Abstract
The spleen is frequently enlarged in genetic disorders, particularly those characterized by hemolysis, storage, infiltration, portal hypertension, immune deficiency, or abnormal phagocytic function (Table 1). The topic of this chapter is metabolic disorders, or inborn errors of metabolism, that result in splenomegaly, especially those disorders with surgical implications for the spleen. Hemolytic disorders are discussed in another chapter in this volume. I will summarize the genetic and biochemical aspects of the diseases with storage or infiltration, as well as the indications for and results of surgical intervention, and any alternative therapies available. For a more complete discussion of each disorder, the reader is referred to Scriver et al. [45]
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Wilcox, W.R. (1997). Metabolic Disorders and the Spleen. In: Hiatt, J.R., Phillips, E.H., Morgenstern, L. (eds) Surgical Diseases of the Spleen. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-60574-1_10
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DOI: https://doi.org/10.1007/978-3-642-60574-1_10
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