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H Traupe

Publications and source records attributed to H Traupe.

At least 55 records · Page 3Linked to original sources
PubMed1999

Total antioxidative capacity is normal in sera from psoriasis patients despite elevated bilirubin, tocopherol and urate levels.

BACKGROUND: Psoriasis is a chronic inflammatory skin disease. Reactive oxygen species are thought to play a critical role in inflammation and are scavenged by antioxidants. OBJECTIVE: We wanted to know whether the total antioxidative capacity or single antioxidative components in sera from psoriasis patients are decreased. MATERIAL AND METHODS: Well-known antioxidants of the serum were measured using commercial kits or determined by HPLC in the sera of 33 inpatients with severe psoriasis and in 36 healthy control persons. The total antioxidative capacity was measured using the ability of endogenous antioxidants to scavenge the 2, 2'-azinobis(3-ethylbenzothiazoline-6-sulfonic acid) radical cation. RESULTS: Several single antioxidative components were slightly elevated when considered groupwise, namely tocopherol (p = 0.001), bilirubin (p = 0.001) and urate which was increased over controls by about 33% (p= 0.01). Other parameters such as total protein and thiol content and ascorbic acid did not differ from controls. CONCLUSION: To our surprise the total antioxidative activity was normal in sera from psoriasis patients although some components such as urate were even elevated.

Antioxidants↗
PubMed1998

Ichthyosis follicularis, alopecia, and photophobia (IFAP) syndrome: clinical and neuropathological observations in a 33-year-old man.

The syndrome of ichthyosis follicularis, alopecia, and photophobia (IFAP) is an uncommon neuroichthyosis described in only 10 males so far. We report on a man with congenital ichthyosis and alopecia with apparently normal development in early infancy. Photophobia and generalized myoclonicastatic seizures began during or after the first year of age and were associated with progressive impairment of motor skills and mental abilities. He died at 33 years of age. Neuropathological findings showed an unusual deformation of the temporal lobes and olivocerebellar atrophy. Cytogenetic and molecular studies did not uncover deletions in either Xp22.2 to 3 or in Xq27.3 to qter.

Abnormalities, Multiple↗
PubMed1998

A novel in situ method for the detection of deficient transglutaminase activity in the skin.

Autosomal recessive congenital ichthyoses are disorders of epidermal cornification, but are clinically and etiologically heterogeneous. Some cases, known as lamellar ichthyosis, are caused by mutations in the TGM1 gene encoding transglutaminase 1, which result in markedly diminished or lost enzyme activity and/or protein. In some cases, this enzyme is present but there is little detectable activity, and in other clinically similar cases, transglutaminase 1 levels appear to be normal. Since conventional enzyme assays and mutational analyses are tedious, we developed a novel assay for the rapid screening of transglutaminase 1 activity using covalent incorporation of biotinylated substrate peptides into skin cryostat sections. Coupled with immunohistochemical assays using transglutaminase 1 antibodies, our method allows rapid identification of those cases caused by alterations in this enzyme.

Chromosome Mapping↗
PubMed1998

Genetic and immunohistochemical detection of mutations inactivating the keratinocyte transglutaminase in patients with lamellar ichthyosis.

Autosomal recessive lamellar ichthyosis is a clinically heterogeneous group of severe congenital keratinization disorders that is characterized by generalized hyperkeratosis and variable erythema. About half of the patients have mutations in the TGM1 gene, which encodes the keratinocyte transglutaminase. Linkage studies have shown that at least two further loci for autosomal recessive lamellar ichthyosis must exist. We present here two patients with lamellar ichthyosis caused by mutations in the TGM1 gene. The first patient is compound heterozygous for the novel missense mutation C53S and the splice mutation A3447G. The second patient, a child of consanguineous parents from Tunisia, is homozygous for the unknown nonsense mutation W263X. This is the first report of a mutation, C53S, that affects the region of the keratinocyte transglutaminase that is essential for anchorage of the enzyme to the plasma membrane. A novel, rapid in situ transglutaminase activity assay revealed the absence of keratinocyte transglutaminase activity in both patients. The mutations described are hence causative for the ichthyosis phenotype.

Adolescent↗
PubMed1998

A mutational hotspot in the 2B domain of human hair basic keratin 6 (hHb6) in monilethrix patients.

