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Biomedical subjects

M Simon

Publications and source records attributed to M Simon.

At least 505 records · Page 28Linked to original sources

Thymopentin treatment in a patient with pluriorificial pyoderma vegetans.

This paper presents longitudinal clinical and immunological findings in a patient with a life-long history of pluriorificial pyoderma vegetans, a disease characterized by a distinct immunodeficiency of T lymphocytes. After treatment with thymopentin, 50 mg s.c. three times weekly for 12 weeks, the number of T lymphocytes in peripheral blood normalized, while other impaired immunological functions failed to improve, however. The most impressive observation was the significant clinical improvement of the patient's condition.

Adjuvants, Immunologic↗

Enzymatic cross-linking of involucrin and other proteins by keratinocyte particulates in vitro.

A transglutaminase-catalyzed cross-linking process characteristic of keratinocytes leads to the formation of the insoluble corneocyte envelope. The essentials of this process take place in vitro in a reconstituted system derived from subcellular fractions. A particulate fraction containing membrane-bound envelope precursor proteins and the enzyme transglutaminase is combined with cytosolic proteins; when the enzyme is activated by Ca++, cytosolic proteins are removed from solution and cross-linked to particulate proteins. This interaction is cell-type-specific, since particulates derived from fibroblasts and also containing transglutaminase activity cannot substitute for those of keratinocytes. Involucrin, a cytosolic protein known to be a precursor of the envelope, is more efficiently cross-linked than other cytosolic proteins. The cross-linking of proteins of the particulate fraction (membrane proteins) is promoted by the presence of involucrin.

Acyltransferases↗

The engrailed locus of Drosophila: in situ localization of transcripts reveals compartment-specific expression.

The engrailed locus plays a unique and critical role in organizing the segmented body plan of Drosophila. Embryos lacking engrailed function die with fused, abnormal segments. Adult mosaics with patches of engrailed cells similarly suffer defects in all of their segments, but only with mutant cells that are in the posterior developmental compartment of each segment. The non-uniform requirement for engrailed function reflects the position-dependent expression of the engrailed locus and we demonstrate it here unambiguously by directly visualizing engrailed transcripts in frozen sections of embryos and larvae and in whole imaginal discs. These results demonstrate that developmental compartments subdivide the embryonic insect segments. In these and in the compartments of the later developmental stages the engrailed locus is expressed in the posterior but not the anterior compartments. With its role in controlling the developmental pathway of the posterior compartment cells, the engrailed locus may be an example of a binary developmental switch.

Animals↗

Is the HLA-linked haemochromatosis allele implicated in idiopathic refractory sideroblastic anaemia?

In order to assess the previously reported association of HLA-linked idiopathic haemochromatosis with idiopathic refractory sideroblastic anaemia (IRSA), the prevalence of HLA-A3 antigen in a group of 22 patients with IRSA was compared to that observed in healthy controls and in patients with homozygous idiopathic haemochromatosis and to that calculated for a population heterozygous for idiopathic haemochromatosis. The prevalence of A3 in patients with IRSA (0.23) was quite similar to that observed in controls (0.29) and significantly different from that observed in homozygous (0.73; P less than 10(-5] and heterozygous (0.57; P less than 10(-3] haemochromatosis. Serum iron, transferrin saturation, serum ferritin and liver iron concentration showed no difference in IRSA patients with or without A3. It is concluded that there is neither systematic association between the haemochromatosis allele and IRSA nor systematic implication of such an allele in the development of iron overload observed in IRSA.

Aged↗

Specific uptake rates of amino acids by attached and free-living bacteria in a mesotrophic lake.

Seasonal and spatial patterns of specific uptake rates of amino acids by bacteria in Lake Constance were studied. The total bacterial population was divided into small (0.2- to 1.0-micron) and large (1.0- to 3.0-micron) free-living bacteria and attached bacteria by fractionated filtration. Data for attached bacteria, received by retention on 3.0-micron-pore Nuclepore filters, were corrected for free-living bacteria in this fraction. Specific uptake rates based on autoradiography were also recorded. Specific uptake rates for attached bacteria ranged from 9.41 X 10(-11) to 6.11 X 10(-8) ng of C h-1 cell-1 and were therefore significantly greater than those for free-living bacteria during most time periods. However, they were not significantly different from those for cells proven to be active by autoradiography. Specific uptake rates for small free-living bacteria ranged between 7.68 X 10(-11) and 4.60 X 10(-9) ng of C h-1 cell-1. They were nearly in the same range of those for large free-living bacteria (5.10 X 10(-11) to 1.07 X 10(-8) ng of C h-1 cell-1), although both fractions exhibited pronounced differences in their seasonal and vertical distributions.

