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Tube feeding is a life-saving treatment for children with neurological disabilities (ND), who often suffer from malnutrition and feeding disorders. Nonetheless, it is still not widely used. Our aim was to evaluate the outcomes of exclusive tube feeding in a cohort of ND children. All consecutive ND children who started tube feeding at our center within the last 5 years were included in this retrospective study. Weight-for-age, body mass index (BMI), mid-upper arm circumference (MUAC) Z-scores, and symptoms were collected at baseline (V0), 6 (V1), and 12 months (V2) after gastrostomy placement. Fifty children (62% males) were included. The ND-underlying disease was genetic (n = 29, 58%), hypoxic-ischemic encephalopathy (n = 17, 34%), or metabolic (n = 4, 8%). Indications for tube feeding were malnutrition (n = 35, 70%), recurrent respiratory infections (n = 11, 22%), or both (n = 4, 8%). Enteral formulae were polymeric (n = 29, 58%), semi-elemental (n = 17, 34%), hypercaloric (n = 3, 6%), or elemental (n = 1, 2%). Homemade blended feed was offered to three children (6%) in addition to the formula. Weight and BMI increased over the study period. Except for constipation, all symptoms (cough, vomiting, and diarrhea) improved at 6 and 12 months (p < 0.05). Non-serious complications (n = 8; track disruption, granuloma, and skin infection) were observed. Longer disease duration (p < 0.001) at the start of tube feeding was associated with the absence of normalization of nutritional status (BMI Z-score > 2 SD) at 12 months. Tube feeding with commercially available enteral formulae should be started as early as possible for better outcomes.

What is known and objective The off-label use of vedolizumab (VDZ) for inflammatory bowel disease in children is increasing. We report on possibly the first case of VDZ-associated pulmonary manifestations in paediatrics. Case summary This report details the case of a 13-year-old child with ulcerative colitis who was initiated on VDZ due to persistent active disease. After the first three doses, he developed a persistent and productive cough. Microbiological work-up was normal. VDZ discontinuation led to the resolution of symptoms. What is new and conclusion To our knowledge, this is the first case report of VDZ-associated pulmonary manifestations in paediatrics. A direct, pro-inflammatory effect of VDZ has been hypothesized, but further studies are warranted.

SummaryObjective Cadmium (Cd) has been shown to impair pubertal development in experimental animals. However, no data are available for male adolescents with increased urinary cadmium levels. Design The aim of this cross-sectional study was to evaluate pubertal onset and pituitary–gonadal axis hormones in male adolescents with increased urinary levels of Cd. Subjects We studied 111 males, aged 12–14 years living in the Milazzo-Valle del Mela area. A control age-matched population (n = 60) living 28–45 km far from the industrial site was also enrolled. Measurements Pubertal stages were assessed by clinical examination according to Tanner's score. Mean testicular volume was also investigated by ultrasound examination. Urinary Cd concentration and blood levels of FSH, LH, testosterone and inhibin B were also investigated. Results Cd levels were significantly higher in adolescents living in the Milazzo-Valle del Mela area, compared to both age-matched subjects living far from the industrial plants and the reference values. Our population showed also a delayed onset of puberty, a smaller testicular volume and lower testosterone levels. An inverse correlation was found between urinary Cd and testicular volume (r = −0·25; P = 0·0008), testosterone levels (Spearman's r = −0·0·37; two-tailed P

Background: Numerous studies have shown that GH, in addition to promoting linear growth, exerts a key role in many metabolic processes. However, there are only few studies aiming at evaluating the metabolic panel of children with GH deficiency (GHD). The aims of the study were: to verify the presence of metabolic alterations in GHD children in comparison with age-matched controls and to check the possible effects of two year GH therapy on the metabolic parameters in GHD. Study design: cross-sectional and prospective; one center experience Population and Methods: We enrolled 32 pediatric GHD patients (group A) and 33 sex- and age-matched healthy controls (group B). Baseline serum assays (lipid, insulin, glucose) were performed in both groups. GHD children underwent replacement therapy with GH. The same assays were repeated after 12 and 24 months of GH treatment. Results: No differences, in basal assays were found between the two groups. In group A, after initiation of GH, there was a significant increase of basal insulin and HOMA- insulin resistance (IR) index (p

Rapid-onset Obesity with Hypothalamic Dysfunction, Hypoventilation, and Autonomic Dysregulation (ROHHAD) is a rare and complex pediatric disorder . Children typically show ROHHAD after the first years of life with rapid weight gain and subsequently autonomic nervous system dysregulation (altered pain perception, pupillary dysfunction, hypothermia and bradycardia); alveolar hypoventilation with risk of cardiorespiratory arrest and hypothalamic dysfunction (central diabetes insipidus, hypothyroidism, growth hormone and corticotrophin deficiency). Tumours of neural crest origin, such as ganglioneuroblastoma and ganglioneuronoma, are reported in 33% of the patients and may be found in the chest or abdomen. Here we describe two girls who presented with rapid weight gain, at the age of 5 and 9 years respectively. The first was admitted due to obesity and central hypothyroidism. After two months she rapidly developed a clinical picture characterized by thermal dysregulation, hypodipsia and severe hypernatriemia, hypertrigliceridemia, alveolar hypoventilation supported by mechanical ventilation. The second presented with rapid-onset obesity and a mild hyperprolactinemia. After three months of follow- up she was admitted due to a clinical picture of hypothermia, seizures and hyponatremia. Subsequentely she developed altered water balance (severe hypernatremia) and severe hypoventilation . Chest CT and MR imaging showed a posterior mediastinal mass. Endocrinological investigation showed corticotrophin deficiency and central hypothyroidism treated with specific replacement therapies. Conclusions: On the basis of our experiences we can infer that it is necessary perform specific further investigations of hypothalamic function in all the children with rapid onset obesity in order to early prevent the catastrophic consequences that may occur in this syndrome.

Background: The area of Milazzo-Valle del Mela (Sicily, Italy) is considered at high risk of environmental crisis by regional authorities. Objective: To measure oxidative-stress, DNA repair and detoxification genes in school children living near the industrial area and in age-matched controls. Methods: The parent study was a biomonitoring investigation evaluating heavy metal urine levels in 226 children aged 12–14 years, living in the high risk area, and in 29 age-matched controls living 45 km far from the industrial site. In the present study 67 exposed adolescents and 29 controls were included. Samples were analyzed for urinary 8-hydroxydeoxyguanosine (8OHdG) levels, and gene expression of OGG1 (DNA repair gene), NQO1, ST13, and MT1A (detoxifying genes). Results: Urinary cadmium was higher (p = 0.0004) in exposed [geometric mean, 0.46 µg/L; 25th–75th percentile: 0.3–0.56] than in control adolescents [geometric mean, 0.26 µg/L; 25th–75th percentile: 0.2–0.3]. Chromium was also significantly elevated in exposed [geometric mean, 1.52 µg/L; 25th–75th percentile: 1.19–1.93] compared with controls [geometric mean, 1.25 µg/L; 25th–75th percentile: 1.05–1.48; p = 0.02]. Urinary 8-OHdG concentration was greater in exposed than in controls (71.49 vs 61.87 µg/L, p = 0.02), and it was correlated with cadmium levels (r = 0.46, p