Pathology EMQ template.

Name: James Burton

Theme: Porphyrias

 

OPTION LIST

 

A	Acute Intermittent Porphyria	I	Harderoporphyria
B	ALA dehydrates Deficiency	J	Erythropoietic Protoporphyria
C	Porphyria variegata	K
D	Hereditary Coproporphyria	L
E	Congenital Erythroblastic porphyria	M
F	Porphyria Cutanea tarda	N
G	Toxic Porphyria	O
H	Hepatoma-erythroblastic porphyria	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 premenstrual female patient in the UK presents with Abdominal pains, nausea, vomiting, fever, sweating, restlessness and a slight tremor after the administration of hydrocortisol. Examination reveals a tachycardic and hypertensive patient while investigations reveal increased urinary ALA and PBG with reduced HMBS in her erythrocytes.

 

 

2. A 31yr old male patient who had been drinking presents with vesicles over his exposed hands with associated crusting and superficial scarring with pigmentation. Investigations reveal increased urinary uroporphyrins and coproporphyrins with increased ferritin levels.

 

 

3. Which porphyria is associated with the enzyme Ferrochelatase?

 

 

4. Which porphyria is associated with the enzyme Coproporphyrinogen oxidase?

 

 

5. Which porphyria is associated with the enzyme Protoporphyrinogen oxidase?

 

 

 

ANSWERS

1. A

2. F

3. J

4. I

5. C