@article {10844, title = {Retrospective study showed that palpitations with tachycardia on admission to a paediatric emergency department were related to cardiac arrhythmias.}, journal = {Acta Paediatr}, volume = {108}, year = {2019}, month = {2019 Feb}, pages = {328-332}, abstract = {

AIM: This retrospective study reviewed the prevalence and long-term prognosis of children aged 0-18 with palpitations who were admitted to the emergency department (ED) of an Italian paediatric hospital.

METHODS: We examined all admissions to the ED of the IRCCS Burlo Garofolo between January 2009 and December 2015 by selecting triage diagnoses of palpitations. The hospital discharge cards were reviewed to assess vital parameters, physical examinations, diagnostic tests, cardiology consultations and final diagnoses.

RESULTS: Of the 142 803 patients who attended our ED for any reason, 96 (0.07\%) complained of palpitations. Despite this low prevalence, it was noteworthy that 13.5\% had a real underlying arrhythmic cause and needed medical assistance. Over half (52.1\%) were women and the mean age was 12.7 years. At the long-term follow-up, at a mean of 47 {\textpm} 23 months, 53.8\% of patients with a cardiac arrhythmia had received medical therapy and 46.1\% had undergone trans-catheter ablation for supraventricular tachycardia. A heart rate above 146 beats per minute or palpitations for more than an hour was statistically related to a cardiac arrhythmia.

CONCLUSION: Palpitations were an infrequent cause of admission to our ED, but 13.5\% who displayed them had an underlying cardiac arrhythmia.

}, issn = {1651-2227}, doi = {10.1111/apa.14486}, author = {Bobbo, Marco and Amoroso, Stefano and Tamaro, Gianluca and Gesuete, Valentina and D{\textquoteright}agata Mottolese, Biancamaria and Barbi, Egidio and Ventura, Alessandro} } @article {10510, title = {Role of the Long Non-Coding RNA Growth Arrest-Specific 5 in Glucocorticoid Response in Children with Inflammatory Bowel Disease.}, journal = {Basic Clin Pharmacol Toxicol}, volume = {122}, year = {2018}, month = {2018 Jan}, pages = {87-93}, abstract = {

Glucocorticoids (GCs) are widely employed in inflammatory, autoimmune and neoplastic diseases, and, despite the introduction of novel therapies, remain the first-line treatment for inducing remission in inflammatory bowel disease (IBD). Given the high incidence of suboptimal response, associated with a significant number of side-effects, that are particularly severe in paediatric patients, the identification of subjects that are most likely to respond poorly to GCs is extremely important. Recent evidence suggests that the long non-coding RNA (lncRNA) GAS5 could be a potential marker of GC resistance. To address this issue, we evaluated the association between the lncRNA GAS5 and the efficacy of steroids, in terms of inhibition of proliferation, in two cell lines derived from colon and ovarian cancers, to confirm the sensitivity and specificity of these lncRNAs. These cells showed a different sensitivity to GCs and revealed differential expression of GAS5 after treatment. GAS5 was up-regulated in GC-resistant cells and accumulated more in the cytoplasm compared to the nucleus in response to the drug. The functions of GAS5 were assessed by silencing, and we found that GAS5 knock-down reduced the proliferation during GC treatment. Furthermore, for the first time, we measured GAS5 levels in 19 paediatric IBD patients at diagnosis and after the first cycle of GCs, and we demonstrated an up-regulation of the lncRNA in patients with unfavourable steroid response. Our preliminary results indicate that GAS5 could be considered a novel pharmacogenomic marker useful for the personalization of GC therapy.

}, keywords = {Biomarkers, Cell Line, Tumor, Cell Proliferation, Child, Drug Resistance, Female, Gene Knockdown Techniques, Glucocorticoids, Humans, Inflammatory Bowel Diseases, Male, Patient Selection, Pharmacogenomic Testing, Precision Medicine, RNA, Long Noncoding, RNA, Small Interfering, Treatment Outcome, Up-Regulation}, issn = {1742-7843}, doi = {10.1111/bcpt.12851}, author = {Lucaf{\`o}, Marianna and Di Silvestre, Alessia and Romano, Maurizio and Avian, Alice and Antonelli, Roberta and Martelossi, Stefano and Naviglio, Samuele and Tommasini, Alberto and Stocco, Gabriele and Ventura, Alessandro and Decorti, Giuliana and De Iudicibus, Sara} } @article {7766, title = {Relapse and metastasis of atypical teratoid/rhabdoid tumor in a boy with neurofibromatosis type 1 treated with recombinant human growth hormone.}, journal = {Neuropediatrics}, volume = {46}, year = {2015}, month = {2015 Apr}, pages = {126-9}, abstract = {

