Pleuropulmonary Ultrasound Imaging in Patients With Sickle Cell Disease in a Vaso-occlusive Crisis. "ECHODREP"

NCT07782489 | Not Yet Recruiting

• 1 other identifier: APHP 250934
• Study Type: observational
• Target: 200
• Locations: 0 countries
• Sites: N/A

Brief Summary

Introduction Sickle cell disease is the most common inherited genetic disorder in France and worldwide. Its main acute manifestation is vaso-occlusive crisis, a frequent reason for emergency department visits. Acute chest syndrome is the leading complication and cause of death during vaso-occlusive crisis, occurring in approximately 15-20% of hospitalized episodes, often within the first few days after symptom onset. Early identification of patients at risk of developing acute chest syndrome remains a major clinical challenge. Although predictive clinical and biological scores exist, their ability to identify high-risk patients remains limited in daily practice. Chest X-ray has traditionally been used to support the diagnosis of acute chest syndrome, but its diagnostic performance and repeated radiation exposure are increasingly questioned. Lung ultrasound is a non-invasive bedside imaging tool that has shown good performance for detecting pleural effusion, alveolar consolidation and interstitial syndrome, and is increasingly used by emergency physicians. However, its diagnostic performance when systematically performed by emergency physicians in adult sickle cell patients presenting with vaso-occlusive crisis has not yet been specifically assessed. Hypothesis / Objective We hypothesize that systematic pleuropulmonary ultrasound performed early after emergency department admission may help identify patients with vaso-occlusive crisis who are at risk of developing acute chest syndrome, and may allow earlier diagnostic assessment. The primary objective of the ECHODREP study is to evaluate the diagnostic performance of systematic pleuropulmonary ultrasound, performed by an emergency physician within six hours of admission, for the diagnosis of acute chest syndrome during hospitalization in adult sickle cell patients presenting with vaso-occlusive crisis. Secondary objectives are to assess the association between ultrasound findings and the final diagnosis of acute chest syndrome, to describe patient outcomes according to ultrasound results, including hospitalization, admission to intermediate care, emergency department revisits and mortality at day 3, day 7 and day 28, and to compare ultrasound performance according to the operator's level of experience. Method ECHODREP is a prospective, observational, single-arm, monocentric diagnostic performance study conducted in the Emergency Department of Henri-Mondor University Hospital. The study will include 200 adult patients with sickle cell disease - SS, SC or S-beta-thalassemia - presenting to the emergency department for vaso-occlusive crisis. Patients with a diagnosis of acute chest syndrome already established on arrival, patients previously included in the study, protected adults, and patients refusing participation will not be included. After informed consent, a pleuropulmonary ultrasound will be performed within six hours of emergency department admission by an emergency physician investigator who is not involved in the patient's clinical care. The examination will assess twelve predefined lung zones. In order to avoid influencing standard care and to preserve the objectivity of the study, ultrasound findings obtained for research purposes will not be communicated to the treating medical team and will not be used for diagnostic or therapeutic decisions. Patients will receive standard care for vaso-occlusive crisis according to local practice. A telephone follow-up will be performed at day 28 to collect vital status, emergency department revisits, hospitalizations and any secondary diagnosis of acute chest syndrome. At the end of follow-up, an independent adjudication committee, blinded to the research ultrasound findings, will determine the final diagnosis: uncomplicated vaso-occlusive crisis or acute chest syndrome. The primary endpoint will be the diagnosis of acute chest syndrome within seven days after emergency department admission or before hospital discharge, based on the medical record. Diagnostic performance will be assessed by calculating sensitivity, specificity, positive and negative predictive values, likelihood ratios and area under the ROC curve, with 95% confidence intervals. Additional analyses will compare outcomes according to ultrasound findings and operator experience. Conclusion This exploratory study will provide original data on the diagnostic performance of early pleuropulmonary ultrasound performed by emergency physicians in adult sickle cell patients presenting with vaso-occlusive crisis. If ultrasound demonstrates adequate diagnostic accuracy, it could contribute to earlier identification of acute chest syndrome, improve risk stratification in the emergency department and help optimize the management pathway of these patients. By evaluating both diagnostic performance and patient outcomes, ECHODREP may support the integration of lung ultrasound into future diagnostic strategies for acute chest syndrome in sickle cell disease.

Conditions

Sickle Cell Disease (SCD); Vaso-Occlusive Crises; Acute Chest Syndrome

Keywords

Lung Ultrasonography; Point-of-Care Ultrasound; Sickle Cell Disease; Vaso-Occlusive Crises; Acute Chest Syndrome,; Pleuropulmonary Ultrasound; Emergency Medicine; Emergency Department; Diagnostic Accuracy; Prospective Studies

Outcome Measures

Primary Outcomes (1)

• Diagnosis of acute chest syndrome

  Formulation of the diagnosis of acute chest syndrome within 7 days after the emergency department visit or, where applicable, until hospital discharge, based on the patient's medical record. The outcome will be considered present if the medical record explicitly mentions at least one of the following: Diagnosis of acute chest syndrome; or occurrence of a new pulmonary auscultation abnormality, such as crackles or tubular breath sounds; or occurrence of a new radiological infiltrate, either alveolar or interstitial, associated with chest pain and/or decreased breath sounds.

  Within 7 days after the emergency department visit, or until hospital discharge, as applicable.

Secondary Outcomes (7)

• Time to diagnosis of acute chest syndrome

  Within 7 days after the emergency department visit, or until hospital discharge, as applicable.

• Intermediate care unit stay

  From emergency department visit to hospital discharge.

