Intestinal failure in childhood

Intestinal failure (IF) requires the use of parenteral nutrition (PN) for as long as it persists and in case of irreversible IF may be an indication for intestinal transplantation (ITx). Biological evaluation of IF is becoming possible with the use of plasma citrulline as a marker of intestinal mass. Short bowel syndrome (SBS) is the leading cause of intestinal failure in infants while few epidemiological data are to date available. Data on morbidity and mortality in paediatric patients with SBS are very limited but long-term outcomes seem to be improving. Other causes of intestinal failure include neuro-muscular intestinal disease and congenital disease of enterocyte development. The management of IF should include therapies adapted to each type and stage of IF based on a multidisciplinary approach in centres involving paediatric surgery, paediatric gastroenterology, parenteral nutrition expertise, home-parenteral nutrition programme, liver-intestinal transplantation experience. Timing for referral of patients in specialised centres remains a crucial issue.Keywords: citrulline; congenital enteropathy; home parenteral nutrition chronic intestinal pseudoobstructio

Nutrition in paediatric Crohn’s disease

Inflammatory bowel disease (IBD) such as Crohn’s disease (CD) results from the interaction between an individual’s immune response and precipitant environmental factors, which generate an anomalous chronic inflammatory response in those who are genetically predisposed. Protein-energy malnutrition (PEM) is a frequent consequence of CD. Macrophage products such as Tumour Necrosis Factor-α (TNF-α) and interleukins 1 and 6 may be the central molecules that link the inflammatory process to derangements of homeostasis. CD is associated with frequent nutritional deficiencies, the pattern and severity of which depends on the extent, duration and activity of the inflammation. Nutritional support is especially important in childhood CD as an alternative to pharmacological treatment, especially steroids. Current treatment regimens limit the use of corticosteroids, by using immunomodulatory drugs, recommend the use of enteral nutrition, and, if necessary, consider surgery for intestinal complications of localised CD. Biologic agents with the potential for mucosal healing hold promise of growth enhancement even among children whose growth remained compromised with previously available therapies. For all treatment modalities, there is a window of opportunity to achieve normal growth before puberty is too advanced.Keywords: Crohn’s disease; linear growth; Tumour Necrosis Factor-α; nutritional support; enteral feeding; immunomodulation

Congenital diarrhoeal disorders: advances in this evolving web of inherited enteropathies.

Congenital diarrhoeal disorders (CDDs) represent an evolving web of rare chronic enteropathies, with a typical onset early in life. In many of these conditions, severe chronic diarrhoea represents the primary clinical manifestation, whereas in others diarrhoea is only a component of a more complex multi-organ or systemic disorder. Typically, within the first days of life, diarrhoea leads to a life-threatening condition highlighted by severe dehydration and serum electrolyte abnormalities. Thus, in the vast majority of cases appropriate therapy must be started immediately to prevent dehydration and long-term, sometimes severe, complications. The number of well-characterized disorders attributed to CDDs has gradually increased over the past several years, and many new genes have been identified and functionally related to CDDs, opening new diagnostic and therapeutic perspectives. Molecular analysis has changed the diagnostic scenario in CDDs, and led to a reduction in invasive and expensive procedures. Major advances have been made in terms of pathogenesis, enabling a better understanding not only of these rare conditions but also of more common diseases mechanisms

Effect of prevention measures on incidence of human listeriosis, France, 1987-1997.

To assess the impact of preventive measures by the food industry, we analyzed food monitoring data as well as trends in the incidence of listeriosis estimated through three independent sources: the National Reference Center of Listeriosis; a laboratory-based active surveillance network; and two consecutive nationwide surveys of public hospital laboratories. From 1987 to 1997, the incidence of listeriosis decreased by an estimated 68%. A substantial reduction in the proportion of Listeria monocytogenes-contaminated products was observed at the retail level. The temporal relationship between prevention measures by the food industry, reduction in L. monocytogenes-contaminated foodstuffs, and reduction in listeriosis incidence suggests a causal relationship and indicates that a substantial part of the reduction in illness is related to prevention efforts

