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European Respiratory Journal 12/04/2019 19:05
Rationale. Precapillary pulmonary hypertension (PH) is a devastating complication of sickle cell disease (SCD). Little is known about the influence of the SCD genotype on PH characteristics. Objectives. To describe clinical phenotypes and outcomes of precapillary PH due to SCD according to disease genotype. Methods. A nationwide multicentre retrospective study including all patients with SCD-related precapillary PH from the French PH Registry was conducted. Clinical characteristics and outcomes according to SCD genotype were analysed. Results. 58 consecutive SCD patients with precapillary PH were identified, of whom 41 had homozygous for haemoglobin S (SS) SCD, three had S-β 0 thalassaemia (S-β 0 thal) and 14 had haemoglobin SC disease (SC). Co.
In 2010, this last European Society of Hypertension protocol was revised (ie, European Society of Hypertension International Protocol 2 [ESH-IP2]), being more exigent than the previous protocol.

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