Publications by authors named "Capriati Teresa"

32 Publications

Cutaneous venous malformations as a clue for possible gastrointestinal tract involvement: diagnosis and treatment of six cases.

Dermatol Ther 2021 Mar 9:e14932. Epub 2021 Mar 9.

Dermatology Unit and Genodermatosis Unit, Genetics and Rare Diseases Research Division, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy VASCERN VASCA and ERN-Skin European Reference Centre.

Background: Venous malformation is the most common type among vascular malformations classified by International Society for the Study of Vascular Anomalies. Most venous malformations are sporadic (94%), caused in 40% of cases by somatic mutation of TEK gene. Venous malformations can be cutaneous, visceral or combined. Visceral involvement is rare, and gastrointestinal tract is the most common localization. Visceral venous malformations, usually asymptomatic, may manifest with bleeding, anaemia, consumptive coagulopathy, which sometimes require an emergency treatment.

Objectives: Our aim is to study a possible gastrointestinal involvement in patients with only one cutaneous venous malformation.

Methods: We collect a series of six patients who presented with a single cutaneous venous malformation and that subsequently manifested intestinal involvement at our Reference Centre for vascular anomalies since 2010.

Results: In our patients, cutaneous venous malformations were located on lower or upper limbs, and gastrointestinal involvement manifested from 3 to 10 years after skin diagnosis.

Conclusions: Our experience urges to early diagnose a gastrointestinal involvement also in patients with only one skin venous malformation and to prevent severe complications. A multidisciplinary approach is mandatory for the diagnosis and treatment of these patients. This article is protected by copyright. All rights reserved.
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http://dx.doi.org/10.1111/dth.14932DOI Listing
March 2021

Etiology and Management of Pediatric Intestinal Failure: Focus on the Non-Digestive Causes.

Nutrients 2021 Feb 27;13(3). Epub 2021 Feb 27.

Hepatology Gastroenterology and Nutrition Unit, "Bambino Gesù" Children Hospital, 00165 Rome, Italy.

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.
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http://dx.doi.org/10.3390/nu13030786DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7997222PMC
February 2021

Plasma and Red Blood Cell PUFAs in Home Parenteral Nutrition Paediatric Patients-Effects of Lipid Emulsions.

Nutrients 2020 Dec 5;12(12). Epub 2020 Dec 5.

Artificial Nutrition Division, Ospedale Bambino Gesù, 00165 Roma, Italy.

: Mixed lipid emulsions (LE) containing fish oil present several advantages compared to the sole soybean oil LE, but little is known about the safety of essential fatty acids (EFA) profile in paediatric patients on long-term Parenteral Nutrition (PN). : to assess glycerophosfolipid polyunsaturated fatty acids (PUFA) levels on plasma and red blood cell (RBC) membrane of children on long term PN with composite LE containing fish oil (SMOF), and to compare it with a group receiving olive oil LE (Clinoleic) and to the reference range for age, previously determined on a group of healthy children. : A total of 38 patients were enrolled, median age 5.56 (0.9-21.86) years, 15 receiving Clinoleic, 23 receiving SMOF. Patients on SMOF showed significantly higher levels of eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA), lower levels of arachidonic acid (ARA) and Mead acid (MEAD)/ARA ratio in plasma and RBC compared with patients on Clinoleic and with healthy children. Triene:tetraene (T:T) ratio of both groups of patients did not differ from that of healthy children-median plasma (MEAD/ARA: 0.01, interquartile rage (IQR) 0.01, = 0.61 and 0.02, IQR 0.02, = 0.6 in SMOF and Clinoleic patients, respectively), and was considerably lower than Holman index (>0.21). SMOF patients showed no statistically significant differences in growth parameters compared with Clinoleic patients. Patients of both groups showed stiffness class F0-F1 of liver stiffness measure (LSM) 5.6 (IQR 0.85) in SMOF patients and 5.3 (IQR 0.90) in Clinoleic patients, = 0.58), indicating absence of liver fibrosis. : Fatty acids, measured as concentrations (mg/L), revealed specific PUFA profile of PN patients and could be an accurate method to evaluate nutritional status and eventually to detect essential fatty acid deficiency (EFAD). SMOF patients showed significantly higher EPA, DHA and lower ARA concentrations compared to Clinoleic patients. Both LEs showed similar hepatic evolution and growth.
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http://dx.doi.org/10.3390/nu12123748DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7762095PMC
December 2020

Management of Enteral Nutrition in the Pediatric Intensive Care Unit: Prokinetic Effects of Amoxicillin/Clavulanate in Real Life Conditions.

Pediatr Gastroenterol Hepatol Nutr 2020 Nov 5;23(6):521-530. Epub 2020 Nov 5.

Artificial Nutrition Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

Purpose: Malnutrition is a common feature in critically ill children. Enteral nutrition (EN) is the main strategy to nutritionally support critical ill children, but its use can be hindered by the development of intolerance. The study aimed to assess the effectiveness and safety of amoxicillin/clavulanate (A/C) to treat EN intolerance.

Methods: We retrospectively evaluated patients admitted to the pediatric intensive care unit from October 2018 to October 2019. We conducted a case-control study: in the first 6 months (October 2018-April 2019) we implemented the nutritional protocol of our Institution with no drug, whereas in the second half (May 2019-October 2019) we employed A/C for 1 week at a dose of 10 mg/kg twice daily.

