Interdisziplinäre AWMF-Leitlinie zur Therapie primärer Antikörpermangelerkrankungen

J. Krudewig; U. Baumann; H. Bernuth von; M. Borte; U. Burkhard-Meier; G. Dueckers; E. Foerster-Waldl; K. Franke; P. Habermehl; M. Hönig; W. Kern; K. Kösters; K. Kugel; T. Lehrnbecher; J. Liese; R. Marks; G. A. Müller; R. Müller; D. Nadal; H.-H. Peter; D. Pfeiffer-Kascha; M. Schneider; H. Sitter; P. Späth; V. Wahn; T. Welte; T. Niehues

doi:10.1055/s-0032-1323837

Klinische Pädiatrie, Table of Contents

Klin Padiatr 2012; 224(06): 404-415
DOI: 10.1055/s-0032-1323837

Report

Interdisziplinäre AWMF-Leitlinie zur Therapie primärer Antikörpermangelerkrankungen

Interdisciplinary AWMF Guideline for the Treatment of Primary Antibody Deficiencies

J. Krudewig

¹Abteilung für Pneumologie, HELIOS Klinkum Krefeld

,

U. Baumann

²Klinik für Pädiatrische Pneumologie, Allergologie und Neonatologie, Medizinische Hochschule Hannover, Hannover

,

H. Bernuth von

³Department for Pediatric Pneumology and Immunology, University Hospital Berlin, Berlin

,

M. Borte

⁴Immuno Deficiency Center Leipzig (IDCL), Municipal Hospital St. Georg Leipzig, Leipzig

,

U. Burkhard-Meier

⁵Praxis für Hämatologie und Onkologie, Mönchengladbach

,

G. Dueckers

⁶Zentrum für Kinder- und Jugendmedizin, HELIOS Klinikum Krefeld

,

E. Foerster-Waldl

⁷Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria

,

K. Franke

⁸St. Marien-Krankenhaus Siegen gemGmbH, Klinik für Hämatologie und internistische Onkologie, Siegen

,

P. Habermehl

⁹Kinderarztpraxis, Mainz-Hechtsheim

,

M. Hönig

¹⁰Klinik für Kinder- und Jugendmedizin, Universitätsklinikum Ulm, Ulm

,

W. Kern

¹¹Klinik für Infektiologie, Universitätsklinikum Freiburg, Freiburg

,

K. Kösters

¹²Medizinische Klinik II, HELIOS Klinikum Krefeld, Krefeld

,

K. Kugel

¹³Deutsche Selbsthilfe Angeborene Immundefekte, Regionalgruppe Düsseldorf, Velbert

,

T. Lehrnbecher

¹⁴Zentrum für Kinder- und Jugendmedizin, Klinikum der Johann-Wolfgang-Goethe-Universität, Klinik III, Frankfurt

,

J. Liese

¹⁵Pädiatrische Infektiologie und Immunologie, Universitäts-Kinderklinik Würzburg, Würzburg

,

R. Marks

¹⁶Kompetenzzentrum Leukämien und Präleukämien, Universitätsklinikum Freiburg, Freiburg

,

G. A. Müller

¹⁷Abteilung Nephrologie und Rheumatologie, Universitätsmedizin Göttingen, Göttingen

,

R. Müller

¹⁸Klinik und Poliklinik für HNO-Heilkunde, Universitätsklinikum Carl Gustav Carus an der Technischen Universität Dresden, Dresden

,

D. Nadal

¹⁹Abteilung für Infektiologie, Kinderspital Zürich, Zürich, Switzerland

,

H.-H. Peter

²⁰Centrum für chronische Immundefizienz (CCI), Universitätsklinikum Freiburg, Freiburg

,

D. Pfeiffer-Kascha

²¹Physiotherapiepraxis, Wuppertal

,

M. Schneider

²²Abteilung für Endokrinologie, Diabetologie und Rheumatologie, Universitätsklinikum Düsseldorf, Düsseldorf

,

H. Sitter

²³Abteilung für Chirurgische Forschung, Universität Marburg, Marburg

,

P. Späth

²⁴Institut für Pharmakologie, Universität Bern, Bern, Switzerland

,

V. Wahn

³Department for Pediatric Pneumology and Immunology, University Hospital Berlin, Berlin

,

T. Welte

²⁵Klinik für Pneumologie, Medizinische Hochschule Hannover, Hannover

,

T. Niehues

⁶Zentrum für Kinder- und Jugendmedizin, HELIOS Klinikum Krefeld

› Author Affiliations

Abstract

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.

Schlüsselwörter

Primäre Antikörpermangelerkrankungen - Leitlinie - Therapie - Immunglobuline - Kinder - Erwachsene

Key words

primary antibody deficiencies - guideline - therapy - immunoglobulins - children - adults

Full Text

References

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