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https://www.readbyqxmd.com/read/28320193/good-outcome-of-brain-stem-progressive-multifocal-leukoencephalopathy-in-an-immunosuppressed-renal-transplant-patient-importance-of-early-detection-and-rapid-immune-reconstitution
#1
Roland Sauer, Philipp Gölitz, Johannes Jacobi, Stefan Schwab, Ralf A Linker, De-Hyung Lee
Progressive multifocal leukoencephalopathy (PML) is a rare, opportunistic and often fatal disease of the CNS which may occur under immunosuppression in transplant patients. Brain stem PML is associated with a particularly bad prognosis. Here, we present a case of a renal transplant patient treated with mycophenolate mofetil (MMF) and tacrolimus who developed brain stem PML with limb ataxia, dysarthria and dysphagia. Diagnosis was established by typical MRI features and detection of JCV-DNA in the CSF. Immune reconstitution after stopping MMF and tacrolimus led to a complete and sustained remission of symptoms with improvement of the brain stem lesion over a follow-up over 20months...
April 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28320164/impact-of-stn-dbs-on-mood-drive-anhedonia-and-risk-of-psychiatric-side-effects-in-the-population-of-pd-patients
#2
Anna Antosik-Wójcińska, Łukasz Święcicki, Monika Dominiak, Emilia Sołtan, Przemyslaw Bieńkowski, Tomasz Mandat
BACKGROUND: DBS is a surgical method of choice for various movement disorders, especially for Parkinson's disease (PD). Many publications showing improvement in motor symptoms and quality of life have been presented while there is little comprehensive research evaluation of the impact of DBS on mental state and psychiatric side-effects. OBJECT: The purpose of this study was to assess the impact of DBS on mood, drive, anhedonia and psychotic symptoms in the group of PD patients...
April 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28320076/paediatric-vertebral-artery-aneurysms-a-literature-review
#3
REVIEW
Kevin Phan, Konrad Schultz, Victor M Lu, Ryan J Campbell, Nelson Wang, Prashanth J Rao
Vertebral artery (VA) aneurysms in the paediatric population are a rare but a serious condition. However, the epidemiology of paediatric VA aneurysms is poorly understood and there is little consensus on what constitutes the appropriate treatment. Although multiple treatment options are available, including surgery, endovascular approaches, coil embolization and parent artery occlusion, there is limited clinical evidence regarding which approach is most optimal. This review outlines the current literature and evidence outlining the epidemiology, presentation, pathogenesis and treatment of paediatric VA aneurysms...
March 20, 2017: ANZ Journal of Surgery
https://www.readbyqxmd.com/read/28319866/contrast-enhanced-ultrasound-and-computed-tomography-findings-of-granulomatosis-with-polyangiitis-presenting-with-multiple-intrarenal-microaneurysms-a-case-report
#4
Youe Ree Kim, Young Hwan Lee, Jong-Ho Lee, Kwon-Ha Yoon
Granulomatosis with polyangiitis (GPA) is a systemic disorder that affects small- and medium- sized vessels in many organs. Although the kidneys are the second most commonly involved organ in patients with GPA, its manifestation as multiple intrarenal aneurysms is rare. We report an unusual manifestation of GPA with multiple intrarenal microaneurysms, as demonstrated by contrast-enhanced ultrasound and computed tomography.
March 9, 2017: Clinical Imaging
https://www.readbyqxmd.com/read/28319713/observational-evidence-on-the-effects-of-mega-fires-on-the-frequency-of-hydrogeomorphic-hazards-the-case-of-the-peloponnese-fires-of-2007-in-greece
#5
M Diakakis, E I Nikolopoulos, S Mavroulis, E Vassilakis, E Korakaki
Even though rare, mega-fires raging during very dry and windy conditions, record catastrophic impacts on infrastructure, the environment and human life, as well as extremely high suppression and rehabilitation costs. Apart from the direct consequences, mega-fires induce long-term effects in the geomorphological and hydrological processes, influencing environmental factors that in turn can affect the occurrence of other natural hazards, such as floods and mass movement phenomena. This work focuses on the forest fire of 2007 in Peloponnese, Greece that to date corresponds to the largest fire in the country's record that burnt 1773km(2), causing 78 fatalities and very significant damages in property and infrastructure...
March 16, 2017: Science of the Total Environment
https://www.readbyqxmd.com/read/28319638/actinomycetoma-an-update-on-diagnosis-and-treatment
#6
Roberto Arenas, Ramón F Fernandez Martinez, Edoardo Torres-Guerrero, Carlos Garcia
Mycetoma is a chronic infection that develops after traumatic inoculation of the skin with either true fungi or aerobic actinomycetes. The resultant infections are known as eumycetoma or actinomycetoma, respectively. Although actinomycetoma is rare in developed countries, migration of patients from endemic areas makes knowledge of this condition crucial for dermatologists worldwide. We present a review of the current concepts in the epidemiology, clinical presentation, diagnosis, and treatment of actinomycetoma...
