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Johann W Bauer - One of the best experts on this subject based on the ideXlab platform.
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pseudosyndaktylie eine entzundliche und fibrotische wundheilungsstorung bei rezessiver Epidermolysis Bullosa Dystrophica
Journal Der Deutschen Dermatologischen Gesellschaft, 2015Co-Authors: Jenny Breitenbach, Christina Gruber, Alfred Klausegger, Andrea Trost, Barbara Bogner, Herbert A Reitsamer, Johann W BauerAbstract:Zusammenfassung Hintergrund Die rezessive Epidermolysis Bullosa Dystrophica (engl.: dystrophic Epidermolysis Bullosa, RDEB) ist eine genetisch bedingte bullose Dermatose und durch schwere Wundheilungsstorungen und Fingerkontrakturen gekennzeichnet. Der Zweck dieser Arbeit war es, die Rolle bekannter Entzundungs- und Fibrosemarker in Pseudosyndaktyliegewebe von RDEB-Patienten zu untersuchen und so den Mechanismus der Wundheilungsstorung und der Pseudosyndaktylie bei RDEB zu beleuchten. Patienten und Methoden Wir untersuchten die Expression der Fibrose- und/oder Entzundungsmarker Tenascin-C, α-smooth muscle actin, transformierender Wachstumsfaktor β1, Interleukin-1β und Interleukin-6 in vernarbtem und nicht vernarbtem Gewebe von gesunden Spendern und RDEB-Patienten durch semiquantitative Real-Time-PCR und gegebenenfalls durch Immunblots. Daruber hinaus untersuchten wir das Verteilungsmuster von α-smooth muscle actin und Tenascin-C durch Immunfluoreszenzmikroskopie. Ergebnisse Mithilfe von mRNA- und Proteinanalysen stellten wir in Proben von Narbengewebe, die Patienten mit Epidermolysis Bullosa Dystrophica an ihren von Pseudosyndaktylie betroffenen Handen entnommen worden waren, eine Hochregulation von Tenascin-C, Interleukin-1β und Interleukin-6, nicht jedoch von transformierendem Wachstumsfaktor β1 fest. Unerwarteterweise wurde auch α-smooth muscle actin nicht hochreguliert. Schlussfolgerungen Unsere Ergebnisse bestatigen das Auftreten von Entzundung und Fibrose bei rezessiver Epidermolysis Bullosa Dystrophica, insbesondere in Narben Dies legt nahe, dass diese Prozesse bei Pseudosyndaktylie eine wichtige Rolle spielen. Unsere Daten weisen daher auf die Moglichkeit hin, entzundungshemmende und antifibrotisch wirksame Arzneimittel zur Pravention der Pseudosyndaktylie einzusetzen.
P B Johnston - One of the best experts on this subject based on the ideXlab platform.
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cornea plana and sclerocornea in association with recessive Epidermolysis Bullosa Dystrophica case report
Cornea, 1992Co-Authors: J A Sharkey, G N Kervick, A J Jackson, P B JohnstonAbstract:We report a case of bilateral cornea plana and sclerocornea in a 14-year-old boy with recessive Epidermolysis Bullosa Dystrophica. This is the first reported association of these anomalies. Bilateral cataracts were present at initial presentation. These spontaneously reabsorbed over the subsequent 18 months.
Jenny Breitenbach - One of the best experts on this subject based on the ideXlab platform.
