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ABSTRACT: Long term home parenteral nutrition (PN) is at potential risk for developing osteoporosis. Various attempts have been made to treat bone disease both by modifying the composition of parenteral nutrition and 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 on very long term home parenteral nutrition (at least 3 years) that 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 two cycles of intravenous pamidronate, 30 mg/m2 once a month for six months consecutively. They all showed a significant improvement in bone mineral density evaluated after 6 and 12 months of pamidronate treatment.In our little sample anthropometrical variables (weight, height, 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, DXA scan showed that no patients had Z score below - 2.5; moreover nobody developed bone fractures during the 108-month-follow up.The patients did not have 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.
Journal of pediatric gastroenterology and nutrition 05/2012; · 2.18 Impact Factor
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F. Ambrosino,
A. Antonelli,
M. Antonelli,
C. Bacci,
P. Beltrame,
G. Bencivenni,
S. Bertolucci,
C. Bini,
C. Bloise,
S. Bocchetta, [......],
M. Testa,
L. Tortora,
P. Valente,
B. Valeriani,
G. Venanzoni,
S. Veneziano, A. Ventura,
R. Versaci,
G. Xu,
The KLOE Collaboration
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[hide abstract]
ABSTRACT: Using a sample of over 400 million φ→KSKL decays produced during the years 2001 and 2002 at the DAΦNE e+e- collider, the ratio RS
π=Γ(KS→π+π-(γ))/Γ(KS→π0π0) has been measured with the KLOE detector. The result is RS
π=2.2555±0.0012stat±0.0021corr-stat±0.0050syst, which is in good agreement with the previously published result based on the KLOE data sample from the year 2000. The average
of the KLOE results is RS
π=2.2549±0.0054, reducing the total error by a factor of three, to 0.25%.
European Physical Journal C 04/2012; 48(3):767-780. · 3.63 Impact Factor
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F. Ambrosino,
A. Antonelli,
M. Antonelli,
C. Bacci,
P. Beltrame,
G. Bencivenni,
S. Bertolucci,
C. Bini,
C. Bloise,
S. Bocchetta, [......],
M. Testa,
L. Tortora,
P. Valente,
B. Valeriani,
G. Venanzoni,
S. Veneziano, A. Ventura,
R. Versaci,
G. Xu,
The KLOE Collaboration
[show abstract]
[hide abstract]
ABSTRACT: We have studied the Dalitz plot of the e+e-→π0π0γ events (Nπ0π0γ∼65000) collected at Ös @ Mf\sqrt{s} \simeq{M}_{\phi} with the KLOE detector. In the dipion invariant mass (Mππ) region below 700MeV, the process under study is dominated by the non-resonant process e+e-→ωπ0 with ω→π0γ whereas, for higher Mππ values, the radiative φ decay to the f0(980) is the dominant mechanism. Different theoretical models are used to fit the Dalitz plot, taking also into account a
possible contribution of the σ(600). For each model, we extract the f0(980) mass and its coupling to ππ, KK̄ and to the φ.
European Physical Journal C 04/2012; 49(2):473-488. · 3.63 Impact Factor
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F. Ambrosino,
A. Antonelli,
M. Antonelli,
C. Bacci,
P. Beltrame,
G. Bencivenni,
S. Bertolucci,
C. Bini,
C. Bloise,
V. Bocci, [......],
M. Testa,
L. Tortora,
P. Valente,
B. Valeriani,
G. Venanzoni,
S. Veneziano, A. Ventura,
R. Versaci,
G. Xu,
The KLOE Collaboration
[show abstract]
[hide abstract]
ABSTRACT: We describe the method of measuring the integrated luminosity of the e+e- collider DAΦNE, the Frascati φ-factory. The measurement is done with the KLOE detector selecting large angle Bhabha scattering
events and normalizing them to the effective cross section. The e+e-→e+e-(γ) cross section is calculated using different event generators which account for the
O(a)\mathcal{O}(\alpha)
radiative initial and final state corrections, and the φ resonance contribution. The accuracy of the measurement is 0.6%,
where 0.3% comes from systematic errors related to the event counting and 0.5% from theoretical evaluations of the cross section.