Monilethrix is an inherited hair dystrophy in which affected, fragile, hairs have an unique beaded morphology. Ultrastructural studies suggest a defect in filament structure in the cortex of the hair, and the hard keratins of hair and nail are thus candidate genes. In several families with autosomal dominant monilethrix, the disorder has been linked to the type II keratin gene cluster at chromosome 12q13. Recently, causative mutations in the critical helix termination motif in the 2B domain of the human hair basic keratin 6 (hHb6) have been identified. We now report the results of sequencing this domain in 13 unrelated families or cases with monilethrix. Five of the 13 had the same mutation as previously found, a G to A transversion leading to a lysine for glutamic acid substitution (E413K) in the 2B domain (residue 117 of the 2B helix) of hHb6. The mutation was confirmed by a restriction fragment length polymorphism assay developed for this purpose, and, as this mutation is evidently a common cause of the syndrome, for use in screening other cases. In eight families or cases, however, including three in whom linkage data are consistent with a defect at the type II keratin locus, no mutation was found in this domain of hHb6.

Family Health↗
PubMed1998

[Magnetic resonance imaging of of the right extraocular muscle paths in healthy persons and in patients with high myopia].

PURPOSE: To investigate the paths of the rectus extraocular muscles (EOMs) in patients with high axial myopia, using high-resolution magnetic resonance imaging (MRI). METHODS: Coronal MR images (T1 weighting) of the orbit were obtained with controlled gaze. Positions of recti EOMs were measured digitally in the middle of the orbit, using the NIH-image analysis software. Orbits of three different patient groups were analysed. Group 1 (n = 14 orbits): Patients with high axial myopia and restrictive eye motility. Group 2 (n = 8 orbits) subjects with high axial myopia and normal eye motility. Controls (n = 11 orbits) with normal eye motility and no refractive error. RESULTS: In comparison to the controls, patients with high axial myopia were found to have significant misplacement of the recti EOMs. Thus in group 1 (group 2 within brackets) the lateral rectus muscle (LR) was misplaced 2.9 (1.4) mm into the lower temporal quadrant p < 0.001 (p = 0.07). The course of the superior rectus muscle (SR) was shifted 1.5 (1.5) mm medially p = 0.02 (p = 0.03) and the path of the inferior rectus muscle (IR) 1.3 (1.3) mm medially p = 0.06 (p = 0.06). The medial rectus muscle (MR) showed a 1.3 (1.2) mm downward mislocation p = 0.01 (p = 0.07). CONCLUSIONS: In patients with high axial myopia (group 1 and group 2) misplacement of all rectus EOMs could be demonstrated by high resolution MRI with controlled gaze. All patients showed an approximately equal amount of MR, SR and IR mislocation. However, misplacement of the LR was significantly greater in patients with high myopia and restrictive eye motility (group 1) than in those without restrictive ocular motility (group 2), p = 0.03. We therefore assume that LR downward mislocation is a major determinant for restrictive eye motility in high myopia.

Adult↗
PubMed1998

Soluble and cell surface ICAM-1 as markers for disease activity in multiple sclerosis.

OBJECTIVE: The intercellular adhesion molecule-1 (ICAM-1) is a member of the Ig supergene family. ICAM-1 is expressed on various cells like peripheral blood lymphocytes, endothelial cells or thymic cells and the cell surface form is supposed to be shed into a soluble form. The expression of ICAM-1 is induced by cytokines like Interleukin-1, TNF alpha or interferon gamma. The aim of the study was to investigate whether changes of cell surface and soluble ICAM-1 in the cerebrospinal fluid (CSF) and blood are indicative for disease activity in patients with multiple sclerosis (MS). MATERIAL AND METHODS: In all patients with relapsing-remitting MS (relapse: n=31, remission: n=11) and controls (n=13) the expression of cell surface ICAM-1 (c-ICAM-1) was determined by two colour flow cytometry. Soluble ICAM-1 (s-ICAM-1) was measured by ELISA. Follow-up examinations were done 3 months later. RESULTS: In 31 patients with a current relapse we found significantly decreased expression levels of c-ICAM-1 on leukocytes in CSF (P<0.001) and blood (P<0.10), when compared to those 11 individuals experiencing remission. In contrast we observed significantly (P<0.05) increased levels of s-ICAM-1 in CSF of patients with relapses. Comparing patients who had been in remission for more than 4 weeks (n=11) with remission lasting longer than 3 months (n=28) we detected stable c-ICAM-1 expression on CD3+ T cells in blood. CONCLUSION: Our results demonstrate for the first time that c-ICAM-1 on CD3+ T-cells in CSF and blood is an activity marker in MS.

