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Background Microvillous inclusion disease (MVID) is a congenital intestinal epithelial cell disorder leading to lifelong intestinal failure. Despite long-term total parenteral nutrition, life expectancy is extremely reduced because of metabolic or septic complications or liver failure. Methods Twelve patients with early-onset MVID were evaluated between 1995 and 2002 for the possibility of small bowel transplantation (SbTx). Three patients died before they could be placed on the waiting list for SbTx, and one patient is still awaiting SbTx. SbTx was contraindicated in one patient. Results Seven of 12 patients (six boys and one girl) underwent transplantation (three SbTxs and four combined liver-SbTxs). Actuarial survival rates were 100% and 75% in the SbTx and combined liver-SbTx groups, respectively, with a mean follow-up of 3 years (1.1-8.5 years). In contrast, the survival rate was only 40% in the subgroup of five patients who did not undergo transplantation. After transplantation, all patients were weaned from parenteral nutrition: the five patients with an additional colon graft were weaned within 36 days as opposed to the others without colonic transplant who obtained full intestinal autonomy several months after transplantation. The only two surviving patients who did not undergo SbTx remain highly dependent on total parenteral nutrition, which is complicated by repeated episodes of metabolic decompensation. Conclusions SbTx alone or in combination with the liver is highly successful in children with MVID, offering them a long-term perspective for the first time. Associated colon grafting markedly improves the outcome and quality of life after SbTx in patients with MVID.

Hoyeraal-Hreidarsson syndrome represents a severe variant of dyskeratosis congenita (Zinsser-Cole-Engman syndrome). This X-linked recessive, progressive, multisystemic disorder reported so far in 12 pedigrees is characterised by intrauterine growth retardation, microcephaly, cerebellar hypoplasia, mental retardation, progressive combined immune deficiency and aplastic anaemia. Mutations in the DKC1gene on Xq28 have been identified in the X-linked form of dyskeratosis congenita and in some Hoyeraal-Hreidarsson syndrome patients. We report on two sibs and two other unrelated patients with the striking clinical features of Hoyeraal-Hreidarsson syndrome. Noticeably, all four had early digestive problems, with chronic, bloody diarrhoea and feeding problems causing one of the most difficult problems in the supportive treatment of this uniformly lethal condition. Pathological changes in the proliferative compartment of the digestive mucosa included alterations of the glandular architecture and focal rarefaction of the glands. This aspect seems consistent with altered telomerase function associated with a dyskerin mutation which may decrease the proliferative capacity of digestive epithelial cells. A missense mutation 146 C-->T (Thr49Met) in the DKC1gene was found in two unrelated patients, whereas mutation screening was negative for one single case. The absence of mutations of the DKC1gene in patients with Hoyeraal-Hreidarsson syndrome emphasises the probable implication of one or more other loci.

To evaluate the efficacy and toxicity of infliximab in children with severe Crohn disease (CD), the authors prospectively monitored 21 children aged 15 +/- 2 years with severe CD who they treated with infliximab (5 mg/kg) on days 0, 15, and 45. One patient received only one injection. Eighteen patients were corticosteroid dependent, and 6 were receiving parenteral nutrition. Three patients were corticoid resistant (1 mg/kg/d15 days). Sixteen had perianal disease.The Harvey-Bradshaw index (HB) decreased from 8 +/- 3 on day 0 to 1 +/- 2 on day 45 (P = 0.001). The inflammation factors decreased (P = 0.001), and albumin increased (P = 0.002). Nineteen children were in complete remission (HB4) on day 45, and 2 had improved (HB = -6 points). Tumor necrosis factor-alpha (TNFalpha) in the stools (n = 16) decreased (P = 0.04). All perianal fistulas (n = 12) were closed by day 90. Fourteen of 21 patients had stopped taking steroids at 3 months, and all had stopped parenteral nutrition. Growth velocity was significantly greater after infliximab administration (Z score, +0.5) than before (-0.45; P = 0.004). Nineteen of 21 patients had relapsed (90%) at 1 year despite continued immunosuppressors. Seven had surgery because of an uncontrolled relapse ( 5), stenosis ( 1), or fistula ( 1). Six patients developed antinuclear antibodies (1/40-1/640e), and two had anti-DNA antibodies. Epstein-Barr virus (EBV) polymerase chain reaction (PCR) values increased (100-fold) in eight patients. One child developed an anaphylactic reaction to the medication, and one had a catheter-related sepsis.Infliximab produces spectacular results for children with severe CD and is well tolerated. However, its effect is transitory for many (90%), with frequent relapses despite continued immunosuppressors. Long-term management with infliximab should be tested despite its worrying side effects.

