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Biomedical subjects

W A Herrmann

Publications and source records attributed to W A Herrmann.

12 recordsLinked to original sources
PubMed2005

Elastosis perforans serpiginosa-like pseudoxanthoma elasticum in a child with severe Moya Moya disease.

A 2-year-old girl with Moya Moya disease who had relapsing cerebrovascular strokes presented with loose skin folds, 'chicken' skin appearance and perforating elastosis serpiginosa-like lesions in the genitocrural region. Histologically, calcified material perforating the epidermis and adjacent short curled and mineralized elastic fibres suggested a variant of pseudoxanthoma elasticum (PXE). As PXE is known to be caused by various mutations in the transmembrane transporter ABCC6 gene, we hypothesized that a novel ABCC6 mutation may underlie this unique combination of PXE and elastopathic vascular damage. Therefore, the complete ABCC6 coding region of the patient and her parents was screened for genetic alterations. No bona fide disease-causing mutation of ABCC6 could be found in the child and in her parents. However, two novel allelic amino acid substitutions (Arg1273Lys and Glu1293Lys; exon 27) were found in the girl and her father, localized in close proximity to the region that codes for the functionally critical second nucleotide-binding fold of ABCC6. Although a causal involvement of these amino acid substitutions could not be proven based on this study, both heterozygote substitutions may possibly have interacted with other undetected recessive maternal ABCC6 changes in the child. To the best of our knowledge, this is the first report of an association between early-onset PXE and severe Moya Moya syndrome possibly related to ABCC6 changes.

Amino Acid Substitution↗
PubMed2001

Mechanism of the Heck reaction using a phosphapalladacycle as the catalyst: classical versus palladium(IV) intermediates.

The mechanism of the (Mizoroki-)Heck vinylation of aryl bromides catalyzed by the phosphapalladacycle [[PdCH2C6H4P(o-Tol)2(OAc)]2] (1) was investigated in order to determine whether palladium(IV) intermediates play a significant role in the catalytic cycle. The phosphapalladacycle 1 was compared to the related palladium(0) catalyst [Pd[P(o-Tol)3]2] (2) and an intermediate of the classical catalytic cycle [[PdAr[P(o-Tol)3]Br]2] (3) obtained by oxidative addition of an aryl bromide to 2. Small, yet measurable deviations of the phosphapalladacycle 1 from the other catalysts 2 and 3 in the product distribution of styrene, in competition experiments of styrene and n-butyl acrylate, in the isotope effect on styrene, and the Hammett correlation on aryl bromides led to the conclusion that a different catalytic cycle is operative. Nevertheless, due to only small differences in these competition experiments a modified classical catalytic cycle with a novel, cyclometallated, anionic palladium(0) species 6 is proposed rather than palladium(IV) intermediates. Species 6 accounts for the observed high activity and stability of palladacycle catalysts in the Heck reaction. Palladium(IV) intermediates can be ruled out by taking into account the experiments performed here and recently published by others.

Alkenes↗
PubMed1994

Terbinafine-associated hepatic injury.

We describe two cases of terbinafine-associated hepatic injury, in which a mixed cholestatic-hepatocellular type of hepatitis was present. In both cases extrahepatic cholestasis and viral hepatitis were excluded and involvement of other drugs was unlikely. In the first patient all abnormalities have disappeared, but in the second patient alkaline phosphatase, aminotransferase and gamma-glutamyl transferase levels have remained elevated (follow up 3 months after cessation of treatment with terbinafine). Most likely, the terbinafine-associated hepatic injury in these patients was caused by an idiosyncratic rather than a direct hepatotoxic reaction.

Alanine Transaminase↗
PubMed1992

[Loss of taste sensation in terbinafine administration].

Terbinafine is a new antifungal agent for oral treatment of dermatomycosis. Seven patients are reported who developed taste loss after treatment with terbinafine. In four of them, taste loss was complete while one patient was still able to taste sweet products, and a second and third patient no longer tasted salty and sweet products, respectively. In all patients, the onset of the adverse reaction occurred 4-8 weeks after starting treatment. As far as known, taste loss is a transient effect which disappears within 3-6 weeks after discontinuation of terbinafine.

Adult↗
PubMed1980

Allergic vasculitis. A histological and immunofluorescent study of lesional and non-lesional skin in relation to circulating immune complexes.

This study concerns 57 patients who fulfilled histological criteria for the diagnosis allergic vasculitis. For 37 of these patients, biopsy specimen were available from lesional and adjacent non-lesional skin. Histological signs of vasculitis were found at both sites, but in clinically normal skin the perivascular infiltrate was less dense and neutrophils and eosinophils were sparse or absent. Fibrin was found in only ten patients and occurred less frequently in non-lesional skin. Deposits of immune complexes and/or complement were detected by immunofluorescence in 49 of the 57 patients. Hardly any differences between lesional and non-lesional skin were found with immunofluorescent microscopy. Circulating immune complexes were detected in 45 of the 56 available sera. A relationship was found between the class of immunoglobulin in immune complexes in the vessel wall and in the circulation. Moreover, the class of immunoglobulin seemed to be related to the course, the extracutaneous involvement, and the presence of associated diseases. No explanation was found for the histological differences observed between lesional and non-lesional skin.

