muramidase and Syndrome

Diffuse haemangiomatosis of the spleen is a rare benign vascular condition occurring as a manifestation of systemic angiomatosis or, less commonly, confined to the spleen. It is sometimes accompanied by severe disturbance of blood coagulation. The goal of this study was to characterize an additional case of isolated diffuse haemangiomatosis of the spleen and to determine the histogenesis of this lesion which remains obscure.. We describe a case of isolated diffuse haemangiomatosis of the spleen in which histological and immunohistological findings suggested the possibility of a malformative tumour-like lesion. The pathological cavernous vessels were distributed randomly through the red pulp, without continuity with sinuses. The endothelial cells expressed vimentin, factor VIII related antigen and CD34, but not CD8. Some cells lining the sinus lumen expressed CD68, lysozyme and myeloperoxidase. In addition, trabecular veins presented with intimal thickening. These results allow making a diagnosis between diffuse haemangiomatosis and other tumours/tumour-like lesions of the spleen, especially littoral cell angioma, splenoma and peliosis.. If diffuse haemangiomatosis is usually classified as a benign proliferation of endothelial cells, we suggest that diffuse haemangiomatosis, when confined to the spleen, could be a tumour-like vascular lesion. In this hypothesis, the aetiology may be hamartomatous or malformative as is suspected in arterio-venous haemangioma of the lower extremities. The histogenesis is still questionable and no definitive proof in favour of one or the other hypothesis has been reported.

Topics: Aged; Antigens, CD; Antigens, CD34; Antigens, Differentiation, Myelomonocytic; Diagnosis, Differential; Factor VIII; Hemangioma; Humans; Male; Muramidase; Peroxidase; Splenic Neoplasms; Syndrome; Vimentin

Retrospective analysis of patients with acute myelomonoblastic (or nonlymphocytic) leukemia has helped to clarify the early stages of the evolution of this disease, and a clinical picture has emerged that is sufficiently discriminating to permit prospective observations. The clinical and laboratory features of the hematologic disorder preceding overt, blast-cell leukemia (i.e., the "preleukemic syndrome" or "hemopoietic dysplasia") are described, and diagnostic criteria and approaches to management are considered. This hematologic syndrome appears to be a stage in a multiphasic panmyelopathy that involves all products of the marrow stem cell. Available data indicate that most of these patients develop acute leukemia within 24 months of the onset of the hematologic abnormalities, but longer courses are not uncommon. Prospective documentation in a large group of patients is needed before the natural course can be accurately defined; such studies are now being planned.

Topics: Adrenal Cortex Hormones; Aged; Antineoplastic Agents; Blood Transfusion; Bone Marrow; Child; Chromosome Aberrations; Colony-Forming Units Assay; Erythrocyte Transfusion; Erythrocytes; Female; Humans; Iron; Male; Middle Aged; Muramidase; Preleukemia; Pyridoxine; Splenectomy; Syndrome

While lung transplant is an effective therapy for advanced lung disease, chronic allograph rejection remains a primary basis for lower survival rates than those for other solid organ transplants. This study used carefully controlled Zip-Tip extraction of bronchoalveolar lavage fluid (BALF) followed by MALDI-TOF MS to identify biomarkers of chronic lung transplant rejection. Many differences were observed between controls, those who did not develop chronic rejection within 100 months, and patients who had developed chronic rejection, diagnosed as bronchiolitis obliterans syndrome (BOS). Intensity ratios of peaks within the same MALDI-TOF profile were used to quantify the result. One of the best identifiers of BOS was a lowered ratio of clara cell protein (CCP m/z = 15,835) to lysozyme (m/z = 14,700), which gave 94% specificity and 74% sensitivity for diagnosis. Furthermore, low values for CCP/Lysozyme (<0.3) were observed in 66% of samples taken at 1 to 15 months prior to the diagnosis of BOS. Many other components of the profile gave similar or better outcomes for diagnosis but tended to be less valuable for the prediction of future disease. Overall, this study demonstrated the feasibility of this approach for the detection of disease biomarkers.

