@article {8311, title = {A congenital purplish tumour.}, journal = {Arch Dis Child Educ Pract Ed}, volume = {102}, year = {2017}, month = {2017 Apr}, pages = {79-81}, keywords = {Congenital Abnormalities, Humans, India, Infant, Infant, Newborn, Knee, Male, Neonatology, Neoplasms, Treatment Outcome}, issn = {1743-0593}, doi = {10.1136/archdischild-2015-309475}, author = {Matarazzo, L and Delise, A and Zennaro, F and Bussani, R and Demarini, S and Berti, I and Ventura, A} } @article {7764, title = {Long noncoding RNA GAS5: a novel marker involved in glucocorticoid response.}, journal = {Curr Mol Med}, volume = {15}, year = {2015}, month = {2015}, pages = {94-9}, abstract = {

Glucocorticoids (GCs) exert their effects through regulation of gene expression after activation in the cytoplasm of the glucocorticoid receptor (GR) encoded by NR3C1 gene. A negative feedback mechanism resulting in GR autoregulation has been demonstrated through the binding of the activated receptor to intragenic sequences called GRE-like elements, contained in GR gene. The long noncoding RNA growth arrest-specific transcript 5 (GAS5) interacts with the activated GR suppressing its transcriptional activity. The aim of this study was to evaluate the possible role of GAS5 and NR3C1 gene expression in the antiproliferative effect of methylprednisolone in peripheral blood mononuclear cells and to correlate the expression with individual sensitivity to GCs. Subjects being poor responders to GCs presented higher levels of GAS5 and NR3C1 in comparison with good responders. We suggest that abnormal levels of GAS5 may alter GC effectiveness, probably interfering with the mechanism of GR autoregulation.

}, keywords = {Adult, Cell Proliferation, Female, Gene Expression Regulation, Glucocorticoids, Humans, Leukocytes, Mononuclear, Male, Methylprednisolone, Middle Aged, Receptors, Glucocorticoid, RNA, Long Noncoding, Transcription, Genetic}, issn = {1875-5666}, author = {Lucafo, M and De Iudicibus, S and Di Silvestre, A and Pelin, M and Candussio, L and Martelossi, S and Tommasini, A and Piscianz, E and Ventura, A and Decorti, G} } @article {7702, title = {Prevalence of celiac disease in patients with severe food allergy.}, journal = {Allergy}, volume = {70}, year = {2015}, month = {2015 Oct}, pages = {1346-9}, abstract = {

The association between food allergy and celiac disease (CD) is still to be clarified. We screened for CD 319 patients with severe food allergy (IgE~>~85~kU/l against food proteins and a history of severe allergic reactions) who underwent specific food oral immunotherapy (OIT), together with 128 children with mild allergy who recovered without OIT, and compared the prevalence data with our historical data regarding healthy schoolchildren. Sixteen patients (5\%) with severe allergy and one (0.8\%) with mild allergy tested positive for both genetic and serological CD markers, while the prevalence among the schoolchildren was 1\%. Intestinal biopsies were obtained in 13/16 patients with severe allergy and in the one with mild allergy, confirming the diagnosis of CD. Sufferers from severe food allergy seem to be at a fivefold increased risk of CD. Our findings suggest that routine screening for CD should be recommended in patients with severe food allergy.

}, issn = {1398-9995}, doi = {10.1111/all.12692}, author = {Pillon, R and Ziberna, F and Badina, L and Ventura, A and Longo, G and Quaglia, S and De Leo, L and Vatta, S and Martelossi, S and Patano, G and Not, T and Berti, I} } @article {1875, title = {Pain in cognitively impaired children: a focus for general pediatricians.}, journal = {Eur J Pediatr}, volume = {172}, year = {2013}, month = {2013 Jan}, pages = {9-14}, abstract = {

UNLABELLED: Pain in children with cognitive impairment and cerebral palsy is a particularly relevant issue due to its high prevalence and impact on quality of life. We review available evidence about prevalence of pain, causes and specific treatment, recognition and use of specific pain scales, physiology, and consequences of pain in this subset of patients.

