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 language | English (US) |
|---|---|
| Pages (from-to) | 195-206 |
| Number of pages | 12 |
| Journal | Pediatrics in Review |
| Volume | 33 |
| Issue number | 5 |
| DOIs | |
| State | Published - May 2012 |
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ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
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Sickle cell disease. / McCavit, Timothy L.
In: Pediatrics in Review, Vol. 33, No. 5, 05.2012, p. 195-206.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Sickle cell disease
AU - McCavit, Timothy L.
PY - 2012/5
Y1 - 2012/5
N2 - • 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.
AB - • 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.
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U2 - 10.1542/pir.33-5-195
DO - 10.1542/pir.33-5-195
M3 - Article
C2 - 22550263
AN - SCOPUS:84860809568
VL - 33
SP - 195
EP - 206
JO - Pediatrics in Review
JF - Pediatrics in Review
SN - 0191-9601
IS - 5
ER -