Publications by authors named "Attilio Di Girolamo"

Lysosomal Storage Diseases (LSDs) encompass a range of genetic disorders characterized by enzyme deficiencies that lead to substrate accumulation and progressive tissue damage. Enzyme Replacement Therapy (ERT) is the primary treatment for LSDs, yet it is often associated with hypersensitivity reactions (HSRs), ranging from mild rashes to severe anaphylaxis. These reactions, frequently driven by anti-drug antibodies, pose significant challenges in treatment adherence and patient outcomes.

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Introduction: Acyclovir is a synthetic purine nucleoside analog that is used to treat infections caused by herpes simplex virus (HSV) and varicella zoster virus (VZV) by targeting the viral enzyme thymidine kinase. However, its use can lead to hypersensitivity reactions (HR) in rare cases, resulting in treatment discontinuation. Rapid drug desensitization (DD) by intravenous or oral administration protocols are used in these patients in order to avoid treatment discontinuation.

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Background: Mucopolysaccharidosis (MPS) type 1 S and type 2 are rare lysosomal storage disorders characterized by impaired enzyme production, resulting in glycosaminoglycans accumulation within lysosomes. Enzyme Replacement Therapy (ERT) with laronidase and idursulfase are first line treatments, respectively. However, infusion-related hypersensitivity reactions (HR) may lead to ERT discontinuation.

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Article Synopsis
  • Eosinophilic granulomatous polyangiitis (EGPA) is a rare autoimmune disease marked by inflammation and high eosinophil levels, traditionally treated with corticosteroids and immunosuppressants, which can have serious side effects.
  • A systematic review and meta-analysis of eight studies with 396 EGPA patients found that benralizumab, a biologic agent targeting the interleukin-5 receptor, significantly reduced oral corticosteroid use and led to remission in over half the patients.
  • The study concluded that benralizumab is an effective and well-tolerated treatment for EGPA, recommending further large-scale research to assess its long-term efficacy and safety.
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Iron deficiency is the main cause of anemia in both sexes, with women being more commonly affected. Iron therapy is currently considered an effective and safe remedy to replenish the iron storages. Iron can be administrated both orally and intravenously.

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Background: Brentuximab vedotin (BV) is an antibody-drug conjugate formed by an anti-CD30 chimeric IgG conjugated with monomethyl-auristatin-E. BV targets the CD30 cells, which characterize Hodgkin lymphoma as well as anaplastic large cell lymphoma. Once bound to the CD30 cells BV exerts its cytotoxic effect via the monomethyl-auristatin-E moiety.

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