Reference Values and Hematologic Changes From Birth to 5 Years in Patients With Sickle Cell Disease

Brown, Audrey K.; Sleeper, Lynn A.; Miller, Scott T.; Pegelow, Charles H.; Gill, Frances M.; Waclawiw, Myron A.

doi:10.1001/archpedi.1994.02170080026005

Cited by 95 publications

(74 citation statements)

References 27 publications

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“…33 The majority of SCIs occur in children younger than 6 years, 4 as the hemoglobin levels decline continuously until levels reach steady state. 31 In addition to our findings that low hemoglobin levels are associated with SCI, two singlecenter studies of children with SCA have demonstrated similar findings. Using a multivariable analysis from a retrospective cohort, Bernaudin et al demonstrated that low hemoglobin was associated with an increased rate of SCI.…”

Section: Org Fromsupporting

confidence: 82%

“…Other potential mechanisms that are probably important and associated with hemodynamic insufficiency include vascular stenosis, dural venous sinus thrombosis, and posterior reversible encephalopathy syndrome, often referred to as PRES. 30 Additional support for the precarious balance between oxygen delivery and demand that may result in SCI is the observation that infants with SCA may begin to develop their anemia as young as 10 weeks of age 31 and that, at 3 months of age, the mean hemoglobin of infants with HbSS is approximately 1.5 g/dL below expected for children without SCA of the same age. 31 The lower hemoglobin levels may explain in part why SCIs have been detected in an infant as young as 7 months 32 and in several infants younger than 15 months.…”

Section: Org Frommentioning

confidence: 99%

“…30 Additional support for the precarious balance between oxygen delivery and demand that may result in SCI is the observation that infants with SCA may begin to develop their anemia as young as 10 weeks of age 31 and that, at 3 months of age, the mean hemoglobin of infants with HbSS is approximately 1.5 g/dL below expected for children without SCA of the same age. 31 The lower hemoglobin levels may explain in part why SCIs have been detected in an infant as young as 7 months 32 and in several infants younger than 15 months. 33 The majority of SCIs occur in children younger than 6 years, 4 as the hemoglobin levels decline continuously until levels reach steady state.…”

Section: Org Frommentioning

confidence: 99%

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Associated risk factors for silent cerebral infarcts in sickle cell anemia: low baseline hemoglobin, sex, and relative high systolic blood pressure

DeBaun

Sarnaik

Rodeghier³

et al. 2012

Blood

182

144

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The most common form of neurologic injury in sickle cell anemia (SCA) is silent cerebral infarction (SCI). In the Silent Cerebral Infarct Multi-Center Clinical Trial, we sought to identify risk factors associated with SCI. In this cross-sectional study, we evaluated the clinical history and baseline laboratory values and performed magnetic resonance imaging of the brain in participants with SCA (HbSS or HbS␤°thalassemia) between the ages of 5 and 15 years with no history of overt stroke or seizures. Neuroradiology and neurology committees adjudicated the presence of SCI. SCIs were diagnosed in 30.8% (251 of 814) participants who completed all evaluations and had valid data on all prespecified demographic and clinical covariates. The mean age of the participants was 9.1 years, with 413 males (50.7%). In a multivariable logistic regression analysis, lower baseline hemoglobin concentration (P < .001), higher baseline systolic blood pressure (P ‫؍‬ .018), and male sex (P ‫؍‬ .030) were statistically significantly associated with an increased risk of an SCI. Hemoglobin concentration and systolic blood pressure are risk factors for SCI in children with SCA and may be therapeutic targets for decreasing the risk of SCI. This study is registered at www.clinicaltrials.gov as #NCT00072761. IntroductionSilent cerebral infarcts (SCIs) have been recognized by neuroimaging in neurologically normal older adult populations since 1981 1 and were documented in sickle cell anemia (SCA) soon afterward. 2 As with overt stroke, SCIs represent a clinical finding that is common in older adults without SCD, but they appear during early childhood in persons with SCA. SCIs are defined as an MRI signal abnormality visible on 2 views on the T2-weighted images (axial and coronal) that must measure at least 3 mm in one dimension; further, the person deemed to have an SCI must have an absence of focal neurologic deficit compatible with the anatomic location of the brain lesion. 3 SCI is the most common form of neurologic injury among children with SCA, occurring in at least 27% before 6 years of life 4 and 37% by 14 years of life. 5 SCIs in children with SCA are associated with increased risk of future overt strokes and new or progressive SCIs. 6,7 In addition, children with SCA and SCI have been found to have poorer cognitive function The publication costs of this article were defrayed in part by page charge payment. Therefore, and solely to indicate this fact, this article is hereby marked ''advertisement'' in accordance with 18 USC section 1734. 3684BLOOD, 19 APRIL 2012 ⅐ VOLUME 119, NUMBER 16For personal use only. on March 28, 2019. by guest www.bloodjournal.org From than children with SCA with normal MRI of the brain 8-10 or sibling controls. 10,11 Clinical and laboratory risk factors for SCI have been evaluated only sparingly. In the most rigorous study to date, the investigators from the Cooperative Study for Sickle Cell Disease (CSSCD) described risk factors associated with SCI in 42 participants, comparing them with 188 controls with ...

