Prevalence and economic cost of malnutrition in Italy: A systematic review and metanalysis from the Italian Society of Artificial Nutrition and Metabolism (SINPE).

OBJECTIVES: Disease-related malnutrition (DRM) is a major public health issue with dramatic consequences on outcomes. However, in Italy a comprehensive and updated overview on national prevalence, in both the adult and pediatric populations, and its burden on the health care environment, is missing. The aim of this systematic literature review and meta-analysis was to identify and summarize the available evidence regarding the prevalence of DRM in Italy from pediatric to adult and older ages, and to project its global costs on the health care system. METHODS: We performed a systematic literature search for articles on epidemiology of DRM in Italy published up to June 2021. Studies reporting data on the prevalence of DRM in community-dwelling individuals with chronic diseases, nursing home patients, and hospitalized patients (medical, surgery, and oncology patients), were selected for inclusion. Methodological quality of the studies was assessed by two independent reviewers using published criteria. An epidemiologic meta-analysis to obtain an aggregate estimate of prevalence of DRM was performed and a model for estimating the cost of illness, based on the application of epidemiologic results to official national hospitalization data, and attribution of relevant unit costs in the national context was constructed. RESULTS: Sixty-seven studies reporting on the prevalence of DRM in Italian populations were included in the final selection; meta-analytical pooling yields mean prevalence estimates of about 50% and 30% in adult and pediatric hospitalized populations, respectively, with even higher findings for residents of long-term care facilities. Modeled projections of DRM-attributable yearly economic effects on the Italian health care system exceed 10 billion € in base case analysis, with the most optimistic estimate still exceeding 2.5 billion €. CONCLUSION: Although comparable in magnitude to data from previous studies in analogous international settings, the diffusion and effects of DRM in the Italian setting is impressive. Increased awareness of these data and proactive fostering of clinical nutrition services are warranted, as prompt identification and treatment of malnutrition have been shown to effectively improve clinical and economic results.

Nutritional Management of Patients with Fontan Circulation: A Potential for Improved Outcomes from Birth to Adulthood.

Fontan circulation (FC) is a surgically achieved palliation state offered to patients affected by a wide variety of congenital heart defects (CHDs) that are grouped under the name of univentricular heart. The procedure includes three different surgical stages. Malnutrition is a matter of concern in any phase of life for these children, often leading to longer hospital stays, higher mortality rates, and a higher risk of adverse neurodevelopmental and growth outcomes. Notwithstanding the relevance of proper nutrition for this subset of patients, specific guidelines on the matter are lacking. In this review, we aim to analyze the role of an adequate form of nutritional support in patients with FC throughout the different stages of their lives, in order to provide a practical approach to appropriate nutritional management. Firstly, the burden of faltering growth in patients with univentricular heart is analyzed, focusing on the pathogenesis of malnutrition, its detection and evaluation. Secondly, we summarize the nutritional issues of each life phase of a Fontan patient from birth to adulthood. Finally, we highlight the challenges of nutritional management in patients with failing Fontan.

Case Report: Morphologic and Functional Characteristics of Intestinal Mucosa in a Child With Short Bowel Syndrome After Treatment With Teduglutide: Evidence in Favor of GLP-2 Analog Safety.

Teduglutide is a glucagon-like peptide-2 (GLP-2) analog employed in patients with short bowel syndrome (SBS) to reduce the need of parenteral nutrition in these patients, by virtue of its effects on enteric function. The experimental studies reported that the stimulating action of GLP-2 on epithelial turnover implies the potential development of dysplastic and neoplastic lesion. However, the clinical trials could not detect preneoplastic lesions on histologic material, and in a recent pilot study the occurrence of polyps was similar before and after treatment and included only low-grade dysplastic lesions. Another clue in GLP-2 function in stimulating mucosal restore is its enhancement through cooperation with epidermal growth factor (EGF). In this study, we analyzed gastroscopy and colonoscopy samplings from a child successfully weaned off parenteral nutrition with teduglutide. Villous and crypt structure was regular both in duodenal and in colonic samplings; in properly oriented villi, villus/crypt ratio was regular. The absorptive epithelium demonstrated a regular morphology. No atypia was detected in enterocytes, along epithelial structures. At the ultrastructural analysis, only a few enterocytes with vacuolized cytoplasm were observed. An S-phase marker Ki67 stained nuclei in the transitional amplifying zone, while nuclei stained by the cell cycle regulatory proteins p21 and p27 were placed in the differentiated epithelium of the duodenal villi and colonic crypts, as in the control cases. The counts of enterocytes immunostained with the same antisera, evaluated with image analysis software, were in the range of control cases. The ratio of the number of epidermal growth factor receptor (EGFR) signals/the number of centromere probe of chromosome 7 (CEP7) signals was less than 2. The findings available from this single patient are consistent with good preservation of functional capability of intestinal epithelium after treatment with GLP-2, given the histologic and ultrastructural features of enterocytes. In addition, the findings from cell cycle regulatory proteins immunolocalization and quantitative analysis show that cell renewal machinery in our case is comparable to control cases. The gene of the receptor EGFR is regularly expressed in enteric epithelium of our case. Morphologic and functional data from our patient improve evidence in favor of the safety of GLP-2 employ in SBS.

Management of Nutritional Needs in Pediatric Oncology: A Consensus Statement.

