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List of works by Austen Worth

A prospective study on the natural history of patients with profound combined immunodeficiency: An interim analysis.

scientific article

Activating mutations of N-WASP alter Shigella pathogenesis

scientific article published on 18 April 2009

Adenosine deaminase deficient severe combined immunodeficiency presenting as atypical haemolytic uraemic syndrome

scientific article

Autoimmune lymphoproliferative syndrome: molecular basis of disease and clinical phenotype

scientific article

Biallelic mutations in DNA ligase 1 underlie a spectrum of immune deficiencies

scientific article published on 05 November 2018

Bleeding and splenectomy in Wiskott-Aldrich syndrome: A single-centre experience

scientific article published on 23 July 2018

Chronic Infection with Rotavirus Vaccine Strains in UK Children with Severe Combined Immunodeficiency

scientific article published in September 2015

Clinical and immunological features in a cohort of patients with partial DiGeorge syndrome followed at a single center

scientific article published on 23 April 2019

Correction of both immunodeficiency and hypoparathyroidism by thymus transplantation in complete DiGeorge syndrome

scientific article published on 30 October 2019

Current and emerging treatment options for Wiskott-Aldrich syndrome

scientific article

Deletion of Wiskott-Aldrich syndrome protein triggers Rac2 activity and increased cross-presentation by dendritic cells

scientific article published on 18 July 2016

Detection of Low Frequency Multi-Drug Resistance and Novel Putative Maribavir Resistance in Immunocompromised Pediatric Patients with Cytomegalovirus

scientific article published on 09 September 2016

Disease-associated missense mutations in the EVH1 domain disrupt intrinsic WASp function causing dysregulated actin dynamics and impaired dendritic cell migration

scientific article

Effect of stem cell source on long-term chimerism and event-free survival in children with primary immunodeficiency disorders after fludarabine and melphalan conditioning regimen

scientific article

Erratum to: Adenosine Deaminase Deficient Severe Combined Immunodeficiency Presenting as Atypical Haemolytic Uraemic Syndrome

scientific article

Favipiravir and Zanamivir Cleared Infection with Influenza B in a Severely Immunocompromised Child

scientific article published on 03 March 2020

Frequent occurrence of cytomegalovirus retinitis during immune reconstitution warrants regular ophthalmic screening in high-risk pediatric allogeneic hematopoietic stem cell transplant recipients

scientific article published on 3 April 2014

Host natural killer immunity is a key indicator of permissiveness for donor cell engraftment in patients with severe combined immunodeficiency

scientific article

Human cytomegalovirus haplotype reconstruction reveals high diversity due to superinfection and evidence of within-host recombination

scientific article published on 28 February 2019

Identifying functional defects in patients with immune dysregulation due to LRBA and CTLA-4 mutations.

scientific article published on 3 February 2017

Incomplete penetrance for isolated congenital asplenia in humans with mutations in translated and untranslated RPSA exons

Incomplete penetrance for isolated congenital asplenia in humans with mutations in translated and untranslated exons

article published in the Proceedings of the National Academy of Sciences of the United States of America

Increased proportions of γδ T lymphocytes in atypical SCID associate with disease manifestations

scientific article published on 15 February 2019

Inherited p40phox deficiency differs from classic chronic granulomatous disease

scientific article published on 06 August 2018

Long-term follow up of IPEX syndrome patients after different therapeutic strategies: an international multicenter retrospective study.

scientific article published on 11 December 2017

Modelling of human Wiskott-Aldrich syndrome protein mutants in zebrafish larvae using in vivo live imaging

scientific article

Non-posttransplant lymphoproliferative disorder malignancy after hematopoietic stem cell transplantation in patients with primary immunodeficiency: UK experience

scientific article published on 07 March 2018

Omission of in vivo T-cell depletion promotes rapid expansion of naïve CD4+ cord blood lymphocytes and restores adaptive immunity within 2 months after unrelated cord blood transplant

scientific article published on 09 January 2012

One hundred percent survival after transplantation of 34 patients with Wiskott-Aldrich syndrome over 20 years

scientific article published on 25 July 2018

Phosphorylation of WASp is a key regulator of activity and stability in vivo

scientific article published on September 2009

Pre-emptive rituximab based on viraemia and T cell reconstitution: a highly effective strategy for the prevention of Epstein-Barr virus-associated lymphoproliferative disease following stem cell transplantation

scientific article published on 13 September 2011

Risk factors predisposing to the development of hypogammaglobulinemia and infections post-Rituximab.

scientific article published on 11 August 2017

Severe Epstein-Barr virus infection in primary immunodeficiency and the normal host

scientific article

Stem cell transplantation for primary immune deficiency

scientific article

Stem cell transplantation for tetratricopeptide repeat domain 7A deficiency: long-term follow-up

scientific article published on 14 July 2016

Successful stem cell transplant with antibody-based conditioning for XIAP deficiency with refractory hemophagocytic lymphohistiocytosis ⬇️

scientific article published on June 13, 2013

Successful treatment of juvenile myelomonocytic leukemia relapsing after stem cell transplantation using donor lymphocyte infusion

scientific article published on 03 October 2002

T-cell receptor αβ+ and CD19+ cell-depleted haploidentical and mismatched hematopoietic stem cell transplantation in primary immune deficiency.

scientific article published on 2 August 2017

The Wiskott-Aldrich syndrome: The actin cytoskeleton and immune cell function

scientific article

The extended phenotype of LPS-responsive beige-like anchor protein (LRBA) deficiency

scientific article

Thymus transplantation for complete DiGeorge syndrome: European experience.

scientific article

Treatment of severe forms of LPS-responsive beige-like anchor protein deficiency with allogeneic hematopoietic stem cell transplantation

scientific article published on 10 May 2017

Treosulfan and Fludarabine Conditioning for Hematopoietic Stem Cell Transplantation in Children with Primary Immunodeficiency: UK Experience.

scientific article published on 16 November 2017

Two novel activating mutations in the Wiskott-Aldrich syndrome protein result in congenital neutropenia

scientific article published on 27 June 2006

Umbilical cord blood transplantation without in vivo T-cell depletion for children with MHC class II deficiency

scientific article published on 20 January 2018

WIP regulates the stability and localization of WASP to podosomes in migrating dendritic cells

scientific article published on December 2006

Whole Genome Sequencing of Primary Immunodeficiency reveals a role for common and rare variants in coding and non-coding sequences

Wiskott Aldrich syndrome protein regulates non-selective autophagy and mitochondrial homeostasis in human myeloid cells

scientific article published on 02 November 2020

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