CONCLUSIONS: Pain is very common and is a critical determinant of quality of life in children with cognitive impairment and cerebral palsy. The diseases and associated complications that frequently expose these patients to pain can be treated and pain prevented. For patients with communication difficulties, appropriate, effective, validated tools are available and should be used to diagnose pain in itself, to >choose analgesic treatment and to determine effectiveness of these therapies. The level of awareness of pediatricians towards this issue seems to be quite low.

}, keywords = {Analgesics, Cerebral Palsy, Child, Cognition Disorders, Humans, Pain, Pain Measurement, Quality of Life}, issn = {1432-1076}, doi = {10.1007/s00431-012-1720-x}, author = {Massaro, M and Pastore, S and Ventura, A and Barbi, E} } @article {1793, title = {Adverse effects during specific oral tolerance induction: in home phase.}, journal = {Allergol Immunopathol (Madr)}, volume = {40}, year = {2012}, month = {2012 Jan-Feb}, pages = {41-50}, abstract = {

BACKGROUND: Specific oral tolerance induction (SOTI) is a promising approach for severe food allergies. There are little data in the literature regarding the home-phase of SOTI, not only with regard to type and frequency of adverse reactions but also regarding the most suitable treatment and protocol.

AIMS: To define the incidence and severity of adverse reactions, possible risk factors, and the safety and effectiveness of the home-phase of an original SOTI protocol in a large group of children with severe cow{\textquoteright}s milk (CM) allergy, after the hospital "rush" phase.

METHODS: The study was conducted by recording in-home phase adverse events, success and failure as reported by parents, and calling families. Adverse reactions were treated following the International Guidelines, arbitrarily modified by introducing nebulised epinephrine for respiratory reactions, oral beclomethasone for acute gastric pain and oral cromolyn for recurrent gastric pain.

RESULTS: Out of 140 patients, 132 were contacted; eight were inaccessible (follow-up 2-84 months). The number of adverse reactions was 1 in every 100 doses. The reactions were treated with nebulised epinephrine (221 reactions), IM epinephrine (6 reactions), and other drugs. Patients with high specific IgE levels (greater than 100 kU(A)/L) and lower CM dose (less than 5 ml) at the end of in-hospital phase showed a higher risk both for number of reactions and use of nebulised epinephrine.

CONCLUSIONS: The home phase of SOTI was characterised by a significant number of adverse reactions, mostly managed with an acceptable rate of side effects. Nebulised epinephrine played a pivotal role in respiratory reactions.

}, keywords = {Adolescent, Adult, Age Factors, Allergens, Child, Child, Preschool, Desensitization, Immunologic, Epinephrine, Female, Food Hypersensitivity, Humans, Immune Tolerance, Immunoglobulin E, Male, Milk Hypersensitivity, Nebulizers and Vaporizers}, issn = {1578-1267}, doi = {10.1016/j.aller.2011.05.004}, author = {Barbi, E and Longo, G and Berti, I and Matarazzo, L and Rubert, L and Saccari, A and Lenisa, I and Ronfani, L and Radillo, O and Ventura, A} } @article {1895, title = {Adverse effects during specific oral tolerance induction: in-hospital "rush" phase.}, journal = {Eur Ann Allergy Clin Immunol}, volume = {44}, year = {2012}, month = {2012 Feb}, pages = {18-25}, abstract = {

BACKGROUND: Specific oral tolerance induction (SOTI) is a promising approach in the treatment of severe food allergies. Different protocols have demonstrated its efficacy. Nevertheless, SOTI is still considered an experimental method and should be limited to highly controlled settings.

AIMS: To define the incidence and severity of adverse reactions, possible risk factors, and the safety and effectiveness of nebulized epinephrine as a first-line treatment of respiratory reactions during in-hospital SOTI for cow{\textquoteright}s milk allergy.

