Indications for pediatric intestinal transplantation: a position paper of the American Society of Transplantation.

Parenteral nutrition represents standard therapy for children with short bowel syndrome and other causes of intestinal failure. Most infants with short bowel syndrome eventually wean from parenteral nutrition, and most of those who do not wean tolerate parenteral nutrition for protracted periods. However, a subset of children with intestinal failure remaining dependent on parenteral nutrition will develop life-threatening complications arising from therapy. Intestinal transplantation (Tx) can now be recommended for this select group. Life-threatening complications warranting consideration of intestinal Tx include parenteral nutrition-associated liver disease, recurrent sepsis, and threatened loss of central venous access. Because a critical shortage of donor organs exists, waiting times for intestinal Tx are prolonged. Therefore, it is essential that children with life-threatening complications of intestinal failure and parenteral nutrition therapy be identified comparatively early, i.e. in time to receive suitable donor organs before they become critically ill. Children with liver dysfunction should be considered for isolated intestinal Tx before irreversible, advanced bridging fibrosis or cirrhosis supervenes, for which a combined liver and intestinal transplant is necessary. Irreversible liver disease is suggested by hyperbilirubinemia persisting beyond 3-4 months of age combined with features of portal hypertension such as splenomegaly, thrombocytopenia, or prominent superficial abdominal veins; esophageal varices, ascites, and impaired synthetic function are not always present. Death resulting from complications of liver failure is especially common during the wait for a combined liver and intestinal transplant, and survival following combined liver and intestinal Tx is probably lower than following an isolated intestinal transplant. The incidence of morbidity and mortality following intestinal Tx is greater than that following liver or kidney Tx, but long-term survival following intestinal Tx is now at least 50-60%. It is probable that outcomes shall improve in the future with continued refinements in operative technique and post-operative management, including immunosuppression.

Syndrome of intractable diarrhoea with persistent villous atrophy in early childhood: a clinicopathological survey of 47 cases.

BACKGROUND: The syndrome of intractable diarrhoea of infancy is heterogeneous and includes several diseases with diverse aetiologies. This study determines whether diagnostic categories can be defined on the basis of clinicopathological analysis. METHODS: European Society of Paediatric Gastroenterology, Hepatology and Nutrition members were surveyed to identify cases of intractable diarrhoea with persisting small intestinal enteropathy. A retrospective clinicopathological analysis was performed on cases showing life-threatening diarrhoea within the first 24 mo of life and requiring total parenteral nutrition, which were characterized by persistent villous atrophy, and resistance to therapy. RESULTS: Forty-seven infants were identified with intractable diarrhoea. Villous atrophy was of varying degrees with (group I, n = 24) or without (group II, n = 18) lamina propria mononuclear cell infiltration. Group I presented later, had gut autoantibodies, and a higher prevalence of protein-losing enteropathy; a subset (group Ia, n = 12) also had extraintestinal symptoms of autoimmunity associated with a later onset of larger volume diarrhoea. Group II presented early; 8 cases (group IIa) had phenotypic abnormalities and a low birth weight; the remaining 10 (group IIb) showed mild-to-moderate villous atrophy, epithelial tufting, and abnormal crypts. Group III included five patients in whom no specific features were recognised. Twenty-one (45%) died at a median age of 24 months, 20 (43%) remained dependent on parenteral (n = 16) or enteral tube (n = 4) feeding, 4 (9%) received elimination diets plus other therapies, and 2 (4%) were lost to follow-up. CONCLUSIONS: Clinicopathological analysis allowed distinct disease groups to be identified, allowing a provisional classification to be made. This straightforward approach forms a basis for future research in this exceptionally difficult paediatric condition.

Neonatal short bowel syndrome.

