Liver transplantation for arteriohepatic dysplasia (Alagille's syndrome)

Thirteen out of 268 children (<18 years old) underwent hepatic transplantation (OLT) for end-stage liver disease (ESLD) associated with arteriohepatic dysplasia (AHD). Seven children are alive and well with normal liver function. Six children died, four within 11 days of the operation and the other two at 4 and 10 months after the OLT. Vascular complications with associated septicemia were responsible for the deaths of three children. Two died of heart failure and circulatory collapse, secondary to pulmonary hypertension and congenital heart disease. The remaining patient died of overwhelming sepsis not associated with technical complications. Seven patients had a portoenterostomy or portocholecystostomy early in life; five of these died after the OLT. Severe cardiovascular abnormalities in some of our patients suggest that complete hemodynamic monitoring with invasive studies should be performed in all patients with AHD, especially in cases of documented hypertrophy of the right ventricle. The improved quality of life in our surviving patients confirms the validity of OLT as a treatment of choice in cases of ESLD due to AHD. © 1992 Springer-Verlag

Forefronts in portion size. An overview and synthesis of a roundtable discussion

Establishing eating habits in early life that include appropriate portion sizes of foods which are nutrient dense and low in energy density is considered important in the prevention of obesity in children. This special supplement presents the proceedings of a symposium focusing on advances in scientific understanding of the development of healthy food portion sizes in children and their families. Recent basic research highlights individual differences in children's responsiveness to portion size as well as potential mechanisms of portion size effects. Quantitative approaches highlight the influence of maternal serving in determining intake, while qualitative approaches seek to elaborate caregiver decisions around child portion sizes at meals and snacks. Family-based environmental interventions for child weight control involving food portion size are outlined. An overview of the overarching issues and roundtable discussion on the forefronts of portion size research are presented as well as policy considerations to promote healthy portion control

Transplantation of multiple abdominal viscera.

Two children with the short-gut syndrome and secondary liver failure were treated with evisceration and transplantation en bloc of the stomach, small intestine, colon, pancreas, and liver. The first patient died perioperatively, but the second lived for more than 6 months before dying of an Epstein-Barr virus-associated lymphoproliferative disorder that caused biliary obstruction and lethal sepsis. There was never evidence of graft rejection or of graft-vs-host disease in the long-surviving child. The constituent organs of the homograft functioned and maintained their morphological integrity throughout the 193 days of survival

Onstage and off: The shifting relevance of gender in women’s prisons

uncorrected proofEven though international research on men’s prisons is no longer oblivious to gender, approaches to women’s prisons have tended to be more gender-bound as a whole. Besides having informed a specific reflexive agenda of representation, the angle of gender has presided to most research issues as an analytical overall parti pris: from the gendered nature of prison regimes to the gendered character of prison cultures, socialities and ‘pains of imprisonment’. This more ‘gendercentric’ agenda is however becoming more diversified for theoretical and empirical reasons alike. These involve a recognition of the diversity of women prisoners’ experiences and identities, and an attention to a wider variety of aspects of carceral life. Drawing on field approaches to the Portuguese carceral world spanning three decades, I propose to take this debate further by focusing on contextual shifts in the actual saliency of gender as a category of identity and social life in women’s prisons.(undefined)(undefined)info:eu-repo/semantics/publishedVersio

Extracellular Matrix Heterogeneity Regulates Three-Dimensional Morphologies of Breast Adenocarcinoma Cell Invasion

Plasticity and reciprocity of breast cancer cells to various extracellular matrice (ECMs) are three-dimensionally analyzed in quantitative way in a novel and powerful microfluidic in vitro platform. This successfully demonstrates the metastatic potential of cancer cells and their effective strategies of ECM proteolytic remodeling and morphological change, while interacting with other cells and invading into heterogeneous ECMs.International Research & Development Program (Grant 2009-00631)National Research Foundation of Korea (Basic Science Research Program Grant 2012-022481

