Disease severity and slower psychomotor speed in adults with sickle cell disease

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First study of cognitive functioning in adult patients with SCD genotypes other than homozygous for hemoglobin S. Patients with “severe” genotypes had significantly poorer speed of processing when compared with those with “moderate” genotypes.

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Global epidemiology of haemoglobin disorders and derived service indicators

Bernadette Modell, Matthew Darlison (2008)

To demonstrate a method for using genetic epidemiological data to assess the needs for equitable and cost-effective services for the treatment and prevention of haemoglobin disorders. We obtained data on demographics and prevalence of gene variants responsible for haemoglobin disorders from online databases, reference resources, and published articles. A global epidemiological database for haemoglobin disorders by country was established, including five practical service indicators to express the needs for care (indicator 1) and prevention (indicators 2-5). Haemoglobin disorders present a significant health problem in 71% of 229 countries, and these 71% of countries include 89% of all births worldwide. Over 330 000 affected infants are born annually (83% sickle cell disorders, 17% thalassaemias). Haemoglobin disorders account for about 3.4% of deaths in children less than 5 years of age. Globally, around 7% of pregnant women carry b or a zero thalassaemia, or haemoglobin S, C, D Punjab or E, and over 1% of couples are at risk. Carriers and at-risk couples should be informed of their risk and the options for reducing it. Screening for haemoglobin disorders should form part of basic health services in most countries.

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Mortality Rates and Age at Death from Sickle Cell Disease: U.S., 1979–2005

Sophie Lanzkron, C Carroll, Carlton Haywood (2013)

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Central nervous system complications and management in sickle cell disease.

Michael DeBaun, Fenella Kirkham (2016)

With advances in brain imaging and completion of randomized clinical trials (RCTs) for primary and secondary stroke prevention, the natural history of central nervous system (CNS) complications in sickle cell disease (SCD) is evolving. In order of current prevalence, the primary CNS complications include silent cerebral infarcts (39% by 18 years), headache (both acute and chronic: 36% in children with sickle cell anemia [SCA]), ischemic stroke (as low as 1% in children with SCA with effective screening and prophylaxis, but ∼11% in children with SCA without screening), and hemorrhagic stroke in children and adults with SCA (3% and 10%, respectively). In high-income countries, RCTs (Stroke Prevention in Sickle Cell Anemia [STOP], STOP II) have demonstrated that regular blood transfusion therapy (typically monthly) achieves primary stroke prevention in children with SCA and high transcranial Doppler (TCD) velocities; after at least a year, hydroxycarbamide may be substituted (TCD With Transfusions Changing to Hydroxyurea [TWiTCH]). Also in high-income countries, RCTs have demonstrated that regular blood transfusion is the optimal current therapy for secondary prevention of infarcts for children with SCA and strokes (Stroke With Transfusions Changing to Hydroxyurea [SWiTCH]) or silent cerebral infarcts (Silent Infarct Transfusion [SIT] Trial). For adults with SCD, CNS complications continue to be a major cause of morbidity and mortality, with no evidence-based strategy for prevention.

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Author and article information

Contributors

Dana R. Jorgensen: (View ORCID Profile)

Andrea Metti: (View ORCID Profile)

Caterina Rosano: (View ORCID Profile)

Enrico M. Novelli: (View ORCID Profile)

Journal

Title: Blood Advances

Publisher: American Society of Hematology

ISSN (Print): 2473-9529

ISSN (Electronic): 2473-9537

Publication date Created: September 26 2017

Publication date (Print): September 26 2017

Publication date (Electronic): September 14 2017

Volume: 1

Issue: 21

Pages: 1790-1795

Affiliations

[1 ]Department of Epidemiology, School of Public Health,

[2 ]Department of Psychiatry, School of Medicine,

[3 ]Department of Radiology, School of Medicine, and

[4 ]Pittsburgh Heart, Lung and Blood Vascular Medicine Institute, Department of Medicine, School of Medicine, University of Pittsburgh, Pittsburgh, PA

Article

DOI: 10.1182/bloodadvances.2017008219

PubMed ID: 29296825

SO-VID: 8dc1bcea-0a66-4af3-ad7a-a75192154c1c

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Disease severity and slower psychomotor speed in adults with sickle cell disease

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Procedural speed in criminal law

Most cited references 23

Global epidemiology of haemoglobin disorders and derived service indicators

Mortality Rates and Age at Death from Sickle Cell Disease: U.S., 1979–2005

Central nervous system complications and management in sickle cell disease.

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Cited by 7

Most referenced authors 634