Hereditary Angioedema-HAE is not rare in children
It is characterised by appearance of rashes over the boby with swelling of face,tongue,lips uvula and periphery of limbs.Body rashes may not be present everytime
It becomes life threatening ,when there is laryngeal swelling.
In case of Laryngeal swelling ,it becomes a respiratory emergency needing immediate treatment
The etiology, as suggested by name is absence of C1 Inhibitor-C1-INH or non functioning C1-INH from the birth due to mutation in gene SERPING 1
In most of the cases,a family history of repeated episodes of swelling over the face,lips, tongue ,is present
In case of clinical manifestations ,the fist action by a physican is to give antihistamine . If there is no adequate response to antihistamine,this condition should be suspected. This situation becomes frightening to parents as well as to treating physicians. Steroids are also given but usually not effective
The pathogenesis of HAE is different as the reactions are not mediated by histamine,rather it is mediated by Bradykinin.In absence of C1 Esterase inhibitor ,kallikrin is not inhibited leading to overproduction of Bradykinin.
Treatment is not antihistamine but anti Bradykinin after confirmation of diagnosis
Diagnosis is confirmed by laboratory estimation of C1-INH and C4. Sometimes ,the level of both are normal and the disease is present called nl-C1-INH-HAE
In July 2025,FDA has approved recombinant CI-INH ,replacement therapy ,Ruconest for children 13 years and above .In Europe ,it is recommended for use in children above 2 years of age in the dose of 50 units per kg,maximum dose 4200 units in 24 hours,equal to 2 vials.It is given IV after reconstitution over 5 minutes, the dose can be repeated once within 24 hoirs
It is published in bionews,inc January 2026
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