Pulmonary function in Williams-Beuren syndrome: Spirometric data of 22 Italian patients.

Williams-Beuren syndrome (WBS) is caused by an haploinsufficiency of the 7q11.2 region which involves the elastin gene (ELN). A deficiency of elastin is a known pathophysiological mechanism of emphysema/chronic obstructive pulmonary disease (COPD). A previous study hypothesized a higher risk of COPD in WBS patients. Herein, this phenomenon was further investigated looking for a possible correlation between COPD and WBS. Dynamic lung volumes (forced vital capacity [FVC], FEV1, FEV1/FVC) were measured in 22 patients (age range 18.9 ± 7.4 years) affected with WBS, genetically confirmed, correlating these parameters to respiratory risk factors. Dyspnea, cough and wheezing were detected in 6/22 (27%) patients. Obstructive and restrictive patterns were identified in 6/22 (27%) and 2/22 (9%) cases, respectively with no evidence of irreversible obstruction. CVF, FEV1 and FEV1/CVF mean values were all normal, with values of 91.3% (n.v. > 80%), 84.2% (n.v. > 80%) and 0.82 (n.v. > 0.7), respectively. The severity of the comorbidities did not show a cause-effect relation with the respiratory patterns, nevertheless patients treated with anti-hypertensive drugs had poorer pulmonary function. Our findings are in accordance with previous observations, showing that emphysema/COPD is not a typical finding in young patients with WBS. However, a respiratory function assessment should be included in the follow-up of WBS patients, especially in adolescents/young adults under treatment with anti-hypertensive drugs.

Vitamin D Deficiency and Glycemic Status in Children and Adolescents with Type 1 Diabetes Mellitus.

BACKGROUND: Vitamin D (25OHD) effects on glycemic control are unclear in children and adolescents with type 1 diabetes. Aims of this study were to investigate 25OHD status among children with T1DM and its relationship with insulin sensitivity and glycemic status. SUBJECTS AND METHODS: A cross sectional study was carried out between 2008-2014. A total of 141 patients had a T1DM >12 months diagnosis and were enrolled in the present study. Of these 35 (24.8%) were migrants and 106 (75.2%) Italians (T2). We retrospectively analyzed data at the onset of the disease (T0)(64 subjects) and 12-24 months before the last visit (T1,124 subjects). Fasting glucose, glycated hemoglobin (HbA1c), 25OHD levels and daily insulin requirement were evaluated and Cholecalciferol 1000 IU/day supplementation for the management of vitamin D insufficiency (<75 nmol/L) was systematically added. RESULTS: A generalized 25OHD insufficiency was found at each study time, particularly in migrants. At T0, the 25OHD levels were inversely related to diabetic keto-acidosis (DKA) severity (p<0.05). At T1 and T2, subjects with 25OHD ≤25nmol/L (10 ng/mL) showed higher daily insulin requirement (p<0.05) and HbA1c values (p<0.01) than others vitamin D status. The 25OHD levels were negatively related with HbA1c (p<0.001) and daily insulin dose (p<0.05) during follow up. There was a significant difference in 25OHD (p<0.01) between subjects with different metabolic control (HbA1c <7.5%,7.5-8%,>8%), both at T1 and T2. In supplemented subjects, we found a significant increase in 25OHD levels (p<0.0001) and decrease of HbA1c (p<0.001) between T1 and T2, but this was not significant in the migrants subgroup. Multivariate regression analysis showed a link between HbA1c and 25OHD levels (p<0.001). CONCLUSIONS: Children with T1DM show a generalized 25OHD deficiency that impact on metabolic status and glycemic homeostasis. Vitamin D supplementation improves glycemic control and should be considered as an additional therapy.

Pediatric emergency room activities in Italy: a national survey.

BACKGROUND: In Italy, the number of accesses to the Emergency Units has been growing for the past 30 years. This, together with a low coordination between hospital and peripheral pediatric services, has brought to an unnecessarily high number of hospital admissions. For this reason, it is essential to plan and implement strategies able to improve the appropriateness of hospital admissions. In the '90s, the Short Stay Observation was extended to pediatric patients. As highlighted by the report "Guidelines for Pediatric Observation Units" (2005), patients receive considerable benefits from a short hospital permanence. The purpose of the study is to report data about the Pediatric Emergency Room activities in Italy. METHODS: In 2011, the Italian Society of Pediatrics promoted an online data collection to investigate organization and activity of Italian Pediatric and Neonatal Units. A form, containing 140 questions, was sent to 624 Pediatric and Neonatology Units. This study will be focused only on data regarding pediatric Emergency Rooms (E.R.) and Observation Units. RESULTS: 237 units replied, 183 if we focus on units with pediatric inpatient service. Based on the results, E.R Units were provided with a dedicated pediatrician in 56 % of the cases: of these, 85 % for 24 h. The majority of the patients were seen by a pediatrician. In only 8 % of the units, patients visited by a pediatrician were less than 40 %. The age limit was 14 years in 60 % of the cases. In 72 % of participating units a E.R. triage was carried out. Only 18 % of units registered more than 10000 E.R. visits/year. The percentage of children hospitalized after accessing the E.R. was significantly higher in southern regions (more than 20 % of the units hospitalized more than 40 % of children entering the E.R.). 66 % of the units were provided with an Observation Unit. In 61 % of the cases, the duration did not exceed 24 h. In more than half of the structures, less than 10 % of the E.R. visits went into observation. The type of remuneration was not homogeneous. CONCLUSIONS: The study highlights the heterogeneity of the Italian reality, with great possibilities for improvement, especially in southern regions.