MATERIALS AND METHODS: A retrospective study was conducted by reviewing the medical records of patients admitted for SOTI beginning in 2001. Reactions were classified as mild, moderate and severe on a partially modified Clark scale. Adverse reactions were treated following the International Guidelines with the introduction of nebulized epinephrine for level four reactions.

RESULTS: Of 209 patients, 17 were excluded due to the absence of objective reactions. The remaining 192 were classified as follows: Mild Reactions (Clark Scale 1 to 3): 100 patients received either no treatment, oral antihistamines or nebulized steroids; Moderate Reactions (Clark Scale 4): 87 patients treated with nebulized epinephrine and, depending on their symptoms, oral antihistamines, corticosteroids (nebulized, oral or IV) or nebulized beta 2 agonists; Severe Reactions (Clark Scale 5): 5 children, 4 of whom initially underwent one nebulization of epinephrine and eventually required an IM dose. The fifth patient was immediately treated with IM epinephrine due to hypotension.

DISCUSSION: adverse reactions during this in-hospital SOTI protocol were frequent but easily manageable. Nebulized epinephrine can play a relevant role in the treatment of respiratory reactions.

}, keywords = {Administration, Inhalation, Anaphylaxis, Animals, Bronchodilator Agents, Child, Desensitization, Immunologic, Epinephrine, Humans, Milk, Milk Hypersensitivity, Retrospective Studies}, issn = {1764-1489}, author = {Barbi, E and Longo, G and Berti, I and Neri, E and Saccari, A and Rubert, L and Matarazzo, L and Montico, M and Ventura, A} } @article {1774, title = {Coeliac disease diagnosis: ESPGHAN 1990 criteria or need for a change? Results of a questionnaire.}, journal = {J Pediatr Gastroenterol Nutr}, volume = {54}, year = {2012}, month = {2012 Jan}, pages = {15-9}, abstract = {

BACKGROUND AND OBJECTIVES: A revision of criteria for diagnosing coeliac disease (CD) is being conducted by The European Society for Pediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN). In parallel, we have performed a survey aimed to evaluate present practices for CD among paediatric gastroenterologists and to learn their views on the need for modification of present criteria for CD diagnosis.

PATIENTS AND METHODS: Questionnaires were distributed to experienced paediatric gastroenterologists (ESPGHAN members) via the Internet.

RESULTS: Overall, 95 valid questionnaires were available for analysis, pertaining to 28 different countries, with the majority of responders treating patients with CD for >15 years. Only about 12\% of the responders comply with present criteria, noncompliance being related mainly to the challenge policy. Approximately 90\% request a revision and modification of the present criteria. Forty-four percent want to omit the small bowel biopsy in symptomatic children with positive anti-tissue transglutaminase immunoglobulin (Ig) A or endomysial IgA antibodies, especially if they are DQ2/DQ8 positive. For silent cases detected by screening with convincingly positive anti-tissue transglutaminase IgA or EMA IgA, about 30\% consider that no small bowel biopsy should be required in selected cases. Adding human leukocyte antigen typing in the diagnostic workup was asked for by 42\% of the responders. As for gluten challenge, a new policy is advocated restricting its obligation to cases whenever the diagnosis is doubtful or unclear.

CONCLUSIONS: Based on these opinions, revision of the ESPGHAN criteria for diagnosing CD is urgently needed.

