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5 out of 7

Hepatoma is associated with?(AIIMS May 2010)

A Porphyria Cutanea tarda


C Variegate porphyria

D Erythropoietic protoporphyria

Ans. A

Porphyria Cutanea tarda

Classification and Principal Features of The Porphyrias

1. Acute intermittent porphyria (AIP)

a. Autosomal dominant

b. Porphobilinogen synthase Abnormality

c. Precipitated by drugs and Alcohol

d. Abdominal pain, polyneuropathy

e. Hypertension, neuropsychiatric Disorders

1. Congenital porphyria

a. Autosomal recessive

b. Uroporphyrin synthase abnormality

c. Extreme sensitivity to sunlight (severe scarring)

d. Dystrophy of nails and teeth, blindness

2. Variegate porphyria

a. Autosomal dominant

b. Protoporphyrinogen oxidase Abnormality

c. Features of AIP and cutaneous

d. Bullous eruption with sunlight

2. Erythropoietic protoporphyria

a. Autosomal dominant

b. Ferrochelatase abnormality

c. Cutaneous photosensitivity, Porphyria

d. Hepatic dysfunction

3. Hereditary coproporphyria

a. Autosomal dominant

b. Similar to variegate porphyria

c. Very rare

d. Due to deficiency of coproporphyrinogen oxidase

4. Porphyria cutanea tarda

a. Autosomal dominant c. Hepatic uroporphyrinogen Decarboxylase abnormality

c. Associated with alcohol; hepatic tumour

(Ref. Hari 18th ed., Pg. 3168, Fig 358.1)

Take table from (Page 3168, Fig 358.1 Hari-18th )

The human heme biosynthetic pathway indicating in linked boxes the enzyme hat, when deficient, causes the respective porphyria. Hepatic porphyrias are shown in yellow boxes and erythropoietic porphyrias in pink boxes.