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Hailey hailey disease

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https://www.readbyqxmd.com/read/29154712/prognosis-for-dogs-with-stage-iii-osteosarcoma-following-treatment-with-amputation-and-chemotherapy-with-and-without-metastasectomy
#1
Hailey Turner, Bernard Séguin, Deanna R Worley, Nicole P Ehrhart, Mary H Lafferty, Stephen J Withrow, Laura E Selmic
OBJECTIVE To determine survival times of selected dogs with metastatic (stage III) osteosarcoma, whether disease-free interval (DFI) was associated with survival time after diagnosis of stage III disease (ie, stage III survival time), and whether a survival benefit of metastasectomy existed. DESIGN Retrospective case series with nested cohort study. ANIMALS 194 client-owned dogs treated for histologically confirmed appendicular osteosarcoma from 1997 through 2009. PROCEDURES Dogs were included if they had stage I or II osteosarcoma at the time of initial evaluation, had amputation of the affected appendage and ≥ 1 dose of chemotherapy afterward, and developed metastasis within the follow-up period or prior to death...
December 1, 2017: Journal of the American Veterinary Medical Association
https://www.readbyqxmd.com/read/29139371/hailey-hailey-disease-presenting-as-lichenoid-plaques-on-the-thigh
#2
Anupama Ghosh, Anupam Das, Piyush Kumar, Swapan Sardar
A 40-year-old woman presented with multiple itchy violaceous papules and plaques on the medial aspects of both thighs. This was accompanied by a burning sensation on friction and increased itching that interfered with her daily activities, particularly in the summer. The family history was noncontributory. Cutaneous examination showed multiple flat-topped hyperkeratotic violaceous papules and plaques, appearing in linear fashion on the medial aspects of the thighs bilaterally (Figure 1). Histopathologic examination showed acanthosis, widespread suprabasal acantholysis resembling a "dilapidated brick wall," without any evidence of dyskeratosis...
2017: Skinmed
https://www.readbyqxmd.com/read/29110168/caregiver-quality-of-life-with-tyrosinemia-type-1
#3
Hailey Campbell, Rani H Singh, Eric Hall, Nadia Ali
Tyrosinemia type I (HT1) is an inborn error of metabolism (IEM). Current management guidelines include lifelong specialized diet and use of the orphan drug, nitisinone. This study explores the quality of life (QOL) of caregivers of children with HT1. Caregivers for 26 children with HT1 completed a questionnaire (TYR-QOL) adapted to this patient population from an existing validated QOL questionnaire (PKU-QOL). Responses were analyzed via domain scores, based on predetermined scoring guidelines. Results suggest HT1 has a moderate overall impact on caregiver QOL, with emotional aspects of the disease having the greatest impact...
November 6, 2017: Journal of Genetic Counseling
https://www.readbyqxmd.com/read/29104283/identification-of-2-novel-mutations-in-atp2c1-gene-in-hailey-hailey-disease-and-a-literature-review-of-variations-in-a-chinese-han-population
#4
Kejia Xu, Bingjun Shi, Qingchun Diao, Xue Jiang, Yujuan Xiao
BACKGROUND Hailey-Hailey disease (HHD) is a rare autosomal dominant skin condition. The ATP2C1 gene was identified as the defective gene in HHD. To date, 166 pathogenic mutations in ATP2C1 have been observed worldwide. The aim of this study was to identify variations in HHD and summarize the features of the mutations identified in China. MATERIAL AND METHODS We examined 2 familial and 2 sporadic cases of HHD. Genomic DNA polymerase chain reaction and direct sequencing of the ATP2C1 were performed from HHD patients, unaffected family members, and 200 healthy individuals...
November 6, 2017: Medical Science Monitor Basic Research
https://www.readbyqxmd.com/read/29078901/hispanic-ethnicity-and-complication-profile-following-laparoscopic-and-open-cholecystectomy
#5
Hailey C Budnick, Isaac L Lee, Stacey A Milan
BACKGROUND: Hispanic ethnicity is associated with increased incidence of gallbladder disease. Additionally, ethnicity has been shown to be an outcome determinant in several conditions and procedures but has never been studied as a potential determinant of morbidity or mortality after laparoscopic or open cholecystectomy. METHODS: Using the American College of Surgeons National Surgical Quality Improvement Program database, patients who underwent cholecystectomy and related procedures were studied in Hispanic and non-Hispanic cohorts...