Numerous studies indicate beneficial effects of glutamine (Gln) in many models of catabolic adult rats. No data were available for aged rats. The effects of oral L-Gln-enriched diet were tested in endotoxemic 24-mo old rats. First, rats received for 7 d (from d0 to d7) an oral diet supplemented with either L-Gln [1g/(kg. d)] or casein (Cas: isonitrogenous supply) prior to lipopolysaccharide (LPS) challenge. The rats were then killed after 24 h food deprivation (from d7 to d8). Endotoxemia induced a catabolic response as shown by muscle glutamine depletion, hyperphenylalaninemia, small bowel atrophy and impaired functionality and bacterial translocation. The Gln-enriched diet did not prevent muscle Gln depletion but significantly (P

Purpose: The aim of the authors was to report an up-to-date review of their experience with 26 intestinal transplantations in children since 1987. Methods: A retrospective study was conducted of 26 patients with a mean age of 5 years (range, 0.3 to 14 years). Three groups were isolated. In group A (1987 to 1990), seven patients received nine isolated intestinal transplants for short bowel syndrome. Immunosuppression therapy consisted of cyclosporine, aziathioprine, and corticosteroids. In group B (1994-current), nine patients received nine isolated intestinal transplants for short bowel syndrom (n = 2), intestinal pseudoobstruction (n = 2), neonatal intractable diarrhea (n = 3), and Hirschsprung' disease (n = 1); hepatic biopsy results showed weak cholestasis or fibrosis. In group C (1994-current), 10 patients received 10 combined liver-small bowel transplants for short bowel syndrome (n = 3), neonatal intractable diarrhea (n = 4), and Hirschsprung' disease (n = 3); hepatic cirrhosis related to total parenteral nutrition (TPN) was shown in all cases. Groups B and C received immunosupressive treatment consisting of tacrolimus, aziathioprine, and corticosteroids. Posttransplant follow-up included intestinal biopsies of the small bowel twice a week and more frequently or combined with liver biopsy if rejection was suspected. Results: Overall patient survival (PS) and graft survival (GS) are 61% (16 of 26) and 50% (13 of 26), respectively. In group A, severe intestinal allograft rejection occurred in six patients leading to graft removal (GS, 11%). Five patients died of TPN complications after graft removal (PS, 28%). One survivor is off TPN, and one currently is waiting for a second graft. In group B, six patients survived (PS, 66%). Causes of death include hepatic failure (n = 1), renal and liver failure (n = 1), and systemic infection (n = 1). Severe intestinal allograft rejection occurred in five patients, which neccessitated aggressive immunosuppression (antilymphocyte serum) leading to an incomplete functional recovery of the graft. Only two patients currently are off TPN. In group C, eight patients survived (PS, 80%) all of which are currently off TPN. One patient died during the procedure, and one died of severe systemic infection. Intestinal graft rejection occurred in six patients; rejection of the liver allograft occurred in five patients, yet all rejections were weak and successfully treated by corticosteroids (GS, 80%). Conclusions: Intestinal transplantation is a valid therapeutic option for children with definitive intestinal failure and not only for short bowel syndrome. Tacrolimus improves graft and patient survival (group A v group B). The lower severity of graft rejection in combined liver-small bowel transplantation improves functional results of intestinal transplantation in children without additional mortality or morbidity (group B v group C).

