Table 1.

Treatment of Hermansky-Pudlak syndrome at the time of delivery

Article	Year	Article type	Treatment	Type of delivery	Result
Reiss et al7	1985	Case report	Prophylactic platelet transfusion None	Spontaneous labour at 40 weeks. Vaginal delivery Spontaneous labour at 39 weeks. Vaginal delivery	Transfusion reaction after platelet administration, treated with diphenhydramine and hydrocortisone. No abnormal intrapartum or postpartum bleeding Epistaxis during second stage of labour. Uncomplicated delivery with an estimated blood loss of 400 mL
Wax et al8	2001	Case report	Prophylactic platelet transfusion	Vaginal delivery at term	Uneventful delivery
Zatik et al2	2002	Case report	Prophylactic DDAVP (0.3 μg/kg) on induction Prophylactic DDAVP (0.3 μg/kg); 4 units of packed cells and 2 units of platelets standby	Induction at 41 weeks resulting in an emergency caesarean section for fetal distress SROM at 39 weeks, resulting in repeat caesarean section for fetal distress	Estimated blood loss of 1600 mL, for which 4 units of packed cells and 2 units of platelets were administered. Normal postpartum lochia Uneventful delivery, no need for blood replacement or platelet transfusion
Poddar et al3	2004	Case report	None	SROM at 40 weeks. Vaginal delivery	Initial estimated blood loss of 250 mL due to second-degree tear, forming a vulval haematoma. Small but continuing blood loss postpartum: re-exploration of the tear revealed no active bleeding, but continuous ooze from raw areas. The vagina was packed with swabs. Systolic blood pressure was 60–70 mm Hg and estimated blood loss was 1800 mL. Two units of blood and 1 L of gelofusine were administered. The surgical pack was removed under DDAVP 0.3 μg/kg in 50 mL of saline intravenous over 20 min with minimal blood loss
Beesley et al4	2008	Case report	DDAVP during second stage of labour with repeat dosing every 8 hours DDAVP infused over 30 min in late second stage of labour	Induction at term. Vaginal delivery Induction at term. Vaginal delivery	Uncomplicated delivery, estimated blood loss of 1500 mL resulting from second-degree perineal laceration; patient received 2 units of packed cells and 2 units of pooled platelets Episode of brisk bleeding after placental separation, controlled with bimanual massage, 1 unit of platelets and 60 units of oxytocin diluted in 1 L of saline solution. Total blood loss estimated 1000 mL. Normal postpartum lochia
Spencer and Rosengren11	2009	Case report	4 units of platelets 2.5 hours prior to delivery	Induction at 39 weeks. Vaginal delivery	Estimated blood loss of 700 mL, causing a drop of haematocrit from 32.6% to 27.9%. Platelet count went from 210 000 before delivery to 326 000 after platelet transfusion. A second-degree laceration was repaired. Discharge after 2 days without complications
Nisal et al9	2012	Case report	Transfusion of platelets before surgery Infusion of tranexamic acid postoperatively 5-day course of oral tranexamic acid postpartum	Elective caesarean section of DCDA twins with general anaesthesia	Uneventful postoperative recovery
Harris-Glocker et al6	2013	Case report	DDAVP immediately prior to caesarean delivery Units of packed cells crossed and typed in anticipation of severe bleeding	Urgent caesarean section of spontaneous DCTA triplets at 313/7 weeks for oligohydramnios and fetal distress	Severe uterine atony responsive to oxytocin, uterine massage and methylergonovine, with a total blood loss of 800 mL. Preoperative haematocrit value of 26 dropped to a postoperative value of 19 and remained stable. Discharge after 4 days without complications
Bachmann et al5	2014	Case report	Prophylactic DDAVP Postpartum DDAVP	Vacuum delivery due to birth arrest	No bleeding complications. Estimated blood loss 300 mL
Civaschi et al10	2015	Clinical trial Multicentre study	None Prophylactic platelet transfusion	Vaginal delivery Caesarean section with general anaesthesia	No bleeding complications
Van Avermaete	2016	Case report	Prophylactic platelet transfusion in 3 doses over 36 hours	Caesarean section with general anaesthesia	No bleeding complications during the procedure. Estimated blood loss of 600 mL. Haematoma formation at the site of the wound after 72 hours with secondary infection