363 research outputs found
Lincoln and the Constitution: Presentation by Dr. Timothy S. Huebner
No full textDr. Huebner speaks on the way that Lincoln struggled with, and found ways to surmount, three major constitutional issues – the legitimacy of secession, the constitutionality of the suspension of the writ of habeas corpus in wartime, and the legality of emancipation – and how his struggles demonstrated his belief that a vigorous Union would promote and protect the ideals embodied in the Declaration of Independence. Includes text document with selected, relevant quotations from Lincoln. Dr. Huebner is the author of several books on American law, courts, and the Constitution, including The Taney Court: Justices, Rulings and Legacy, and is co-editor and contributor to Major Problems in American Constitutional History
Lincoln and the Constitution: Presentation by Dr. Timothy S. Huebner
No full textDr. Huebner speaks on the way that Lincoln struggled with, and found ways to surmount, three major constitutional issues – the legitimacy of secession, the constitutionality of the suspension of the writ of habeas corpus in wartime, and the legality of emancipation – and how his struggles demonstrated his belief that a vigorous Union would promote and protect the ideals embodied in the Declaration of Independence. Includes text document with selected, relevant quotations from Lincoln. Dr. Huebner is the author of several books on American law, courts, and the Constitution, including The Taney Court: Justices, Rulings and Legacy, and is co-editor and contributor to Major Problems in American Constitutional History.https://digitalcommons.memphis.edu/speccoll-exhibit-lincoln2012-doc1/1000/thumbnail.jp
Managing MPLS/VPNs with policies
No full textThis paper proposes an approach for the dynamic management of MPLS-based VPNs, MPLS and VPNs significantly contribute to achieve QoS within networks but there remain dynamic management problems associated with their use. We believe that these problems can be solved by using a policy model; such an approach also enables subscribers to keep control of their VPNs and share information with service providers. We used a PCIM-enable network model to account for the peculiarities of the two technologies and combined the resulting schema with COPS and the necessary policy tools. The resulting framework was then tested on a MPLS network. The results show that, with some limitations, the approach does provide the expected functionality
Axonal neuropathy with unusual pattern of amyotrophy and alacrima associated with a novel AAAS mutation p.Leu430Phe
No full textThe triple A syndrome is caused by autosomal recessively inherited mutations in the AAAS gene and is characterized by achalasia, alacrima and adrenal insufficiency as well as progressive neurological impairment. We report on a 14-year-old girl with slowly progressive axonal motor neuropathy with conspicuous muscle wasting of hypothenars and calves as well as alacrima. The mutation analysis of the AAAS gene revealed a compound heterozygous mutation: a c.251G>A mutation in exon 2 that had been reported previously, and a novel c.1288C>T mutation in exon 14. At the transcriptional level, the c.251G>A transition results in an aberrant splicing and decay of this RNA strand so that the particular clinical picture results from the novel c. 1288C>T, (p.Leu430Phe, L430F) mutation in a hemizygous form. With transfection experiments, we demonstrate that GFP-ALADIN(L430F) correctly localizes to nuclear pore complexes. Therefore, we conclude that this point mutation impairs ALADIN function at the nuclear pore
associated with previously unreported features of Cohen syndrome
No full textCohen syndrome (CS) is a rare autosomal recessive disorder associated with mutations in the vacuolar protein sorting 13 homolog B (VPS13B; formerly COH1) gene. The core clinical phenotype comprises a characteristic facial gestalt, marked developmental delay, and myopia. Additional, nonobligatory features include obesity, microcephaly, short stature, muscular hypotonia, scoliosis, narrow hands and feet, progressive retinopathy, as well as neutropenia. Here we report a novel homozygous nonsense mutation in the VPS13B gene and previously undescribed clinical features in a 19-year-old woman with developmental delay, intellectual disability, and a particular facial appearance. The patient showed several features consistent with CS. In addition, the parents observed congenital alacrima and anhidrosis persisting until onset of puberty. The diagnosis was not established based on the clinical phenotype. We performed whole-genome sequencing and identified a novel homozygous nonsense mutation c.62T>G (NM_152564.4), p.(Leu21 ) in the VPS13B gene. Our findings extended the previously reported phenotype of CS. We conclude that transient, prepubertal alacrima and anhidrosis are part of the phenotypic spectrum of CS associated with a novel homozygous nonsense mutation in the VPS13B gene.Deutsche Forschungsgemeinschaft (DFG) http://dx.doi.org/10.13039/50110000165
Endocrine correlates in a case of Allgrove Syndrome with diagnosis in adulthood
No full textLate-onset triple A syndrome caused by a novel homozygous missense mutation in the AAAS gene (A167V in exon 6) was diagnosed at least 17 years after symptom onse
Study cites teens' mixed emotions about parent deployment
No full textUnderstanding how a parent's deployment affects the emotional and behavioral development of their teenage children is the focal point of research conducted by Angela Huebner, associate professor of human development at Virginia Tech, National Capital Region, and Jay A. Mancini, professor of human development, Blacksburg campus
Dataset for Urinary incontinence in female weightlifters
No full textOVERVIEW
Title of Dataset: Urinary incontinence in female weightlifters
Reference: doi: 10.1371/journal.pone.0278376. PMID: 36449558; PMCID: PMC9710785.