Repeated or prolonged organic obstruction of the small intestine in the neonatal period can lead to severe refeeding problems, despite a transient ostomy. These problems are thought to result from a postobstructive enteropathy (POE) of the apparently normal small intestine segment above the obstruction. Ten infants with a POE, characterized by limited oral caloric and carbohydrate intakes and increased ostomy effluent, were compared with 8 controls with an enterostomy and a normal postoperative refeeding pattern. There was no statistical difference in the histomorphometric appearance of the mucosa or its digestive or absorptive capacity (brush-border hydrolases, glucose transport) between the two groups. The effluent and duodenal floras of the two groups were similar. However, all POE patients showed significant abnormal peristalsis characterized by barium and carmin transit times. This suggests that repeated or prolonged obstruction in the neonatal period could lead to a POE, caused by chronic motricity abnormalities of the small intestine above the obstruction. Although this POE is more frequent after small bowel atresia, it may also occur with other conditions causing prenatal and postnatal intestinal obstruction.

Resume Chez des rats denutris apres un jeune de 72 h, 24 h de realimentation avec un regime contenant des acides amines libres (AA) determinent au niveau de la muqueuse jejunale une stimulation plus importante de l'index mitotique que celle induite par des regimes isocaloriques contenant des proteines entieres (PE) ou un hydrolysat de petits peptides (HP). Le but de ce present travail a ete de rechercher si des peptides jejunaux a action mitogenique intestinale, recemment mis en evidence lors de la realimentation apres denutrition ou resection du grele, ou encore lors du sevrage, pouvaient etre impliques dans la difference de stimulation de l'index mitotique. Ceci a ete etudie chez 72 rats (Wistar 250 g) soumis a 3 jours de jeune et realimentes 24 h et 48 h avec les regimes precedemment decrits. L'activite des extraits acides muqueux bruts ainsi que celle des fractions obtenues apres passage sur colonne de Sephadex G25 ont ete mesurees par l'etude de la stimulation de synthese de l'ADN dans un systeme de culture de cellules intestinales (IEC 6) et aussi par culture organotypique pour les extraits bruts. Cette activite de croissance a ete exprimee sous forme d'un index de stimulation (%), apres incorporation de 3 H-thymidine. L'etude des extraits bruts a montre une augmentation de cet index avec les trois regimes a la 48 e heure de realimentation, retardee par rapport a l'augmentation de l'index mitotique (24 h). L'analyse des eluats a confirme cette reponse differee et de plus a mis en evidence un index de stimulation significativement plus important avec le regime AA. Leur activite etait plus specifiquement attachee au pic d'elution de M.M.

Background Management of patients with total intestinal aganglionosis (TIA) is a medical challenge because of their dependency on parenteral nutrition (PN). Intestinal transplantation (ITx) represents the only alternative treatment for patients with irreversible intestinal failure for achieving intestinal autonomy. Methods Among 66 patients who underwent ITx in our center, 12 had TIA. They received either isolated ITx (n = 4) or liver-ITx (LITx, n=8) after 10 to 144 months of total PN. All grafts included the right colon. Results After a median follow-up of 57 months, the survival rate was 62.5% in the LITx group and 100% in the ITx patients. The graft survival rate was 62.5% in the LITx group and 75% in the ITx group. All the surviving patients were fully weaned from total PN, after a median of 57 days. Pull through of the colon allograft was carried out in all patients. Fecal continence is normal in all but one of the surviving children. Conclusion These results suggest that ITx with colon grafting should be the preferred therapeutic option in TIA. Early referral to a transplantation center after diagnosis of TIA is critical to prevent PN-related cirrhosis and thereby to permit ITx, which is associated with a good survival rate.

