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  • 1
    Electronic Resource
    Electronic Resource
    IgA-epidermolysis bullosa acquisita in a child resulting in blindness (1997)
    Oxford, UK : Blackwell Science Ltd
    British journal of dermatology 137 (1997), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Epidermolysis bullosa acquisita (EBA) is an acquired subepidermal bullous disease characterized by IgG autoantibodies directed against type VII collagen, the major component of anchoring fibrils. The classical phenotype of EBA is a non-inflammatory, mechanobullous disease resembling the dystrophic forms of inherited epidermolysis bullosa. Mucous membrane involvement is frequent but usually mild. We report a 1-year-old girl suffering from IgA-EBA, who presented with an initial eruption of disseminated urticarial lesions and tense blisters of the skin but subsequently developed severe oral and ocular lesions reminiscent of cicatricial pemphigoid. Direct immunofluorescence of the skin and buccal mucosa revealed linear IgA and C3 at the basement membrane zone (BMZ). IgA anti-BMZ autoantibodies stained the dermal side of salt-split skin by indirect immunofluorescence and recognized a dermal protein of 290 kDa co-migrating with type VII collagen by immunoblotting. Direct and indirect immunoelectron microscopy revealed IgA deposits overlying the anchoring fibrils. The ocular involvement led to total blindness in spite of intense treatment. This case of childhood IgA-EBA is particularly striking because of the cicatricial pemphigoid phenotype with severe ocular involvement which resulted in blindness. It reinforces the necessity to use modern immunological methods to classify autoimmune bullous diseases in order to allow early and appropriate treatment.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2133.1997.18191915.x
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  • 2
    Electronic Resource
    Electronic Resource
    MR imaging of breast hemangioma in female infants (1992)
    Springer
    Pediatric radiology 22 (1992), S. 463-464 
    Details
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Breast hemangioma in female infants is a rare benign lesion, prone to spontaneous regression. But when the lesion regresses there is a risk of breast atrophy if the breast bud is included in or very close to the hemangioma. A trial of corticosteroid therapy could be proposed to prevent this risk, but one must be sure that the breast bud is included in or very close to the hemangioma before treatment. We studied 4 children with breast hemangioma to evaluate the ability of MR Imaging in the diagnosis of breast bud inclusion. 0.5 Tesla axial Spin Echo T2-weighted images (TR=2000 ms; TE=120 ms) clearly depicted interface between high signal appearance of hemangioma and hypo-intensity of the breast bud: in our four patients we were able to determine whether or not the hemangioma involved the breast bud. Our preliminary study seems to demonstrate that MR imaging is a valuable imaging technique to determine which patients could be eligible for a trial of corticosteroid therapy.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02013514
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    The current management of cervico-cephalic venous malformations (1996)
    Springer
    Pediatric surgery international 11 (1996), S. 304-307 
    Details
    ISSN: 1437-9813
    Keywords: Cervicofacial venous malformations ; Venous angiomas ; Sclerotherapy ; Vascular malformation ; Ethibloc
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Venous malformations are often detected at birth and progressively increase in size if not treated. They can have severe aesthetic and functional consequences. Our purpose is to evaluate therapeutic results with Ethibloc. From 1982 to 1994, we have been using Ethibloc injections under fluoroscopic control in a group of 421 patients with malformations in cervicofacial veins. The diagnosis was made on the basis of clinical examinations as well as by using CT and MRI. In addition, 40 patients had a diagnostic angiogram. The therapeutic procedure consisted of direct puncture and opacification of venous the basis of lakes before injection of Ethibloc. Two hundred patients have been analyzed. In 139 patients treated with Ethibloc alone, the venous malformations were reduced significantly in size in 64%, while we obtained good results in 67% of the 61 patients treated with a combined procedure (Ethibloc followed by surgical excision). In those, Ethibloc effectively prevented extensive blood loss during surgery and delineated the malformations. Minor complications occurred such as fever or aseptic superficial abscesses. Ethibloc is safe, has no neurotoxicity, is efficient can be repeated many times and facilitates surgery. It must be used as the therapy of choice in venous malformations.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00497798
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