Liver transplantation for fulminant Wilson's disease in children.

Background: Fulminant Wilson's disease (FWD) is rare and fatal condition in children unless liver transplantation is performed, however introduction of new technologies could change this poor prognosis. The aim of our study was retrospective analysis of clinical course, treatment and outcome of children with FWD treated in our institution.

Material/methods: Between 1999-2007 we've treated in our hospital 13 patients with mean age of 15.

Good and bad prognosis of alpha-1-antitrypsin deficiency in children: when to list for liver transplantation.

Alpha1-antitrypsin deficiency (alpha1-ATD) is a genetic disorder that may predispose to chronic liver disease. The clinical manifestations and prognosis of this disorder are variable. The aim of the study was to evaluate the clinical presentation and liver tests in two groups of children with alpha1-ATD: those with a good prognosis who survived long term with their native liver, and those with a bad one, requiring liver transplantation (OLT) or dying before OLT.

Cryptosporidium infection in patients with primary immunodeficiencies.

Background: Cryptosporidium species infection is usually self-limited in immunocompetent populations, but can be severe and life-threatening among immunocompromised individuals, particularly in patients with AIDS and in these patients with primary immunodeficiencies (PIDs).

Patients And Methods: A group of 5 patients with genetically confirmed hyper-IgM syndrome type 1 (XHIM) and one patient with primary CD4 lymphopenia were enrolled in the study. At least 2 stool samples and a bile sample in one patient were examined for Cryptosporidium oocysts by a modified Ziehl-Neelsen technique, by immunofluorescence assay using a commercial kit, as well as by molecular analysis followed by genotyping.

Guidelines for nutritional care for infants with cholestatic liver disease before liver transplantation.

Infants with cholestatic liver disease are at risk of various nutritional deficiencies. They require regular review with appropriate interventions to prevent complications and ensure optimum possible status if liver transplantation becomes necessary. We propose evidence based and internationally agreed guidelines for their care with recommendations for therapeutic strategies and for service organization.

Recurrence of non-alcoholic steatohepatitis after liver transplantation in a 13-yr-old boy.

Nonalcoholic steatohepatitis (NASH) is the most severe form of non-alcoholic fatty liver disease (NAFLD). The aim of our study was to highlight NASH as a rare but possible problem in children. We present a case of 13-yr-boy with a well-established diagnosis of liver cirrhosis secondary to NASH, who underwent orthotopic liver transplantation (OLT) at the age of 13 years.

Fecal pyruvate kinase: a potential new marker for intestinal inflammation in children with inflammatory bowel disease.

Objective: Inflammatory bowel disease (IBD) in children creates diagnostic and clinical challenges. Clinical data, endoscopic appearance and the histopathological assessment of biopsies are essential for diagnosis. However, new methods are required for non-invasive follow-up.

Nonalcoholic fatty liver disease as a feature of the metabolic syndrome.

The main criteria of the metabolic syndrome are obesity, insulin resistance and disturbed lipid metabolism. The same disturbances are regarded to be involved into the pathomechanism of nonalcoholic fatty liver disease which is shown by epidemiological studies and animal models. Thus NAFLD can be regarded a specific feature of the metabolic syndrome and it should be looked for in high risk populations.

Nutrition in pediatric patients before liver transplantation.

Malnutrition leading to growth failure is one of the main problems in maintainig children with chronic liver diseases. The pathogenesis of malnutrition is complex and includes reduced calorie intake, fat malabsorption, impaired protein metabolism and increased energy expenditure. The nutritional status is an important risk factor for survival post liver transplantation.

Lipid, carbohydrate metabolism, and antioxidant status in children after liver transplantation.

Organ transplantation is a risk factor for atherogenesis that may be related to immunosuppressive therapy. Increased free radical generation may even aggravate atherogenesis. The aim of the study was to assess lipid metabolism in relation to risk factors for atherogenesis as well as carbohydrate metabolism and antioxidant status among children after liver transplantation.

Short-term effects of parenteral nutrition of cholestatic infants with lipid emulsions based on medium-chain and long-chain triacylglycerols.

Objective: Infants with chronic cholestasis may require parenteral nutrition with lipid emulsions to provide energy and essential fatty acids but the optimal strategy is controversial.

Methods: We studied the effects of parenteral lipid emulsions with long-chain triacylglycerols (LCTs) or a mixture of LCTs and medium-chain triacylglycerols (MCTs/LCTs) on serum bilirubin and lipid metabolism in cholestatic infants who received these 20% emulsions in alternating order for 3 d each, together with a glucose and amino acid infusion.

Results: Of 11 recruited infants, two dropped out because enteral feeding could be established.

[Oxidative stress and hypertensive arteriopathy in children with primary hypertension--preliminary results].

