Validation of a Predictive Score of Acute Chest Syndrome
Validation of a Predictive Score of Acute Chest Syndrome
Vaso-Occlusive Crisis (VOC), the most common manifestation of sickle cell disease (SCD), is
 the first cause of death, particularly when complicated by an acute chest syndrome (ACS).

 The PRESEV score could help the physicians to better manage VOC and could be used for future
 therapeutic trials. This predictive score of secondary ACS has to be validated in a
 multicenter international study.
International multicentre prospective observational study.

 Prediction of an ACS within 15 days after admission by the PRESEV2 score at arrival.

 Validation of a Predictive Score of Acute Chest Syndrome (Presev2) associates a categorical
 pain score of the spine /or pelvis and 3 biological parameters: Reticulocytes, Leucocytes and
 Hemoglobin.

 ACS is defined by crepitant or bronchial breathing or the association of new radiologic
 infiltrate and chest pain

 Inclusion criteria:

 -  Children (>2 years) and adults

 -  Male and Female

 -  Homozygous SCD patients

 -  The patient can only be included once

 -  VOC admitted at the emergency unit (a severe VOC is defined as pain or tenderness
 affecting at least one part of the body (e.g. limbs, ribs, sternum, head (skull), spine
 and/or pelvis) that required hospitalization and opioids (level 3), and is not
 attributable to other causes.

 -  Patient has health care insurance (in Europe)

 -  Written consent given after being informed of the purpose, progress and potential risks

 Exclusion criteria:

 -  No inaugural Acute Chest Syndrome

 -  Homeless patients

 -  Deprived of their liberty by a court or administrative order or under guardianship

 -  Unable to understand the purpose and conditions of carrying out the study, unable to
 give consent

 STUDY SCHEDULE Screening and inclusion once patients are admitted at the accident and
 emergency department or medical day unit.

 Inclusion visit (day 1) Once admitted at the accident and emergency department, the patient
 will be informed about the protocol and asked to participate in the study. Informed consent
 will be obtained according to local regulations by signing the informed consent form. The
 inclusion and non-inclusion criteria will be verified.

 Demographic data, current and previous treatments taken within one month, and medical history
 will be recorded.

 The following parameters of the score will be collected:

 Reticulocytes and/or leucocytes counts, urea (mmol/L) and Categorical pain score.

 The Score is adjusted with Hydroxyurea treatment (Yes/No) and Hb level (g/dL). Plasmodium
 falciparum test will be performed only in Africa. Hemoculture if fever (>38°C) is recorded
 within the 2 first days after admission.

 Temperature, Blood pressure, Oxygen saturation, Respiratory rate, Visual analogue score will
 be recorded.

 STUDY DURATION Inclusion period: 2 years Per patient: 15 days Total duration of the study: 2
 years
Inclusion Criteria:

 -  Inclusion criteria:

 -  Children (>2 years) and adults

 -  Male and Female

 -  Homozygous SCD patients

 -  VOC admitted at the emergency unit (a severe VOC is defined as pain or tenderness
 affecting at least one part of the body (e.g. limbs, ribs, sternum, head (skull),
 spine and/or pelvis) that required hospitalization and opioids (level 3), and is not
 attributable to other causes.

 -  Patient has health care insurance (in Europe)

 -  Written consent given after being informed of the purpose, progress and potential
 risks

 Exclusion criteria:

 -  No inaugural Acute Chest Syndrome

 -  Homeless patients

 -  Deprived of their liberty by a court or administrative order or under guardianship

 -  Unable to understand the purpose and conditions of carrying out the study, unable to
 give consent