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  1. Article ; Online: Chronic active Epstein–Barr virus and hydroa vacciniforme‐like lymphoproliferative disorder in a pediatric patient complicated by fatal ruptured cerebral artery aneurysm

    Troy Yi / Jeffrey Steinberg / Scott Olson / Howaida El‐Said / Jun Mo / Eric Anderson / Nicholas Gloude / Deborah Schiff

    Clinical Case Reports, Vol 11, Iss 6, Pp n/a-n/a (2023)

    2023  

    Abstract: Key Clinical Message Hydroa vacciniforme‐like lymphoproliferative disorder (HV‐LPD) is a rare cutaneous form of chronic active Epstein–Barr virus (CAEBV) that presents with vesicular lesions induced by sun‐exposure. Arterial aneurysm is a rare but ... ...

    Abstract Key Clinical Message Hydroa vacciniforme‐like lymphoproliferative disorder (HV‐LPD) is a rare cutaneous form of chronic active Epstein–Barr virus (CAEBV) that presents with vesicular lesions induced by sun‐exposure. Arterial aneurysm is a rare but potentially fatal complication of CAEBV and HV‐LPD.
    Keywords arterial aneurysm ; CAEBV ; HV‐LPD ; Medicine ; R ; Medicine (General) ; R5-920
    Language English
    Publishing date 2023-06-01T00:00:00Z
    Publisher Wiley
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  2. Article ; Online: An End-To-End Pipeline for Fully Automatic Morphological Quantification of Mouse Brain Structures From MRI Imagery

    Shahinur Alam / Tae-Yeon Eom / Jeffrey Steinberg / David Ackerman / J. Eric Schmitt / Walter J. Akers / Stanislav S. Zakharenko / Khaled Khairy

    Frontiers in Bioinformatics, Vol

    2022  Volume 2

    Abstract: Segmentation of mouse brain magnetic resonance images (MRI) based on anatomical and/or functional features is an important step towards morphogenetic brain structure characterization of murine models in neurobiological studies. State-of-the-art image ... ...

    Abstract Segmentation of mouse brain magnetic resonance images (MRI) based on anatomical and/or functional features is an important step towards morphogenetic brain structure characterization of murine models in neurobiological studies. State-of-the-art image segmentation methods register image volumes to standard presegmented templates or well-characterized highly detailed image atlases. Performance of these methods depends critically on the quality of skull-stripping, which is the digital removal of tissue signal exterior to the brain. This is, however, tedious to do manually and challenging to automate. Registration-based segmentation, in addition, performs poorly on small structures, low resolution images, weak signals, or faint boundaries, intrinsic to in vivo MRI scans. To address these issues, we developed an automated end-to-end pipeline called DeepBrainIPP (deep learning-based brain image processing pipeline) for 1) isolating brain volumes by stripping skull and tissue from T2w MRI images using an improved deep learning-based skull-stripping and data augmentation strategy, which enables segmentation of large brain regions by atlas or template registration, and 2) address segmentation of small brain structures, such as the paraflocculus, a small lobule of the cerebellum, for which DeepBrainIPP performs direct segmentation with a dedicated model, producing results superior to the skull-stripping/atlas-registration paradigm. We demonstrate our approach on data from both in vivo and ex vivo samples, using an in-house dataset of 172 images, expanded to 4,040 samples through data augmentation. Our skull stripping model produced an average Dice score of 0.96 and residual volume of 2.18%. This facilitated automatic registration of the skull-stripped brain to an atlas yielding an average cross-correlation of 0.98. For small brain structures, direct segmentation yielded an average Dice score of 0.89 and 5.32% residual volume error, well below the tolerance threshold for phenotype detection. Full pipeline execution is ...
    Keywords MRI ; deep learning ; image segmentation ; image registration ; brain atlas ; data augmentation ; Computer applications to medicine. Medical informatics ; R858-859.7
    Subject code 004
    Language English
    Publishing date 2022-06-01T00:00:00Z
    Publisher Frontiers Media S.A.
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  3. Article ; Online: Proton magnetic resonance spectroscopy detects cerebral metabolic derangement in a mouse model of brain coenzyme a deficiency

    Yanan Li / Jeffrey Steinberg / Zane Coleman / Shubo Wang / Chitra Subramanian / Yimei Li / Zoltan Patay / Walter Akers / Charles O. Rock / Suzanne Jackowski / Puneet Bagga

    Journal of Translational Medicine, Vol 20, Iss 1, Pp 1-

    2022  Volume 7

    Abstract: Abstract Background Pantothenate kinase (PANK) is the first and rate-controlling enzymatic step in the only pathway for cellular coenzyme A (CoA) biosynthesis. PANK-associated neurodegeneration (PKAN), formerly known as Hallervorden–Spatz disease, is a ... ...

    Abstract Abstract Background Pantothenate kinase (PANK) is the first and rate-controlling enzymatic step in the only pathway for cellular coenzyme A (CoA) biosynthesis. PANK-associated neurodegeneration (PKAN), formerly known as Hallervorden–Spatz disease, is a rare, life-threatening neurologic disorder that affects the CNS and arises from mutations in the human PANK2 gene. Pantazines, a class of small molecules containing the pantazine moiety, yield promising therapeutic effects in an animal model of brain CoA deficiency. A reliable technique to identify the neurometabolic effects of PANK dysfunction and to monitor therapeutic responses is needed. Methods We applied 1H magnetic resonance spectroscopy as a noninvasive technique to evaluate the therapeutic effects of the newly developed Pantazine BBP-671. Results 1H MRS reliably quantified changes in cerebral metabolites, including glutamate/glutamine, lactate, and N-acetyl aspartate in a neuronal Pank1 and Pank2 double-knockout (SynCre + Pank1,2 dKO) mouse model of brain CoA deficiency. The neuronal SynCre + Pank1,2 dKO mice had distinct decreases in Glx/tCr, NAA/tCr, and lactate/tCr ratios compared to the wildtype matched control mice that increased in response to BBP-671 treatment. Conclusions BBP-671 treatment completely restored glutamate/glutamine levels in the brains of the mouse model, suggesting that these metabolites are promising clinically translatable biomarkers for future therapeutic trials.
    Keywords Pantothenate kinase ; Coenzyme A ; Neurodegeneration ; Pantothenate kinase-associated neurodegeneration ; 1H magnetic resonance spectroscopy ; Metabolites ; Medicine ; R
    Subject code 616
    Language English
    Publishing date 2022-02-01T00:00:00Z
    Publisher BMC
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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    Inter-library loan at ZB MED

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