Topics: Biomarkers; Bronchiolitis Obliterans; Bronchoalveolar Lavage Fluid; Chronic Disease; Feasibility Studies; Graft Rejection; Humans; Lung Transplantation; Muramidase; Postoperative Complications; Retrospective Studies; Sensitivity and Specificity; Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization; Syndrome; Uteroglobin

Rheumatoid arthritis was diagnosed in a 30-year-old woman with erythema nodosum and arthritic symptoms since 1994, and she was treated with anti-rheumatic agents. Mediastinal and bilateral hilar lymphadenopathy and abnormal pulmonary shadows were detected in 1996, and she was admitted to our hospital in 1997. We also recognized the elevation of ACE and lysozyme, and found granulomas in a transbronchial lung biopsy and an arthrosis synovia biopsy. From these findings, sarcoidosis was diagnosed. Sarcoidosis demonstrating erythema nodosum, arthritis, and bilateral hilar lymphadenopathy is called Löfgren's syndrome. In Caucasians, Löfgren's syndrome is frequently encountered, but it is rare in Japanese. Our case had coexisting arthrosis symptoms, and satisfied the diagnosis criteria of rheumatic arthritis. Therefore, the differential diagnosis was important. We emphasize that it is necessary to consider Löfgren's syndrome when diagnosing patients with rheumatic features, even in Japan.

Topics: Adult; Arthritis; Arthritis, Rheumatoid; Biomarkers; Diagnosis, Differential; Erythema Nodosum; Female; Humans; Lung; Lymphatic Diseases; Muramidase; Peptidyl-Dipeptidase A; Sarcoidosis; Syndrome; Synovial Membrane; Tomography, X-Ray Computed

In the deteriorating group of sarcoidosis patients, progress towards pulmonary fibrosis is a major problem. In order to benefit from corticosteroids, it is important for the treatment to start early. We studied a group of 45 patients with sarcoidosis. Most of them were newly detected patients and none were under or had currently received corticosteroid therapy. The patients were followed for at least six months. We found that increased amounts of polymorphonuclear neutrophils (PMN) or lysozyme-positive macrophages (Lys+MF) and mast cells (MC) in bronchoalveolar lavage (BAL) could implicate a bad prognosis.

Topics: Bronchi; Erythema Nodosum; Female; Follow-Up Studies; Humans; Lung Neoplasms; Macrophages; Male; Mast Cells; Middle Aged; Muramidase; Neutrophils; Prognosis; Pulmonary Alveoli; Sarcoidosis; Syndrome; Therapeutic Irrigation

A case of pagetoid reticulosis is presented. Histopathology showed infiltration of the epidermis by mononuclear cells. Twenty percent of the mononuclear cells showed the presence of lysozyme indicating a histiocytic origin. Electron microscopy confirmed the presence of lymphocytes and histiocytes but these cells were outnumbered by Sézary cells. The presence of large numbers of Sézary cells indicates that pagetoid reticulosis is a cutaneous T-cell lymphoma closely related to mycosis fungoides.

Topics: Adult; Histiocytes; Humans; Immunoenzyme Techniques; Lymphatic Diseases; Male; Microscopy, Electron; Muramidase; Skin; Skin Neoplasms; Syndrome

Topics: Acetylglucosaminidase; Alkaline Phosphatase; alpha-Glucosidases; Aminopeptidases; beta-Galactosidase; beta-Glucosidase; CD13 Antigens; Clinical Enzyme Tests; gamma-Glutamyltransferase; Glucuronidase; Humans; Isoenzymes; Kidney Diseases; Kidney Neoplasms; L-Lactate Dehydrogenase; Muramidase; Syndrome

An 18-year-old boy showed childhood onset of mental retardation, neurogenic muscle atrophy with hyperreflexia, Marfan-like features, multiple epiphyseal dysplasia, increased urinary excretion of dermatan sulfate, and decreased lysosomal enzyme activities in beta-galactosidase, beta-glucuronidase, and N-acetyl-beta-D-glucosaminidase. This case may be a new syndrome, the combination of neurogenic muscle atrophy with lysosomal enzyme deficiencies.

