muramidase and Liver-Diseases

Topics: Amyloidosis; Apolipoproteins A; Cardiomyopathies; Dialysis; Fibrinogen; Humans; Kidney Diseases; Liver Diseases; Muramidase; Organ Transplantation; Prealbumin

Topics: Animals; Avitaminosis; Bronchoscopy; Burns; Central Nervous System Diseases; Chick Embryo; Child; Crystallization; Ear Diseases; Eye Diseases; Female; Gastrointestinal Diseases; Genital Diseases, Female; Humans; Liver Diseases; Mouth Diseases; Muramidase; Nose Diseases; Postoperative Complications; Respiratory Tract Diseases; Typhus, Epidemic Louse-Borne; Vascular Diseases; Wounds and Injuries

Topics: Animals; Autoimmune Diseases; Biopsy; Colitis, Ulcerative; Colonic Diseases; Colonic Neoplasms; Diagnosis, Differential; Eye Manifestations; Humans; Lactose Intolerance; Liver Diseases; Milk; Muramidase; Prognosis; Psychophysiologic Disorders; Radiography; Sigmoidoscopy

Topics: Aged; Amyloidosis, Familial; DNA Mutational Analysis; Female; Hematoma; Humans; Liver Diseases; Molecular Diagnostic Techniques; Muramidase; Mutation, Missense; Recurrence

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

Tissue deposition of soluble proteins as amyloid fibrils underlies a range of fatal diseases. The two naturally occurring human lysozyme variants are both amyloidogenic, and are shown here to be unstable. They aggregate to form amyloid fibrils with transformation of the mainly helical native fold, observed in crystal structures, to the amyloid fibril cross-beta fold. Biophysical studies suggest that partly folded intermediates are involved in fibrillogenesis, and this may be relevant to amyloidosis generally.

Topics: Amyloid; Amyloidosis; Circular Dichroism; Cloning, Molecular; Crystallography, X-Ray; Enzyme Stability; Hot Temperature; Humans; Liver Diseases; Models, Molecular; Muramidase; Point Mutation; Protein Conformation; Protein Denaturation; Protein Folding; Protein Structure, Secondary; Recombinant Proteins

The study was designed to evaluate (a) the role of reduced renal phosphate reabsorptive capacity assessed as the ratio of maximum capacity for renal phosphate reabsorption (TmPO4) to glomerular filtration rate (GFR) in the pathogenesis of hypophosphatemia in alcoholics, (b) possible mechanisms leading to reduced TmPO4/GFR, and (c) the effect of liver function impairment on TmPO4/GFR. The TmPO4/GFR, its major extrarenal determinants, ratios of urinary excretion gamma-glutamyl transpeptidase and of alpha-glucosidase to GFR (uGGT/GFR and uAGL/GFR), indices of structural damage of renal tubular cells, and fractional clearance of lysozyme, an index of proximal renal function, were evaluated in 31 alcoholics with alcohol-related liver disease, 24 alcoholics without alcohol-related liver disease, 14 patients with non-alcohol-related liver disease, and 25 control subjects. Hypophosphatemia was found in 35% of alcoholics with alcohol-related liver disease, 29% of alcoholics without alcohol-related liver disease, and no patients with non-alcohol-related liver disease. A reduced TmPO4/GFR was the major determinant of hypophosphatemia in both groups of alcoholics. No difference in extrarenal determinants of TmPO4/GFR was found between alcoholics with and without hypophosphatemia. Alcoholics with and without alcohol-related liver disease had increased uGGT/GFR and normal uAGL/GFR regardless of serum phosphate level. Fractional clearance of lysozyme, instead, was increased only in hypophosphatemic alcoholics with and without alcohol-related liver disease. The TmPO4/GFR correlated inversely with the fractional clearance of lysozyme in both groups of alcoholics (P less than 0.01). The TmPO4/GFR and urinary enzymes were normal in patients with non-alcohol-related liver disease. It was concluded that a reduced TmPO4/GFR is involved in the pathogenesis of hypophosphatemia in alcoholics. A proximal tubular dysfunction seems to be responsible for the reduced TmPO4/GFR. Liver function impairment is not required for the expression of this tubular dysfunction.

