Publications by authors named "Isabel Badell Serra"
Chronic hypogammaglobulinemia after allogeneic stem cell transplantation and their treatment with subcutaneous immunoglobulin in pediatric patients.
An Pediatr (Engl Ed)
August 2022
Article Synopsis
- Hypogammaglobulinemia is common in pediatric patients following allogeneic hematopoietic stem cell transplantation (HSCT), often requiring immunoglobulin replacement therapy to prevent infections, especially when it persists over time.
- A study from 2008-2019 evaluated pediatric patients with persistent chronic hypogammaglobulinemia, analyzing the transition from intravenous immunoglobulin (Privigen®) to subcutaneous immunoglobulin (Hizentra®) and its effects on patients’ quality of life.
- The findings indicate that chronic hypogammaglobulinemia results from various factors, and while the study had a small sample size, switching to Hizentra 20% significantly improved the patients
Experience of the Spanish Group for Hematopoietic Transplantation (GETMON-GETH) in allogenic Hematopoietic stem cell Transplantation in Philadelphia acute lymphoblastic leukemia.
An Pediatr (Engl Ed)
April 2022
Introduction: Outcomes in patients diagnosed of acute lymphoblastic leukemia with Philadelphia chromosome (Ph-ALL) remains unfavourable compared to other subtypes of acute lymphoblastic leukemia despite improvements in drug treatments as well as advances in hematopoietic stem cell transplantation (HSCT).
Patients And Methods: The role of allogeneic HSCT in Ph-ALL patients has been analysed through a multicentric study where data belonging to 70 patients diagnosed of this entity in different centers that received HSCT between years 1998 and 2014, were reported by the Grupo Español de Trasplante Hematopoyético (GETH).
Results: The performance of HSCT from year 2004, in first complete remission (CR) status with thymoglobulin (ATG) based conditioning had a favorable impact on overall survival (OS).
[Experience of the Spanish Group for Hematopoietic Transplantation (GETMON-GETH) in allogenic hematopoietic stem cell transplantation in Philadelphia acute lymphoblastic leukemia].
An Pediatr (Engl Ed)
March 2021
Introduction: Outcomes in patients diagnosed of acute lymphoblastic leukemia with Philadelphia chromosome (Ph-ALL) remains unfavourable compared to other subtypes of acute lymphoblastic leukemia despite improvements in drug treatments as well as advances in hematopoietic stem cell transplantation (HSCT).
Patients And Methods: The role of allogeneic HSCT in Ph-ALL patients has been analysed through a multicentric study where data belonging to 70 patients diagnosed of this entity in different center that received HSCT between years 1998 and 2014, were reported by the Grupo Español de Trasplante Hematopoyético (GETH).
Results: The performance of HSCT from year 2004, in first complete remission (CR) status with thymoglobulin (ATG) based conditioning had a favorable impact on overall survival (OS).
Primary immunodeficiencies (PIDs) are rare, undiagnosed and potentially fatal diseases. Clinical manifestations of PID can be fatal or leave sequelae that worsen the quality of life of patients. Traditionally, the treatment of PIDs has been largely supportive, with the exception of bone marrow transplantation and, more recently, gene therapy.
View Article and Find Full Text PDFExecutive Summary of the Consensus Document on the Diagnosis and Management of Patients with Primary Immunodeficiencies.
Enferm Infecc Microbiol Clin (Engl Ed)
November 2020
Primary immunodeficiencies (PIDs) are rare, undiagnosed and potentially fatal diseases. Clinical manifestations of PID can be fatal or leave sequelae that worsen the quality of life of patients. Traditionally, the treatment of PIDs has been largely supportive, with the exception of bone marrow transplantation and, more recently, gene therapy.
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- Telomeres are essential structures at the ends of chromosomes, and mutations affecting their maintenance can lead to various disorders known as telomeropathies, with unclear genetic origins for many patients.
- The study examined 47 patients with significantly shortened telomeres who had different telomeropathies and found known and potential pathogenic variants in key telomere-related genes, as well as rare variants in genes linked to DNA repair.
- The research suggests that new variants in both telomere maintenance and DNA repair genes could contribute to the development of telomeropathies, and those with variants in telomere-related genes had a poorer prognosis after diagnosis.
We report data obtained from a retrospective multicenter pediatric survey on behalf of the European Society for Blood and Marrow Transplantation (EBMT). Information on solid organ transplantation (SOT) performed in pediatric recipients of either autologous or allogeneic hematopoietic stem cell transplantation (HSCT) between 1984 and 2016 was collected in 20 pediatric EBMT Centers (25.6%).
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