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  1. Article: Surgical Management of Harlequin Ichthyosis.

    Margulies, Ilana G / Harburg, Leah / Zellner, Elizabeth

    Plastic and reconstructive surgery. Global open

    2019  Volume 7, Issue 5, Page(s) e2161

    Language English
    Publishing date 2019-05-21
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2851682-5
    ISSN 2169-7574 ; 2169-7574
    ISSN (online) 2169-7574
    ISSN 2169-7574
    DOI 10.1097/GOX.0000000000002161
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: An unusual case of hemophagocytic lymphohistiocytosis diagnosed by spinal nerve root biopsy.

    Harburg, Leah / Cooper, Jared B / Flower, Allyson / Tobias, Michael E / Mohan, Avinash

    Journal of neurosurgery. Pediatrics

    2019  Volume 23, Issue 6, Page(s) 699–703

    Abstract: Hemophagocytic lymphohistiocytosis (HLH) is a rare disease process characterized by aberrant immune system activation and an exaggerated inflammatory response. Establishing the diagnosis may be challenging and is achieved by satisfying a number of ... ...

    Abstract Hemophagocytic lymphohistiocytosis (HLH) is a rare disease process characterized by aberrant immune system activation and an exaggerated inflammatory response. Establishing the diagnosis may be challenging and is achieved by satisfying a number of clinical criteria, in addition to demonstrating tissue hemophagocytosis. This syndrome is rapidly fatal if prompt diagnosis and treatment are not achieved. The authors present the case of a 17-year-old male patient with CNS HLH involving both the brain and spinal cord, highlighting the variable CNS manifestations in pediatric patients with HLH and the challenges that accompany establishing diagnosis.
    Language English
    Publishing date 2019-04-05
    Publishing country United States
    Document type Case Reports ; Journal Article
    ZDB-ID 2403985-8
    ISSN 1933-0715 ; 1933-0707
    ISSN (online) 1933-0715
    ISSN 1933-0707
    DOI 10.3171/2019.1.PEDS18591
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article: Evolution of Traumatic Parenchymal Intracranial Hematomas (ICHs): Comparison of Hematoma and Edema Components.

    Wilkes, Sean / McCormack, Erin / Kenney, Kimbra / Stephens, Brian / Passo, Ross / Harburg, Leah / Silverman, Erika / Moore, Carol / Bogoslovsky, Tanya / Pham, Dzung / Diaz-Arrastia, Ramon

    Frontiers in neurology

    2018  Volume 9, Page(s) 527

    Abstract: This study seeks to quantitatively assess evolution of traumatic ICHs over the first 24 h and investigate its relationship with functional outcome. Early expansion of traumatic intracranial hematoma (ICH) is common, but previous studies have focused on ... ...

    Abstract This study seeks to quantitatively assess evolution of traumatic ICHs over the first 24 h and investigate its relationship with functional outcome. Early expansion of traumatic intracranial hematoma (ICH) is common, but previous studies have focused on the high density (blood) component. Hemostatic therapies may increase the risk of peri-hematoma infarction and associated increased cytotoxic edema. Assessing the magnitude and evolution of ICH and edema represented by high and low density components on computerized tomography (CT) may be informative for designing therapies targeted at traumatic ICH. CT scans from participants in the COBRIT (Citicoline Brain Injury Trial) study were analyzed using MIPAV software. CT scans from patients with non-surgical intraparenchymal ICHs at presentation and approximately 24 h later (±12 h) were selected. Regions of high density and low density were quantitatively measured. The relationship between volumes of high and low density were compared to several outcome measures, including Glasgow Outcome Score-Extended (GOSE) and Disability Rating Score (DRS). Paired scans from 84 patients were analyzed. The median time between the first and second scan was 22.79 h (25%ile 20.11 h; 75%ile 27.49 h). Over this time frame, hematoma and edema volumes increased >50% in 34 (40%) and 46 (55%) respectively. The correlation between the two components was low (
    Language English
    Publishing date 2018-07-04
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2564214-5
    ISSN 1664-2295
    ISSN 1664-2295
    DOI 10.3389/fneur.2018.00527
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  4. Article ; Online: Imaging of Cerebrovascular Function in Chronic Traumatic Brain Injury.

