Impact statement:
We report the largest single centre cohort of type 1 hereditary
angioedema (HAE) from India. Most patients had novel mutation inSERPING1 gene. Clinical profile of patients in the present series
was found to be similar to several previously reported series. Because
of lack of availability of C1-INH therapy in India, patients were
managed using attenuated androgens, tranexamic acid and fresh frozen
plasma. Only one disease related mortality was reported in the entire
cohort. Results of this study suggest that use of TA, stanozolol and FFP
may still be an effective treatment option for patients with type 1 HAE
in resource constrained settings.