Etiology and management of pediatric intestinal failure: Focus on the non-digestive causes

Background: Intestinal failure (IF) is defined as reduction in functioning gut mass below the minimal amount necessary for adequate digestion and absorption. In most cases, IF results from intrinsic diseases of the gastrointestinal tract (digestive IF) (DIF); few cases arise from digestive vascular components, gut annexed (liver and pancreas) and extra-digestive organs or from systemic diseases (non-digestive IF) (NDIF). The present review revised etiology and treatments of DIF and NDIF, with special focus on the pathophysiological mechanisms, whereby NDIF develops. Methods: We performed a comprehensive search of published literature from January 2010 to the present by selecting the following search strings: “intestinal failure” OR “home parenteral nutrition” OR “short bowel syndrome” OR “chronic pseudo-obstruction” OR “chronic intestinal pseudo-obstruction” OR “autoimmune enteropathy” OR “long-term parenteral nutrition”. Results: We collected overall 1656 patients with well-documented etiology of IF: 1419 with DIF (86%) and 237 with NDIF (14%), 55% males and 45% females. Among DIF cases, 66% had SBS and among NDIF cases 90% had malabsorption/maldigestion. Conclusions: The improved availability of diagnostic and therapeutic tools has increased prevalence and life expectancy of rare and severe diseases responsible for IF. The present review greatly expands the spectrum of knowledge on the pathophysiological mechanisms through which the diseases not strictly affecting the intestine can cause IF. In view of the rarity of the majority of pediatric IF diseases, the development of IF Registries is strongly required; in fact, through information flow within the network, the Registries could improve IF knowledge and management

Efficacy of adalimumab as second-line therapy in a pediatric cohort of crohn’s disease patients who failed infliximab therapy: The Italian society of pediatric gastroenterology, hepatology, and nutrition experience

Background: Adalimumab (Ada) treatment is an available option for pediatric Crohn’s disease (CD) and the published experience as rescue therapy is limited. Objectives: We investigated Ada efficacy in a retrospective, pediatric CD cohort who had failed previous infliximab treatment, with a minimum follow-up of 6 months. Methods: In this multicenter study, data on demographics, clinical activity, growth, laboratory values (CRP) and adverse events were collected from CD patients during follow-up. Clinical remission (CR) and response were defined with Pediatric CD Activity Index (PCDAI) score ≤10 and a decrease in PCDAI score of ≥12.5 from baseline, respectively. Results: A total of 44 patients were consecutively recruited (mean age 14.8 years): 34 of 44 (77%) had active disease (mean PCDAI score 24.5) at the time of Ada administration, with a mean disease duration of 3.4 (range 0.3–11.2) years. At 6, 12, and 18 months, out of the total of the enrolled population, CR rates were 55%, 78%, and 52%, respectively, with a significant decrease in PCDAI scores (P<0.01) and mean CRP values (mean CRP 5.7 and 2.4 mL/dL, respectively; P<0.01) at the end of follow-up. Steroid-free remission rates, considered as the total number of patients in CR who were not using steroids at the end of this study, were 93%, 95%, and 96% in 44 patients at 6, 12, and 18 months, respectively. No significant differences in growth parameters were detected. In univariate analysis of variables related to Ada efficacy, we found that only a disease duration >2 years was negatively correlated with final PCDAI score (P<0.01). Two serious adverse events were recorded: 1 meningitis and 1 medulloblastoma. Conclusion: Our data confirm Ada efficacy in pediatric patients as second-line biological therapy after infliximab failure. Longer-term prospective data are warranted to define general effectiveness and safety in pediatric CD patients

Prevalence of hypertrophic cardiomyopathy (HCM) in feline population examined by the osservatorio italiano HCM felina

Hypertrophic cardiomyopathy (HCM) is the most common feline inherited cardiac disease and it is a major cause of morbidity and mortality. The Osservatorio Italiano HCM Felina was formed in 2008 by a network of clinicians, geneticists and breedings to monitor and study HCM in Italian cats. Since April 2008, 1308 adult cats, belonging to various breeds, including Maine Coon, Siberian, Norwegian Forest Cat, Ragdoll, Sphynx, British SH, Birmans and others have been prospectively enrolled. Recheck evaluations were performed in 287 cats. Each cat underwent a clinical examination, echocardiography, and blood collection for genetic testing (when appropriate) and storage in the Italian Feline Biobank. The disease status was defined by echocardiography according to established guidelines (left ventricular diastolic wall thickness<5.5mm = HCM negative, =5.5 but < 6mm = HCM equivocal, = 6mm = HCM positive). The prevalence of HCM in the population was 6% (74 cats); equivocal diagnoses were conferred on 4% (57 cats). These prevalences did not differ between breeds. The prevalence of HCM in the Italian feline population was lower compared to those reported by other investigators. Evaluation of data from the entire population demonstrated that left ventricular end-diastolic wall thickness and aortic diameter showed a weak positive correlation with body weight (p< 0.0001, r2<0.12 for all variables), suggesting that weight-dependent limits on wall thickness should be considered in cats as is currently practiced in dogs. The lower prevalence of HCM in Italian cat breeds compared with those examined elsewhere might be explained by different criteria for determining presence or absence of disease, differences in ages at which the subject were examined, or a selection bias by breeders in presenting cats they consider \u201cnormal\u201d

Inflammatory bowel disease in children and adolescents in Italy: data from the pediatric national IBD register (1996-2003).

Abstract BACKGROUND: The purpose was to assess in Italy the clinical features at diagnosis of inflammatory bowel disease (IBD) in children. METHODS: In 1996 an IBD register of disease onset was established on a national scale. RESULTS: 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. CONCLUSIONS: The IBD incidence in children and adolescents in Italy shows an increasing trend for all 3 pathologies. UC diagnoses exceeded CD

Transesophageal Echocardiography as the Sole Guidance for Occlusion of Patent Ductus Arteriosus using a Canine Ductal Occluder in Dogs

Background: Transcatheter occlusion of patent ductus arteriosus (PDA) is usually performed by fluoroscopy alone or together with transesophageal echocardiography (TEE). Transthoracic echocardiography (TTE) guidance has been used for deployment of Amplatz Canine Ductal Occluder (ACDO), but sometimes is limited by suboptimal acoustic windows. Transesophageal echocardiography can overcome such issues and provides higher image resolution at the level of the great vessels. Objectives: To determine if TEE without fluoroscopy could be used to successfully perform ductal occlusion for the treatment of PDA in dogs. Animals: Twenty client-owned dogs with PDA. Methods: A prospective consecutive case series of PDA occlusion was performed using only TEE guidance. Dogs were positioned in right lateral recumbency and the TEE probe was positioned to visualize the descending aorta, PDA, and pulmonary artery. The guide wire, long introducer sheath, and ACDO were imaged by TEE to direct deployment. Results: Ductal occlusion was performed successfully without need for fluoroscopy and without complications in 19 dogs. One dog required a second larger ACDO because of embolization of the first device 18 hours after positioning. Conclusions and Clinical Importance: We have demonstrated that TEE monitoring without concurrent fluoroscopy can guide each step of transcatheter ACDO embolization thereby providing an alternate method of visualization for this procedure. Use of TEE alone can reduce radiation exposure or is an option when fluoroscopy is not available, and, therefore, should be evaluated in a larger case series to better assess procedural failure rates