    Amyot, Franck / Kenney, Kimbra / Moore, Carol / Haber, Margalit / Turtzo, L Christine / Shenouda, Christian / Silverman, Erika / Gong, Yunhua / Qu, Bao-Xi / Harburg, Leah / Lu, Hanzhang Y / Wassermann, Eric M / Diaz-Arrastia, Ramon

    Journal of neurotrauma

    2018  Volume 35, Issue 10, Page(s) 1116–1123

    Abstract: Traumatic cerebrovascular injury (TCVI) is a common pathologic mechanism of traumatic brain injury (TBI) and presents an attractive target for intervention. The aims of this study were to assess cerebral blood flow (CBF) and cerebrovascular reactivity ( ... ...

    Abstract Traumatic cerebrovascular injury (TCVI) is a common pathologic mechanism of traumatic brain injury (TBI) and presents an attractive target for intervention. The aims of this study were to assess cerebral blood flow (CBF) and cerebrovascular reactivity (CVR) using magnetic resonance imaging (MRI) to assess their value as biomarkers of TCVI in chronic TBI, characterize the spatial distribution of TCVI, and assess the relationships between each biomarker and neuropsychological and clinical assessments. Forty-two subjects (27 chronic TBI, 15 age- and gender-matched healthy volunteers) were studied cross-sectionally. CBF was measured by arterial spin labeling and CVR by assessing the MRI-blood oxygen level-dependent signal with hypercapnia challenge. A focused neuropsychological battery adapted from the TBI Common Data Elements and neurobehavioral symptom questionnaires were administered at the time of the imaging session. Chronic TBI subjects showed a significant reduction in mean global, gray matter (GM), and white matter (WM) CVR, compared with healthy volunteers (p < 0.001). Mean GM CVR had the greatest effect size (Cohen's d = 0.9). CVR maps in chronic TBI subjects showed patchy, multifocal CVR deficits. CBF discriminated poorly between TBI subjects and healthy volunteers and did not correlate with CVR. Mean global CVR correlated best with chronic neurobehavioral symptoms among TBI subjects. Global, GM, and WM CVR are reliable and potentially useful biomarkers of TCVI in the chronic stage after moderate-to-severe TBI. CBF is less useful as biomarker of TCVI. CVR correlates best with chronic TBI symptoms. CVR has potential as a predictive and pharmacodynamic biomarker for interventions targeting TCVI.
    MeSH term(s) Adult ; Brain Injuries, Traumatic/diagnostic imaging ; Brain Injuries, Traumatic/physiopathology ; Brain Injury, Chronic/diagnostic imaging ; Brain Injury, Chronic/physiopathology ; Cerebrovascular Circulation/physiology ; Cross-Sectional Studies ; Female ; Humans ; Image Interpretation, Computer-Assisted ; Magnetic Resonance Imaging/methods ; Male ; Middle Aged
    Language English
    Publishing date 2018-03-20
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Intramural
    ZDB-ID 645092-1
    ISSN 1557-9042 ; 0897-7151
    ISSN (online) 1557-9042
    ISSN 0897-7151
    DOI 10.1089/neu.2017.5114
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article: Phosphodiesterase-5 inhibition potentiates cerebrovascular reactivity in chronic traumatic brain injury.

    Kenney, Kimbra / Amyot, Franck / Moore, Carol / Haber, Margalit / Turtzo, L Christine / Shenouda, Christian / Silverman, Erika / Gong, Yunhua / Qu, Bao-Xi / Harburg, Leah / Wassermann, Eric M / Lu, Hanzhang / Diaz-Arrastia, Ramon

    Annals of clinical and translational neurology

    2018  Volume 5, Issue 4, Page(s) 418–428

    Abstract: Background: Traumatic cerebrovascular injury (TCVI), a common consequence of traumatic brain injury (TBI), presents an attractive therapeutic target. Because phosphodiesterase-5 (PDE5) inhibitors potentiate the action of nitric oxide (NO) produced by ... ...

