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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The aim of this study was to propose a classification system for childhood arterial ischaemic stroke (AIS). Subtypes from the Trial of Org 10172 in Acute Stroke Therapy (TOAST) classification, previously shown to be applicable to children, were retained in the proposed Paediatric Stroke Classification (PSC). Additional important paediatric AIS aetiologies were identified from a literature review. Preliminary validation was performed by three raters who categorized clinical vignettes from 135 patients (66 male; median age 6.3 y, range 0.1 to 16 y). Eight aetiological subtypes were identified and defined, as follows: (1) sickle cell disease; (2) cardioembolic; (3) moyamoya syndrome; (4) cervical arterial dissection; (5) steno-occlusive cerebral arteriopathy; (6) other determined aetiology; (7) multiple probable/possible aetiologies; and (8) undetermined aetiology. There was very good agreement between the raters about categorization of the vignettes. Causes of disagreement were identified and final categories and definitions were modified accordingly. We conclude that the PSC enables the categorization of children with AIS into aetiological subtypes relevant to this age group. This will be useful in multicentre studies of natural history and treatment but will require further independent validation.
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PMID:A proposed classification for subtypes of arterial ischaemic stroke in children. 1583 48

A 10-year, retrospective review of the etiology, outcome, and complications of ischemic stroke in children from a nonurban population was conducted. Twenty-seven children were identified (14 boys, 13 girls), ages 1.25 to 17 years (mean 7.7 years). Etiologies included undetermined (22%), arterial dissection (19%), coagulopathy (15%), embolism (15%), moyamoya disease (11%), sickle cell disease (11%), isolated angiitis of the central nervous system or vasculitis (11%), or other known source (11%; two fibromuscular dysplasia, one L-asparaginase). More than one risk factor was present in five children. Seventeen (65%) children were anticoagulated, with no adverse events occurring. Nine children were anticoagulated initially with low-molecular-weight heparin. Other treatments included corticosteroids; physical, occupational, and speech therapy; and anticonvulsants for concomitant seizures. Follow-up ranged from 3 to 60 months (mean 17 months) and was as follows: 6 (22%) were normal, 9 (33%) had mild impairment, and 12 (44%) had moderate to severe deficits. There were no deaths. Neurologic complications included seizure (two), behavioral problems (two), and hemorrhagic conversion (one). In this population, the outcome from ischemic stroke was similar to that of other studies, with the majority of children demonstrating persistent neurologic deficits. Etiology could be determined for the majority of patients, with 19% having more than one risk factor.
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PMID:Childhood ischemic stroke in a nonurban population. 1583 8

Moyamoya angiopathy is a well-known complication of sickle cell disease but has rarely been observed in other hemoglobinopathies. The authors describe a previously unreported association of hemolytic anemia due to a rare unstable hemoglobinopathy with abnormal oxygen affinity (Hb Alesha) and moyamoya syndrome in a 10-year-old girl. At age 4 she had recurrent migraine-with-aura-like symptoms. Cranial MRI, Doppler, and EEG examinations were not conclusive. Deterioration of her neurologic symptoms prompted a renewed EEG examination at 10 years of age, which revealed a re-buildup phenomenon. MRI and MR angiography now showed moyamoya angiopathy with stenotic and occlusive lesions of both internal carotid and middle cerebral arteries. Conventional angiography confirmed these findings. Reperfusion with three extra-intracranial bypasses terminated the transient ischemic attacks. The authors suggest that chronic hypoxemia may be the cause of occlusive moyamoya angiopathy in Hb Alesha and possibly other unstable hemoglobinopathies with altered oxygen affinity.
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PMID:Moyamoya syndrome associated with hemolytic anemia due to Hb Alesha. 1609 27