European Physical Journal C 04/2012; 47(3):589-596. · 3.63 Impact Factor
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Alimentary Pharmacology & Therapeutics 04/2012; 35(8):966-7; author reply 967-9. · 3.77 Impact Factor
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ABSTRACT: 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.
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' satisfaction were recorded.
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' satisfaction was very high.
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.
European annals of allergy and clinical immunology 04/2012; 44(2):54-60.
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ABSTRACT: 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.
European Journal of Pediatrics 03/2012; · 1.88 Impact Factor
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ABSTRACT: Our study evaluated the prevalence, the characteristics and implications of the upper gastrointestinal localisation (UGI+) in paediatric Crohn's Disease (CD) patients.
This prospective study evaluated 45 newly diagnosed CD patients at diagnosis and follow up with respect to CD localisation.
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.
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.
Journal of Crohn s and Colitis 02/2012; 6(1):51-5. · 2.57 Impact Factor
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[show abstract]
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ABSTRACT: 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.
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's milk allergy.
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.
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.
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.
European annals of allergy and clinical immunology 02/2012; 44(1):18-25.
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ABSTRACT: In vitro lymphocyte steroid sensitivity has been suggested as a useful tool to predict in vivo response to glucocorticoid treatment in different inflammatory chronic diseases. A correlation between genetic polymorphisms and clinical response to glucocorticoids has been demonstrated in these patients.
The BclI polymorphism in the glucocorticoid receptor (NR3C1) gene is associated with higher methylprednisolone potency in vitro. The combined evaluation of the in vitro sensitivity to methylprednisolone and BclI polymorphism could represent an aid for physicians to adjust therapy a priori. AIM To evaluate the association between the in vitro sensitivity of peripheral blood mononuclear cells (PBMCs) to methylprednisolone (MP) and the presence of genetic polymorphisms involved in glucocorticoid (GC) response.
In vitro MP inhibition of the proliferation of lymphocytes stimulated with concanavalin A was determined. Non linear regression of dose-response data was performed computing the MP concentration required to reduce proliferation to 50% (IC(50) ). The maximum inhibition achievable at the highest MP concentration (I(max) ) was also calculated. Moreover, the Taqman technique was used to analyze the BclI polymorphism in the NR3C1 gene and the Leu155His polymorphism in the NALP1 gene.
A significant association between the BclI mutated genotype and an increased in vitro sensitivity to GCs was observed.
The a priori evaluation of the BclI polymorphism, associated with a lymphocyte proliferation assay, could represent a useful diagnostic tool for the optimization of steroid treatment.
British Journal of Clinical Pharmacology 10/2011; 73(4):651-5. · 2.96 Impact Factor
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ABSTRACT: 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.
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's milk (CM) allergy, after the hospital "rush" phase.
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.
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.
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.
Allergologia et Immunopathologia 07/2011; 40(1):41-50. · 1.04 Impact Factor
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G Aad,
B Abbott,
J Abdallah,
A A Abdelalim,
A Abdesselam,
O Abdinov,
B Abi,
M Abolins,
H Abramowicz,
H Abreu, [......],
R Zitoun,
L Živković,
V V Zmouchko,
G Zobernig,
A Zoccoli,
Y Zolnierowski,
A Zsenei,
M zur Nedden,
V Zutshi,
L Zwalinski
[show abstract]
[hide abstract]
ABSTRACT: Measurements are presented from proton–proton collisions at centre-of-mass energies of , 2.36 and 7 TeV recorded with the ATLAS detector at the LHC. Events were collected using a single-arm minimum-bias trigger. The charged-particle multiplicity, its dependence on transverse momentum and pseudorapidity and the relationship between the mean transverse momentum and charged-particle multiplicity are measured. Measurements in different regions of phase space are shown, providing diffraction-reduced measurements as well as more inclusive ones. The observed distributions are corrected to well-defined phase-space regions, using model-independent corrections. The results are compared to each other and to various Monte Carlo (MC) models, including a new AMBT1 pythia6 tune. In all the kinematic regions considered, the particle multiplicities are higher than predicted by the MC models. The central charged-particle multiplicity per event and unit of pseudorapidity, for tracks with pT>100 MeV, is measured to be 3.483±0.009 (stat)±0.106 (syst) at and 5.630±0.003 (stat)±0.169 (syst) at .