Results: Twelve cases were compared with 12 controls. At the final evaluation, enteral intake was significantly higher than that at baseline in the cases (from 2.1±3.7 to 66.1±27.4% of requirement, =0.0001 by Wilcoxon matched-pairs signed rank test) but not in the controls (from 0.2±0.8 to 6.0±14.1% of the requirement, =NS). Final gastric residual volume at the end of the observation was significantly lower in the cases than in the controls (=0.0398). The drug was well tolerated as shown by the similar safety outcomes in both cases and controls.

Conclusion: Malnutrition exposes critically ill children to several complications that affect the severity of disease course, length of stay, and mortality; all may be prevented by early EN. The development of intolerance to EN could be addressed with the use of A/C. Future prospective clinical trials are needed to confirm these conclusions.
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http://dx.doi.org/10.5223/pghn.2020.23.6.521DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7667232PMC
November 2020

Moving on: How to switch young people with chronic intestinal failure from pediatric to adult care. a position statement by italian society of gastroenterology and hepatology and nutrition (SIGENP) and italian society of artificial nutrition and metabolism (SINPE).

Dig Liver Dis 2020 10 29;52(10):1131-1136. Epub 2020 Aug 29.

Center for Chronic Intestinal Failure, Department of Digestive System, St. Orsola-Malpighi University Hospital, Bologna, Italy(SINPE).

In 2019 the Italian Society of Pediatric Gastroenterology, Hepatology and Nutrition (SIGENP) and the Italian Society of Artificial Nutrition and Metabolism (SINPE) created a joint panel of experts with the aim of preparing an official statement on transition in Chronic Intestinal Failure (CIF). The transition from pediatric to adult care has a key role in managing all chronic diseases and in optimizing the compliance to care. Thus SIGENP and SINPE, in light of the growing number of patients with IF who need long-term Parenteral Nutrition (PN) and multidisciplinary rehabilitation programs throughout adulthood, shared a common protocol to provide an accurate and timely process of transition from pediatric to adult centers for CIF. The main objectives of the transition process for CIF can be summarized as the so-called "acronym of the 5 M": 1)Motivate independent choices which are characteristics of the adult world; 2)Move towards adult goals (e.g. self-management of his pathology and sexual issues); 3)Maintain the habitual mode of care; 4) Minimize the difficulties involved in the transition process and 5)Modulate the length of the transition so as to fully share with the adult's team the children's peculiarities.
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http://dx.doi.org/10.1016/j.dld.2020.07.032DOI Listing
October 2020

To Wean or Not to Wean: The Role of Autologous Reconstructive Surgery in the Natural History of Pediatric Short Bowel Syndrome on Behalf of Italian Society for Gastroenterology, Hepatology and Nutrition (SIGENP).

Nutrients 2020 Jul 18;12(7). Epub 2020 Jul 18.

Artificial Nutrition Unit, Bambino Gesù Children Hospital, 00165 Rome, Italy.

Pediatric Short Bowel Syndrome (SBS) can require prolonged parenteral nutrition (PN). Over the years, SBS management has been implemented by autologous gastrointestinal reconstructive surgery (AGIR). The primary objective of the present review was to assess the effect of AGIR on weaning off PN. We also evaluated how AGIR impacts survival, the need for transplantation (Tx) and the development of liver disease (LD). We conducted a systematic literature search to identify studies published from January 1999 to the present and 947 patients were identified. PN alone was weakly associated with higher probability of weaning from PN (OR = 1.1, = 0.03) and of surviving (OR = 1.05, = 0.01). Adjusting for age, the probability of weaning off PN but of not surviving remained significantly associated with PN alone (OR = 1.08, = 0.03). Finally, adjusting for age and primary diagnosis (gastroschisis), any association was lost. The prevalence of TX and LD did not differ by groups. In conclusion, in view of the low benefit in terms of intestinal adaptation and of the not negligible rate of complications (20%), a careful selection of candidates for AGIR should be required. Bowel dilation associated with failure of advancing EN and poor growth, should be criteria to refer for AGIR.
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http://dx.doi.org/10.3390/nu12072136DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7400841PMC
July 2020

Non-interventional, retrospective data of long-term home parenteral nutrition in patients with benign diseases: Analysis of a nurse register (SERECARE).

Nutrition 2019 10 7;66:131-141. Epub 2019 Mar 7.

Ospedale San Nicola Pellegrino, Trani, Italy.

Objectives: The aim of this study was to evaluate the safety and efficacy of home parenteral nutrition (HPN) service in patients with benign chronic intestinal failure (CIF).

Methods: This was a 10-y retrospective, non-interventional, multicenter study conducted with adult and pediatric patients with CIF who received HPN service. We analyzed data prospectively collected from a dedicated register by HPN nurses.

Results: From January 2002 to December 2011 a total of 794 patients (49.7% male, median age 1 y for children and 57 y for adults) were included in the analysis. Over the 10-y period, 723 central venous catheter (CVC) complications occurred, of which 394 were infectious (54.5%), 297 were mechanical (41.1%), and 32 (3.3%) were defined as CVC-related thrombosis. The complication rate was higher in children (1.11 per patient) than in adults (0.70 per patient). During the observation period, the rates of both infectious and mechanical complications showed a global declining trend and ∼75% of patients had neither infectious nor mechanical CVC complications. HPN efficacy was evaluated in 301 patients with a minimum follow-up of 36 mo. Body mass index and Karnofsky score showed that the median growth significantly increased (P < 0.001) over baseline for adults and pediatric patients in the 0 to 2 age range.

