Overview of pediatric short bowel syndrome.
ABSTRACT Short bowel syndrome (SBS) is a malabsorptive state occuring as a result of surgical resection or congenital disease of a significant portion of the small intestine . The amount of resection or remaining bowel generally dictates the degree of malabsorption and consequentely the need for specialized enteral nutrition or parenteral nutrition (PN). Intestinal failure in the context of SBS is defined as a dependence on PN to maintain minimal energy and fluid requirement for growth in children. Common causes of SBS in infants and children include necrotizing enterocolitis, midgut volvulus, intestinal atresia, and gastroschisis. Early identification of patients at risk for long-term PN dependency is the first step toward avoiding severe complications. Close monitoring of nutritional status, steady and early introduction of enteral nutrition, and aggressive prevention, diagnosis, and treatment of infections such as central venous catheter sepsis and bacterial overgrowth can significantly improve the prognosis. Intestinal transplantation is an emerging treatment that may be considered when intestinal failure is irreversible and children are experiencing serious complications related to TPN administration.
- SourceAvailable from: Daniel H Teitelbaum
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- "SBS is a highly morbid disease, characterized by septic episodes, metabolic derangements, repeated hospitalizations, and a poor quality of life . The care of children with SBS remains challenging, requiring meticulous management of PN and vigilance with regards to infectious complications  . Counseling these patients and their families is made difficult by the relatively unpredictable course of the disease. "
ABSTRACT: In a large cohort of children with intestinal failure (IF), we sought to determine the cumulative incidence of achieving enteral autonomy and identify patient and institutional characteristics associated with enteral autonomy. A multicenter, retrospective cohort analysis from the Pediatric Intestinal Failure Consortium was performed. IF was defined as severe congenital or acquired gastrointestinal diseases during infancy with dependence on parenteral nutrition (PN) >60 days. Enteral autonomy was defined as PN discontinuation >3 months. A total of 272 infants were followed for a median (IQR) of 33.5 (16.2-51.5) months. Enteral autonomy was achieved in 118 (43%); 36 (13%) remained PN dependent and 118 (43%) patients died or underwent transplantation. Multivariable analysis identified necrotizing enterocolitis (NEC; OR 2.42, 95% CI 1.33-4.47), care at an IF site without an associated intestinal transplantation program (OR 2.73, 95% CI 1.56-4.78), and an intact ileocecal valve (OR 2.80, 95% CI 1.63-4.83) as independent risk factors for enteral autonomy. A second model (n = 144) that included only patients with intraoperatively measured residual small bowel length found NEC (OR 3.44, 95% CI 1.36-8.71), care at a nonintestinal transplantation center (OR 6.56, 95% CI 2.53-16.98), and residual small bowel length (OR 1.04 cm, 95% CI 1.02-1.06 cm) to be independently associated with enteral autonomy. A substantial proportion of infants with IF can achieve enteral autonomy. Underlying NEC, preserved ileocecal valve, and longer bowel length are associated with achieving enteral autonomy. It is likely that variations in institutional practices and referral patterns also affect outcomes in children with IF. Copyright © 2015 Elsevier Inc. All rights reserved.2014 American Academy of Pediatrics National Conference and Exhibition; 10/2014
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ABSTRACT: Obstruktive Fehlbildungen und Erkrankungen des Intestinaltraktes sind im Neugeborenen- und Säuglingsalter relativ häufig und bilden einen der Schwerpunkte der viszeralen Kinderchirurgie. In den ersten Lebenstagen machen sie bis zu einem Drittel der intensivpflichtigen Erkrankungen aus. Fehlender Abgang von Mekonium bzw. Stuhlgang, geblähtes und schmerzhaftes Abdomen sowie galliges Erbrechen sind die wichtigsten gemeinsamen Symptome, hinter denen sich eine Vielzahl Fehlbildungen und Erkrankungen verbirgt, die im Folgenden abgehandelt werden. Bei den meisten von diesen ist eine zielgerichtete Diagnostik und ggf. rasche chirurgische Intervention dringlich, um schwere Folgeschäden wie Sepsis und Kreislaufschock, insbesondere aber Verlust von Darmanteilen mit drohendem Kurzdarmsyndrom zu vermeiden. Die recht uniforme, klinisch zunächst nicht sehr ausgeprägte Symptomatik macht es aber oft schwierig, rasch die korrekte Diagnose zu stellen und die richtigen Konsequenzen daraus zu ziehen.
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ABSTRACT: Since 1992, reliable high heat flux PFCs based on copper alloy heat sink structures and a CFC armour, have been developed. The final result is an actively cooled high heat flux element that is capable of removing up to 10 MW·m<sup>-2</sup> stationary operating conditions. About 600 of these high performance individual components have then been manufactured and assembled in order to equip a toroidal pump limiter (TPL). This major in-vessel component consists of a flat toroidally continuous disk (R<sub>i</sub>=2.2 m, R<sub>o</sub>=2.7 m) covering a 7.6 m<sup>2</sup> area and made of 574 radial high heat flux elements called "fingers". Following a promising previous manufacture of 200 "short fingers elements" dedicated to RF antenna limiters, the fabrication of actively cooled long fingers elements required to equip the TPL was launched in 1997 in the framework of CIEL project. Although some issues appeared during series fabrication, requiring repair processes development, the final deliveries have been achieved successfully end 2001. The paper deals with the experience feedback that was gained all along the four years duration of the TPL components manufacture. We will show where issues were encountered, how solutions were found to achieve the fabrication of components and will highlight what are the main technical lessons to be learned: acceptance criteria, choice of materials, margins of processes. Finally a proposal of an alternative optimised design is presented, fruit of the experience gained and progress performed from this up to now, unique series manufacture of actively cooled plasma facing HHF components. Such feedback experience is considered highly relevant and available to ITER as well as to Wendelstein 7-X.Fusion Engineering, 2003. 20th IEEE/NPSS Symposium on; 11/2003