Performance of non-contact infrared thermometer for detecting febrile children in hospital and ambulatory settings.

AIMS: To assess the performance of the non-contact infrared thermometer compared with mercury-in-glass thermometer in children; to assess the diagnostic accuracy of non-contact infrared thermometer for detecting children with fever; to compare the discomfort caused by the two procedures in children aged > one month. BACKGROUND: Non-contact infrared thermometer is a quick and non-invasive method to measure body temperature, not requiring sterilisation or disposables. It is a candidate for temperature recording in children. DESIGN: Prospective multicenter study. METHODS: Body temperature readings were taken from every child consecutively admitted to the Pediatric Emergency Departments or Pediatric Clinics participating in the study. Two bilateral axillary temperature measurements using the mercury-in-glass thermometers and three mid-forehead temperature measurements using the non-contact infrared thermometer were performed. RESULTS: Two hundred and fifty-one children were enrolled in the study. Mean body temperature obtained by mercury-in-glass thermometer and non-contact infrared thermometer was 37.18 (SD 0.96) °C and 37.30 (SD 0.92) °C, respectively (p = 0.153). Non-contact infrared thermometer clinical repeatability was 0.108 (SD 0.095) °C, similar to that of the mercury-in-glass thermometer (0.11 SD 01 °C; p = 0.517). Bias was 0.0150 (SD 0.09) °C. The proportion of outliers >1 °C was 4/251 children (1.59%). A significant correlation between temperature values obtained with the two procedures was observed (r(2) = 0.84; p < 0.0001). The limits of agreement, by the Bland and Altman method, were -0.62 (95% CI: -0.47 to -0.67) and 0.76 (95% CI: 0.61-0.91). No significant correlation was evidenced between the difference of the body temperature values recorded by the two methods and age (p = 0.226), or room temperature (p = 0.756). Calculating the receiver operating characteristic curve to determine the best threshold for axillary temperature >38.0 °C, for a non-contact infrared thermometer temperature = 37.98 °C the sensitivity was 88.7% and the specificity 89.9%. Mean distress score (on a 5-point scale) was significantly lower using the non-contact infrared thermometer than using the mercury-in-glass thermometer (1.92 SD 0.56 and 2.40 SD0.93, respectively; p < 0.0001). CONCLUSION: Non-contact infrared thermometer showed a good performance in our study population, has the advantage of measuring body temperature in two seconds and is comfortable for children. RELEVANCE TO CLINICAL PRACTICE: Non-contact infrared thermometer may be taken into consideration when assessing body temperature in children aged > one month in hospital or ambulatory.

Efficacy of injectable trivalent virosomal-adjuvanted inactivated influenza vaccine in preventing acute otitis media in children with recurrent complicated or noncomplicated acute otitis media.

BACKGROUND: Most cases of acute otitis media (AOM) follow an upper respiratory infection due to viruses, including influenza viruses. As effective and safe influenza vaccines are available, their use has been considered among the possible measures of AOM prophylaxis. OBJECTIVES: To evaluate the efficacy of an inactivated virosomal-adjuvanted influenza vaccine in preventing AOM in children with a history of noncomplicated recurrent AOM (rAOM) or rAOM complicated by spontaneous perforation. METHODS: In this prospective, randomized, single-blinded, placebo-controlled study, 180 children aged 1 to 5 years with a history of rAOM and previously unvaccinated against influenza were randomized to receive the inactivated virosomal-adjuvanted subunit influenza vaccine (n = 90) or no treatment (n = 90), and AOM-related morbidity was monitored every 4 to 6 weeks for 6 months. RESULTS: The number of children experiencing at least 1 AOM episode was significantly smaller in the vaccinated group (P < 0.001), as was the mean number of AOM episodes (P = 0.03), the mean number of AOM episodes without perforation (P < 0.001), and the mean number of antibiotic courses (P < 0.001); the mean duration of bilateral OME was significantly shorter (P = 0.03). The only factor that seemed to be associated with the significantly greater efficacy of influenza vaccine in preventing AOM was the absence of a history of recurrent perforation (crude odds ratio, P = 0.01; adjusted odds ratio, P = 0.006). CONCLUSIONS: The intramuscular administration of injectable trivalent inactivated virosomal-adjuvanted influenza vaccine in children with a history of rAOM significantly reduces AOM-related morbidity. However, the efficacy of this preventive measure seems to be reduced in children with rAOM associated with repeated tympanic membrane perforation.