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  • 11
    ISSN: 1432-198X
    Keywords: Key words: Hyperammonemia ; Carbamoyl phosphate synthetase deficiency ; Hemodialysis ; Recirculation ; Clearance
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. A 2-year-old girl with carbamoyl phosphate synthetase deficiency underwent emergency hemodialysis (HD) for treatment of acute life-threatening hyperammonemia. HD was performed via catheters placed in each femoral vein serving as vascular access. The tip of one of the catheters (aspirating line) was in the left external iliac vein and the tip of the other catheter (the return line) was in the inferior vena cava (IVC). High blood flow rates were used in order to rapidly lower the blood ammonia (NH3) levels. However, unanticipated marked recirculation in the IVC, between the dialysis aspirating and return catheters, was encountered, preventing significant reduction in blood NH3. The recognition of this problem, suggested solutions, and prevention are described.
    Type of Medium: Electronic Resource
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  • 12
    ISSN: 1432-198X
    Keywords: Sialidosis ; Proteinuria ; Lectin histochemistry ; Visceral epithelial cell
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 9-year-old body with sialidosis had nephrotic-range proteinuria. Histological studies demonstrated massive distension of renal cells, particularly glomerular visceral epithelial cells, by cytoplasmic vesicles which contained material reactive with concanavalin A and wheat-germ agglutinin. In addition, some glomeruli exhibited segmental mesangial thickening or glomerulosclerosis. Ultrastructurally, focal detachment of visceral epithelial cells from the underlying glomerular basement membrane was observed. We postulate that glomerular visceral epithelial cell dysfunction may underlie the proteinuria and focal glomerulosclerosis exhibited by this patient. Hyperfiltration, as suggested by the child's elevated creatinine clearances, may be a contributing factor.
    Type of Medium: Electronic Resource
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