Klin Padiatr 2012; 224(06): 404-415
DOI: 10.1055/s-0032-1323837
Report
© Georg Thieme Verlag KG Stuttgart · New York

Interdisziplinäre AWMF-Leitlinie zur Therapie primärer Antikörpermangelerkrankungen

Interdisciplinary AWMF Guideline for the Treatment of Primary Antibody Deficiencies
J. Krudewig
1   Abteilung für Pneumologie, HELIOS Klinkum Krefeld
,
U. Baumann
2   Klinik für Pädiatrische Pneumologie, Allergologie und Neonatologie, ­Medizinische Hochschule Hannover, Hannover
,
H. Bernuth von
3   Department for Pediatric Pneumology and Immunology, University ­Hospital Berlin, Berlin
,
M. Borte
4   Immuno Deficiency Center Leipzig (IDCL), Municipal Hospital St. Georg Leipzig, Leipzig
,
U. Burkhard-Meier
5   Praxis für Hämatologie und Onkologie, Mönchengladbach
,
G. Dueckers
6   Zentrum für Kinder- und Jugendmedizin, HELIOS Klinikum Krefeld
,
E. Foerster-Waldl
7   Department of Pediatrics and Adolescent Medicine, Medical University of ­Vienna, Vienna, Austria
,
K. Franke
8   St. Marien-Krankenhaus Siegen gemGmbH, Klinik für Hämatologie und internistische Onkologie, Siegen
,
P. Habermehl
9   Kinderarztpraxis, Mainz-Hechtsheim
,
M. Hönig
10   Klinik für Kinder- und Jugendmedizin, Universitätsklinikum Ulm, Ulm
,
W. Kern
11   Klinik für Infektiologie, Universitätsklinikum Freiburg, Freiburg
,
K. Kösters
12   Medizinische Klinik II, HELIOS Klinikum Krefeld, Krefeld
,
K. Kugel
13   Deutsche Selbsthilfe Angeborene Immundefekte, Regionalgruppe ­Düsseldorf, Velbert
,
T. Lehrnbecher
14   Zentrum für Kinder- und Jugendmedizin, Klinikum der Johann-Wolfgang-Goethe-Universität, Klinik III, Frankfurt
,
J. Liese
15   Pädiatrische Infektiologie und Immunologie, Universitäts-Kinderklinik Würzburg, Würzburg
,
R. Marks
16   Kompetenzzentrum Leukämien und Präleukämien, Universitätsklinikum Freiburg, Freiburg
,
G. A. Müller
17   Abteilung Nephrologie und Rheumatologie, Universitätsmedizin ­Göttingen, Göttingen
,
R. Müller
18   Klinik und Poliklinik für HNO-Heilkunde, Universitätsklinikum Carl Gustav Carus an der Technischen Universität Dresden, Dresden
,
D. Nadal
19   Abteilung für Infektiologie, Kinderspital Zürich, Zürich, Switzerland
,
H.-H. Peter
20   Centrum für chronische Immundefizienz (CCI), Universitätsklinikum ­Freiburg, Freiburg
,
D. Pfeiffer-Kascha
21   Physiotherapiepraxis, Wuppertal
,
M. Schneider
22   Abteilung für Endokrinologie, Diabetologie und Rheumatologie, ­Universitätsklinikum Düsseldorf, Düsseldorf
,
H. Sitter
23   Abteilung für Chirurgische Forschung, Universität Marburg, Marburg
,
P. Späth
24   Institut für Pharmakologie, Universität Bern, Bern, Switzerland
,
V. Wahn
3   Department for Pediatric Pneumology and Immunology, University ­Hospital Berlin, Berlin
,
T. Welte
25   Klinik für Pneumologie, Medizinische Hochschule Hannover, Hannover
,
T. Niehues
6   Zentrum für Kinder- und Jugendmedizin, HELIOS Klinikum Krefeld
› Author Affiliations
Further Information

Publication History

Publication Date:
09 November 2012 (online)

Zusammenfassung

Hintergrund:

Gegenwärtig unterscheidet sich die Versorgung von Patienten mit primären Antikörpermangelerkrankungen erheblich von Behandler zur Behandler. Zur Verbesserung der Versorgung wurde eine evidenz- und konsensusbasierte Leitlinie zur Therapie primärer Antikörpermangelerkrankungen entwickelt.

