Pulmonary Ultrasound in the Diagnosis of Acute Thoracic Syndrome in Vaso-occlusive Sickle Cell Crisis (ECHO-STA)

Evaluation of the Interest of Pulmonary Ultrasound in the Diagnosis and Early Detection of Acute Thoracic Syndrome in Hospitalized Children for Vaso-occlusive Sickle Cell Crisis: Multicenter Prospective Study

ATS (acute thoracic syndrome) refers to acute pulmonary involvement in a sickle cell patient. The diagnosis is based on the association of clinical signs (fever or respiratory symptoms) with a recent pulmonary infiltrate on the chest x-ray.

The main objective of the study is to evaluate the place of the pulmonary ultrasound for the diagnosis of ATS, in comparison with frontal chest x-ray.

Study Overview

• Status: Recruiting
• Conditions: Sickle-Cell Disease Nos With Crisis
• Intervention / Treatment: Diagnostic test: pulmonary ultrasound

Detailed Description

The ATS occurs in half of the cases during hospitalization for a VOC (Vaso Occlusive crisis). The morbidity of ATS is important (respiratory insufficiency, duration of hospitalization, stays in intensive care, brain complications, pain, hypoxia, long-term sequelae, etc.) but no study has shown the benefits of curative treatments such as transfusion ( recommended at the time of diagnosis and practiced by some teams) or noninvasive ventilation (practiced by other teams). The first step before studying curative treatments is to have tools for early detection of ATS. Recent studies show non-inferiority of pulmonary ultrasonography compared to radiography and thoracic computed tomography in the diagnosis of pulmonary condensation (in adults and children). In a study performed in sickle cell adults admitted for ATS, pulmonary ultrasound was more sensitive than X-ray in detecting images of pulmonary consolidations visible on the chest CT scan.

Daswani et al have also shown the value of pulmonary ultrasound in comparison with radiography in the detection of consolidation lesions, suggestive of STA, in febrile children or young adults with sickle cell disease. They showed a good sensitivity (87%) and specificity (94%) of the pulmonary ultrasound.

• Study Type: Interventional
• Enrollment (Anticipated): 150
• Phase: Not Applicable

Contacts and Locations

• Study Contact: Name: Camille JUNG, MD, PhD; Phone Number: +3357022268; Email: camille.jung@chicreteil.fr
• Study Contact Backup: Name: Fouad MADHI, MD; Email: fouad.madhi@chicreteil.fr

Study Locations

• France
  • Clamart, France, 92141
    • Not yet recruiting
    • Hôpital Antoine Béclère
    • Contact: Mélanie TAVEIRA, MD; Email: melanie.taveira@aphp.fr
  • Créteil, France, 94000
    • Recruiting
    • Centre Hospitalier Intercommunal Creteil
    • Contact: Fouad MADHI, MD; Email: fouad.madhi@chicreteil.fr
  • Le Kremlin-Bicêtre, France, 94270
    • Not yet recruiting
    • Hôpital Bicêtre
    • Contact: Irina CRAIU, MD; Email: irina.craiu@aphp.fr
  • Paris, France, 75012
    • Not yet recruiting
    • Hôpital Armand Trousseau
    • Contact: Guillaume Thouvenin, MD
    • Contact: Céline Delestrain, MD; Email: celine.delestrain@aphp.fr

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 1 year to 17 years (CHILD)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

Description

Inclusion Criteria:

• Age: ≥12 months and <18 years
• Has a major sickle cell disease whatever the genotype
• Admission to hospital for a febrile vaso-occlusive crisis (VOC) or not
• Evolving at home for less than 48h
• Concomitant treatment with hydroxycarbamide / hydroxyurea possible
• Signed consent
• Patients affiliated to a French social security scheme

Exclusion Criteria:

• Child presenting an acute thoracic syndrome (ATS) from the outset at the admission
• Child who presented an ATS in the month preceding the inclusion
• Child in regular transfusion program or child who has received a hematopoietic stem cell transplant
• Child hospitalized at least 5 times for VOC in the year preceding inclusion (psychic problems making difficult the evaluation of the pain)
• Child who has already been included in the study during a previous VOC (each child participates in the study only once)
• VOC evolving for more than 48 hours before admission to the emergency room
• Acute splenic sequestration crisis at admission

Study Plan

How is the study designed?

