Chronic organ injuries in children with sickle cell disease
| Autor: | Mariane de Montalembert, Melissa Taylor, Slimane Allali, Joséphine Brice |
|---|---|
| Rok vydání: | 2021 |
| Předmět: |
medicine.medical_specialty
Cell Anemia Sickle Cell Review Article Disease Asymptomatic 03 medical and health sciences 0302 clinical medicine Antisickling Agents Internal medicine medicine Humans Hydroxyurea Blood Transfusion Child Stroke business.industry Hematology medicine.disease Pulmonary hypertension Transcranial Doppler medicine.anatomical_structure 030220 oncology & carcinogenesis Albuminuria Cardiology Eye disorder medicine.symptom business 030215 immunology |
| Zdroj: | Haematologica |
| ISSN: | 1592-8721 0390-6078 |
| Popis: | Median life expectancy of patients with sickle cell disease has increased to up to 55 years but there are still frequent cases of premature death, mostly in patients with pre-existing organ failure such as pulmonary hypertension, kidney injury, and cerebral vasculopathy. Most organ injuries remain asymptomatic for a long time and can only be detected through early systematic screening. Protocols combining assessment of velocities on transcranial Doppler and regular transfusions in patients with abnormal velocities have been demonstrated to dramatically reduce the risk of stroke. In contrast, no consensus has been reached on systematic screening or therapy for silent cerebral infarcts. The prognostic significance of increased tricuspid regurgitant jet velocity on echocardiography has not yet been identified in children, whereas increased albuminuria is a good predictor of kidney injury. Finally, screening for hip and eye disorder is recommended; however, different countries adopt different screening strategies. Hydroxyurea is probably of potential benefit in preventing chronic organ damage but this requires further study in order to be fully demonstrated. Efficacy and safety of the other new drugs available are also under investigation. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
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