November 2017: Journal of Surgical Research
https://www.readbyqxmd.com/read/29044476/treatment-of-hailey-hailey-disease-with-botulinum-toxin
#6
Olivia A Charlton, Thomas J Stewart, Robert H Rosen
No abstract text is available yet for this article.
October 18, 2017: Australasian Journal of Dermatology
https://www.readbyqxmd.com/read/29020610/neonatal-c57bl-6j-and-parkin-mice-respond-differently-following-developmental-manganese-exposure-result-of-a-high-dose-pilot-study
#7
Melanie L Foster, Thomas B Bartnikas, Hailey C Maresca-Fichter, Courtney Mercadante, Miriam Dash, Chelsea Miller, David C Dorman
It has been suggested that childhood exposure to neurotoxicants may increase the risk of Parkinson's disease (PD) or other neurodegenerative disease in adults. Some recessive forms of PD have been linked to loss-of-function mutations in the Park2 gene that encodes for parkin. The purpose of this pilot study was to evaluate whether responses to neonatal manganese (Mn) exposure differ in mice with a Park2 gene defect (parkin mice) when compared with a wildtype strain (C57BL/6J). Neonatal parkin and C57BL/6J littermates were randomly assigned to 0, 11, or 25mg Mn/kg-day dose groups with oral exposures occurring from postnatal day (PND) 1 through PND 28...
October 8, 2017: Neurotoxicology
https://www.readbyqxmd.com/read/28993233/the-distribution-of-the-alpha7-nicotinic-acetylcholine-receptor-in-healthy-aging-an-in-vivo-positron-emission-tomography-study-with-18-f-asem
#8
Jennifer M Coughlin, Yong Du, Hailey B Rosenthal, Stephanie Slania, Soo Min Koo, Andrew Park, Ghedem Solomon, Melin Vranesic, Inga Antonsdottir, Caroline L Speck, Kelly Rootes-Murdy, Alexandria Lerner, Steven P Rowe, Yuchuan Wang, Wojciech G Lesniak, Il Minn, Arnold Bakker, Gwenn S Smith, Robert F Dannals, Hiroto Kuwabara, Andrew Horti, Dean F Wong, Martin G Pomper
Altered function of the alpha7 nicotinic acetylcholine receptor (α7-nAChR) is implicated in several neuropsychiatric diseases. Nevertheless, studies of the human cerebral α7-nAChR even in healthy aging are limited in number and to postmortem tissue. METHODS: The distribution of the cerebral α7-nAChR was estimated in nine brain regions in 25 healthy volunteers (ages 21-86 years; median 57 years, interquartile range 52 years) using [(18)F]ASEM with positron emission tomography (PET) imaging...
October 7, 2017: NeuroImage
https://www.readbyqxmd.com/read/28991360/low-dose-naltrexone-a-novel-treatment-for-hailey-hailey-disease
#9
V Campbell, C McGrath, A Corry
Hailey-Hailey disease (chronic benign familial pemphigus) is a rare inherited dermatosis typically characterised by erosions at intertriginous sites preceded by minor trauma or stress. We report a case of treatment resistant Hailey-Hailey disease, having failed topical and oral steroids, prophylactic acyclovir and doxycycline, and systemic therapies including dapsone, acitretin and ciclosporin. Low dose naltrexone (LDN) 4.5mg one daily was commenced following an incidental benefit in this patient's similarly affected sister...
October 9, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28970723/high-yield-reproducible-rat-model-recapitulating-human-barrett-s-carcinogenesis
#10
Daisuke Matsui, Ashten N Omstead, Juliann E Kosovec, Yoshihiro Komatsu, Emily J Lloyd, Hailey Raphael, Ronan J Kelly, Ali H Zaidi, Blair A Jobe
AIM: To efficiently replicate the biology and pathogenesis of human esophageal adenocarcinoma (EAC) using the modified Levrat model of end-to-side esophagojejunostomy. METHODS: End-to-side esophagojejunostomy was performed on rats to induce gastroduodenoesophageal reflux to develop EAC. Animals were randomly selected and serially euthanized at 10 (n = 6), 17 (n = 8), 24 (n = 9), 31 (n = 6), 38 (n = 6), and 40 (n = 6) wk postoperatively. The esophagi were harvested for downstream histopathology and gene expression...
