Publications by authors named "E Fagundes"
Early death and intracranial hemorrhage prediction in acute promyelocytic leukemia: validation of a risk score in a cohort from an international consortium treated with chemotherapy plus all- retinoic acid.
Haematologica
March 2025
Background: Hepatopulmonary syndrome (HPS) is characterized by the triad of abnormal arterial oxygenation caused by intrapulmonary vascular dilatations (IPVD) in the setting of advanced liver disease or portal hypertension, impacting the patient's quality of life and survival. There are still many gaps in the literature on this topic, especially in pediatrics, with practices frequently based on extrapolation of data obtained from adults.
Objective: Provide a synthesis of the current knowledge about HPS in children.
Article Synopsis
- The combination of all-trans retinoic acid and anthracyclines has greatly improved acute promyelocytic leukemia (APL) treatment outcomes, particularly in areas without access to arsenic trioxide.
- Despite these advances, low- and middle-income countries (LMIC) still struggle with high mortality rates during treatment due to inadequate management of complications.
- A study involving 806 APL patients across several South American countries showed a reduction in induction mortality to 14.6% and an 81% overall survival rate over four years, demonstrating the benefits of international clinical collaboration.
Article Synopsis
- Non-alcoholic fatty liver disease (NAFLD), now often referred to as metabolic dysfunction-associated fatty liver disease (MAFLD), is prevalent among children and teens with obesity and is linked to metabolic syndrome factors like insulin resistance.
- A consensus of 65 international experts was reached through surveys to create recommendations covering various aspects of pediatric MAFLD, including its causes, epidemiology, and treatment strategies.
- The final consensus aims to enhance clinical outcomes and life quality for affected youth, highlighting the importance of standardized diagnosis and treatment methods.
Background: Alpha 1-antitrypsin deficiency (AATD) is a hereditary codominant autosomal disease. This liver disease ranges from asymptomatic cases to terminal illness, which makes early recognition and diagnosis challenging. It is the main cause of pediatric liver transplantation after biliary atresia.
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