0000000000061153
AUTHOR
Peter J. Späth
Untersuchungen zum hereditären Angioödem im deutschsprachigen Raum
In 6 Zentren der BRD, der Schweiz und Osterreichs wurden 242 Personen erfast, bei denen ein quantitativer und funktioneller Defekt des C1-Esterase-Inhibitors (C1-INH) biochemisch nachgewiesen und uber 2–6 Generationen verfolgt werden konnte. Bezogen auf die Gesamteinwohnerzahl der 3 Lander betragt die Frequenz des HAE auf der Basis der von uns erfasten Falle 0,02×10−4. Da unsere epidemiologischen Untersuchungen nicht flachendeckend erfolgten, ist mit einer um mindestens 1–2 Zehnerpotenzen hoheren Dunkelziffer zu rechnen. Innerhalb eines Kollektivs von 110 Personen mit klinischen Manifestationen eines hereditaren Angioodems (HAE) wurden retrospektiv anamnestische, klinische, Labor- und Thera…
Intravenous immunoglobulin in primary and secondary chronic progressive multiple sclerosis: a randomized placebo controlled multicentre study
In patients with relapsing-remitting multiple sclerosis (MS), IVIG was shown to reduce the relapse rate and progression of disability. In patients with chronic progressive MS, a beneficial effect of IVIG was not documented in placebo controlled studies. This trial investigated the influence of IVIG in primary (PPMS) and secondary (SPMS) chronic progressive MS. Two-hundred and thirty-one patients stratified for PPMS ( n = 34) and SPMS ( n = 197) were randomly assigned to IVIG 0.4 g/kg per month or to placebo for 24 months. Primary endpoints were 1) the time to sustained progression of disease identified as worsening of the expanded disability status scale (EDSS) sustained for 3 months, and …
Hereditary angioedema in children and adolescents - A consensus update on therapeutic strategies for German-speaking countries.
Background/methods At a consensus meeting in August 2018, pediatricians and dermatologists from German-speaking countries discussed the therapeutic strategy for the treatment of pediatric patients with type I and II hereditary angioedema due to C1 inhibitor deficiency (HAE-C1-INH) for Germany, Austria, and Switzerland, taking into account the current marketing approval status. HAE-C1-INH is a rare disease that usually presents during childhood or adolescence with intermittent episodes of potentially life-threatening angioedema. Diagnosis as early as possible and an optimal management of the disease are important to avoid ineffective therapies and to properly treat swelling attacks. This art…
Hereditary angiodema: a current state-of-the-art review, VII: Canadian Hungarian 2007 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema.
Background We published the Canadian 2003 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema (HAE; C1 inhibitor [C1-INH] deficiency) in 2004. Objective To ensure that this consensus remains current. Methods In collaboration with the Canadian Network of Rare Blood Disorder Organizations, we held the second Canadian Consensus discussion with our international colleagues in Toronto, Ontario, on February 3, 2006, and reviewed its content at the Fifth C1 Inhibitor Deficiency Workshop in Budapest on June 2, 2007. Papers were presented by international investigators, and this consensus algorithm approach resulted. Results This consensus algorithm …