Even though no increased recurrence rate seems to be reported in patients with brain tumors receiving recombinant human growth hormone (rhGH) replacement, in some patients multiple risk factors could put at higher risk for recurrence. In such cases, the decision to start rhGH therapy should be very cautious. A boy with neurofibromatosis type 1 developed an atypical teratoid/rhabdoid tumor (AT/RT) of right cerebellum, treated with surgery, radiotherapy, and chemotherapy. After 3 years of remission, he started rhGH for growth hormone deficiency, having a negative magnetic resonance imaging (MRI) scan. Ten weeks after starting therapy, the boy became symptomatic and MRI showed relapse of AT/RT in the right cerebellum and a new lesion in the brainstem. The boy died of progressive disease. In this case, the connection between AT/RT recurrence and the beginning of rhGH therapy, with a negative pretreatment MRI, cannot be excluded. Additional caution should be used for rhGH in patients with multiple risk factors.

}, keywords = {Brain Stem Neoplasms, Cerebellar Neoplasms, Child, Human Growth Hormone, Humans, Magnetic Resonance Imaging, Male, Neurofibromatosis 1, Recombinant Proteins, Recurrence, Rhabdoid Tumor, Risk Factors, Teratoma}, issn = {1439-1899}, doi = {10.1055/s-0034-1393706}, author = {Tornese, Gianluca and Faleschini, Elena and Matarazzo, Lorenza and Bibalo, Cristina and Zanazzo, Giulio Andrea and Rabusin, Marco and Tonini, Giorgio and Zennaro, Floriana and Ventura, Alessandro} } @article {7687, title = {Reply to Thalidomide Treatment of Pediatric Ulcerative Colitis: A New Use for an Old Drug.}, journal = {Inflamm Bowel Dis}, volume = {21}, year = {2015}, month = {2015 Aug}, pages = {1752-3}, keywords = {Crohn Disease, Female, Humans, Immunosuppressive Agents, Male, Thalidomide}, issn = {1536-4844}, doi = {10.1097/MIB.0000000000000451}, author = {Lazzerini, Marzia and Martelossi, Stefano and Ventura, Alessandro} } @article {7779, title = {Role of oxidative stress mediated by glutathione-s-transferase in thiopurines{\textquoteright} toxic effects.}, journal = {Chem Res Toxicol}, volume = {28}, year = {2015}, month = {2015 Jun 15}, pages = {1186-95}, abstract = {

Azathioprine (AZA), 6-mercaptopurine (6-MP), and 6-thioguanine (6-TG) are antimetabolite drugs, widely used as immunosuppressants and anticancer agents. Despite their proven efficacy, a high incidence of toxic effects in patients during standard-dose therapy is recorded. The aim of this study is to explain, from a mechanistic point of view, the clinical evidence showing a significant role of glutathione-S-transferase (GST)-M1 genotype on AZA toxicity in inflammatory bowel disease patients. To this aim, the human nontumor IHH and HCEC cell lines were chosen as predictive models of the hepatic and intestinal tissues, respectively. AZA, but not 6-MP and 6-TG, induced a concentration-dependent superoxide anion production that seemed dependent on GSH depletion. N-Acetylcysteine reduced the AZA antiproliferative effect in both cell lines, and GST-M1 overexpression increased both superoxide anion production and cytotoxicity, especially in transfected HCEC cells. In this study, an in vitro model to study thiopurines{\textquoteright} metabolism has been set up and helped us to demonstrate, for the first time, a clear role of GST-M1 in modulating AZA cytotoxicity, with a close dependency on superoxide anion production. These results provide the molecular basis to shed light on the clinical evidence suggesting a role of GST-M1 genotype in influencing the toxic effects of AZA treatment.

}, issn = {1520-5010}, doi = {10.1021/acs.chemrestox.5b00019}, author = {Pelin, Marco and De Iudicibus, Sara and Fusco, Laura and Taboga, Eleonora and Pellizzari, Giulia and Lagatolla, Cristina and Martelossi, Stefano and Ventura, Alessandro and Decorti, Giuliana and Stocco, Gabriele} } @article {1685, title = {Refractory iron-deficiency anaemia in a child with portal cavernoma.}, journal = {Gut}, volume = {60}, year = {2011}, month = {2011 Mar}, pages = {317, 377}, keywords = {Anemia, Iron-Deficiency, Antihypertensive Agents, Child, Hemangioma, Cavernous, Humans, Hypertension, Portal, Ileal Diseases, Male, Propranolol, Vascular Neoplasms}, issn = {1468-3288}, doi = {10.1136/gut.2009.184697}, author = {Pastore, Serena and Londero, Margherita and Cont, Gabriele and Di Leo, Grazia and Ventura, Alessandro} }