Successful orthotopic liver transplantation in an adult patient with sickle cell disease and review of the literature

Sickle cell disease can lead to hepatic complications ranging from acute hepatic crises to chronic liver disease including intrahepatic cholestasis, and iron overload. Although uncommon, intrahepatic cholestasis may be severe and medical treatment of this complication is often ineffective. We report a case of a 37 year-old male patient with sickle cell anemia, who developed liver failure and underwent successful orthotopic liver transplantation. Both pre and post-operatively, he was maintained on red cell transfusions. He remains stable with improved liver function 42 months post transplant. The role for orthotopic liver transplantation is not well defined in patients with sickle cell disease, and the experience remains limited. Although considerable challenges of post-transplant graft complications remain, orthotopic liver transplantation should be considered as a treatment option for sickle cell disease patients with end-stage liver disease who have progressed despite conventional medical therapy. An extended period of red cell transfusion support may lessen the post-operative complications

Fluid Accumulation After Neonatal Congenital Cardiac Surgery; Clinical Implications and Outcomes

BACKGROUND To determine the association between fluid balance metrics and mortality and other postoperative outcomes after neonatal cardiac surgery in a contemporary multi-center cohort. METHODS Observational cohort study across 22 hospitals in neonates (≤30 days) undergoing cardiac surgery. We explored overall % fluid overload, postoperative day 1 % fluid overload, peak % fluid overload, and time to first negative daily fluid balance. The primary outcome was in-hospital mortality. Secondary outcomes included postoperative duration of mechanical ventilation, and intensive care unit (ICU) and hospital length of stay. Multivariable logistic or negative binomial regression was used to determine independent associations between fluid overload variables and each outcome. RESULTS The cohort included 2223 patients. In-hospital mortality was 3.9% (n=87). Overall median peak % fluid overload was 4.9%, (interquartile range 0.4-10.5%). Peak % fluid overload and postoperative day 1 % fluid overload were not associated with primary or secondary outcomes. Hospital resource utilization increased on each successive day of not achieving a first negative daily fluid balance and was characterized by longer duration of mechanical ventilation (incidence rate ratio 1.11, 95% confidence interval 1.08-1.14, ICU length of stay (incidence rate ratio 1.08, 95% confidence interval 1.03-1.12), and hospital length of stay (incidence rate ratio 1.09, 95% confidence interval 1.05-1.13). CONCLUSIONS Time to first negative daily fluid balance, but not % fluid overload is associated with improved postoperative outcomes in neonates after cardiac surgery. Specific treatments to achieve an early negative fluid balance may decrease postoperative care durations

Workers’ Views of the Impact of Trade on Jobs

Although public policy is influenced by the perception that workers worry about the impact of trade on their jobs, there is little empirical evidence on what shapes such views. This paper uses new data to examine how workers' perceptions of the impact of trade are related to their career paths, job characteristics, and local labor market conditions. Surprisingly, given prior literature, we find that workers' perceptions primarily reflect local labor market conditions and education rather than labor market experiences or job characteristics

Input and Output Inventories in General Equilibrium

We build and estimate a two‐sector (goods and services) dynamic stochastic general equilibrium model with two types of inventories: materials (input) inventories facilitate the production of finished goods, while finished goods (output) inventories yield utility services. The model is estimated using Bayesian methods. The estimated model replicates the volatility and cyclicality of inventory investment and inventory‐to‐target ratios. Although inventories are an important element of the model’s propagation mechanism, shocks to inventory efficiency or management are not an important source of business cycles. When the model is estimated over two subperiods (pre ‐ and post‐1984), changes in the volatility of inventory shocks, or in structural parameters associated with inventories play a minor role in reducing the volatility of output

Coats’ Disease-Related Macular Edema Treated with Combined Aflibercept and Laser Photocoagulation

Purpose. To describe the clinical response of refractory macular edema associated with Coats’ disease following treatment with aflibercept and laser photocoagulation. Methods. Case report. Results. A 17-year-old female presented with decreased vision of the left eye. Ophthalmic exam demonstrated intraretinal hemorrhages and exudation with associated edema centrally. Angiographic evaluation revealed central leaking microaneurysms and peripheral capillary dropout. These findings and a systemic work-up that yielded an incidental Factor V Leiden mutation lead to a diagnosis of Coats’ disease. Initial treatment consisted of laser photocoagulation and intravitreal bevacizumab but with poor response. Switching to intravitreal aflibercept resulted in resolution of the refractory macular edema and improvement of visual acuity to 20/25 in the left eye. Conclusion. We describe a case of refractory macular edema which responded more favorably to intravitreal aflibercept compared with bevacizumab when combined with laser photocoagulation in a patient with Coats’ disease