Prognostic Factors of Acute Splenic Sequestration

Not Applicable

Completed

• Conditions: Anemia; Drepanocytic
• Interventions: Procedure: blood samples and scintigraphy

• Registration Number: NCT01207037
• Lead Sponsor: Assistance Publique - Hôpitaux de Paris

Brief Summary

Acute splenic sequestration is a frequent and life threatening complication occurring in approximately 10 % of homozygous children. Maximal incidence is between 6 and 18 months.

The investigators formulate the hypothesis that there are clinical, biological and genetic markers predictive of severe complications notably acute splenic sequestration in SCD children. The present research project thus aims at analyzing in a forward-looking way the profile of severity by analysing clinical, biological and genetic characteristics in a multicentric cohort of 60 SCD children

Detailed Description

A prospective multicentric analysis will be conducted in a cohort of 150 SS or S ß ° children diagnosed at birth, included at 3 -5 months and followed up to the age of 24 months.

Five visits, superimposed to the usual follow-up of SCD children, (Recommendations of the High Authority of Health) at 3 months, 6 months, 12 months, 18 months and 24 months will allow a clinical evaluation and an additional sampling of blood (5 mL) at each visit.

The samples will allow 1.analysis of the red blood cell phenotype (adhesion and deformability) and densities 2. the genetic profile 3.to establish a cell bank, a sera bank and a DNA bank, Spleen function in the cohort will be estimated by spleen scintigraphy, coupled with blood markers (pitted cells, Howell-Jolly bodies counts)

Study Timeline

• Study First Submitted: 2010-06-03
• Study Start: 2010-08-12
• Study First Submitted QC: 2010-09-21
• Study First Posted: 2010-09-22
• Primary Completion: 2014-12-22
• Study Completion: 2017-11-07
• Status Verified: 2019-10
• Last Update Submitted: 2019-10-18
• Last Update Posted: 2019-10-21

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 58

Inclusion Criteria

Not provided

Exclusion Criteria

Not provided

Study & Design

• Study Type: INTERVENTIONAL
• Study Design: SINGLE_GROUP

Arm && Interventions

Group	Intervention	Description
Intervention	blood samples and scintigraphy	-

Primary Outcome Measures

Name	Time	Method
Study of prognostic factors of acute splenic sequestration	after three years	* Complete blood count, reticulocyte count, haemoglobin level, VGM, TGMH, CCMH, hematocrit, % foetal haemoglobin (HbF), red blood cell densities (2 mL); * Deformability of red blood cells; * Expression study of the cell surface molecule: Phosphatidyl serine, CD 36, READ B-CAM, CD 47, ICAM 4, VLA 4. (Cellulotheque = red blood cells frozen in cryopreservative conditions); * Total LDH, Bilirubine (stigmas of hemolysis); * Genetic Profil ( DNAtheque)

Secondary Outcome Measures

Name	Time	Method
Study of hematology parameters	after three years	* Percentage of Howell Jolly's bodies (0,1 mL); * Percentage of pitted cells (0,5 mL fixed in 4 %)formaldehyde; * Splenic volume, semi-quantitative measure in scintigraphy; * Antibody titers in answer to the antipneumococcic vaccination ( serotheque)

Trial Locations

Locations (1)

Necker Hospital; 🇫🇷 Paris, France