Adolescent↗
PubMed1998

Efficacy of urea therapy in children with ichthyosis. A multicenter randomized, placebo-controlled, double-blind, semilateral study.

BACKGROUND: Ichthyoses are genetic disorders of keratinization which are uncomfortable due to their conspicuous scaling, itching and cosmetic problems. Especially in childhood, ichthyoses can lead to social discrimination and psychological problems. Efficient therapies are necessary which are safe and well tolerated. OBJECTIVE: The aim of the study was to investigate the keratolytic and moisturizing properties as well as the tolerance of a new urea lotion when applied to hyperkeratotic and ichthyotic skin in childhood. METHODS: The study was conducted as a multicenter, randomized, placebo-controlled, double-blind, semilateral investigation. Sixty children between 1 and 16 years treated one side of the most affected extremity with Laceran 10% urea lotion for 8 weeks. On the other side the urea-free Laceran lotion base was given. On each side of the body a control area was left untreated. The investigators evaluated the global severity of ichthyotic symptoms with the help of a visual analogue scale. RESULTS: The analysis of the global estimation of severity of ichthyosis showed improvements being stronger in the body areas treated with Laceran 10% urea lotion (from 4.8 to 2.0 points) than in the areas treated with the urea-free Laceran lotion base (from 4.8 to 2.5 points). The response rates were 65% after 4 weeks and 78% after 8 weeks for Laceran 10% urea lotion, 50% after 4 weeks and 72% after 8 weeks for the urea-free Laceran lotion base. CONCLUSION: It can be ascertained that Laceran 10% urea lotion has a strong positive effect on generalized ichthyotic keratinization disorders.

Adolescent↗
PubMed1997

Elucidation of restrictive motility in high myopia by magnetic resonance imaging.

OBJECTIVE: To elucidate the cause of an acquired, restrictive motility disorder in patients with high myopia. METHODS: Magnetic resonance imaging (MRI) scans were obtained from 37 patients with high myopia (axial length of globe, 29.4 mm; refractive error, > -15 diopters). Additional dynamic MRI scans were obtained in which the patient fixated in various positions with the less restricted eye for 50 seconds. Twenty normal orbits were studied in control MRI scans. RESULTS: The path of the lateral rectus, in the anterior and midorbital regions, was displaced downward an average of 3.4 mm in 13 patients with typical esotropia and hypotropia. This mislocation reduces abducting torque of the lateral rectus and creates depressing and extorting moments. The muscle insertions were normal, except where previous strabismus surgery relocated them. Contact between the enlarged globe and the bones of the orbital apices that would cause esotropia was not observed. CONCLUSIONS: This eye muscle abnormality in patients with high myopia is another strabismus syndrome related to abnormalities of orbital connective tissues and muscle paths. Orbital MRI scans may be useful before strabismus surgery in patients with high myopia. If an abnormal lateral rectus path is found, surgery should be directed to normalize it. Magnetic resonance imaging morphometry in high myopia may give additional information on orbital anatomy and biomechanical mechanisms of strabismus.

Adult↗
PubMed1997

[Green hair caused by frequent swimming pool use].

Three patients presented with an acquired green discoloration of their scalp hair. History revealed that all of them swam regularly in private swimming pools. Examination of the hair by atomic emission spectroscopy showed that the green discoloration was caused by an excessively high copper content of the hair. This exogeneous discoloration is characteristically related to the uptake of copper from private swimming pools.

Adolescent↗
PubMed1997

[Nuclear magnetic resonance tomography diagnosis of eye motility disorder in high-grade myopia for planning an eye muscle operation].

