Hepatomegaly is an essential feature of: (A) Von Gierke's disease, (B) Nieman Pick disease, (C) Hurler syndrome, (D) Hepatic porphyria
Answer is D. 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.