Health-related quality of life in adolescents with inflammatory bowel disease depends on disease activity and psychiatric comorbidity.

Adolescent patients with inflammatory bowel disease (IBD) show an increased risk for behavioral and emotional dysfunction. Health-related quality of life (HRQoL) is influenced by medical illnesses, as well as by psychiatric disorders, but for adolescents with IBD, the extent to which HRQoL is influenced by these two factors is unclear. For 47 adolescent IBD patients, we analyzed disease activity, HRQoL and whether or not a psychiatric disorder was present. Disease activity was estimated using pediatric Ulcerative Colitis Activity Index and pediatric Crohn's Disease Activity Index. The IMPACT-III and the EQ-5D were used to measure HRQoL and QoL, respectively. In addition, patient and parent diagnostic interviews were performed. 55.3 % patients fulfilled DSM-IV criteria for one or more psychiatric disorders. In all patients, psychiatric comorbidity together with disease activity contributed to a reduction in quality of life. Adolescents with IBD are at a high risk for clinically relevant emotional or behavioral problems resulting in significantly lower HRQoL. We conclude that accessible, optimally structured psychotherapeutic and/or psychiatric help is needed in adolescent patients with IBD.

Indications for pediatric liver transplantation. Data from the Heidelberg pediatric liver transplantation program.

Nowadays liver transplantation is an established treatment for children with end-stage liver disease with very good 1- and 5-year survival. This has been achieved through constant improvement of surgical techniques, new immunosuppressive drugs and clinical management. Indications for liver transplantation in infants and children include acute liver failure (ALF), chronic liver failure with pruritus, complications of cholestasis and failure to thrive. In young children, the most common liver disease leading to transplantation is biliary atresia. Biliary atresia accounts for at least 50 percent of all liver transplants in children and is characterized by the failure of the bile ducts to develop normally and drain bile from the liver. Several models to assess prognosis of liver disease have been developed. In acute liver failure leukocyte count, bilirubin, International Normalized Ratio (INR) and age have a strong correlation with outcome. In chronic liver failure, PELD (Pediatric end-stage liver disease) Score and the occurrence of complications of liver disease are important prognostic tools. Since the start of our own paediatric liver transplantation program at the University of Heidelberg in 2003, already 15 Children between 5 months and 14 years have been transplanted. Indications and outcome of these patients are reviewed in this paper.

Plasma carotenoids in German children and adolescents.

UNLABELLED: Carotenoids are important antioxidants and precursors of vitamin A, but only few studies have been carried out on plasma carotenoid levels in paediatric age groups. Using high-performance liquid chromatography we analysed concentrations of four important carotenoids (alpha-carotene, beta-carotene, cryptoxanthin, lycopene) in 129 healthy German children and adolescents (81 boys, 48 girls; age 1-18 years). For all carotenoids analysed, no significant differences between girls and boys were found. alpha-Carotene values ranged from 0 mumol/l (below detection limits) to 0.73 mumol/l (0-395 micrograms/l), beta-carotene from 0.09 mumol/l to 2.68 mumol/l (48-1443 micrograms/l), lycopene from 0 mumol/l to 1.51 mumol/l (0-815 micrograms/l), and cryptoxanthin from 0 mumol/l to 0.30 mumol/l (0-164 micrograms/l), respectively. Data analyses according to age groups showed a tendency towards higher levels of alpha- and beta-carotene, and lower levels of lycopene in young children. These differences were significant when children younger than 4 years were compared with those above 8 and 12 years, respectively. The data presented may serve as reference values for studies on children with nutritional disorders and diseases associated with a risk of vitamin deficiencies. CONCLUSION: Carotenoids are important antioxidants and singlet oxygen scavengers. Plasma levels of alpha-carotene, beta-carotene, lycopene and cryptoxanthin were determined in 129 healthy German children and adolescents. Highest values were found for beta-carotene and lycopene.