}, keywords = {Adolescent, Adult, Biopsy, Celiac Disease, Child, Child, Preschool, Glutens, Guideline Adherence, Guidelines as Topic, Health Care Surveys, Humans, Immunoglobulin A, Intestine, Small, Physician{\textquoteright}s Practice Patterns, Questionnaires, Societies, Medical, Transglutaminases, Young Adult}, issn = {1536-4801}, doi = {10.1097/MPG.0b013e31822a00bb}, author = {Ribes-Koninckx, C and Mearin, M L and Korponay-Szab{\'o}, I R and Shamir, R and Husby, S and Ventura, A and Branski, D and Catassi, C and Koletzko, S and M{\"a}ki, M and Troncone, R and Zimmer, K P} } @article {1947, title = {Diagnosed child, treated child: food challenge as the first step toward tolerance induction in cow{\textquoteright}s milk protein allergy.}, journal = {Eur Ann Allergy Clin Immunol}, volume = {44}, year = {2012}, month = {2012 Apr}, pages = {54-60}, abstract = {

BACKGROUND: Food challenge is required to assess tolerance in cow milk (CM) allergy. A positive challenge contraindicates the reintroduction of CM. Specific oral tolerance induction (SOTI) is a promising treatment.

METHODS: All children admitted for a challenge were prospectively enrolled. To those tolerating between 2 and 150 ml a SOTI protocol was offered. Outcome, adverse reactions, parents{\textquoteright} satisfaction were recorded.

RESULTS: Out of 245 challenged patients, 175 reacted 122 out of 125, able to tolerate a minimum dose of 2 ml, underwent SOTI. After one year 75.4\% were in an unrestricted diet, 16.1\% tolerated between 5 and 150 ml, 8.5\% stopped SOTI. Side effects were mild, parents{\textquoteright} satisfaction was very high.

CONCLUSIONS: The majority of children tolerating limited amounts of CM at the challenge acquires tolerance with SOTI without relevant side effects. Maintaining on an exclusion diet partially tolerant children should be considered debatable.

}, keywords = {Administration, Oral, Adolescent, Animals, Cattle, Child, Child, Preschool, Humans, Immune Tolerance, Infant, Milk Hypersensitivity, Milk Proteins, Self Administration}, issn = {1764-1489}, author = {Longo, G and Berti, I and Barbi, E and Calligaris, L and Matarazzo, L and Radillo, O and Ronfani, L and Ventura, A} } @article {1843, title = {European Society for Pediatric Gastroenterology, Hepatology, and Nutrition guidelines for the diagnosis of coeliac disease.}, journal = {J Pediatr Gastroenterol Nutr}, volume = {54}, year = {2012}, month = {2012 Jan}, pages = {136-60}, abstract = {

OBJECTIVE: Diagnostic criteria for coeliac disease (CD) from the European Society for Paediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN) were published in 1990. Since then, the autoantigen in CD, tissue transglutaminase, has been identified; the perception of CD has changed from that of a rather uncommon enteropathy to a common multiorgan disease strongly dependent on the haplotypes human leukocyte antigen (HLA)-DQ2 and HLA-DQ8; and CD-specific antibody tests have improved.

METHODS: A panel of 17 experts defined CD and developed new diagnostic criteria based on the Delphi process. Two groups of patients were defined with different diagnostic approaches to diagnose CD: children with symptoms suggestive of CD (group 1) and asymptomatic children at increased risk for CD (group 2). The 2004 National Institutes of Health/Agency for Healthcare Research and Quality report and a systematic literature search on antibody tests for CD in paediatric patients covering the years 2004 to 2009 was the basis for the evidence-based recommendations on CD-specific antibody testing.

RESULTS: In group 1, the diagnosis of CD is based on symptoms, positive serology, and histology that is consistent with CD. If immunoglobulin A anti-tissue transglutaminase type 2 antibody titers are high (>10 times the upper limit of normal), then the option is to diagnose CD without duodenal biopsies by applying a strict protocol with further laboratory tests. In group 2, the diagnosis of CD is based on positive serology and histology. HLA-DQ2 and HLA-DQ8 testing is valuable because CD is unlikely if both haplotypes are negative.

CONCLUSIONS: The aim of the new guidelines was to achieve a high diagnostic accuracy and to reduce the burden for patients and their families. The performance of these guidelines in clinical practice should be evaluated prospectively.

