Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a severe multiorgan hypersensitivity reaction mostly caused by several eliciting drugs in patients with a genetic predisposition. Anti-convulsants are the main drugs involved both in adults and in children. Incidence of DRESS in children is very variable, from 1:1,000 to 1:10,000, and the mortality rate seems to be lower than 10%. The treatment of choice is the prompt withdrawn of the offending drug and using intravenous immunoglobulins and corticosteroids used in synergy. In recent years, emerging studies have outlined the disease more clearly. We present a paediatric case in which the patient developed DRESS syndrome as a result of exposure to lamotrigine before and carbamazepine after and a relapse after exposure to omeprazole. Starting from this case report we provide an overview on DRESS syndrome.

Intraosseous (IO) access offers a fast and reliable route for administration of fluids and drugs when intravenous (IV) accesses like umbilical, peripheral, or peripherally inserted central lines fail in critically ill neonates. Several medications can be successfully administered via the IO route, however only limited information is available regarding IO administration of antiviral agents.We present the case of a 2-week-old neonate, admitted to the Neonatal Intensive Care Unit (NICU) due to suspected meningitis, who received acyclovir through IO infusion after the venous access was lost and a new one could not be established. No complications were reported within 12 months of follow up.This report highlights the feasibility of IO acyclovir infusion when IV accesses fail in a critically ill neonate.

Pediatric obesity is a growing and alarming global health problem and represents an important determinant of morbidity. Since nutrition plays an important role in regulating growth and development, the excess weight gain related to overnutrition can affect growth patterns, bone maturation and pubertal development. The purpose of this review was to summarize the current knowledge about the effect of primary obesity on linear growth and pubertal development in children and adolescents. Evidence about regulatory hormones and adipokines that may be involved in the physiology of childhood growth in the context of obesity were also discussed. The most recent literature confirms previous studies indicating that linear growth is accelerated (mainly due to longer trunks rather than longer legs) and bone age is advanced in prepubertal children with obesity, while there is a reduction of pubertal height gain and attainment of normal adult height. Conflicting results are reported on the timing of puberty, specifically in boys. Indeed, previous studies suggested earlier onset of puberty in obese girls and overweight boys, and a delayed puberty in obese boys. Conversely, the most recent studies show more consistently an earlier onset and completion of pubertal development also in boys with obesity. Considering the false belief of health associated with transient taller stature in children and the adverse outcomes related to early puberty, interventions on diet and physical activity are urgently needed to tackle the epidemics of childhood obesity in public health and clinical setting.

L’iperplasia surrenalica congenita (CAH) è una malattia ereditaria autosomica recessiva dovuta a un difetto enzimatico della catena biosintetica che dal colesterolo porta alla sintesi di aldosterone, androgeni e cortisolo. Il difetto comporta un blocco totale o parziale della produzione di cortisolo e, eventualmente, anche dell’aldosterone, con conseguente ipersecrezione di CRH e ACTH che determina iperplasia della corteccia surrenalica. Il blocco enzimatico porta all’aumentata sintesi di precursori del cortisolo a monte del difetto, che prendono altre vie biosintetiche e vengono trasformati in androgeni, androstenedione e testosterone con virilizzazione ed eventuale squilibrio idroelettrolitico, ipovolemia e shock.

Background: non-autoimmune thyroid disorder is a common finding in celiac patients, more frequent than in the general population. An impairment of iodine absorption has been hypothesized, but it has never been investigated so far. We aimed to evaluate the iodine absorption in children and adolescents with newly diagnosed celiac disease. Methods: 36 consecutive celiac patients (age 7.4 years, range 2.4–14.5 years) before starting a gluten-free diet (GFD) were enrolled. We assayed the urinary iodine concentration (UIC) in a 24-h urine sample, at baseline (T0) after 3 (T1) and 12 months (T2) of GFD. Results: UIC at T0 was 64 μg/L (IQR 45–93.25 μg/L) with an iodine deficiency rate of 77.8%. UIC was not different according to histological damage, clinical presentation (typical vs atypical); we found no correlation with the thyroid function tests and auxological parameters. UIC was not statistically different at T1 (76 μg/L) and T2 (89 μg/L) vsT0. UIC at T2 was similar between patients with positive and negative anti-transglutaminase antibodies at T2. No patients presented overt hypothyroidism during the study. Conclusions: We found that iodine absorption in celiac children is impaired compared to the general population; it increases slightly, but not significantly, during the GFD. We should regularly reinforce the need for a proper iodine intake in celiac disease patients to reduce iodine deficiency risk.

