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Postoperatively, the patient's factor X levels were controlled daily and corrected using SD-FFP as needed, with no clinically significant blood loss. CONCLUSIONS SD-FFP can be used to manage congenital factor X deficiency in the peripartum period and maintain perioperative blood loss within normal limits.BACKGROUND Birt-Hogg-Dubé Syndrome (BHDS) characterised by skin fibrofolliculomas, kidney tumour and pulmonary cysts/pneumothorax is caused by folliculin (FLCN) germline mutations. The pathology of both neoplasia and focused tissue loss o