Multiple factors are incriminated in the etiopathogeny of necrotizing enterocolitis (NEC) in premature infants, including oral feeding, vascular abnormalities, increase in pro-inflammatory cytokines, and inappropriate response of the intestinal barrier to bacterial microflora. CARD15/NOD2 is a gene recently recognized as important in the innate response to gut flora and is involved in Crohn's disease susceptibility. We thus tested its putative role in NEC. Ten children (seven boys and three girls) suffering from NEC who were admitted to Robert Debre hospital between 1999 and 2002 were retrospectively included in the study. Genetic screening of the 11 constant exons and the exon–intron junctions of CARD15/NOD2 by direct sequencing revealed no novel mutations of that gene in NEC patients. Furthermore, the three main mutations of CARD15/NOD2 (R702W, G908R, and 1007fs) associated with susceptibility to Crohn's disease were not found in these patients. Our results suggest that CARD15/NOD2 does not play a major role in genetic susceptibility to NEC.

Intestinal transplantation (ITx) has become an alternative for patients with permanent intestinal failure who are dependent on parenteral nutrition (PN) [1], Our center has been involved in the management of pediatric patients with intestinal failure for a long time. After developing a home PN program [2, 3], we started ITx in 1987 using cyclosporine [4]. One of our earliest patients still survives with a fully functioning graft 12 years later [5]. With the development of FK506 (tacrolimus) in the early 1990s [6], we restarted our ITx program. We now report the results of the largest current European series of consecutive intestinal transplantations in pediatric patients at the Necker-Enfants Malades University Hospital in Paris.

Background & Aims: Oral rehydration therapy is the only treatment recommended by the World Health Organization in acute diarrhea in children. Antisecretory drugs available could not be used because of their side effects, except for racecadotril, which is efficient in acute diarrhea in adults. Methods: The efficacy and tolerability of racecadotril (1.5 mg/kg administered orally 3 times daily) as adjuvant therapy to oral rehydration were compared with those of placebo in 172 infants aged 3 months to 4 years (mean age, 12.8 months) who had acute diarrhea. The treatment groups were comparable in terms of age, duration of diarrhea, number of stools, and causative microorganism at inclusion. Results: During the first 48 hours of treatment, patients receiving racecadotril had a significantly lower stool output (grams per hour) than those receiving placebo. The 95% confidence interval was 43%— 88% for the full data set (n 5 166; P 5 0.009) and 33%—75% for the per-protocol population (n 5 116; P 5 0.001). There was no difference between treatments depending on rotavirus status. Significant differences between treatment groups were also found after 24 hours of treatment: full data set (n 5 167; P 5 0.026) and per-protocol population (n 5 121; P 5 0.015). Tolerability was good in both groups of patients. Conclusions: This study demonstrates the efficacy (up to 50% reduction in stool output) and tolerability of racecadotril as adjuvant therapy to oral rehydration solution in the treatment of severe diarrhea in infants and children.

Pharmacological effects of dietary amino acids (AA) and peptides must be compared to an isonitrogenous control that is as inert as possible. To establish a rationale for the choice of such a control, potential metabolic and nutritional effects of three currently used nitrogenous controls (glycine, alanine, and casein) were evaluated in an endotoxemic rat model that has well-defined alterations in AA and protein metabolism. Five-week-old male Sprague-Dawley rats (113 +/- 1 g) were randomly assigned to four groups and received at d 0 an intraperitoneal injection of endotoxin (3 mg/kg). After withdrawal of food for 24 h, the rats were enterally refed for 48 h with a liquid diet (Osmolite((R))) supplemented with 0.19 g N. kg(-1). d(-1) in the form of glycine [lipopolysaccharide (LPS)-GLY group], alanine (LPS-ALA group) or casein (LPS-CAS group). One group (LPS group) received only Osmolite((R)). Plasma, two skeletal muscles, the liver and the intestine were then removed. Body and tissue weights and tissue protein contents did not differ among the four groups. Intestine histomorphometry showed no significant difference among groups. Jejunal hydrolase activities were significantly affected by the nitrogenous supplementations, but no effect was observed in the ileum. Only limited significant effects were observed on plasma and tissue-free AA concentrations, except for an accumulation of glycine in the plasma and tissues from the LPS-GLY group, compared to other groups. Overall, whereas glycine as a nitrogenous control should be used with care, either alanine or casein may be used as the "placebo," with the choice depending on the study to be performed.

