Clinical spectrum of and outcomes for Indian children with deficiency of adenosine deaminase 2 (DADA2): a multicentric study
Authors' conclusion
DADA-2 closely resembles PAN; early age of onset and CNS stroke are striking differentiating features from classic PAN. Most children respond well to anti-TNF agents without serious adverse events being observed during short-term follow-up.
Study context
- Population
- Indian children with deficiency of adenosine deaminase 2 (DADA2)11 females
- Participants
- 16 children
- Outcome
- Clinical spectrum of DADA2
Methodological detailsmulticentre
- Multicentre
Main finding
As reported in the abstract
- Mean (SD) age at symptom onset, males
- 46.2 (47) months
- Mean (SD) age at symptom onset, females
- 73.6 (50.4) months
- Fever and rash
- 80%
- CNS stroke
- 8 (53%)
- Hypertension
- 5 (33%)
- Anaemia
- 3 (20%)
- Arthralgia/arthritis
- 4 (26%)
Observational data: associations, not effects.
Further findings
Treatment with immunomodulators
- Steroids
- 13 (86%)
- Anti-TNF agents
- 12 (80%)
- CYC
- 2 (13%)
- MMF
- 3 (20%)
Outcomes during follow-up · median (IQR) follow-up 17 (10, 29) months
- Children achieving remission
- 14
- Recurrent strokes after initiation of anti-TNF drugs
- 0
Original publication
Clinical spectrum of and outcomes for Indian children with deficiency of adenosine deaminase 2 (DADA2): a multicentric study. Rheumatology (Oxford). Published 11 September 2024. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →