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Eye and Vision 2019年第1期6卷 1-5页

作者： Luca Buzzonetti Gianni Petrocelli Paola Valente sergio Petroni Rosa Parrilla Giancarlo Iarossi Ophthalmology Department Bambino GesùIRCCS Children’s HospitalVia Torre di Palidoro snc-00050 PassoscuroRomeItaly

Background:To evaluate refractive outcome 24 months after Deep Anterior Lamellar Keratoplasty(DALK)in pediatric patients by comparing results achieved using mechanical trephine and femtosecond ***:Twenty eyes of 20 patients affected by keratoconus were *** perform big-bubble DALK,10 eyes(Group 1;mean age 11.2±2.2 years)were subjected to the Hessburg-Barron mechanical trephine and the remaining 10 eyes(Group 2;mean age 11.3±3.1 years)to a 150 kHz femtosecond laser that performed mushroom *** thinnest point in the corneal thickness map and K readings were measured by the sirius scheimpflug *** also evaluated corrected distance visual acuity(CDVA)as logMAR value using spectacles,spherical equivalent and refractive ***:Mean preoperative thinnest point and pre-and post-operative K readings did not show significant difference(P>0.05)between the two ***,spherical equivalent and refractive astigmatism were respectively,0.14±0.08 logMAR and 0.13±0.10 logMAR(P=0.8),−4.2±1.1 D and−2.8±1.2 D(P=0.03),4.4±2.0 D and 3.6±1.2 D(P=0.4)in Groups 1 and *** DALK procedures were ***:Our findings suggest that femtosecond laser compared to mechanical trephine could significantly reduce the spherical equivalent amount in pediatric big-bubble DALK.

关键词： Pediatric keratoplasty Deep anterior lamellar keratoplasty Femtosecond laser

Combined endovascular-surgical treatment for complex congenital intrahepatic arterioportal fistula: A case report and review of the literature

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World Journal of Hepatology 2020年第4期12卷 160-169页

作者： Roberta Angelico Guglielmo Paolantonio Monica Paoletti Chiara Grimaldi Maria Cristina saffioti Lidia Monti Manila Candusso Massimo Rollo Marco spada Department of Abdominal Transplantation and Hepatobiliary and Pancreatic surgery Bambino GesùChildren’s Hospital IRCCSRome 00165Italy Department of surgery Hepato-bilio-pancreatic Surgery and Transplant UnitUniversity of Rome Tor VergataFondazione PVTRome 00133Italy Interventional Radiology Unit Department of ImagingBambino GesùChildren’s Hospital IRCCSRome 00165Italy Department of Imaging Bambino GesùChildren’s Hospital IRCCSRome 00165Italy Division of Hepatogastroenterology Bambino GesùChildren’s Hospital IRCCSRome 00165Italy

BACKGROUND Congenital intrahepatic arterioportal fistula(IAPF) is a rare vascular malformation in infants that causes severe portal hypertension(PH) with poor prognosis if untreated. Currently, radiological embolisation is considered the first-line therapy for simple IAPF; however, it might be not resolutive for complex hepatic vascular lesions. When endovascular embolization is not sufficient to completely obliterate the IAPF, surgical intervention is needed, but it has been associated with severe morbidity and mortality in small ***, indications are not *** sUMMARY We present the first case of a 6-month-old girl with trisomy 21 affected by a complex congenital IAFP, which caused severe PH, successfully treated with an endovascular-surgical hybrid procedure. The novel technique comprised a multistep endovascular embolisation, including a superselective transarterial embolisation of the afferent vessels and a direct transhepatic embolisation of the dilated portal vein segment, combined with selective surgical ligation of the arterial branches that supply the fistula, which were too small to be *** complex IAPF was also associated with severe cholestasis and intra/extrahepatic biliary tree dilatation, which was successfully treated by a temporary biliary drainage. At 24-mo follow-up, the hybrid endovascularsurgical procedure achieved complete occlusion of the complex IAPF and resolution of the PH. A comprehensive review of the literature on congenital IAPF management, focussed on alternative treatment strategies, is also *** The combined radiological-surgical approach is a safe and effective treatment option for complex IAPF and avoids major invasive surgery.

关键词： Liver Intrahepatic arterioportal fistula Congenital malformation Portal hypertension Radiological embolization Hepatic surgery Case report

Chronic pancreatitis as presentation of Crohn's disease in a child

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World Journal of Gastroenterology 2013年第31期19卷 5204-5206页

作者： Daniela Knafelz Fabio Panetta Lidia Monti Fiammetta Bracci Bronislava Papadatou Giuliano Torre Luigi Dall'Oglio Antonella Diamanti Hepatology Gastroenterology and Nutrition Unit Bambino Gesù Children’s Hospital 00165 Rome Italy Department of Radiology Hepato-bilio-pancreatic Imaging Unit Bambino Gesù Children’s Hospital 00165 RomeItaly Digestive surgery and Endoscopy Unit Bambino Gesù Children’s Hospital 00165 Rome Italy

It is reported that a pancreatic disease may precede the diagnosis of inflammatory bowel disease(IBD) both in children and in *** chronic pancreatitis,however,occasionally co-exists with the IBD,mainly at pediatric *** report a case of a patient who progressively developed the features of a chronic pancreatitis,before the diagnosis of Crohn's Disease(CD).Ten months after the onset of the first episode of pancreatitis the patient developed bloody diarrhea,mucus stools and biochemical findings of *** colonoscopy revealed a diffuse colitis without involvement of the last loop and the gastroscopy showed inflammation of the iuxta-papillary *** histological findings confirmed the diagnosis of CD that involved the colon and the *** conclusion,in children the idiopathic chronic pancreatitis may be an unusual presentation of ***,if other known cause of chronic pancreatitis are not found,a not invasive work up to exclude the IBD should be *** early coincidental diagnosis of the IBD may delay the progression of the pancreatic disease.

关键词： Inflammatory bowel disease Crohn’s Disease Pediatric age Bloody diarrhea Pancreatic disease

Pediatric non-alcoholic fatty liver disease:Preventive and therapeutic value of lifestyle intervention

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World Journal of Gastroenterology 2009年第48期15卷 6017-6022页

作者： Valerio Nobili Anna Alisi Massimiliano Raponi Liver Unit Research InstituteBambino Gesù Children's Hospital Liver Unit Research InstituteBambino Gesù Children’s Hospital Health Planning Department Research Institute Bambino Gesù Children’s Hospital

Nonalcoholic fatty liver disease(NAFLD),ranging from simple steatosis to nonalcoholic steatohepatitis(NAsH),and eventually cirrhosis and liver failure,is seen to be increasing amongst Western *** rates are rising in parallel with the epidemic of childhood obesity,and in particular,fatty liver evolves more easily in NAsH when poor dietary habits and sedentary lifestyle are *** fact,its general prevalence in the child population varies between 2.6% and 10%,but increases up to 80% in obese *** NAsH is expected to become the most common cause of pediatric chronic liver disease in the near future,there is broad interest amongst clinical researchers to move forward,both in diagnosis and ***,to date,the expensive and invasive procedure of liver biopsy is seen as the gold standard for NAsH diagnosis and few noninvasive diagnostic methods can be applied ***,there are still no approved pharmacological interventions for NAFLD/***,current management paradigms are based upon the presence of associated risk factors and aims to improve an individual's quality of life,thus reducing NAFLD-associated morbidity and ***,lifestyle intervention(diet and exercise) is the treatment of choice for NAFLD/ *** far,no study has evaluated the potential preventive effect of lifestyle intervention on children at risk of NAFLD/*** studies will be required in this area with the perspective of developing a national program to promote nutrition education and increase physical activity as means of preventing the disease in individuals at ***,we outline the clinical course,pathogenesis and management of NAFLD in children,highlighting the preventive and therapeutic value of lifestyle intervention.

关键词： Fatty liver children Lifestyle Diet Exercise Prevention

Classifying Cardiac Anomalies in Right and Left Isomerism:Concordant and Discordant Patterns

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Congenital Heart Disease 2023年第1期18卷 97-111页

作者： Lilia Oreto Giuseppe Mandraffino Paolo Ciliberti Teresa P.santangelo Placido Romeo Antonio Celona Placido Gitto Lorenzo Galletti Fiore s.Iorio Alfredo Di Pino Aurelio secinaro Paolo Guccione Robert H.Anderson salvatore Agati Mediterranean Pediatric Cardiology Center Bambino GesùChildren’s HospitalVia SirinaTaorminaItaly Internal Medicine Unit Department of Clinical and Experimental MedicineUniversity of MessinaMessinaItaly Department of Pediatric Cardiology and Cardiac surgery Bambino GesùChildren’s HospitalRomaItaly Advanced Cardiovascular Imaging Unit Department of ImagingBambino GesùChildren’s HospitalRomaItaly Radiology Department S.Vincenzo HospitalTaorminaItaly Biosciences Institute Newcastle UniversityNewcastle upon TyneUK

Aims:Evidence is emerging that,in the setting of isomerism,the atrial and bronchial arrangement are not always concordant,nor are these patterns always harmonious with the arrangement of the abdominal *** aimed to evaluate the concordance between these features in a cohort of patients with cardiac malformations in the setting of known isomerism,seeking to determine whether it was feasible to assess complexity on this basis,in this regard taking note of the potential value of bronchial as opposed to appendage *** and Results:We studied 78 patients known to have isomerism of the bronchuses,43 with right and 35 with left *** anatomy could be determined in 49 cases(63%),all but one of these being concordant with bronchial *** assessing abdominal features,in only 59 cases(76%)was splenic morphology in keeping with the thoracic *** expected,right isomerism was associated with greater complexity of cardiac malformations,with an odds ratio of 6.53,with confidence intervals from 2.2–19.3(ps were slightly decreased with thoraco-abdominal disharmony,when lesions shown to carry higher risk were then found in the setting of left ***:Harmony is excellent between bronchial and appendage isomerism,but less so with the arrangement of the abdominal *** isomerism in our cohort,was indicative of a sixfold increase in intracardiac *** discordance was found between the systems,however,the cardiac anomalies were less typical of the anticipated findings for right *** isomerism of the appendages.

关键词： Isomerism asplenia polysplenia congenital heart disease bronchial isomerism atrial appendages

Pediatric gastrointestinal bleeding: Perspectives from the Italian society of Pediatric Gastroenterology

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World Journal of Gastroenterology 2017年第8期23卷 1328-1337页

作者： Claudio Romano salvatore Oliva stefano Martellossi Erasmo Miele serena Arrigo Maria Giovanna Graziani sabrina Cardile Federica Gaiani Gian Luigi de'Angelis Filippo Torroni Unit of Pediatrics Department of Human Pathology in Adulthood and Childhood“G.Barresi”University of Messina Department of Pediatrics and Infantile Neuropsychiatry Pediatric Gastroenterology and Liver UnitSapienza University of Rome Institute for Maternal and Child Health irccs‘Burlo Garofalo’ Department of Translational Medical science Section of PediatricsUniversity of Naples“Federico II” Gastroenterology and Endoscopy Unit G.Gaslini Children’s Hospital-IRCCS Gastroenterology and Endoscopy Unit “San Giovanni Addolorata”Hospital Hepatology Gastroenterology and Nutrition UnitBambino GesùChildren’s Hospital Gastroenterology and Endoscopy Unit University of Parma Department of surgery and Transplantation Digestive Surgery and Endoscopy UnitBambino GesùChildren’s Hospital