Amino Acids↗

Disseminated cicatricial pemphigoid.

A patient is presented with generalized blistering and scarring eruptions, showing immunopathological, histological and electron microscopical characteristics of disseminated cicatricial pemphigoid, successfully treated by combined therapy of systemic steroids and azathioprine.

Azathioprine↗

[Cytosine-arabinoside in high doses in refractory acute granulocytic leukemia. Apropos of 17 cases].

A total of 17 patients, 6 female and 11 male (age range 13 to 56 years), received high dose Ara-C for treatment of refractory acute myelogenous leukemia. Ara-C was given at 3 g/m2 twice daily for 6 days as a 1 infusion. 1 patient (with induced acute leukemia) was treated directly, two after failure of a chemotherapy schedule containing the usual dose Ara-C, 12 for first relapse and 2 for subsequent relapse. Maximum follow up is 16 months. Beside hematological toxicity, systemic tolerance was good with no neurological of cutaneous effects. Despite preventive corticoid eyewash, ocular complications occurred in 6 cases, mild and resolvable in 5 of them. The immediate results were as follows: 3 deaths during induction (18%); 6 failures (35%); 8 complete remissions (CR) (47%). After primary chemo-resistance (two cases) failure was always noted. In 3 cases, after less than 12 infusions had been given, 2 failures and 1 very short CR were noted. In 2 patients, when doxorubicin was added to Ara-C, we observed 1 death during induction and 1 failure. Of the patients achieving CR 8 were treated by periodic courses with high dose Ara-C and 4 of them relapsed. The longest failure free duration was 11 months. Median survival duration of the 17 patients is 5 months.

Adolescent↗

[Coincidence of light urticaria and lichen ruber planus].

A 49-year-old patient with concomitant solar urticaria (SU) and lichen planus (LP) is presented. The sunlight- and UV-A-provoked wheal and flare SU reactions were interpreted as an antigen-antibody reaction, probably involving a reaginic antibody, because SU was passively transferred by a serum factor utilizing the Prausnitz-Küstner reaction. The reduced percentage of suppressor cytotoxic T-cell subset in peripheral blood typical of active LP may facilitate the increased production of reaginic antibodies after presentation of antigen by sunlight. Not only was the usual immunological reaction of LP (similar to graft-versus-host reaction) suppressed by PUVA treatment, but increased tolerance to sunlight was also achieved.

Biopsy↗

[Autologous replantation of spleen particles--an established procedure? An opinion].

Autologous splenic replantation after splenectomy for trauma is generally recommended in order to prevent postsplenectomy sepsis. Several aspects, however, make this method uncertain: The extent of splenosis induced is not to be predicted. Even if the whole spleen is replanted, the resulting mass may not exceed splenosis, which sometimes develops spontaneously after sole splenectomy. But patients with extended splenic regenerates do not differ from those without any splenosis, considering various immunologic parameters. Postoperative complications after splenic replantation (abscess, intestinal occlusion) are possible, although they may be rare. Fatal cases of postsplenectomy sepsis despite massive splenosis are known. The most important reasons apparently are the altered vascularity and scarring in splenic regenerates. There is no specific test for immunological splenic function. Therefore there is no test to judge the success of replantation, too. Overall, the preservation of the spleen and the vaccination of splenectomized individuals are the most important surgical intentions.

Bacterial Infections↗

Genetic analysis of idiopathic hemochromatosis using both qualitative (disease status) and quantitative (serum iron) information.

An ongoing family study of idiopathic hemochromatosis in Brittany, France, allowed us to investigate the segregation of this trait and its linkage and association to the HLA-A locus in 147 pedigrees, comprising 1,408 individuals with over 900 characterized for relevant biological parameters and typed for HLA. The joint consideration of affection status and serum iron concentration reveals no dominance effect on the latter trait and documents the increased information afforded by the consideration of a biological correlate of liability to affection for disease exhibiting incomplete penetrance. Our overall results are in general agreement with published results on a Utah family study.

Female↗