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Triulzi F.,Vittore Buzzi Childrens Hospital
International journal of immunopathology and pharmacology | Year: 2010

The vast majority of nasal and paranasal sinuses (PNS) diseases in childhood are of inflammatory origin. In particular, during the early rapid development of PNS rhinosinusitis are exceedingly frequent in both acute and chronic forms. However other diseases such as primarily congenital malformation, but also neoplasms can affect the nasal cavity (NC) and PNS during pediatric age. Modern radiological techniques such as multidetector computerized tomography (MDCT) or magnetic resonance imaging (MRI) are becoming of greater importance in the diagnosis of chronic or complicated inflammatory processes of NC and PNS and in the differential diagnosis with the other less frequent diseases. The characteristic features of MDCT and MRI and their diagnostic role in the more frequent pediatric inflammatory, neoplastic and congenital diseases of NC and PNS are reviewed.

Racca F.,Pediatric Anesthesiology and Intensive Care Unit | Mongini T.,University of Turin | Wolfler A.,Vittore Buzzi Childrens Hospital | Vianello A.,University of Padua | And 10 more authors.
Minerva Anestesiologica | Year: 2013

Patients with neuromuscular disorders are at high risk of intraoperative and postoperative complications. General anesthesia in these patients may exacerbate respiratory and cardiovascular failure due to a marked sensitivity to several anesthetic drugs. Moreover, succinylcholine and halogenated agents can trigger life-threatening reactions, such as malignant hyperthermia, rhabdomyolysis and severe hyperkalemia. Therefore, regional anesthesia should be used whenever possible. If general anesthesia is unavoidable, special precautions must be taken. In particular, for patients at increased risk of respiratory complications (i.e., postoperative atelectasis, acute respiratory failure, nosocomial infections), noninvasive ventilation associated with aggressive airway clearance techniques can successfully treat upper airway obstruction, hypoventilation and airway secretion retention, avoiding prolonged intubation and tracheotomy. Anesthesia and perioperative management of patients with neuromuscular disorders are described in this article. To grade the strength of recommendations and the quality of evidence we adopted the GRADE approach. In case of low-quality evidence, these recommendations represent the collective opinion of the expert panel.

Leo G.,Pediatric Allergy and Respiratory Pathophysiology Unit | Incorvaia C.,Pulmonary Rehabilitation Unit | Cazzavillan A.,Vittore Buzzi Childrens Hospital | Consonni D.,Epidemiology Unit
International Journal of Pediatric Otorhinolaryngology | Year: 2015

Objectives: Chronic rhinosinusitis (CRS) is a common disease in childhood but is often underdiagnosed because the symptoms are subtle and similar to other nasal pathologies. No clinical symptom is pathognomonic, and consensus documents suggest nasal fibroendoscopy (NF) or imaging criteria (computed tomography, magnetic resonance imaging) as the gold standards for diagnosis of CRS. However, considering the frequent unavailability of such tools to physicians, we designed this study to evaluate whether combinations of symptoms may achieve a clinical diagnosis of CRS in children as confirmed by NF. Methods: The study population consisted of 275 children with a clinical diagnosis of CRS, in 228 of whom diagnosis of CRS was confirmed by NF, while in 47 diagnosis was not confirmed by NF and they served as the control group. The symptoms considered were nasal obstruction, nasal discharge, cough, facial pain, and halitosis, using for statistical analysis multivariate logistic regression, Wald tests, and receiver operating characteristic (ROC) curve. Results: The multivariate logistic regression for CRS symptoms indicated rhinorrea as the strongest predictor of CRS. With three symptoms the probability of CRS was from 60% to 75% without rhinorrea and 77-91% in the presence of this symptom, with four symptoms the probability was over 93%, and with all the five symptoms the probability of having CRS was virtually 100%. Conclusions: These findings suggest that an initial symptoms assessment may help to recognize children with a high probability of CRS, thus reducing the need of NF or imaging techniques. © 2015 Elsevier Ireland Ltd.

Barzaghi F.,San Raffaele Scientific Institute | Barzaghi F.,Vita-Salute San Raffaele University | Passerini L.,San Raffaele Scientific Institute | Gambineri E.,University of Florence | And 18 more authors.
Journal of Autoimmunity | Year: 2012

Immune dysregulation, Polyendocrinopathy, Enteropathy X-linked (IPEX) syndrome is a unique example of primary immunodeficiency characterized by autoimmune manifestations due to defective regulatory T (Treg) cells, in the presence of FOXP3 mutations. However, autoimmune symptoms phenotypically resembling IPEX often occur in the absence of detectable FOXP3 mutations. The cause of this " IPEX-like" syndrome presently remains unclear.To investigate whether a defect in Treg cells sustains the immunological dysregulation in IPEX-like patients, we measured the amount of peripheral Treg cells within the CD3 + T cells by analysing demethylation of the Treg cell-Specific-Demethylated-Region (TSDR) in the FOXP3 locus and demethylation of the T cell-Specific-Demethylated-Region (TLSDR) in the CD3 locus, highly specific markers for stable Treg cells and overall T cells, respectively.TSDR demethylation analysis, alone or normalized for the total T cells, showed that the amount of peripheral Treg cells in a cohort of IPEX-like patients was significantly reduced, as compared to both healthy subjects and unrelated disease controls. This reduction could not be displayed by flow cytometric analysis, showing highly variable percentages of FOXP3 + and CD25 +FOXP3 + T cells. These data provide evidence that a quantitative defect of Treg cells could be considered a common biological hallmark of IPEX-like syndrome. Since Treg cell suppressive function was not impaired, we propose that this reduction per se could sustain autoimmunity. © 2011 Elsevier Ltd.

Racca F.,University of Turin | Berta G.,University of Turin | Sequi M.,Mario Negri Institute | Bignamini E.,University of Turin | And 8 more authors.
Pediatric Pulmonology | Year: 2011

Background Improved technology, as well as professional and parental awareness, enable many ventilator-dependent children to live at home. However, the profile of this growing population, the quality and adequacy of home care, and patients' needs still require thorough assessment. Objectives To define the characteristics of Italian children receiving long-term home mechanical ventilation (HMV) in Italy. Methods A detailed questionnaire was sent to 302 National Health Service hospitals potentially involved in the care of HVM in children (aged <17 years). Information was collected on patient characteristics, type of ventilation, and home respiratory care. Results A total of 362 HMV children was identified. The prevalence was 4.2 per 100,000 (95% CI: 3.8-4.6), median age was 8 years (interquartile range 4-14), median age at starting mechanical ventilation was 4 years (1-11), and 56% were male. The most frequent diagnostic categories were neuromuscular disorders (49%), lung and upper respiratory tract diseases (18%), hypoxic (ischemic) encephalopathy (13%), and abnormal ventilation control (12%). Medical professionals with nurses (for 62% of children) and physiotherapists (20%) participated in the patients' discharge from hospital, though parents were the primary care giver, and in 47% of cases, the sole care giver. Invasive ventilation was used in 41% and was significantly related to young age, southern regional residence, longer time spent under mechanical ventilation, neuromuscular disorders, or hypoxic (ischemic) encephalopathy. Conclusions Care and technical assistance of long-term HMV children need assessment, planning, and resources. A wide variability in pattern of HMV was found throughout Italy. An Italian national ventilation program, as well as a national registry, could be useful in improving the care of these often critically ill children. Copyright © 2011 Wiley-Liss, Inc.

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