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Klinefelter syndrome is the most common sex chromosomal aneuploidy in males. It is well known that patients with this syndrome have greater mortality and morbidity compared to the general population due to cardiovascular diseases and endocrine metabolism disorders. This augmented risk is due both to hypogonadism and to the syndrome itself. Therefore, correct hormonal replacement therapy and early primary prevention are crucial to these patients. Even though different studies are available on this topic in adult patients, only a few authors have focused on the paediatric population. Thus, in this narrative review, we report the current knowledge of metabolic and nutritional aspects in children with Klinefelter syndrome.

Type 1 diabetes (T1D) is one of the most common systemic diseases in childhood which predisposes the patient to serious short-term and long-term complications, affecting all body systems. Taste and olfactory impairments were first described a long time ago in adult patients affected by diabetes (both type 1 and type 2 diabetes). However, studies evaluating taste perception, behavioral attitudes (e.g., food neophobia), and preferences toward foods in children and adolescents affected by T1D are globally lacking. Therefore, the purpose of this study was to assess taste sensitivity, food neophobia, and preferences among children and adolescents affected by T1D and healthy controls in a cross-sectional study. T1D patients presented a significantly lower ability in general to correctly identify taste qualities, especially bitter and sour tastes. Moreover, they were characterized by fewer fungiform papillae compared to controls, as well as a lower responsiveness to the bitter compound 6-n-propylthiouracil (PROP). There were no significant differences in food neophobia scores between the two groups, but differences were observed in the mean hedonic ratings for some product categories investigated. Diabetic patients showed a greater liking for certain type of foods generally characterized by sourness and bitterness, an observation probably linked to their impaired ability to perceive taste stimuli, e.g., sourness and bitterness. These results may help to enhance the understanding of these relationships in populations with elevated diet-related health risks.

Neurologically impaired (NI) children are at increased risk of malnutrition due to several nutritional and non-nutritional factors. Among the nutritional factors, insufficient dietary intake as a consequence of feeding difficulties is one of the main issues. Feeding problems are frequently secondary to oropharyngeal dysphagia, which usually correlates with the severity of motor impairment and presents in around 90% of preschool children with cerebral palsy (CP) during the first year of life. Other nutritional factors are represented by excessive nutrient losses, often subsequent to gastroesophageal reflux and altered energy metabolism. Among the non-nutritional factors, the type and severity of neurological impairment, ambulatory status, the degree of cognitive impairment, and use of entiepileptic medication altogether concur to determination of nutritional status. With the present review, the current literature is discussed and a practical approach for nutritional assessment in NI children is proposed. Early identification and intervention of nutritional issues of NI children with a multidisciplinary approach is crucial to improve the overall health and quality of life of these complex children.

We performed a cross-sectional study to compare the intake of energy, macronutrients, fiber, sodium and iron and the anthropometric status of infants and toddlers living in North (Milano) and South Italy (Catania). Nutrient intake was evaluated using a 7-day weighed food record. Out of 400 planned children aged 6 to 36 months, 390 (98%) were recruited, 189 in Milano and 201 in Catania. The mean (standard deviation) age was 17 (9) months in Milano and 17 (10) months in Catania. Anthropometry, energy intake and macronutrient intake were similar in Milano and Catania. However, iron intake was 27% lower and fiber intake 16% higher in Milano than in Catania. Despite normal anthropometry and energy intake, in the pooled sample there was a high intake of proteins, simple carbohydrates, saturated fats and sodium, and a low intake of iron and fiber compared to Italian reference values. This is the first study to report the macro- and micro-nutrient intake of children aged <12 months using the 7-day weighed food record and one of the very few studies that have employed such reference method in children from the general population.

Gluten-free diet (GFD) is the cornerstone treatment for celiac disease (CD). GFD implies a strict and lifelong elimination from the diet of gluten, the storage protein found in wheat, barley, rye and hybrids of these grains, such as kamut and triticale. The absence of gluten in natural and processed foods, despite being the key aspect of GFD, may lead to nutritional consequences, such as deficits and imbalances. The nutritional adequacy of GFD is particularly important in children, this the age being of maximal energy and nutrient requirements for growth, development and activity. In recent years, attention has focused on the nutritional quality of gluten-free products (GFPs) available in the market. It is well recognized that GFPs are considered of lower quality and poorer nutritional value compared to the gluten-containing counterparts. The present review focuses on the nutritional adequacy of GFD at the pediatric age, with the aim being to increase awareness of the potential complications associated with this diet, to identify strategies in order to avoid them and to promote a healthier diet and lifestyle in children with CD.