Conclusions: The use of a structured register has proved to be a key strategy for monitoring the outcomes of long-term treatment, improving time efficiency, and preventing potential malpractice. To our knowledge, this is the largest survey ever documented; the results were consistent despite the heterogeneity of the centers because of duly applied standard rules and protocols.
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http://dx.doi.org/10.1016/j.nut.2019.01.006DOI Listing
October 2019

A Modified Holder Pasteurization Method for Donor Human Milk: Preliminary Data.

Nutrients 2019 May 22;11(5). Epub 2019 May 22.

Artificial Nutrition Unit, Bambino Gesù Children's Hospital, IRCCS, Piazza Sant'Onofrio 4, 00165 Rome, Italy.

Background: Holder pasteurization (HoP) is the recommended method of pasteurization for donor human milk (DHM). The aim of the present study was to compare nutritional and microbiological impact on DHM of a new technique of pasteurization based on technical changes of HoP.

Methods: We analyzed milk samples from 25 donors. Each sample, derived from one breast milk expression, was subdivided into three aliquots according to pasteurization: The first was not pasteurized, the second pasteurized by HoP, and the third was pasteurized by modified HoP (MHoP). Each aliquot was assessed as to its microbiological and nutritional profile. Nutritional profile included calcium and triglycerides concentrations detected by spectrophotometry and amino acid levels assessed by high-performance liquid chromatography (HPLC).

Results: Triglycerides were significantly lower in pasteurized, by both methods, than in not pasteurized aliquots, while calcium and amino acids concentration were similar. Microbiological profile did not differ between HoP and MHoP aliquots.

Conclusions: HoP and MHoP seem to have similar efficacy in preserving some nutritional characteristics of DHM and to confer similar microbiological safety. MHoP is time-saving and potentially costs-effective when compared to HoP, and it is; therefore, potentially of more interest from a practical point of view. Further studies are needed to confirm these findings.
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http://dx.doi.org/10.3390/nu11051139DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6566761PMC
May 2019

Pediatric Intestinal Pseudo-obstruction: Impact of Neonatal and Later Onset on Clinical and Nutritional Outcomes.

J Pediatr Gastroenterol Nutr 2019 08;69(2):212-217

Division of Neurogastroenterology and Motility, Department of Paediatric Gastroenterology, Great Ormond Street Hospital, NHS Foundation Trust, London, United Kingdom.

Objective: The aim of the study was to evaluate long-term nutritional outcomes and clinical characteristics in a cohort of children with pediatric intestinal pseudo-obstruction (PIPO) at neonatal-onset (NO-PIPO) and at later-onset (LO-PIPO).

Methods: All children fulfilling new PIPO criteria over a 30-year period were reviewed. Baseline demographic and clinical features as well as nutritional outcomes were collected. Nutritional outcomes included overall survival, prevalence of enteral autonomy and parenteral nutrition (PN) dependency, rate of major PN complications, and growth course.

Results: Forty-four patients were still alive at the end of the follow-up. Twenty-five patients (57%) achieved enteral autonomy, whilst 18 remained on PN. Among the patients requiring PN at the beginning of the study period, we found that 55% (CI 34-70) has the probability of remaining on PN at the latest follow-up. Prevalence of gastrointestinal obstruction symptoms (P < 0.01), urinary involvement (P < 0.05), stoma placements [gastrostomy (P < 0.01), ileostomy P < 0.05)] and complex gastrointestinal surgery (P < 0.05) were significantly higher in NO-PIPO than in LO-PIPO. The number of patients requiring long-term PN (P < 0.001) and the number of PN days (P < 0.05) were significantly higher in NO-PIPO, whilst the number of patients achieving enteral autonomy was significantly higher in LO-PIPO (P < 0.05).

Conclusions: In our study, we have reported the nutritional outcome of a cohort of children with PIPO over a 30-year period showing that about 20% of patients develop irreversible intestinal failure requiring life-long PN. Nutritional and clinical outcomes seem to be influenced by the time of onset of the disease.
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http://dx.doi.org/10.1097/MPG.0000000000002373DOI Listing
August 2019

New Nutritional and Therapeutical Strategies of NEC.

Curr Pediatr Rev 2019 ;15(2):92-105

Pediatric Department for the Treatment and Study of abdominal Disease and Abdominal Transplants, ISMETT-UPMC, Palermo, Italy.

Necrotizing enterocolitis (NEC) is an acquired severe disease of the digestive system affecting mostly premature babies, possibly fatal and frequently associated to systemic complications. Because of the severity of this condition and the possible long-term consequences on the child's development, many studies have aimed at preventing the occurrence of the primary events at the level of the bowel wall (ischemia and necrosis followed by sepsis) by modifying or manipulating the diet (breast milk versus formula) and/or the feeding pattern (time for initiation after birth, continuous versus bolus feeding, modulation of intake according clinical events). Feeding have been investigated so far in order to prevent NEC. However, currently well-established and shared clinical nutritional practices are not available in preventing NEC. Nutritional and surgical treatments of NEC are instead well defined. In selected cases surgery is a therapeutic option of NEC, requiring sometimes partial intestinal resection responsible for short bowel syndrome. In this paper we will investigate the available options for treating NEC according to the Walsh and Kliegman classification, focusing on feeding practices in managing short bowel syndrome that can complicate NEC. We will also analyze the proposed ways of preventing NEC.
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http://dx.doi.org/10.2174/1573396315666190313164753DOI Listing
March 2020

Autologous Intestinal Reconstructive Surgery in the Management of Total Intestinal Aganglionosis.

