Is PGD curable

Congenital, primary immunodeficiency (PGD)

Author: Prof. Dr. med. Volker Wahn, Julia Dobke, Prof. Dr. med. Tim Niehues, last changed: 01/31/2018

The term “primary immunodeficiency” (PGD) is a collective term for a group of diseases in which the function of the immune system is disturbed from birth due to a genetic defect.

In the event of a disorder, our immune system cannot or only inadequately perform its tasks. These tasks include:

  • Defense against infectious agents such as bacteria, mycobacteria, viruses, fungi and parasites
  • Establishing long-term protection against re-infections (= immunity)
  • Prevention of rheumatic and autoimmune diseases
  • Prevention of allergies
  • Prevention of spontaneous fever and inflammation reactions
  • Defense against tumor cells

All of these mechanisms work in a healthy immune system. There are currently around 300 different known genetic defects that can cause functional disorders (as of October 2015).

The following information text is aimed at those affected by a primary, congenital immunodeficiency, their families, friends, teachers and other caregivers as well as the interested public. The text is intended to contribute to a better understanding of this group of diseases, the options for their treatment and the problems and special needs of the patients concerned. It should be checked again and updated after this period has expired.

Due to the large number of known primary immunodeficiencies, we will in some cases refer to their content with the approval of the Charité's ImmunDefektZentrum.

Annotation: Our information does not replace the necessary clarifying discussions with the treating physicians and other employees of the treatment team; however, they can help to prepare these conversations and to understand them better. You can also use this information text to inform educators, teachers, friends and other caregivers of your child as fully as possible about the disease. In this way you can help prevent potential prejudice and social exclusion and thereby contribute to the best possible quality of life for your child.

Basic literature

  1. Baumann U, et al .: Primary Immunodeficiency Warning Signs and Algorithms for Diagnosis. 2010 [ISBN: 978-3-8374-2130-9] BAU2015
  2. Schmidt R, Baumann U, Botzug K, Henning C, Stoll M, Witte, T: Primary and secondary immune defects. [ISBN: 978-3-8374-2197-2] SCH2011h
  3. Kalden J, Eger, G: Greten, H et altri (ed.): Internal medicine: Increased susceptibility to infections - primary and secondary immune defects. Thieme Verlag 2010 [DOI: 10.1055 / b-0034-86240] KAL2010
  4. Grimbacher, B: J. Schölmerich (Ed.): Medical therapy in clinic and practice. Springer Verlag 2003 [ISBN: 978-3540422181] GRI2003b

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Introduction of newborn screening

for severe combined immunodeficiency (SCID)

From August 2019, all newborns will be tested for a severe, combined immunodeficiency as part of the newborn screening. An important step in the early detection of this rare but life-threatening disease.More

Primary Immunodeficiency (PID)

In the case of congenital disorders of the immune system, certain symptoms occur, such as frequent, severe infections or failure to thrive. (Photo: fotolia)

Classification of congenital immunodeficiencies:

The review article by T. Niehues and M. Weiß (from "Allergologie", 11/2003, pp. 497-503) gives an overview of the classification of immunodeficiencies according to the WHO classification. To the article here

Network initiative

by resident doctors, hospitals and the patient organization DSAI (German Self-Help for Congenital Immune Defects). More information and locations of therapy centers in Germany: here