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Biomedical subjects

Y Matzner

Publications and source records attributed to Y Matzner.

At least 37 records · Page 2Linked to original sources
PubMed1995

Purification and characterization of a C5a-inactivating enzyme from human peritoneal fluid.

Earlier work has suggested that familial Mediterranean fever, an inherited disorder characterized by sporadic episodes of inflammation involving the pleural and peritoneal cavities and the joints, is caused by the lack of a C5a inactivator normally found in serosal fluid. We have purified this inactivator from ascites fluid and obtained a protein of molecular weight 53 to 56 kD with a specific activity 10,000-fold greater than the crude material. On Western blot, an inhibitory antibody recognized a single antigenic species at the same molecular weight. The enzyme had no activity against denatured bovine serum albumin. With recombinant C5a as substrate, the Km and Vm were 3.4 mumol/L and 52 nmol C5a/min/mg protein, respectively.

Ascitic Fluid↗
PubMed1995

[Laparoscopic splenectomy for immune thrombocytopenic purpura].

Splenectomy is effective in treating immune thrombocytopenic purpura (ITP). Recent advances in laparoscopic technique and technology have made laparoscopic splenectomy feasible. We performed it in 8 cases of ITP (16-34 years old, and 2-24 months after the diagnosis was made and appropriate treatment started). Indications for splenectomy were no response to corticosteroid therapy (2 patients), decrease in platelet count when attempting to taper off therapy (3), or severe side-effects of the treatment (3). 4-5 ports were used. The splenic artery was first double-clipped through an opening in the gastrocolic ligament and then the lower splenic pole and the posterolateral attachments were dissected using endoclips and electrocautery. The hilum and short gastric vessels were separated using an endostapler. The spleen was placed in a plastic bag whose open end was pulled out through an umbilical incision and the spleen fragmented and aspirated out of the bag, while it was still inside the abdomen. Blood or platelet transfusions were not needed and the postoperative course was uneventful in all, with early return to full normal activity. Postoperatively, platelets increased to more than 150,000/mm3 in all patients, and there was no further need for corticosteroids during a follow-up of 2-12 months. We recommend laparoscopic splenectomy for ITP because of the reduced operative trauma, better recovery and rehabilitation, less postoperative pain, cosmetic advantage, and possibly fewer postoperative complications.

Adolescent↗
PubMed1995

Disorders of neutrophil function.

Major bacterial infections are most commonly associated with agranulocytosis or an abnormality of immunoglobulins or complement. Occasionally, repeated infections cannot be attributed to these relatively common causes. In such cases, a quantitative abnormality in neutrophil function should be sought. Complete evaluation of neutrophil function, including: chemotaxis, adhesion, aggregation, phagocytosis, granule content and degranulation, respiratory burst activity and bacterial killing is expensive and requires the services of a specialized laboratory. However, preliminary screening of a patient with a predisposition towards infection can be carried out using simple and inexpensive methods. These include examination of blood films, chemotaxis assay, NBT test and peroxidase staining. For final diagnosis and determination of genetic transmission and treatment, specific tests are indicated. Investigation of neutrophil functions may be useful for the diagnosis of congenital and acquired neutrophil disorders. These assays may also be useful in research, diagnosis and follow up of non-infectious diseases with active inflammatory component.

Bacterial Infections↗
PubMed1995

Hydroxyurea-induced fever: case report and review of the literature.

OBJECTIVE: To describe a patient with hydroxyurea-induced fever. CASE SUMMARY: A patient with essential thrombocythemia developed a fever 12 hours after beginning hydroxyurea therapy. The diagnosis of drug fever was established by resolution of the fever after withdrawal of hydroxyurea and its recurrence on reexposure to the drug. DISCUSSION: Hydroxyurea-induced fever is rare. In the present case, a short lag period between commencement of hydroxyurea therapy and appearance of fever may suggest either direct toxicity or an idiosyncratic reaction. In previous reports, a hypersensitivity drug reaction was implicated. CONCLUSIONS: The mechanism of hydroxyurea-induced fever remains unclear. Clinicians should be aware of this rare adverse effect.

