Publications by authors named "Ignacio Sancho Val"

Article Synopsis
  • Hydroa vacciniforme (HV) lymphoproliferative disorder is a rare type of NK/T-cell lymphoma primarily found in children, mainly reported in Latin America and parts of Asia.
  • The disorder is categorized under EBV-positive lymphoid proliferations and is classified in the fifth edition of the WHO guidelines for lymphoid neoplasms.
  • A noteworthy case of a 22-year-old woman from Spain illustrates the disease's progression, highlighting the importance of recognizing its uncommon presentation to improve diagnosis and early treatment.
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Article Synopsis
  • Enzymatic replacement therapy (ERT) has significantly improved early diagnosis and management of Gaucher disease (GD) in pediatric patients since its introduction in Spain in 1993, with 386 patients registered in the Spanish Registry of Gaucher Disease.
  • The study reviewed data from 98 pediatric patients, revealing that those diagnosed after 1995 had earlier diagnoses and were generally less severe than those diagnosed before 1994, who demonstrated worse complications at presentation.
  • Patients in the later cohort (≥1995) started ERT much sooner after diagnosis compared to earlier diagnosed patients (1.6 years vs. 5.2 years), indicating an overall improvement in treatment timing and outcomes for pediatric GD cases.
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