Claude Ricour

Long-term outcome of children receiving home parenteral nutrition: a 20-year single-center experience in 302 patients.

Background: More information is needed regarding the prognosis of children receiving home parenteral nutrition (HPN). This article describes 20-year outcome data in children receiving HPN and provides separate profiles for the major pediatric diagnostic subgroups. Patients and Methods: This retrospective study included children who started receiving HPN between January 1, 1980, and December 31, 1999, in a single pediatric HPN center. Results: A total of 302 children were recruited, 230 (76%) with primary digestive disorders and 72 (24%) with nonprimary digestive disorders. Median age at HPN onset was 1.5 years. Median duration of HPN was 1.3 years. By January 1, 2000, 54% had weaned from HPN, 26% were still receiving HPN, 16% had died, and 4% had undergone intestinal transplantation. The survival probabilities at 2, 5, 10, and 15 years were 97%, 89%, 81%, and 72%, respectively. The likelihood and cause of death depended on the underlying diagnosis. Nine percent of children with primary digestive disorders died, 24% from their primary disease and 48% from liver disease or sepsis. Children with intractable diarrhea of infancy had the highest mortality rate (25%) and the highest incidence of liver disease (48%; P = 0.0002). Thirty-eight percent of children with primary nondigestive diseases died, 94% from their primary disease and 6% from liver disease or sepsis. Conclusions: Outcome and survival of children receiving HPN are mainly determined by their underlying diagnosis. Nearly all children with primary digestive disease survive if referred early to an expert center.

Effect of recombinant human growth hormone on intestinal absorption and body composition in children with short bowel syndrome.

This prospective study aimed to establish the effect of recombinant human growth hormone (rhGH) on intestinal function in children with short bowel syndrome (SBS). Eight children with neonatal SBS were included. All were dependent on parenteral nutrition (PN) for >3 years (range, 3.8-11.6 years), with PN providing >50% of recommended dietary allowance for age (range, 50%-65%). The subjects received rhGH (Humatrope) 0.13 mg/kg/d subcutaneously over a 12-week period. The follow-up was continued over a 12-month period after rhGH discontinuation. Clinical and biological assessments were performed at baseline, at the end of the treatment period, and 12 months after the end of treatment. No side effects related to rhGH were observed. PN requirements were decreased in all children during the course of rhGH treatment. Between baseline and the end of treatment, significant increases were observed in concentrations (mean ± standard deviation) of serum insulin-like growth factor 1 (103.1 ± 49.9 µg/L vs 153.5 ± 82.2 µg/L; P < .01), serum insulin-like growth factor-binding protein 3 (1.7 ± 0.6 mg/L vs 2.5 ± 0.9 mg/L; P < .001), and plasma citrulline (16.5 ± 14.8 µmol/L vs 25.2 ± 18.3 µmol/L; P < .05). A median 54% increase in enteral intake (range, 10%-244%) was observed (P < .001) and net energy balance improved significantly (P < .002). It was necessary for 6 children to be maintained on PN or restarted after discontinuation of rhGH treatment, and they remained on PN until the end of the follow-up period. A 12-week high-dose rhGH treatment allowed patients to decrease PN, but only 2 patients could be definitively weaned from PN. Indications and cost-effectiveness of rhGH treatment for SBS pediatric patients need further evaluation.

Intestinal transplantation in children: preliminary experience in Paris.