}, keywords = {Adolescent, Celiac Disease, Child, Duodenum, HLA-DQ Antigens, Humans, Immunoglobulin A, Transglutaminases}, issn = {1536-4801}, doi = {10.1097/MPG.0b013e31821a23d0}, author = {Husby, S and Koletzko, S and Korponay-Szab{\'o}, I R and Mearin, M L and Phillips, A and Shamir, R and Troncone, R and Giersiepen, K and Branski, D and Catassi, C and Lelgeman, M and M{\"a}ki, M and Ribes-Koninckx, C and Ventura, A and Zimmer, K P} } @article {1890, title = {Letter: TPMT activity and age in IBD patients.}, journal = {Aliment Pharmacol Ther}, volume = {35}, year = {2012}, month = {2012 Apr}, pages = {966-7; author reply 967-9}, keywords = {Azathioprine, Humans, Immunosuppressive Agents, Inflammatory Bowel Diseases, Thioguanine}, issn = {1365-2036}, doi = {10.1111/j.1365-2036.2012.05027.x}, author = {Stocco, G and De Iudicibus, S and Cuzzoni, E and Decorti, G and Martelossi, S and Ventura, A} } @article {1910, title = {Treatment with pamidronate for osteoporosis complicating long-term intestinal failure.}, journal = {J Pediatr Gastroenterol Nutr}, volume = {55}, year = {2012}, month = {2012 Nov}, pages = {615-8}, abstract = {

Long-term home parenteral nutrition (PN) is a potential risk for developing osteoporosis. Various attempts have been made to treat bone disease both by modifying the composition of PN and by administering hormones, such as calcitonin, parathyroid hormone, and sexual hormones. Bisphosphonates are recognized as a medication useful for the treatment of several bone disorders associated with excessive reabsorption. Nevertheless, there have been no paediatric studies on bisphosphonates use for intestinal failure-associated bone disease. Our study includes 6 paediatric patients receiving extremely long-term home PN (at least 3 years) who showed radiological and clinical signs of osteoporosis. Diagnosis of bone disease was made after a median period of 127.5 PN months. Treatment consisted in 2 cycles of intravenous pamidronate, 30 mg/m once per month for 6 months consecutively. They all showed a significant improvement in bone mineral density, evaluated after 6 and 12 months of pamidronate treatment. In our sample anthropometrical variables (weight, height, and body mass index) are not related with the z-score trend. Our patients had normal levels of calcium, phosphorus, and vitamin D, and proper nutrient intake. At the last follow-up, dual-energy x-ray absorptiometry scan showed that no patients had a z-score lower than -2.5; moreover, nobody developed bone fractures during the 108-month follow-up. The patients did not have any prominent adverse effect. Finally, in our experience, pamidronate is effective for improving bone mineral density and safe in patients with intestinal failure-associated bone disease.

}, keywords = {Absorptiometry, Photon, Adolescent, Bone Density, Bone Density Conservation Agents, Child, Child, Preschool, Diphosphonates, Female, Humans, Intestinal Diseases, Male, Osteoporosis, Parenteral Nutrition, Home}, issn = {1536-4801}, doi = {10.1097/MPG.0b013e31825f1c7d}, author = {Pastore, S and Londero, M and Barbieri, F and Di Leo, G and Paparazzo, R and Ventura, A} } @article {1850, title = {Upper gastrointestinal involvement in paediatric onset Crohn{\textquoteright}s disease: prevalence and clinical implications.}, journal = {J Crohns Colitis}, volume = {6}, year = {2012}, month = {2012 Feb}, pages = {51-5}, abstract = {

BACKGROUND AND AIMS: Our study evaluated the prevalence, the characteristics and implications of the upper gastrointestinal localisation (UGI+) in paediatric Crohn{\textquoteright}s Disease (CD) patients.

METHODS: This prospective study evaluated 45 newly diagnosed CD patients at diagnosis and follow up with respect to CD localisation.