The authors report the unique case of a 6-year-old African-American girl with sickle cell disease (SCD) and an associated moyamoya arteriopathy who developed a de novo arteriovenous malformation (AVM) of the cerebral circulation. Based on preoperative cerebral angiography, computerized tomography angiography, and magnetic resonance imaging, the incidentally discovered lesion was originally thought to be a direct arteriovenous fistula with an associated varix. At surgery, however, a 1.5-cm AVM was identified adjacent to the deep surface of the varix, and it was successfully resected. The diagnosis of cerebral AVM was then confirmed histopathologically. Based on a review of the literature, no published correlation between cerebral AVMs and SCD exists. In addition to reporting this case, the authors provide a description of AVM pathogenesis, with particular emphasis on acquired AVMs of the cerebral circulation.
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PMID:Development of a de novo cerebral arteriovenous malformation in a child with sickle cell disease and moyamoya arteriopathy. Case report. 1615 38

Intelligence is reported to decline after onset of moyamoya in Japanese populations, but there is less evidence for this in Western populations where the condition may be secondary to stroke and sickle cell anaemia (SCA). Preoperative longitudinal IQ data were obtained from 15 children (seven males, eight females) who developed moyamoya syndrome (MMS) following a stroke (six with SCA, nine without SCA), and 19 controls (10 males, nine females; nine healthy control participants, 10 with SCA). At baseline assessment (Time 1) median age of patients was 7 years 6 months (range 3y 7mo to 12y 5mo); median age of controls was 6 years 3 months (range 4y to 11y 6mo). At follow-up (Time 2), ages were 11 years 8 months (range 3y 7mo to 12y 5mo) and 12 years 8 months (range 6y 4mo to 16y 8mo) in patients and controls respectively. Median duration of follow-up for the patient group was 3 years (range 7 to 10y) and in controls, 4 years 1 month (range 1 to 10y). In children with SCA, Verbal and Performance IQs (VIQ and PIQ) were significantly lower than in controls at Time 1; there was an additional independent statistically significant reduction in PIQ associated with MMS (p=0.004). Although there were further significant reductions in IQ by the second assessment for patients with MMS compared with controls, IQ did not differ significantly between groups with and without SCA. While the reduction in IQ attributed to SCA does not appear to become more marked with increasing age, the difference between those with and without MMS is associated with increasing effect over time.
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PMID:Intellectual decline in children with moyamoya and sickle cell anaemia. 1628 73

Prompt recognition and early intervention, with pertinent management and medication, may reduce subsequent neurologic deficits in stroke, which constitutes a devastating event in children. This is due to the tasking and demanding consequences including death or residual neurological deficits, which may last for many decades, in over 60% of survivors. Evidence-based treatment for children with stroke is still lacking, reflecting scarcity in baseline epidemiological data on pediatric stroke, the multitude of underlying risk factors, and the ethical and practical challenges incurred in conducting clinical trials. Based on the experience we gained from a combined prospective and retrospective study on childhood stroke (covering 10 years and 7 months and involving a cohort of 104 Saudi children), a diagnostic algorithm, which outlines the approach to a child with suspected stroke/cerebrovascular lesion, was designed. This algorithm might also be of use for managing other children with stroke from the Arabian Peninsula and Middle Eastern Region with similar demographic, socioeconomic, and ethnic backgrounds. Underlying risk factors, which need special attention, include thrombophilia and hypercoagulable states and sickle cell disease (SCD), which contrary to previous studies from Saudi Arabia, were found to constitute a common risk factor with severe manifestations. Other risk factors include infections (especially neurobrucellosis), cardiac diseases, and hypernatremic dehydration. Recognition of an identifiable syndrome or inherited metabolic cause may unravel an underlying cerebrovascular disease. This is particularly important in this region, given the large pool of autosomal recessive diseases and the high rate of consanguinity. In the evaluation of a suspected case of stroke, important imaging modalities include cranial CT, MRI (including diffusion-weighted images), magnetic resonance angiography (MRA), magnetic resonance venography (MRV) and conventional angiography. Transcranial Doppler sonography of the intracranial vessels and Duplex scan of the neck are valuable modalities for detecting large vessel vasculopathy, which occur in SCD, moyamoya syndrome, arterial dissection, and stenosis. Antithrombotic drugs are increasingly being used in the acute phase of childhood ischemic stroke. These include unfractionated heparin, low-molecular-weight heparins, aspirin or warfarin, or both. Specialized stroke care and follow-up are needed for children with stroke, as well as their families.
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PMID:Diagnostic approach and management strategy of childhood stroke. 1653 30

