muramidase and Agammaglobulinemia

Topics: Agammaglobulinemia; Anemia, Sickle Cell; Chemotaxis; Female; Glucosephosphate Dehydrogenase Deficiency; Humans; Immunity, Cellular; Immunoglobulin E; Immunologic Deficiency Syndromes; Infections; Job Syndrome; Lactoferrin; Leukocytes; Male; Muramidase; Opsonin Proteins; Peroxidase; Phagocytes; Phagocytosis; Staphylococcus

Topics: Adult; Agammaglobulinemia; Biomarkers; Carboxylesterase; Carboxylic Ester Hydrolases; Epithelium; Female; Fibroblasts; Granuloma; Humans; Hypersplenism; Immunologic Deficiency Syndromes; Macrophages; Muramidase; Organ Size; Spleen; Splenectomy; Splenic Diseases

The ocular findings in three brothers with Bruton's disease are reported. All three boys had purulent conjunctivitis, but the two older brothers also developed marked corneal scarring with visual impairment. Haemophilus influenzae was cultured from conjunctival swabs; it was resistant to neomycin but sensitive to chloramphenicol. Tear analysis showed that the three subjects had normal levels of lysozyme but no detectable IgA.

Topics: Agammaglobulinemia; Child; Chloramphenicol; Conjunctivitis, Bacterial; Corneal Diseases; Haemophilus Infections; Humans; Immunoglobulins; Infant; Male; Muramidase; Secretory Component; Tears

1) Certain selective IgA-deficient subjects are capable of synthesizing functional secretory antibodies (s-IgM or s-IgA class) to indigenous oral microorganisms. 2) The presence or absence of these secretory antibodies in saliva can be correlated with the extent of caries involvement. 3) Lysozyme activity is significantly increased in saliva from immune deficient subjects though no correlation can be made with caries experience.

Topics: Agammaglobulinemia; Dental Caries; Humans; Immunoglobulin A; Immunoglobulin A, Secretory; Immunoglobulin M; Lactoferrin; Muramidase; Parotid Gland; Saliva; Submandibular Gland

The electrophoretic mobility of serum lysozyme in 2 patients with raised enzyme levels was identical to that of gamma-globulins. Similar mobility was observed after incubation of lysozyme and normal serum. Incubation with one hypogammaglobulinemic serum showed that lysozyme could also acquire alpha2 mobility.

Topics: Agammaglobulinemia; Alpha-Globulins; Electrophoresis, Cellulose Acetate; gamma-Globulins; Humans; Leukemia, Myeloid, Acute; Leukocytosis; Muramidase

Topics: Agammaglobulinemia; Age Factors; Bacterial Infections; Bronchitis; Child, Preschool; Chronic Disease; Complement System Proteins; Humans; Immunoglobulin A; Immunoglobulin G; Infant; Muramidase; Virus Diseases

Spontaneous amyloidosis was noted in a significant number of germfree mice in comparison with their conventional contemporaries. The adjusted prevalence of this disease was increased in both groups by whole-body exposure at 6 weeks of age to 700 rad of ionizing radiation. The germfree groups demonstrated persistent hypogammaglobulinemia throughout their lifespans and no evidence of significant inflammatory processes at necropsy. The possible interpretation of these observations is discussed and it is concluded that defective or deficient immunoglobulin production may be the essential prerequisite for the development of amyloidosis.

Topics: Agammaglobulinemia; Age Factors; Amyloid; Amyloidosis; Animals; Aspartate Aminotransferases; Blood Chemical Analysis; Blood Proteins; Blood Urea Nitrogen; Cobalt Isotopes; Disease Models, Animal; Female; Germ-Free Life; Immunosuppression Therapy; Kidney; L-Lactate Dehydrogenase; Lymphoma, Non-Hodgkin; Mice; Muramidase; Radiation Effects; Serum Albumin; Serum Globulins; Thymus Neoplasms

Serum levels, urinary excretion, and clearances of several proteins of different molecular weights were studied in 18 patients with mono- and myelomonocytic leukemia. Nine patients had normal renal function (group A) and nine had impaired renal function with azotemia (group B). The majority of patients in both groups had increased concentration of immunoglobulins, particularly IgG, IgA, and IgM; IgD level was normal. Serum transferrin and alpha(2)-macroglobulin were frequently reduced while the level of ceruloplasmin was often increased, especially in patients with azotemia. The activity of lysozyme in the serum was high in all patients, but was considerably higher in group B. Proteinuria was found in most patients but was more prominent in group B. Almost invariably albumin constituted less than 25% of the total protein excreted. Qualitative analysis of various urinary proteins by immunochemical techniques and clearance studies suggested the presence of glomerular as well as tubular dysfunction. Determination of urinary lysozyme frequently showed no direct correlation between the serum level of the enzyme and its concentration in the urine or its clearance by the kidney. In addition to glomerular filtration, impaired tubular reabsorption may account for the high level of lysozyme in the urine. It is postulated that the very high level of lysozyme in the glomerular filtrate and possibly hypergammaglobulinemia may play a role in the induction of tubular damage. Renal impairment has been correlated with histological changes in the kidneys. From a comparative study of various leukemias, it seems that the combined glomerular-tubular dysfunction is a manifestation unique to mono- and myelomonocytic leukemia.

Topics: Adult; Agammaglobulinemia; Aged; Albuminuria; Blood Chemical Analysis; Blood Proteins; Ceruloplasmin; Female; Humans; Hypergammaglobulinemia; Immunoglobulins; Kidney Glomerulus; Kidney Tubules; Leukemia, Myeloid; Male; Middle Aged; Muramidase; Nitrogen; Proteinuria; Transferrin; Uremia

Topics: Agammaglobulinemia; Asthma; Bronchitis; Chronic Disease; Cystic Fibrosis; Humans; Marfan Syndrome; Muramidase