MedElevator Войти в исследования
← Поиск публикаций

DOI 10.3390/genes14010199

Медицинская библиотека · Genes · 2023

What to Expect of Feeding Abilities and Nutritional Aspects in Achondroplasia Patients: A Narrative Review.

Sforza Elisabetta, Margiotta Gaia, Giorgio Valentina, Limongelli Domenico, Proli Francesco, Kuczynska Eliza Maria, Leoni Chiara, De Rose Cristina, Trevisan Valentina, Romeo Domenico Marco, Calandrelli Rosalinda, De Corso Eugenio, Massimi Luca, Palmacci Osvaldo, Rigante Donato, Zampino Giuseppe, Onesimo Roberta

СтатьяОбзорПеревод в очереди

Аннотация

Achondroplasia is an autosomal dominant genetic disease representing the most common form of human skeletal dysplasia: almost all individuals with achondroplasia have identifiable mutations in the fibroblast growth factor receptor type 3 (FGFR3) gene. The cardinal features of this condition and its inheritance have been well-established, but the occurrence of feeding and nutritional complications has received little prominence. In infancy, the presence of floppiness and neurological injury due to foramen magnum stenosis may impair the feeding function of a newborn with achondroplasia. Along with growth, the optimal development of feeding skills may be affected by variable interactions between midface hypoplasia, sleep apnea disturbance, and structural anomalies. Anterior open bite, prognathic mandible, retrognathic maxilla, and relative macroglossia may adversely impact masticatory and respiratory functions. Independence during mealtimes in achondroplasia is usually achieved later than peers. Early supervision of nutritional intake should proceed into adolescence and adulthood because of the increased risk of obesity and respiratory problems and their resulting sequelae. Due to the multisystem involvement, oral motor dysfunction, nutrition, and gastrointestinal issues require special attention and personalized management to facilitate optimal outcomes, especially because of the novel therapeutic options in achondroplasia, which could alter the progression of this rare disease.

Для цитирования: Sforza Elisabetta, Margiotta Gaia, Giorgio Valentina, Limongelli Domenico, Proli Francesco, Kuczynska Eliza Maria, Leoni Chiara, De Rose Cristina, Trevisan Valentina, Romeo Domenico Marco, Calandrelli Rosalinda, De Corso Eugenio, Massimi Luca, Palmacci Osvaldo, Rigante Donato, Zampino Giuseppe, Onesimo Roberta. What to Expect of Feeding Abilities and Nutritional Aspects in Achondroplasia Patients: A Narrative Review.. Genes. 2023. DOI: 10.3390/genes14010199.

Предметные рубрики MeSH

Подготовка и перевод публикации
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…

История · было → стало

Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.

Тип публикации указан по данным источника. Уровень доказательности автоматически не присваивается.