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Hailey-Hailey disease

M Selim Asmer, Julia Kirkham, Hailey Newton, Zahinoor Ismail, Heba Elbayoumi, Roxanne H Leung, Dallas P Seitz
OBJECTIVE: Little is known about the overall prevalence of major depressive disorder (MDD) in persons with dementia (ie, "depression in dementia": DpD). The aim of this systematic review and meta-analysis was to determine the prevalence and factors associated with DpD among older adults (age range 58.7-87.8 years). The protocol was registered in the PROSPERO registry (2015:CRD42015020681). DATA SOURCES: We searched the following electronic databases: MEDLINE (1946-February 2017), Embase (1980-2017 week 5), and PsycINFO (1967-February 2017) using medical subject headings and free-text search terms for studies in the English language...
July 31, 2018: Journal of Clinical Psychiatry
Susan E Warrington, Hailey K Collier, Adam S Himebauch, Heather A Wolfe
OBJECTIVES: To evaluate if institutionally established calculations for transitioning continuous IV midazolam to enteral benzodiazepines maintain Withdrawal Assessment Tool-Version 1 scores equal to or less than preconversion values. DESIGN: Retrospective cohort study evaluating the effectiveness and safety of benzodiazepine conversion calculations embedded within an institution-specific clinical pathway for sedation and weaning of mechanically ventilated pediatric patients...
August 3, 2018: Pediatric Critical Care Medicine
Yuhui Wang, Junyi Tan, Zhiming Wu, Kyle VandenLangenberg, Todd C Wehner, Changlong Wen, Xiangyang Zheng, Ken Owens, Alyson Thornton, Hailey H Bang, Eric Hoeft, Peter A G Kraan, Jos Suelmann, Junsong Pan, Yiqun Weng
The Gy14 cucumber (Cucumis sativus) is resistant to oomyceteous downy mildew (DM), bacterial angular leaf spot (ALS) and fungal anthracnose (AR) pathogens, but the underlying molecular mechanisms are unknown. Quantitative trait locus (QTL) mapping for the disease resistances in Gy14 and further map-based cloning identified a candidate gene for the resistant loci, which was validated and functionally characterized by spatial-temporal gene expression profiling, allelic diversity and phylogenetic analysis, as well as local association studies...
July 19, 2018: New Phytologist
Graziella Ficociello, Azzurra Zonfrilli, Samantha Cialfi, Claudio Talora, Daniela Uccelletti
The term orthodisease defines human disorders in which the pathogenic gene has orthologs in model organism genomes. Yeasts have been instrumental for gaining insights into the molecular basis of many human disorders, particularly those resulting from impaired cellular metabolism. We and others have used yeasts as a model system to study the molecular basis of Hailey-Hailey disease (HHD), a human blistering skin disorder caused by haploinsufficiency of the gene ATP2C1 the orthologous of the yeast gene PMR1 ...
June 20, 2018: International Journal of Molecular Sciences
Kyle T Amber, Manuel Valdebran, Sergei A Grando
Pemphigus vulgaris (PV) is a potentially life-threatening mucocutaneous autoimmune blistering disease. Patients develop non-healing erosions and blisters due to cell-cell detachment of keratinocytes (acantholysis), with subsequent suprabasal intraepidermal splitting. Identified almost 30 years ago, desmoglein-3 (Dsg3), a Ca2+ -dependent cell adhesion molecule belonging to the cadherin family, has been considered the "primary" autoantigen in PV. Proteomic studies have identified numerous autoantibodies in patients with PV that have known roles in the physiology and cell adhesion of keratinocytes...
2018: Frontiers in Immunology
Nicole Leung, Adela R Cardones, Nicole Larrier
No abstract text is available yet for this article.
March 2, 2018: Practical Radiation Oncology
Kaien Gu, Shane Silver
Hailey-Hailey disease, or benign familial pemphigus, is a rare blistering disease originally described in 1939. The disease is due to an autosomal dominant mutation in the ATP2C1 gene on chromosome 3, which encodes for an adenosine triphosphate-dependent calcium pump in the Golgi apparatus whose function is to maintain intercellular calcium homeostasis. Common treatments for Hailey-Hailey disease involve calcineurin inhibitors, topical corticosteroids, topical or systemic antibiotics, topical antifungals, ablative lasers, or botulin toxin...
