Publication
Adaptation of Pediatric Palliative Care Groups to Metabolic Diseases: Beyond the Pathways
Spolador G, Polastrini R, Zoboli I, Henrique A, Freitas E, do Nascimento A, Monti F, Pugliese C, Kok F, Barbosa S, Bueno C · Annals of Pediatrics · 2025
The Palliative Care (PC) aims to control suffering in the physical, psychological, spiritual and social spheres. The growing number of studies that emphasize the importance of PC in the field of Inborn Metabolic Diseases (IMD) reinforce the substantial frequency of these conditions in the palliative setting. However, the representation of IMD in PC is restricted to a single group of complex chronic conditions, which does not reach the complexity of these diseases. From the four pre-defined groups in pediatric palliative care, the IMD corresponding to the clinical courses were adapted. In group 1, conditions such as organic aciduria and urea cycle defects; in group 2 fatty acid oxidation disorders and MCT/GLUT; group 3 includes lysosomal, peroxisomal and CDGs; in group 4 are amino acids synthesis defects, metabolite repair, cellular processing and trafficking and mitochondrial disorders. These groups are not static, and conditions may behave with characteristics of one of them at some point in life. The proposal enables the health professionals who care for people with metabolic diseases to recognize potential patients who can benefit from palliative care.
Synced from the TRENDS in Pediatric Palliative Care Zotero library, curated by The Siden Research Team. ICPCN does not host or verify the full text.