Jack L. Bartram

Glucose 6 phosphate dehydrogenase deficiency is not associated with cerebrovascular disease in children with sickle cell anemia

To the editor: Cerebrovascular disease is an important complication in children with sickle cell anemia (SCA).[1][1] Hemolysis is increasingly implicated in the pathogenesis.[2][2],[3][3] Recently, Bernaudin et al found that glucose 6 phosphate dehydrogenase (G6PD) deficiency was associated with

Extracranial internal carotid arterial disease in children with sickle cell anemia

Background Sickle cell anemia is one of the commonest causes of stroke in children. It is usually, but not always, associated with intracranial vasculopathy. We have assessed the value of ultrasound screening for extracranial internal carotid artery disease. Design and Methods Using Doppler ultrasound scanning, we assessed peak systolic blood velocity, tortuosity and stenosis in the extracranial internal carotid arteries of 236 children with sickle cell anemia. Seventeen of the children had previously had a stroke. All measurements were performed as part of routine clinical care. Results The median extracranial internal carotid artery velocity was 148cm/s (5th centile 84, 95th centile 236). Higher velocities were significantly correlated with younger age, higher white blood cell counts and higher rates of hemolysis. Fourteen (5.9%) had tortuous extracranial internal carotid arteries and 13 (5.4%) had stenosis or occlusion. None of the children with tortuous vessels but 8 of those with stenosis had previously had a stroke; the presence of stenosis was strongly associated with overt clinical stroke (OR 35.9, 95% C.I. 9.77–132, P<0.001). In 6 children, extracranial stenosis was part of extensive intracranial vasculopathy, but in 2 there was no evidence of intracranial disease. Stenosis seemed to be more common in older children. Conclusions Extracranial internal carotid artery stenosis is strongly associated with stroke in children with sickle cell anemia, and may explain some cases of stroke without overt intracranial vasculopathy. Doppler ultrasound scanning of extracranial internal carotid arteries is non-invasive and fairly quick to perform and may identify children at increased risk of stroke who would otherwise be missed. The value of extracranial internal carotid artery scanning should be studied prospectively.

Portacaths are safe for long-term regular blood transfusion in children with sickle cell anaemia

Peripheral venous access in children with sickle cell anaemia (SCA) requiring regular blood transfusions can become difficult over time. Previous reports have suggested the use of totally implantable venous access devices, Portacaths (PAC) in this patient group are associated with unacceptable high rates of complications. We present our experience in seven children with SCA over a 9-year period. Seven devices were placed for a total of 9754 PAC days during the study period. The median age at insertion was 6.3 years (range 3–15 years). The rate of PAC associated infection was 0.2 per 1000 PAC days. There were no episodes of thrombosis. The median length of time in situ during the study period was 3.7 years (range 1.3–7.5 years). Our experience highlights the safe and reliable use of PAC in children with SCA requiring regular blood transfusions when venous access has become a major problem.

Outcome of adults with sickle cell disease admitted to critical care – Experience of a single institution in the UK

Sickle cell disease (SCD) patients are perceived to have a high mortality when admitted to the Critical Care Unit (CCU). We performed a retrospective analysis of all adult sickle admissions to CCU at a single centre over an 8‐year period (1 January 2000 to 31 December 2007). Thirty‐eight patients (14 male) were admitted 46 times to CCU; the commonest reasons for admission were acute chest syndrome (14, 30%), multi‐organ failure (8, 17%) and planned post‐elective surgery (7, 15%). CCU mortality for SCD patients was 19·6%, comparable to a CCU‐wide mortality of 17·6% during the study period in the same institution. Re‐admission to CCU was high (16% over the 8‐year period) but did not increase mortality risk.

