Examinando por Autor "Luna Ovalle, Paula"
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Ítem Cognitive functioning in adults with phenylketonuria in a cohort of Spanish patients(Hindawi Limited, 2023) Luna Ovalle, Paula; López Paz, Juan Francisco; García Martín, Maitane; Amayra Caro, Imanol; Martínez, Óscar; Pérez Álvarez, Manuel; Rodríguez Bermejo, Alicia Aurora; Pérez Núñez, Paula; Ceberio, I.; Mansilla, Noelia; Soria, CristinaThe early introduction of a low phenylalanine (Phe) diet has been demonstrated to be the most successful treatment in subjects with phenylketonuria (PKU), especially for preventing severe cognitive and neurological damages. However, it still concerns that even if treated in the first months of life with supplements and following a diet, they can show slight scores below people without PKU in neuropsychological assignments. We investigated 20 adults with classical PKU aged 19-48 years (mean age 29 years) and 20 heathy controls matched by age, gender, and years of education. Patients and controls were assessed with an extended neuropsychological battery, as well as psychological aspects and quality of life, also the last Phe level result was obtained. Results showed that the most affected cognitive domains are processing speed, executive functioning, memory, and also theory of mind, but very well-preserved verbal fluency, language, and visuospatial functioning. In quality of life, some significant results were seen specially in anxiety of Phe levels, anxiety of Phe levels during pregnancy, guilt if poor adherence to supplements, and if dietary protein restriction not followed. No significant results were obtained for the psychological variables. In conclusion, it has been shown that a combination of a low Phe diet, supplement intake, and keeping Phe levels in a low range seems appropriate to have the most normal and alike cognitive performance to persons without PKU.Ítem Effects of teleassistance on the quality of life of people with rare neuromuscular diseases according to their degree of disability(Frontiers Media S.A., 2021-03) Martínez, Óscar; Amayra Caro, Imanol; López Paz, Juan Francisco; Lázaro Pérez, Esther; Caballero, Patricia; García Urquiza, Irune; Rodríguez Bermejo, Alicia Aurora; García Martín, Maitane; Luna Ovalle, Paula; Pérez Núñez, Paula; Barrera, Jaume; Passi, Nicole; Berrocoso Cascallana, Sarah; Pérez Álvarez, Manuel; Al-Rashaida, Mohammad Ali HusseinRare neuromuscular diseases (RNMDs) are a group of pathologies characterized by a progressive loss of muscular strength, atrophy, fatigue, and other muscle-related symptoms, which affect quality of life (QoL) levels. The low prevalence, high geographical dispersion and disability of these individuals involve difficulties in accessing health and social care services. Teleassistance is presented as a useful tool to perform psychosocial interventions in these situations. The main aim of this research is to assess the effects of a teleassistance psychosocial program on the QoL levels of people with RNMDs who have different levels of disability. A sample of 73 participants was divided into an experimental group (n = 40), which participated in the intervention, and a control wait list group (n = 33). QoL was evaluated through the SIP and the SF-36, and disability through the WHO-DAS II. The participants with a moderate to severe level of disability were those who most benefited from the intervention. The results also revealed that the psychosocial teleassistance program was suitable to improve physical and psychosocial aspects of people suffering from a rare neuromuscular disease with a moderate level of disability, but just psychosocial aspects in those with a severe level of disability.