1.
Understanding the Pathogenesis of Cardiac Complications in Patients with Propionic Acidemia and Exploring Therapeutic Alternatives for Those Who Are Not Eligible or Are Waiting for Liver Transplantation.
Maines, E, Moretti, M, Vitturi, N, Gugelmo, G, Fasan, I, Lenzini, L, Piccoli, G, Gragnaniello, V, Maiorana, A, Soffiati, M, et al
Metabolites. 2023;(4)
Abstract
The guidelines for the management of patients affected by propionic acidemia (PA) recommend standard cardiac therapy in the presence of cardiac complications. A recent revision questioned the impact of high doses of coenzyme Q10 on cardiac function in patients with cardiomyopathy (CM). Liver transplantation is a therapeutic option for several patients since it may stabilize or reverse CM. Both the patients waiting for liver transplantation and, even more, the ones not eligible for transplant programs urgently need therapies to improve cardiac function. To this aim, the identification of the pathogenetic mechanisms represents a key point. Aims: This review summarizes: (1) the current knowledge of the pathogenetic mechanisms underlying cardiac complications in PA and (2) the available and potential pharmacological options for the prevention or the treatment of cardiac complications in PA. To select articles, we searched the electronic database PubMed using the Mesh terms "propionic acidemia" OR "propionate" AND "cardiomyopathy" OR "Long QT syndrome". We selected 77 studies, enlightening 12 potential disease-specific or non-disease-specific pathogenetic mechanisms, namely: impaired substrate delivery to TCA cycle and TCA dysfunction, secondary mitochondrial electron transport chain dysfunction and oxidative stress, coenzyme Q10 deficiency, metabolic reprogramming, carnitine deficiency, cardiac excitation-contraction coupling alteration, genetics, epigenetics, microRNAs, micronutrients deficiencies, renin-angiotensin-aldosterone system activation, and increased sympathetic activation. We provide a critical discussion of the related therapeutic options. Current literature supports the involvement of multiple cellular pathways in cardiac complications of PA, indicating the growing complexity of their pathophysiology. Elucidating the mechanisms responsible for such abnormalities is essential to identify therapeutic strategies going beyond the correction of the enzymatic defect rather than engaging the dysregulated mechanisms. Although these approaches are not expected to be resolutive, they may improve the quality of life and slow the disease progression. Available pharmacological options are limited and tested in small cohorts. Indeed, a multicenter approach is mandatory to strengthen the efficacy of therapeutic options.
2.
Vegan Nutrition for Mothers and Children: Practical Tools for Healthcare Providers.
Baroni, L, Goggi, S, Battaglino, R, Berveglieri, M, Fasan, I, Filippin, D, Griffith, P, Rizzo, G, Tomasini, C, Tosatti, MA, et al
Nutrients. 2018;11(1)
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Plain language summary
Vegan diets have grown in popularity and so healthcare practitioners need to be adequately educated to be able to give advice, as if they are properly planned, they can give adequate nutrition throughout all stages of life. This review study aimed to summarise the findings of the Scientific Society for Vegetarian Nutrition (SSNV) on vegan diets throughout pregnancy, breastfeeding, infancy, and childhood to provide recommendations for healthcare professionals. The paper starts by defining a well-planned vegan diet as high in a variety of whole or minimally processed plant foods, which meets the required amount of energy. In addition, minimising vegetable fats and avoiding trans fats to not displace other nutrient-dense foods is a requirement of a vegan diet with sufficient nutrition. Adequate amounts of calcium are also needed to be a complete vegan diet and vitamin B12, and vitamin D should be obtained from alternative sources, which are lacking in plant-based diets. The paper then goes on to recommend sources and requirements of protein, fibre, omega-3 fatty acids, iron, zinc, iodine, calcium, vitamin D, and vitamin B12 in vegans during pregnancy, lactation, infancy, and childhood. Many positives of a vegan diet were outlined such as increased fibre benefitting gut bacteria and high iron intakes. It was concluded that adequately planned vegan diets can provide sufficient nutrition at all stages of pregnancy and early life and instances of malnutrition in vegans is usually due to an inappropriate diet. Healthcare professionals could use this paper to understand what defines a complete vegan diet and sources of critical nutrients to ensure that vegan clients and patients are receiving adequate nutrient amounts.
Abstract
As the number of subjects choosing vegan diets increases, healthcare providers must be prepared to give the best advice to vegan patients during all stages of life. A completely plant-based diet is suitable during pregnancy, lactation, infancy, and childhood, provided that it is well-planned. Balanced vegan diets meet energy requirements on a wide variety of plant foods and pay attention to some nutrients that may be critical, such as protein, fiber, omega-3 fatty acids, iron, zinc, iodine, calcium, vitamin D, and vitamin B12. This paper contains recommendations made by a panel of experts from the Scientific Society for Vegetarian Nutrition (SSNV) after examining the available literature concerning vegan diets during pregnancy, breastfeeding, infancy, and childhood. All healthcare professionals should follow an approach based on the available evidence in regard to the issue of vegan diets, as failing to do so may compromise the nutritional status of vegan patients in these delicate periods of life.