Outcomes of haemoglobin Bart's hydrops fetalis following intrauterine transfusion in Ontario, Canada.

OBJECTIVES: With improved access to intrauterine transfusion (IUT), more fetuses with haemoglobin Bart's hydrops fetalis (HBHF; homozygous α0-thalassaemia) will survive. DESIGN: To evaluate the long-term outcome of affected fetuses with and without IUT in Ontario, Canada, we retrospectively collected data on IUTs and pregnancy outcomes in all cases of HBHF, from 1989 to 2014. Clinical outcome and neurocognitive profiles of long-term survivors were also collected and compared with data from 24 patients with transfusion-dependent ß-thalassaemia (TDT-ß). RESULTS: Of the 99 affected pregnancies (93 prenatally diagnosed), 68 resulted in miscarriage or elective termination of pregnancy. Twelve mothers (12%) continued their pregnancies without IUT, and none of those newborns survived the first week of life. All 13 fetuses that received IUT(s) were live-born, but 3 died due to severe hydrops at birth and 1 died due to infection. The remaining nine survivors, in comparison with TDT-ß patients, had earlier iron overload requiring iron chelation therapy. Endocrinopathies and short stature were more frequent in these patients. Neurocognitive outcome was not significantly affected in five patients who were assessed, and none were diagnosed with intellectual impairment. In three patients, MRI studies demonstrated brain white matter changes in keeping with 'silent' ischaemic infarcts. CONCLUSIONS: In patients with HBHF, IUT is associated with improved survival. While acceptable neurocognitive outcome can be expected, these patients have more clinical complications compared with their TDT-ß counterparts. The clinical and neurocognitive outcomes of HBHF should be discussed in detail when counselling and offering IUT for patients.

Maternal outcomes in pregnancies affected by varicella zoster virus infections: population-based study on 7.7 million pregnancy admissions.

AIM: Previous estimates of the incidence of varicella zoster virus (VZV) pneumonia and maternal death associated with VZV infection among the pregnant population have varied considerably and been based predominantly on reports from case series. We sought to measure the incidence of VZV-related morbidity and mortality to provide more representative population estimates. METHODS: We carried out a large cohort study on all births using the United States Healthcare Cost and Utilization Project-Nationwide Inpatient Sample database between 2003 and 2010. Descriptive statistics were used to measure baseline characteristics and outcomes of women with VZV infection. Multivariate logistic regression analyses were used to identify risk factors for the development of VZV-related morbidity and mortality. RESULTS: We identified 935 patients admitted for VZV infection among 7.7 million pregnancy admissions, representing an incidence of 1.21 cases/10 000 pregnancies (95% confidence interval [CI], 1.13-1.29). The incidence of VZV pneumonia was 2.5% (95% CI, 1.6-3.7). No maternal deaths were recorded during the 8-year study period. There were no significant risk factors identified for those who developed VZV pneumonia compared to those who had an uncomplicated VZV infection during pregnancy. CONCLUSION: The incidence of VZV pneumonia and VZV infection associated with maternal death is significantly lower than previously estimated and may reflect better immunization and earlier interventions.