Sickle Cell Disease in Children

Berger, Elizabeth; Saunders, Natasha; Wang, Lisa; Friedman, Jeremy

doi:10.1001/archpediatrics.2008.545

Cited by 69 publications

(47 citation statements)

References 10 publications

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“…Clinical features that increase the likelihood of osteomyelitis over an uncomplicated painful event are a single focus of pain, fever, and bacteremia. 10 However, painful events are far more common (50 times more common) than osteomyelitis in SCD, so the pretest probability of osteomyelitis always needs to be considered. Special imaging and biopsy of bone are best reserved for patients for whom there is high clinical suspicion of osteomyelitis.…”

Section: Clinical Scenarios and Complications Of Scdmentioning

confidence: 99%

Sickle Cell Disease in Childhood

Quinn

2013

Pediatric Clinics of North America

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Section: Clinical Scenarios and Complications Of Scdmentioning

confidence: 99%

Sickle Cell Disease in Childhood

Quinn

2013

Pediatric Clinics of North America

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“…The underlying pathology is the obstruction of the microvasculature by sickled red blood cells, resulting in chronic tissue ischaemia and tissue infarction; this may present as pain and/or swelling referred to as vaso-occlusive crisis (VOC). Vaso-occlusive events are the most common acute clinical presentation of SCD in children [2]. A combination of tissue infarction, immunodeficiency due to splenic dysfunction and excess iron leads to increase risk of osteomyelitis in SCD.…”

Section: Introductionmentioning

confidence: 99%

“…A combination of tissue infarction, immunodeficiency due to splenic dysfunction and excess iron leads to increase risk of osteomyelitis in SCD. Salmonella osteomyelitis is several hundred times more likely to occur in children with SCD than in the general population [2]–[4].…”

Section: Introductionmentioning

confidence: 99%

Dilemma in Differentiating between Acute Osteomyelitis and Bone Infarction in Children with Sickle Cell Disease: The Role of Ultrasound

et al. 2013

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BackgroundDistinguishing between acute presentations of osteomyelitis (OM) and vaso-occlusive crisis (VOC) bone infarction in children with sickle cell disease (SCD) remains challenging for clinicians, particularly in culture-negative cases. We examined the combined role of ultrasound scan (USS), C - reactive protein and White blood counts (WCC) in aiding early diagnosis in children with SCD presenting acutely with non-specific symptoms such as bone pain, fever or swelling which are common in acute osteomyelitis or VOC.MethodsWe reviewed the records of all children with SCD who were discharged from our department from October 2003 to December 2010 with a diagnosis of osteomyelitis based on clinical features and the results of radiological and laboratory investigations. A case control group with VOC who were investigated for OM were identified over the same period.ResultsIn the osteomyelitis group, USS finding of periosteal elevation and/or fluid collection was reported in 76% cases with the first scan (day 0–6). Overall 84% were diagnosed with USS (initial +repeat). 16% had negative USS. With VOC group, USS showed no evidence of fluid collection in 53/58 admissions (91%), none of the repeated USS showed any fluid collection. Mean C-reactive protein (CRP), and white cell count (WCC) were significantly higher in the OM.ConclusionThe use of Ultrasound in combination with CRP and WCC is a reliable, cost-effective diagnostic tool for differentiating osteomyelitis from VOC bone infarction in SCD. A repeat ultrasound and/or magnetic resonance imaging (MRI) scan may be is necessary to confirm the diagnosis.

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“…Distinguishing the acute presentation of osteomyelitis from VOC relies on clinical assessment (fever and pain on admission, swelling of affected limb, and painful sites) and radiological findings (ultrasound scans, magnetic resonance imaging [MRI]), in combination with elevated C-reactive protein (CRP) and white blood cell (WBC) counts (5,10). Distinct cytokine profiles were reported during early (1 to 4 months) and late (5 to 12 months) osteomyelitis episodes; the early-episode profiles are highlighted by an increased frequency of high tumor necrosis factor alpha (TNF-␣) and interleukin-4 (IL-4) producers, while the late-episode profiles are exemplified by increased frequencies of IL-10, IL-6, and IL-2 producers (11).…”

mentioning

confidence: 99%

Contribution of Reduced Interleukin-10 Levels to the Pathogenesis of Osteomyelitis in Children with Sickle Cell Disease

Sarray

Almawi

2015

Clin Vaccine Immunol

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Osteomyelitis is a significant complication of sickle cell disease (SCD), and several factors contribute to its pathogenesis, including altered expression of proinflammatory and anti-inflammatory cytokines. In view of the role of interleukin-10 (IL-10) as an anti-inflammatory cytokine, we tested the notion that SCD osteomyelitis is associated with a reduction in IL-10 secretion and, hence, precipitation of a proinflammatory state. Study subjects comprised 52 SCD patients with confirmed diagnosis of osteomyelitis and 165 age-and gender-matched SCD patients with negative histories of osteomyelitis. Results obtained showed that IL-10 serum levels in SCD osteomyelitis patients were significantly lower than those of control SCD patients. Receiver operating characteristic (ROC) analysis demonstrated that altered IL-10 serum levels predicted the development of osteomyelitis, and the mean area under ROC curves of IL-10 was 0.810 among SCD patients with osteomyelitis. A systematic shift in IL-10 serum levels toward lower values was seen in osteomyelitis cases, with an increased osteomyelitis risk associated with decreased IL-10 levels. Multivariate logistic regression analyses confirmed the independent association of reduced IL-10 with osteomyelitis after controlling for sickle hemoglobin (HbS), fetal hemoglobin (HbF), platelet count, and white blood cell (WBC) count. These data support the strong association of decreased IL-10 levels with osteomyelitis, thereby supporting a role for IL-10 in osteomyelitis follow-up.

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Sickle Cell Disease in Children

Cited by 69 publications

References 10 publications

Sickle Cell Disease in Childhood

Sickle Cell Disease in Childhood

Dilemma in Differentiating between Acute Osteomyelitis and Bone Infarction in Children with Sickle Cell Disease: The Role of Ultrasound

Contribution of Reduced Interleukin-10 Levels to the Pathogenesis of Osteomyelitis in Children with Sickle Cell Disease

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