The effects of dietary proteins given as whole proteins (WP) or as a peptide hydrolysate (PH) on growth, nitrogen retention, and small bowel adaptation were assessed using two groups of male Wistar rats. Measurements were made 18, 42, and 66 h after acute inflammation induced by subcutaneous injections of 0.125 mL turpentine and in two control groups (n = 12). The two diets had the same caloric, nitrogen, vitamin, and mineral content. The WP diet resulted in better weight gain, nitrogen retention, and small intestinal adaptation by control rats than did the PH diet. Loss of body weight after 18 h of acute inflammation was significantly lower and nitrogen retention significantly higher in animals on the WP diet than in those on the PH diet. Small intestine morphology was maintained with the WP diet, whereas villus height was significantly lower after 66 h, and there were fewer mitoses per crypt in the rats on the PH diet. Glucoamylase activity at all times, and N-aminopeptidase activity at 18 h, were significantly higher in rats on the WP diet. The putrescine (at 42 h) and spermidine (at 18 h) concentrations in the mucosa were higher in the rats on the WP diet. These data suggest that synthetic diets should be tested for their nutritional value during acute inflammation before they are used in human nutrition.

Two liquid diets containing selected milk proteins (SMP) or its small peptide hydrolysate (SPH) were fed to growing rats for 2 wk and the effects on growth, nitrogen balance, and small intestine adaptation were determined. Residual antigenicity of the SPH diet as measured by immunodot was reduced by 98.8%. Nitrogen intakes were not different. Weight gain was significantly higher in rats fed the SMP diet. In contrast, the absolute nitrogen balance was similar, suggesting that protein storage was identical with the two diets. A better nitrogen digestion-absorption rate with the SPH diet was observed as evidenced by the significantly increased fecal excretion with the SMP diet. Small intestine adaptation showed no difference between the two diets for mucosal weight, protein content/10 cm as well as for sucrase, glucoamylase, and N-aminopeptidase total activity/10 cm or specific activity (mU/mg protein). The DNA content of the mucosa/10 cm was significantly higher suggesting a mucosal hyperplasia in the SPH diet. The data suggest that in rats the SPH diet leads to nitrogen retention and small intestine adaptation similar to that of the SMP diet, despite better body weight gain by the latter.

This study was carried out to determine the frequency and composition (biliary and/or pancreactic) of duodenogastric reflux (DGR) in children with severe gastro-intestinal disorders on total parenteral nutrition (TPN), and to assess its consequences in terms of gastric histology (gastric per endoscopic biopsies) and secretion (acid, pepsin and sialic acid output). Sixteen children (mean age: 20 months) with severe gastro-intestinal disorders requiring TPN (mean duration: 9.5 months) were studied. DGR was demonstrated by measuring gastric choline and trypsin outputs. Serum gastrin levels were measured in all patients. Seven children (44%) had a DGR, with a significant increase in choline output (p < 0.02). Trypsin output was elevated in one patient only. Exudative gastritis and increased sialic acid output occurred in the presence and in the absence of DGR. DGR did not alter the basal acid and pepsin secretions. The serum gastrin levels were normal except in one case. These results show that DGR occurs frequently in children suffering from severe gastro-intestinal disorders on TPN, that it is mainly of biliary origin and that exudative gastritis is very frequent but not correlated with DGR. It suggests that DGR causes little injury in children on TPN, perhaps because of their decreased pancreatic secretion.

