Publications by authors named "Patricia Benhaim"

Background: Systemic inflammatory diseases (SIDs) have been reported in patients with sickle cell disease (SCD), but clinical data in children are scarce.

Objectives: To identify clinical and laboratory features at diagnosis of SID in children with SCD and to describe their evolution.

Methods: Data from children with SCD and SIDs were retrospectively collected in a French multicenter study from 1991 to 2018.

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Objectives: Newborn screening (NBS) for β-thalassemia is based on measuring the expression of the hemoglobin A (HbA) fraction. An absence or very low level of HbA at birth may indicate β-thalassemia. The difficulty is that the HbA fraction at birth is correlated with gestational age (GA) and highly variable between individuals.

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Article Synopsis
  • * A total of 13 SCD-related deaths occurred, primarily in SS-genotype patients, with treatment non-compliance being a major risk factor, increasing mortality risk tenfold.
  • * Following the implementation of online guidelines, there was a noticeable improvement in patient care from 2006-2009, including better survival rates, increased TCD coverage, and more timely intensification of therapies.
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