Evaluation and management of deficiency of adenosine deaminase 2: an international consensus statement
PY Lee, BA Davidson, RS Abraham, B Alter… - JAMA network …, 2023 - jamanetwork.com
Importance Deficiency of adenosine deaminase 2 (DADA2) is a recessively inherited
disease characterized by systemic vasculitis, early-onset stroke, bone marrow failure, and/or …
disease characterized by systemic vasculitis, early-onset stroke, bone marrow failure, and/or …
Mechanisms of vascular inflammation in deficiency of adenosine deaminase 2 (DADA2)
Deficiency of adenosine deaminase 2 (DADA2) was first described as a monogenic form of
systemic vasculitis that closely resembles polyarteritis nodosa (PAN). The phenotypic …
systemic vasculitis that closely resembles polyarteritis nodosa (PAN). The phenotypic …
The spectrum of the deficiency of adenosine deaminase 2: an observational analysis of a 60 patient cohort
KS Barron, I Aksentijevich, NT Deuitch… - Frontiers in …, 2022 - frontiersin.org
The deficiency of adenosine deaminase 2 (DADA2) is an autosomal recessively inherited
disease that has undergone extensive phenotypic expansion since being first described in …
disease that has undergone extensive phenotypic expansion since being first described in …
[HTML][HTML] Immune dysregulation
A Long, A Kleiner, RJ Looney - Journal of Allergy and Clinical Immunology, 2023 - Elsevier
The understanding of immune dysregulation in many different diseases continues to grow.
There is increasing evidence that altered microbiome and gut barrier dysfunction contribute …
There is increasing evidence that altered microbiome and gut barrier dysfunction contribute …
Intrinsic defects in B cell development and differentiation, T cell exhaustion and altered unconventional T cell generation characterize human adenosine deaminase …
JY Yap, L Moens, MW Lin, A Kane, A Kelleher… - Journal of Clinical …, 2021 - Springer
Purpose Deficiency of adenosine deaminase type 2 (ADA2)(DADA2) is a rare inborn error of
immunity caused by deleterious biallelic mutations in ADA2. Clinical manifestations are …
immunity caused by deleterious biallelic mutations in ADA2. Clinical manifestations are …
Deficiency of human adenosine deaminase type 2–a diagnostic conundrum for the hematologist
RK Pilania, AZ Banday, S Sharma, R Kumrah… - Frontiers in …, 2022 - frontiersin.org
Deficiency of adenosine deaminase type 2 (DADA2) was first described in 2014 as a
monogenic cause of polyartertitis nodosa (PAN), early onset lacunar stroke and livedo …
monogenic cause of polyartertitis nodosa (PAN), early onset lacunar stroke and livedo …
Inborn errors of immunity and autoimmune disease
PE Gray, C David - The Journal of Allergy and Clinical Immunology: In …, 2023 - Elsevier
Autoimmunity may be a manifestation of inborn errors of immunity, specifically as part of the
subgroup of primary immunodeficiency known as primary immune regulatory disorders …
subgroup of primary immunodeficiency known as primary immune regulatory disorders …
Allogeneic hematopoietic cell transplantation for patients with deficiency of adenosine deaminase 2 (DADA2): approaches, obstacles and special considerations
Deficiency of adenosine deaminase 2 (DADA2) is an inherited autosomal recessive disease
characterized by autoinflammation (recurrent fever), vasculopathy (livedo racemosa …
characterized by autoinflammation (recurrent fever), vasculopathy (livedo racemosa …
Lentiviral Mediated ADA2 Gene Transfer Corrects the Defects Associated With Deficiency of Adenosine Deaminase Type 2
Y Hong, M Casimir, BC Houghton, F Zhang… - Frontiers in …, 2022 - frontiersin.org
Deficiency of adenosine deaminase type 2 (DADA2) is an autosomal recessive disease
caused by bi-allelic loss-of-function mutations in ADA2. Treatment with anti-TNF is effective …
caused by bi-allelic loss-of-function mutations in ADA2. Treatment with anti-TNF is effective …
Hematopoietic stem cell transplantation in systemic autoinflammatory diseases-the first one hundred transplanted patients
Introduction Primary immune regulatory disorders encompass a range of clinical conditions
caused by different defects of immune regulatory mechanisms, including systemic …
caused by different defects of immune regulatory mechanisms, including systemic …