J Pediatr Gastroenterol Nutr 2019 05;68(5):635-641

Section of Pediatric Surgery, Pediatric Liver and Gut Research Group, Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.

Objectives: Total/near total intestinal aganglionosis (TIA/NTIA) is the most uncommon and life-threatening form of Hirschsprung disease (HD). The management of TIA/NTIA is challenging and the role of autologous intestinal reconstructive (AIR) surgery is controversial. The objective is to evaluate the effectiveness of AIR in patients with TIA/NTIA.

Methods: Records from children affected by TIA and enrolled in the multicenter international Pediatric Intestinal Rehabilitation and Transplantation Registry were retrospectively reviewed.

Results: Fourteen patients with TIA were identified. TIA diagnosis was confirmed histologically at the median age of 14 days of life. All received a proximal decompressive jejunostomy. Two patients died, 4 patients had satisfactory stoma output with enteral tolerance without additional procedures, 8 underwent 10 AIR procedures (4 Ziegler myotomy-myectomy, 3 transposition of aganglionic ileum with or without myotomy, 2 simple tapering, 1 longitudinal lengthening and tailoring procedure with associated myotomy). AIR significantly reduced median stoma output, from 197 to 31 mL · kg · day (P = 0.0001). The reduction was seen in all patients. In addition, AIR improved enteral tolerance in the long term in 5 of 8 patients (63%), and temporarily in 1, leading to a reduction of parenteral nutrition requirement from 100% to 70% (P = 0.0231).

Conclusions: AIR surgery in carefully selected patients may be useful and effective way to enhance residual bowel absorptive function and to reduce parenteral nutrition requirements. AIR and intestinal transplantation are complementary surgical tools in the complex treatment algorithm of TIA/NTIA.
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http://dx.doi.org/10.1097/MPG.0000000000002260DOI Listing
May 2019

Growth failure in Crohn's disease children: may the first treatment have a role?

Expert Rev Clin Immunol 2019 01 15;15(1):97-104. Epub 2018 Nov 15.

a Artificial Nutrition Unit , "Bambino Gesù" Children Hospital , Rome , Italy.

: Growth failure in children is a frequent feature of childhood-onset Crohn's disease (CD), and stunting can persist into adulthood. Growth is an important outcome by which to judge the effectiveness of therapies in children; currently available studies in CD children have focused on the short-term impact of treatments on growth, and there are limited data regarding the long-term effects of treatments upon growth. : We designed the present article to review whether the first treatment performed in newly diagnosed CD children may have a role on the future growth course. We conducted a systematic literature search to identify relevant studies published on the PubMed database from January 2002 up to now. We found only six surveys that documented mid-term growth course in newly diagnosed CD patients. : In the last years there have been relevant advances in the clinical management of CD children; however, there is a lack of knowledge about the best strategy to reverse growth failure. Children treated with enteral nutrition have appropriate height and weight gain but do not reverse the growth course. Further surveys are required to better explore not only clinical outcomes but also long-term growth course following each therapeutic strategy.
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http://dx.doi.org/10.1080/1744666X.2019.1543590DOI Listing
January 2019

An additional patient with a homozygous mutation in DCPS contributes to the delination of Al-Raqad syndrome.

Am J Med Genet A 2018 12 5;176(12):2781-2786. Epub 2018 Oct 5.

Genetics and Rare Diseases Research Division, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

DCPS gene encodes for a protein involved in gene expression regulation through promoting cap degradation during mRNA decapping processes. Mutations altering the DCPS function have been associated to a distinct disorder, Al-Raqad syndrome, so far described only in two families. We report on a patient harboring a novel homozygous missense mutation in DCPS, presenting with growth retardation, craniofacial anomalies, skin dyschromia, and neuromuscular defects. This case study explains the molecular spectrum of DCPS mutations and might contribute to the phenotypic delineation of this rare condition.
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http://dx.doi.org/10.1002/ajmg.a.40488DOI Listing
December 2018

Paediatric Home Artificial Nutrition in Italy: Report from 2016 Survey on Behalf of Artificial Nutrition Network of Italian Society for Gastroenterology, Hepatology and Nutrition (SIGENP).

Nutrients 2018 Sep 16;10(9). Epub 2018 Sep 16.

Artificial Nutrition Unit Bambino Gesù, Children's Hospital, IRCCS, 00165 Rome, Italy.

Home Artificial Nutrition (HAN) is a safe and efficacious technique that insures children's reintegration into the family, society and school. Epidemiological data on paediatric HAN in Italy are not available.

Aim: to detect the prevalence and incidence of Home Parenteral Nutrition (HPN) and Home Enteral Nutrition (HEN), either via tube or mouth, in Italy in 2016.

Materials And Methods: a specific form was sent to all registered SIGENP members and investigators of local HAN centres, inviting them to provide the requested centre's data and demographics, underlying diseases and HAN characteristics of the patients.