RESULTS: All patients presented CD at the colon and/or ileum. In 24/45 patients (53.3\%, 12 F and 12 M) an UGI+ involvement was also found. UGI+ patients had a younger age of onset (10.9 years versus 12.6 years; P<0.05). PCDAI at diagnosis was significantly higher in the UGI+ (41 vs. 25 P<0.01). UGI+ patients were overall more symptomatic. Pancolitis and extraintestinal manifestations were also more frequent (19/24 (80\%) vs. 12/21 (57\%) P<0.01). Growth was more impaired at diagnosis in UGI+ patients. By the end of the follow-up (mean 3 years, range 2 to 4) no significant difference was found in PCDAI (17 in UGI+ patients vs. 11 in UGI- P=NS), or the number of relapses. Weight and growth catch-up in UGI+ patients were comparable to UGI- ones. However, UGI+ patients required a more aggressive therapeutic approach.

CONCLUSION: At least half of paediatric onset CD patients have an upper gastrointestinal localisation. UGI+ patients present an earlier onset and a more severe disease. The final outcome does not differ, but UGI+ patients require a more aggressive therapeutic approach.

}, keywords = {Adolescent, Age of Onset, Child, Crohn Disease, Female, Humans, Male, Prospective Studies, Upper Gastrointestinal Tract}, issn = {1876-4479}, doi = {10.1016/j.crohns.2011.06.013}, author = {Crocco, S and Martelossi, S and Giurici, N and Villanacci, V and Ventura, A} } @article {1937, title = {Videogame playing as distraction technique in course of venipuncture.}, journal = {Pediatr Med Chir}, volume = {34}, year = {2012}, month = {2012 Mar-Apr}, pages = {77-83}, abstract = {

BACKGROUND: Needle-related procedures (venipuncture, intravenous cannulation) are the most common source of pain and distress for children. Reducing needle related pain and anxiety could be important in order to prevent further distress, especially for children needing multiple hospital admissions. The aim of the present open randomized controlled trial was to investigate the efficacy of adding an active distraction strategy (videogame) to EMLA premedication in needle-related pain in children.

METHODS: One-hundred and nine children (4 -10 years of age) were prospectively recruited to enter in the study. Ninety-seven were randomized in two groups: CC group (conventional care: EMLA only) as control group and AD group (active distraction: EMLA plus videogame) as intervention group. Outcome measures were: self-reported pain by mean of FPS-R scale (main study outcome), observer-reported pain by FLACC scale, number of attempts for successful procedure.

RESULTS: In both groups FPS-R median rate was 0 (interquartile range: 0-2), with significant pain (FPS-R > 4) reported by 9\% of subjects. FLACC median rate was 1 in both groups (interquartile range 0-3 in CC group; 0-2 in AD group). The percentage of children with major pain (FLACC > 4) was 18\% in CC group and 9\% in AD group (p = 0.2). The median of necessary attempts to succeed in the procedures was 1 (interquartile range 1-2) in both groups..

CONCLUSION: Active distraction doesn{\textquoteright}t improve EMLA analgesia for iv cannulation and venipuncture. Even though, it resulted in an easily applicable strategy appreciated by children. This technique could be usefully investigated in other painful procedures.

}, keywords = {Anesthetics, Local, Child, Child, Preschool, Female, Humans, Lidocaine, Male, Pain, Phlebotomy, Prilocaine, Prospective Studies, Video Games}, issn = {0391-5387}, doi = {10.4081/pmc.2012.64}, author = {Minute, M and Badina, L and Cont, G and Montico, M and Ronfani, L and Barbi, E and Ventura, A} } @article {1650, title = {Does infant gastro-oesophageal reflux really deserve medical attention?}, journal = {Arch Dis Child}, volume = {95}, year = {2010}, month = {2010 Sep}, pages = {765}, keywords = {Beds, Gastroesophageal Reflux, Humans, Infant, Infant Care, Posture, Prognosis}, issn = {1468-2044}, doi = {10.1136/adc.2010.186502}, author = {Declich, V and Badina, L and Ventura, A} }