Percutaneous Large Bore Aspiration Thrombectomy of Tumor Embolism Causing Massive Pulmonary Embolism.

We used aspiration thrombectomy to treat a 66-year-old man with renal cell carcinoma undergoing radical nephrectomy and caval thrombectomy with a massive pulmonary artery tumor embolism. (Level of Difficulty: Intermediate.).

The First Reported Case of Hyperreninemic Hypoaldosteronism Due to Mucopolysaccharidosis Disorder.

Mucopolysaccharidoses (MPS) are rare genetic lysosomal storage disorders caused by a deficiency of enzymes that catalyze the breakdown of glycosaminoglycans. MPS-III, also known as Sanfilippo syndrome, is caused by a deficiency of one of four enzymes that catalyze heparan sulfate proteoglycan degradation. MPS-IIIA results from a deficiency of heparan sulfatase. The natural history of MPS-IIIA is marked by progressive neurodegeneration. A nine-year-old boy with developmental delay presented with progressive three-month functional decline culminating in emergency department presentation for lethargy and immobility. Laboratory workup revealed hepatic and renal failure, coagulopathy, pancytopenia, hypernatremia, and uremia requiring emergent dialysis. He developed hyperkalemia during the second month of hospitalization, the workup of which led to a diagnosis of hyperreninemic hypoaldosteronism with normal cortisol. Blood chemistry consistent with renal hypoperfusion prompted exploration of adrenal ischemia, specifically affecting the zona glomerulosa and sparing the zona fasciculata, to explain low aldosterone with normal cortisol. Heparan sulfate (HS) normally acts as a storage site for basic fibroblast growth factor (bFGF), a paracrine stimulator of aldosterone, but accumulates in MPS-IIIA due to deficiency of heparan sulfatase. If bFGF is sequestered in HS deposits in MPS-III, then paracrine signaling is reduced, accounting for the state of hypoaldosteronism. To our knowledge, this is the first reported case of hyperreninemic hypoaldosteronism caused by an MPS disorder.

The Changing Face of Hepatocellular Carcinoma: Forecasting Prevalence of Nonalcoholic Steatohepatitis and Hepatitis C Cirrhosis.

BACKGROUND/OBJECTIVES: The purpose of this research is to analyze the past and forecast the future prevalence of Hepatitis C Virus (HCV) and Nonalcoholic Steatohepatitis (NASH) and their respective contribution to Hepatocellular Carcinoma (HCC) incidence in the setting of novel anti-viral agents and rising obesity rates in the United States. METHODS: Existing data of HCV and NASH prevalence in the United States utilizing the National Health and Nutrition Examination Survey (NHANES) and Organ Procurement and Transplantation Network (OPTN) was collected and analyzed to project future prevalence trends. RESULTS: Prevalence of NASH and HCV are expected to increase and decline respectively over the next two decades with alcoholic cirrhosis expected to stay relatively unchanged. The estimated prevalence of NASH equaled and overtook the projected prevalence of HCV in 2007 at approximately 3 million persons. Estimates of NASH's contribution to HCC overtook HCV-HCC in 2015 at an approximately 25 million persons. Projection models suggest HCV prevalence declining to 1 million active cases by 2025, while NASH potentially increases to 17-42 million depending on a linear or exponential trendline. Projections of NASH-HCC similarly outpace HCV-HCC by 2025 with 45 million or 106 million (linear, exponential) versus 18 million persons respectively. CONCLUSIONS: The future prevalence of HCV and NASH are expected to become further divergent with NASH emerging as the major contributor of cirrhosis and HCC in the United States.