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pseudosyndaktylie eine entzundliche und fibrotische wundheilungsstorung bei rezessiver Epidermolysis Bullosa Dystrophica
Journal Der Deutschen Dermatologischen Gesellschaft, 2015Co-Authors: Jenny Breitenbach, Christina Gruber, Alfred Klausegger, Andrea Trost, Barbara Bogner, Herbert A Reitsamer, Johann W BauerAbstract:Zusammenfassung Hintergrund Die rezessive Epidermolysis Bullosa Dystrophica (engl.: dystrophic Epidermolysis Bullosa, RDEB) ist eine genetisch bedingte bullose Dermatose und durch schwere Wundheilungsstorungen und Fingerkontrakturen gekennzeichnet. Der Zweck dieser Arbeit war es, die Rolle bekannter Entzundungs- und Fibrosemarker in Pseudosyndaktyliegewebe von RDEB-Patienten zu untersuchen und so den Mechanismus der Wundheilungsstorung und der Pseudosyndaktylie bei RDEB zu beleuchten. Patienten und Methoden Wir untersuchten die Expression der Fibrose- und/oder Entzundungsmarker Tenascin-C, α-smooth muscle actin, transformierender Wachstumsfaktor β1, Interleukin-1β und Interleukin-6 in vernarbtem und nicht vernarbtem Gewebe von gesunden Spendern und RDEB-Patienten durch semiquantitative Real-Time-PCR und gegebenenfalls durch Immunblots. Daruber hinaus untersuchten wir das Verteilungsmuster von α-smooth muscle actin und Tenascin-C durch Immunfluoreszenzmikroskopie. Ergebnisse Mithilfe von mRNA- und Proteinanalysen stellten wir in Proben von Narbengewebe, die Patienten mit Epidermolysis Bullosa Dystrophica an ihren von Pseudosyndaktylie betroffenen Handen entnommen worden waren, eine Hochregulation von Tenascin-C, Interleukin-1β und Interleukin-6, nicht jedoch von transformierendem Wachstumsfaktor β1 fest. Unerwarteterweise wurde auch α-smooth muscle actin nicht hochreguliert. Schlussfolgerungen Unsere Ergebnisse bestatigen das Auftreten von Entzundung und Fibrose bei rezessiver Epidermolysis Bullosa Dystrophica, insbesondere in Narben Dies legt nahe, dass diese Prozesse bei Pseudosyndaktylie eine wichtige Rolle spielen. Unsere Daten weisen daher auf die Moglichkeit hin, entzundungshemmende und antifibrotisch wirksame Arzneimittel zur Pravention der Pseudosyndaktylie einzusetzen.
E J Ring - One of the best experts on this subject based on the ideXlab platform.
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chronic recurrent esophageal strictures treated with balloon dilation in children with autosomal recessive Epidermolysis Bullosa Dystrophica
The American Journal of Gastroenterology, 1993Co-Authors: Melvin B Heyman, Maurice S Zwass, M Applebaum, Colin D Rudolph, Roy L Gordon, E J RingAbstract:Two children (8- and 17-yr old) with autosomal recessive Epidermolysis Bullosa Dystrophica and chronic esophageal strictures were treated with repeated balloon dilations. General anesthesia was by face mask adapted specifically for this procedure, with careful attention to avoid skin and mucus membrane damage. A balloon dilator positioned fluoroscopically over an angiographic guidewire was gently inflated until narrowings resolved. Dilations have been performed every 1-11 [4.3 +/- 3.2 (mean +/- SD)] months in the younger patient over the last 4.3 yr, and every 8-20 (14.5 +/- 5.9) months in the older patient over the last 4.8 yr, without serious complications. Both patients were able to swallow normal foods following dilations. Repeated balloon dilations of esophageal strictures in patients with Epidermolysis Bullosa Dystrophica can be done safely. Further studies are indicated to determine its effectiveness and appropriateness as an alternative to colonic interposition.
Klaus Hahnenkamp - One of the best experts on this subject based on the ideXlab platform.