Intestinal motility was studied in 11 children with a transient enterostomy secondary to a neonatal organic small intestine obstruction (5 total colon Hirschsprung's disease, 2 necrotizing enterocolitis, 1 intussusception, 3 ileal atresia). Eight children presented with a postobstructive enteropathy (severe grade I [5], moderate grade II [3]) and three were considered as controls (grade III). They were assigned to one of the three groups on the basis of the duration of parenteral nutrition and constant rate enteral nutrition needed and the oral feeding tolerance. Barium small intestine transit showed no persistent partial obstruction or peritoneal adhesions. The abnormal inert marker transit times were statistically correlated with the clinical groups as well as duodenal manometric abnormalities. Manometric recordings were characterised by the absence (grade I) or abnormal phase III (grade II) of the migrating motor complex and decreased motility index (grades I and II). This study confirms that this enteropathy is due to a chronic alteration in motility induced by prenatal or postnatal obstructions.

Shwachman Diamond syndrome (SDS) is a rare multi organ genetic disease bearing a very high risk of haematological complications i.e. MDS/leukaemia and Bone Marrow Failure. The aim of this study is to explore genotype predisposition of the major complications observed in SDS’s patients and to explore prognosis factors of MDS/leukaemia. Methods: Among 90 SDS patients included in the French Severe Chronic Neutropenia Registry, SBDS gene was screened in 63 patients and mutations have been found in 60 patients. Cut-off date was july 30th, 2007. Differences between groups of patients were analysed as survival data, by log rank test. The medical events analysed were: death (n=6), myelodysplasia or acute leukemia (n=6), bone marrow failure (n=6), all hematological events combined (n=12), the use of G-CSF as infectious prophylaxis (n= 11), the necessity of an orthopedic surgery (n=4) and the necessity of nutritional medical support (parenteral or enteral feeding, by mean of gastrostomy (n= 5), intrauterine growth retardation (n=19) and finally, major development retardation if it leads to a specialized school (n=10). Results: Mutations were found in 60 patients (35 males, 25 females) belonging to 54 distinct families (in 6 families, two siblings were genotyped). The median age at last analysis was 10.3 years (0.5yr-38.6 yr). The great majority of patients present the recurrent genotype K62X/C84fs (n=38, 68%) while 19 other mutations were founded, which could be classified in truncating mutations leading to premature stop codons (nonsense, frameshift or splicing defect; n=8) or missense mutations (n=11). We compared patients with truncating mutations on both alleles to compound heterozygous patients carrying at least one missense mutation. Even if differences were observed for the distribution of events between genotype subgroups of patients, none of them raised statistically significance. However, to date, all leukemia has been observed in the group of patients with “truncating” mutations. The genotype of patients with leukemia was [K62X]+[C84fs] in 5 and [C84fs]+[V93fs] in one; while the genotype of patients with BM failure was [K62X]+[C84fs] (n=2), [C84fs]+[624+1G>C], [C84fs]+[C119R], K62X/undetermined, and [C84fs]+[E99fs], [C84fs]+[E44fs]. Among the 6 pairs of siblings tested, four had a similar outcome and two pairs were discordant for the haematological events (leukaemia in one family, Bone marrow failure in the second family). Further, we have analysed genotype, gender, G-CSF therapy and initial Neutrophils and monocytes count, Hemoglobin level, Platelet level as risk factors of Leukemia/MDS. In a multivariate model, none of these features predicts Leukemia/MDS in SDS patients. Conclusion: The genotype of SDS did not appear to be correlated with clinical presentation or outcome. It remains possible than patients without truncating mutations (about 18%) may have a low rate of leukaemia but our survey lack of statistical powerful to demonstrate this hypothesis. We also failed to determine prognostic factors of Leukemia/MDS in SDS patients.

Total Parenteral Nutrition (TPN) and constant rate enteral nutrition (CREN) are widely used: their effects on gastric function, especially pepsin secretion, are unknown. Basal and pentagastrin-stimulated pepsin (BPO, MPO) and acid (BAO, MAO) secretions were measured in three groups of infants: controls (14 infants fed normally), TPN groups (seven infants on TPN), CREN groups (14 infants on CREN). The MAO and MPO of the TPN group were significantly lower than controls (p less than 0.02), and the ratio of pentagastrin-stimulated PO/AO did not change, suggesting a large decrease of acid gastric function in the TPN group. BPO was not different from controls and BAO was significantly higher because of amino acids perfusion. The data for CREN group were not different from those of the control group, despite the fact that 11 infants were on TPN before CREN. These results demonstrate that TPN causes decreases in both acid and pepsin secretions in human infants. When TPN children are placed on CREN, these secretions return to normal.