A case of de novo duplication of 15q24-q26.3

Distal duplication, or trisomy 15q, is an extremely rare chromosomal disorder characterized by prenatal and postnatal overgrowth, mental retardation, and craniofacial malformations. Additional abnormalities typically include an unusually short neck, malformations of the fingers and toes, scoliosis and skeletal malformations, genital abnormalities, particularly in affected males, and, in some cases, cardiac defects. The range and severity of symptoms and physical findings may vary from case to case, depending upon the length and location of the duplicated portion of chromosome 15q. Most reported cases of duplication of the long arm of chromosome 15 frequently have more than one segmental imbalance resulting from unbalanced translocations involving chromosome 15 and deletions in another chromosome, as well as other structural chromosomal abnormalities. We report a female newborn with a de novo duplication, 15q24-q26.3, showing intrauterine overgrowth, a narrow asymmetric face with down-slanting palpebral fissures, a large, prominent nose, and micrognathia, arachnodactyly, camptodactyly, congenital heart disease, hydronephrosis, and hydroureter. Chromosomal analysis showed a 46,XX,inv(9)(p12q13),dup(15)(q24q26.3). Array comparative genomic hybridization analysis revealed a gain of 42 clones on 15q24-q26.3. This case represents the only reported patient with a de novo 15q24-q26.3 duplication that did not result from an unbalanced translocation and did not have a concomitant monosomic component in Korea

Deceptive body movements reverse spatial cueing in soccer

This article has been made available through the Brunel Open Access Publishing Fund.The purpose of the experiments was to analyse the spatial cueing effects of the movements of soccer players executing normal and deceptive (step-over) turns with the ball. Stimuli comprised normal resolution or point-light video clips of soccer players dribbling a football towards the observer then turning right or left with the ball. Clips were curtailed before or on the turn (-160, -80, 0 or +80 ms) to examine the time course of direction prediction and spatial cueing effects. Participants were divided into higher-skilled (HS) and lower-skilled (LS) groups according to soccer experience. In experiment 1, accuracy on full video clips was higher than on point-light but results followed the same overall pattern. Both HS and LS groups correctly identified direction on normal moves at all occlusion levels. For deceptive moves, LS participants were significantly worse than chance and HS participants were somewhat more accurate but nevertheless substantially impaired. In experiment 2, point-light clips were used to cue a lateral target. HS and LS groups showed faster reaction times to targets that were congruent with the direction of normal turns, and to targets incongruent with the direction of deceptive turns. The reversed cueing by deceptive moves coincided with earlier kinematic events than cueing by normal moves. It is concluded that the body kinematics of soccer players generate spatial cueing effects when viewed from an opponent's perspective. This could create a reaction time advantage when anticipating the direction of a normal move. A deceptive move is designed to turn this cueing advantage into a disadvantage. Acting on the basis of advance information, the presence of deceptive moves primes responses in the wrong direction, which may be only partly mitigated by delaying a response until veridical cues emerge

Cohort differences in 3-year adaptation to health problems among Dutch middle-aged, 1992–1995 and 2002–2005

Midlife is a period during which ageing-related health problems first emerge. In view of increasing life expectancy, it is of great importance that people in midlife adapt to possible health problems, to be able to lead productive and engaged lives as long as possible. It may be expected that given the better circumstances in which more recent cohorts grew up, they are better equipped to adapt to health problems than earlier cohorts. This study addresses the question if the way people in midlife adapt to health problems is or is not improving in the Netherlands. The study is based on the nationally representative 1992–1993 and 2002–2003 cohorts of the Longitudinal Aging Study Amsterdam (ages 55–64 years), with follow-up cycles in 1995–1996 (n = 811) and 2005–2006 (n = 829), respectively. Mastery is considered as a measure of adaptation, and 3-year change in mastery is compared in subjects without and with health problems at baseline. A rise was observed in the prevalence of diabetes, chronic lung disease, arthritis, subthreshold depression, and disability. Subjects without health problems in the recent cohort had better mastery than their counterparts in the early cohort. Regardless of cohort membership, mastery declined over 3 years for those with subthreshold depression, mild disability, chronic lung disease, and stroke. In the recent cohort only, mastery declined for those with cognitive impairment, but improved for those with heart disease. These findings do not support the expectation that recent cohorts are better equipped to deal with health problems for conditions other than heart disease