Bleeding complications during pregnancy and delivery in haemophilia carriers and their neonates in Western France: An observational study.

BACKGROUND: Pregnancy, delivery and the postpartum period expose haemophilia carriers, as well as their potentially affected neonates to a high risk of haemorrhagic complications. OBJECTIVES: To describe bleeding complications in haemophilia carriers and their newborns throughout pregnancy and postpartum and to identify potential factors increasing the risk of bleeding in this population. PATIENTS/METHODS: The ECHANGE multicentre observational cohort study was conducted between January 2014 and February 2019 using the BERHLINGO database comprised of patients from seven French haemophilia centres. RESULTS: During the 5 years study period, a total of 104 haemophilia carriers and 119 neonates were included, representing 124 pregnancies and 117 deliveries. Thirty-five (30%) bleeding events were observed, most of them (83%) occurred during the postpartum period, and 37% were reported during the secondary postpartum. Neuraxial anaesthesia was not complicated by spinal haematoma. Three (2.5%) neonates experienced cerebral bleeding. Caesarean section was associated with an increased risk of maternal bleeding in primary and secondary postpartum periods. Basal factor level <0.4 IU/mL was also found to be associated with an increased risk of bleeding during secondary postpartum. CONCLUSION: In our cohort, bleeding events occurred in more than a third of haemophilia carriers mainly in the postpartum period, and a significant portion of this bleeding occurred during the secondary postpartum. Haemophilia carriers warrant specific attention during primary and secondary postpartum, in particular in case of caesarean section and low basal factor level. The ECHANGE study is registered at clinicaltrials.gov identifier: NCT03360149.

Contribution of ultrasonography to the prediction of the induction-delivery interval: The ECOLDIA prospective multicenter cohort study.

INTRODUCTION: To evaluate the ability of preinduction ultrasonographic cervical length to predict the interval between induction and delivery in women at term with a Bishop score of 4 to 6 at induction. STUDY DESIGN: This multicenter prospective observational cohort recruited 334 women from April 2010 to March 2014. Inclusion criteria were women with singleton pregnancies at a gestational age ≥37 weeks, with no previous caesarean, a medical indication for induction of labor, and a Bishop score of 4, 5, or 6. All women underwent cervical assessment by both transvaginal ultrasound and digital examination (Bishop score). The induction protocol was standardized. The primary outcome measure was the induction-delivery interval. Hazard ratios (HR) and their 95% confidence intervals (95% CI) were used to assess potential predictors. RESULTS: Mean gestational age at induction was 40.1 weeks, 60.8% of the women were nulliparous, and the cesarean rate was 13.4%. The mean induction-delivery interval was 20.8 h (± 10.6). Delivery occurred within 24 h for 56.9% (n=190) of the women. An ultrasonographic cervical length measurement less than 25 mm (HR=1.50, 95% CI 1.18-1.91, P<0.01) and parity (HR=1.41, 95% CI 1.21-1.65, P<0.01) appeared to predict induction-delivery interval. The cervical length cutoff to reduce the induction-delivery interval was 25 mm. CONCLUSION: A cervical length cutoff of 25 mm was associated with shorter induction-delivery interval in women at term with a Bishop score of 4 to 6.

Ursodeoxycholic acid therapy in intrahepatic cholestasis of pregnancy: Results in real-world conditions and factors predictive of response to treatment.

BACKGROUND: Ursodeoxycholic acid (UDCA) therapy is commonly used in intrahepatic cholestasis of pregnancy (ICP). AIM: To evaluate the efficacy and tolerance of UDCA in real-world conditions and to search for factors predictive of response to treatment. METHODS: This observational study included 98 consecutive patients suffering from pruritus during pregnancy associated with increased ALT levels or total bile acid (TBA) concentrations, without other causes of cholestasis. The entire ABCB4 gene coding sequence was analyzed by DNA sequencing. RESULTS: UDCA was prescribed until delivery in all patients (mean dose 14.0mg/kg/day; mean duration 30.4 days). Pruritus improved in 75/98 (76.5%) patients, and totally disappeared before delivery in 25/98 (25.5%). After 2-3 weeks of treatment, ALT levels decreased by more than 50% of base line in 67/86 (77.9%) patients and normalized in 34/86 (39.5%), and TBA concentrations decreased in 28/81 (34.6%). Only one patient stopped the treatment before delivery. On multivariate analysis, ALT >175IU/l before treatment was associated with improvement of pruritus (OR 2.97, 95% CI 1.12-7.89, P=0.029) and with decreased ALT (OR 18.61, 95% CI 3.94-87.99, P=0.0002). ABCB4 gene mutation was not associated with response to treatment. CONCLUSION: This study supports the use of UDCA as first line therapy in ICP.

Pregnancies after transjugular intrahepatic portosystemic shunt for noncirrhotic portal hypertension.

With the growing role of transjugular intrahepatic portosystemic shunt (TIPS) in the management of portal hypertension complications, a number of women of childbearing age are now being treated with TIPS. However, if pregnancy is unusual in patients with cirrhosis, it can occur in the case of noncirrhotic portal hypertension. To our knowledge, there are no data on pregnancy safety after TIPS insertion. We report the first case of a patient with noncirrhotic portal hypertension treated by TIPS who had two successful pregnancies. She presented with HIV-associated obliterative portopathy with recurrent variceal bleeding treated by TIPS. Pregnancies occurred later and progressed normally without maternal or fetal morbidity. There was no effect on TIPS patency, but only a moderate increase in the flow velocity in the portal vein, the stent, and the hepatic artery. Thus, TIPS does not seem to impair progression of pregnancy.