A5058777943- Immunodeficiency and Autoimmune Disorders
- Platelet Disorders and Treatments
- Blood disorders and treatments
- Blood groups and transfusion
- Chronic Lymphocytic Leukemia Research
- Autoimmune Bullous Skin Diseases
- Immune Cell Function and Interaction
- Blood Coagulation and Thrombosis Mechanisms
- Pharmaceutical studies and practices
- Immune Response and Inflammation
- Monoclonal and Polyclonal Antibodies Research
- Peptidase Inhibition and Analysis
- Cytomegalovirus and herpesvirus research
- Diabetes and associated disorders
- T-cell and B-cell Immunology
- Protease and Inhibitor Mechanisms
- NF-κB Signaling Pathways
- Peripheral Neuropathies and Disorders
- Myasthenia Gravis and Thymoma
- Drug-Induced Adverse Reactions
- Cell death mechanisms and regulation
- DNA Repair Mechanisms
- Acute Lymphoblastic Leukemia research
- Pharmaceutical Economics and Policy
- Dermatological and COVID-19 studies
University of California, Irvine
2007-2025
Takeda (United States)
2016-2024
Takeda (France)
2023-2024
Australian National University
2022
Baxalta (United States)
2015-2021
Children's Hospital of Philadelphia
2021
Dicerna Pharmaceuticals (United States)
2016
Baxter (United States)
2013-2015
Irvine University
2012
Hacettepe University Hospital
1995-2011
Most patients with congenital hypogammaglobulinemia and absent B cells are males X-linked agammaglobulinemia, which is caused by mutations in the gene for Bruton's tyrosine kinase (Btk); however, there females a similar disorder who do not have this gene. We studied two families autosomal recessive defects B-cell development presumed agammaglobulinemia did Btk.A series of candidate genes that encode proteins involved signal-transduction pathways were analyzed linkage studies mutation...
ADVANCE-CIDP 1 evaluated facilitated subcutaneous immunoglobulin (fSCIG; human G 10% with recombinant hyaluronidase) efficacy and safety in preventing chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) relapse.ADVANCE-CIDP was a phase 3, double-blind, placebo-controlled trial conducted at 54 sites 21 countries. Eligible adults had definite or probable CIDP adjusted Inflammatory Neuropathy Cause Treatment (INCAT) disability scores of 0-7 (inclusive), received stable intravenous...
IgM deficiency is a dysgammaglobulinemia characterized by isolated low levels of serum IgM. Patients with may exhibit various clinical manifestations. However, still remains to be explored regard diagnosis and treatment.Through retrospective chart review, we investigated the immunological features 15 symptomatic adult IgM-deficient patients who were referred our immunology clinics over 4-year period.The comprised 6 males 9 females, mean age 57.2 years. On initial evaluation, 12 (80%)...
Patients with primary immunodeficiency disease (PIDD) typically require life-long intravenous (IV) or subcutaneous (SC) immunoglobulin (Ig) replacement therapy to prevent recurrent infections. The efficacy, safety, and pharmacokinetics of a highly concentrated (20 %) Ig preparation for SC administration (IGSC 20 were evaluated in prospective trial patients PIDD. A total 74 (aged 3–83 years) received 4327 IGSC % infusions over median 380.5 days. rate validated serious bacterial infections was...
Summary A highly concentrated (20%) immunoglobulin (Ig)G preparation for subcutaneous administration (IGSC 20%), would offer a new option antibody replacement therapy in patients with primary immunodeficiency diseases (PIDD). The efficacy, safety, tolerability and pharmacokinetics of IGSC 20% were evaluated prospective trial Europe 49 PIDD aged 2–67 years. Over median 358 days, received 2349 infusions at monthly doses equivalent to those administered previous intravenous or IgG treatment....
Treatment of primary immunodeficiency diseases (PIDD) with subcutaneous (SC) infusions IgG preceded by injection recombinant human hyaluronidase (rHuPH20) (IGHy) to increase SC tissue permeability was evaluated in two consecutive, prospective, non-controlled, multi-center studies. Subjects >4 years age received replacement at a weekly dose equivalent 108 % their previous intravenous (IV) dose, facilitated prior 75 U/g rHuPH20. Starting infusions, the interval increased (ramped-up) 3- or...
To assess the long-term efficacy, safety and tolerability of recombinant human hyaluronidase-facilitated subcutaneous infusion immunoglobulin (Ig) (fSCIG; HYQVIA(®); IGHy) in children aged <18 years.Patients with primary immunodeficiency diseases were included studies. IGHy was administered every 3 or 4 weeks.Validated acute serious bacterial infections reported at 0.08/patient-year (four pneumonia episodes three patients). No adverse drug reaction (ADR) reported, rates local systemic ADRs...
There is a worldwide increasing concern over the neurological risks of thimerosal (ethylmercury thiosalicylate) which an organic mercury compound that commonly used as antimicrobial preservative. In this study, we show thimerosal, at nanomolar concentrations, induces neuronal cell death through mitochondrial pathway. Thimerosal, in concentration- and time-dependent manner, decreased viability assessed by calcein-ethidium staining caused apoptosis detected Hoechst 33258 dye....
Aim: To assess the long-term safety of hyaluronidase-facilitated subcutaneous immunoglobulin (fSCIG) 10% in European routine clinical practice. Materials & methods: This prospective, noninterventional, open-label, post-authorization study (EUPAS5812) sourced data on adverse events, immunogenicity, treatment regimens and product administration for 106 adult patients prescribed fSCIG across 17 sites six countries from July 2014 to February 2020. Results: In total, 1171 treatment-emergent...
Abstract Purpose To investigate the efficacy, safety, tolerability, and serum IgG trough levels of hyaluronidase-facilitated subcutaneous immunoglobulin (fSCIG) 10% in US pediatric patients with primary immunodeficiency diseases (PIDDs). Methods This phase 3, open-label, prospective study (NCT03277313) was conducted at 17 centers. Eligible aged 2 to < 16 years had PIDDs received G (IgG) a consistent dose for ≥ 3 months before screening. Participants fSCIG via ramp-up up 6 weeks (Epoch 1),...
Interleukin-12 receptor beta-1 (IL-12Rβ1) defect is generally associated with selective susceptibility to weakly pathogenic mycobacteria and Salmonella species. Patients rarely experience infections caused by other organisms. We report a 5-year-old patient IL-12Rβ1 deficiency who developed recurrent visceral leishmaniasis 6 months apart. The responded lyposomal amphotericin B treatment reasonably well.
Yellow nail syndrome (YNS) is a rare disorder of unknown aetiology that characterized by yellow nails associated with lymphoedema and chronic respiratory manifestations. There are no detailed immunological studies in YNS. In this study, we present first extensive analysis both adaptive innate immunity two patients One patient has common variable immunodeficiency, whereas second specific antibody deficiency syndrome. Severe lymphopaenia, striking naïve CD4+ CD8+ T cells total B cells,...