Methodik:

Auf dem Boden der aktuell bestehenden studienbasierten Evidenz (systematische Literaturrecherche über PubMed; Deadline November 2011) wurden mittels strukturierter Konsensusfindung (nominaler Gruppenprozess) während einer Konsensuskonferenz im November 2011 in Frankfurt 14 Kernaussagen verabschiedet. Die teilnehmenden Vertreter wurden durch ihre jeweilige Fachgesellschaft/Patientenorganisation nominiert ([Tab. 1]).

Tab. 1 Teilnehmende Fachgesellschaften und Verbände.

Arbeitsgemeinschaft Pädiatrische Immunologie e. V. (API)

Berufsverband der Kinder- und Jugendärzte e. V. (BVKJ)

Berufsverband der Niedergelassenen Hämatologen und Onkologen e. V. (BNHO)

Deutsche Gesellschaft für Hämatologie und Onkologie e. V. (DGHO)

Deutsche Gesellschaft für Hals-Nasen-Ohren-Heilkunde, Kopf- und ­Halschirurgie e. V.

Deutsche Gesellschaft für Immunologie e. V. (DGfI)

Deutsche Gesellschaft für Infektiologie e. V. (DGI)

Deutsche Gesellschaft für Innere Medizin (DGIM)

Deutsche Gesellschaft für Kinder- und Jugendmedizin (DGKJ)

Deutsche Gesellschaft für Kinder- und Jugendrheumatologie (DGKJ)

Deutsche Gesellschaft für Pädiatrische Infektiologie e. V. (DGPI)

Deutsche Gesellschaft für Pneumologie und Beatmungsmedizin e. V. (DGP)

Deutsche Gesellschaft für Rheumatologie e. V. (DGRh)

Deutsche Selbsthilfe Angeborene Immundefekte e. V. (dsai)

Deutscher Verband für Physiotherapie -Zentralverband der ­Physiotherapeuten/Krankengymnasten e. V. (ZVK)

Gesellschaft für Pädiatrische Onkologie und Hämatologie e. V. (GPOH)

Gesellschaft für Pädiatrische Pneumologie e. V. (GPP)

Österreichische Gesellschaft für Kinder- und Jugendheilkunde (ÖGKJ)

Schweizerische Gesellschaft für Allergologie und Immunologie (SGAI)

Schweizerische Gesellschaft für Pädiatrie/Swiss Society of Paediatrics (SGP)

Ergebnisse:

Die Empfehlungen der Leitlinie beziehen sich auf die Indikation, Durchführung und Steuerung der Immunglobulinsubstitutionstherapie sowie auf verschiedene Applikationsarten. Weiterhin werden Empfehlungen zu supportiven Maßnahmen wie antiinfektiöser Therapie, Impfungen und Physiotherapie gegeben. Die Kombination aus studienbasierter Evidenz und Expertenerfahrung in dieser evidenz- und konsensusbasierten Leitlinie ermöglicht eine Verbesserung der Versorgungsqualität für Patienten mit Antikörpermangelerkrankungen.

Abstract

Backround:

Currently, management of antibody deficient patients differs significantly among caregivers. Evidence and consensus based (S3) guidelines for the treatment of primary antibody deficiencies were developed to improve the management of these patients.

Methods:

Based on a thorough analysis of current evidence (systematic literature search in PubMed; deadline November 2011) 14 recommendations were finalized during a consensus meeting in Frankfurt in November 2011 using structured consensus methods (nominal group technique). Experts were nominated by their scientific societies/patient initiatives ([Tab. 1]).

Results:

The guidelines focus on indication, practical issues and monitoring of immunoglobulin replacement therapy as well as on different routes of administration. Furthermore recommendations regarding supportive measures such as antiinfective therapy, vaccinations and physiotherapy are given. Combining literature evidence and experience of caregivers within this evidence and consensus based guidelines offers the chance to improve the quality of care for anti­body deficient patients.

 
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