Spontaneous Intracranial Extradural Hematoma in Sickle Cell Disease.
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ABSTRACT:
SUBMITTER: N'dri Oka D
PROVIDER: S-EPMC4520986 | biostudies-other | 2015 Jul
REPOSITORIES: biostudies-other
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Project description:Matrix metalloproteinases (MMPs) are proteolytic zinc-endopeptidases regulated by tissue Inhibitors of matrix metalloproteinases (TIMPs). We evaluated the potential of MMPs and TIMPs as clinical tools for Intracranial Haemorrhage (ICH). Spontaneous non-traumatic ICH patients were recruited from two hospitals: Complejo Hospitalario de Navarra (CHN?=?29) and Vall d´Hebron (VdH?=?76). Plasmatic levels of MMP-1, -2, -7, -9, -10 and TIMP-1 and their relationship with clinical, radiological and functional variables were evaluated. We further studied the effect of TIMP-1 (0.05-0.2?mg/Kg) in an experimental tail-bleeding model. In CHN, TIMP-1 was associated with admission-hematoma volume and MMP-7 was elevated in patients with deep when compared to lobar hematoma. In VdH, admission-hematoma volume was associated with TIMP-1 and MMP-7. When data from both hospitals were combined, we observed that an increase in 1?ng/ml in TIMP-1 was associated with an increase of 0.14?ml in haemorrhage (combined ??=?0.14, 95% CI?=?0.08-0.21). Likewise, mice receiving TIMP-1 (0.2?mg/Kg) showed a shorter bleeding time (p?<?0.01). Therefore, the association of TIMP-1 with hematoma volume in two independent ICH cohorts suggests its potential as ICH biomarker. Moreover, increased TIMP-1 might not be sufficient to counterbalance MMPs upregulation indicating that TIMP-1 administration might be a beneficial strategy for ICH.
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Project description:BackgroundPrevalence and contribution of intracranial and extracranial arterial stenosis to stroke risk were assessed prospectively in children and young adults with sickle cell disease.MethodsIn this cross-sectional study, children and young adults (mean = 19.4 years) with sickle cell disease underwent neurological examination, brain MRI, and magnetic resonance angiography of the head and neck. Two neuroradiologists independently recorded infarcts and arterial stenosis. Clinical features and stroke outcomes were compared between participants with and without stenosis and between children and young adults. Logistic regression analysis assessed the association of variables of interest with overt stroke and silent cerebral infarct.ResultsOf 167 participants (79 children and 88 young adults), 20 (12.0%) had intracranial stenosis, all in the anterior circulation, and nine had concurrent extracranial stenosis. No participants had isolated extracranial stenosis. Participants with intracranial stenosis were more likely than those without stenosis to have an overt stroke (70% vs 5%, P < 0.001) or silent cerebral infarct (95% vs 35%, P < 0.001). Logistic regression analysis indicated that intracranial stenosis was strongly associated with overt stroke when compared with participants with silent cerebral infarct alone and strongly associated with silent cerebral infarct when compared with participants with normal brain MRI; male sex and age were also significant predictors of silent cerebral infarct.ConclusionsIntracranial stenosis was strongly associated with both overt stroke and silent cerebral infarct; prevalence of intracranial stenosis was similar to prior estimates in sickle cell disease. Extracranial stenosis without concurrent intracranial stenosis did not occur and thus could not be evaluated as an independent risk factor for stroke.
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Project description:To establish a nomogram model to predict early cognitive impairment after supratentorial spontaneous intracranial hematoma in adult patients.A retrospective cohort study was held between January 2016 and October 2018. One hundred twenty seven out of 170 consecutive patients with supratentorial spontaneous intracranial hematoma were enrolled in this study. They were divided into development (n = 92) and validation (n = 35) dataset according to their admission time. Mini-mental State Examination (MMSE) was conducted between the third and the sixth month after the onset of stroke. MMSE ≤ 24 was considered as cognitive impairment. Univariate and multivariate logistic regression was used to screen for independent risk factors which correlate with cognitive impairment on the development dataset. A nomogram was built based on Akaike Information Criterion (AIC). Receiver operating characteristic (ROC) curve and calibration curve on development and validation dataset was drawn with each area under the curves (AUC) calculated. The decision curve analysis was also conducted with the development dataset.The bleeding volume, Glasgow Coma Scale (GCS), and intraventricular hemorrhage (IVH) are the most significant risk factors which may cause cognitive impairment both in the univariate and multivariate analysis. The finial model performed good discrimination ability on both development and validation dataset with AUC 0.911 and 0.919. Most patients would benefit from the model according to the decision curve analysis.A nomogram, constructed based on bleeding volume, GCS, and IVH can provide a feasible tool to evaluate cognitive impairment after supratentorial spontaneous intracranial hematoma in adult patients.
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Project description:Early identification of infants with sickle cell disease (SCD) by newborn screening, now universal in all 50 states in the US, has improved survival, mainly by preventing overwhelming sepsis with the early use of prophylactic penicillin. Routine transcranial Doppler screening with the institution of chronic transfusion decreases the risk of stroke from 10% to 1% in paediatric SCD patients. Hydroxyurea decreases the number and frequency of painful crises, acute chest syndromes and number of blood transfusions in children with SCD. Genetic research continues to be driven toward the prevention and ultimate cure of SCD before adulthood. This review focuses on clinical manifestations and therapeutic strategies for paediatric SCD as well as the evolving topic of gene-focused prevention and therapy.
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