Topics: Adult; Aged; Alcoholism; alpha-Glucosidases; Female; gamma-Glutamyltransferase; Glomerular Filtration Rate; Humans; Kidney Tubules; Liver Diseases; Liver Diseases, Alcoholic; Male; Middle Aged; Muramidase; Parathyroid Hormone; Phosphates; Transaminases

Serum lysozyme activities and semiquantitative analysis of tissue lysozyme distribution were studied in patients with primary biliary cirrhosis (PBC), chronic hepatitis (CH), miscellaneous liver diseases, and normal subjects. Serum lysozyme was significantly raised in PBC and CH. Portal venous blood has similar lysozyme activities to peripheral venous blood in a group of various liver diseases. Lysozyme-containing intralobular cells were decreased in all liver diseases studied but portal tract lysozyme was increased only in PBC and CH. Thus the increase in serum lysozyme in PBC and CH appears to originate from the portal inflammatory infiltrate, seen in these diseases.

Topics: Chronic Disease; Hepatitis; Humans; Immunoenzyme Techniques; Liver; Liver Cirrhosis, Biliary; Liver Diseases; Muramidase

Topics: Adult; Aged; Cathepsins; Clinical Enzyme Tests; Female; Humans; Liver Diseases; Lysosomes; Male; Middle Aged; Muramidase; Ribonucleases

Topics: Adult; Aged; Collagen; Female; Gaucher Disease; Humans; Liver; Liver Diseases; Male; Middle Aged; Muramidase; Peptidyl-Dipeptidase A

Measurements of urinary lysozyme were used to evaluate renal tubular integrity in 34 patients with cirrhosis or fulminant hepatic failure who had developed renal impairment. In 18 of the patients the lysozyme values were normal but in the remaining 16 were increased, supporting previous concepts that renal failure complicating hepatocellular disease may occur both without and with tubular necrosis. The lysozyme values were inversely related to the creatinine clearance, suggesting that the development of tubular necrosis may be determined by the level of renal perfusion. The validity of simpler laboratory tests often used to assess renal tubular integrity--namely, the urine sodium concentration, the urine:plasma osmolality ratio, and casts in the urine sediment--was evaluated by comparison with the lysozyme measurements. The urine sodium concentration was of most value and the findings in the sediment were of no value at all.

Topics: Acute Kidney Injury; Humans; Kidney Tubular Necrosis, Acute; Kidney Tubules; Liver Cirrhosis; Liver Diseases; Muramidase; Sodium

Topics: Adult; Aged; Aorta; Arteriosclerosis; Arthritis, Rheumatoid; Collagen; Connective Tissue; Ear Ossicles; Female; Galactosidases; Gastric Mucosa; Glycoside Hydrolases; Hepatitis A; Histocytochemistry; Humans; Hyaluronoglucosaminidase; Hydrochloric Acid; Hydroxides; Liver Diseases; Macromolecular Substances; Male; Microbial Collagenase; Middle Aged; Muramidase; Neuraminidase; Otosclerosis; Sodium Chloride; Stomach Ulcer; Synovial Membrane; Urea

Topics: Adolescent; Adult; Age Factors; Asthma; Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Leukemia; Liver Diseases; Male; Muramidase; Nephrotic Syndrome; Sex Factors

Topics: Amides; Animals; Antibodies; Bilirubin; Carbon Tetrachloride Poisoning; Chemical and Drug Induced Liver Injury; Cryptococcosis; Ethanol; Hexachlorocyclohexane; Hyperbilirubinemia; Iron; Liver; Liver Diseases; Male; Muramidase; Naphthalenes; Necrosis; Nitrosamines; Protein Deficiency; Rats; Sulfhydryl Compounds; Thiocyanates

Topics: Blood Coagulation; Hematologic Agents; Liver Diseases; Muramidase