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. 2019 Apr 18;380(16):1582-1584.
doi: 10.1056/NEJMc1801927.

Treatment Strategies for Deficiency of Adenosine Deaminase 2

Amanda K Ombrello  1 , Jing Qin  2 , Patrycja M Hoffmann  3 , Parag Kumar  4 , Deborah Stone  3 , Anne Jones  3 , Tina Romeo  3 , Beverly Barham  3 , Gineth Pinto-Patarroyo  3 , Camilo Toro  3 , Ariane Soldatos  5 , Qing Zhou  3 , Natalie Deuitch  3 , Ivona Aksentijevich  3 , Sherry L Sheldon  4 , Susan Kelly  6 , Ada Man  7 , Karyl Barron  2 , Michael Hershfield  6 , Willy A Flegel  4 , Daniel L Kastner  3
Affiliations

Treatment Strategies for Deficiency of Adenosine Deaminase 2

Amanda K Ombrello et al. N Engl J Med. .
No abstract available

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Figures

Figure 1.
Figure 1.. Effects of FFP Administration and Anti-TNF Initiation in Patients with ADA2 Deficiency.
Panel A shows the half-life of adenosine deaminase 2 (ADA2) in 3 patients with a deficiency of this enzyme after an infusion of 100 ml of fresh-frozen plasma (FFP). Blood samples were drawn immediately after completion of the infusion and at 2 hours, 4 hours, and 6 hours after infusion, with values plotted after correction for the patient’s baseline ADA2 value. The median half-life was approximately 6.4 hours (range, 5.0 to 9.0). Panel B shows the cumulative number of strokes and recurrence rate in 15 study patients before and after the inhibition of tumor necrosis factor (TNF), according to the number of patient-months observed (P<0.001 by the likelihood ratio test). Panel C shows the number of strokes in the same 15 patients on the basis of matched follow-up time (733 patient-months) before and after anti-TNF therapy (P<0.001 by the Blyth–Still–Casella method).
See this image and copyright information in PMC

References

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