Results: we recorded 3403 Italian patients on HAN aged 0 to 19 years from 22 centres: 2277 HEN, 950 Oral Nutritional Supplements (ONS) and 179 HPN programs. The prevalence of HEN (205 pts/million inhabitants) and HPN (16 pts/million inhabitants) has dramatically increased in Italy in the last 9 years. Neurodisabling conditions were the first indication for HEN by tube or mouth while HPN is mainly requested in digestive disorders.

Conclusions: HAN is a widespread and rapidly growing treatment in Italy, as well as in other European countries. Awareness of its extent and characteristics helps improving HAN service and patients' quality of life.
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http://dx.doi.org/10.3390/nu10091311DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6163787PMC
September 2018

Prevalence and outcome of malnutrition in pediatric patients with chronic diseases: Focus on the settings of care.

Clin Nutr 2019 08 27;38(4):1877-1882. Epub 2018 Jul 27.

Medical Direction, Pediatric Hospital "Bambino Gesù", IRCCS, Rome, Italy.

Background: Information on disease-related malnutrition and related outcomes in pediatric patients with chronic diseases in different settings of care is not available.

Methods: Consecutive eligible patients attending the out-patient clinic (n = 177) or admitted to the day-hospital clinic (n = 163) or to hospital (n = 201) were screened for the presence of malnutrition (BMI and/or height/length for age z-scores < -2). We recorded data on emergency care admissions to hospital that occurred during the 3 years before screening and related total days of stay, as well as data on emergency care admissions to hospital occurring within 6 months after screening.

Results: Prevalence of malnutrition was 2-fold higher (P < 0.001) in in-patients (56.7% [95% CI, 49.6-63.7]) than in patients assessed at the out-patient (33.3% [95% CI, 26.4-40.8]) and day-hospital (28.3% [95% CI, 21.5-35.8]) clinics. Estimates were heterogeneous across diagnostic groups with higher rates in patients with neurologic (61%) and cardiac (56%) diseases. Stunting was more frequent among in-patients, who also had more evident nutritional derangements. Multivariate logistic regression (covariates: age, gender, healthcare setting and disease group), showed that malnutrition (OR = 1.86 [95% CI, 1.21-2.88]; P = 0.005) was significantly associated with prolonged hospitalization (≥15 days) in the 3 years before screening. In-patients were also more likely to have been hospitalized ≥15 days (using out-patients as reference category, OR = 2.24 [95% CI, 1.39-3.63], P = 0.001), but we did not find any modifying effect (interaction) of the setting of care on the association between malnutrition and prolonged hospitalization.

Discussion: The rates of malnutrition in children with chronic diseases are very high and increase hospital care needs, especially when they are admitted to hospital. Nutritional care in this patient population is recommended.
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http://dx.doi.org/10.1016/j.clnu.2018.07.008DOI Listing
August 2019

Pediatric Chronic Intestinal Failure in Italy: Report from the 2016 Survey on Behalf of Italian Society for Gastroenterology, Hepatology and Nutrition (SIGENP).

Nutrients 2017 Nov 5;9(11). Epub 2017 Nov 5.

Pediatric Surgery Unit, Women's and Children's Health Department, University of Padua, 35122 Padua, Italy.

Background: Intestinal failure (IF) is the reduction in functioning gut mass below the minimal level necessary for adequate digestion and absorption of nutrients and fluids for weight maintenance in adults or for growth in children. There is a paucity of epidemiologic data on pediatric IF. The purpose of this study was to determine the prevalence, incidence, regional distribution and underlying diagnosis of pediatric chronic IF (CIF) requiring home parenteral nutrition (HPN) in Italy.

Methods: Local investigators were selected in 19 Italian centers either of reference for pediatric HPN or having pediatric gastroenterologists or surgeons on staff and already collaborating with the Italian Society for Pediatric Gastroenterology, Hepatology and Nutrition with regard to IF. Data requested in this survey for children at home on Parenteral Nutrition (PN) on 1 December 2016 included patient initials, year of birth, gender, family's place of residence and underlying diagnosis determining IF.

Results: We recorded 145 CIF patients on HPN aged ≤19 years. The overall prevalence was 14.12/million inhabitants (95% CI: 9.20-18.93); the overall incidence was 1.41/million inhabitant years (95% CI: 0.53-2.20).

Conclusion: Our survey provides new epidemiological data on pediatric CIF in Italy; these data may be quantitatively useful in developing IF care strategy plans in all developed countries.
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http://dx.doi.org/10.3390/nu9111217DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5707689PMC
November 2017

Pediatric Short Bowel Syndrome: Predicting Four-Year Outcome after Massive Neonatal Resection.

Eur J Pediatr Surg 2018 Oct 18;28(5):455-463. Epub 2017 Jul 18.

Artificial Nutrition Unit, Ospedale Pediatrico Bambino Gesu, Roma, Italy.

Objectives:  The aim of this study was to ascertain predictors of survival, liver disease (LD), and enteral autonomy 48 months after resection in neonatal short bowel syndrome (SBS) patients with residual small bowel length (SBL) ≤40 cm.

Patients And Methods:  Medical records of all SBS patients followed up between 1996 and 2016 were retrospectively reviewed. Survival rate, prevalence of LD, and of enteral autonomy were evaluated.

