Blood pressure thresholds for the diagnosis of hypertensive disorders of pregnancy in sickle cell disease.

In this retrospective cohort study of singleton pregnancies in people with sickle cell disease (SCD) delivered at two academic centres between 1990 and 2021, we collected demographic and SCD-related data, pregnancy outcomes, and the highest systolic and diastolic blood pressure (SBP and DBP) at seven time periods. We compared the characteristics of subjects with new or worsening proteinuria (NWP) during pregnancy to those without. We then constructed receiver operating characteristic (ROC) curves to determine the blood pressure (BP) that best identifies those with NWP. The SBP or DBP thresholds which maximized sensitivity and specificity were 120 mmHg SBP (sensitivity: 55.2%, specificity: 73.5%) and 70 mmHg DBP (sensitivity: 27.6%, specificity: 67.7%). The existing BP threshold of 140/90 mmHg lacked sensitivity in both genotype groups (HbSS/HbSß0 : SBP = 21% sensitive, DBP = 5.3% sensitive; HbSS/HbSß+ : SBP = 10% sensitive, DBP = 0% sensitive). Finally, percent change in SBP, DBP and MAP were all poor tests for identifying NWP. Existing BP thresholds used to diagnose hypertensive disorders of pregnancy (HDP) are not sensitive for pregnant people with SCD. For this population, lowering the BP threshold that defines HDP may improve identification of those who need increased observation, consideration of early delivery and eclampsia prophylaxis.

Knowledge of fertility and perception of fertility treatment among adults with sickle cell disease (KNOW FERTILITY).

Introduction: This study assessed fertility knowledge in adults with sickle cell disease using the Cardiff Fertility Knowledge Scale and Fertility Treatment Perception Survey and compared knowledge scores in respondents with sickle cell disease to previously reported unaffected cohorts. Methods: This cross-sectional study surveyed adults over age 18 with sickle cell disease at an adult sickle cell disease center using a 35-question survey addressing infertility risk factor knowledge and perceptions of fertility treatment. Analyses included summary statistics for continuous and categorical variables, univariate linear regression, and Mann-Whitney U tests for group comparisons of Fertility Knowledge Scale scores. Fertility Treatment Perception Survey scores were measured by medians of the two positive statements and four negative statements to generate separate positive and negative treatment belief scores. Statistical significance was set at p < 0.05 for all analyses. Results: Ninety-two respondents (71 female, 21 male) with median age of 32 years (IQR: 25.0, 42.5) completed the survey between October 2020-May 2021. Sixty-five percent of respondents reported taking sickle cell disease treatment and 18% reported refusing at least one sickle cell disease treatment due to fertility concerns. The mean Fertility Knowledge Score was 49% (SD: 5.2), lower than reported in an international cohort (57% vs. 49%, p = 0.001), and higher than in a cohort of reproductive-aged Black women in the USA (49% vs. 38%, p = 0.001). Less than 50% of respondents correctly identified common infertility risk factors including sexually transmitted infections, advanced age, and obesity. Mean positive fertility perception score was 3 (IQR 3, 4), and negative fertility perception score was 3.5 (IQR 3, 4). Factors associated with agreement with negative fertility perception statements included: trying to conceive, refusing sickle cell disease treatment, and undergoing fertility treatment. Discussion: Opportunities exist to improve knowledge of infertility risk factors among adults with sickle cell disease. This study raises the possibility that nearly one in five adults with sickle cell disease refuse SCD treatment or cure due to infertility concerns. Education about common infertility risks factors needs to be addressed alongside disease- and treatment- associated fertility risks.

Risk factors associated with glycated hemoglobin A1c trajectories progressing to type 2 diabetes.

BACKGROUND AND OBJECTIVE: The notion of prediabetes, defined by the ADA as glycated hemoglobin A1c (HbA1c) of 5.7-6.4%, implies increased vascular inflammatory and immunologic processes and higher risk for developing diabetes mellitus and major cardiovascular events. We aimed to determine the risk factors associated with rapid progression of normal and prediabetes patients to type 2 diabetes mellitus (T2DM). METHODS: Retrospective cohort study in a single 8-hospital health system in southeast Michigan, between 2006 and 2020. All patients with HbA1c <6.5% at baseline and at least 2 other HbA1c measurements were clustered in five trajectories encompassing more than 95% of the study population. Multivariate linear regression analysis was performed to examine the association of demographic and comorbidities with HbA1c trajectories progressing to diabetes. RESULTS: A total of 5,347 prediabetic patients were clustered based on their HbA1c progression (C1: 4,853, C2: 253, C66: 102, C12: 85, C68: 54). The largest cluster (C1) had a baseline median HbA1c value of 6.0% and exhibited stable HbA1c levels in prediabetic range across all subsequent years. The smallest cluster (C68) had the lowest median baseline HbA1c value and also remained stable across subsequent years. The proportion of normal HbA1c in each of the pre-diabetic trajectories ranged from 0 to 12.7%, whereas 81.5% of the reference cluster (C68) were normal HbA1c at baseline. The C2 (steady rising) trajectory was significantly associated with BMI (adj OR 1.10, 95%CI 1.03-1.17), and family history of DM (adj OR 2.75, 95%CI 1.32-5.74). With respect to the late rising trajectories, baseline BMI was significantly associated with both C66 and C12 trajectory (adj OR 1.10, 95%CI 1.03-1.18) and (adj OR 1.13, 95%CI 1.05-1.23) respectively, whereas, the C12 trajectory was also significantly associated with age (adj OR 1.62, 95%CI 1.04-2.53) and history of MACE (adj OR 3.20, 95%CI 1.14-8.93). CONCLUSIONS: We suggest that perhaps a more aggressive preventative approach should be considered in patients with a family history of T2DM who have high BMI and year-to-year increase in HbA1c, whether they have normal hemoglobin A1c or they have prediabetes.KEY MESSAGESProgression to diabetes from normal or prediabetic hemoglobin A1c within four years is associated with baseline BMI.A steady rise in HbA1c during a four-year period is associated with age and family history of T2DM, whereas age and personal history of MACE are associated with a rapid rise in HbA1c.A more aggressive preventative approach should be considered in patients with a family history of T2DM who have high BMI and year-to-year increase in HbA1c.