High pulmonary artery pressures do occur in children with sickle cell disease. Screening by echocardiography can lead to early detection and intervention that may potentially reverse this disease process. There was no correlation between elevated pulmonary artery pressures and abnormal transcranial Doppler examination in our study.

Summary Elevated pulmonary artery pressures (PAP) occur in approximately 30% of children with sickle cell disease. In adults, pulmonary hypertension is significantly associated with mortality. There are no data on the long term significance in children. Nineteen children with SS/Sß0 thalassaemia had elevated PAP, defined as tricuspid regurgitant jet velocity (TRV) ≥2·5 m/s on screening echocardiograms. They were prospectively followed for 23 months (range 19–31 months). Patients with initial TRV ≥ 3 or TRV ≥ 2·5 m/s on repeat echocardiogram had cardiopulmonary evaluation and were offered treatment with hydroxyurea. Associated conditions like asthma and obstructive sleep apnea were treated. 18/19 patients had follow‐up echocardiograms. These showed normalization of TRV in 8 patients. Risk factors associated with persistent elevation were higher TRV on initial echocardiogram (P = 0·01), lower haemoglobin (P = 0·003) and lower oxygen saturation (P = 0·03). Five patients with persistently elevated PAP were treated with hydroxyurea. Mean right ventricular pressure dropped from 40·16 to 29·26 (P = 0·017) after 3–6 months and to 23·6 mmHg (P = 0·002) after 9–12 months on treatment. In conclusion (i) At borderline elevation of TRV there is intrapatient variability and echocardiograms should be repeated for confirmation. (ii) Elevated PAP are reversible in children with early detection and treatment with hydroxyurea.

The submandibular approach, when added to a standard transcranial Doppler examination, may increase the sensitivity of this technique to identify important potential sources of cerebral infarction.

Elevated TRV ≥ 2.5 m/second is significantly associated with proteinuria on longitudinal follow up in children with SCD.

Purpose: To study the relationship between complete blood count (CBC) indices over time, particularly serum hemoglobin (Hb) levels, and severity of macular thinning on spectral domain optical coherence tomography (SD-OCT) in patients with sickle cell disease (SCD). Methods: This is a single-center, retrospective analysis of 141 consecutive SCD patients over a 10-year period, of which 40 patients (79 eyes) had SD-OCT imaging of the macula and 29 (58 eyes, mean age 17.5 years) were eligible for the study. Investigators reviewed electronic medical records for documentation of retinopathy stage, disease genotype, CBC values, and SD-OCT imaging. SD-OCT parameters and CBC values were compared between different retinopathy stages and disease genotypes. Regression analyses were performed on SD-OCT parameters and CBC values. Results: Of the 58 eligible eyes (34HbSS, 18HbSC, 4HbSβ + thal, 2HbS βthal), 18 had PSR (proliferative sickle retinopathy), 14 had NPSR (nonproliferative sickle retinopathy), and 26 had NSR (no sickle retinopathy). Hb values were higher in SC group compared to SS group. Macular thickness in the temporal inner (Δ=26±33 um, p =0.01) and outer (Δ=21±30 um, p =0.02) subfields was higher in SC compared to SS group. Patients with SD-OCT thinning below the 5th percentile in the temporal outer subfields had lower recorded Hb nadirs (6.0±0.9) compared to those with thickness within the top 95th percentile (9.1±2.3). Regression analysis showed temporal macular thickness to be positively correlated with Hb values in the SS group. Conclusion: Macular thinning observed on SD-OCT in SCD patients with SS genotype may be related to the level of anemia in this population.