Case Report: Deficiency of Adenosine Deaminase 2 (DADA2) as a Cause of Brainstem Stroke in a 3-Year-Old Girl and the Importance of Early Fast-Track Genetic Diagnostics to Influence Therapy.


Journal

Neuropediatrics
ISSN: 1439-1899
Titre abrégé: Neuropediatrics
Pays: Germany
ID NLM: 8101187

Informations de publication

Date de publication:
12 2022
Historique:
pubmed: 12 7 2022
medline: 11 11 2022
entrez: 11 7 2022
Statut: ppublish

Résumé

Deficiency of adenosine deaminase 2 (DADA2) is a rare Mendelian, autoinflammatory multiorgan disease. We report the case of a 3.8-year-old female patient who was admitted with an acute brainstem stroke and was diagnosed with DADA2 by early initiation of exome sequencing. We recommend that DADA2 and a genetic workup should be taken into account, when evaluating strokes in children even if no other than neurological symptoms are evident.

Identifiants

pubmed: 35817355
doi: 10.1055/a-1896-5817
doi:

Substances chimiques

Adenosine Deaminase EC 3.5.4.4
Intercellular Signaling Peptides and Proteins 0

Types de publication

Case Reports Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

432-435

Informations de copyright

Thieme. All rights reserved.

Déclaration de conflit d'intérêts

None declared.

Auteurs

Janina Gburek-Augustat (J)

Division of Neuropaediatrics, Hospital for Children and Adolescents, University Hospital Leipzig, Leipzig, Germany.

Konrad Platzer (K)

Institute of Human Genetics, University of Leipzig Medical Center, Leipzig, Germany.

Isabell Schumann (I)

Institute of Human Genetics, University of Leipzig Medical Center, Leipzig, Germany.

Sven Starke (S)

Department of Paediatric Oncology, Haematology and Haemostaseology, Hospital for Children and Adolescents, University Hospital Leipzig, Leipzig, Germany.

Michael Steven Hershfield (MS)

Department of Medicine and Biochemistry, Duke University Medical Center, Durham, North Carolina, United States.

Ina Sorge (I)

Department of Pediatric Radiology, Hospital for Children and Adolescents, University Hospital Leipzig, Leipzig, Germany.

Andreas Merkenschlager (A)

Division of Neuropaediatrics, Hospital for Children and Adolescents, University Hospital Leipzig, Leipzig, Germany.

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Classifications MeSH