Hepatomegaly is a feature of all of the following, Except :
Correct Answer: Hepatic porphyrias
Description: Answer is D (Hepatic Pot phyrias): Frank Hepatomegaly is rare in hepatic porphyrias Although hepatomegaly may occasionally be seen in hepatic porphyrias, frank hepatomegaly is rare and not a prominent manifestation of hepatic porphyria . Hepatic porphyria is therefore the single best answer by exclusion Frank signs of hepatomegaly are rare in Hepatic Porphyrias but have been noted. Hepatic involvement in porphyria is variable Patients with acute hepatic porphyria generally have elevation of serum aminotransferases and bile acid levels. Patients with chronic hepatic porphyrias (porphyria cutanea tarda) may show liver enlargement with fatty infiltration but frank signs of hepatomegaly and iron overload are rare Hepatomegaly is a prominent manifestation of Hurler's syndrome, Nieman lick disease and Von Girke's Disease.
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