Publications by authors named "Roxane Labrosse"

Wiskott-Aldrich syndrome (WAS) is a rare X-linked disorder characterized by combined immunodeficiency, eczema, microthrombocytopenia, autoimmunity, and lymphoid malignancies. Gene therapy (GT) to modify autologous CD34+ cells is an emerging alternative treatment with advantages over standard allogeneic hematopoietic stem cell transplantation for patients who lack well-matched donors, avoiding graft-versus-host-disease. We report the outcomes of a phase 1/2 clinical trial in which 5 patients with severe WAS underwent GT using a self-inactivating lentiviral vector expressing the human WAS complementary DNA under the control of a 1.

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The advantage of the newer biological therapies is that the immunosuppressive effect is targeted, in contrast, to the standard, traditional immunomodulatory agents, which have a more global effect. However, there are unintended targets and consequences, even to these "precise" therapeutics, leading to acquired or secondary immunodeficiencies. Besides depleting specific cellular immune subsets, these biological agents, which include monoclonal antibodies against biologically relevant molecules, often have broader functional immune consequences, which become apparent over time.

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Article Synopsis
  • Severe combined immunodeficiency (SCID) is a rare immune disorder that often requires treatments like hematopoietic cell transplantation (HCT) or gene therapy for survival, but many patients struggle with incomplete immune recovery post-treatment.
  • This study investigated the relationship between low CD4 T-cell counts and T-cell exhaustion in 61 SCID patients a median of 10.4 years after HCT, finding that those with poor T-cell reconstitution exhibited significant markers of exhaustion and increased inhibitory receptors on their T cells.
  • Results suggest that patients with fewer CD4 T cells may face late-onset T-cell exhaustion, especially following unconditioned HCT, emphasizing that elevated inhibitory receptor expression could
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  • - Tree nut (TN) and seed allergies are becoming more common and often lead to severe reactions that can persist into adulthood, impacting daily life.
  • - New diagnostic techniques like component resolved diagnostics (CRD) and the basophil activation test (BAT) can help predict allergy severity, while strict avoidance of certain TN isn't always necessary, as some patients can tolerate specific types.
  • - Strategies like oral immunotherapy (OIT) allow patients to safely increase their allergy threshold, and better understanding of co-reactivity can lead to improved management through selective TN introduction and optimized treatment plans.
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  • The study focused on creating a way to convert scores from the Food Allergy Quality of Life Questionnaire Parent Form (FAQLQ-PF) into health state utilities for better understanding food allergy impacts.
  • Researchers analyzed data from questionnaires using various regression methods to find the best model for accuracy, especially looking at individual item scores.
  • Results showed that their mapping had strong predictive accuracy, suggesting it can be useful for assessing cost-effectiveness in food allergy treatments.
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Background: Rituximab is a B-cell depleting agent used in B-cell malignancies and autoimmune diseases. A subset of adult patients may develop prolonged and symptomatic hypogammaglobulinemia following rituximab treatment. However, this phenomenon has not been well delineated in the pediatric population.

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Current recommendations for the management of penicillin allergy are to perform penicillin skin testing (PST) with penicilloyl-polylysine (PPL) and benzylpenicillin (BP) prior to drug challenge with amoxicillin. However, the role of PST is increasingly questioned in the pediatric setting. To resolve the question of PST's diagnostic accuracy, consecutive children with a history of non-life-threatening penicillin allergy referred to a tertiary-care allergy center were recruited to undergo double-blinded PST with PPL and BP prior to drug provocation to amoxicillin.

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Colorectal cancer is driven by mutations that activate canonical WNT/β-catenin signaling, but inhibiting WNT has significant on-target toxicity, and there are no approved therapies targeting dominant oncogenic drivers. We recently found that activating a β-catenin-independent branch of WNT signaling that inhibits GSK3-dependent protein degradation induces asparaginase sensitivity in drug-resistant leukemias. To test predictions from our model, we turned to colorectal cancer because these cancers can have WNT-activating mutations that function either upstream (i.

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Non-immunoglobulin E-mediated gastrointestinal food allergic disorders (non-IgE-GI-FA) include food protein-induced enterocolitis syndrome (FPIES), food protein-induced enteropathy (FPE) and food protein-induced allergic proctocolitis (FPIAP), which present with symptoms of variable severity, affecting the gastrointestinal tract in response to specific dietary antigens. The diagnosis of non-IgE-GI-FA is made clinically, and relies on a constellation of typical symptoms that improve upon removal of the culprit food. When possible, food reintroduction should be attempted, with the documentation of symptoms relapse to establish a conclusive diagnosis.

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Flexible dosing of IgPro20 (Hizentra®, CSL Behring, King of Prussia, Pennsylvania) maintains normal serum immunoglobulin G (IgG) levels in patients with primary immunodeficiencies (PID). Until now, clinical trials testing the pharmacokinetic (PK) characteristics of serum IgG of weekly and biweekly subcutaneous IgG therapy were not published. This is the first study assessing PK characteristics following weekly and biweekly IgPro20 in patients with PID.

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Background: Penicillin allergy is the most frequent drug allergy, among which aminopenicillins are reputed for causing delayed rashes in children, particularly in the context of viral infections. Despite a negative allergy evaluation, a significant proportion of individuals continue to avoid penicillin antibiotics for fear of an allergic reaction.

Objective: To evaluate the safety and efficacy of a 5-day challenge to amoxicillin and the proportion of subsequent use of amoxicillin.

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Chronic granulomatous disease (CGD) is a rare primary immune deficiency caused by mutations in genes coding for components of the nicotinamide adenine dinucleotide phosphate oxidase, characterized by severe and recurrent bacterial and fungal infections, together with inflammatory complications. Dysregulation of inflammatory responses are often present in this disease and may lead to granulomatous lesions, most often affecting the gastrointestinal (GI) and urinary tracts. Treatment of inflammatory complications usually includes corticosteroids, whereas antimicrobial prophylaxis is used for infection prevention.

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Food allergy is an important health issue that affects up to 8 % of the population. The management of allergic patients involves allergen avoidance and prompts the treatment of accidental reactions, as no curative treatment is available so far in routine practice. Oral immunotherapy (OIT) is a promising therapeutic alternative, but it is associated with frequent allergic reactions and cost-effectiveness issues.

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