Results:  Forty-seven patients were included, and 43 were still alive at the end of the study period, with cumulative 48-month survival of 91.5%. Twenty-one (45%) patients developed LD, all within the first 6 months. On the final follow-up visit, three (6%) patients were still jaundiced and progressed toward end-stage LD. LD prevalence was higher in patients with recurrent bloodstream infections (odds ratio [OR] 5.4, 95% confidence interval [CI] 1.5-19.3). Of the 43 surviving patients, 22 (51%) had enteral autonomy 48 months after resection. The probability of weaning off parenteral nutrition (PN) was strongly correlated with the remaining SBL.

Conclusion:  Survival of patients who have undergone neonatal massive small bowel resection has improved in recent years. Multidisciplinary strategies can improve the course of LD, but not the probability of weaning off PN, which seems to be strongly dependent on the anatomical profile of residual bowel. Therefore, the primary surgical approach should be as conservative as possible to gain even small amounts of intestinal length, which may be crucial in promoting intestinal adaptation.
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http://dx.doi.org/10.1055/s-0037-1604113DOI Listing
October 2018

Enteral nutrition in pediatric intestinal failure: does initial feeding impact on intestinal adaptation?

Expert Rev Gastroenterol Hepatol 2017 Aug 8;11(8):741-748. Epub 2017 Jun 8.

a Department of Pediatrics , Pediatric Hospital "Bambino Gesù" , Rome , Italy.

Introduction: Primary IF can be due to impaired gut length or impaired gut function; short bowel syndrome (SBS) is the leading cause of IF. In IF patients complete enteral starvation should be avoided whenever possible and enteral/oral nutrition (EN/ON) should be employed at the maximum tolerated amount in each phase of the clinical evolution of IF. Intraluminal nutrients have stimulatory effects on epithelial cells and on trophism that enhance intestinal adaptation. Areas covered: Evidence for nutritional interventions in pediatric IF is limited and of poor quality. Clinical practice in SBS feeding are more 'experience-based' rather than 'evidence-based' and this dearth of clinical evidence is partly due to the rarity of this condition. This review updates knowledge concerning the impact of the initial diet with EN/ON in neonatal onset SBS in the process of bowel adaption. Expert commentary: Human milk resulted the preferred starting diet and it is generally combined with amino-acids (AAs) in Northern America and with hydrolyzed proteins (HFs) in Europe; polymeric diet is rarely employed. HFs were not more effective than AAs in promoting intestinal adaptation.
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http://dx.doi.org/10.1080/17474124.2017.1335196DOI Listing
August 2017

Pediatric inflammatory bowel disease: specificity of very early onset.

Expert Rev Clin Immunol 2016 09 31;12(9):963-72. Epub 2016 May 31.

a Artificial Nutrition Unit and Hepato-Metabolic Disease Unit , Pediatric Hospital Bambino Gesu , Rome , Italy.

Introduction: The incidence of inflammatory bowel disease (IBD) has increased over the last 50 years. It is now recognized that several genetic defects can express an IBD-like phenotype at very early onset (<6 years).

Areas Covered: The aim of this review was to update knowledge concerning the specificity of IBD at onset <6 years, which can include conventional/standard IBD as well as monogenic IBD-like diseases. Expert commentary: We found that females are less prone than males to develop monogenic disorders, which have X-linked heritability in several cases. Furthermore, the Crohn's Diseases (CD) subtype seems to be suggestive of monogenic disorders while Unclassified IBD (IBDU) subtype is predominantly found in conventional/standard IBD at onset <6 years. Isolated colonic location is prevalent in both the subsets of IBD at onset <6 years if compared to IBD at later onset. Monogenic disorders require more aggressive medical and surgical treatments and can be complicated by the occurrence of lymphomas.
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http://dx.doi.org/10.1080/1744666X.2016.1184571DOI Listing
September 2016

From an imbalance to a new imbalance: Italian-style gluten-free diet alters the salivary microbiota and metabolome of African celiac children.

Sci Rep 2015 Dec 18;5:18571. Epub 2015 Dec 18.

Department of Soil, Plant and Food Sciences, University of Bari Aldo Moro, Bari, 70126, Italy.

Fourteen Saharawi celiac children following an African-style gluten-free diet for at least two years were subjected to a change of diet to an Italian-style gluten-free diet for 60 days. Significant differences were identified in the salivary microbiota and metabolome when Saharawi celiac children switched from African- to Italian-style dietary habits. An Italian-style gluten-free diet caused increases in the abundance of Granulicatella, Porphyromonas and Neisseria and decreases in Clostridium, Prevotella and Veillonella, altering the 'salivary type' of the individuals. Furthermore, operational taxonomic unit co-occurrence/exclusion patterns indicated that the initial equilibrium of co-occurring microbial species was perturbed by a change in diet: the microbial diversity was reduced, with a few species out-competing the previously established microbiota and becoming dominant. Analysis of predicted metagenomes revealed a remarkable change in the metabolic potential of the microbiota following the diet change, with increased potential for amino acid, vitamin and co-factor metabolism. High concentrations of acetone and 2-butanone during treatment with the Italian-style gluten-free diet suggested metabolic dysfunction in the Saharawi celiac children. The findings of this study support the need for a translational medicine pipeline to examine interactions between food and microbiota when evaluating human development, nutritional needs and the impact and consequences of westernisation.
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http://dx.doi.org/10.1038/srep18571DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4683525PMC
December 2015

Autoimmune diseases and celiac disease which came first: genotype or gluten?