• Thoracic imaging abnormalities

  After inclusion and pleuropulmonary ultrasound corresponded to D0 (Day 0), patients will be followed until Day 7 (D7) during hospitalization or, if discharged earlier, will be contacted between Days 3 (D3) and Days 7 (D7) after discharge of hospital

• Hospitalization outcomes

  From admission to the emergency department corresponded to Day 1 (D1) until hospital discharge or Day 7 (D7), and until Day 28 (D28) after inclusion that corresponded to follow visit.

• All-cause mortality

  At Day 3, Day 7, and Day 28 after the emergency department visit.

Interventions

Pleuropulmonary ultrasound OTHER

A systematic bedside pleuropulmonary ultrasound will be performed within 6 hours of emergency department admission in adult patients with sickle cell disease presenting with vaso-occlusive crisis. The examination will be performed by an emergency physician investigator who is not involved in the patient's clinical care and will assess 12 predefined lung zones for pleural effusion, pulmonary consolidation and/or interstitial abnormalities. Ultrasound findings obtained as part of the study will not be communicated to the treating medical team and will not be used for diagnostic or therapeutic decisions. The examination is non-invasive, performed according to usual clinical ultrasound practice, and is expected to take less than 10 minutes.

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Adult patients with sickle cell disease presenting to the Emergency Department of Henri-Mondor University Hospital for a vaso-occlusive crisis. Eligible patients will be aged 18 years or older and have sickle cell disease of genotype SS, SC, or S-beta-thalassemia. Patients must be affiliated with a social security system and must have received study information and provided written informed consent before participation.

You may qualify if:

• Age ≥ 18 years
• Sickle cell disease: SS, SC, or S-beta-thalassemia
• Presentation to the Emergency Department for vaso-occlusive crisis
• Patient affiliated with a social security system, either as beneficiary or entitled person
• Patient informed about the study and having agreed to sign a free and informed consent form to participate in the study

You may not qualify if:

• Diagnosis of acute chest syndrome already established upon arrival at the Emergency Department
• Pleuropulmonary ultrasound showing no abnormality but not having explored all twelve areas of interest \[Appendix 1\]
• Patient already included in the study during a previous Emergency Department visit
• Patient under guardianship or curatorship
• Refusal to participate in the research

Sponsors & Collaborators

• Assistance Publique - Hôpitaux de Paris: lead

Related Publications (1)

• 1. Avis n°2022.0060/AC/SESPEV du 10 novembre 2022 du collège de la Haute Autorité de santé relatif à la généralisation du dépistage de la drépanocytose en France métropolitaine [Internet]. Haute Autorité de Santé. [cited 2023 Oct 9];Available from: https://www.has-sante.fr/jcms/p_3385427/fr/avis-n2022-0060/ac/sespev-du-10-novembre-2022-du-college-de-la-haute-autorite-de-sante-relatif-a-la-generalisation-du-depistage-de-la-drepanocytose-en-france-metropolitaine 2. Platt OS, Brambilla DJ, Rosse WF, et al. Mortality in sickle cell disease. Life expectancy and risk factors for early death. N Engl J Med 1994;330(23):1639-44. 3. Vichinsky EP, Neumayr LD, Earles AN, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med 2000;342(25):1855-65. 4. Bartolucci P, Habibi A, Khellaf M, et al. Score Predicting Acute Chest Syndrome During Vaso-occlusive Crises in Adult Sickle-cell Disease Patients. EBioMedicine 2016;10:305-11. 5. Kassasseya C, Sekou K, Besse-Hammer T, et al. Validation of a Predictive Score of Acute Chest Syndrome (PRESEV-2 study) in Adults. Blood 2020;136:23-23. 6. Pelinski Y, Mescam C, Kassasseya C, et al. Drepadom - Establishment of Home Care Services and Hospitalizations for Sickle Cell Disease Patients As Standard Care Since the Covid-19 Pandemic. Blood 2021;138(Supplement 1):916. 7. Maitre B, Habibi A, Roudot-Thoraval F, et al. Acute Chest Syndrome in Adults With Sickle Cell Disease. CHEST 2000;117(5):1386-92. 8. Cheminet G, Mekontso-Dessap A, Pouchot J, Arlet J-B. [Acute chest syndrome in adult sickle cell patients]. Rev Med Interne 2022;43(8):470-8. 9. Pelinski Y, Guindo A, Kassasseya C, et al. Is chest X-ray still relevant for acute chest syndrome diagnosis? Health Sci Rep 2024;7(5):e2053. 10. Lichtenstein D, Goldstein I, Mourgeon E, Cluzel P, Grenier P, Rouby J-J. Comparative diagnostic performances of auscultation, chest radiography, and lung ultrasonography

  RESULT

MeSH Terms

Conditions

Anemia, Sickle Cell; Vaso-Occlusive Crises; Acute Chest Syndrome; Emergencies

Condition Hierarchy (Ancestors)

Anemia, Hemolytic, Congenital; Anemia, Hemolytic; Anemia; Hematologic Diseases; Hemic and Lymphatic Diseases; Hemoglobinopathies; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Lung Diseases; Respiratory Tract Diseases; Respiration Disorders; Disease Attributes; Pathologic Processes; Pathological Conditions, Signs and Symptoms

Central Study Contacts

Christian Kassasseya, Doctor

CONTACT

(+33)1.49.81.21.11; christian.kassasseya@aphp.fr

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: May 20, 2026
• First Posted: August 24, 2026
• Study Start (Estimated): September 1, 2026
• Primary Completion (Estimated): November 1, 2028
• Study Completion (Estimated): November 1, 2028
• Last Updated: August 24, 2026

Record last verified: 2026-06

Data Sharing

• IPD Sharing: Will not share