Soluble CD163 levels in children with sickle cell disease

Sickle cell disease (SCD) is characterized by vasculopathy, which has been causally linked to intravascular haemolysis and high levels of free plasma haemoglobin. Soluble CD163 (sCD163) is implicated in the clearance of free plasma haemoglobin and high plasma concentrations have been linked to arterial disease. We therefore investigated the value of sCD163 as a biomarker in children with SCD, and also measured haptoglobin levels in this population. We measured sCD163 in 25 control children with no haemoglobinopathy, 41 with sickle cell anaemia (HbSS) in the steady state, 27 with HbSS taking hydroxycarbamide, and 7 with HbSC disease. There was no significant difference between sCD163 levels in steady‐state HbSS (1·78 mg/l) and controls (1·81 mg/l) (P = 0·86). However, sCD163 levels were significantly lower in those HbSS children taking hydroxycarbamide (1·35 mg/l) compared to both steady state HbSS (P = 0·004) and controls (P = 0·036). In children on hydroxycarbamide, sCD163 correlated negatively and highly significantly with percentage HbF (R = −0·76, P < 0·001), and this relationship was absent in those not taking hydroxycarbamide (R = 0·07, P = 0·65). sCD163 is a potentially useful biomarker in children with SCD, and may have a role in monitoring responses to hydroxycarbamide.

Outcome of children with sickle cell disease admitted to intensive care - a single institution experience.

We retrospectively audited children with sickle cell disease (SCD) admitted to paediatric intensive care (PICU) at King’s College Hospital between January 2000 and December 2008. Forty‐six children with SCD were admitted, on 49 separate occasions. Ages ranged from 4 months to 15 years (median 7·6 years). Three children died in PICU, however two presented to hospital in cardiorespiratory arrest; overall mortality was 6%. The most common reason for admission was acute chest syndrome (43%). 88% of admissions required blood transfusion, of which 74% had exchange blood transfusions. The mortality among children with SCD admitted to PICU is low.

Outcome of children with sickle cell disease admitted to intensive care - a single institution experience: Short Report

We retrospectively audited children with sickle cell disease (SCD) admitted to paediatric intensive care (PICU) at King’s College Hospital between January 2000 and December 2008. Forty‐six children with SCD were admitted, on 49 separate occasions. Ages ranged from 4 months to 15 years (median 7·6 years). Three children died in PICU, however two presented to hospital in cardiorespiratory arrest; overall mortality was 6%. The most common reason for admission was acute chest syndrome (43%). 88% of admissions required blood transfusion, of which 74% had exchange blood transfusions. The mortality among children with SCD admitted to PICU is low.

Outcome of children with sickle cell disease admitted to intensive care

We retrospectively audited children with sickle cell disease (SCD) admitted to paediatric intensive care (PICU) at King’s College Hospital between January 2000 and December 2008. Forty‐six children with SCD were admitted, on 49 separate occasions. Ages ranged from 4 months to 15 years (median 7·6 years). Three children died in PICU, however two presented to hospital in cardiorespiratory arrest; overall mortality was 6%. The most common reason for admission was acute chest syndrome (43%). 88% of admissions required blood transfusion, of which 74% had exchange blood transfusions. The mortality among children with SCD admitted to PICU is low.

Outcome of adults with sickle cell disease admitted to critical care - experience of a single institution in the UK: Short Report

Sickle cell disease (SCD) patients are perceived to have a high mortality when admitted to the Critical Care Unit (CCU). We performed a retrospective analysis of all adult sickle admissions to CCU at a single centre over an 8‐year period (1 January 2000 to 31 December 2007). Thirty‐eight patients (14 male) were admitted 46 times to CCU; the commonest reasons for admission were acute chest syndrome (14, 30%), multi‐organ failure (8, 17%) and planned post‐elective surgery (7, 15%). CCU mortality for SCD patients was 19·6%, comparable to a CCU‐wide mortality of 17·6% during the study period in the same institution. Re‐admission to CCU was high (16% over the 8‐year period) but did not increase mortality risk.

Outcome of adults with sickle cell disease admitted to critical care

Sickle cell disease (SCD) patients are perceived to have a high mortality when admitted to the Critical Care Unit (CCU). We performed a retrospective analysis of all adult sickle admissions to CCU at a single centre over an 8‐year period (1 January 2000 to 31 December 2007). Thirty‐eight patients (14 male) were admitted 46 times to CCU; the commonest reasons for admission were acute chest syndrome (14, 30%), multi‐organ failure (8, 17%) and planned post‐elective surgery (7, 15%). CCU mortality for SCD patients was 19·6%, comparable to a CCU‐wide mortality of 17·6% during the study period in the same institution. Re‐admission to CCU was high (16% over the 8‐year period) but did not increase mortality risk.