San Luca, Italy
San Luca, Italy

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Costa G.,University of Rome La Sapienza | Ruscelli P.,Marche Polytechnic University | Balducci G.,University of Rome La Sapienza | Buccoliero F.,M Bufalini Hospital | And 54 more authors.
Annali Italiani di Chirurgia | Year: 2016

BACKGROUND In: Testinal obstructions/pseudo-obstruction of the smalUlarge bowel are jequent conditions but their management could be challenging. Moreover, a general agreement in this field is currently lacking, thus SICUT Society designed a consensus study aimed to define their optimal workout. METHODST:h e Delphi methodology was used to reach consensus among 47 Italian surgical experts in two study rounds. Consensus was defined as an agreement of 75.0% or greater. Four main topic areas included nosology, diagnosis, management and treatment. RESULTS: A bowel obstruction was defined as an obstacle to the progression of intestinal contents and flzrids generally beginning with a sudden onset. The panel identiJed four major criteria of diagnosis including absence of flatus, presence of > 3.5 cm ileal levels or > G cm colon dilatation and abdominal distension. Panel also recommended a surgical admission, a multidisciplinary approach, and a gastrografin swallow for patients presenting occlusions. Criteria for immediate surgery included: presence of strangulated hernia, a > 10 cm cecal dilatation, signs of vascular pedicles obstructions and persistence of metabolic acidosis. Moreover, rules for non-operative management (to be conducted for maximum 72 hours) included a naso-gastric drainage placement and clinical and laboratory controls each 12 hours. Non-operative treatment should be suspended if any suspects of intra-abdominal complications, high level of lactates, leukocytosis (> 18. 000/mm3 or Neutrophils > 85%) or a doubling of creatinine level comparing admission. Conversely, consensus was not reached regarding the exact timing of CT scan and the appropriateness of colonic stenting. CONCLUSIOTNhSis: consensus is in line with current international strategies and guidelines, and it could be a usefil tool in the safe basic ahily management of these common and peculiar diseases.


Scolamiero E.,CEINGE Biotecnologie Avanzate scarl | Cozzolino C.,CEINGE Biotecnologie Avanzate scarl | Albano L.,University of Naples Federico II | Ansalone A.,A.O. SantAnna e S. Sebastiano | And 33 more authors.
Molecular BioSystems | Year: 2015

Inborn errors of metabolism are genetic disorders due to impaired activity of enzymes, transporters, or cofactors resulting in accumulation of abnormal metabolites proximal to the metabolic block, lack of essential products or accumulation of by-products. Many of these disorders have serious clinical consequences for affected neonates, and an early diagnosis allows presymptomatic treatment which can prevent severe permanent sequelae and in some cases death. Expanded newborn screening for these diseases is a promising field of targeted metabolomics. Here we report the application, between 2007 and 2014, of this approach to the identification of newborns in southern Italy at risk of developing a potentially fatal disease. The analysis of amino acids and acylcarnitines in dried blood spots by tandem mass spectrometry revealed 24 affected newborns among 45 466 infants evaluated between 48 and 72 hours of life (overall incidence: 1 : 1894). Diagnoses of newborns with elevated metabolites were confirmed by gas chromatography-mass spectrometry, biochemical studies, and genetic analysis. Five infants were diagnosed with medium-chain acyl CoA dehydrogenase deficiency, 1 with methylmalonic acidemia with homocystinuria type CblC, 2 with isolated methylmalonic acidemia, 1 with propionic acidemia, 1 with isovaleric academia, 1 with isobutyryl-CoA dehydrogenase deficiency, 1 with beta ketothiolase deficiency, 1 with short branched chain amino acid deficiency, 1 with 3-methlycrotonyl-CoA carboxylase deficiency, 1 with formimino-transferase cyclodeaminase deficiency, and 1 with cystathionine-beta-synthase deficiency. Seven cases of maternal vitamin B12 deficiency and 1 case of maternal carnitine uptake deficiency were detected. This study supports the widespread application of metabolomic-based newborn screening for these genetic diseases. © The Royal Society of Chemistry.


