We report on a 42-year-old female patient with glycogen storage disease type 1a (von Gierke disease, GSD 1a) who developed hepatic adenomas and finally a hepatocellular carcinoma 10 years after renal transplantation. The tumor was resected; however, the patient died 6 months later as a result of fulminant carcinoma recurrence. In patients who have GSD 1a with terminal renal failure, combined liver and kidney transplantation may be considered at an early stage of the disease.
In selected cases, cuffed tunneled catheters via the iliac vein are implanted as a last resort access for hemodialysis. To monitor the correct position, sonography of the inferior vena cava (IVC) is sufficient in most cases. Position control using an X-ray of the abdomen is not routinely recommended when femoral catheters are implanted. In this report, we describe the case of a 59-year-old patient on chronic hemodialysis due to granulomatosis with polyangiitis and complex shunt history with multiple shunt occlusions and revisions. The implantation of an iliac-cuffed tunneled catheter led to complications because the catheter was malpositioned into the left ascending lumbar vein (ALV). It is important to be aware of potential incorrect positioning of dialysis catheters into the ALV. Due to the anatomical relation to the IVC, this happens more frequently on the left side than on the right side. In case of doubt, the correct placement of large-bore catheters via iliac access route should be verified by means of appropriate imaging before hemodialysis is performed.
Isolated, denervated, cat spleens were perfused at constant flow with modified Ringer solution. Perfusion, pressure, outflow rate, and outflow red cell concentration were measured against time. After splenic perfusion by 500 ml solution the cell washout curve became a single exponential function, indicating that only cells from the most slowly exchanging red cell compartment remained (these are immature and abnormal cells which adhere to the fine structures of the red pulp). Splenic contraction was induced by injection of 5 mug noradrenaline into the inflow after perfusion by 600 and 1000 ml of fluid, respectively; outflow cell concentration rose 17-fold before returning to baseline value and 32% of red cells in the spleen were expelled. The time course of changes in cell concentration was similar in shape but delayed with respect to that of outflow rate. The transit time of the cells from the site of release to the splenic vein must have exceeded 40 s, which is consistent only with release from the red pulp. Furthermore, at the peak of the cell concentration curve the mean reticulocyte count was 37.8%. Thus immature and abnormal red cells, which comprise the slowly-exchanging compartment, are indeed released from the spleen during contraction.
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