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U Hillen

Publications and source records attributed to U Hillen.

At least 19 recordsLinked to original sources
PubMed2006

[Manifestation of Fabry disease in a heterozygous female patient. New perspectives using enzyme replacement therapy].

HISTORY AND ADMISSION FINDINGS: A 32-year-old woman with bilateral corneal opacities was at 9 years of age diagnosed to have reduced activity of the enzyme alpha-galactosidase A. She was admitted to our hospital because of skin lesions that had developed over the last 8 years. INVESTIGATIONS: The clinical features included angiokeratomas on the buttocks, hips, and periumbilical region, whorl-like corneal opacity (cornea verticillata), and mitral valve prolapse. Activity of alpha-galactosidase A was reduced to about a quarter of the normal value. Histological examination revealed lipid deposits within the endothelial cells of the skin. Molecular analysis of the alpha-galactosidase A gene revealed a point mutation at nucleotide-position 691 in exon 5 (p.Asp231Asn). TREATMENT AND COURSE: Enzyme replacement therapy with agalsidase alfa 0.2 mg/kg body-weight, infused over 40 min every other week, was initiated. So far no side effects due to the infusion therapy have been noted. The therapeutic success (reduction of lipid storage) cannot be assessed as yet. CONCLUSION: Fabry disease results from deficient activity of the enzyme alpha-galactosidase A. Affected (hemizygous) males often show the complete spectrum of symptoms and signs and have a deficient alpha-galactosidase A activity. Occasionally milder oligosymptomatic courses are observed, when residual enzyme activity is present. In contrast to previous belief, heterozygous females may be affected in the same manner as hemizygotes and may also have a significantly reduced enzyme activity.

Abdomen↗
PubMed2006

[Contact allergies in patients with chronic wounds: results of a study from 1999 to 2004].

Patients with chronic wounds frequently acquire clinically relevant contact sensitization. We examined the results of the skin patch tests in patients with chronic wounds, who were treated between 1999-2004 in the Department of Dermatology, University School of Medicine, Essen, Germany. Altogether 105 patients with chronic wounds underwent patch testing. In 68 individuals, contact sensitization to at least one substance was detected. The most frequent contact allergens were to balsam of Peru, Amerchol L-101, fragrance mix, wool wax alcohols and rosins. Contact sensitization to wound dressing materials was also identified in 4 patients. Our results demonstrate the current spectrum of contact sensitization of patients with chronic wounds. Our results show the importance of paying attention to the ingredients in wound dressings and performing patch testing if there is any clinical suggestion of allergic contact dermatitis.

Adult↗
PubMed2006

Late reactions to the patch-test preparations para-phenylenediamine and epoxy resin: a prospective multicentre investigation of the German Contact Dermatitis Research Group.

BACKGROUND: Late patch-test reactions, developing at day (D) 7 or later have been described for several allergens. Late reactions may reflect patch-test sensitization. Para-phenylenediamine (PPD) and epoxy resins (ER) are potent allergens and therefore may potentially induce patch-test sensitization. Up to now, there has been no prospective study on the frequency of late reactions in routine patch testing with these allergens. OBJECTIVES: To assess the frequency of late reactions to PPD and ER. PATIENTS/METHODS: In 1748 patients PPD (PPD-base, 1% pet.) and ER [based on diglycidylether of bisphenol A (DGEBA, 1% pet.)], and in 812 patients, nickel sulphate (5% pet.) were removed from the test panel of the standard series and applied on the medial side of the upper arm. Patch-test occlusion time was 24 h in 588 (PPD and ER) and 241 patients (nickel sulphate), respectively, and 48 h in 1160 (PPD and ER) and 571 (nickel sulphate) patients, respectively. Patch tests were read on D1-3 and D2-3, respectively; additional late readings were performed on D7, D14 and D21 after patch-test application. Patients who were not able to return for all scheduled late readings were telephoned on D7, D14 or D21, and questioned about a reaction at the test sites. Patients were instructed to perform daily self-examination from D4 onwards and to return immediately to the clinic if a reaction at the upper arm became visible. RESULTS: Data of 1428 patients (ER and PPD) and 638 patients (nickel) were evaluable. In 25 patients (1.8%), patch tests became positive not before D7, among them 21 reactions to PPD (1.5%) and four reactions to ER (0.3%). In five of seven patients, repeated patch tests with PPD disclosed patch-test sensitization as the cause of the late reaction. All late reactions, except for one, occurred in patients in whom patch tests were applied for 48 h. No late reactions were seen with nickel sulphate. CONCLUSIONS: PPD (1% pet.) elicited late reactions in 1.5% of routine patch tests, the majority of them probably being caused by patch-test sensitization. Therefore, the German Contact Dermatitis Research Group decided to remove PPD 1% pet. from the German standard series and to take efforts to optimize the patch-test conditions of PPD. One way to optimize PPD testing could be to reduce the exposure of PPD 1% to 24 h. Alternatively the patch-test concentration of PPD might be reduced.