Malnutrition, intended as both overnutrition and undernutrition, is a common problem in children with cancer, impacting quality of life as well as survival. In addition, nutritional imbalances during childhood can significantly affect proper growth. Nevertheless, there is currently a lack of a systematic approach to this issue in the pediatric oncology population. To fill this gap, we aimed to provide practice recommendations for the uniform management of nutritional needs in children with cancer. Twenty-one clinical questions addressing evaluation and treatment of nutritional problems in children with cancer were formulated by selected members from four Italian Association of Pediatric Hematology and Oncology (AIEOP) centers and from the Survivorship Care and Nutritional Support Working Group of Alliance Against Cancer. A literature search in PubMed was performed; during two consensus meetings, all recommendations were discussed and finalized using the nominal group technique. Members representing every institution voted on each recommendation. Finally, recommendations were approved by all authors.

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).

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, p = 0.03) and of surviving (OR = 1.05, p = 0.01). Adjusting for age, the probability of weaning off PN but of not surviving remained significantly associated with PN alone (OR = 1.08, p = 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.

Plasma citrulline as marker of bowel adaptation in children with short bowel syndrome.

PURPOSE: The aim of this study is to determine if prospective determinations of citrulline could be predictive of the bowel adaptation in children with short bowel syndrome (SBS). METHODS: Between March 2005 and March 2010, we prospectively included 28 SBS patients on parenteral nutrition. The citrulline and the enteral intake determinations were scheduled at the inclusion and at 6-month intervals. We assessed the correlation between citrulline and bowel length as well as enteral caloric intake, longitudinal trend of citrulline and association between patients characteristics according to the course of bowel adaptation. RESULTS: Citrulline significantly correlated with the residual duodenum-jejunum length (r (2) = 0.22, P = 0.0113) and with enteral intake (r (2) = 0.20, P = 0.016 and r (2) = 0.48, P = 0.0001). Baseline citrulline at the cutoff >10 µmol/L and a longitudinal increase >25% provided a weak association with bowel adaptation (likelihood ratio (LR), 2.6 and 2.4, respectively), unlike residual small bowel length ≥20 cm and the presence of >50% of the colon (LR, 10 and 6, respectively). CONCLUSIONS: Citrulline seems to be a powerful biomarker of the intestinal function, showed by the correlation with the residual duodenum-jejunum length and the enteral absorption, but not of its prospective changes during the bowel adaptation process. Future studies may be necessary to confirm this finding.

Cushing syndrome due to ectopic adrenocorticotropic hormone secretion in a 3-year-old child.

Ectopic adrenocorticotrophic hormone (ACTH) secretion is a rare cause of Cushing syndrome in paediatric age, due to tumours arising from different tissues. To date, only 11 reports of ACTH-secreting pancreatic tumours in children and adolescents exist in the literature. We present a paediatric case of Cushing syndrome caused by ectopic ACTH secretion. This was caused by a large acinar cell carcinoma that developed in the pancreas of a 3-year-old girl.

Variable phenotypic expression of chylomicron retention disease in a kindred carrying a mutation of the Sara2 gene.

Chylomicron retention disease is a recessive inherited disorder characterized by fat malabsorption and steatorrhea and is associated with failure to thrive in infancy. We describe a kindred carrying a mutation of Sara2 gene causing a chylomicron retention phenotype. The proband was a 5-month-old baby, born of consanguineous, apparently healthy parents from Morocco, with failure to thrive. There was a large quantity of fats in feces and malabsorption of fat-soluble vitamins. Intestinal biopsies showed a diffused enterocyte vacuolization with large cytosolic lipid droplets. Chylomicron retention disease or Anderson disease was hypothesized, and the Sara2 gene was analyzed by direct sequencing. Analysis of the Sara2 gene in the proband identified a 2-nucleotide homozygous deletion in exon 3 leading to a premature stop codon (c.75-76 del TG-L28fsX34). The father was heterozygous for the same mutation, whereas the proband's mother was homozygous, suggesting a variable phenotypic expression of the molecular defect. More studies are needed to understand the reasons of the phenotypic variability of the same molecular defect in the same family.

Neonatal screening for cystic fibrosis does not affect time to first infection with Pseudomonas aeruginosa.

OBJECTIVE: Newborn screening for cystic fibrosis was introduced in the Piedmont region of Italy in the year 2000. Our aim with this study was to estimate the effect of newborn screening on the risk of Pseudomonas aeruginosa infection at the regional cystic fibrosis pediatric reference center. METHODS: The time to first infection with P aeruginosa within the historical cohort of cystic fibrosis children diagnosed between January 1, 1997, and June 30, 2004, was investigated, comparing survival functions and the adjusted hazard ratio of children diagnosed before and after newborn screening introduction. The role of pancreatic insufficiency was also concurrently investigated. RESULTS: Overall, 71 children diagnosed with cystic fibrosis were identified, 27 cases were clinically diagnosed before newborn screening introduction, and 5 of them presented with meconium ileus, whereas 44 were identified by newborn screening. Among them 35 needed pancreatic enzyme supplementation, whereas 34 children were infected with P aeruginosa. Both the nonparametric and semiparametric survival estimates failed to show any significant increase in the risk of P aeruginosa infection among screened children compared with historical controls. However, the median time from cystic fibrosis diagnosis to P aeruginosa infection among screened children was significantly shorter (183 vs 448 days). Children with impaired pancreatic function were at high risk of P aeruginosa infection. CONCLUSIONS: The results of the study suggest that health authorities should regard newborn screening for cystic fibrosis as an opportunity to improve care and outcomes among affected children and shift the focus from whether it is appropriate to screen to how to optimize biomedical and psychosocial outcomes of screening.