobulin; Female; Glomerulonephritis; Humans; Kidney Diseases; Kidney Failure, Chronic; Kidney Function Tests; Male; Molecular Weight; Muramidase; Pyelonephritis; Ribonucleases

Topics: Acetylglucosaminidase; Adult; Alkaline Phosphatase; Aminopeptidases; CD13 Antigens; Diagnosis, Differential; Female; gamma-Glutamyltransferase; Glomerulonephritis; Humans; Kidney Failure, Chronic; Male; Muramidase; Pyelonephritis; Urine

The effect of gamma versus ethylene oxide sterilization of different dialyzers (polyacrylonitrile, cuprophan) and blood lines on plasma levels of granulocyte elastase and of lysozyme during hemodialysis was investigated in 17 chronically uremic patients. Plasma levels of granulocyte elastase increased during hemodialysis but significantly less in the presence of polyacrylonitrile compared with cuprophan membranes. In contrast, enhanced lysozyme plasma levels decreased during dialysis using the polyacrylonitrile dialyzer to values of healthy controls and remained unchanged using the cuprophan dialyzer. Both effects were not influenced by the way of sterilization. We conclude that granulocyte activation during hemodialysis occurs independently of the sterilization procedure of dialyzers and blood lines in patients showing no clinical signs of hypersensitivity.

Topics: Acrylic Resins; Adult; Aged; Cellulose; Ethylene Oxide; Female; Gamma Rays; Granulocytes; Humans; Kidney Failure, Chronic; Kidneys, Artificial; Male; Middle Aged; Muramidase; Pancreatic Elastase; Sterilization

Topics: Aged; Diabetic Nephropathies; Humans; Kidney Failure, Chronic; Kidney Function Tests; Leukocyte Count; Muramidase; Reagent Kits, Diagnostic; Urinary Tract Infections

I describe an improved turbidimetric assay for lysozyme in urine. This method is simple, reproducible, linear over a 100-fold concentration range, sensitive to concentrations as low as 10 micrograms/L, and only commercially available products are required. Evaluation of urinary lysozyme concentration with this assay demonstrated a within-run and between-run CV of 9.9% and 4.8%, respectively, for normal urine, minimal chemical interference, and a mean analytical recovery of 104.8%. There were differences in the rate of clearing and in the linearity of the assay with use of three commercial preparations of Micrococcus lysodeikticus, and significant differences in results when different lysozyme standards were used. Reference values were established by use of data on 169 healthy subjects, ages six months to 61 years. Clinical efficacy of the assay was demonstrated in children with renal disorders, especially those associated with chronic renal failure or tubular dysfunction.

Topics: Adolescent; Adult; Child; Child, Preschool; Humans; Infant; Kidney Diseases; Kidney Failure, Chronic; Micrococcus; Middle Aged; Muramidase; Nephelometry and Turbidimetry; Reference Values; Statistics as Topic

In patients on regular dialysis treatment, uremic symptoms (anemia, osteopathy, myopathy, neuropathy, and disorders of carbohydrate, fat, and protein metabolism) may be partly due to an accumulation of low molecular weight (MW) proteins (10,000 to 60,000 daltons). We tested this hypothesis using membranes with a higher permeability than conventional Cuprophan membranes. The primary aim of the study was to test the cutoff of various hemofilters (Cuprophan [Highflux], polyamide [FH 20], cellulose acetate [Duoflux], and polyacrylonitrile [Hospal RP 7 + 8 and Asahi PAN]) under in vivo conditions. In addition the effects of hemofiltration on plasma low molecular weight protein concentrations, polyneuropathy, and autonomic insufficiency were also tested in a long-term (six-month) study using the membrane with the highest cutoff and most constant sieving coefficient, i.e., Highflux. Low MW proteins with a defined MW were used as marker substances. Sieving coefficients of beta 2-microglobulin, lysozyme, retinol-binding protein, alpha 1-glycoprotein, alpha 1-antitrypsin, prealbumin, albumin, and transferrin were determined during a four-hour hemofiltration (20 L ultrafiltrate). Proteins were analyzed using an immunodiffusion technique. In the long-term study, motor nerve conduction velocity, the Schellong test, and Valsalva maneuver were tested prior to and three and six months after hemofiltration therapy. Highflux, Duoflux, and FH 202 membranes were permeable to proteins with molecular weights up to 15,000 daltons, and the Highflux module had the most constant sieving coefficient during hemofiltration. In the six-month hemofiltration study with the Highflux filter, plasma beta 2-microglobulin and lysozyme decreased significantly as expected. Parameters of polyneuropathy and autonomic insufficiency were slightly improved.

Topics: Adult; Aged; beta 2-Microglobulin; Blood; Blood Pressure; Blood Proteins; Female; Heart Rate; Humans; Kidney Failure, Chronic; Long-Term Care; Male; Membranes, Artificial; Middle Aged; Molecular Weight; Muramidase; Neural Conduction; Retinol-Binding Proteins; Retinol-Binding Proteins, Plasma; Ultrafiltration

Lysozyme activity was determined in the serum, urine and leukocytes of 53 patients with immunocytoma and 24 patients with lymphoproliferative syndromes without associated monoclonal gammapathy. In patients with multiple myeloma the frequency of low serum lysozyme activity and high leukocyte lysozyme activity was higher. In the cases with renal failure, lysozyme activity was raised in serum and urine, and the 24-hour urinary lysozyme excretion was increased. In 7 patients with increased urinary lysozyme excretion no clinical or laboratory evidence of renal complications was found. Relative monocytosis in peripheral blood was observed in half of the cases of multiple myeloma, and in these patients also in about half of the cases the lysozyme activity was raised in the leukocytes and urine, and the 24-hour urinary lysozyme excretion was increased. In patients with Hodgkin's disease, lymphosarcoma and chronic lymphatic leukemia the frequency of low serum lysozyme activity was increased.

