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M A Muniain

Publications and source records attributed to M A Muniain.

33 records · Page 2Linked to original sources
PubMed1993

Late onset Papillon-Lefèvre syndrome? A chromosomic, neutrophil function and microbiological study.

We had the opportunity to study a family with one of the most destructive forms of periodontal disease known, the Papillon-Lefèvre syndrome. The parents had no consanguinity and were not affected, and were therefore to be considered carriers of the disease. 2 sisters, the eldest and youngest, showed periodontal breakdown and hyperkeratotic skin lesions, but their deciduous dentition was not affected. 2 brothers had skin lesions only and another brother and sister were healthy. Furthermore, 2 babies died at birth one after a 9-month pregnancy and the other after a 6-month pregnancy, and the mother also suffered 3 miscarriages. For 4 years, we studied the family: in the case of both sisters, mechanical periodontal treatment and antibiotics were unable to control the disease. In the chromosomic study of the 2 sisters affected, the GTG banding technique found no trace of anomalies in the cells analyzed, whose chromosomic formation was 46,XX. Before treatment, the chemotaxis of the PMN, the phagocytosis of opsonized Staphylococcus aureus, and production of superoxide radicals by PMN was significantly impaired in both sisters. Despite scaling and root planing, the periodontal lesions still progressed, but the PMN functions evaluated were now normal in both sisters. An orally asymptomatic but dermatologically affected brother showed no significant defect in the phagocytic activity and the production of superoxide radicals.

Adult↗
PubMed1992

Primary idiopathic osteolysis: description of a family.

A clinical, analytical, and radiological study was carried out on three members of the same family with multicentric idiopathic osteolysis. Transmission appeared to be via the dominant autosome present in the mother and two daughters. In the daughters osteolysis was seen in the carpal and tarsal bones, whereas in the mother radiology showed it to be in the phalanges of the hands and feet.

Adolescent↗
PubMed1991

Circadian variations in the superoxide production, enzyme release and neutrophil aggregation in patients with rheumatoid arthritis and controls.

Patients with rheumatoid arthritis (RA) experience fluctuations of symptoms throughout the day. These could be due to mechanical changes or modification of the inflammatory mechanisms. In the present paper we studied fluctuations of superoxide production (O2), enzyme release (beta-glucuronidase and lysozyme) and neutrophil aggregation in the peripheral blood of eight healthy volunteers and eight patients with RA. Although enzyme release was greater in patients with RA, no significant difference was found throughout the day, neither in control nor in patients. Similar results were obtained when studying neutrophil aggregation. On the contrary, the superoxide production, determined at 8:00, 14:00, and 20:00 h, was 6.3 +/- 0.5, 4.40 +/- 0.4 and 6.26 +/- 0.3, respectively, in patients with RA and 6.65 +/- 0.7, 4.7 +/- 0.7 and 7.27 +/- 0.4 in the controls. The values obtained at 14:00 h were significantly lower (alpha = 0.01). There were no differences in the curve form between patients and controls.

Arthritis, Rheumatoid↗
PubMed1989

[Circadian variations in the production of superoxide and liberation of lysosomal enzymes of neutrophils in peripheral blood].

Superoxide formation and lysosomal enzyme liberation circulating neutrophils has been assessed in healthy subjects. Blood samples were obtained at 8:00, 14:00, and 20:00 o'clock. The formation of superoxide followed a circadian rythm. Lowest concentration was found a 14:00 hours (4.59 +/- 2.09 nM reduced cytochrome/10(6) neutrophils). No 24 hour variation of betaglucuronidase and lysozyme liberation was proven. The number of neutrophil receptors for formyl-methyonil-leucyl-phenyl-alamine (FMLP) at the same hours did not show any circadian variation. The circadian changes of the functional capacity of the neutrophils might be one of the mechanisms that could justify the 24 hour variation of symptomatology characteristic of some diseases.

Adult↗
PubMed1988

[Effect of corticosteroids, quinacrine and L-652, 731 on experimental aggregation of polymorphonuclear leukocytes induced by complement and platelet activating factor (PAF)].