Syndromic (phenotypic) diarrhea in early infancy

Syndromic diarrhea (SD), also known as phenotypic diarrhea (PD) or tricho-hepato-enteric syndrome (THE), is a congenital enteropathy presenting with early-onset of severe diarrhea requiring parenteral nutrition (PN). To date, no epidemiological data are available. The estimated prevalence is approximately 1/300,000–400,000 live births in Western Europe. Ethnic origin does not appear to be associated with SD. Infants are born small for gestational age and present with facial dysmorphism including prominent forehead and cheeks, broad nasal root and hypertelorism. Hairs are woolly, easily removed and poorly pigmented. Severe and persistent diarrhea starts within the first 6 months of life (≤ 1 month in most cases) and is accompanied by severe malabsorption leading to early and relentless protein energy malnutrition with failure to thrive. Liver disease affects about half of patients with extensive fibrosis or cirrhosis. There is currently no specific biochemical profile, though a functional T-cell immune deficiency with defective antibody production was reported. Microscopic analysis of the hair show twisted hair (pili torti), aniso- and poilkilotrichosis, and trichorrhexis nodosa. Histopathological analysis of small intestine biopsy shows non-specific villous atrophy with low or no mononuclear cell infiltration of the lamina propria, and no specific histological abnormalities involving the epithelium. The etiology remains unknown. The frequent association of the disorder with parental consanguinity and/or affected siblings suggests a genetic origin with an autosomal recessive mode of transmission. Early management consists of total PN. Some infants have a rather milder phenotype with partial PN dependency or require only enteral feeding. Prognosis of this syndrome is poor, but most patients now survive, and about half of the patients may be weaned from PN at adolescence, but experience failure to thrive and final short stature

Direct Tension Recording From Smooth Muscle of Resistance Vessels From Various Organs

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/67115/2/10.1177_000331976101201009.pd

Nurturing Children's Healthy Eating: Position statement

The relationship between eating a healthy diet and positive health outcomes is well known; nurturing healthy eating among children therefore has the potential to improve public health. A healthy diet occurs when one''s usual eating patterns include adequate nutrient intake and sufficient, but not excessive, energy intake to meet the energy needs of the individual. However, many parents struggle to establish healthy eating patterns in their children due to the pressures of modern life. Moreover, healthcare providers often do not have the time or the guidance they need to empower parents to establish healthy eating practices in their children. Based on existing evidence from epidemiologic and intervention research, the Nurturing Children''s Healthy Eating collaboration, established by Danone Institute International, has identified four key themes that encourage and support healthy eating practices among children in the modern Western world. The first — positive parental feeding — explores how parenting practices and styles, such as avoiding food restriction, allowing children to make their own food choices, and encouraging children to self-limit their portion sizes, can influence children''s dietary intake. The second — eating together — highlights the link between eating socialization through regular family meals and healthful diet among children. The third — a healthy home food environment — explores the impact on eating practices of family resources, food availability/accessibility, parental modeling, and cues for eating. The fourth — the pleasure of eating — associates children''s healthy eating with pleasure through repeated exposure to healthful foods, enjoyable social meals, and enhancement of the cognitive qualities (e.g. thoughts or ideas) of healthful foods. This paper reviews the evidence leading to the characterization of these nurturing themes, and ways in which recommendations might be implemented in the home

Multivisceral intestinal transplantation: Surgical pathology

We report the diagnostic surgical pathology of two children who underwent multivisceral abdominal transplantation and survived for 1 month and 6 months. There is little relevant literature, and diagnostic criteria for the various clinical possibilities are not established; this is made more complicated by the simultaneous occurrence of more than one process. We based our interpretations on conventional histology, augmented with immunohistology, including HLA staining that distinguished graft from host cells in situ. In some instances functional analysis of T cells propagated from the same biopsies was available and was used to corroborate morphological interpretations. A wide spectrum of changes was encountered. Graft-versus-host disease, a prime concern before surgery, was not seen. Rejection was severe in 1 patient, not present in the other, and both had evidence of lymphoproliferative disease, which was related to Epstein-Barr virus. Bacterial translocation through the gut wall was also a feature in both children. This paper documents and illustrates the various diagnostic possibilities.. © 1989 Informa UK Ltd All rights reserved: reproduction in whole or part not permitted