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GRC 25 : DREPS

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GRC 25 DREPS – Sickle cell disease : Paris Research Group – Sorbonne University

Sickle cell disease is a genetic disorder of hemoglobin leading to anemia by destruction of red blood cells and to microcirculatory dysfunction. It is very common in sub-Saharan Africa, the Maghreb and India, but is also the most common genetic disorder among newborns in France. It causes acute complications (acute painful episode, acute chest syndrome, infections, vascular events) and progressive target organ damage (kidney, heart, brain…).

The sickle cell center of the Tenon Hospital coordinates the multidisciplinary care of over 1000 adult patients. Research within the center adresses two main areas:

  1. History of chronic complications over the lifetime.
  2. Analysis of the clinincal care pathways, with a focus on emergency visits and hospitalizations.
Team History and Prevention of Chronic Complications

Team History and Prevention of Chronic Complications

Director : François Lionnet

Life expectancy of sickle cell patients has dramatically increased thanks to the successful management of severe acute complications. Sickle cell disease is now a chronic disease, and progressive kidney and heart dysfunction has become the prime factor determining life expectancy. Systematic patient follow-up data allows to better understand the natural history of these complications and the factors that determine it. Subgroups at risk may require more frequent monitoring and a stronger focus on preventive interventions.

Team study and optimization of urgent care recourse

Team study and optimization of urgent care recourse

Director : Olivier Steichen

Acute painful episodes impair the quality of life of sickle cell patients, leading to emergency department visits and subsequent hospitalizations. The use of emergency care varies from one patient to another and for the same patient over time. Hospital and national administrative databases can be used to characterize the patterns of health care use related to sickle cell disease, their frequency and the factors that determine it. Some of these factors, such as psychiatric comorbidities, may benefit from specific interventions to improve patient outcomes.

Contacts

Director
Pr Olivier STEICHEN
Phone : +33 1 56 01 60 77

Co-director
Dr François LIONNET
Phone : +33 1 56 01 67 91

Address
Centre de la drépanocytose
Service de médecine interne
Hôpital Tenon
4 rue de la Chine
75020 Paris

Mails
olivier.steichen@aphp.fr
francois.lionnet@aphp.fr

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