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Adems de hepatomegalia, cursan con: nefromegalia, nefrocalcinosis, litiasis renal, osteoporosis y con un fenotipo peculiar (cara de mueca con abdomen prominente, talla baja, obesidad facio-troncular y xantomas)

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The median lethal dose (LD50) was determined by identifying the lowest dose level that resulted in two or three mortalities

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