Publications by authors named "Carla Duff"

Background: Patients with inborn errors of immunity (IEI) have increased risk of developing cancers secondary to impaired anti-tumor immunity. Treatment of patients with IEI and cancer is challenging as chemotherapy can exacerbate infectious susceptibility. However, the literature on optimal cancer treatment in the setting of IEI is sparse.

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Congenital athymia can present with severe T cell lymphopenia (TCL) in the newborn period, which can be detected by decreased T cell receptor excision circles (TRECs) on newborn screening (NBS). The most common thymic stromal defect causing selective TCL is 22q11.2 deletion syndrome (22q11.

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Nuts and Bolts of Subcutaneous Therapy.

Immunol Allergy Clin North Am

August 2020

Immunoglobulin replacement therapy is standard of care in treatment of many primary immunodeficiency diseases. The goal of replacement therapy is to reduce infections in individuals with primary immune deficiency and improve their quality of life. Immunoglobulin replacement therapy is most often lifelong, therefore ease of administration is vital for adherence to treatment.

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We treated three pediatric cardiac transplant patients with chronic parvovirus viremia with high-dose intravenous immunoglobulin (HD-IVIG). One patient with severe T-cell lymphopenia suffered recurrent viremia and aseptic meningitis, which resolved remarkably when he was switched to high-dose hyaluronidase-facilitated subcutaneous immunoglobulin (HD-SCIG-Hy). We discuss the advantages of HD-SCIG-Hy vs HD-IVIG treatment for similar cases.

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Article Synopsis
  • The text discusses the importance of connecting SCID symptoms to specific genetic causes, especially with advances in newborn screening and gene therapy options.
  • In western countries, X-linked IL2RG and ADA deficiency SCID are prevalent types that can be treated with gene therapy, but diagnosing genetic variants can be challenging due to their polymorphic nature and complexities in coding and non-coding regions.
  • The authors provide examples of X-linked SCID cases where initial tests did not reveal pathogenic variants, highlighting the need for further functional studies and maternal X-inactivation tests to confirm diagnosis and ensure timely eligibility for gene therapy.
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Subcutaneous immunoglobulin (SCIg) infusions are an option for patients requiring immunoglobulin therapy. Nurses are uniquely positioned to advocate for patients and to teach them how to successfully manage their infusions. The purpose of this review is to describe SCIg therapy and to provide teaching instructions as well as creative tips to ensure treatment success.

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Increased use of specialized infusion therapies has necessitated training of health care providers and patients. The Starting Hizentra Administration with Resources and Education (SHARE) program provided 709 US participants with information to educate patients with primary immunodeficiency disease (PIDD) on self-administration of 20% subcutaneous immunoglobulin (SCIG). Postprogram surveys assessed participants' experience and opinion of 20% SCIG.

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A number of ancillary supplies are used in the process of administering subcutaneous immunoglobulin. The particular type of ancillary supplies used (needles, tubing, and tape) may contribute to the development of issues at the local infusion site. Patient case studies demonstrate that changes in the choice of ancillary supplies can often alleviate these issues.

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Immunoglobulin (Ig) replacement therapy, given as regular infusions of pooled human Ig, is the recognized treatment of humoral immunodeficiencies characterized by hypogammaglobulinemia and impaired antibody responses. It is a safe, effective therapy when delivered by nurses who have been educated to oversee and/or provide these infusions. Guidelines for administration have been developed by the Immune Deficiency Foundation Nurse Advisory Committee to provide a framework and guidance to those nurses administering this therapy.

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Purpose: The tolerability of L-proline-stabilized Privigen, a new 10% liquid immunoglobulin for intravenous administration, was assessed at high infusion rates in a Phase III, open-label, single-arm, multicenter study in 45 patients with primary immune deficiencies.

Patients And Methods: Maximum infusion rates were not assigned prospectively. For analysis, patients were grouped according to maximum infusion rate in a low infusion rate group (8 mg/kg/min) and high infusion rate group (12 mg/kg/min).

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