Adolescent↗
PubMed2005

Quantitative patch and repeated open application testing in methyldibromo glutaronitrile-sensitive patients.

Contact allergy to methyldibromo glutaronitrile (MDBGN), often combined with phenoxyethanol (PE) (e.g., Euxyl K 400), increased throughout the 1990s in Europe. Consequently, in 2003, the European Commission banned its use in leave-on products, where its use concentration was considered too high and the non-sensitizing use concentration as yet unknown. The 2 objectives of the study are (a) to find a maximum non-eliciting concentration in a leave-on product in MDBGN/PE-sensitized patients, which could possibly also be considered safe regarding induction and (b) to find the best patch test concentration for MDBGN. We, therefore, performed a use-related test (ROAT) in patients sensitized to MDBGN/PE (n = 39) with 3 concentrations of MDBGN/PE (50, 100 and 250 p.p.m. MDBGN, respectively). A subset of these patients (n = 24) was later patch-tested with various concentrations (0.1, 0.2, 0.3 and 0.5% MDBGN, respectively). 15 patients (38%, 95% confidence interval (CI) = 23-55%) had a negative and 24 (62%; 95% CI = 45-77%) a positive overall repeated open application test (ROAT) result. 13 reacted to the lowest (50 p.p.m.), 8 to the middle (100 p.p.m.) and 3 to the highest concentration (250 p.p.m.) only. In those 13 reacting to the lowest ROAT concentration, dermatitis developed within a few days (1-7). The strength of the initial and the confirmatory patch test result, respectively, and the outcome of the ROAT were positively associated. Of the 24 patients with a use and confirmatory patch test, 15 reacted to 0.1% MDBGN, 16 to 0.2%, 17 to 0.3% and 22 to 0.5%. With the patch test concentration of 0.5%, the number of ROAT-negative patients but patch-test-positive patients increases considerably, particularly due to + reactions. A maximum sensitivity of 94% (95% CI = 70-100%) is reached with a patch test concentration of 0.2%, and is not further improved by increasing the concentration. However, the specificity decreases dramatically from 88 (95% CI = 47-100%) with 0.2% to a mere 12.5% (95% CI = 0-53%) with 0.5%. It can be concluded (a) that for MDBGN 0.2% is very likely the best patch test concentration and (b) that 50 p.p.m. in a leave-on product can elicit contact dermatitis in sensitized persons. We were, therefore, unable to find a safe, still microbicidal, concentration for leave-on products. By contrast, with other contact allergens, dose-response use tests may be able to identify a non-eliciting concentration, which could give valuable clues to a non-inducing (i.e., safe) concentration in products.

Adult↗
PubMed2004

The Van der Woude syndrome: a case report and review of the literature.

The Van der Woude syndrome is a rare autosomal dominant developmental malformation usually associated with bilateral lower lip pits. These congenital lip pits appear clinically as a malformation in the vermilion border of the lip, with or without excretion. As a genetic defect has been identified as a microdeletion of chromosome bands 1q32-q41, genetic counselling of patients may be considered. A nonsense mutation in the interferon regulatory factor-6 (IRF-6) is discussed as a pathogenic relevant factor. Therapeutic intervention is generally not necessary, although surgical excision is especially indicated in patients with recurrent inflammation. Physicians should be aware of the Van der Woude syndrome because it has been reported to be associated with a variety of malformations or other congenital disorders.

Adult↗
PubMed2003

[Sensitization to tea tree oil in Germany and Austria. A multicenter study of the German Contact Dermatitis Group].