There are many causes of gastrointestinal bleeding(GIB) in children, and this condition is not rare, having a reported incidence of 6.4%. Causes vary with age, but show considerable overlap; moreover, while many of the causes in the pediatric population are similar to those in adults, some lesions are unique to children. The diagnostic approach for pediatric GIB includes definition of the etiology, localization of the bleeding site and determination of the severity of bleeding; timely and accurate diagnosis is necessary to reduce morbidity and mortality. To assist medical care providers in the evaluation and management of children with GIB, the "Gastro-Ped Bleed Team" of the Italian society of Pediatric Gastroenterology, Hepatology and Nutrition(sIGENP) carried out a systematic search on MEDLINE via Pub Med(http://***/pubmed/) to identify all articles published in English from January 1990 to 2016; the following key words were used to conduct the electronic search: "upper GIB" and "pediatric" [all fields]; "lower GIB" and "pediatric" [all fields]; "obscure GIB" and "pediatric" [all fields]; "GIB" and "endoscopy" [all fields]; "GIB" and "therapy" [all fields]. The identified publications included articles describing randomized controlled trials, reviews, case reports, cohort studies, casecontrol studies and observational studies. References from the pertinent articles were also reviewed. This paper expresses a position statement of sIGENP that can have an immediate impact on clinical practice and for which sufficient evidence is not available in literature. The experts participating in this effort were selected according to their expertise and professional qualifications.

关键词： Gastrointestinal bleeding Endoscopy Lower gastrointestinal bleeding Upper gastrointestinal bleeding Pediatric

TGA/Chemometrics addressing innovative preparation strategies for functionalized carbon nanotubes

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Journal of Pharmaceutical Analysis 2020年第4期10卷 351-355页

作者： Roberta Risoluti Giuseppina Gullifa Elena Carcassi Andrea Masotti stefano Materazzi Department of Chemistry "Sapienza"University of Romep.le A.Moro 500185RomeItaly bambino gesùchildren’s hospital IRCCSResearch Laboratoriesv.le di San Paolo 1500146RomeItaly

In this work,functionalized carbon nanotubes(CNTs)using two polyamine polymers,polyethyleneimine(PEI)and polyamidoamine dendrimer(PAMAM),were investigated by thermal analysis in order to address preparation strategies to obtain low cytotoxic compounds with the ability to conjugate micro-RNAs and,at the same time,to transfect efficiently endothelial *** analysis(TGA)was coupled to chemometrics as a novel analytical strategy to characterize functionalized CNTs from different preparation *** particular,two starting materials were considered:very small CNTs and carboxylated CNTs(CNT-COOH)in order to examine the affinity with *** permitted to compare results from TGA and to investigate the effect of a number of factors affecting the synthesis of coated nanotubes including a different amount of involved polymer and the time required for the suspension for a satisfactory and reproducible preparation *** results demonstrated the effectiveness of TGA as a tool able to address synthesis of coated CNTs to be employed as efficient drug delivery vectors in biomedical applications.

关键词： Carbon nanotubes Thermogravimetry Chemometrics Polyethyleneimine(PEI) Polyamidoamine dendrimer(PAMAM)

Gastrointestinal neuromuscular apparatus: An underestimated target of gut microbiota

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World Journal of Gastroenterology 2016年第45期22卷 9871-9879页

作者： Michele Pier Luca Guarino Michele Cicala Lorenza Putignani Carola severi Digestive Disease Unit of Campus Bio Medico University of Rome 00128 Rome Italy Unit of Parasitology Bambino Gesù Children’s Hospital IRCCS 00100 Rome Italy Department of Internal Medicine and Medical specialties University Sapienza 00100 Rome Italy

