Teresa Capriati

Long-term outcome of home parenteral nutrition in patients with ultra-short bowel syndrome.

Objective: The patients with ultra-short bowel syndrome (U-SBS) have been considered potential candidates for a preemptive/rehabilitative intestinal transplantation owing to the high risk of death from the underlying disease. We hypothesized that children with U-SBS, in the absence of intestinal failure-associated liver disease (IFALD), could also have a good rate of survival on home parenteral nutrition (HPN). Methods: A prospective database from the “Bambino Gesù” Artificial Nutrition and Intestinal Failure Program was used to evaluate outcomes and morbidities of consecutive patients with ⩽10 cm of small bowel enrolled since 2000. Results: Eleven patients were identified with a median bowel length of 7.5 (3–9) cm. Eight patients developed IFALD, which reversed in 7 of them; the IFALD progressively worsened in 1 patient until death. One patient underwent isolated intestinal transplantation and 1 patient is no longer receiving parenteral nutrition (PN) and both are fully enterally fed. The other patients remained at least partially dependent on HPN. The number of days of inpatient care decreased in all of the patients except for the 1 who had repeated episodes of central line infections. Conclusions: The survival of patients with U-SBS receiving HPN was good. Although IFALD was frequent, it had been manageable in most of the patients, but in a single complex case, it led to death. The multidisciplinary management warranted to these patients to approach the school age, to grow, and to maintain the oral intake. Patients with U-SBS are rare, and to better understand their long-term survival, further studies, including more large patient populations, are required.

Celiac Disease and Overweight in Children: An Update

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.

Increased Prevalence of Celiac Disease Among Pediatric Patients With Irritable Bowel Syndrome: A 6-Year Prospective Cohort Study

IMPORTANCEnRecurrent 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.nnnOBJECTIVEnTo assess the prevalence of celiac disease among children with abdominal pain-related functional gastrointestinal disorders classified according to the Rome criteria.nnnDESIGN, SETTING, PARTICIPANTSnSix-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.nnnEXPOSUREnPatients were classified according to Rome III criteria as having IBS, functional dyspepsia, functional abdominal pain, or abdominal migraine.nnnMAIN OUTCOMES AND MEASURESnPrevalence 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.nnnRESULTSnA 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]; Pu2009<u2009.001).nnnCONCLUSIONS AND RELEVANCEnThe 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.

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

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.

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

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.

Pediatric inflammatory bowel disease: specificity of very early onset

ABSTRACT 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.

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

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.

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

T o the Editor: Ubesie et al (1) reported a high prevalence of vitamin D deficiency (39.8%) in children with intestinal failure (IF). We routinely monitored vitamin A, E, and D levels every 6 months for children who were receiving home parenteral nutrition for IF, following European Society for Pediatric Gastroenterology, Hepatology, and Nutrition guidelines (2). In 2013, we completed a cross-sectional assessment of 32 home parenteral nutrition patients who had fat-soluble vitamin levels measured for 1 year. Twenty children with nonsurgical IF (dysmotility and malabsorption) (group A) and 12 with surgical IF (short bowel syndrome) (group B) were included in the study. All of the patients received daily parenteral nutrition with a standard pediatric multivitamin formulation containing vitamin A, 3500 IU; vitamin D, 250 IU; and vitamin E, 11.20 IU. Vitamin levels were low in groups A and B, respectively, as follows: vitamin A in 5 (25%) and 4 (30%), vitamin D in 9 (45%) and 4 (30%), and vitamin E in 2 (10%) and 0. Overall, 15 patients in group A (75%) and 6 patients in group B (50%) showed low levels of fat-soluble vitamins. The likelihood of low fat-soluble vitamin levels was significantly greater in nonsurgical IF than in patients with short bowel syndrome (odds ratio 6, 95% confidence interval 1.5–24.5). Thus, we agree that routine surveillance of fat-soluble vitamins and consideration of enteral or parenteral supplementation, including periodic intramuscular injection, may be useful in children with IF.

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

ABSTRACT 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.

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

BACKGROUNDnInformation on disease-related malnutrition and related outcomes in pediatric patients with chronic diseases in different settings of care is not available.nnnMETHODSnConsecutive eligible patients attending the out-patient clinic (nxa0=xa0177) or admitted to the day-hospital clinic (nxa0=xa0163) or to hospital (nxa0=xa0201) were screened for the presence of malnutrition (BMI and/or height/length for age z-scoresxa0<xa0-2). 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.nnnRESULTSnPrevalence of malnutrition was 2-fold higher (Pxa0<xa00.001) 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 (ORxa0=xa01.86 [95% CI, 1.21-2.88]; Pxa0=xa00.005) 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, ORxa0=xa02.24 [95% CI, 1.39-3.63], Pxa0=xa00.001), but we did not find any modifying effect (interaction) of the setting of care on the association between malnutrition and prolonged hospitalization.nnnDISCUSSIONnThe 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.