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Section: Org Fromsupporting

confidence: 82%

Section: Org Frommentioning

confidence: 99%

Section: Org Frommentioning

confidence: 99%

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Associated risk factors for silent cerebral infarcts in sickle cell anemia: low baseline hemoglobin, sex, and relative high systolic blood pressure

DeBaun

Sarnaik

Rodeghier³

et al. 2012

Blood

182

144

View full text Add to dashboard Cite

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“…Over time, the spleen loses its function via progressive atrophy, a state known as autosplenectomy and associated with a lifelong susceptibility to infections due to encapsulated bacteria. Among SS children aged 5 years, 90% have functional or anatomical asplenia (Brown et al, 1994) and therefore require daily prophylactic penicillin therapy and appropriate immunizations, including the pneumococcus vaccine.…”

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confidence: 99%

Acute splenic sequestration crisis in sickle cell disease: cohort study of 190 paediatric patients

et al. 2012

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SummaryAcute splenic sequestration crisis (ASSC) is an unpredictable life-threatening complication of sickle cell disease (SCD) in infants. Here, our objective was to update available clinical information on ASSC. We retrospectively studied the 190 patients who were diagnosed at birth with SS or Sbeta 0 in the Paris conurbation between 2000 and 2009 and who experienced ASSC. They had 437 ASSC episodes (0AE06/patient-year). Median age at the first episode was 1AE4 years (0AE1-7) and 67% of patients had more than one episode. Age was the only factor predicting recurrence: the risk was lower when the first episode occurred after 2 years versus before 1 year of age (hazard ratio, 0AE60; 95% confidence interval, 0AE41-0AE88; P = 0AE025). A concomitant clinical event was found in 57% of episodes. The mortality rate was 0AE53%. The treatment consisted in watchful waiting without prophylactic blood transfusions or splenectomy in 103 (54%) patients and in a blood transfusion programme in 55 (29%) patients. Overall, splenectomy was performed in 71 (37%) patients, at a median age of 4AE5 years (range, 1AE9-9AE4). In conclusion, aggressive treatment may be warranted in patients experiencing ASSC before 2 years of age. Randomized controlled trials are needed to define the best treatment modalities.

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“…Splenic uptake was absent at the end of the study period for 47% of HU-SOFT participants, which compared favorably to children in the Cooperative Study of Sickle Cell Disease, in which 78% of children had pitted RBC counts Ͼ 3.5% by the age of 3 years. 54 An extended study with dose escalation continued to demonstrate hematologic responses with increased fetal hemoglobin, normal growth, and no novel toxicities. 55 In examining those subjects with serial assessments of splenic function, 21.5% had normal uptake after a mean duration of 4.9 years on hydroxyurea, whereas 43% at extension exit had absent splenic uptake, at an age when the majority of children with homozygous sickle cell historically were presumed to have functional asplenia.…”

Section: Primary Prophylactic Interventions In Scd Penicillinmentioning

confidence: 99%

Primary Prophylaxis in Sickle Cell Disease: Is It Feasible? Is It Effective?

Thompson

2011

Hematology

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Consideration of primary prophylaxis to prevent complications of sickle cell disease (SCD) requires a clear understanding of the earliest manifestations of SCD-related organ injury, a reliable clinical or laboratory tool to detect organ dysfunction, and evidence that an intervention instituted in the presymptomatic state can mitigate disease progression. This review examines the pathophysiology of SCD in organs that may be potential targets for intervention, our current capacity to evaluate early SCD manifestations, results of clinical trials, and opportunities for future interventions.

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Reference Values and Hematologic Changes From Birth to 5 Years in Patients With Sickle Cell Disease

Cited by 95 publications

References 27 publications

Associated risk factors for silent cerebral infarcts in sickle cell anemia: low baseline hemoglobin, sex, and relative high systolic blood pressure

Associated risk factors for silent cerebral infarcts in sickle cell anemia: low baseline hemoglobin, sex, and relative high systolic blood pressure

Acute splenic sequestration crisis in sickle cell disease: cohort study of 190 paediatric patients

Primary Prophylaxis in Sickle Cell Disease: Is It Feasible? Is It Effective?

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