The effects of 6-methylprednisolone sodium succinate, quinacrine and the synthetic anti-PAF compound L-652,731 were studied on the PAF and complement induced aggregation of rabbit polymorphonuclear leukocytes in vivo. High doses (100-250 mg/kg) of corticosteroid were able to abrogate PAF and complement induced aggregation. Quinacrine (1.25 mg/kg), and L-652,731, partially precluded complement-depending aggregation. The L-652,731 dose employed was just enough to prevent PAF induced aggregation. These results suggest that in those pathological conditions in which polymorphonuclear aggregation occurs, factors other than those derived from plasmatic complement system activation, and laboring jointly with it, may be involved.

Agranulocytosis↗
PubMed1986

Wegener's granulomatosis in two sisters.

We describe two sisters with necrotising granulomas. The intervals between the onset of symptoms and diagnosis were eight and nine years. The pathological findings showed necrotising granulomas in both patients, this being consistent with a diagnosis of pathergic (Wegener's) granulomatosis. The one patient that accepted treatment responded well to cyclophosphamide, though the facial lesions responded only to radiation therapy. Since the second was almost asymptomatic we conclude that relatives of patients with Wegener's disease should be investigated.

Adult↗
PubMed1985

[Effects of pepstatin A on neutrophils; cross-deactivation with FMLP].

The ability of pepstatin A, a protease inhibitor produced by Streptomyces testaceus, to elicit a number of responses by the human PMN has been studied. In lysozyme and beta-glucuronidase release, pepstatin A 10(-5)M is equivalent to the synthetic oligopeptide N-formyl-methionyl-leucyl-phenylalanine (FMLP) 10(-7)M. In superoxide release, pepstatin A 10(-5)M produces 80% of that originated by FMLP 10(-7). After two minutes of incubation the superoxide release is important, there being no further increase after 10 minutes. Preincubation of the cells with cytochalasin B before stimulation with pepstatin A elicits a noticeable increase in O2- release. In chemotaxis, pepstatin A 10(-6) originates the same cell motility as FMLP 10(-9). Pepstatin A produces a cross deactivation with FMLP which adds further evidence to the hypothesis that both stimuli compete for the same receptor in the PMN.

Binding, Competitive↗
PubMed1984

Receptor blockade as a mechanism of deactivation of human neutrophils by pepstatin and formyl-Met-Leu-Phe.

The pentapeptide pepstatin was shown to be chemotactic for human neutrophils by two techniques: ED50 for chemotaxis was found to be 3 microM by the agarose method and 0.2 microM by the Boyden chamber technique. Pepstatin also induced superoxide radical generation, release of lysosomal enzymes, and a transient increase in intercellular adenosine-3',5'-cyclic monophosphate (cAMP) levels in a dose-dependent manner. Carbobenzoxy-phenylalanyl-methionine (CBZ-PM), which competitively inhibits formyl-methionyl-leucyl-phenylalanine (FMLP) -induced neutrophil functions, also inhibited pepstatin-induced neutrophil function of superoxide generation in a dose-dependent fashion. Likewise, pepstatin inhibited the binding of [3H]FMLP to the cells. Furthermore, preincubation of neutrophils with suboptimal concentrations of FMLP or pepstatin diminished the cellular response toward either factor when tested for their chemotactic activity and for their ability to induce superoxide generation, to release granule enzymes, and to induce a transient increase in intracellular cAMP levels. The concentrations of pepstatin or FMLP tested had no effect on superoxide generation, granule enzyme release, or intracellular levels of cAMP on subsequent challenge with C5a; both of these factors, however, cross-deactivated the chemotactic response of the cells towards C5a. Similar results were observed when cells were preincubated with C5a and subsequently challenged with pepstatin or FMLP. These results suggest that FMLP and pepstatin interact with the same receptor molecules to activate human neutrophil functions. Furthermore, our data indicate that the deactivation of the neutrophil functions of superoxide production and granule enzyme release are receptor specific, but the heterologous deactivation of chemotaxis involves a postreceptor mechanism(s).

Binding, Competitive↗
PubMed1983

Alcohol-induced colitis.

Acute colitis following the unintentional administration of an alcohol enema in a 38-year-old woman is reported. To our knowledge this is the first well-documented case of this iatrogenic condition in an adult.

Adult↗