May 2018: Journal of Cutaneous Medicine and Surgery
Elisabeth P Dellon, Sarah W Helms, Claire E Hailey, Rosemary Shay, Scott D Carney, Howard Joel Schmidt, David E Brown, Mary G Prieur
BACKGROUND: Individuals with cystic fibrosis (CF) face the challenges of managing a chronic, progressive disease. While palliative care is a standard of care in serious illnesses, there are no guidelines for its incorporation into CF care. Patients with CF, caregivers, and CF care providers may lack knowledge about palliative care and perceive barriers to integrated care. OBJECTIVES: To: 1) explore knowledge and perceptions of palliative care among patients with CF, caregivers, and CF care providers; 2) solicit opinions about incorporating palliative care into routine CF care; and 3) solicit recommendations for CF-specific palliative care education for patients and caregivers...
June 3, 2018: Pediatric Pulmonology
Hailey R Banack, Sam Harper, Jay S Kaufman
BACKGROUND: In cardiovascular research, pre-hospital mortality represents an important potential source of selection bias. Inverse probability of censoring weights are a method to account for this source of bias. The objective of this article is to examine and correct for the influence of selection bias due to pre-hospital mortality on the relationship between cardiovascular risk factors and all-cause mortality after an acute cardiac event. METHODS: The relationship between the number of cardiovascular disease (CVD) risk factors (0-5; smoking status, diabetes, hypertension, dyslipidemia, and obesity) and all-cause mortality was examined using data from the Atherosclerosis Risk in Communities (ARIC) study...
June 2018: Canadian Journal of Cardiology
Debbie S Gipson, David T Selewski, Susan F Massengill, Mary Margaret Modes, Hailey Desmond, Lauren Lee, Elaine Kamil, Matthew R Elliott, Sharon G Adler, Gia Oh, Richard A Lafayette, Patrick E Gipson, Aditi Sinha, Arvind Bagga, Anne Pesenson, Cheryl Courtlandt, Cathie Spino, Richard Eikstadt, Renée Pitter, Samara Attalla, Anne Waldo, Richard Winneker, Noelle E Carlozzi, Jonathan P Troost, Irving Smokler, Mark Stone
Introduction: NephCure Accelerating Cures Institute (NACI) is a collaborative organization sponsored by NephCure Kidney International and the University of Michigan. The Institute is composed of 7 cores designed to improve treatment options and outcomes for patients with glomerular disease: Clinical Trials Network, Data Warehouse, Patient-Reported Outcomes (PRO) and Endpoints Consortium, Clinical Trials Consulting Team, Quality Initiatives, Education and Engagement, and Data Coordinating Center...
March 2018: KI Reports
M Kono, M Niizawa, T Takeichi, Y Muro, M Akiyama
No abstract text is available yet for this article.
April 28, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
Payal Chauhan, Dilip Meena, Neirita Hazarika
No abstract text is available yet for this article.
March 2018: Indian Dermatology Online Journal
Rebecca A Morgan, Julie A Karl, Hailey E Bussan, Katelyn E Heimbruch, David H O'Connor, Dawn M Dudley
Baboons are valuable models for complex human diseases due to their genetic and physiologic similarities to humans. Deep sequencing methods to characterize full-length major histocompatibility complex (MHC) class I (MHC-I) alleles in different nonhuman primate populations were used to identify novel MHC-I alleles in baboons. We combined data from Illumina MiSeq sequencing and Roche/454 sequencing to characterize novel full-length MHC-I transcripts in a cohort of olive and hybrid olive/yellow baboons from the Southwest National Primate Research Center (SNPRC)...
July 2018: Immunogenetics
Susanne Smaardijk, Jialin Chen, Sara Kerselaers, Thomas Voets, Jan Eggermont, Peter Vangheluwe
The Secretory Pathway Ca2+ ATPases SPCA1 and SPCA2 transport Ca2+ and Mn2+ into the Golgi and Secretory Pathway. SPCA2 mediates store-independent Ca2+ entry (SICE) via STIM1-independent activation of Orai1, inducing constitutive Ca2+ influx in mammary epithelial cells during lactation. Here, we show that like SPCA2, also the overexpression of the ubiquitous SPCA1 induces cytosolic Ca2+ influx, which is abolished by Orai1 knockdown and occurs independently of STIM1. This process elevates the Ca2+ concentration in the cytosol and in the non-endoplasmic reticulum (ER) stores, pointing to a functional coupling between Orai1 and SPCA1...
