| Affiliation: | 1. https://orcid.org/0000-0002-1242-5626;2. Greenberg Center for Skeletal Dysplasias, McKusick‐Nathans Department of Genetics, Johns Hopkins University, Baltimore, Maryland;3. Julie Hoover‐Fong, Greenberg Center for Skeletal Dysplasias, McKusick‐Nathans Department of Genetics, Johns Hopkins University, 733 N. Broadway Room 579, Baltimore, MD 21205.;4. Greenberg Center for Skeletal Dysplasias, Johns Hopkins University, Baltimore, Maryland;5. Department of Orthopedics, Johns Hopkins University, Baltimore, Maryland;6. Department of Anesthesiology and Critical Care Medicine, Johns Hopkins University, Baltimore, Maryland;7. Division of Genetics, Alfred I duPont Hospital for Children, Wilmington, Delaware;8. Center for Skeletal Dysplasias, Hospital for Special Surgeries, New York, New York;9. Department of Pediatrics, University of Texas Medical School at Houston, Houston, Texas;10. Department of Orthopedic Surgery, University of Missouri‐Columbia, Columbia, Missouri;11. Department of Orthopaedic Surgery, Ronald Reagan UCLA Medical Center, Los Angeles, California;12. McKusick‐Nathans Institute of Genetic Medicine, Johns Hopkins University, Baltimore, Maryland;13. Department of Orthopedic Surgery, Alfred I duPont Hospital for Children, Wilmington, Delaware;14. Clinical and Metabolic Genetics, The Hospital for Sick Children, Toronto, Ontario, Canada;15. Regional Skeletal Dysplasia Program, Kaiser Permanente Genetics, Oakland, California;16. Bloomberg School of Public Health, Johns Hopkins University, Baltimore, Maryland |
| Abstract: | Hypertension, compounded by obesity, contributes to cardiovascular disease and mortality. Data describing hypertension prevalence in adults with short stature skeletal dysplasias are lacking, perhaps due to poor fit of typical adult blood pressure cuffs on rhizomelic or contracted upper extremities. Through health screening research, blood pressure was measured in short stature adults attending support group meetings and skeletal dysplasia clinics. Blood pressure was measured with a commercially available, narrower adult cuff on the upper and/or lower segment of the arm. Height, weight, age, gender, diagnosis, exercise, and medications were collected. Subjects were classified as normotensive, prehypertensive, or hypertensive for group analysis; no individual clinical diagnoses were made. In 403 short stature adults, 42% were hypertensive (systolic >140, diastolic >90 OR taking antihypertensive medications). For every BMI unit and 1 kg weight increase in males, there was a 9% and an 8% increase, respectively, in the odds of hypertension versus normotension. In females, the increase was 10% and 6%, respectively. In those with achondroplasia, the most common short stature dysplasia, males (n = 106) had 10% greater odds of hypertension versus normotension for every BMI unit and kilogram increase. In females with achondroplasia (n = 128), the odds of hypertension versus normotension was 8% greater for each BMI unit and 7% for each additional kilogram. These data suggest a high population prevalence of hypertension among short stature adults. Blood pressure must be monitored as part of routine medical care, and measuring at the forearm may be the only viable clinical option in rhizomelic short stature adults with elbow contractures. |