Expert Rev Clin Immunol 2016 16;12(1):67-77. Epub 2015 Oct 16.

d 4 Scientific Directorate, Pediatric Hospital "Bambino Gesù", Rome, Italy.

Celiac disease (CD) is associated with several autoimmune diseases (ADs) and, in particular, thyroid autoimmunity (TA) and Type 1 diabetes (T1D). TA and T1D are defined as 'associated conditions' to CD (conditions at increased prevalence in CD but not directly related to gluten ingestion). The diagnosis of CD may precede or follow that of TA/T1D. To date, the available evidence suggests that the common genetic background is the main factor determining the high prevalence of the association. Conversely, no conclusive findings clarify whether extrinsic gluten-related factors (age at the first introduction, concomitant breastfeeding, length of gluten exposure and gluten-free diet) may link CD to the ADs. The aim of this review is to evaluate whether genetic background alone could explain the association between CD and ADs or if gluten-related factors ought to be considered. The pathophysiological links clarifying how the gluten-related factors could predispose to ADs will also be discussed.
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http://dx.doi.org/10.1586/1744666X.2016.1095091DOI Listing
August 2016

Impact of the birth's season on the development of celiac disease in Italy.

Eur J Pediatr 2015 Dec 5;174(12):1657-63. Epub 2015 Jul 5.

Gastroenterology-Hepatology and Nutrition Unit, "Bambino Gesù" Children's Hospital, Piazza Sant'Onofrio 4, Rome, 00165, Italy.

Unlabelled: Celiac disease (CD) is an immune-mediated systemic disorder induced by a trigger factor in genetically susceptible individuals. There is emerging evidence about the impact of the month of birth on the development of several autoimmune diseases. Our aim was to investigate whether, in Italian CD children, the season of birth is associated with development of CD later in life. We report a survey conducted at two Italian referral centers for CD in Rome and Bari. The CD database was created to enable retrospective examination of the data of all the consecutive patients, born between 2003 and 2010, who had received a diagnosis of CD. This CD patient group comprising 596 children was compared with a reference group that included all subjects born in the same period and in the same cities (439,990 controls). Overall, there was a summer birth preponderance in CD patients compared to controls (28.2 % of CD patients vs 23.0 % of the control population; OR 1.315; 95 % CI 1.100 to 1.572). Stratifying the caseload by gender and age, the summer birth preponderance was maintained for females (28.6 % CD females vs 22.6 % control females; OR 1.368; 95 % CI 1.069 to 1.750).

Conclusions: our survey confirms that in Italy, children born in summer are at higher risk to develop CD than subjects born in other seasons. The identification of a responsible seasonal factor or factors, such as timing of the first introduction of gluten and/or acute viral gastrointestinal infections, would be very important for disease prevention strategies.

What Is Known: • Environmental factors could be involved in the pathogenesis of CD. • Data about the impact of season of birth on CD development is so far derived from North American, Northern European and Israeli surveys.

What Is New: • This is the first study in Southern Europe to find a relationship between season of birth (summer) and development of CD. • Summer-born infants are introduced to complementary feeding (gluten) in winter, when the rotavirus infection is at its highest peak; this may be the link between season of birth and development of CD.
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http://dx.doi.org/10.1007/s00431-015-2589-2DOI Listing
December 2015

Overweight and Pediatric Inflammatory Disease: Is It a True Association?

J Pediatr Gastroenterol Nutr 2015 Aug;61(2):e10

Hepatology, Gastroenterology and Nutrition Unit, Bambino Gesù Children's Hospital, Rome, Italy.

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http://dx.doi.org/10.1097/MPG.0000000000000843DOI Listing
August 2015

Clinical management of post-pyloric enteral feeding in children.

Expert Rev Gastroenterol Hepatol 2015 Jul 30;9(7):929-41. Epub 2015 Apr 30.

Hepatology, Gastroenterology and Nutrition Unit, Bambino Gesù Children's Hospital, Piazza S. Onofrio 4, 00165 Rome, Italy.

Post-pyloric feeding (PF) allows the administration of enteral nutrition beyond the pylorus, either into the duodenum or, ideally, into the jejunum. The main indications of PF are: upper gastrointestinal tract obstructions, pancreatic rest (e.g., acute pancreatitis), gastric dysmotility (e.g., critically ill patients and chronic intestinal pseudo-obstruction) or severe gastroesophageal reflux with risk of aspiration (e.g., neurological disability). Physiological and clinical evidence derives from adults, but can also be pertinent to children. This review will discuss the practical management and potential clinical applications of PF in pediatric patients. Some key studies pertaining to the physiological changes during PF will also be considered because they support the strategy of PF management.
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http://dx.doi.org/10.1586/17474124.2015.1041506DOI Listing
July 2015

The clinical implications of thalidomide in inflammatory bowel diseases.

Expert Rev Clin Immunol 2015 Jun 11;11(6):699-708. Epub 2015 Apr 11.

Hepatology, Gastroenterology and Nutrition Unit, Bambino Gesù Children's Hospital, Piazza S. Onofrio 4, 00165 Rome, Italy.