BACKGROUND: This study was conducted to elucidate the etiology of the acquired, restrictive motility disorder in patients with severe myopia by magnetic resonance imaging (MRI) and intraoperative situs and to verify existing theories about strabismus to develop appropriate methods of eye muscle surgery. METHODS: Thirty-five patients with unilateral or bilateral high-degree myopia and strabismus, i.e., axial length of the globe averaged 29.4 mm. Multiple coronal, transversal and parasagittal MRI planes were obtained using a Siemens Magnetom (SP 63) 1.5 Tesla MRI scanner (TR = 550 ms, TE = 15 ms; field of view = 21 x 21 cm; pixel matrix = 256 x 512; 3 acquisitions; slice thickness 2 mm; distance factor 0.25). In addition, in a dynamic MRI the patient had to fixate with the less restricted eye for 50 s in different gaze positions. All data were validated by measurements during strabismus surgery. Twenty normal orbits were studied in control MRI scans. RESULTS: The major MRI finding was dislocation of the lateral rectus in the anterior and midorbital region at an average of 3.4 mm into the temporocaudal quadrant in 13 cases with the typical eso- and hypotropia. This dislocation will reduce the abducting torque of the lateral rectus and create depressing and extorting torques. Two-anatomical explanations are possible: (1) increasing stretch of the lateral rectus because of temporocranial distension of the globe and inability of intermuscular membranes and pulleys to stabilize the path of the lateral rectus; (2) dehiscence of the lateral levator aponeurosis. The restrictive motility disorder was never caused by contact between the enlarged globe and the bones of the orbital apices. CONCLUSIONS: Until now, an abnormal path of recti EOMs has been known only in Duane's syndrome. Prior to strabismus surgery in patients with high-degree myopia, an orbital MRI scan may be useful. If misalignment of the lateral rectus is detected, the most important aim of eye muscle surgery is to normalize the pathological path of the lateral rectus. MRI morphometry in severe myopia may give additional information on the anatomy of the orbit and biomechanical mechanisms of strabismus. Our findings demonstrate the necessity of fixation-controlled MRI scans.

Adult↗
PubMed1997

[The revised WHO classification of brain tumors. Radiological aspects of 4 new tumor entities].

PURPOSE: Characterisation of the classification of brain tumours authorized by the WHO. METHOD OF APPRAISAL: This classification was revised and published in its second version. In the revision, some tumours were regrouped histogenetically and some tumour variants were added. Radiologically relevant changes of the classification include the differentiation of four new tumour entities that are easily distinguished by MR imaging. These four tumours belong to the group of childhood tumours or tumours occurring in early adulthood and are characterized by a good prognosis after extirpation. RESULTS OF APPRAISAL: Central neurocytomas are small-cyst ventricular tumours associated with the foramen of Monroi and show moderate contrast enhancement. Infantile desmoplastic gangliogliomas/astrocytomas commonly consist of a solid tumour portion related to the leptomeninges with pronounced contrast enhancement and a typically very large cyst. Pleomorphic xanthoastrocytomas are circumscribed cortical tumours and usually show very moderate gyriform enhancement with only slight signs of a mass effect. Dysembryoblastic neuroepithelial tumours, which originate in the cortical/ subcortical region, likewise show no mass effect; they are characterised by thickening of the cortex from surrounding dysplastic tissue and erosion of the calotte.

Adult↗
PubMed1997

A novel dinucleotide mutation in keratin 10 in the annular epidermolytic ichthyosis variant of bullous congenital ichthyosiform erythroderma.

Annular epidermolytic ichthyosis has recently been delineated as a distinct clinical phenotype within the spectrum of epidermolytic keratinization disorders. The pattern of inheritance of the disorder is consistent with an autosomal dominant mode of transmission. Here we report a second incidence of this disorder in a family with two affected generations. The proband suffered from bullous ichthyosis and had bouts of disease activity associated with the development of numerous annular and polycyclic erythematous, hyperkeratotic plaques on the trunk and the proximal extremities. Histologic examination showed the typical pathology of epidermolytic hyperkeratosis, and ultrastructural analysis revealed abnormal keratin filament networks and tonofilament clumping with a perinuclear distribution. Molecular analysis revealed a novel tandem CG to GA 2-bp mutation in the same allele of keratin 10 in affected individuals, resulting in an arginine to glutamate substitution at residue 83 (R83E) of the 2B helical segment. We conclude that annular epidermolytic ichthyosis should be considered a variant of bullous congenital ichthyosiform erythroderma.

Adult↗
PubMed1997

New approach in strabismus surgery in high myopia.