Topics: Adult; Aged; Humans; Kidney Diseases; Kidney Failure, Chronic; Leukocytes; Lymphoproliferative Disorders; Middle Aged; Multiple Myeloma; Muramidase; Waldenstrom Macroglobulinemia

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.

Topics: Acute Disease; Amylases; Creatinine; Glomerulonephritis; Humans; Kidney Diseases; Kidney Failure, Chronic; Kidney Glomerulus; Muramidase; Nephrotic Syndrome; Pancreatitis; Proteinuria; Pyelonephritis

A quick and simple method for the estimation of lysozyme activity using the Fragiligraph, was described. Diminution of turbidity in a suspension of Micrococcus lysodeikticus produced by the addition of standard lysozyme (hen egg white) or serum sample, was continuously recorded for 5 min by the Fragiligraph. The normal mean serum lysozyme activity value obtained by this method is 6,80 mug/ml +/- 1.85.

Topics: Autoanalysis; Bacteriolysis; Humans; Kidney Failure, Chronic; Leukemia; Micrococcus; Muramidase; Spectrophotometry

The mean concentrations of serum lysozyme were markedly higher in patients with Crohn's disease and ulcerative colitis than in normal controls, and mean levels tended to be slightly higher in those with Crohn's disease than in those with colitis. The significance of these differences is unclear but the overlap between values in normal individuals and those with inflammatory bowel disease prevents the measurement having any discriminant value.

Topics: Adult; Colitis, Ulcerative; Female; Humans; Kidney Failure, Chronic; Leukocyte Count; Male; Monocytes; Muramidase; Neutrophils; Spectrophotometry

The serum muramidase levels were measured in 128 patients with primary or metastatic colorectal cancer, 166 tumour-free patients after resection of a colorectal cancer, and 172 controls. Muramidase levels over 10 mug/ml were detected in 30%-39% of the tumour-bearing patients, in 8.2% of the tumour free, and in only 1.7% of the controls (normal level 6.68 +/- 1.42 mug/ml). Long-term follow up indicated that raised levels may occur as a transient phenomenon in recurrent or metastatic disease. The likely relation of abnormal serum muramidase activity and stimulation of the reticuloendothelial system is discussed.

Topics: Adenocarcinoma; Carcinoembryonic Antigen; Colonic Neoplasms; Creatinine; gamma-Glutamyltransferase; Humans; Kidney Failure, Chronic; Leucyl Aminopeptidase; Liver Neoplasms; Muramidase; Neoplasm Metastasis; Rectal Neoplasms; Recurrence

Topics: Adolescent; Adult; Aged; Blood Urea Nitrogen; Child; Creatinine; Glomerular Filtration Rate; Glomerulonephritis; Humans; Kidney Diseases; Kidney Failure, Chronic; Middle Aged; Muramidase; Renal Dialysis

Topics: Bence Jones Protein; Blood Protein Disorders; Creatinine; Humans; Immunoelectrophoresis; Immunoglobulin Fragments; Immunoglobulins; Kidney; Kidney Failure, Chronic; Kidney Transplantation; Metabolic Clearance Rate; Molecular Weight; Multiple Myeloma; Muramidase; Transplantation, Homologous

On the assumption that increased urinary lysozyme concentration (;lysozymuria') indicates tubular proteinuria and therefore impaired tubular function, urinary lysozyme has been estimated in acute disorders where transient disturbances of renal function might be expected, in cases diagnosed clinically as extrarenal uraemia, and in a few examples of acute renal disease. Reversible lysozymuria occurred with hypokalaemia, postoperative ;collapse', electrolyte depletion, severe extrarenal infection, acute pyelonephritis, the nephrotic syndrome, after a few apparently uncomplicated surgical operations, and very transiently after ventricular fibrillation abolished by DC shock. There was no lysozymuria with severe uraemic heart failure, aspirin and paracetamol poisoning, or severe jaundice, nor in two cases of acute glomerulonephritis. Although lysozymuria may occasionally be useful in the clinical diagnosis of acutely disordered renal function, the results suggest that its value is limited; on the other hand, they have provided information on renal pathophysiology in acute disease.

Topics: Acetaminophen; Acute Disease; Aspirin; Electroshock; Glomerulonephritis; Heart Failure; Humans; Hypokalemia; Jaundice; Kidney; Kidney Diseases; Kidney Failure, Chronic; Kidney Tubules; Muramidase; Myocardial Infarction; Nephrotic Syndrome; Pneumonia; Postoperative Complications; Proteinuria; Pyelonephritis; Uremia; Ventricular Fibrillation

Topics: Adult; Chronic Disease; Female; Humans; Kidney Failure, Chronic; Kidney Transplantation; Lymph; Male; Middle Aged; Muramidase; Renal Dialysis; Transplantation, Homologous; Uremia

Topics: Adult; Aged; gamma-Globulins; Humans; Kidney Failure, Chronic; Kidney Transplantation; Leukocyte Count; Lymphocytes; Muramidase; Transplantation Immunology; Transplantation, Homologous

Topics: Acute Kidney Injury; Diagnosis, Differential; Glomerular Filtration Rate; Humans; Kidney Diseases; Kidney Failure, Chronic; Kidney Tubules; Muramidase