June 2018: Biochimica et Biophysica Acta
Devesh Vashishtha, William Sieber, Brittany Hailey, Kristen Guirguis, Alexander Gershunov, Wael K Al-Delaimy
Introduction: The purpose of this study was to determine whether heat waves are associated with increased frequency of clinic visits for ICD-9 codes of illnesses traditionally associated with heat waves. Methods: During 4 years of family medicine clinic data between 2012 and 2016, we identified six heat wave events in San Diego County. For each heat wave event, we selected a control period in the same season that was twice as long. Scheduling a visit on a heat wave day (versus a non-heat wave day) was the primary predictor, and receiving a primary ICD-9 disease code related to heat waves was the outcome...
March 10, 2018: Family Practice
Jennifer Marie Coughlin, Stephanie Slania, Yong Du, Hailey B Rosenthal, Wojciech G Lesniak, Il Minn, Gwenn S Smith, Robert F Dannals, Hiroto Kuwabara, Dean F Wong, Yuchuan Wang, Andrew G Horti, Martin G Pomper
Reduced density of the α4β2 nicotinic acetylcholine receptor (α4β2-nAChR) in the cortex and hippocampus of human brain has been reported in aging and patients with neurodegenerative disease. This study assessed the pharmacokinetic behavior of 18 F-XTRA, a new radiotracer for in vivo positron emission tomography (PET) imaging of the α4β2-nAChR, particularly in extrathalamic regions of interest (ROIs) where the α4β2-nAChR is less densely expressed than in thalamus. 18 F-XTRA was also used to evaluate the α4β2-nAChR in hippocampus in human aging...
March 1, 2018: Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine
Dallas P Seitz, Calvin Ch Chan, Hailey T Newton, Sudeep S Gill, Nathan Herrmann, Nadja Smailagic, Vasilis Nikolaou, Bruce A Fage
BACKGROUND: Alzheimer's disease and other forms of dementia are becoming increasingly common with the aging of most populations. The majority of individuals with dementia will first present for care and assessment in primary care settings. There is a need for brief dementia screening instruments that can accurately diagnose dementia in primary care settings. The Mini-Cog is a brief, cognitive screening test that is frequently used to evaluate cognition in older adults in various settings...
February 22, 2018: Cochrane Database of Systematic Reviews
Megan K Mills, Bryn Putbrese, Hailey Allen, Sarah E Stilwill
Lymphangiomatosis is an uncommon disease process characterized by multisystem lymphatic malformations that can involve numerous body systems, including organs, muscles, soft tissues, and bones. Involvement of the nervous system is rare and has even been previously described as a site of sparing. We present a case of a 24-year-old female with known lymphangiomatosis, presenting with acute onset of lower extremity paresthesias, weakness, and new urinary retention. MRI of the pelvis revealed lymphangiomatosis of the sacral plexus, which has not been previously reported...
September 2018: Skeletal Radiology
M C Abaca, L Flores, V Parra
No abstract text is available yet for this article.
February 18, 2018: Actas Dermo-sifiliográficas
Hailey Pinz, Louise C Pyle, Dong Li, Kosuke Izumi, Cara Skraban, Jennifer Tarpinian, Stephen R Braddock, Aida Telegrafi, Kristin G Monaghan, Elaine Zackai, Elizabeth J Bhoj
Myelin Regulatory Factor (MYRF) is a transcription factor that has previously been associated with the control of the expression of myelin-related genes. However, it is highly expressed in human tissues and mouse embryonic tissues outside the nervous system such as the stomach, lung, and small intestine. It has not previously been reported as a cause of any Mendelian disease. We report here two males with Scimitar syndrome [MIM 106700], and other features including penoscrotal hypospadias, cryptorchidism, pulmonary hypoplasia, tracheal anomalies, congenital diaphragmatic hernia, cleft spleen, thymic involution, and thyroid fibrosis...
April 2018: American Journal of Medical Genetics. Part A
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