Patients with Intestinal failure (IF) require parenteral nutrition (PN) support to obtain enough nutrients to sustain growth. long-term PN use is associated with significant liver damage. Objective To analyze the utility of a non-invasive test, the aspartate aminotransferase (AST) to platelet ratio index (APRI), in the diagnosis of liver disease in pediatric patients with IF. Methods Medical records of all Boston Children’s Hospital patients who received PN and underwent a liver biopsy from January 2006 until November 2010 were reviewed. Patients with a clinical diagnosis with IF were selected. APRI was calculated as follows (AST (U/L)/ upper normal limit) × 100/ platelets (109/L). Presence of fibrosis and cirrhosis was estimated using the METAVIR score in liver biopsies. Results 62 liver biopsies from 48 patients (22 female) were studied. Mean APRI values in the different METAVIR categories (0-1; 2-3; 4) were: 1.80, 1.17, and 4.24 respectively (ANOVA; P=0.053; Bonferroni test for cirrhosis versus fibrosis P=0.048). APRI could significantly predict cirrhosis (OR 1.2.; 95% CI 1.001-1.43) but not significant fibrosis (METAVIR 2-3, OR 1.00; 95% CI =0.86-1.18). Area under the receiver operating characteristic curve for cirrhosis was 0.67 (95% CI= 0.45-0.89; p=0.13). Conclusion APRI, a non invasive, easy to obtain bedside test significantly predicts cirrhosis but not fibrosis in pediatric patients with IFALD. As the clinicians need a non invasive test to differentiate among different stages of liver fibrosis rather than differentiating cirrhosis from normal, we cannot recommend the use of this test in pediatric patients with IFALD for this purpose.

Fetal growth restriction followed by accelerated postnatal growth contributes to impaired metabolic function in adulthood. The extent to which these outcomes may be mediated centrally within the hypothalamus, as opposed to in the periphery within the digestive tract, remains unknown. In a sheep model, we achieved intrauterine growth restriction experimentally by maternal nutrient restriction (R) that involved a 40% reduction in food intake through late gestation. R offspring were then either reared singly to accelerate postnatal growth (RA) or as twins and compared with controls also reared singly. From weaning, all offspring were maintained indoors until adulthood. A reduced litter size accelerated postnatal growth for only the first month of lactation. Independently from postnatal weight gain and later fat mass, R animals developed insulin resistance as adults. However, restricted accelerated offspring compared with both the control accelerated and restricted restricted offspring ate less and had higher fasting plasma leptin as adults, an adaptation which was accompanied by changes in energy sensing and cell proliferation within the abomasum. Additionally, although fetal restriction down-regulated gene expression of mammalian target of rapamycin and carnitine palmitoyltransferase 1-dependent pathways in the abomasum, RA offspring compensated for this by exhibiting greater activity of AMP-activated kinase-dependent pathways. This study demonstrates a role for perinatal nutrition in the peripheral control of food intake and in energy sensing in the gastric mucosal and emphasizes the importance of diet in early life in regulating energy metabolism during adulthood.

An unnoticed FB aspiration and absence of and/or non-specific initial symptoms may contribute to a late diagnosis. The significant reduction in the number of cases over the later years may be related to the implementation of preventive strategies.

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Rh isoimmunisation leads to haemolytic anaemia and hyperbilirubinaemia in the first h of life. Isolated early onset neonatal anaemia has rarely been reported. The authors describe the case of a term infant, born to an 'A' negative, second gravida mother. On the second day of life, pallor was noticed. His haemoglobin (Hb) was 6.8 g/dl, he had reticulocytosis and a positive direct antiglobulin test. However, he did not have a high total serum bilirubin (TSB) (87.2 μmol/l). He was transfused with red blood cells and kept under phototherapy for 3 days. Three weeks later, he received another transfusion for severe anaemia (Hb 6 5 g/dl). During this period, he was never jaundiced and the maximum level of TSB was 122 μmol/l. On follow-up, his Hb stabilised and he had no further problems. This report highlights the possibility of early onset anaemia without jaundice as the sole manifestation of Rh isoimmunisation.