Conclusion:
This is the first study on long-term follow-up of HAE from India. The major strength of the study is its duration of follow-up (1824 patient-months) which is one of the longest reported so far in children and genetic profile of HAE in India has been studied for the first time. In resource constraint settings where C1-INH concentrate is not available, FFP may be an effective option for acute management. Though recent guidelines for management of HAE in children recommend use of C1-INH for long term prophylaxis, our experience suggests that in developing countries and resource limited settings, attenuated androgens and fibrinolytic agents may also have a role in management. In the present situation, when several options are available and more effective options are being explored for long term prophylaxis and for acute management of HAE, all attempts must be made to avail these medications for patients. Use of TA, attenuated androgens and FFP may not be advocated for patients with HAE in developed countries who have access to modern treatments. However, despite best of the efforts, the modern treatments for HAE may not be available in several developing countries as is also the case in India. Our experience suggests that use of TA, stanozolol and FFP may still be an effective treatment option for patients with HAE in these countries.
Acknowledgements: None