    Abstract Background: Traumatic cerebrovascular injury (TCVI), a common consequence of traumatic brain injury (TBI), presents an attractive therapeutic target. Because phosphodiesterase-5 (PDE5) inhibitors potentiate the action of nitric oxide (NO) produced by endothelial cells, they are candidate therapies for TCVI. This study aims to: (1) measure cerebral blood flow (CBF), cerebrovascular reactivity (CVR), and change in CVR after a single dose of sildenafil (ΔCVR) in chronic TBI compared to uninjured controls; (2) examine the safety and tolerability of 8-week sildenafil administration in chronic symptomatic moderate/severe TBI patients; and as an exploratory aim, (3) assess the effect of an 8-week course of sildenafil on chronic TBI symptoms.
    Methods: Forty-six subjects (31 chronic TBI, 15 matched healthy volunteers) were enrolled. Baseline CBF and CVR before and after administration of sildenafil were measured. Symptomatic TBI subjects then completed an 8-week double-blind, placebo-controlled, crossover trial of sildenafil. A neuropsychological battery and neurobehavioral symptom questionnaires were administered at each study visit.
    Results: After a single dose of sildenafil, TBI subjects showed a significant increase in global CVR compared to healthy controls (
    Findings: Single-dose sildenafil improves regional CVR deficits in chronic TBI patients. CVR and ΔCVR are potential predictive and pharmacodynamic biomarkers of PDE5 inhibitor therapy for TCVI. Sildenafil is a potential therapy for TCVI.
    Language English
    Publishing date 2018-03-07
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2740696-9
    ISSN 2328-9503
    ISSN 2328-9503
    DOI 10.1002/acn3.541
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: Reliability of the NINDS common data elements cranial tomography (CT) rating variables for traumatic brain injury (TBI).

    Harburg, Leah / McCormack, Erin / Kenney, Kimbra / Moore, Carol / Yang, Kelly / Vos, Pieter / Jacobs, Bram / Madden, Christopher J / Diaz-Arrastia, Ramon / Bogoslovsky, Tanya

    Brain injury

    2017  Volume 31, Issue 2, Page(s) 174–184

    Abstract: Background: Non-contrast head computer tomography (CT) is widely used to evaluate eligibility of patients after acute traumatic brain injury (TBI) for clinical trials. The NINDS Common Data Elements (CDEs) TBI were developed to standardize collection of ...

    Abstract Background: Non-contrast head computer tomography (CT) is widely used to evaluate eligibility of patients after acute traumatic brain injury (TBI) for clinical trials. The NINDS Common Data Elements (CDEs) TBI were developed to standardize collection of CT variables. The objectives of this study were to train research assistants (RAs) to rate CDEs and then to evaluate their performance. The aim was to assess inter-rater reliability (IRR) of CDEs between trained RAs and a neurologist and to evaluate applicability of CDEs in acute and sub-acute TBI to test the feasibility of using CDE CT ratings in future trials and ultimately in clinical practice. The second aim was to confirm that the ratings of CDEs reflect pathophysiological events after TBI.
    Methods and results: First, a manual was developed for application of the CDEs, which was used to rate brain CTs (n = 100). An excellent agreement was found in combined kappas between RAs on admission and on 24-hour follow-up CTs (Iota = 0.803 and 0.787, respectively). Good IRR (kappa > 0.61) was shown for six CDEs on admissions and for seven CDEs on follow-up CTs. Low IRR (kappa < 0.4) was determined for five CDEs on admission and for four CDEs on follow-up CT. Combined IRR of each assistant with the neurologist were good on admission (Iota = 0.613 and 0.787) and excellent on follow-up CT (Iota = 0.906 and 0.977). Second, Principal Component Analysis (PCA) was applied to cluster the rated CDEs (n = 255) and five major components were found that explain 53% of the variance.
    Conclusions: CT CDEs are useful in clinical studies of TBI. Trained RAs can reliably collect variables. PCA identifies CDE clusters with clinical and biologic plausibility.
    Abbreviations: RA, research assistant; CT, Cranial Tomography; TBI, Traumatic Brain Injury; CDE, Common Data Elements; IRR, inter-rater reliability; PCA, Principal Component Analysis; GCS, Glasgow Coma Scale; R, rater; CI, confidence interval; CCC, Concordance correlation coefficient; IVH, Intraventricular haemorrhage; DCA, Discriminant Component analysis; SAH, Subarachnoid Haemorrhage.
    Language English
    Publishing date 2017
    Publishing country England
    Document type Journal Article
    ZDB-ID 639115-1
    ISSN 1362-301X ; 0269-9052
    ISSN (online) 1362-301X
    ISSN 0269-9052
    DOI 10.1080/02699052.2016.1225989
    Database MEDical Literature Analysis and Retrieval System OnLINE

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