Hepatomegaly is an essential feature of
Hepatomegaly is an essential feature of von Gierke's disease (all glycogen storage diseases except type 2, 5, 7), Hurler (Scheic) syndrome (& all mucopolysaccharidosis) and Nieman-Pick disease. But the hepatic involvement of hepatic porphyria is variable. Patients with chronic hepatic porphyria (porphyria cutanea tarda), however may show liver enlargement d/t fatty infiltration but frank sings of hepatomegaly and iron overload are rare.
Von Gierke's disease
Nieman Pick disease
Hurler syndrome
Hepatic porphyria