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a severe multiorgan hypersensitivity reaction mostly caused by several eliciting drugs in patients with a genetic predisposition. Incidence of DRESS in children is very variable, frome 1:1000 to 1:10.000, and the mortality rate seems to be lower than 10%. Anti-convulsants are the main drugs involved both in adults and in children. The treatment of choice is the prompt withdrawn of the offending drug and using intravenous immunoglobulins and corticosteroids used in synergy. In recent years, emerging studies have outlined the disease more clearly. We present a pediatric case in which the patient developed DRESS syndrome as a result of exposure to lamotrigine before and carbamazepine after and a relapse after exposure to omeprazole. Starting from this case report we provide an overview on DRESS Syndrome.

Aim: No data are available on caffeine consumption among Italian adolescents. We investigated caffeine intake from coffee, soft drinks and energy drinks in a sample of Italian adolescents and determined if they exceeded the recommended limits. Methods: The study comprised 1213 adolescents with a mean age of 15.1 years (range 12-19) from four schools in Foggia, southern Italy. Caffeine intake was assessed using an anonymous self-reported questionnaire during the 2013/2014 school year. We calculated the percentage of daily caffeine consumers, their mean intake of caffeine from beverages and the contribution of each beverage category to the total caffeine intake. Results: Approximately 76% of the sample consumed caffeine every day, amounting to 125.5 +/- 69.2 mg/day and 2.1 +/- 1.2 mg/kg/day. When we applied the reference values from the Academy of Pediatrics, we found that 46% of the adolescents exceeded the recommended upper limits. Coffee was the most frequently consumed caffeinated drink and the main contributor to daily caffeine intake. Conclusion: More than three quarters (76%) of the Italian adolescents in our study drank coffee on a daily basis and nearly half (46%) exceeded the recommended upper limits. Strategies are needed to reduce caffeine consumption by adolescents.

Background: The adipose tissue has effect on growth hormone secretion but the metabolic correlates of circulating insulin-like growth factor-1 (IGF1) and IGF1 binding protein 3 (IGFBP-3) are unclear. Several studies have suggested that IGF1 modulates adipocyte and there is increasing evidence that IGF1 and IGFBP-3 could be related to metabolic syndrome (MS) in adults.

The aim of the replacement therapy with levothyroxine in congenital hypothyroidism (CH) is to correct hypothyroidism and ensure normal growth and neuropsychological development. Few data are available about the appropriate dose during childhood and early adolescence; therefore, we performed a multicenter observational study in a large population of patients with CH to assess the required levothyroxine dose to obtain euthyroidism. We recruited 216 patients with permanent CH classified into three groups (agenesia, ectopia, and in situ gland) on the basis of the thyroid imaging. The levothyroxine dose was recorded at 6 and 12 months and then yearly until 12 years of age. The daily levothyroxine requirement progressively decreased during the follow-up, irrespective of etiology. It was significantly lower in patients with in situ gland than in patients with athyreosis during the entire study period and with ectopic gland from the age of 1 year. The levothyroxine requirement at 6 months of age was correlated with the requirement at each later time-point. The daily dose was modified less frequently in patients with in situ thyroid (36 %) than in patients with ectopic gland (41.4 %) or with athyreosis (43.6 %). Patients with in situ gland required a lower dose than the other two subgroups. The dose at 6 months seems predictive of the requirement until 12 years of age. Euthyroidism may be achieved in pre-school and in-school patients by 3–4 and 2–3 µg/kg/day (70–90 and 60–80 µg/m2/day) of levothyroxine, respectively.

Linear growth and final height are reported as normal in congenital hypothyroid patients in the neonatal screening era.

Objective: Coeliac disease (CD) is an autoimmune disorder elicited by gluten and related prolamines in genetically susceptible individuals. The prevalence has been estimated to be 0.5–1% worldwide. For not-well known reasons, girls are at increased risk for CD compared to boys.