Fever↗
PubMed1995

Identical HLA class II alleles predispose to drug-triggered and idiopathic pemphigus vulgaris.

In pemphigus vulgaris, a dermatological autoimmune disease, specific human leukocyte antigen (HLA) class II alleles, DR4 (DRB1*0402) and DRw14 (DRB1*1401, in linkage disequilibrium with DQB1*0503), are thought to be susceptibility genes involved in the onset of the disease. We studied the HLA class II alleles (DQA1, DQB1, DRB1 and DPB1) of 6 patients with pemphigus, in whom the disease was "triggered" by drugs containing sulphydryl or another sulphur-containing group. All patients carried the DRB1*0402 susceptibility allele, and one patient also carried the second susceptibility allele, namely DQB1*0503 (in linkage with DRB1*1401). Bacterial, viral or environmental agents are suspected to trigger the onset of autoimmune diseases. Our study demonstrated the presence in patients with drug-triggered pemphigus vulgaris of the same HLA alleles thought to predispose to idiopathic pemphigus vulgaris. This finding strengthens the notion that these HLA alleles may be true disease susceptibility genes.

Adult↗
PubMed1995

The effect of Padma-28, a traditional Tibetan herbal preparation, on human neutrophil function.

Human neutrophils were studied in vitro in the presence of the herbal preparation Padma-28. At concentrations higher than 0.3 mg/ml, the Padma-28 induced O2- production in unstimulated neutrophils. At lower concentrations, O2- production was inhibited in phorbol myristate acetate (PMA) stimulated cells. Lysozyme release by PMA and opsonized zymosan-stimulated cells was inhibited by Padma-28 at a concentration dependent manner. On the other hand, random and directed migration and adhesion to nylon fibers were not affected. These results suggest that Padma-28 may have anti-inflammatory activity whose mechanism remains to be elucidated.

Anti-Inflammatory Agents, Non-Steroidal↗
PubMed1994

Inhibition of neutrophil activation by fibrinogen.

Physiological levels of human fibrinogen markedly inhibited the chemotactic activity of human neutrophils triggered by zymosan-activated serum (ZAS), C5a, or IL-8 in a Boyden chamber assay. Fibrinogen also slightly inhibited the N-formyl-methionyl leucyl-phenylalanine (FMLP)-induced migration of human neutrophils. Albumin was devoid of the inhibitory activities displayed by fibrinogen in this system. The inhibition of chemotaxis by fibrinogen was dose-dependent and saturable. Fibrinogen placed in the upper compartment of the Boyden chamber produced a larger inhibition than that obtained with fibrinogen placed in the lower compartment. Lysine as well as the lysine analog 6-aminohexanoic acid (AHA) decreased the inhibitory capacity of fibrinogen. In contrast, both arginine and glutamine failed to suppress the fibrinogen-mediated inhibition of neutrophil chemotaxis. AHA counteracts the inhibition of ZAS-induced chemotaxis by anti-CD18 monoclonal antibody, suggesting that lysine binding sites are required for integrin function in chemotaxis. Fibrinogen also inhibited, in a dose-dependent manner, the oxygen consumption of neutrophils activated by opsonized zymosan. Taken together, the present results indicate that fibrinogen modulates neutrophil functions and suggest that in addition to its role in blood coagulation, circulating fibrinogen may be involved in regulation of the inflammatory response.

Amino Acids↗
PubMed1993

Therapy-related myelodysplastic syndrome. Two cytogenetically unrelated abnormal clones in a patient with multiple myeloma.

A multiple myeloma patient presented for cytogenetic analysis at diagnosis of secondary MDS, which followed cytotoxic treatment including melphalan. Two abnormal unrelated clones were detected, one of them had 5q-, 7q- with clonal evolution of an additional aberration, t(12;13); in the second clone there was a translocation between the two homologues of chromosome 1 as the only aberration. We suggest that the clone with 5q- and 7q- represented the secondary MDS cells, whereas the abnormal clone with t(1;1) represented the plasmablasts of the multiple myeloma.

