Publications by authors named "L Gonzalez-Dieguez"
Lysosomal acid lipase deficiency (LAL-D) is an ultra-rare lysosomal storage disease with two distinct phenotypes, an infantile-onset form (formerly Wolman disease) and a later-onset form (formerly cholesteryl ester storage disease). The objective of this narrative review is to examine the most important aspects of the diagnosis and treatment of LAL-D and to provide practical expert recommendations. The infantile-onset form occurs in the first weeks of life and is characterized by malnourishment and failure to thrive due to gastrointestinal impairment (vomiting, diarrhea, malabsorption), as well as systemic inflammation, hepatosplenomegaly, and adrenal calcifications.
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- - Spain leads the world in deceased organ donation rates and has a well-established network of liver transplant centers; however, about 10% of patients on the liver transplant waiting list may die or deteriorate before receiving a transplant.
- - The country currently lacks a unified national system for prioritizing liver transplants, leading to varying rules among institutions and some regional practices to address urgent needs.
- - A new consensus document from the Spanish Society of Liver Transplantation (SETH) aims to standardize waiting list prioritization, enhance equity, and improve outcomes for patients with severe liver disease across Spain.
Article Synopsis
- The study investigates gender disparities in access to liver transplantation in Spain, focusing on the performance of the Gender-Equity Model adjusted by serum sodium (GEMA-Na) compared to the traditional Model for End-stage Liver Disease 3.0 (MELD 3.0).
- It includes a nationwide cohort of 6,071 patients and finds that women have lower access to transplantation and a higher risk of mortality or delisting within the first 90 days.
- GEMA-Na shows better predictive accuracy for waiting list outcomes than MELD 3.0, suggesting it could be the preferred method for prioritizing patients on the liver transplant waiting list.
Data comparing long-term effectiveness and safety of once-daily tacrolimus formulations in de novo liver transplantation are scarce. We compared the effectiveness, pharmacokinetic profile, and safety of LCPT (Envarsus) and PR-Tac (Advagraf) for up to 12 months post-transplant. Adult de novo liver transplant recipients who started IR-Tac (Prograf) and were converted to LCPT or PR-Tac 3-5 days post-transplant were included.
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