Home parenteral nutrition in children: 8 years of experience with 112 patients

Ricour, C.; Górski, A; Goulet, Olivier; Potter, S. De; Corriol, Odile; Postaire, M.; Nihoul-Fékété, C; Jan, D.; Révillon, Y; Lortat-Jacob, S.; Pellerin, D

doi:10.1016/0261-5614(90)90055-w

Cited by 72 publications

(44 citation statements)

References 27 publications

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“…200 In children dependent on home parenteral nutrition who developed cholestasis, supplementation did not improve liver function tests despite a significant increase in blood taurine concentrations. 201 In a retrospective review of the frequency of cholestasis in infants who received two different commercial parenteral amino acid formulations supplemented with taurine (25 and 70 mg/100 ml bulk solution), PNAC occurred in 21.4% of subjects (15/70 patients) in equal numbers of those who received either formulation for at least 14 days. 37 This frequency fell within the range reported for PNAC in infants receiving parenteral nutrition without taurine supplementation.…”

Section: Taurine Deficiencymentioning

confidence: 99%

Parenteral Nutrition‐Associated Liver Complications in Children

Btaiche

Khalidi

2002

Pharmacotherapy

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Parenteral nutrition is a life-saving therapy for patients with intestinal failure. It may be associated with transient elevations of liver enzyme concentrations, which return to normal after parenteral nutrition is discontinued. Prolonged parenteral nutrition is associated with complications affecting the hepatobiliary system, such as cholelithiasis, cholestasis, and steatosis. The most common of these is parenteral nutrition-associated cholestasis (PNAC), which may occur in children and may progress to liver failure. The pathophysiology of PNAC is poorly understood, and the etiology is multifactorial. Risk factors include prematurity, long duration of parenteral nutrition, sepsis, lack of bowel motility, and short bowel syndrome. Possible etiologies include excessive caloric administration, parenteral nutrition components, and nutritional deficiencies. Several measures can be undertaken to prevent PNAC, such as avoiding overfeeding, providing a balanced source of energy, weaning parenteral nutrition, starting enteral feeding, and avoiding sepsis.

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Section: Taurine Deficiencymentioning

confidence: 99%

Parenteral Nutrition‐Associated Liver Complications in Children

Btaiche

Khalidi

2002

Pharmacotherapy

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“…Functional bowel capacity does not depend on its real length, but on its capacity and mucosal integrity. Citrulline is a nonessential amino acid synthesized in the enterocyte from glutamine and other amino acids and is not used for protein synthesis [10, 16]. It is a specific biomarker of intestinal function in patients with normal renal function [11, 17] and a good marker of enterocyte load.…”

Section: Reviewmentioning

confidence: 99%

“…Other factors that contribute to intestinal adaptation are the individual ability to develop hyperphagia, the increase in and hypertrophy of intestinal villi, the delay of digestive transit and, last but not least, a dedicated multidisciplinary team. The clinical course and serum biomarkers such as citrulline (nonessential amino acid produced exclusively by enterocytes) [10] will guide the therapeutic approach. In case of deficient intestinal adaptation, different surgical options are available.…”

Section: Introductionmentioning

confidence: 99%

Long-Term Results of Serial Transverse Enteroplasty with Neovalve Creation for Extreme Short Bowel Syndrome: Report of Two Cases

Botey

Alastrué

Haetta³

et al. 2017

Case Rep Gastroenterol

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Objective: The aim of this article was to determine whether serial transverse enteroplasty (STEP) and the creation of a new ileocecal valve in extreme short bowel syndrome (SBS) cases (<45 cm) is effective in intestinal adaptation and improvement of nutritional parameters and serum citrulline levels. Patients and Methods: We present 2 cases of SBS treated with STEP. Enterectomy was performed for massive intestinal ischemia secondary to a gastrointestinal stromal tumor in the first case and to catastrophic antiphospholipid syndrome in the second. After enterectomy, the short residual bowel measured 34 cm in the first patient and 45 cm in the second. In both cases STEP, cholecystectomy, and gastrostomy were performed. In the first case a Brooke neovalve was created, and in the other the ileocecal valve was preserved. Results: Both patients could finally be weaned off total parenteral nutrition (TPN) and gastrostomy feeding, maintaining a good nutritional status 1 year after surgery. Conclusions: In extreme SBS, a minimum length of 80–90 cm of functioning small bowel and an intact ileocecal valve are necessary. We plead for the use of STEP with preservation of the ileocecal valve or creation of a neovalve using the Brooke technique in order to achieve the ultimate goal, which is to wean patients off TPN. After a critical review of different surgical techniques, a treatment algorithm is proposed.

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“…[21][22][23] Death in childhood is related to the development of liver dysfunction, as more than 40% of patients with complete intestinal failure will develop severe liver disease within two years.22…”

Section: Intestinal Failurementioning

confidence: 99%