Design Details

• Primary Purpose: DIAGNOSTIC
• Allocation: NA
• Interventional Model: SINGLE_GROUP
• Masking: NONE

Number of Arms

1

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
OTHER: Children with sickle cell disease; Child from 12 months to 18 years old admitted for vaso-occlusive crisis	Diagnostic test: pulmonary ultrasound; The subjects will have an ultrasound on admission and on day 1, 2 and 3

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Sensitivity of pulmonary ultrasound	Proportion of subjects with positive pulmonary ultrasound among those with a positive chest X-ray at time t with 95% confidence interval at day 1	Day 1
Sensitivity of pulmonary ultrasound	Proportion of subjects with positive pulmonary ultrasound among those with a positive chest X-ray at time t with 95% confidence interval at day 2	Day 2
Sensitivity of pulmonary ultrasound	Proportion of subjects with positive pulmonary ultrasound among those with a positive chest X-ray at time t with 95% confidence interval at day 3	Day 3

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Specificity of pulmonary ultrasound	Proportion of subjects with a positive pulmonary ultrasound among those with a negative chest X-ray at time t at day 1	Day 1
Specificity of pulmonary ultrasound	Proportion of subjects with a positive pulmonary ultrasound among those with a negative chest X-ray at time t at day 2	Day 2
Specificity of pulmonary ultrasound	Proportion of subjects with a positive pulmonary ultrasound among those with a negative chest X-ray at time t at day 3	Day 3
Sensitivity of pulmonary ultrasound in relation with ATS diagnosis	Proportion of subjects with positive pulmonary ultrasound among those with a positive chest X-ray at time t with 95% confidence intervalat day -3 of ATS diagnosis	day -3 of ATS diagnosis
Sensitivity of pulmonary ultrasound in relation with ATS diagnosis	Proportion of subjects with positive pulmonary ultrasound among those with a positive chest X-ray at time t with 95% confidence interval at day -1 of ATS diagnosis	day -1 of ATS diagnosis
Sensitivity of pulmonary ultrasound in relation with ATS diagnosis	Proportion of subjects with positive pulmonary ultrasound among those with a positive chest X-ray at time t with 95% confidence interval at day -2 of ATS diagnosis	day -2 of ATS diagnosis
Specificity of pulmonary ultrasound in relation with ATS diagnosis	Proportion of subjects with a positive pulmonary ultrasound among those with a negative chest X-ray at time t at day -1 of ATS diagnosis	day -1 of ATS diagnosis
Specificity of pulmonary ultrasound in relation with ATS diagnosis	Proportion of subjects with a positive pulmonary ultrasound among those with a negative chest X-ray at time t at day -3 of ATS diagnosis	-3 day of ATS diagnosis
Specificity of pulmonary ultrasound in relation with ATS diagnosis	Proportion of subjects with a positive pulmonary ultrasound among those with a negative chest X-ray at time t at day -2 of ATS diagnosis	day -2 of ATS diagnosis

Collaborators and Investigators

• Sponsor: Centre Hospitalier Intercommunal Creteil

Study record dates

Study Major Dates

• Study Start (ACTUAL): April 4, 2019
• Primary Completion (ANTICIPATED): April 30, 2023
• Study Completion (ANTICIPATED): July 1, 2023

Study Registration Dates

• First Submitted: May 23, 2019
• First Submitted That Met QC Criteria: May 31, 2019
• First Posted (ACTUAL): June 3, 2019

Study Record Updates

• Last Update Posted (ACTUAL): February 8, 2023
• Last Update Submitted That Met QC Criteria: February 7, 2023
• Last Verified: February 1, 2023

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Hematologic Diseases; Genetic Diseases, Inborn; Anemia; Anemia, Hemolytic, Congenital; Anemia, Hemolytic; Hemoglobinopathies; Anemia, Sickle Cell
• Other Study ID Numbers: ECHO-STA

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No