Chromosome Deletion↗
PubMed1993

Inactivation of interleukin-8 by the C5a-inactivating protease from serosal fluid.

The complement fragment C5a and the cytokine interleukin-8 (IL-8) are proinflammatory peptides with potent chemotactic activity toward neutrophils. We have previously shown that C5a can be inactivated by a protease that is found in normal synovial and peritoneal fluids but is absent from serosal fluids obtained from patients with familial Mediterranean fever (FMF). We report here that serosal fluids can also eliminate the chemotactic activity of IL-8. The agent responsible for IL-8 elimination appears to be the C5a-inactivating protease, because the pure protease can inactivate IL-8, inactivation of IL-8 by normal peritoneal fluid is partly prevented by an antibody raised against the purified C5a-inactivating protease, and IL-8 is not inactivated by peritoneal fluids from patients with FMF. The ability of this protease to inactivate both, early (C5a) and late (IL-8) inflammatory mediators identifies it as a potentially significant regulator of inflammation.

Ascitic Fluid↗
PubMed1993

Correction of neutrophil chemotaxis defect in patients with Gaucher disease by low-dose enzyme replacement therapy.

We have recently described a chemotactic defect in severely afflicted Gaucher disease patients. Two of the patients were treated with low-dose intravenous enzyme replacement (Ceredase). Marked improvement in their hematological status, organomegaly, and growth was observed. In addition, their chemotactic defect and their tendency towards infections were corrected within 1 year of treatment.

Chemotaxis, Leukocyte↗
PubMed1993

Abnormal neutrophil chemotaxis in Gaucher disease.

The tendency towards infection described in Gaucher disease patients has been attributed to their post-splenectomy state. We noticed that certain patients with intact spleen have also suffered from recurrent pyogenic infections, thus an attempt to study their neutrophil function has been made. Nine of 29 patients studied expressed significant decrease in neutrophil chemotaxis directed towards zymosan activated serum or N-formyl-methionyl-leucyl-phenylalanine. Random migration was significantly impaired in five of those nine patients. Adherence of neutrophils to nylon fibres and O2- production were intact. The patients with impaired chemotaxis were significantly afflicted by their disease (early onset of symptoms and severity score index > 10) and most of them had genotypes associated with severe disease (1448/1448 and 1226/84GG). No correlation was found with the spleen status. Three of the patients with impaired chemotaxis, and none of the patients with normal neutrophil function, suffered from recurrent pyogenic infections. It is suggested that the described neutrophil migration impairment may contribute to the tendency towards infection in certain patients with advanced Gaucher disease.

Adolescent↗
PubMed1993

Impaired neutrophil chemotaxis in patients with thalassaemia major.

Random and directed migration, O2- production, degranulation and adhesion were studied in neutrophils obtained from patients with homozygous beta-thalassaemia and iron overload, in the presence or absence of thalassaemic serum. The only significant defect found was an impairment in directed chemotaxis, further depressed after addition of thalassaemic serum. The chemotactic defect was encountered in all the patients that have suffered from pyogenic infections except one, and was not correlated with the severity of the iron overload. It is suggested that the described neutrophil migration impairment may contribute to the tendency towards infection in certain patients with homozygous beta-thalassaemia.

Adolescent↗
PubMed1992

Subcellular localization of heparanase in human neutrophils.

The subcellular localization of a heparan sulfate degrading endoglycosidase, heparanase, was studied in human neutrophils. Unstimulated cells were disrupted by nitrogen cavitation and fractionated on a Percoll density gradient into three components, separating the plasma membranes, specific granules, and azurophilic granules. Heparanase activity was measured by gel filtration analysis of 35S-labeled degradation fragments released from subendothelial extracellular matrix (ECM) or produced during incubation with soluble, ECM-derived, heparan sulfate proteoglycans. Heparanase activity was found mainly in fractions containing the specific granules; this activity was inhibited by heparin. Freezing and thawing was not needed for recovery of the enzyme from the subcellular fraction, confirming previous data about its ready release. The mechanism of the ready release of heparanase from the specific granules requires further investigation.

Extracellular Matrix↗