S-Acetylglutathione normalizes intracellular glutathione content in cultured fibroblasts from patients with glutathione synthetase deficiency.

Glutathione synthetase deficiency is an autosomal recessive inherited metabolic defect in the gamma-glutamyl cycle. Decreased intracellular glutathione levels are one of the characteristic biochemical features. In this study we show that addition of S-acetylglutathione to the medium raised intracellular glutathione content in cultured fibroblasts from patients with glutathione synthetase deficiency. This has implications for the treatment of patients with this inborn error of metabolism.

Influence of dialysis on plasma lipid peroxidation products and antioxidant levels.

In patients with end-stage renal failure (ESRF), the incidence of atherosclerosis and cancer is increased. The importance of lipid peroxidation (LPO) products in the pathogenesis of these complications has recently been emphasized. The LPO products malondialdehyde (MDA) and hexanal, lipophilic antioxidants and erythrocyte glutathione (GSH) were estimated in 10 pediatric hemodialysis (HD) patients before and after HD and in 11 peritoneal dialysis (CPD) patients. Before HD, MDA was elevated [median (interquartile range): 384.5 (110 to 501) nM; normal < 150 nM], whereas plasma hexanal levels were normal in all patients [130.5 (88 to 222) nM; < 320 nM]. HD decreased MDA concentrations on average by 88% but did not change hexanal levels. CPD patients exhibited high plasma MDA concentrations [371 (287 to 468) nM], whereas hexanal was in the low normal range [56 (51 to 81) nM]. Antioxidants were normal in both groups and unchanged during HD. GSH decreased slightly during HD. We hypothesize that MDA may accumulate in ESRF due to reduced plasma clearance. Our results argue against a general increase of LPO in uremia.

The clinical manifestation of the kwashiorkor syndrome is related to increased lipid peroxidation.

Along with the onset of severe kwashiorkor symptoms, a 20-month-old child showed biochemical signs of markedly increased lipid peroxidation, with a decrease of plasma antioxidants and decreased proportions of polyunsaturated fatty acids in plasma and red cell phospholipids. Additionally, plasma concentrations of the lipid peroxidation products malondialdehyde and hexanal, as well as the urinary excretion of leukotriene E4, were found to be increased. All biochemical alterations normalized along with subsequent clinical improvement. These findings suggest that the extent of lipid peroxidation is strongly related to the severity of the kwashiorkor syndrome.

Carotenoid supply in breast-fed and formula-fed neonates.

UNLABELLED: Carotenoids have various biological functions including their role as antioxidants. For humans fruits and vegetables are the only source of carotenoids. In the first months breast milk and/or formula preparations are the only nutrition for infants. To study the influence of nutrition on the plasma carotenoid profile in newborns, breast milk, different formula preparations, and the plasma of breast-fed (BF) and formula-fed (FF) newborns were analyzed by high-performance liquid chromatography. The method used allowed beta-carotene, alpha-carotene, lycopene, and beta-cryptoxanthine to be detected and all four were found in breast milk. In colostrum carotenoids were up to five times higher than in mature breast milk (P<0.05). In contrast, not all carotenoids could be found in formula preparations. Beta-carotene was detected in four out of eight, and beta-cryptoxanthine in three out of eight formula preparations. Lycopene and alpha-carotene were not detectable in any of the formula preparations. Four formula preparations did not contain any carotenoids. FF infants had different plasma carotenoid profiles compared to BF infants. beta-carotene was significantly lower in FF infants [14 (0-32) microg/l, median and interquartile ranges] than in infants after birth [24 (19-310) microg/l, P<0.05], and BF infants [32 (22-63) microg/l, P<0.05]. While newborns after birth had measurable plasma concentrations of lycopene (16 [14-18] microg/l) and of alpha-carotene [5 (0-8) microg/l), these carotenoids were no longer detectable in FF infants after day 14. CONCLUSION: FF and BF infants show significant biochemical differences in plasma carotenoid concentrations.