Publications by authors named "P Teira"
Background: Triokinase and FMN cyclase (TKFC) is a bifunctional enzyme involved in fructose metabolism. Triokinase catalyses the phosphorylation of fructose-derived glyceraldehyde (GA) and exogenous dihydroxyacetone (DHA), while FMN cyclase generates cyclic FMN. TKFC regulates the antiviral immune response by interacting with IFIH1 (MDA5).
View Article and Find Full Text PDFSevere combined immunodeficiency (SCID) is characterized by a severe deficiency in T cell numbers. We analyzed data collected (n = 307) for PHA-based T cell proliferation from the PIDTC SCID protocol 6901, using either a radioactive or flow cytometry method. In comparing the two groups, a smaller number of the patients tested by flow cytometry had <10% of the lower limit of normal proliferation as compared to the radioactive method (p = 0.
View Article and Find Full Text PDFBackground: P47phox (neutrophil cytosolic factor-1) deficiency is the most common cause of autosomal recessive chronic granulomatous disease (CGD) and is considered to be associated with a milder clinical phenotype. Allogeneic hematopoietic cell transplantation (HCT) for p47phox CGD is not well-described.
Objectives: We sought to study HCT for p47phox CGD in North America.
Article Synopsis
- This study focuses on two pediatric patients with systemic-onset juvenile idiopathic arthritis (sJIA) who underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT) after failing multiple treatments.
- Both patients experienced severe complications initially, but ultimately both recovered and achieved complete remission (CR), with one patient remaining off all medications five years post-transplant.
- The outcomes of these cases align with a broader review of literature on allo-HSCT for sJIA, indicating a high success rate, but emphasizing the need for careful consideration and long-term follow-up due to potential disease flares and complications.
Background: The Primary Immune Deficiency Treatment Consortium (PIDTC) enrolled children in the United States and Canada onto a retrospective multicenter natural history study of hematopoietic cell transplantation (HCT).
Objective: We investigated outcomes of HCT for severe combined immunodeficiency (SCID).
Methods: We evaluated the chronic and late effects (CLE) after HCT for SCID in 399 patients transplanted from 1982 to 2012 at 32 PIDTC centers.