Strain induced esophageal growth in a novel rodent model.

Purpose: Longitudinal esophageal strain has been shown to increase esophageal length but the contribution of tissue hyperplasia to this growth is unknown. We used a novel model of esophageal stretch to determine the cellular response to the strain stimulus.

Methods: Male Sprague-Dawley rats underwent transection of the distal esophagus.

Tissue expander stimulated lengthening of arteries (TESLA) induces early endothelial cell proliferation in a novel rodent model.

Background: We examine the mechanism of aortic lengthening in a novel rodent model of tissue expander stimulated lengthening of arteries (TESLA).

Methods: A rat model of TESLA was examined with a single stretch stimulus applied at the time of tissue expander insertion with evaluation of the aorta at 2, 4 and 7day time points. Measurements as well as histology and proliferation assays were performed and compared to sham controls.

The effect of graft type on mortality in liver transplantation for hepatocellular carcinoma.

Background: Liver transplantation (LT) with living-donor (LD-P) and deceased-donor (DD-P) partial grafts for hepatocellular carcinoma (HCC) may be associated with worse outcomes. Using the United Network for Organ Sharing (UNOS), we aimed to: (1) examine the risk of mortality in LT for HCC, (2) to establish if this risk is affected by partial graft use, and (3) to determine if this effect is mitigated by improved tumor-associated risk stratification.

Material And Methods: All first-time adult LT recipients were analyzed (3/2002-12/2012), including 2,353 LD-P, 727 DD-P, and 47,833 DD whole (DD-W) grafts.

Pulmonary support on day of life 30 is a strong predictor of increased 1 and 5-year morbidity in survivors of congenital diaphragmatic hernia.

Purpose: Pulmonary support (PS) on day-of-life-30 (DOL-30) has been shown to be the strongest predictor of subsequent morbidity and in-patient mortality in congenital diaphragmatic hernia (CDH). We hypothesized that PS on DOL-30 can also predict long-term outcomes in CDH survivors.

Methods: We analyzed records of 201 CDH survivors followed by a single multidisciplinary clinic (1995-2010).

Immediate extubation after pediatric liver transplantation: a single-center experience.

The care of pediatric liver transplant recipients has traditionally included postoperative mechanical ventilation. In 2005, we started extubating children undergoing liver transplantation in the operating room according to standard criteria for extubation used for general surgery cases. We reviewed our single-center experience to determine our rates of immediate extubation and practice since that time.

Frostbite of the liver: an unrecognized cause of primary non-function?

Appropriate hypothermic packaging techniques are an essential part of organ procurement. We present a case in which deviation from standard packaging practice may have caused sub-zero storage temperatures during transport, resulting in a clinical picture resembling PNF. An 18-month-old male with alpha-1-antitrypsin deficiency underwent liver transplant from a size-matched pediatric donor.

Ductus venosus closure results in transient portal hypertension--is this the silent trigger for necrotizing enterocolitis?

Introduction: The etiology of necrotizing enterocolitis (NEC) remains elusive and no definite trigger has been identified. There are no studies to date examining the potential role of closure of the ductus venosus (DV), its effect on increasing portal venous pressure (PVP) and its association to mesenteric venous ischemia in the development of NEC. Our aim was to develop an animal model to examine this physiology.

Deceased-donor split-liver transplantation in adult recipients: is the learning curve over?

Background: Infants have the highest wait-list mortality of all liver transplantation candidates. Deceased-donor split-liver transplantation, a technique that provides both an adult and pediatric graft, might be the best way to decrease this disproportionate mortality. Yet concern for an increased risk to adult split recipients has discouraged its widespread adoption.

Pulmonary support on day 30 as a predictor of morbidity and mortality in congenital diaphragmatic hernia.

Purpose: Congenital diaphragmatic hernia (CDH) is associated with significant in-hospital mortality, morbidity and length-of-stay (LOS). We hypothesized that the degree of pulmonary support on hospital day-30 may predict in-hospital mortality, LOS, and discharge oxygen needs and could be useful for risk prediction and counseling.

Methods: 862 patients in the CDH Study Group registry with a LOS ≥ 30 days were analyzed (2007-2010).

Deceased donor liver transplantation in infants and small children: are partial grafts riskier than whole organs?

Infants have the highest wait-list mortality of all liver transplant candidates. Although previous studies have demonstrated that young children may be at increased risk when they receive partial grafts from adult and adolescent deceased donors (DDs), with few size-matched organs available, these grafts have increasingly been used to expand the pediatric donor pool. We aimed to determine the current adjusted risks of graft failure and mortality in young pediatric recipients of partial DD livers and to determine whether these risks have changed over time.

Report of 111 consecutive patients enrolled in the International Serial Transverse Enteroplasty (STEP) Data Registry: a retrospective observational study.

Background: The International Serial Transverse Enteroplasty (STEP) Data Registry is a voluntary online database created in 2004 to collect information on patients undergoing the STEP procedure. The aim of this study was to identify preoperative factors that are significantly associated with transplantation or death or attainment of enteral autonomy after STEP.

Study Design: Data were collected from September 2004 to January 2010.

Effect of CX516, an AMPA-modulating compound, on cognition and behavior in fragile X syndrome: a controlled trial.

A Phase II, 4-week randomized, double-blind, placebo-controlled clinical trial was conducted to evaluate the safety and efficacy of the Ampakine compound CX516 as a potential treatment for the underlying disorder in fragile X syndrome (FXS). After baseline screening, subjects with FXS (n = 49) underwent a 1-week placebo lead-in and then were randomized to study drug or placebo for a 4-week period. Cognitive and behavioral outcome measures were administered prior to treatment, at the end of treatment, and 2 weeks posttreatment.

Fragile X-associated tremor/ataxia syndrome in sisters related to X-inactivation.

Fragile X tremor/ataxia syndrome (FXTAS) is a recently described condition consisting of tremor, ataxia, parkinsonism, and executive dysfunction, presenting predominantly in male carriers of a fragile X mental retardation 1 premutation. In this report, we present premutation carrier sisters in whom severity of clinical signs correlated with a molecular pattern of X-inactivation favoring higher expression of the premutation allele. In these women with a common genetic background, we suggest that symptom severity may be dictated by X-inactivation, and thus a higher percentage of cells producing the premutation-containing mRNA result in increased toxicity and disease.

Psychopharmacology in fragile X syndrome--present and future.

In addition to cognitive disability, fragile X syndrome (FXS) is associated with behavioral problems that are often functionally limiting. There are few controlled trials to guide treatment; however, available information does suggest that medications can be quite helpful for a number of categories of behavioral disturbance in FXS. Specifically, stimulants appear to be quite useful for management of distractibility, hyperactivity, and impulsive behavior; antidepressants help with anxiety, obsessive-compulsive behaviors and mood dysregulation; and antipsychotics can reduce aggression.