X-Linked Agammaglobulinemia (XLA) Research This is an RSS file. You can use it to subscribe to this data in your favourite RSS reader or to display this data on your own website or blog.

Reactogenicity and immunogenicity of the second COVID-19 vaccination in patients with inborn errors of immunity or mannan-binding lectin deficiency
BackgroundPatients with inborn errors of immunity (IEI) are at increased risk for severe courses of SARS-CoV-2 infection. COVID-19 vaccination provides effective protection in healthy individuals. However, it remains unclear whether vaccination is efficient and safe in patients with constitutional dysfunctions of the immune system. Thus, we analyzed the humoral response, adverse reactions and assessed the disease activity of the underlying disease after COVID-19 vaccination in a cohort of patients suffering from IEIs or mannan-binding lectin deficiency (MBLdef).MethodsVaccination response was assessed after basic immunizat...
Source: Frontiers in Immunology - September 14, 2022 Category: Allergy & Immunology Source Type: research

Meningoencephalitis in primary antibody deficiency: Our experience from northwest India
CONCLUSIONS: To conclude, meningoencephalitis is an uncommon complication in patients with PAD and is associated with high morbidity and mortality. Early diagnosis of immune deficiency and initiation of replacement immunoglobulin therapy may prevent the development of neurological complications.PMID:36030644 | DOI:10.1016/j.jneuroim.2022.577952 (Source: Herpes)
Source: Herpes - August 28, 2022 Category: Infectious Diseases Authors: Ankur Kumar Jindal Himanshi Chaudhary Rahul Tyagi Amit Rawat Deepti Suri Pratap Kumar Patra Kanika Arora Sanchi Chawla Sameer Vyas Munish Arora Ridhima Aggarwal Suprit Basu Reema Bansal Man Updesh Singh Sachdeva Anju Gupta Vignesh Pandiarajan Naveen Sankh Source Type: research

COVID-19-related health outcomes in people with primary immunodeficiency: A systematic review
Clin Immunol. 2022 Aug 13:109097. doi: 10.1016/j.clim.2022.109097. Online ahead of print.ABSTRACTA better understanding of COVID-19 in people with primary immunodeficiency (PI), rare inherited defects in the immune system, is important for protecting this population, especially as population-wide approaches to mitigation change. COVID-19 outcomes in the PI population could have broader public health implications because some people with PI might be more likely to have extended illnesses, which could lead to increased transmission and emergence of variants. We performed a systematic review on COVID-19-associated morbidity a...
Source: Clinical Immunology - August 16, 2022 Category: Allergy & Immunology Authors: Emily Drzymalla Ridgely Fisk Green Martha Knuth Muin J Khoury W David Dotson Adi Gundlapalli Source Type: research

Response to mRNA COVID-19 vaccination in three XLA patients
We present three patients with XLA who received an mRNA COVID-19 vaccine. One patient demonstrated positive antibody response. Many XLA patients do not receive routine vaccinations due to ongoing immunoglobulin replacement therapy and lack of native antibody production, but in addition to T-cell response to vaccination, select XLA patients may mount a positive antibody response. Therefore, COVID-19 vaccination should be encouraged for all XLA patients.PMID:35934578 | DOI:10.1016/j.vaccine.2022.07.046 (Source: Vaccine)
Source: Vaccine - August 7, 2022 Category: Allergy & Immunology Authors: J D Squire A Y Joshi Source Type: research

Agammaglobulinemia: from X-linked to Autosomal Forms of Disease
AbstractInterruptions or alterations in the B cell development pathway can lead to primary B cell immunodeficiency with resultant absence or diminished immunoglobulin production. While the most common cause of congenital agammaglobulinemia is X-linked agammaglobulinemia (XLA), accounting for approximately 85% of cases, other genetic forms of agammaglobulinemia have been identified. Early recognition and diagnosis of these conditions are pivotal for improved outcomes and prevention of sequelae and complications. The diagnosis of XLA is often delayed, and can be missed if patient has a mild phenotype. The lack of correlation...
Source: Clinical Reviews in Allergy and Immunology - August 1, 2022 Category: Allergy & Immunology Source Type: research

Targeted Gene Sanger Sequencing Should Remain the First-Tier Genetic Test for Children Suspected to Have the Five Common X-Linked Inborn Errors of Immunity
To address inborn errors of immunity (IEI) which were underdiagnosed in resource-limited regions, our centre developed and offered free genetic testing for the most common IEI by Sanger sequencing (SS) since 2001. With the establishment of The Asian Primary Immunodeficiency (APID) Network in 2009, the awareness and definitive diagnosis of IEI were further improved with collaboration among centres caring for IEI patients from East and Southeast Asia. We also started to use whole exome sequencing (WES) for undiagnosed cases and further extended our collaboration with centres from South Asia and Africa. With the increased use...
Source: Frontiers in Immunology - July 8, 2022 Category: Allergy & Immunology Source Type: research