Objective: Given that updated estimates of Ehlers-Danlos syndrome and risks for obstetric complications including postpartum readmission may be of public health significance, we sought to analyze associated obstetric trends and outcomes in a nationally representative population.
Study Design: The 2016 to 2020 Nationwide Readmissions Database was used for this retrospective cohort study. Delivery hospitalizations to women aged 15 to 54 with and without Ehlers-Danlos syndrome were identified. Temporal trends in Ehlers-Danlos syndrome diagnoses during delivery hospitalizations were analyzed using joinpoint regression to estimate the average annual percent change with 95% confidence intervals (CIs). To determine whether adverse obstetric outcomes during the delivery were associated with Ehlers-Danlos syndrome, unadjusted and adjusted logistic regression models were fit with unadjusted (odds ratio [OR]) and adjusted ORs with 95% CIs as measures of association. In addition to analyzing adverse delivery outcomes, risk for 60-day postpartum readmission was analyzed.
Results: An estimated 18,214,542 delivery hospitalizations were included of which 7,378 (4.1 per 10,000) had an associated diagnosis of Ehlers-Danlos syndrome. Ehlers-Danlos syndrome diagnosis increased from 2.7 to 5.2 per 10,000 delivery hospitalization from 2016 to 2020 (average annual percent change increase of 16.1%, 95% CI: 9.4%, 23.1%). Ehlers-Danlos syndrome was associated with increased odds of nontransfusion severe maternal morbidity (OR: 1.84, 95% CI: 1.38, 2.45), cervical insufficiency (OR: 2.14, 95% CI: 1.46, 3.13), postpartum hemorrhage (OR: 1.41, 95% CI: 1.17, 1.68), cesarean delivery (OR: 1.26, 95% CI: 1.17, 1.36), and preterm delivery (OR: 1.35, 95% CI: 1.16, 1.56). Estimates for transfusion, placental abruption, and placenta previa did not differ significantly. Risk for 60-day postpartum readmission was 3.0% among deliveries with Ehlers-Danlos (OR: 1.76, 95% CI: 1.37, 2.25).
Conclusion: Ehlers-Danlos syndrome diagnoses approximately doubled over the 5-year study period and was associated with a range of adverse obstetric outcomes and complications during delivery hospitalizations as well as risk for postpartum readmission.
Key Points: · Ehlers-Danlos syndrome diagnoses approximately doubled over the 5-year study period.. · Ehlers-Danlos was associated with a range of adverse obstetric outcomes.. · Ehlers-Danlos was associated with increased readmission risk..
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http://dx.doi.org/10.1055/a-2185-4149 | DOI Listing |
Orphanet J Rare Dis
December 2024
Center for Biobehavioral Health, Abigail Wexner Research Institute at Nationwide Children's Hospital, Columbus, OH, USA.
Introduction: After diagnosis of Ehlers Danlos Syndrome (EDS), it is unclear what information patients and parents need and understand about EDS. The objective of this study is to characterize patient and parent knowledge and concerns about EDS after a diagnosis of EDS is made to determine patient and parent concerns and identify barriers that cause discomfort with the diagnosis.6 METHODS: A convenience sample of patient and parent dyads were recruited after new diagnosis of EDS.
View Article and Find Full Text PDFEur J Hum Genet
December 2024
National EDS Service, London North West University Healthcare NHS Trust, London, UK.
Vascular Ehlers-Danlos syndrome (vEDS) is a rare inherited connective tissue disorder predominantly caused by pathogenic COL3A1 variants. Characteristic arterial and intestinal fragility and generalised severe tissue friability can lead to clinical events from childhood. We highlight a paucity of literature regarding children diagnosed with vEDS, possibly explained by a restraint in predictive testing, and present data on 63 individuals (23 index cases) with a clinical and genetic diagnosis of vEDS in childhood (<18 years) to address this.
View Article and Find Full Text PDFMed Clin (Barc)
December 2024
Unidad de Cuidados Intensivos Cardiovasculares, Servicio de Medicina Intensiva, Hospital Universitario Virgen de las Nieves, Granada, España.
Front Neurol
December 2024
Neuromuscular Imaging Research Lab, The Kolling Institute, North Sydney Local Health District, St Leonards, NSW, Australia.
Individuals with joint hypermobility and the Ehlers-Danlos Syndromes (EDS) are disproportionately affected by neuraxial dysfunction and Central Nervous System (CNS) disorders: such as Spontaneous Intracranial Hypotension (SIH) due to spinal cerebrospinal fluid (CSF) leaks, Upper Cervical Instability (UCI; including craniocervical or atlantoaxial instability (CCI/AAI)), Occult Tethered Cord Syndrome (TCS), Chiari Malformation (CM) and Idiopathic Intracranial Hypertension (IIH). The neuraxis comprises the parts of the nervous system (brain, nerves, spinal cord) along the craniospinal axis of the body. Neuraxial tissue includes all tissue structures that comprise, support, sheath, and connect along the neuraxis and peripheral nerves.
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