BOF 2.33
A 26-year-old female presents with abdominal pain, nausea
and vomiting and constipation. She complains of discolouration of her urine.
There is a previous history of epilepsy. On examination she is in pain, pulse
rate of 120 beats per minute, BP 160/90 abdomen soft not distended, no lumps
or tender areas, bowel sounds normal.
Investigations reveal:
Hb12.5 g/dL
White cell count 5.5 x 10 9 /L
Neutrophils 3.5 x 10 9 /L
Lymphocytes 1.5 x 10 9 /L
Monocytes 0.7 x 10 9 /L
Eosinophils 0.3 x 10 9 /L
Na 120 mmol/L
K 4.0 mmol/L
Urea 3.0 mmol/L
Serum creatinine 75 mmol/L
Your initial management of this patient would be:
a)
Low carbohydrate diet
b)
High protein diet
c)
Infusion of haem arginate in dilute albumin solution through a
peripheral vein
d)
Infusion of haem arginate through a central vein
e)
Infusion of tin porphyrin
Answer:
d)
The patient has acute intermittent porphyria.
She has pain, nausea, vomiting and constipation with tachycardia m
hypertension, discolouration of urine, epilepsy and hyponatraemia. These are
all suggestive features.
A high carbohydrate diet is a useful adjunct to treatment
of an acute attack and may even abort an incipient attack.
Haem arginate acts by inhibiting ALA synthetase and is
used in the management of acute attacks. It is irritant and should be
administered through a central vein. Administration in dilute albumin solution
further reduces its local irritant effect.
Tin porphyrin is a haem oxygenase inhibitor, which
enhances the efficacy of haem preparations, but they are not at present
recommended for use currently.
Revision Tip
Revise acute intermittent porphyria
KEYS to SUCCESS in Medicine page 61-62
