Pathology EMQ template.

Name: Jaspreet Singh Rayet

Candidate number: 00456526

Theme: Porphyrias

OPTION LIST

A	Acute intermittent porphyria	I
B	Erythropoietic protoporphyria	J
C	Congenital erythropoietic porphyria	K
D	Hepatoerythropoietic porphyria	L
E	Hereditary coproporphyria	M
F	Porphyria cutanea tarda	N
G	Variegate porphyria	O
H		P

For each scenario below, choose the most appropriate answer from the list above. Each option may be used once, more than once or not at all.

1. A 30 yr old lady is admitted to hospital with intermittent abdominal pain, vomiting and fever. A blood count shows a neutrophil leucocytosis. Her urine tests positive for protein and turns deep red on standing. She has recently been taking the oral contraceptive pill.

2. A 40yr old alcoholic man is admitted to hospital with bullous skin lesions. He has several scars over his arms, hands and legs. His face is noted to have areas of hyperpigmentation. A liver biopsy shows iron overload and features of cirrhosis.

3. A 40 yr old man is referred to a hospital by his GP. He is anaemic and also complains of poor vision. On examination of the eye, small lenticular scars are found. He has severe scarring on the face which has caused disfigurement. Further examination reveals splenomegaly and dystrophy of the nails.

4. A 34 yr old lady from South Africa is admitted to accident and emergency with seizures. Her urine porphobilinogen is raised. On examination of the patient, blistering skin lesions are found on her arms.

5. A 10 yr old girl is admitted to hospital with a severe burning pain on the posterior surface of the hands and feet. Examination of the blood reveals raised protoporphyrin in the red blood cells.

ANSWERS

1. A

2. F

3. C

4. G

5. B