New Journal of Physics 05/2011; 13(5):053033. · 4.18 Impact Factor
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Archives of Disease in Childhood 09/2010; 95(9):765. · 2.88 Impact Factor
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F Ferrara,
S Quaglia,
I Caputo,
C Esposito,
M Lepretti,
S Pastore,
R Giorgi,
S Martelossi,
G Dal Molin,
N Di Toro, A Ventura,
T Not
[show abstract]
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ABSTRACT: Anti-transglutaminase antibodies are the diagnostic markers of coeliac disease. A role is suggested for infectious agents in the production of anti-transglutaminase antibodies. The aim was to measure positive anti-transglutaminase antibody levels in children with infectious diseases and to compare immunological and biological characteristics of the anti-transglutaminase antibodies derived from these children with that from coeliac patients. Two hundred and twenty-two children suffering from infectious diseases were enrolled prospectively along with seven biopsy-proven coeliacs. Serum samples were tested for anti-transglutaminase antibodies and anti-endomysium antibodies; positive samples were tested for coeliac-related human leucocyte antigen (HLA)-DQ2/8 and anti-viral antibodies. Purified anti-transglutaminase antibodies from the two study groups were tested for urea-dependent avidity, and their ability to induce cytoskeletal rearrangement and to modulate cell-cycle in Caco-2 cells, using phalloidin staining and bromodeoxyuridine incorporation assays, respectively. Nine of 222 children (4%) tested positive to anti-transglutaminase, one of whom also tested positive for anti-endomysium antibodies. This patient was positive for HLA-DQ2 and was diagnosed as coeliac following intestinal biopsy. Of the eight remaining children, two were positive for HLA-DQ8. Levels of anti-transglutaminase returned to normal in all subjects, despite a gluten-containing diet. Purified anti-transglutaminase of the two study groups induced actin rearrangements and cell-cycle progression. During an infectious disease, anti-transglutaminase antibodies can be produced temporarily and independently of gluten. The infection-triggered anti-transglutaminase antibodies have the same biological properties as that of the coeliacs, with the same in-vivo potential for damage.
Clinical & Experimental Immunology 11/2009; 159(2):217-23. · 3.36 Impact Factor
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ABSTRACT: Inherited mtDNA depletion syndromes (MDS) are a group of severe mitochondrial disorders resulting from defects in nucleus-encoded factors and often associated with severe or fatal liver failure.
In this article, we describe the case of an 18-month-old patient with recurrent hypoketotic hypoglycaemia and fatal hepatic dysfunction with liver mtDNA depletion.
The assessment of mtDNA copy number was performed on leucocytes, liver and muscle biopsy by Quantitative Real Time PCR and total RNA from liver biopsy was used as a template to amplify the cDNA of the POLG1 gene.
Sequence analysis identified two previously undescribed mutations (1868T>G and 2263A>G) located in the gene coding the catalytic subunit of mitochondrial DNA polymerase gamma (POLG), predicting an L623W and K755E amino acid change, respectively. Both mutations were located in the highly conserved linker region of the protein and were absent in more than 200 healthy unrelated control subjects. The identification of these two mutations allowed us to perform genetic counselling and prenatal diagnosis.
Our data further expand the spectrum of POLG1 gene mutations and the unique phenotype reported (late onset isolated liver disease without lactic acidosis) increase the variability of clinical presentations associated with mutations in this gene.
