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Liver transplantation (LT) for children with urea cycle disorders (UCDs) is capable of correcting the enzymatic defect and preventing progressive neurologic injury. We describe the characteristics and outcomes of pediatric LT recipients with UCDs. We identified all pediatric (

Congenital hepatic fibrosis (CHF) is a hereditary fibrocystic disease that can progress to portal hypertension and recurrent cholangitis requiring liver transplantation (LT). It can be associated with renal pathology and need for kidney transplantation (KT). We describe the clinical characteristics and outcomes of patients undergoing liver transplantation alone (LTA) and simultaneous liver-kidney transplantation (SLKT) for CHF using the Unites States Scientific Registry of Transplant Recipients. A total of 197 patients who received LT for CHF between 2002 and 2018 were identified - 87 (44.2%) received SLKT, 110 (55.8%) received LTA. The 1-, 3- and 5-year patient survival were 99.0%, 96.2% and 94.6%. The 1-, 3- and 5-year liver graft survival were 94.9%, 91.1% and 89.6%. No significant differences in patient or liver graft survival were observed between the SLKT and LTA groups, or between paediatric and adult recipients. 53.3% of patients with CHF necessitating LT also have significant renal disease requiring KT. Kidney graft survival for isolated KT prior to LT were poorer compared with KT performed simultaneously or after LT. Both LTA and SLKT for CHF are associated with excellent long-term outcomes in paediatric and adult patients.

We compared the waitlist mortality of 114 adolescent (12–17 years) and 217 adult (≥ 18 years) biliary atresia candidates, and the post-transplant survival of 117 adolescent and 160 adult biliary atresia liver transplant recipients using Scientific Registry of Transplant Recipients data (2002-2018). After adjusting for Model for End-stage Liver Disease score (P = 0.001) and ascites, portal vein thrombosis, encephalopathy, life support at listing (all P ≥ 0.09), adults demonstrated 10.9 times higher risk of waitlist mortality vs. adolescents (95 per cent c.i. 1.55 to 76.46; P = 0.02). Adolescents exhibited superior patient (P = 0.007) and graft survival (P = 0.006).

OBJECTIVES Variceal hemorrhage (VH) is a serious complication of portal hypertension (PH). We evaluated the feasibility, safety, and clinical impact of esophageal capsule endoscopy (ECE) in pediatric and young adult patients with known or suspected PH. METHODS Children and young adults with PH at Boston Children's Hospital (2005-2017) were offered ECE for variceal screening or surveillance. Patient histories, ECE findings, and clinical outcomes were reviewed retrospectively. RESULTS One hundred and forty-nine ECE studies were performed in 98 patients (57.1% male patients) using 3 ECE devices for variceal screening (66.5%) or surveillance (33.5%). Three readers interpreted the studies (88.3%, 10.3%, and 1.4%, respectively). Median age was 16 years (IQR 13.7-18.5). One hundred and three ECE studies involved patients

The metabolic cost associated with locomotion represents a significant part of an animal's metabolic energy budget. Therefore understanding the ways in which animals manage the energy required for locomotion by controlling muscular effort is critical to understanding limb design and the evolution of locomotor behavior. The assumption that energetic economy is the most important target of natural selection underlies many analyses of steady animal locomotion, leading to the prediction that animals will choose gaits and postures that maximize energetic efficiency. Many quadrupedal animals, particularly those that specialize in long distance steady locomotion, do in fact reduce the muscular contribution required for walking by adopting pendulum-like center of mass movements that facilitate exchange between kinetic energy (KE) and potential energy (PE) [1]–[4]. However, animals that are not specialized for long distance steady locomotion may face a more complex set of requirements, some of which may conflict with the efficient exchange of mechanical energy. For example, the “stealthy” walking style of cats may demand slow movements performed with the center of mass close to the ground. Force plate and video data show that domestic cats (Felis catus, Linnaeus, 1758) have lower mechanical energy recovery than mammals specialized for distance. A strong negative correlation was found between mechanical energy recovery and diagonality in the footfalls and there was also a negative correlation between limb compression and diagonality of footfalls such that more crouched postures tended to have greater diagonality. These data show a previously unrecognized mechanical relationship in which crouched postures are associated with changes in footfall pattern which are in turn related to reduced mechanical energy recovery. Low energy recovery was not associated with decreased vertical oscillations of the center of mass as theoretically predicted, but rather with posture and footfall pattern on the phase relationship between potential and kinetic energy. An important implication of these results is the possibility of a tradeoff between stealthy walking and economy of locomotion. This potential tradeoff highlights the complex and conflicting pressures that may govern the locomotor choices that animals make.