The incidence of Crohn's disease (CD) lesions in the upper gastrointestinal (GI) tract of both adults and children is frequently underestimated. In this prospective study, a total of 31 children suspected of having Crohn's disease were systematically examined to identify upper digestive tract lesions. They all underwent barium transit endoscopy with multiple-level biopsies. Typical clinical symptoms suggestive of upper GI tract involvement were found in 5 children (16%), radiological signs in only one child (3%), endoscopic lesions in 13 children (42%), and specific granulomas in 12 children (39%). In eight of these 12 children, the biopsies were taken from macroscopically normal areas of the esophagogastroduodenal mucosa. One of the 31 children had no abnormal radiological and endoscopic features suggestive of CD on the distal small bowel and the colon. There was no correlation between the clinical, radiological, and histological data. Endoscopy plus biopsy provided a positive diagnosis in 39% of cases and a confirmation of the diagnosis in 87% of cases. Endoscopic and histological evidence of CD of the upper GI tract is often present despite an absence of clinical symptoms or radiological changes. Upper GI tract endoscopy with multiple biopsies may be important in the evaluation of this condition and even in some cases for the establishment of the diagnosis.

The effects of alimentary whey proteins given, as whole proteins (WP), controlled trypsin and chymotrypsin hydrolysate oligopeptides (WPH), or a free amino acid mixture (AAM), on growth, nitrogen retention, and steatorrhea were assessed in 24 Wistar rats (250 to 300 g) after 72 hr of starvation and 24 to 96 hr of realimentation and in 24 controls. The three diets had the same caloric, nitrogen, vitamin, and mineral contents. Rats had free access to the liquid diets. Only rats which ate the whole diet (90 cal) were included in the study. No differences in steatorrhea and fecal nitrogen were observed. The absorption rate was over 95% on the three diets. In contrast, weight gain was statistically better on WPH (+9% after 96 hr of realimentation) than on WP (+5%) or AAM (+2%). This was associated with a statistically higher nitrogen retention at all time periods studied, which was a result of a significant lower nitrogen urinary excretion. Similar results were obtained in controls. This better growth was a result of a better protein synthesis and lower ureagenesis.

Plasma cholecystokinin (CCK) levels were measured by radioimmunoassay in 100 children (mean age: 20 months) on various types of artificial nutrition. Of the 81 children given total parenteral nutrition (TPN), 32 were studied while on cyclic TPN (cTPN), 66 while on partial fractioned feeding completed by parenteral nutrition, 25 while on constant rate enteral nutrition, and 18 while on total discontinuous oral feeding. The remaining 19 control cases were on normal alimentation. Plasma CCK levels during TPN (21.4 +/- 1.6 pg/ml), cTPN (21.8 +/- 2.7 pg/ml), and constant rate enteral nutrition (26.4 +/- 2.8 pg/ml) were not significantly different from each other and were similar to preprandial total discontinuous feeding (21 +/- 2 pg/ml) and control (22.6 +/- 3.5 pg/ml) levels. The postprandial CCK level increased significantly in partial fractionated feeding (33.6 +/- 3.3 pg/ml, p less than 0.02) but remained half that of postprandial total discontinuous oral feeding (75.6 +/- 6.6 pg/ml, p less than 0.001) and postprandial controls (75 +/- 7 pg/ml, p less than 0.001). Thus, basal and stimulated CCK levels are similar in children and adults, and the use of long-term artificial nutritional techniques does not modify the feeding stimulation of CCK. Plasma CCK levels during TPN and constant rate enteral nutrition are similar to fasting values, indicating a possible role for CCK in the biliary sludge.

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.