Maternal microchimerism in the livers of patients with Biliary atresia

BACKGROUND: Biliary atresia (BA) is a neonatal cholestatic disease of unknown etiology. It is the leading cause of liver transplantation in children. Many similarities exist between BA and graft versus host disease suggesting engraftment of maternal cells during gestation could result in immune responses that lead to BA. The aim of this study was to determine the presence and extent of maternal microchimerism (MM) in the livers of infants with BA. METHODS: Using fluorescent in situ hybridization (FISH), 11 male BA & 4 male neonatal hepatitis (NH) livers, which served as controls, were analyzed for X and Y-chromosomes. To further investigate MM in BA, 3 patients with BA, and their mothers, were HLA typed. Using immunohistochemical stains, the BA livers were examined for MM. Four additional BA livers underwent analysis by polymerase chain reaction (PCR) for evidence of MM. RESULTS: By FISH, 8 BA and 2 NH livers were interpretable. Seven of eight BA specimens showed evidence of MM. The number of maternal cells ranged from 2–4 maternal cells per biopsy slide. Neither NH specimen showed evidence of MM. In addition, immunohistochemical stains confirmed evidence of MM. Using PCR, a range of 1–142 copies of maternal DNA per 25,000 copies of patients DNA was found. CONCLUSIONS: Maternal microchimerism is present in the livers of patients with BA and may contribute to the pathogenesis of BA

Preliminary Evidence for Cell Membrane Amelioration in Children with Cystic Fibrosis by 5-MTHF and Vitamin B12 Supplementation: A Single Arm Trial

Cystic fibrosis (CF) is one of the most common fatal autosomal recessive disorders in the Caucasian population caused by mutations of gene for the cystic fibrosis transmembrane conductance regulator (CFTR). New experimental therapeutic strategies for CF propose a diet supplementation to affect the plasma membrane fluidity and to modulate amplified inflammatory response. The objective of this study was to evaluate the efficacy of 5-methyltetrahydrofolate (5-MTHF) and vitamin B12 supplementation for ameliorating cell plasma membrane features in pediatric patients with cystic fibrosis.A single arm trial was conducted from April 2004 to March 2006 in an Italian CF care centre. 31 children with CF aged from 3 to 8 years old were enrolled. Exclusion criteria were diabetes, chronic infections of the airways and regular antibiotics intake. Children with CF were supplemented for 24 weeks with 5-methyltetrahydrofolate (5-MTHF, 7.5 mg /day) and vitamin B12 (0.5 mg/day). Red blood cells (RBCs) were used to investigate plasma membrane, since RBCs share lipid, protein composition and organization with other cell types. We evaluated RBCs membrane lipid composition, membrane protein oxidative damage, cation content, cation transport pathways, plasma and RBCs folate levels and plasma homocysteine levels at baseline and after 24 weeks of 5-MTHF and vitamin B12 supplementation. In CF children, 5-MTHF and vitamin B12 supplementation (i) increased plasma and RBC folate levels; (ii) decreased plasma homocysteine levels; (iii) modified RBC membrane phospholipid fatty acid composition; (iv) increased RBC K(+) content; (v) reduced RBC membrane oxidative damage and HSP70 membrane association.5-MTHF and vitamin B12 supplementation might ameliorate RBC membrane features of children with CF.ClinicalTrials.gov NCT00730509

The Glycosyltransferase Repertoire of the Spikemoss Selaginella moellendorffii and a Comparative Study of Its Cell Wall

Spike mosses are among the most basal vascular plants, and one species, Selaginella moellendorffii, was recently selected for full genome sequencing by the Joint Genome Institute (JGI). Glycosyltransferases (GTs) are involved in many aspects of a plant life, including cell wall biosynthesis, protein glycosylation, primary and secondary metabolism. Here, we present a comparative study of the S. moellendorffii genome across 92 GT families and an additional family (DUF266) likely to include GTs. The study encompasses the moss Physcomitrella patens, a non-vascular land plant, while rice and Arabidopsis represent commelinid and non-commelinid seed plants. Analysis of the subset of GT-families particularly relevant to cell wall polysaccharide biosynthesis was complemented by a detailed analysis of S. moellendorffii cell walls. The S. moellendorffii cell wall contains many of the same components as seed plant cell walls, but appears to differ somewhat in its detailed architecture. The S. moellendorffii genome encodes fewer GTs (287 GTs including DUF266s) than the reference genomes. In a few families, notably GT51 and GT78, S. moellendorffii GTs have no higher plant orthologs, but in most families S. moellendorffii GTs have clear orthologies with Arabidopsis and rice. A gene naming convention of GTs is proposed which takes orthologies and GT-family membership into account. The evolutionary significance of apparently modern and ancient traits in S. moellendorffii is discussed, as is its use as a reference organism for functional annotation of GTs

Experiência de pais e outros familiares no cuidado à criança e ao adolescente após o transplante de medula óssea Experiencia de padres y otros familiares en el cuidado al niño y al adolescente después del transplante de medula ósea Experience of parents and other family members in the care to children and adolescents after bone marrow transplantation

O objetivo deste trabalho é descrever como os pais e outros familiares de crianças e adolescentes submetidos ao Transplante de medula Óssea (TMO) vivenciam esta experiência, especialmente na fase pós operatória. Participaram do estudo treze responsáveis por onze pacientes menores de dezoito anos. Utilizamos como instrumento de coleta de dados a entrevista semi-estruturada com os responsáveis. Para a análise dos dados recorremos a abordagem qualitativa. Através das entrevistas identificamos os seguintes temas: impacto do transplante e cotidiano familiar. Os resultados indicam que pela complexidade do TMO é indispensável o conhecimento da realidade familiar do paciente pois esta tem papel fundamental na sua qualidade de vida. Assim identificar condições, vivências e modo de vida das famílias permite trabalhar com base em suas reais necessidades, respeitando suas possibilidades e limites.<br>El objetivo de este trabajo es describir como los padres y otros familiares de niños y adolescentes sometidos al Transplante de Médula Ósea vivencian esa experiencia, especialmente después del transplante. Participaron del estudio trece responsables por once pacientes con menos de 18 años. Utilizamos como instrumento de recolección de datos la entrevista semi-estructurada con los responsables. Para el análisis de datos fue utilizado el abordaje cualitativo. A través de entrevistas fueron identificados los siguientes temas: impacto del transplante y cotidiano familiar. Los resultados indican que por la complejidad del Transplante de Médula Ósea es indispensable el conocimiento de la realidad familiar del paciente pues esta tiene un papel fundamental en su calidad de vida. Así, identificar condiciones, vivencias y el modo de vida de las familias permite trabajar con base en sus reales necesidades, respetando sus posibilidades y limites.<br>The purpose of this study is to describe how parents and other family members of children and adolescents submitted to Bone Marrow Transplantation, specially in the post operative period. Thirteen subjects responsible to the care of eleven patients under 18 years old participated in the study. Authors used semi-structured interviews to collected data. Data were analyzed through a qualitative approach. The following themes were identified: impact of transplantation and family life. Results indicated that due to the complexity of Bone Marrow Transplantation, the knowledge on the family reality is crucial as family plays a fundamental role in patients' quality of life. Therefore, to identify conditions, experiences and the life style of families enable the work based on their real needs, respecting their possibilities and limitations