In this retrospective study the management of infants who had undergone resection of more than 50% of the small bowel as newborn infants between 1970 and 1988 was analyzed to define prognostic factors. Small bowel resections were performed for atresia (36 cases), volvulus (22 cases), gastroschisis (10 cases), necrotizing enterocolitis (11 cases), and other disorders (8 cases). Patients were classified into two groups depending on the length of residual small bowel: group 1 (n = 35) had less than 40 cm of small bowel and group 2 (n = 51) had 40 to 80 cm of residual small bowel. Patients in group 2 had significantly better survival rates than those in group 1 (92.0% vs 66.6%; p less than 0.001). The patients in group 1 who were born after 1980, when home parenteral nutrition was introduced, had better survival rates than those who were treated before 1980 (95.0% vs 65.0%; p less than 0.01). The time required for acquisition of intestinal adaptation depended on the intestinal length (average, 27.3 months for group 1 and 14 months for group 2; p less than 0.01) and on the presence or absence of the ileocecal valve. Parenteral or supportive enteral nutrition, or both, ensured normal growth in both groups. We conclude that more than 90% of infants now survive after extensive small bowel resection with parenteral nutrition and that the remaining small intestine will adapt with time. Home-based parenteral nutrition allowed children to be treated in the best psychosocial environment.

Energy expenditure and substrate utilization in the course of renutrition of malnourished children.

Energy expenditure (EE) and substrate utilization in the course of renutrition of malnourished children is not well understood in children receiving total parenteral nutrition (TPN). The aim of this study was to evaluate, during protein-glucose renutrition, EE and substrate utilization and the relationship between EE and growth and/or protein metabolism. Seven malnourished children were studied during the first 3 weeks of TPN. Weight-for-height = 81.4 +/- 8.0%, with an initial weight of 4.5 +/- 3.3 kg. Caloric support was progressively increased according to a preestablished protocol. Every 7 days the following were determined: (1) EE at 3 different 3-hour intervals per day using an open circuit indirect calorimetric system, (2) anthropometrically defined fat free mass (FFM), and (3) 24-hour urinary 3-methylhistidine (3-M-His) and protein balance. Compared to initial values, EE increased 13% at day 7 and 36% at day 14. A negative relationship was found between the amount of perfused glucose and lipid utilization (r = -0.82; p less than 0.0001). EE per kilogram of total body FFM changes during renutrition were more than EE changes per kilogram of total body weight. There was a relationship between EE and weight gain (r = 0.62; p less than 0.005) and a positive relationship between EE and protein gain (r = 0.48; p = 0.012) and 3-M-His excretion (r = 0.51; p less than 0.026).(ABSTRACT TRUNCATED AT 250 WORDS)

Estimating resting energy expenditure by simple lean-body-mass indicators in children on total parenteral nutrition.

The aim of this study was to determine simple predictive factors of the resting energy expenditure (REE) in children. Two groups, A (n = 14) and B (n = 23), were defined by their weight-for-height index, less than 90% and greater than 90%, respectively. Anthropometrically assessed lean body mass (LBM), 24-h urinary creatinine, and REE were measured. From multiple-regression analysis, the best-fitting equation for calculating REE (REE = 54.4 LBM (kg) + 0.095 creatinine (mmol/kg) + 4.7) was highly significant (r = 0.987, p less than 0.0001). Although the regressions of REE on weight were significantly different between the two groups, the equations using LBM or 24-h urinary creatinine did not discriminate between them. These findings suggest that an equation based on LBM or 24-h urinary creatinine excretion could be a more accurate estimate of REE than are conventional methods based on weight or height, and it may be applicable to diverse nutritional states.

Lipid tolerance in children receiving long-term parenteral nutrition: a biochemical and immunologic study.

The effect of intravenously administered lipids (intralipid) on immunologic function, complement, and coagulation was prospectively studied over 1 year in 15 children. The mean age of the children was 52.4 +/- 37.9 months; they had received total parenteral nutrition for an average of 3 years. Immunoglobulins (IgA, IgM, IgG), coagulation studies (platelets, prothrombin time (PT), partial thromboplastin time (PTT), fibrinogen, fibrinogen degradation products, factor V) and components of complement (C3, C4, and CH100) were analyzed. Activation of monocytes by opsonized zymosan was measured by chemiluminescence and compared with that of normal control subjects. The clinically stable children had normal monocyte activation and normal complement levels. The PT and PTT values were significantly increased but improved with increased intralipid dose; other coagulation factors were normal. Acutely sick children, however, had decreased fat tolerance with significantly increased serum triglyceride levels and PT and PTT values; their monocyte activation and complement factors remained normal. These data indicate that the dose of intralipid should be lowered during acute illnesses; we suggest close monitoring of PT and PTT values and of serum triglyceride and cholesterol levels to avoid the fat overload syndrome.