From November 1994 to November 1998, 20 children (2.5 to 14 years) received a jejunoileal graft alone (SBTx; n = 10) or in combination with the liver (SBLTx; n = 10 and/or the right colon (5 SBTx). Indications were intractable diarrhea of infancy (n = 8), short bowel syndrome (n = 6), extensive Hirschsprung disease (n = 4), and chronic intestinal pseudoobstruction (n = 2). Immunosuppression included tacrolimus, methylprednisolone, and azathioprine. Current follow-up ranges from 6 to 54 months. Five patients died (3 SBTx) within the first 2 months. Acute liver rejection occurred in 5 patients during the first 2 months. Sixteen episodes of intestinal rejection during the first 3 months in 11 patients (8 in 4 SBTx) were successfully treated in all but 3 by increasing tacrolimus dose and/or a 3-day methyprednisolone bolus or required antilymphoglobulins in 3 cases. Surgical complications occurred 8 times after SBLTx and 3 after SBTx. Infectious complications were more frequent in SBLTx recipients. Reversible Epstein-Barr virus-related posttransplant lymphoproliferative disease occurred in 3 recipients. Five presented cytomegalovirus infection. The SB graft was removed in 5 recipients (3 chronic rejection). All patients were started with oral and/or enteral feeding from the 7th postoperative day by using either normal food or protein hydrolysate diet. Currently, 10 of 11 children (8 SBLTx) achieved digestive autonomy after 5 to 30 weeks. All recipients gained weight; however, growth velocity remained reduced during the first 6 months because of the steroid therapy. Overall graft and patient survival is higher after SBLTx. Intestinal transplantation is indicated for patients with permanent intestinal failure. However, because parenteral nutrition is generally well tolerated, even for long periods, each indication for transplantation must be weighed carefully in terms of risk and quality of life.

Intestinal transplantation: indications, results and strategy.

The term ‘intestinal failure’ is now often used to describe gastrointestinal function insufficient to satisfy body nutrient and fluid requirements. The first recognized condition of intestinal failure was short bowel syndrome. Severe motility disorders such as chronic intestinal pseudo-obstruction syndrome in children as well as congenital intractable intestinal mucosa disorders are also forms of intestinal failure, because no curative treatment for these diseases is yet available. Parenteral nutrition and home parenteral nutrition remain the mainstay of therapy for intestinal failure, whether it is partial or total, provisional or permanent. However, some patients develop complications while receiving standard therapy for intestinal failure and are considered for intestinal transplantation. Indeed, recent advances in immunosuppressive treatment and the better monitoring and control of acute rejection have brought intestinal transplantation into the realm of standard treatment for intestinal failure. Although it has been used in humans for the past two decades, this procedure has had a slow learning curve. According to the current results, this challenging procedure may be performed in children or adults, only under certain conditions.

Evaluation of lean body mass in obese children

Multiple skinfold anthropometry (MSA) and bioelectrical impedance analysis (BIA) are useful as clinically non-invasive, inexpensive and portable techniques, although it is not clear if they can be used interchangeably in the same patient to routinely assess her/his body composition. In order to compare BIA, MSA and DXA in the estimation of lean body mass (LBM) of a pediatric obese population, 103 obese [body mass index (BMI) > 97th percentile] children (median age: 11xa0years; range: 5.4–16.7xa0years) underwent nutritional evaluation. After an overnight fast, the subjects’ anthropometric measurements were performed by the same investigator: body weight (BW), height, skinfold thickness (four sites); fat body mass (FBM) using Brook or Durnin equations and dual X-ray absorptiometry (DXA). BIA was performed using a bioelectrical impedance analyzer (Analicor-Eugedia, 50xa0kHz) and Houtkooper’s equation to calculate LBM. Linear regression analysis was performed to evaluate the relationship between the prediction of LBM by MSA, DXA and BIA. The differences between the three techniques were analysed using Student’s t-test for paired observations and the Bland and Altmann method. A considerable lack of agreement was observed between DXA- and BIA-LBM (δu2009=u2009−4.37xa0kg LBM; δ−2σu2009=u2009−11.6xa0kg LBM; δ+2σu2009=u2009+2.8xa0kg LBM); between DXA- and MSA-LBM (δu2009=u2009−1.72xa0kg LBM; δ−2σu2009=u2009−8.2xa0kg LBM; δ+2σu2009=u2009+4.8xa0kg LBM) and between BIA- and MSA-LBM (δu2009=u2009−2.65xa0kg LBM; δ−2σu2009=u2009−10.5xa0kg LBM; δ+2σu2009=u2009+5.2xa0kg LBM). Conclusion: In obese children, DXA, BIA and MSA should not be used interchangeably in the assessment of LBM because of an unacceptable lack of agreement between them. The discrepancies between methods increase with the degree of obesity.