September 7, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28966528/a-case-of-hailey-hailey-disease-with-a-novel-nonsense-mutation-in-the-atp2c1-gene
#11
Hazuki Yasuda, Nobuo Kanazawa, Mitsuhiro Matsuda, Takahiro Hamada, Minao Furumura, Takashi Hashimoto, Takekuni Nakama, Fukumi Furukawa
No abstract text is available yet for this article.
October 2017: Annals of Dermatology
https://www.readbyqxmd.com/read/28833074/methodological-considerations-for-disentangling-a-risk-factor-s-influence-on-disease-incidence-versus-postdiagnosis-survival-the-example-of-obesity-and-breast-and-colorectal-cancer-mortality-in-the-women-s-health-initiative
#12
RANDOMIZED CONTROLLED TRIAL
Elizabeth M Cespedes Feliciano, Ross L Prentice, Aaron K Aragaki, Marian L Neuhouser, Hailey R Banack, Candyce H Kroenke, Gloria Y F Ho, Oleg Zaslavsky, Howard D Strickler, Ting-Yuan David Cheng, Rowan T Chlebowski, Nazmus Saquib, Rami Nassir, Garnet Anderson, Bette J Caan
Often, studies modeling an exposure's influence on time to disease-specific death from study enrollment are incorrectly interpreted as if based on time to death from disease diagnosis. We studied 151,996 postmenopausal women without breast or colorectal cancer in the Women's Health Initiative with weight and height measured at enrollment (1993-1998). Using Cox regression models, we contrast hazard ratios (HR) from two time-scales and corresponding study subpopulations: time to cancer death after enrollment among all women and time to cancer death after diagnosis among only cancer survivors...
December 1, 2017: International Journal of Cancer. Journal International du Cancer
https://www.readbyqxmd.com/read/28782140/dermoscopic-and-reflectance-confocal-microscopic-presentation-of-hailey-hailey-disease-a-case-series
#13
A Oliveira, E Arzberger, B Pimentel, V C de Sousa, P Leal-Filipe
BACKGROUND/PURPOSE: Hailey-Hailey disease is a rare inherited acantholytic skin disorder characterized by heterogeneous clinical presentation. Its differential diagnosis might be wide, including other genodermatoses, inflammatory, and infectious skin diseases. Although histopathology remains as diagnostic gold standard, noninvasive techniques such as dermoscopy and reflectance confocal microscopy may assist clinical examination. Herein, we aim to further characterize the dermoscopic and reflectance confocal microscopic presentation of Hailey-Hailey disease with histologic correlation...
August 7, 2017: Skin Research and Technology
https://www.readbyqxmd.com/read/28768314/low-dose-naltrexone-treatment-of-familial-benign-pemphigus-hailey-hailey-disease
#14
Omer Ibrahim, Sara R Hogan, Alok Vij, Anthony P Fernandez
Importance: Familial benign pemphigus, or Hailey-Hailey disease (HHD), is a rare and debilitating genetic dermatosis characterized by chronic, recurrent vesicles, erosions, and maceration in flexural areas. Despite the reported therapeutic modalities, such as topical and systemic corticosteroids, systemic immunomodulators, topical and systemic retinoids, and laser, HHD can still be markedly difficult to control. Objective: To assess low-dose naltrexone hydrochloride in the treatment of recalcitrant HHD...
October 1, 2017: JAMA Dermatology
https://www.readbyqxmd.com/read/28768313/treatment-of-hailey-hailey-disease-with-low-dose-naltrexone
#15
Lauren N Albers, Jack L Arbiser, Ron J Feldman
Importance: Hailey-Hailey disease is a severe genetic blistering disease of intertriginous skin locations that can lead to poor quality of life and increased morbidities. Multiple therapies are available with inconsistent outcomes and potentially severe adverse effects. Objective: To determine whether low-dose naltrexone is an effective treatment for Hailey-Hailey disease. Design, Setting, and Participants: This study was a case series performed at a dermatology outpatient clinic of 3 patients with severe Hailey-Hailey disease recalcitrant to at least 4 therapies...