Digestive and Liver Disease 02/2009; 41(7):494-9. · 3.05 Impact Factor
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Thorax 01/2009; 63(12):1082, 1090. · 6.84 Impact Factor
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I. Sareneva,
L. L. E. Koskinen,
I. R. Korponay-Szabo,
K. Kaukinen,
K. Kurppa,
F. Ziberna,
S. Vatta,
T. Not, A. Ventura,
R. Ádány,
Z. Pocsai,
G. Széles,
M. Mäki,
P. Saavalainen,
E. Einarsdottir
[show abstract]
[hide abstract]
ABSTRACT: The Fcγ receptor cluster on chromosome 1q23 contains a number of genes that may affect susceptibility to celiac disease, but previous studies have yielded contradictory results. We studied the FcγRIIa*A519G (rs1801274) and FcγRIIIa*A559C (rs396991) single nucleotide polymorphisms in celiac disease families from Hungary and Finland and in celiac disease case–control materials from Hungary and Italy. Neither the Hungarian nor the Italian case–control material or a meta-analysis of the combined case–control material showed significant single-marker or haplotype association. In addition, neither linkage nor family-based association tests showed evidence for association in the Finnish or Hungarian family material. This study thus does not support a previous publication showing FcγR association with celiac disease.
Tissue Antigens 12/2008; 73(1):54 - 58. · 2.59 Impact Factor
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M Castro,
B Papadatou,
M Baldassare,
F Balli,
A Barabino,
C Barbera,
S Barca,
G Barera,
F Bascietto,
R Berni Canani, [......],
A Ravelli,
P Roggero,
C Romano,
N Rotolo,
V Rutigliano,
S Scotta,
C Sferlazzas,
A Staiano, A Ventura,
M G Zaniboni
[show abstract]
[hide abstract]
ABSTRACT: The purpose was to assess in Italy the clinical features at diagnosis of inflammatory bowel disease (IBD) in children.
In 1996 an IBD register of disease onset was established on a national scale.
Up to the end of 2003, 1576 cases of pediatric IBD were recorded: 810 (52%) ulcerative colitis (UC), 635 (40%) Crohn's disease (CD), and 131 (8%) indeterminate colitis (IC). In the period 1996-2003 an increase of IBD incidence from 0.89 to 1.39/10(5) inhabitants aged <18 years was observed. IBD was more frequent among children aged between 6 and 12 years (57%) but 20% of patients had onset of the disease under 6 years of age; 28 patients were <1 year of age. Overall, 11% had 1 or more family members with IBD. The mean interval between onset of symptoms and diagnosis was higher in CD (10.1 months) and IC (9 months) versus UC (5.8 months). Extended colitis was the most frequent form in UC and ileocolic involvement the most frequent in CD. Upper intestinal tract involvement was present in 11% of CD patients. IC locations were similar to those of UC. Bloody diarrhea and abdominal pain were the most frequent symptoms in UC and IC, and abdominal pain and diarrhea in CD. Extraintestinal symptoms were more frequent in CD than in UC.
The IBD incidence in children and adolescents in Italy shows an increasing trend for all 3 pathologies. UC diagnoses exceeded CD.
Inflammatory Bowel Diseases 09/2008; 14(9):1246-52. · 4.86 Impact Factor
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Digestive and Liver Disease 08/2008; 40(7):578. · 3.05 Impact Factor
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[show abstract]
[hide abstract]
ABSTRACT: Chest physiotherapy (CP) is used in cystic fibrosis (CF) even if there is no robust scientific evidence of a beneficial effect. We investigated the effects of a training with a specific device (SpiroTiger) in a group of CF patients. This device, developed for respiratory training through maximal inspirations and espirations without hypocarbia, may improve respiratory function and mucus clearance. Patients where instructed and trained by a physiotherapist with individualized settings of training parameters.
Twenty-four patients were enrolled in an open-label 1 year observational study. Baseline and post intervention measurements were determined by lung function (FVC, FEV1, FEF 25-75), patients' opinions on physiotherapy (questionnaires), need for antibiotic treatment (clinical follow-up and records) and perception of physical fitness (questionnaires) in the year before and in the year of intervention. Adherence to physiotherapy was monitored by means of a specific device software.
Increased lung function (FEV1 p<0.01), perception of physical fitness (p<0.001) and a reduction in the need for intravenous antibiotic treatment (p<0.001) were reported. Adherence to treatment was good/acceptable in 92% of patients.
This study shows an association between training through a specific device and improved lung function. Further trials are needed to confirm this report.
Journal of Cystic Fibrosis 07/2008; 7(4):313-9. · 3.19 Impact Factor