De Rai P.,Fondazione Istit. di Ricovero e Cura a Carattere Scienti. Ca Granda Ospedale Maggiore Policlinico | Zerbi A.,IRCCS Instituto Clinico Humanitas Humanitas Clinical Institute | Castoldi L.,Fondazione Istit. di Ricovero e Cura a Carattere Scienti. Ca Granda Ospedale Maggiore Policlinico | Bassi C.,University of Verona | And 126 more authors.
HPB | Year: 2010

Objective: This study aimed to evaluate the surgical treatment of acute pancreatitis in Italy and to assess compliance with international guidelines. Methods: A series of 1173 patients in 56 hospitals were prospectively enrolled and their data analysed. Results: Twenty-nine patients with severe pancreatitis underwent surgical intervention. Necrosectomy was performed in 26 patients, associated with postoperative lavage in 70% of cases. A feeding jejunostomy was added in 37% of cases. Mortality was 21%. Of the patients with mild pancreatitis, 714 patients with a biliary aetiology were evaluated. Prophylactic treatment of relapses was carried out in 212 patients (36%) by cholecystectomy and in 161 using a laparoscopic approach. Preoperative endoscopic retrograde cholangiopancreatography was associated with cholecystectomy in 83 patients (39%). Forty-seven patients (22%) were treated at a second admission, with a median delay of 31 days from the onset of pancreatitis. Eighteen patients with severe pancreatitis underwent cholecystectomy 37.9 days after the first admission. There were no deaths. Discussion: The results indicate poor compliance with published guidelines. In severe pancreatitis, early surgical intervention is frequently performed and enteral feeding is seldom used. Only a small number of patients with mild biliary pancreatitis undergo definitive treatment (i.e. cholecystectomy) within 4 weeks of the onset of pancreatitis. © 2010 International Hepato-Pancreato-Biliary Association.


Catanzano F.,University of Sannio | Ombrone D.,University of Naples Federico II | Di Stefano C.,Ospedale Umberto i Nocera Inferiore | Rossi A.,Ospedale San Luca | And 8 more authors.
Journal of Inherited Metabolic Disease | Year: 2010

A pilot expanded newborn screening programme to detect inherited metabolic disorders by means of liquid chromatography coupled with tandem mass spectrometry (LC-MS/MS) began in the Campania region, southern Italy, in 2007. By October 2009, >8,800 dried blood samples on filter paper from 11 hospitals had been screened. Within this screening programme, we identified a case of mitochondrial acetoacetyl-coenzyme A (CoA) thiolase deficiency [β-ketothiolase (β-KT) deficiency] by analysing the acylcarnitine profile from a dried blood spot with LC-MS/MS. Gas chromatography coupled with mass spectrometry analysis of urinary organic acids and LC-MS/MS analysis of urinary acylcarnitines were in line with this disorder. In fact, concentrations were well beyond the cut-off values of tiglyl carnitine, 3- hydroxybutyrylcarnitine and 2-methyl-3-hydroxybutyrylcarnitine, 2-methyl-3-hydroxybutyric acid and tiglyl glycine. The absence of 2-methylacetoacetic acid in urine may be attributed to: (i) the instability of this β-ketoacid because it undergoes spontaneous decarboxylation to 2-butanone, which is highly volatile and thus difficult to detect, and (ii) the good health of the patient in the first days of life. β-KT deficiency was subsequently diagnosed in the patient's older sister, who showed increased levels of the same metabolites but also small amounts of 2-methylacetoacetic acid, which is considered a key marker for β-KT diagnosis. Genomic analysis revealed mutation c.1189C >G in exon 12 of the ACAT1 gene, which results in a severe defect because of the p.H397D amino acid change in both alleles of both patients. © The Author(s) 2010.


PubMed | University of Ferrara, Clinica Citta di Alessandria, University of Bologna, Ospedale degli Infermi and 7 more.
Type: Journal Article | Journal: Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology | Year: 2016

We investigated the applicability of the Ventricular Capture Control (VCC) and Atrial Capture Control (ACC) algorithms for automatic management of cardiac stimulation featured by Biotronik pacemakers in a broad, unselected population of pacemaker recipients.Ventricular Capture Control and Atrial Capture Control were programmed to work at a maximum adapted output voltage as 4.8 V in consecutive recipients of Biotronik pacemakers. Ambulatory threshold measurements were made 1 and 12 months after pacemaker implant/replacement in all possible pacing/sensing configurations, and were compared with manual measurements. Among 542 patients aged 80 (73-85) years, 382 had a pacemaker implant and 160 a pacemaker replacement. Ventricular Capture Control could work at long term in 97% of patients irrespectively of pacing indication, lead type, and lead service life, performance being superior with discordant pacing/sensing configurations. Atrial Capture Control could work in 93% of patients at 4.8 V maximum adapted voltage and at any pulse width, regardless of pacing indication, lead type, and service life. At 12-month follow-up, a ventricular threshold increase 1.5 V had occurred in 4.4% of patients uneventfully owing to VCC functioning. Projected pacemaker longevity at 1 month was strongly correlated with the 12-month estimate, and exceeded 13 years in >60% of patients.These algorithms for automatic management of pacing output ensure patient safety in the event of a huge increase of pacing threshold, while enabling maximization of battery longevity. Their applicability is quite broad in an unselected pacemaker population irrespectively of lead choice and service of life.

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