Given the poor prognosis of UCD with conservative therapy, LCT caused considerable beneficial effects. Periods of hyperammonemia and clinically relevant crises could be reduced during an observation period of up to 13 months. Though cell therapy is not a permanent therapeutic option, bridging to liver transplantation may be substantially improved.

Objective: Intensive care delirium is a substantial problem in adults. Intensive care delirium is increasingly recognized in pediatrics in parallel with the development of specific scoring systems for children. However, little is known about the fluctuating course of intensive care delirium in children after surgery and possible implications on diagnostic and therapeutic strategies. Design: Patients that needed treatment in the PICU following elective surgery were screened for intensive care delirium with the Cornell Assessment of Pediatric Delirium. When the patients were awake (Richmond Agitation and Sedation Score > -3), two trained investigators conducted the Cornell Assessment of Pediatric Delirium twice daily for five consecutive days. Patients: Ninety-three patients aged 0 to 17 years. Interventions: Eight hundred forty-five assessments completed. Measurements and Main Results: Of the 845 scores, 230 were consistent with delirium (27.2%). Sixty-one patients (65.5%) were diagnosed with intensive care delirium. Half of these patients (n = 30; 32.2%) had a short-lasting delirium that resolved within 24 hours, and half (n = 31; 33.3%) had delirium of longer duration. Delirium could be clearly distinguished from sedation by analysis of individual test items of the Cornell Assessment of Pediatric Delirium. Time spent delirious had a measurable effect on outcome variables, including hospital length of stay. Conclusion: Most postoperative PICU patients develop intensive care delirium. Some have a short-lasting course, which underlines the need for early screening. Our findings support the view of delirium as a continuum of acute neurocognitive disorder. Further research is needed to investigate prophylactic and treatment approaches for intensive care delirium.

Electrospray-tandem mass spectrometry represents a powerful method for detection of inborn errors of fatty acid metabolism. In the present study, it was used to examine neonatal carnitine metabolism, which reflects fatty acid metabolism. In 70 healthy neonates, blood samples were taken from the umbilical cord and by heel-stick puncture in full-term neonates on postnatal d 5. Cord blood specimens were also obtained from 15 preterm and 10 small-for-gestational-age infants. Acylcarnitine concentrations were measured in dried blood spots by electrospray tandem mass spectrometry. Compared with cord blood, the levels of nearly all acylcarnitine species were significantly higher on the postnatal d 5, whereas free carnitine remained unchanged. Total acylcarnitine/free carnitine-ratio increased, whereas the free carnitine/total carnitine-ratio (0.54 Ϯ 0.05; p Ͻ 0.01) further decreased. A reduced availability of free carnitine in the early neonatal period may affect fatty acid oxidation and thus be of potential pathophysiological relevance under conditions with higher energy demands, e.g. in sepsis. Cord blood concentrations of free carnitine, total carnitine, and total acylcarnitines were strongly related to birth weight (p Ͻ 0.01). Lower umbilical artery pH, i.e. mild hypoxia, caused accumulation of mainly long-chain acylcarnitines. This implicates that long-chain acylcarnitines could serve as a parameter of perinatal asphyxia. In utero, the main substrates for fetal metabolism are amino acids and glucose. The human placenta is also permeable to FFA. However, they are mainly stored as triglycerides in adipose tissues and liver because of the constant supply of glucose and amino acids and a low capacity of fetal tissues for FFA oxidation. The cutoff of the nutrient supply in the presuckling period after birth leads to a transient period of starvation. Thus, the newborn infant is entirely dependent on the mobilization of glycogen and fat stores. With the initiation of milk feeding, neonatal metabolism switches to a high-fat diet. Therefore, it is obvious that fatty acid oxidation is very important in the early postnatal period (1).Carnitine plays an important role in oxidation of especially long-chain fatty acids. Carnitine palmitoyltransferases I and II mediate the transport of fatty acid-carnitine esters (acylcarnitines) across the mitochondrial membranes. Carnitine acyltransferases can also be found in peroxisomes (carnitine octanoyltransferase) and endoplasmatic reticulum (microsomal carnitine acyltransferase), although their function is not yet fully understood. In the mitochondrion, carnitine removes excess acyl groups and thus regulates the concentration of free CoA in the mitochondrial matrix (2). Several studies have shown that the carnitine pool in the neonate is limited (1, 3, 4). Tandem mass spectrometry allows quantitative determination of free carnitine and various carnitine esters like acylcarnitines (5, 6).In the present study, we used this technique to investigate the changes in neonatal carnitine and fat...