AIMS: To develop appropriate methods of eye muscle surgery in highly myopic patients with esotropia and hypotropia, with respect to the pathological findings in high resolution magnetic resonance imaging (MRI). METHODS: 35 patients with unilateral or bilateral high myopia and strabismus--that is, axial length of the globe averaged 29.4 mm. Multiple coronal, transverse, and parasagittal MRI image planes were obtained using a Siemens Magnetom 1.5 tesla MRI scanner. In 15 patients with a pathological plane of recti extraocular muscles found by MRI and confirmed intraoperatively, a new technique of eye muscle surgery was performed to re-establish the physiological muscle plane. This was checked postoperatively in addition to the measurement of alignment and motility by MRI. RESULTS: The new MRI finding of a dislocation of the lateral rectus (LR) into the temporocaudal quadrant by 3.4 mm requires new surgical techniques. Only fixing the LR in the physiological meridian at the equator with a silicone loop ('guide pulley') or a non-absorbable suture is a causal therapy. This yields alignment and improves abduction and elevation. CONCLUSIONS: If the described misalignment of the LR is detected by MRI, a common high dosage recess-resect procedure for esotropia may even aggravate the deviation. The most important aim of eye muscle surgery is to normalise the pathological path of the LR. The restoration of the physiological function of the dislocated LR is remarkable.

Adult↗
PubMed1997

Higher frequency of atopic dermatitis and decrease in viral warts among children exposed to chemicals liberated in a chemical accident in Frankfurt, Germany.

BACKGROUND: In february 1993, a major accident occurred in a chemical plant in Frankfurt, Germany, resulting in the emission of a cloud containing 10 m3 of chlorinated and azo compounds such as o-nitroanisole. An inner zone of the suburb Schwanheim was severely polluted. OBJECTIVE AND METHODS: To assess possible long-lasting effects of this environmental accident on the incidence of skin diseases, we examined a total of 511 children from the inner and outer zones of Schwanheim 18 months later and compared the findings to those obtained from a control group of 143 children from the Frankfurt area and South Hessen. For 402 children of the exposed group, information about the exposure status was available, clearly defining presence of absence in the inner zone at the time of the accident. RESULTS: We observed an overall increase in atopic dermatitis among children present during the accident with an odds ratio of 2.44 (95% CI: 1.25-4.76, p value = 0.009). The prevalence of atopic dermatitis was most marked among exposed children in the age group of 6-12 years with a peak of 22.1% compared to 9.2% in the control group and 11.7% among non-exposed children from the outer zone. All cases of atopic dermatitis were mild, however, and did not require hospital treatment. A history of atopic dermatitis was obtained in 20.3% of all children. In the inner zone, 34.2% of the present cases with atopic dermatitis had a previous history of this disease compared to 31% in the outer zone and only 16.2% in the control group. Other skin diseases, such as birthmarks, congenital skin tumours, psoriasis or acne, did not differ, except for viral warts (verrucae vulgares), which were much more frequent in the control group. CONCLUSIONS: The data suggest that acute exposure to a major chemical burden is a risk factor that contributes even after the considerable time period of 1.5 years to the exacerbation of latent and also the development of 'new' atopic dermatitis.

Accidents, Occupational↗
PubMed1996

Transcranial duplex monitoring discloses hemorrhagic complication following rt-PA thrombolysis.

INTRODUCTION: Transcranial color-coded sonography (TCCS) allows imaging of basal cerebral arteries as well as brain parenchyma. It may therefore serve to monitor thrombolysis in acute stroke. CASE DESCRIPTION: rt-PA thrombolysis was performed in a patient, suffering from paradoxical embolism causing MCA occlusion. Hemorrhage immediately after completion of rt-PA infusion as well as delayed MCA recanalization could be monitored by TCCS. CONCLUSION: TCCS is useful to improve monitoring and safety of systemic thrombolytic treatment.

Adult↗
PubMed1996

Hystrix-like keratosis with nail and joint-involvement: a new genodermatosis?

BACKGROUND AND OBJECTIVE: We describe a familial disorder featuring hystrix-like keratosis, thickened nails and plantar hyperkeratosis. The index patient, a 10-year-old girl, suffered also from joint laxity and had long fingers, while in her mother only the typical skin lesions were observed. METHODS AND RESULTS: Histologic examination of the spiny hyperkeratoses in the index patient showed parakeratosis with marked cornoid lamella. On electron microscopy the keratinocytes exhibited intracellular vacuolization and aggregated tonofilaments, but no concentric shell formation. CONCLUSION: The striking skin lesions present in the 2 cases can be distinguished from other forms of hystrix-like hyperkeratoses such as nevus corniculatus or multiple digitate hyperkeratoses and hence may represent a new autosomal dominant genodermatosis.

Child↗