there is a low level of C1 esterase inhibitor, and sometimes life threatening episodes of oedema of the upper airway may develop in response to stress or local trauma, particularly dental treatment (Frigas 1989). However, attacks of oedema may occur without any obvious reason, and recurrent abdominal pain may be a presenting feature.
3. As with the hereditary form, epinephrine (adrenaline), antihistamines, and steroids are ineffective for prophylaxis, and for treatment of these attacks.
4. The two conditions may be distinguished by the fact that in the acquired form the onset is late, no family history is elicited, no complement abnormalities are found in the patient's blood relatives, and the underlying malignancy may already have been diagnosed.
5. Differentiation may now be made on measurement of the C1q subunit of C1;patients with acquired deficiency have a decreased level of C1q, compared with those with the hereditary form, in whom the C1 level is normal (Alsenz et al 1987).
Was this article helpful?