Intrauterine enzyme replacement therapies for lysosomal storage disorders: Current developments and promising future prospects.

Lysosomal storage disorders (LSDs) are a group of monogenic condition, with many characterized by an enzyme deficiency leading to the accumulation of an undegraded substrate within the lysosomes. For those LSDs, postnatal enzyme replacement therapy (ERT) represents the standard of care, but this treatment has limitations when administered only postnatally because, at that point, prenatal disease sequelae may be irreversible. Furthermore, most forms of ERT, specifically those administered systemically, are currently unable to access certain tissues, such as the central nervous system (CNS), and furthermore, may initiate an immune response.

How do we perform intrauterine transfusions?

Background: Intrauterine transfusion (IUT) is an invasive but critical and potentially life-saving intervention for severe fetal anemia with demonstrated improvement in outcomes. The fetus is vulnerable to hemodynamic alterations and transfusion-related adverse events; therefore, special consideration must be given to blood component selection and modification. There is widespread IUT practice variability, and existing guidance primarily relies on expert opinion and single center experiences.

Understanding Preterm Birth in Pregnancies Complicated by Nonimmune Hydrops Fetalis.

Objective: Nonimmune hydrops fetalis (NIHF) is associated with poor perinatal outcomes including preterm birth (PTB). However, the frequency and causes of PTB in this population are not well understood. We hypothesized that NIHF frequently results in PTB due to medically indicated delivery for fetal distress.

Survey of intrauterine red blood cell (RBC) transfusion practices in the United States.

Background: A paucity of data exists about the current practice of fetal red blood cell (RBC) transfusion in the United States (US). This investigation describes intrauterine transfusion (IUT) RBC product selection and processing practices at different US institutions.

Methods: A transfusion medicine and maternal-fetal medicine (MFM) team designed a survey to interrogate and characterize RBCs utilized for IUT.

Perinatal outcomes and 2017 ACC/AHA blood pressure categories.

Objective: To evaluate the association of blood pressure category < 20 weeks according to the 2017 American College of Cardiology/American Heart Association (ACC/AHA) criteria with adverse perinatal outcomes.

Study Design: A retrospective cohort study of singleton deliveries between 1/2014 and 10/2017 was undertaken. Blood pressure category assigned by 2017 ACC/AHA criteria applied to blood pressures prior to 20 weeks gestation: normal (systolic < 120 and diastolic < 80), elevated blood pressure (systolic 120-129 and diastolic < 80 mmHg), stage 1 hypertension (systolic 130-139 and/or diastolic 80-89), stage 2 hypertension (prior diagnosis of chronic hypertension or systolic ≥ 140 or diastolic ≥ 90 mmHg).

Use of Ballistocardiography to Monitor Cardiovascular Hemodynamics in Preeclampsia.

Pregnancy requires a complex physiological adaptation of the maternal cardiovascular system, which is disrupted in women with pregnancies complicated by preeclampsia, putting them at higher risk of future cardiovascular events. The measurement of body movements in response to cardiac ejection ballistocardiogram (BCG) can be used to assess cardiovascular hemodynamics noninvasively in women with preeclampsia. Using a previously validated, modified weighing scale for assessment of cardiovascular hemodynamics through measurement of BCG and electrocardiogram (ECG) signals, we collected serial measurements throughout pregnancy and postpartum and analyzed data in 30 women with preeclampsia and 23 normotensive controls.

Racial/ethnic disparities among women receiving intrauterine transfusions for alloimmunization at a single fetal treatment center.

Disparities are prevalent in numerous areas of healthcare. We sought to investigate whether there were racial/ethnic disparities among pregnant women with the most severe form of alloimmunization who require intrauterine transfusions (IUT). We reviewed patients who underwent IUT for alloimmunization at a single fetal treatment center between 2015 and 2020.

Gender Differences in Academic Rank and NIH Funding among Academic Maternal-Fetal Medicine Physicians in the United States.

Objective: This article evaluates gender differences in academic rank and National Institutes of Health (NIH) funding among academic maternal-fetal medicine (MFM) physicians.

Study Design: This was a cross-sectional study of board-certified academic MFM physicians. Physicians were identified in July 2017 from the MFM fellowship Web sites.

Fetal Congenital Pulmonary Airway Malformation: The Role of an Objective Measurement of Cardiomediastinal Shift.

Objective: To examine the relationship between cardiomediastinal shift angle (CMSA) and adverse perinatal outcomes and hydrops in cases of congenital pulmonary airway malformation (CPAM).

Study Design: This retrospective study evaluated CPAM cases referred to our institution from 2008 to 2015. The primary outcome was a composite score for adverse perinatal outcome.

Solid pseudopapillary neoplasm of the pancreas head in a pregnant woman: safe pancreaticoduodenectomy postpartum.

Solid pseudopapillary neoplasms (SPNs) of the pancreas are rare and typically arise in young women. The optimal management of a pregnant woman suspected of having an SPN of the pancreas head is unclear. We report such a case where close monitoring for tumor growth was done during pregnancy and a successful pancreaticoduodenectomy was performed after term delivery.