ABSTRACT
Cryoglobulinemia can induce systemic vasculitis affecting various organs such as skin, peripheral nerves, and kidney. The disease can induce chronic organ failure and even be life-threatening. Cryofiltration has been applied for the treatment of cryoglobulinemic vasculitis. We have experienced four cases with mixed cryoglobulinemia showing severe and progressive clinical manifestations, including skin purpura, nephrotic syndrome, acute kidney injury, and peripheral neuropathy. Cryofiltration in conjunction with conventional pharmacological therapies appeared to be safe and effective. After the treatments, plasma cryoglobulins were markedly reduced and the disease was well controlled. Although its efficacy has not yet been well established, this report can be another evidence showing efficacy of cryofiltration for treatment of mixed cryoglobulinemia.
Subject(s)
Cryoglobulinemia/therapy , Cryoglobulins/metabolism , Plasmapheresis/methods , Systemic Vasculitis/therapy , Adult , Aged , Cryoglobulinemia/complications , Cryoglobulinemia/physiopathology , Disease Progression , Filtration/methods , Humans , Male , Middle Aged , Severity of Illness Index , Systemic Vasculitis/etiology , Treatment OutcomeABSTRACT
PURPOSE: To observe a case of congenital extrahepatic portosystemic shunt and discuss it from the embryological and clinical viewpoints. METHODS: An 85-year-old female cadaver was employed for a dissection course at Aichi Medical University in 2009. RESULTS: There was no evidence of liver cirrhosis macroscopically or microscopically. A portosystemic shunt was observed that involved communication between the inferior mesenteric vein, inferior vena cava (IVC), and left ovarian vein by a single Y-shaped shunt vessel. CONCLUSIONS: To the best of our knowledge, this is the first reported case of the above-mentioned three veins being connected by a single Y-shaped shunt vessel. Considering the other venous diameters, the shunt appeared to flow into the splenic vein and IVC. It cannot be denied that this shunt may have led to hepatic encephalopathy, although the shunt effect may have been minimal. Embryological development of IVC appears to occur close to the plexus of anastomosing vitelline veins, forming the portal vein.