Sickle cell disease

Research output: Contribution to journalArticle

22 Citations (Scopus)

Abstract

• Sickle cell disease (SCD) is a heterogeneous group of prevalent, potentially life-threatening, chronic disorders of hemoglobin (Hgb). • Hgb polymerization underlies the pathophysiology of SCD. • Children who have SCD benefit from regular health maintenance visits with a pediatric hematologist and a primary care pediatrician. • The high incidence of invasive pneumococcal disease (IPD) in SCD justifies newborn screening, daily prophylactic penicillin, and immunization with the pneumococcal conjugate and polysaccharide vaccines. • Vaso-occlusive pain crises are the clinical hallmark of SCD and occur with increasing frequency through childhood. These episodes warrant aggressive treatment with analgesics and hydration and may be prevented with hydroxyurea (HU) therapy. • Annual transcranial Doppler (TCD) screening for patients ages 2 to 16 years identifies those at high risk for acute stroke, and regular blood transfusions can reduce this risk greatly. • Common indications for initiating HU therapy have been severe or frequent vaso-occlusive crises or acute chest syndrome, but this therapy may be considered in younger and less symptomatic patients. • The prognosis for children with SCD has improved, with the vast majority surviving into adulthood, prompting a focus on improving the process of transition to adult care.

Original languageEnglish (US)
Pages (from-to)195-206
Number of pages12
JournalPediatrics in Review
Volume33
Issue number5
DOIs
StatePublished - May 2012

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Sickle Cell Anemia
Hydroxyurea
Transition to Adult Care
Hemoglobins
Acute Chest Syndrome
Conjugate Vaccines
Pneumococcal Vaccines
Insurance Benefits
Therapeutics
Blood Transfusion
Polymerization
Penicillins
Analgesics
Immunization
Primary Health Care
Stroke
Newborn Infant
Pediatrics
Pain
Incidence

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Sickle cell disease. / McCavit, Timothy L.

In: Pediatrics in Review, Vol. 33, No. 5, 05.2012, p. 195-206.

Research output: Contribution to journalArticle

McCavit, Timothy L. / Sickle cell disease. In: Pediatrics in Review. 2012 ; Vol. 33, No. 5. pp. 195-206.
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