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