Thalidomide has anti-inflammatory and anti-angiogenetic activity that makes it suitable for treating inflammatory bowel diseases (IBD). The recent guidelines from the European Crohn's and Colitis Organization/European Society for Pediatric Gastroenterology Hepatology and Nutrition conclude that thalidomide cannot be recommended in refractory pediatric Crohn's disease but that it may be considered in selected cohorts of patients who are not anti-TNFα agent responders. The main adverse effect is the potential teratogenicity that renders the long-term use of thalidomide problematic in young adults due to the strict need for contraceptive use. In short-term use it is relatively safe; the most likely adverse effect is the neuropathy, which is highly reversible in children. So far the use of thalidomide is reported in 223 adult and pediatric IBD patients (206 with Crohn's disease). In the following sections, the authors will discuss efficacy and safety of thalidomide, in the short-term treatment of IBD.
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http://dx.doi.org/10.1586/1744666X.2015.1027687DOI Listing
June 2015

Fat-soluble vitamin deficiency in children with intestinal failure receiving home parenteral nutrition.

J Pediatr Gastroenterol Nutr 2014 Nov;59(5):e46

*Surgical Department †Laboratory Department, Pediatric Hospital "Bambino Gesù," IRCCS, Rome, Italy.

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http://dx.doi.org/10.1097/MPG.0000000000000508DOI Listing
November 2014

Citrulline as marker of atrophy in celiac disease.

Intern Emerg Med 2014 Sep 8;9(6):705-7. Epub 2014 May 8.

Hepatology, Gastroenterology and Nutrition Unit, Bambino Gesù Children's Hospital, Piazza S. Onofrio 4, 00165, Rome, Italy.

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http://dx.doi.org/10.1007/s11739-014-1074-7DOI Listing
September 2014

Increased prevalence of celiac disease among pediatric patients with irritable bowel syndrome: a 6-year prospective cohort study.

JAMA Pediatr 2014 Jun;168(6):555-60

Interdisciplinary Department of Medicine, Pediatric Section, University of Bari, Giovanni XXIII Hospital, Bari, Italy.

Importance: Recurrent abdominal pain is a prevalent health issue in childhood. Clinical criteria (ie, the Rome criteria) have been established to aid diagnosis. Studies of adults have shown an increased prevalence of celiac disease among patients with irritable bowel syndrome (IBS); few data are available with regard to children.

Objective: To assess the prevalence of celiac disease among children with abdominal pain-related functional gastrointestinal disorders classified according to the Rome criteria.

Design, Setting, Participants: Six-year (2006-2012) prospective cohort study conducted in a tertiary referral center for the diagnosis and follow-up of gastrointestinal disorders in southern Italy (ie, Bari, Italy). A total of 992 children (42.8% male; median age, 6.8 years) consecutively referred for recurrent abdominal pain by their primary care physicians without previous investigation were evaluated.

Exposure: Patients were classified according to Rome III criteria as having IBS, functional dyspepsia, functional abdominal pain, or abdominal migraine.

Main Outcomes And Measures: Prevalence of celiac disease in each category of abdominal pain-related functional gastrointestinal disorder. Concentrations of IgA, IgA antitissue transglutaminase, and endomysial antibodies were measured, and a duodenal biopsy was performed in case of antibody positivity.

Results: A total of 992 children were evaluated: 270 were classified as having IBS, 201 as having functional dyspepsia, and 311 as having functional abdominal pain, and 210 children were excluded from the study because they had an organic disorder or some other functional gastrointestinal disorder (not related to abdominal pain). Serologic testing was performed for all 782 children included in the study, and 15 patients tested positive for celiac disease (12 of 270 patients with IBS [4.4%], 2 of 201 patients with functional dyspepsia [1%], and 1 of 311 patients with functional abdominal pain [0.3%]). Children presenting with IBS have a 4 times higher risk of having celiac disease than children without IBS (odds ratio, 4.19 [95% CI, 2.03-8.49]; P < .001).

Conclusions And Relevance: The prevalence of celiac disease among children with IBS is 4 times higher than among the general pediatric population. Rome III classification of abdominal pain-related functional gastrointestinal disorders might help to select children who deserve screening for celiac disease.
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http://dx.doi.org/10.1001/jamapediatrics.2013.4984DOI Listing
June 2014

Recurrent catheter related bloodstream infections by Candida glabrata: successful treatment with taurolidine.

Clin Nutr 2014 Apr 21;33(2):367. Epub 2014 Jan 21.

Pharmacy Service, Bambino Gesù Children's Hospital, Piazza S. Onofrio 4, 00165 Rome, Italy.

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http://dx.doi.org/10.1016/j.clnu.2014.01.010DOI Listing
April 2014

Celiac disease and overweight in children: an update.

Nutrients 2014 Jan 2;6(1):207-20. Epub 2014 Jan 2.

Gastroenterology Unit, Pediatric Clinic of University, Piazza Giulio Cesare 11, Bari 70124, Italy.

The clinical presentation of celiac disease in children is very variable and differs with age. The prevalence of atypical presentations of celiac disease has increased over the past 2 decades. Several studies in adults and children with celiac disease indicate that obesity/overweight at disease onset is not unusual. In addition, there is a trend towards the development of overweight/obesity in celiac patients who strictly comply with a gluten-free diet. However, the pathogenesis and clinical implications of the coexistence of classic malabsorption (e.g., celiac disease) and overweight/obesity remain unclear. This review investigated the causes and main clinical factors associated with overweight/obesity at the diagnosis of celiac disease and clarified whether gluten withdrawal affects the current trends of the nutritional status of celiac disease patients.
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http://dx.doi.org/10.3390/nu6010207DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3916856PMC
January 2014