Author Information
Name: Marianne Huebner
Institution: Michigan State University
Address: East Lansing, MI 48824
Period of data collection: 27 April – 20 May 2022
Geographic region of data collection: Online survey in USA with participants from 29 countries in IWF regions Africa, Asia, Europe, Oceania, PanAmerican
LIST OF FILES
Dataset: wlisi_zenodo.xlsx
Data dictionary: wlisi_meta.xlsx
METHODOLOGICAL INFORMATION
Description of methods used for collection/generation of data: The survey was distributed by the Master Committee of the International Weightlifting Federation (IWF) to the National Master Chairs. They then used email or social media to communicate the study to the women weightlifters. The Survey was available in four languages (English, German, French, Spanish), translated and tested by native speakers. In addition, the survey was advertised in weightlifting interest groups via Facebook and Instagram. The survey was administered online via Qualtrics (Provo, UT, USA).
Methods for processing the data: Data were downloaded from Qualtrics (Provo, UT, USA) to Excel and then pre-processed in the statistical software R v. 4.0.3. (https://www.r-project.org)
Variable formats (numeric, character) were checked and transformed, as appropriate.
Data quality checks: Exclusion criteria were younger than 30 years (n=1), missing age (n=1), currently pregnant (n=3). To account for the possibility of male participants missing responses to age of menstruation or prior pregnancies (n=15), were also excluded. Since the focus was on competitive weightlifters, missing response to age of first competition (n=34) or no snatch or clean and jerk in the last 6 months (n=14) were also exclusion criteria. This resulted in an analysis data set of 824 women. Univariate distributions were evaluated numerically and graphically.
DATA-SPECIFIC INFORMATION
Number of variables: 27
Number of cases/rows: 824
Variable List: wlisi_meta.xlsx
Missing data codes: N
Late-Onset Triple A Syndrome: A Risk of Overlooked or Delayed Diagnosis and Management
No full textBACKGROUND/AIMS: A 33-year-old man was referred for the first time to the Division of Neurology because of the presence and progression of neurological symptoms. Dysphagia, weakness, reduced tear production, and nasal speech were present. In order to point the attention of late-onset triple A syndrome we describe this case and review the literature. METHODS: Hormonal and biochemical evaluation, Schirmer test, tilt test and genetic testing for AAAS gene mutations. RESULTS: Late-onset triple A syndrome caused by a novel homozygous missense mutation in the AAAS gene (A167V in exon 6) was diagnosed at least 17 years after symptom onset. CONCLUSIONS: The association between typical signs and symptoms of triple A syndrome should suggest the diagnosis even if they manifest in adulthood. The diagnosis should be confirmed by Schirmer test, endocrine testing (both basal and dynamic), genetic analysis, and detailed gastroenterological and neurological evaluations. Awareness of the possible late onset of the disease and of diagnosis in adulthood is still poor among clinicians, the acquaintance with the disease is more common among pediatricians. The importance of an adequate multidisciplinary clinical approach, dynamic testing for early diagnosis of adrenal insufficiency and periodical reassessment of adrenal function are emphasized
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