Stroke is one of the major complications in children with sickle cell disease (SCD). Ischemic stroke is associated with small asymptomatic subcortical infarcts to large territorial lesions causing major disability. Intracranial hemorrhages may be caused by aneurysm rupture or by leakage from moyamoya vessels or venous sources. There have been no acute stroke treatment studies in SCD, but hydration and exchange transfusion are often recommended. However, there is an evidence base for primary and to some extent secondary stroke prevention. Primary prevention of stroke was demonstrated in the Stroke Prevention Trial in Sickle Cell Anemia (STOP), in which children with transcranial Doppler (TCD) mean blood flow velocities of 200 cm/second (previously shown to indicate high stroke risk) or higher were randomized to either regular blood transfusions or no transfusion. The study showed a very significant 90% reduction in first stroke with transfusion. In STOP2, discontinuing transfusions after 30 months or more (even with normal TCD) resulted in a high rate of reversion to abnormal TCD values and stroke. TCD screening of all children with SCD, and initiation and maintenance of chronic transfusion to maintain hemoglobin S below 30% in the high-risk group, is the only proven prevention strategy for stroke in SCD. Hydroxyurea is being studied as secondary stroke prevention at this time. No recommendation specific to SCD regarding the use of antiplatelet agents or anticoagulants in ischemic stroke can be made. Bone marrow transplantation can be curative for SCD, and limited data support its use to prevent stroke in SCD.
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PMID:Treatment and prevention of stroke in children with sickle cell disease. 1703 71

A 43-year-old lady, known case of sickle cell disease (SCD) was admitted in sickle cell crises and developed a left frontal intracerebral hematoma. She worsened further neurologically and was found to have developed a large left middle cerebral artery (MCA) infarct. Angiogram showed Moyamoya pattern. The patient was managed conservatively with exchange transfusions and made good recovery. She is being maintained on monthly exchange transfusions and hydroxyurea. Such a presentation has been described infrequently, that too mostly in children. Only once, it has been reported with adult SCD. Diagnostic and management controversies are discussed in the light of available literature.
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PMID:Ischemic infarction following an intra-cerebral hemorrhage in an adult sickle cell disease with angiographic Moyamoya. 1754 64

Vascular occlusive diseases affect brain blood flow, brain metabolism and are associated with arterial ischemic stroke. This study was designed to measure the brain blood flow velocity, brain oxygenation, hemoglobin concentrations, hematocrit, and cell free hemoglobin at pre- and post-exchange red cell transfusion in an 18 year old male patient with sickle cell disease and moyamoya syndrome (MMS). Exchange transfusion increased cerebral oxygen saturation 12%, total hemoglobin concentration 2%, hemoglobin AA 80%, and reduced sickle (SS) hemoglobin 12%, arterializations 33%, and cell free hemoglobin 33%. Brain blood flow velocity values were unaffected by transfusion. These observations suggest that exchange transfusion increases the hemoglobin carrying capacity and reduces sickle hemoglobin and shunting of blood, which may improve the peripheral and cerebral oxygenation. Transfusion did not affect the brain blood flow in this patient. Therefore the risk of transient ischemic attack and arterial ischemic stroke from mms still exist.
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PMID:Effect of transfusion on cerebral oxygenation, flow velocity in a patient with sickle cell anemia and Moyamoya disease: a case report. 1760 91

The paediatric neurologist is usually the clinician who makes the diagnosis of moyamoya in children, yet most of the debate in the literature has focused on surgical management of the condition. Semantic confusion and variable use of the term among neuroradiologists continues to be unhelpful. Increasing recognition of moyamoya, for example in sickle cell disease, and the publication of clinical guidelines encouraging referral for surgical evaluation highlight the need to identify and engage with management of the condition. In practical terms, the most frequent management issues for the paediatric neurologist, other than when to refer for surgery, are headache, hypertension, and the concern of the family that other children might be affected. These issues are discussed in the context of the available literature, and areas in which there is a need for research and consensus are identified.
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PMID:Moyamoya: to cut or not to cut is not the only question. A paediatric neurologist's perspective. 1989 33


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