P11 PULMONARY ARTERIAL HYPERTENSION IN CHILDREN WITH SICKLE CELL DISEASE: HOW COMMON IS IT?

R.. Chaudry, presenting, T. Karu, C. Hutchinson, S. Ball, G. Sutherland, A. Bush, M. Rosenthal, S. Crowley.Royal Brompton Hospital, London; St George’s Hospital, London, UK

Introduction: The prevalence of pulmonary arterial hypertension (PAH) in adults with sickle cell disease (SCD) is estimated to be 32%, 2-year mortality of 50% from diagnosis.

Aims: To prospectively determine the prevalence of PAH in children with SCD and define associated risk factors such as hyper-haemolytic state defined by haemoglobin ⩽8.5 g/dl and number of acute chest syndrome events

Methods: Fifty (F = 26) patients with SCD, range 10–18 years, and 50 (F = 25) sex, age, race-matched healthy controls were recruited. Tricuspid regurgitant jet velocity was measured using detailed 2D-Doppler echo; all subjects were at baseline state of health. Patients were free of any vaso-occlusive crisis for ⩾14 days. Hb was measured and a detailed medical history was taken with verification from hospital notes.

Results: The youngest patient with elevated TRV was 10 years.

Conclusion: These data show a significant number of children with SCD have echo evidence of PAH. We were unable to identify an association between elevated TRV and hyper-haemolysis, number of ACS events or increasing age in the patient group. We suggest all children with SCD be screened for PAH from 5 years onwards. Longitudinal studies with therapeutic intervention are required to enable monitoring of disease progression and response to treatment.

P12 THE ROLE OF ANDROGENS IN FETAL GROWTH

H. Miles, presenting, K. Ong, I. Hughes.University of Cambridge, Cambridge, UK

Introduction: Consistent reports of lower mean birth weights in male infants with hypospadias have lead to the suggestion that androgen activity in the fetus promotes fetal growth and explains the 300 g larger birth weight of males compared to females. We tested this hypothesis in genetic models of decreased androgen receptor activity.

Methods: In 64 male infants with hypospadias, gestational age …