4 years ago

A large case series on surgical outcomes in congenital FXIII deficiency patients in Iran

Sezaneh Haghpanah, Ghasem Miri-Aliabad, Majid Naderi, Hakimeh Tavosi, Mehran Karimi
Background The lack of accepted standardized surgical guidelines leads to dependence on the treating physicians’ and centers’ experiences. Aim Our aim is to evaluate the surgical outcomes on a large group of congenital FXIII deficiency (FXIIID) patients. Methods A case series study was conducted on congenital FXIIID patients in two major referral centers located in Iran from 2010 to 2016 prior to surgery. All patients were on prophylaxis using plasma factor XIII concentrate (10 U kg−1, every 28 days) except three patients. Single doses of 10 U kg−1 or 30 U kg−1 plasma factor XIII concentrate were given before a minor procedure and circumcision respectively. Two doses of plasma factor XIII concentrate, one 30 U kg−1 prior to the procedure and the second dose of 30 U kg−1 on post-operative day 3, were given for major surgery. The dose was 50 U kg−1 both before and after neurosurgical procedures. Results One hundred sixty-two FXIIID patients underwent minor, major and obstetrical / gynecological surgeries. Median age of the patients was 14 years (ages ranged 15 days to 47 years). The male-to-female ratio was 89/73. Five post-operative complications, two bleeding and three thrombosis, were recorded. Conclusion Our study showed excellent hemostasis in FXIIID patients undergoing surgeries. In the period of these surgeries, we observed only 1.8% post-operative complications. Surgery can be performed safely in FXIIID patients, and our proposed treatment regimens lead to adequate hemostatic coverage with minimal risk, for both minor and major surgeries. This article is protected by copyright. All rights reserved.

Publisher URL: http://onlinelibrary.wiley.com/resolve/doi

DOI: 10.1111/jth.13872

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