Energy drinks (EDs) are beverages marketed to improve energy, weight loss, attention, athletic performance, and concentration. Caffeine is the main active ingredient in EDs; many of them contain 70–80 mg of caffeine for a 33 cl serving. Heavy caffeine consumption, such as drinking EDs, has been associated with serious consequences such as seizures, mania, stroke, and sudden death. Adolescent's and child's caffeine consumption should not exceed 100 mg/day and 2.5 mg/kg/day, respectively. The daily caffeine's intake should take account of all sources of this substance (coffee, tea, soft and energy drinks). Objective: (1) To investigate the extent of energy drink consumption in adolescents; (2) to understand the reasons why teens’ consumption is widespread; (3) to estimate the amount of daily caffeine taken from coffee and beverages containing caffeine (including EDs). Methods: A self-report questionnaire related to EDs’ consumption has been developed and then proposed to 1232 students aged 12–19 years old (mean age 14.8 ± 3.8). The data have been collected and then analyzed. Results: According to our surveys, EDs are consumed by 378/1232 (30.7%) of subjects; among them 27/378 (7.1%) drink these beverages 1 time/day at least, 122/378 (32.3%) 1–2 times/week and 179/378 (47.3%) 1–2 times/month. Most of them, 341/378 (90.2%) admit to consume EDs for the flavour, 147/378 (38.9%) find them refreshing, 68/378 (18%) and 39/378 (10.3%) declare to achieve an improvement in sports performance and intellectual activity, respectively. 270/378 (71.4%) of participants declare to consume these beverages when outside, 106/378 (28%) at meals, 74/378 (19.6%) in front of TV/PC, only 39/378 (10.3%) at school and 38/378 (10%) during or after physical exercise. We have also evaluated the amount (in mg) of caffeine (from coffee and caffeinated beverages) taken daily. Among consumers of caffeine (891/1232 – 72.3%), 495/891 (55.5%) consume less than the daily maximum level allowed, 265/891 (29.7%) consume between 100 and 200 mg/day of caffeine and 131/891 (14.7%) exceed the maximum dose, consuming daily more than 200 mg and up to 300 mg/day. Conclusions: Our data show that, among a pediatric population consuming caffeine, 396/891 of subjects (44.4%) exceeded the maximum recommended dose. Pediatricians should be aware of the possible effects of EDs and screen for their consumption to educate children and their families to decrease or eliminate the inappropriate use of these beverages.

OBJECTIVE:Mitotane is an adrenocytolytic agent used in adrenocortical carcinoma, inducing adrenal insufficiency, requiring replacement treatment. Such therapy is not easy to monitor because of mitotane interference. Salivary cortisol reflects a free fraction of plasma cortisol and may be useful in such patients.DESIGN:The aim of our study was to evaluate salivary cortisol by HPLC coupled to tandem-mass spectrometry (LC-MS/MS) and by an electrochemiluminescence immunoassay (ECLIA) in patients treated with mitotane. We enrolled 6 patients receiving mitotane and 2 Addison disease patients as negative controls and determined salivary cortisol rhythm. We also determined the salivary cortisol rhythm in 8 healthy subjects. Salivary samples (n=112) were assayed by ECLIA, using Roche Modular E170, and by LC-MS/MS.RESULTS:The mean values obtained by ECLIA were significantly higher than those obtained by LC-MS/MS in the mitotane group (p<0.001). In fact, in the group measured by LC-MS/MS, we observed several peaks eluting at a retention time different from the cortisol group, presumably due to cortisol-like analogues. In Addison disease, since steroidogenesis is absent, salivary cortisol values measured by the two methods did not show any significant difference (p=0.61).CONCLUSIONS:Salivary cortisol measured by LC-MS/MS is a selective method, excluding cortisol analogues accumulating in treated patients. Therefore, LC-MS/MS offers an effective system to monitor replacement therapy in mitotane treated patients.

Background: The poor comparability of growth hormone (GH) results obtained using commercially available methods, is partly due to standard preparations used in calibration. The system relies on the use of the International Reference Preparation (IRP) international standard (IS) 80/505, of human pituitary origin, containing all GH isoforms. Recently, a 22K recombinant GH isoform IRP IS 98/574 was commercialized. Our aim was to evaluate the influence of both calibrators on GH results. Methods: GH concentration in 97 serum samples from children undergoing a growth hormone releasing hormone+arginine stimulation test was measured using Siemens IMMULITE electro-chemiluminescence method, calibrated with both IS 80/505 and IS 98/574 (GRH Growth hormone-Recombinant 98/574-kit). Results: Comparison of our results obtained with the two sets of calibrators showed good correlation, although we found higher percentage variation (var%) than that stated by Siemens. The mean var% value was confirmed when all results were sub-divided into subgroups based on both high and low GH concentrations. Conclusions: Since the GH assay is influenced by a variety of binding proteins, isoforms and conversion factors, standardization of the assay is strongly required. In Italy, the Agenzia Italiana del Farmaco 39 note provides GH laboratory values which are useful for therapy. On the basis of our results, we therefore propose to adjourn these GH values in order to ensure better management of patients with GH-related disorders.