Antigen-Antibody Complex↗
PubMed1980

Circulating IgA-immune complexes in Henoch-Schönlein purpura. A longitudinal study of their relationship to disease activity and vascular deposition of IgA.

In Henoch-Schönlein purpura immune complexes in inflamed vessel walls characteristically contain immunoglobulin A(IgA). To determine whether IgA is also the predominant immunoglobulin in circulating immune complexes, we compared the results of three immune complex assays with specificities for different classes of immunoglobulins in a longitudinal study of 37 patients (30 children and seven adults) with Henoch-Schönlein purpura. Circulating IgA-containing immune complexes were detected by their reactivity with a low avidity anti-IgA antibody in 27 of the 37 patients. IgA was simultaneously present in cutaneous vessel walls in 95 percent of the patients with circulating IgA-containing immune complexes. High levels of IgA-containing immune complexes were found only during the initial phase of the disease. Immune complexes containing bound complement breakdown products were demonstrated by binding to conglutinin. IgA was found in these immune complexes in 17 patients, IgG in 17 and IgM in nine patients. There was no apparent relation with the class of immunoglobulin in the deposits. Conglutinin-binding immune complexes were present later in the course of the disease and after remission. C1q-binding immune complexes were only found in two patients. These findings suggest that immune complexes-containing IgA may initiate the vasculitis of Henoch-Schönlein purpura, whereas complement-reacted immune complexes containing immunoglobulins of the other classes appear in the circulation in a later phase.

Adolescent↗
PubMed1980

Combined treatment of psoriasis with a new aromatic retinoid (Tigason) in low dosage orally and triamcinolone acetonide cream topically: a double-blind trial.

In a multicentre double-blind trial the effect of three therapy regimens was studied for 6 weeks in ninety psoriasis patients: (1) aromatic retinoid (Ro 10-9359) orally (0.50-0.66 mg/kg body weight) and placebo cream topically; (2) aromatic retinoid (Ro 10-9359) (same dosage) with 0.1% triamcinolone acetonide and 5% salicylic acid in lanette wax cream; (3) placebo capsules with 0.1% triamcinolone acetonide and 5% salicylic acid in lanette wax cream. Regimen 1 had virtually no effect and regimen 2 gave better results than regimen 3 for almost all parameters, although statistical significance was reached for only some of them. The 6 week double-blind period was followed by an open study in which all patients were treated according to regimen 2. The clinical result could be maintained up to the end of the study (18 weeks), when more than 60% of the patients showed good to excellant (80-100%) improvement. Most of the side-effects of retinoid were mild and relatively rare. It is concluded that the combination of the aromatic retinoid (Ro 10-9359) given in low dosage orally with corticosteroids topically is as effective as therapy with the retinoid in high dosage alone, but with markedly less side-effects.

Administration, Oral↗
PubMed1980

Circulating and tissue-bound immune complexes in allergic vasculitis: relationship between immunoglobulin class and clinical features.

Sixty-two patients with allergic vasculitis and histological evidence of leukocytoclastic vasculitis were examined to determine whether clinical features, such as course and degree of systemic involvement, could be related to the class of the immunoglobulins in immune complexes (IC) in both the circulation and the vessel wall. Circulating IC were detected with the 125I-C1q-binding assay (C1q-BA), an anti-IgA inhibition binding assay (a-IgA-Inh BA) and the conglutinin-binding assay (Con-BA). Stabilized heat-aggregated IgG, IgA and IgM were used to determine the immunoglobulin class specificity of these assays. With the C1q-BA only aggregated IgG were detected, with the a-IgA-Inh Ba only aggregates containing IgA. With the Con-BA IgG, IgA or IgM in reactive aggregates were identified with class-specific antibodies. For patients with acute cutaneous vasculitis all assays were negative. The a-IgA-Inh BA was frequently positive in sera of patients with chronic cutaneous and acute systemic vasculitis; in the latter group conglutinin-binding IC of the IgG, IgA and IgM class were also detected. Levels in the C1q-BA were high for patients with chronic systemic vasculitis. Comparison of the results of the IC assays with the immunofluorescence studies of the cutaneous vessel walls of the same patients showed agreement between the results of the C1q-BA and deposition of IgG and the results of the a-IgA-Inh BA and IgA deposition. The class of immunoglobulin in conglutinin-binding IC did not correspond as well with the immunoglobulins in vessel walls. This study shows that certain clinical features of allergic vasculitis are related to the composition of the IC in the circulation and in the vessel wall.

Adolescent↗