BACKGROUND AND AIM: Tea tree oil, a distillation product of the Australian tea tree (Melalence alternitolia) is increasingly used as an alternative remedy for various dermatological diseases. Tea tree oil contains several allergenic monoterpenes and sesquiterpenes. In this multicenter study it was evaluated, whether the increasing use of tea tree oil has lead to an increased frequency of sensitization in Germany and Austria which would justify its inclusion into the standard series. PATIENTS AND METHOD: For patch testing a standardized tea tree oil was used, dissolved 5% in diethylphtalate (DEP). Consecutive patients of 11 dermatological departments in Germany and Austria were tested. Readings were taken on day 2 and 3 according to the guidelines of the German Contact Dermatitis Research Group (DKG). RESULTS: 5% tea tree oil was positive in 36/3375 patients (1.1%). Sensitization frequencies showed great regional variations and ranged from 2.3% (Dortmund), 1.7% (Buxtehude), 1.1% (Essen), 0.7% (Graz), to 0% (Berlin, Vienna). 14/36 patients (38.9%) also showed a positive patch test reaction to oil of turpentine. CONCLUSION: Our results show that tea tree oil is an important contact allergen for some centers. It should be tested, if medical history suggests its previous use. Considering the great regional differences in frequencies of sensitization its inclusion into the standard series is not recommended yet.

Adult↗
PubMed2002

Evaluation of metal allergies in patients with coronary stents.

In-stent restenosis is a complication which impairs the success of coronary stenting. Recently, it was supposed that a delayed hypersensitivity reaction to nickel and molybdenum might be one of the triggering factors in in-stent restenosis. We have analyzed the data collected in our centre with respect to this hypothesis. Altogether, 34 patients were investigated (24 male, 10 female). Patch tests were performed with the standard series of the German Contact Dermatitis Research Group and a metal series containing the metal components of 316 L stainless steel. A positive patch test reaction to nickel was observed in 4 (11.8%) patients. None of the patients showed sensitization to the other metals. Retrospective analysis was performed in 20 patients: 2 of these patients had a positive patch test reaction to nickel, one of whom had an in-stent restenosis, and the other not. Restenoses were predominantly observed in patients with negative patch test reactions to nickel (6/18 patients). Out of the patients who were investigated prospectively only one showed sensitization to nickel. Restenosis was observed in 2 patients: neither patient had nickel allergy. Although it still cannot be excluded that metal allergy may play a role in the restenosis process in coronary stenting, there is at present little evidence for it.

Coronary Angiography↗
PubMed2001

Patch test sensitization caused by para-tertiary-butylcatechol. Results of a prospective study with a dilution series.

Para-tertiary-butylcatechol (PTBC) has been patch tested in Europe at 1% in petrolatum (pet.) and is now suspected of induction of patch test sensitization. A prospective study was initiated to obtain detailed data on this undesirable risk. A dilution series of PTBC (1%, 0.5%, 0.25%, 0.1% pet.) was used. Patch tests were read on days (D) 1-3, 7, 14 and 21 after application. Patients who were unable to return for late readings were telephoned and asked to report any reaction at the patch test sites. 40 out of 46 patients included completed the study. Patch tests were negative in 35 patients. 4 patients showed a positive patch test at later readings only (D7-D21); 2 patients reacted to a concentration as low as 0.1%. Rechallenge was performed in 2 of these patients, revealing a clearly positive reaction as early as D2 after patch test application. PTBC clearly induced patch test sensitization in 10% of the patients. It cannot be excluded that patch testing with 0.25% PTBC or with even lower concentrations might induce patch test sensitization. The optimal patch test concentration still has to be determined but may be within the range of 0.01% to 0.25% PTBC.

Adult↗
PubMed2000

[Contact allergies in patients with eczema of the external ear canal. Results of the Information Network of Dermatological Clinics and the German Contact Allergy Group].

BACKGROUND AND OBJECTIVE: The Information Network of Departments of Dermatology (IVDK) centrally collects patch tests results in cooperation with the German Contact Dermatitis Research Group (DKG). Data of patients, who underwent patch testing to evaluate otitis externa were analyzed. PATIENTS/METHODS: Data of 145 patients that had been recorded between 1992 and 1997 were evaluated. The evaluation included data yielded by standardized patch testing as well as by patch testing of products that the patients had used. RESULTS: Allergic contact dermatitis was diagnosed in one third of patients suffering from otitis externa. Topical drugs were shown to be the most important allergens. In comparison to the entire IVDK collective, the population-adjusted frequency of sensitization (PAFS) of neomycin sulfate was higher in the study population whereas that of nickel sulfate was lower. That shows that in a very specific topographic area, different allergens may be of clinical significance. CONCLUSIONS: Whereas nickel sulfate represents an important allergen in the contact dermatitis of the external ear, topical therapeutical agents, especially neomycin sulfate and, as evaluation of the patch tests of the patient's own products revealed, probably polymyxin B are the dominating allergens in otitis externa.

Adult↗