Sickle Cell Disease
Conditions
Keywords
Sickle cell disease, Pulmonary anomalies, Vaso-occlusive crisis, Acute chest syndrom, Pleuropulmonary ultrasound
Brief summary
Describe the proportion of patients with major sickle cell syndrome in vaso-occlusive crisis presenting at least one pleuropulmonary ultrasound abnormality during one of the ultrasounds performed at D0, between D2 and D5 during hospitalization and at D-1 of discharge. We hypothesize that pleuropulmonary ultrasound will make it possible to detect the serious complications associated with vaso-occlusive crises in patients with major sickle cell syndrome earlier and more reliably, in departments where ultrasound tools are still underdeveloped, while avoiding the need for more conventional radiology examinations that cause radiation in multi-hospitalized patients.
Interventions
pleuropulmonary ultrasounds performed at D0, between D2 and D5 during hospitalization and at D-1 on discharge
Sponsors
Study design
Eligibility
Inclusion criteria
* Patient agreeing to participate in the study * Patient with sickle-cell disease consulted to the emergency department or hospitalized in a conventional internal medicine department for a clinical picture of severe vaso-occlusive crisis (CVO) requiring hospitalization. * Hospitalization in the internal medicine department * Possible re-inclusion in the event of a subsequent episode of severe CVO
Exclusion criteria
* Subject under guardianship or subject deprived of freedom. * Primary acute chest syndrom (not following a crisis) * Pulmonary pathologies interfering with pleuro-pulmonary echo analysis: pneumonectomy, pulmonary fibrosis.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Description of the proportion of patients with sickle cell disease in vasoocclusive crisis presenting at least one pleuropulmonary ultrasound abnormality at D0, between D2 and D5 during hospitalization and at D-1 of discharge. | 30 days | Presence of abnormalities found during pleuropulmonary ultrasound scans performed in the medical department on D0, between D2 and D5 during hospitalization and on D-1 of discharge, in patients with major sickle cell syndrome presenting with vasoocclusive crisis, among the following: * Pulmonary condensations; * Pleural effusion: minimal, of moderate abundance (estimated volume \< 500mL) or of great abundance (estimated volume \> 500mL); * Confluent B lines (≥ 3 B lines per lung field). |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Evaluate the association between the ultrasound image detected and the occurrence of acute chest syndrom. | 30 days | Number and characteristics of abnormalities detected by EPP between D0, D2-J5 during hospitalization and at D-1 of discharge, in patients progressing towards an acute chest syndrom, compared with the number and characteristics of abnormalities detected by EPP between D1 and D5 during hospitalization and at D-1 of discharge in patients not progressing towards an acute chest syndrom. |
| Evaluate the association between the ultrasound image detected and the severity of the acute chest syndrom | 30 days | Numbers and characteristics of abnormalities detected by EPP between D1 and D5 during hospitalization and on D-1 of discharge, in relation to the severity of acute chest syndrom (oxygen demand, RF, transfer to ICU or intensive care unit). |
| Evaluate the association between the ultrasound image and the clinical picture | 30 days | Numbers and characteristics of abnormalities detected by EPP between D0, D2-J5 during hospitalization and D-1 of discharge, compared with numbers and characteristics of clinical abnormalities found between D0, D2-J5 during hospitalization and D-1 of discharge. |
| Describe the evolution of abnormalities between visits at D0, between D2 and D5 (V1 and V2) during hospitalization and at D-1 of discharge, and their association with clinical evolution | 30 days | Difference in proportion and characteristics of abnormalities detected by pleuropulmonary ultrasound on D0, between D2 and D5 (V1 and V2) during hospitalization and on D-1 of discharge. |
| Assess the prognostic value of discharge pleuropulmonary ultrasound in relation to re-hospitalization, occurrence of acute chest syndrom or early mortality (within 30 days) | 30 days | Presence of at least one abnormality on day of discharge and number of re-hospitalizations, acute chest syndrom and 30-day mortality. |