Unlabelled: Hypertensive arteriopathy and intima-media (IMT) thickening is observed already in adolescents with primary hypertension (PH) at diagnosis. The injury of arterial wall may cause also generation of free radicals and free radicals may by itself perpetuate arterial wall injury. The aim of the study was to verify the hypothesis that children with PH are exposed to oxidative injury (Sox) due to impaired antioxidant barrier, and that markers of Sox correlate with IMT and metabolic risk factors of arteriosclerosis.

[Efficacy and safety of cyclosporine in the treatment of inflammatory bowel disease in children - a retrospective study].

Aim: the aim of this study was to report single centre experience with cyclosporine used in treatment of children with inflammatory bowel disease with regard to safety and efficacy.

Methods: retrospective analysis included 23 patients, 21 with ulcerative colitis and 2 with Crohn's disease, aged 2.75 to 18.

Challenges and opportunities in Pan-European collaboration for researchers from Central and Eastern Europe.

Ten Central and Eastern [NLG4] European countries have recently joined the European Union. This historical enlargement provided a good opportunity to discuss the challenges and opportunities in Pan-European Research Collaboration for researchers from Central/Eastern Europe. This paper summarises examples of productive research collaboration between East and West, current challenges [NLG5], and ideas on how to facilitate better collaboration.

[Prevalence of Kayser-Fleischer ring in patients with Wilson's disease].

The aim of the study was to assess prevalence of Kayser-Fleischer ring in children with Wilson's disease. 36 patients aged 7-17 years were enrolled into the study. All of them underwent ophthalmic examination by slit-lamp biomicroscopy.

Essential fatty acid depletion in children with inflammatory bowel disease.

Objective: Children with inflammatory bowel disease (IBD) suffer from malabsorption and malnutrition and therefore may be at risk of developing polyunsaturated fatty acid (PUFA) deficiency. The aim of this study was to investigate PUFA status in children with IBD and the possible relationship to disease activity and nutritional status.

Material And Methods: We assessed the fatty acid composition of plasma phospholipids (%wt/wt) of 21 children aged 5.

[Two cases of Wilson disease diagnosed as autoimmune hepatitis].

Authors present two cases of Wilson disease in which the final diagnosis was very difficult and needed the differentiation with autoimmune hepatitis. Observation of those two patients leads to the conclusion that paediatricians have to deepen they knowledge of autoimmune hepatitis and Wilson disease which are seen more often in children now-a-days. It will help to quicker the diagnosis and treatment which is most essential for better prognosis in those patients.

Dietary intake and nutritional status of children and adolescents in Europe.

The objective of this project was to collect and evaluate data on nutrient intake and status across Europe and to ascertain whether any trends could be identified. Surveys of dietary intake and status were collected from across Europe by literature search and personal contact with country experts. Surveys that satisfied a defined set of criteria -- published, based on individual intakes, post-1987, adequate information provided to enable its quality to be assessed, small age bands, data for sexes separated above 12 years, sample size over 25 and subjects representative of the population -- were selected for further analysis.

Allogeneic hematopoietic cell transplantation from alternative donors in children with myelodysplastic syndrome: is that an alternative?

Allogeneic hematopoietic cell transplantation (HCT) in children with myelodysplastic syndrome (MDS) remains a challenge due to the toxic conditioning regimens administered to minimize the risk of relapse in the HLA-matched or of graft rejection in the HLA-mismatched settings. In the absence of matched sibling donors, alternative donors such as unrelated and/or partially matched family sources remain risky, yet the only available, options. Herein we report the results of HCT from alternative donors in 14 children with different subtypes of MDS (juvenile myelomonocytic leukemia [JMML] n = 9; myelodysplastic syndrome [MDS] refractory anemia n = 3; MDS refractory anemia with excess of blasts in transformation n = 2) transplanted at our institution.

Beneficial effect of liver transplantation on bone mineral density in small infants with cholestasis.

Reports of bone mineral density in children after liver transplantation are few. Eleven cholestatic children were analyzed before and 6 months after liver transplantation. No changes in serum levels of calcium, alkaline phosphates, or 25OHD were observed before versus after LTx.

Trans fatty acids in human milk in Poland and their association with breastfeeding mothers' diets.

Aim: To determine the content of trans fatty acids in human milk in relation to breastfeeding mothers' diet.

Methods: Samples of milk were collected from 100 breastfeeding mothers and 7-d dietary records and anthropometry from 69 mothers were obtained.

Results: The following total trans fatty acids contents (median (lower-upper quartile); % wt/wt) in milk samples were determined: 1) data for Spring: colostrum--1.

Reduction of naive CD4/CD45RA+ T cells in children with biliary atresia before and after liver transplantation.

The aim of this study was to examine whether liver transplantation reverses the abnormal distribution of lymphocyte subsets previously observed in biliary atresia children, namely a selective decrease in the naive CD4/CD45RA+ T cell subset and an increase in the B and natural killer cell subpopulations. Eight biliary atresia children aged 1.08 to 6 years were studied before and 1 year after LTx for comparison with 15 age-matched healthy controls.