Over the last few years, the importance of the resident intestinal microbiota in the pathogenesis of several gastrointestinal diseases has been largely investigated. Growing evidence suggest that microbiota can influence gastrointestinal motility. The current working hypothesis is that dysbiosis-driven mucosal alterations induce the production of several inflammatory/immune mediators which affect gut neuro-muscular functions. Besides these indirect mucosal-mediated effects, the present review highlights that recent evidence suggests that microbiota can directly affect enteric nerves and smooth muscle cells functions through its metabolic products or bacterial molecular components translocated from the intestinal lumen. Tolllike receptors, the bacterial recognition receptors, are expressed both on enteric nerves and smooth muscle and are emerging as potential mediators between microbiota and the enteric neuromuscular apparatus. Furthermore, the ongoing studies on probiotics support the hypothesis that the neuromuscular apparatus may represent a target of intervention, thus opening new physiopathological and therapeutic scenarios.

关键词： Microbiota Gastrointestinal motility smooth muscle Enteric nervous system Probiotics Irritable bowel syndrome

Current role of surgery for tuberous sclerosis complex-associated epilepsy

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Pediatric Investigation 2022年第1期6卷 16-22页

作者： Nicola specchio Giusy Carfi Pavia Luca de Palma Alessandro De Benedictis Chiara Pepi Marta Conti Carlo Efisio Marras Federico Vigevano Paolo Curatolo Rare and Complex Epilepsy Unit Department of NeurosciencesBambino GesùChildren’s HospitalIRCCSRomeItaly Neurosurgery Unit Department of NeurosciencesBambino GesùChildren’s HospitalIRCCSRomeItaly Department of Neurosciences Bambino GesùChildren’s HospitalIRCCSRomeItaly Child Neurology and Psychiatry Unit Systems Medicine DepartmentTor Vergata UniversityRomeItaly

Tuberous sclerosis complex(TsC)is a rare multisystem,autosomal dominant neurocutaneous syndrome in which epilepsy is the most common of several neurological and psychiatric *** two thirds of patients develop drug-resistant epilepsy for whom surgical resection of epileptogenic foci is indicated when seizures remain inadequately controlled following trial of two antiseizure *** challenge with presurgical and surgical approaches with patients with TsC is overcoming the complexity from the number of tubers and the multiplex epileptogenic network forming the epileptogenic *** suggest that seizure freedom is achieved by 55%-60%of patients,but predictive factors for success have remained elusive,which makes for unconfident selection of surgical *** article presents three different cases as illustrations of the potential challenges faced when assessing the suitability of TsC patients for epilepsy surgery.

关键词： Epilepsy Everolimus Imaging Prognostics surgery Tuberous sclerosis complex(TsC)

3D additive-manufactured nanocomposite magnetic scaffolds: Effect of the application mode of a time-dependent magnetic field on hMsCs behavior

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Bioactive Materials 2017年第3期2卷 138-145页

作者： Ugo D'Amora Teresa Russo Antonio Gloria Virginia Rivieccio Vincenzo D'Anto Giacomo Negri Luigi Ambrosio Roberto De santis Institute of Polymers Composites and BiomaterialsNational Research CouncilNaplesItaly Villa Betania Evangelical hospital NaplesItaly Department of Neuroscience Reproductive Sciences and Oral SciencesUniversity of Naples Federico IINaplesItaly Unit of Dentistry Bambino GesùChildren's HospitalIRCCSRomeItaly

Over the past few years,the influence of static or dynamic magnetic fields on biological systems has become a topic of considerable *** has recently been implicated to play significant roles in the regulation of cell responses and,for this reason,it is revolutionizing many aspects of healthcare,also suggesting new opportunities in tissue *** aim of the present study was to analyze the effect of the application mode of a time-dependent magnetic field on the behavior of human mesenchymal stem cells(hMsCs)seeded on 3D additivemanufactured poly(3-caprolactone)/iron-doped hydroxyapatite(PCL/FeHA)nanocomposite scaffolds.

关键词： Additive manufacturing scaffold Magnetic field Cell-material interaction