October 1, 2017: JAMA Dermatology
https://www.readbyqxmd.com/read/28764581/skin-diseases-of-the-vulva-inflammatory-erosive-ulcerating-and-apocrine-gland-diseases-zinc-and-vitamin-deficiency-vulvodynia-and-vestibulodynia
#16
Freja Lærke Sand, Simon Francis Thomsen
Chronic, inflammatory and ulcerating mucocutaneous diseases that can affect the vulvar area are reviewed: lichen sclerosus, lichen planus, plasma cell vulvitis, complex aphthosis, Behcet's disease, pyoderma gangrenosum, metastatic Crohn's disease, dyskeratotic skin diseases (Hailey-Hailey disease and Darier's disease), autoimmune bullous diseases (mucous membrane pemphigoid and pemphigus vulgaris) and hidradenitis suppurativa. Also, vulvodynia and vestibulodynia, zinc and vitamin B deficiency are described.
August 1, 2017: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/28669425/-treatment-of-hailey-hailey-disease-with-botulinic-toxin-a-retrospective-study-of-8-cases
#17
L Dousset, A Pham-Ledard, M-S Doutre, M Beylot-Barry, O Cogrel
BACKGROUND: Hailey-Hailey disease (HHD) is characterised by episodes of weeping erythematous lesions, particularly in areas subject to friction or maceration. Treatment is complex. The value of botulinum toxin has been demonstrated in several studies and in individual cases. AIM: To report clinical and progressive data for 8 patients treated for HHD with injections of botulinum toxin A (BTX-A), following the failure of several other therapeutic approaches. PATIENTS AND METHODS: Eight patients (three males and five females), of median age 52...
October 2017: Annales de Dermatologie et de Vénéréologie
https://www.readbyqxmd.com/read/28653466/hailey-hailey-disease-due-to-atp2c1-splice-site-mutation-successfully-treated-with-minocycline-hydrochloride
#18
M Kono, M Niizawa, T Takeichi, Y Muro, M Akiyama
Hailey-Hailey disease (HHD) is an autosomal dominant disease characterized by acantholysis and caused by ATP2C1.(1) Here, we report the first case of HHD successfully treated with minocycline alone in which the diagnosis was confirmed by gene analysis. This article is protected by copyright. All rights reserved.
June 27, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28551824/the-role-of-the-atp2c1-gene-in-hailey-hailey-disease
#19
REVIEW
Hao Deng, Heng Xiao
Hailey-Hailey disease (HHD) is a rare autosomal dominant acantholytic dermatosis, characterized by a chronic course of repeated and exacerbated skin lesions in friction regions. The pathogenic gene of HHD was reported to be the ATPase calcium-transporting type 2C member 1 gene (ATP2C1) located on chromosome 3q21-q24. Its function is to maintain normal intracellular concentrations of Ca(2+)/Mn(2+) by transporting Ca(2+)/Mn(2+) into the Golgi apparatus. ATP2C1 gene mutations are reportedly responsible for abnormal cytosolic Ca(2+)/Mn(2+) levels and the clinical manifestations of HHD...
May 27, 2017: Cellular and Molecular Life Sciences: CMLS
https://www.readbyqxmd.com/read/28541878/dermatosis-papular-acantol%C3%A3-tica-de-la-vulva-con-buena-respuesta-a-tacr%C3%A3-limus-t%C3%A3-pico
#20
Miguel Angel Flores-Terry, Pamela Zamberk Majlis, Monserrat Franco-Muñoz, Elena Vera-Iglesias, Mónica García-Arpa, Francisco Martín-Dávila
Papular acantholytic dermatosis of the vulva is a rare, chronic disorder and is an entity that remains to be fully understood. It shares clinical and histopathological overlap with Darier disease and Hailey-Hailey disease. We describe a 30-year-old woman with papular acantholytic dermatosis of the vulva. The lesions consisted of whitish papules and erosions on the labia majora. Histologically, there was hyperkeratosis and focal parakeratosis with acantholytic and dyskeratotic cells. She did not respond completely to topical steroids but clinical improvement occurred after the use of topical tacrolimus...
April 15, 2017: Dermatology Online Journal
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