Osteogenesis Imperfecta Blood Pressure Study

NCT04169568 | Completed

• 1 other identifier: 1465218-3
• Study Type: observational
• Target: 50
• Locations: 1 country
• Sites: 1

Brief Summary

Osteogenesis Imperfecta (OI) is a genetic disorder caused by mutations to the alpha-1 or alpha-2 chain of type I collagen. Clinically, the disorder is characterized by bones that fracture easily, often from little or no apparent trauma. There is no information about Some institutions perform blood pressure monitoring on these patients with a cuff, while other institutions avoid this method due to concern for fracture. Instead, they use alternative forms of monitoring such as an arterial line. These methods come with their own risks, including clotting, decreased perfusion, and pain. In addition, arterial lines require intensive (and more expensive) monitoring in a pediatric intensive care unit due to risk of dislodgement and rapid blood loss. These blood pressure monitoring recommendations for patients with OI do not appear to be based on strong evidence. Anecdotally, some patients and healthcare professionals report hearing about individuals with OI who have suffered fractures from blood pressure cuffs. However, this is not well documented in the medical literature. Regular and accurate blood pressure monitoring is particularly important in the postoperative period, due to blood loss and fluid shifts, as well as utilization of advanced pain management techniques that can potentially impact blood pressure.

Conditions

Osteogenesis Imperfecta

Outcome Measures

Primary Outcomes (1)

• Sphygmomanometer blood pressures

  Cuff blood pressure measurements obtained without injury to patient with Osteogenesis Imperfecta

  Every 4 hours for first 24 hours, then every 8 hours until discharge

Study Arms (1)

OI manual cuff BP

Patients with diagnosis of Osteogenesis Imperfecta from ages 1 to 35 who are admitted to our institution to the inpatient, non-ICU setting, following orthopedic surgery for spine surgery, upper or lower extremity realignment and IM rodding

Procedure: Manual cuff blood pressure

Interventions

Manual cuff blood pressure PROCEDURE

Blood pressure will be obtained using a sphygmomanometer. Only Registered Nurses will apply BP cuff after careful inspection of the upper extremity for bruising, swelling or tenderness.

OI manual cuff BP

Eligibility Criteria

• Age: 1 Year - 35 Years
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Child (0-17), Adult (18-64)
• Sampling Method: Probability Sample

Study Population

Patients between the ages of 1 and 35 with a diagnosis of Osteogenesis Imperfecta.

You may qualify if:

• Patients with OI who are undergoing extremity surgery or spinal fusion surgery.
• Patients between the ages of 1 and 35 years with OI who are admitted at our institution to a non-ICU inpatient setting in the postoperative period following an orthopedic surgery.
• Patients who initially require ICU-level care but transition to a non-ICU setting following an orthopedic surgery.
• Recent fracture in contralateral upper extremity (\< 8 weeks, or upper extremity fracture that is not yet completely healed).
• Must be able to tolerate upper extremity blood pressures.
• Medical clearance by OI physicians (Kruse, Franzone) after review of fracture history, surgical procedures, x-rays.

You may not qualify if:

• Parent or child refusal, uncooperative child.
• History of frequent upper extremity fracture on ipsilateral side, or recent upper extremity fracture on ipsilateral side if only that side may be used.
• Clinical presence of thrombocytopenia (excessive or easy bleeding or bruising), admission or transfer to PICU.

Sponsors & Collaborators

• Nemours Children's Clinic: lead

Study Sites (1)

Nemours / A I duPont Hospital for Children

Wilmington, Delaware, 19803, United States

Location

MeSH Terms

Conditions

Osteogenesis Imperfecta

Condition Hierarchy (Ancestors)

Osteochondrodysplasias; Bone Diseases, Developmental; Bone Diseases; Musculoskeletal Diseases; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Collagen Diseases; Connective Tissue Diseases; Skin and Connective Tissue Diseases

Study Officials

• Alicia McCarthy, MSN

  Nemours Children's Hospital

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: CASE ONLY
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Nurse Practitioner

Study Record Dates

• First Submitted: November 18, 2019
• First Posted: November 20, 2019
• Study Start: November 15, 2019
• Primary Completion: January 4, 2025
• Study Completion: January 4, 2025
• Last Updated: May 14, 2025

Record last verified: 2025-05

Locations

US (1)