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Cardiometabolic and Nutritional Morbidities of a Large, Adult, PKU Cohort from Andalusia

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Unidad de Gestión Clínica de Endocrinología y Nutrición, Instituto de Biomedicina de Sevilla (IBiS), Hospital Universitario Virgen del Rocio/CSIC/Universidad de Sevilla, 41013 Sevilla, Spain
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Instituto de Investigacion Biomédica Malaga, Servicio Endocrinología y Nutrición, Hospital Regional Universitario Malaga, Universidad de Malaga, 29010 Málaga, Spain
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Unidad de Metabolopatías, Unidad de Gestión Clínica de Pediatría, Hospital Universitario Virgen del Rocio, 41013 Sevilla, Spain
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Unidad Gastroenterología Pediatrica, Servicio Pediatria, Hospital Regional Universitario Malaga, Instituto de Investigacion Biomédica Malaga, Universidad de Malaga, 29010 Málaga, Spain
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Centro de Investigación Biomedica en Red-Diabetes y Enfermedades Metabólicas Asociadas (CIBERDEM), 29010 Málaga, Spain
*
Author to whom correspondence should be addressed.
Academic Editor: Bahram H. Arjmandi
Nutrients 2022, 14(6), 1311; https://doi.org/10.3390/nu14061311
Received: 23 February 2022 / Revised: 15 March 2022 / Accepted: 17 March 2022 / Published: 21 March 2022
(This article belongs to the Section Clinical Nutrition)
The establishment of national neonatal screening systems has resulted in improved quality of life and life expectancy in patients with phenylketonuria (PKU). This has led to the development of multidisciplinary treatment units for adult patients with PKU. We present a retrospective descriptive study of a cohort of 90 adult patients (>16 years) with PKU under active follow-up in two reference centers in Andalusia. We analyzed disease severity, treatment type, demographic variables, cardiovascular risk factors, vitamin and hormone profiles, and bone metabolism. The median (interquartile range)age was 29 (23–38) years, 47 (52.2%) were women and 43 (47.8%) were men. Eighty (88.9%) had classical PKU, five (5.6%) moderate PKU, and five (5.6%) mild PKU. Diagnosis was by neonatal screening in 62 (68.9%) of the patients. The rest had late diagnosis. Treatment with sapropterin was given to 18 (20%) patients and diet and nutrition therapy to 72 (80%). There was adequate metabolic control according to Phe levels in 43 (47.78%) patients. Body mass index was 26.61 (22.7–31.1) kg/m2. Twenty-six (29.2%) patients had obesity, 7 (7.9%) hypertension, 2 (2.2%) type 2 diabetes, 26 (28.89%) dyslipidemia, 14 (15.6%) elevated total cholesterol, 9 (15.8%) decreased high-density lipoprotein cholesterol and 16 (17.8%) hypertriglyceridemia. Seven (10.3%) patients had osteoporosis and 28 (41.17%) osteopenia. Twenty-six (30.6%) had vitamin D (25OH) deficiency and four (4.5%) vitamin B12 deficiency. Although we observed no differences with most vascular risk factors, we found a high prevalence of obesity in relation to the age of the cohort. A continued evaluation of comorbidities in these patients is therefore needed, despite adequate metabolic control. View Full-Text
Keywords: phenylketonuria; sapropterin; phenylalanine phenylketonuria; sapropterin; phenylalanine
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MDPI and ACS Style

Dios-Fuentes, E.; Gonzalo Marin, M.; Remón-Ruiz, P.; Benitez Avila, R.; Bueno Delgado, M.A.; Blasco Alonso, J.; Doulatram Gamgaram, V.K.; Olveira, G.; Soto-Moreno, A.; Venegas-Moreno, E. Cardiometabolic and Nutritional Morbidities of a Large, Adult, PKU Cohort from Andalusia. Nutrients 2022, 14, 1311. https://doi.org/10.3390/nu14061311

AMA Style

Dios-Fuentes E, Gonzalo Marin M, Remón-Ruiz P, Benitez Avila R, Bueno Delgado MA, Blasco Alonso J, Doulatram Gamgaram VK, Olveira G, Soto-Moreno A, Venegas-Moreno E. Cardiometabolic and Nutritional Morbidities of a Large, Adult, PKU Cohort from Andalusia. Nutrients. 2022; 14(6):1311. https://doi.org/10.3390/nu14061311

Chicago/Turabian Style

Dios-Fuentes, Elena, Montserrat Gonzalo Marin, Pablo Remón-Ruiz, Rosa Benitez Avila, Maria A Bueno Delgado, Javier Blasco Alonso, Viyei Kishore Doulatram Gamgaram, Gabriel Olveira, Alfonso Soto-Moreno, and Eva Venegas-Moreno. 2022. "Cardiometabolic and Nutritional Morbidities of a Large, Adult, PKU Cohort from Andalusia" Nutrients 14, no. 6: 1311. https://doi.org/10.3390/nu14061311

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