Topics: Adolescent; Diagnosis, Differential; Humans; Intellectual Disability; Leukocytes; Male; Metabolism, Inborn Errors; Mucopolysaccharidoses; Muramidase; Muscular Atrophy; Reflex, Abnormal; Syndrome

The course of meningococcal infection and nonspecific and specific immunity in children subjected to different regimens of pathogenetic therapy were studied. It was shown that the clinico-immunological indices were more favourable in children treated with lasix as a diuretic agent. Recovery with defects was observed in 6.8 per cent of the children of this group, while in patients subjected to routine treatment the respective value amounted to 14.6 per cent. The use of lasix in combination with penicillin increased the efficacy of penicillin therapy and shortened the recovery period by 8.4 +/- 0.2 days as compared to that of routine treatment. No unfavourable effect of elevated penicillin concentrations on the lysozyme activity, blood bactericidal characteristics and composition of immunoglobulins A, M and G in the children was recorded.

Topics: Adolescent; Blood Bactericidal Activity; Child; Child, Preschool; Drug Therapy, Combination; Furosemide; Humans; Immunoglobulins; Infant; Meningitis, Meningococcal; Meningococcal Infections; Muramidase; Penicillins; Sepsis; Syndrome

A simple, rapid, reproducible method of quantifying tear lysozyme levels with a dual-channel spectrophotometer was used to compare normal subjects and those with dry-eye syndrome. The method was sensitive (80%) and specific (85%) and had a predictive value of a positive result of 83%. One patient with clinical manifestations of dry-eye syndrome and paradoxically elevated levels of tear lysozyme was found to have underlying sarcoidosis.

Topics: Humans; Lacrimal Apparatus Diseases; Muramidase; Sarcoidosis; Sjogren's Syndrome; Syndrome; Tears

Electrophoresis of tears, a rapid and easy technique, shows several peaks of migration. Three main peaks correspond to the proteins synthesized by the lacrimal gland: rapid migrant proteins, lactoferrin migrating to the anode, and lysozyme migrating to the cathode. Secretory tissue injuries of the lacrimal gland show a more or less important decrease of one or several peaks. Any inflammatory reaction induces an increase in the serum albumin and immunoglobulin fractions. This exploration can be helpful in the diagnosis of the sicca syndrome or of drug poisoning thereby avoiding lacrimal biopsy.

Topics: Electrophoresis, Cellulose Acetate; Humans; Immunoglobulins; Lacrimal Apparatus; Lacrimal Apparatus Diseases; Lactoferrin; Muramidase; Proteins; Serum Albumin; Syndrome; Tears

Topics: Adult; Aged; Amino Acids; Betamethasone; Deglutition Disorders; Galantamine; Glossopharyngeal Nerve; Humans; Male; Muramidase; Pharyngeal Diseases; Pyrrolidines; Syndrome; Thiamine; Virus Diseases; Vitamin B 12

Topics: Humans; Keratoconjunctivitis; Muramidase; Sjogren's Syndrome; Syndrome; Tears

Microcystic corneal dystrophy was first described in 1964. Since then, several reports have elaborated on the original findings plus other associated corneal changes. The cause is unknown, but pathological studies have been done in several cases. The corneal changes are subtle and easy to miss on routine examination. This study of 17 patients was undertaken to further evaluate the signs and symptoms of this abnormality and to emphasize techniques of examination. Detailed clinical and laboratory evaluations of tear function were performed. No definite association between tear function and the corneal changes could be determined. In this group of patients, the process seemed benign with minimal symptoms.

Topics: Aged; Cornea; Corneal Dystrophies, Hereditary; Electrophoresis; Epithelium; Female; Fluorescein Angiography; Functional Laterality; Humans; Male; Middle Aged; Muramidase; Ophthalmoscopy; Spectrophotometry; Syndrome; Tears; Visual Acuity

Topics: Child; Child, Preschool; Corneal Opacity; Diet Therapy; Edema; Hospitalization; Humans; Kwashiorkor; Leukocytes; Muramidase; Night Blindness; Proteins; Serum Albumin; Spectrophotometry; Syndrome; Vitamin A; Vitamin A Deficiency; Xerophthalmia

Topics: Acute Disease; Age Factors; Aged; Agranulocytosis; Anemia; Bone Marrow Examination; Cell Transformation, Neoplastic; Ecchymosis; Erythropoiesis; Female; Hematocrit; Humans; Iron; Leukemia; Leukemia, Monocytic, Acute; Leukemia, Myeloid; Leukocytes; Male; Middle Aged; Muramidase; Myeloproliferative Disorders; Splenomegaly; Syndrome; Thrombocytopenia