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feasibility efficacy and safety of ultrasound guided axillary plexus blockade in pediatric patients with Epidermolysis Bullosa Dystrophica
Pediatric Anesthesia, 2016Co-Authors: Ingeborg Van Den Heuvel, Antje Gottschalk, Martin Langer, Klaus Hahnenkamp, Bjorn EllgerAbstract:Summary Background In patients suffering from Epidermolysis Bullosa Dystrophica (DEB), the most severe form of Epidermolysis Bullosa, trauma or friction cause separation of the skin from underlying tissue with consecutive painful blisters, scarifications, contractures, and pseudosyndactyly. To retain functionality of the hands surgical procedures are necessary. Anesthesia is challenging as difficult airways make general anesthesia risky. Regional anesthesia is considered controversial in patients with EB as accidental subcutaneous injections can cause severe blisters. As ultrasound-guided procedures became standard of care this might have changed however. Aim In this case series, we describe feasibility, efficacy, and safety of ultrasound-guided plexus axillaris block in DEB patients undergoing hand surgery. Method We performed a retrospective analysis of the charts of all children with DEB undergoing hand surgery under plexus axillaris block and sedation between 2009 and 2013 in our institution. Results Nineteen procedures in nine children were performed. Induction of anesthesia (securing monitoring, sedation, plexus block) took a mean time of 34 min. Perioperative analgesia was adequate in all procedures. No complications such as airway incidents, conversion to general anesthesia, movement during surgery, incomplete block, or formation of new blisters were seen. Conclusion Ultrasound-guided plexus axillaris block in DEB patients undergoing hand surgery in our institution has been feasible, effective, and safe.
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a two step multidisciplinary approach to treat recurrent esophageal strictures in children with Epidermolysis Bullosa Dystrophica
Endoscopy, 2015Co-Authors: Thorsten Vowinkel, Antje Gottschalk, Klaus Hahnenkamp, Mike G Laukoetter, Rudolf Mennigen, M Boschin, Michael Frosch, Norbert Senninger, Dirk TubergenAbstract:In children with severe generalized recessive dystrophic Epidermolysis Bullosa (RDEB), esophageal scarring leads to esophageal strictures with dysphagia, followed by malnutrition and delayed development. We describe a two-step multidisciplinary therapeutic approach to overcome malnutrition and growth retardation. In Step 1, under general anesthesia, orthograde balloon dilation of the esophagus is followed by gastrostomy creation using a direct puncture technique. In Step 2, further esophageal strictures are treated by retrograde dilation via the established gastrostomy; this step requires only a short sedation period. A total of 12 patients (median age 7.8 years, range 6 weeks to 17 years) underwent successful orthograde balloon dilation of esophageal strictures combined with direct puncture gastrostomy. After 12 and 24 months in 11 children, a substantial improvement of growth and nutrition was achieved (body mass index [BMI] standard deviation score [SDS] + 0.59 and + 0.61, respectively). In one child, gastrostomy was removed because of skin ulcerations after 10 days. Recurrent esophageal strictures were treated successfully in five children. The combined approach of balloon dilation and gastrostomy is technically safe in children with RDEB, and helps to promote catch-up growth and body weight. In addition, recurrent esophageal strictures can be treated successfully without general anesthesia in a retrograde manner via the established gastrostomy.
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anesthesia for balloon dilatation of esophageal strictures in children with Epidermolysis Bullosa Dystrophica from intubation to sedation
Current Opinion in Anesthesiology, 2010Co-Authors: Antje Gottschalk, Thorsten Vowinkel, Michael Frosch, Dirk Tubergen, Stefan Venherm, Klaus HahnenkampAbstract:PURPOSE OF REVIEW Dystrophic Epidermolysis Bullosa is a rare disease mainly affecting small children. They often have to undergo different surgical procedures, for example balloon dilatation following esophageal strictures to maintain oral intake and prevent malnutrition. RECENT FINDINGS So far, no specific treatment is available for patients with any forms of Epidermolysis Bullosa. However, more and more minimal invasive surgical techniques are used to manage symptoms of the disease, for example endoscopic balloon dilatation for severe esophageal strictures. SUMMARY In this article implications for the anesthetic management of children with dystrophic Epidermolysis Bullosa are described. General anesthesia and sedation techniques are feasible if specific prerequisites are fulfilled. The team providing anesthesia and endoscopy must be familiar with the nature and disabilities of this disease. Coordinated care of the involved disciplines is crucial for all planned procedures regarding perioperative management.