Primary Immunodeficiency Diseases and Bacillus Calmette-Guérin (BCG)-Vaccine–Derived Complications: A Systematic Review
Publication date: April 2020Source: The Journal of Allergy and Clinical Immunology: In Practice, Volume 8, Issue 4Author(s): Saba Fekrvand, Reza Yazdani, Peter Olbrich, Andrew Gennery, Sergio D. Rosenzweig, Antonio Condino-Neto, Gholamreza Azizi, Hosein Rafiemanesh, Gholamreza Hassanpour, Nima Rezaei, Hassan Abolhassani, Asghar Aghamohammadi
Immunoglobulin replacement therapy (IgG) is the mainstay of treatment in many primary immunodeficiency diseases (PIDD), including common variable immunodeficiency, hypogammaglobulinemia, and antibody deficiency. Compared to intravenous administration, subcutaneous delivery is becoming the preferred route of delivery for patients due to its flexibility and mitigation of side effects1. The subcutaneous route is administered without the need for intravenous access and there ‘s a decreased risk for systemic reactions.
Post hematopoietic cell transplantation (HCT) autoimmune cytopenia (AIC) is a potential life-threatening complication but studies focusing on large cohorts of patients transplanted for primary immunodeficiency (PID) are lacking.
Both intravenous and subcutaneous human immune globin G (IgG) replacement (IVIG and SCIG, respectively) reduce severe infection and increase serum IgG levels in primary immune deficiency disorder (PIDD) patien...
CONCLUSION: Ten warning sign scoring system is of less value to consider a patient suspected of having primary immune deficiency. There is a meaningful delay in diagnosis of primary immune deficiencies especially in antibody deficiency defects which seeks further upgrading of knowledge in physicians. PMID: 32404246 [PubMed - as supplied by publisher]
Conclusions.PID accounted for about 40% of NCFB. Early diagnosis/appropriate treatment have impact on clinical course of a PID patient. Thus, follow-up in also immunology clinics should be a routine for patients who experience pneumonia in the first year of their lives and those with NCFB. Most patients with NCFB (84.28%) had their first pulmonary infection within the first year of their lives. PMID: 32372587 [PubMed - as supplied by publisher]
In a national cohort, we demonstrate the safety and efficacy of tailored chemo-immunotherapy followed by RIC HSCT for PID-associated lymphoproliferative disorders in children.
Abstract The prevalence of primary immunodeficiency (PID) is rather high in Iran compared to the world average, mainly due to the high rate of consanguineous marriage. Despite that, little genetic information is available about primary immunodeficiencies in Iran. Autosomal recessive hyper IgE syndrome (AR-HIES) is a severe type of immunodeficiency, mainly caused by mutations in the dedicator of cytokinesis 8 (DOCK8). Rapid and precise diagnoses of patients suffering from AR-HIES can help to manage the patients and reach properly the treatment decision. However, in regions with low financial resources and limited e...
Activated PI3K delta syndrome (APDS) belongs to the heterogeneous group of primary immunodeficiency disorders (PIDs). Progress in next-generation sequencing (NGS) enabled identification of gain-of-function mut...
Serum immunoglobulin G (IgG) concentrations are integral to the workup of immune deficiencies and IgG4-related disease (IgG4-RD). Demographic differences in IgG concentrations are poorly described but could influence test interpretation, contribute to racial disparities in primary immunodeficiency diagnosis, and explain demographic differences in IgG concentrations in IgG4-RD.
Serum immunoglobulin G (IgG) concentrations are integral to the workup of immune deficiencies and IgG4-related disease (IgG4-RD). Demographic differences in IgG concentrations are poorly described but can influence test interpretation, contribute to racial disparities in primary immunodeficiency diagnosis, and explain demographic differences in IgG concentrations in IgG4-RD.