February 2017: Cutis; Cutaneous Medicine for the Practitioner
https://www.readbyqxmd.com/read/28319635/lupus-erythematosus-tumidus-of-the-scalp-masquerading-as-alopecia-areata
#7
Kara Hoverson, Abel D Jarell, Wendi E Wohltmann
Lupus erythematosus tumidus (LET) is a unique subset of chronic cutaneous lupus erythematosus (CCLE) that generally presents as urticarialike papules and plaques with induration and erythema on the face, trunk, and upper extremities. Lesions rarely present on the scalp or below the waist. We report a unique case of LET on the scalp of a woman that presented clinically as alopecia areata. Resistance to the standard treatment for alopecia areata prompted a biopsy that proved the diagnosis.
February 2017: Cutis; Cutaneous Medicine for the Practitioner
https://www.readbyqxmd.com/read/28319579/vulvar-carcinosarcoma-composed-of-intestinal-type-mucinous-adenocarcinoma-associated-with-anaplastic-pleomorphic-and-spindle-cell-carcinoma-and-heterologous-chondrosarcomatous-and-osteosarcomatous-elements-a-case-report-and-review-of-the-literature
#8
Leonardo Lordello, Patricia Webb, Esther Oliva
Carcinosarcomas (CS) are exceedingly rare in the vulva, with only 3 cases reported in the English literature, associated with squamous cell carcinoma (2) or spiradenocarcinoma (1). We first report a vulvar CS with intestinal-type mucinous adenocarcinoma associated with anaplastic pleomorphic and spindle cell carcinoma and heterologous chondro- and osteosarcomatous elements in a 62-year-old woman, who presented with a painless, slow-growing vulvar cyst for almost 2 years, that rapidly enlarged and hardened in the last 4 months forming a mass...
March 17, 2017: International Journal of Gynecological Pathology
https://www.readbyqxmd.com/read/28319572/sarcomatous-component-in-uterine-carcinosarcomas-correlates-with-advanced-stage-and-poorer-prognosis
#9
Mirna B Podoll, Mana Moghadamfalahi, Erin Faber, Julie Ann Bishop, Houda Alatassi
Uterine carcinosarcomas, also known as malignant-mixed mullerian tumors, are rare and highly aggressive tumors whose prognostic factors remain controversial. The stage at the time of presentation is the most important prognostic factor thus far, but little information exists on the prognostic impact of the sarcomatous component (SC) in these tumors. We reviewed 21 cases of uterine carcinosarcomas and estimated the volume of the SC in each case. This information was correlated with the stage of the tumor at presentation...
March 17, 2017: International Journal of Gynecological Pathology
https://www.readbyqxmd.com/read/28319526/anaplastic-variant-of-diffuse-large-b-cell-lymphoma-displays-intricate-genetic-alterations-and-distinct-biological-features
#10
Mingyang Li, Yixiong Liu, Yingmei Wang, Gang Chen, Qiongrong Chen, Hualiang Xiao, Fang Liu, Chubo Qi, Zhou Yu, Xia Li, Linni Fan, Ying Guo, Qingguo Yan, Shuangping Guo, Zhe Wang
Anaplastic diffuse large B-cell lymphoma (A-DLBCL) is a rare morphologic variant characterized by the presence of polygonal, bizarre-shaped tumor cells. However, the clinicopathologic and genetic features of this variant are largely unknown. In this study, we investigated 35 cases of A-DLBCL with regard to their clinical, pathologic, and genetic characteristics. The age of the patients ranged from 23 to 89 years (median age, 62 y) with a male to female ratio of 23:12. Twenty-two of 26 (85%) patients had Ann Arbor stage III or IV disease, and 17/26 (65%) patients had a high-intermediate or high International Prognostic Index score...
March 17, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28319502/remitting-seronegative-symmetrical-synovitis-with-pitting-edema-appearance-on-fdg-pet-ct
#11
Jianjie Wang, Chaoyu Pu, Zhenchang Wang, Jigang Yang
Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) is a rare condition in the elderly and can appear as a first presentation of various types of rheumatic and malignant diseases. We presented a 62-year-old man with the diagnosis of RS3PE based on the clinical sign and laboratory data. Because of the possibility of associated malignancies in RS3PE, FDG PET/CT was performed to exclude occult tumors. The images showed multiple, symmetrically, diffusely increased F-FDG uptake in the soft tissue around joints and bones in the shoulders, hips, knees, and ankles...
March 17, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28319272/gluteal-abscess-and-fistula-after-release-of-sacrospinous-fixation-sutures
#12
Simone Salimans, Leonie Speksnijder, Louwerens Vos, Mariska Shekary-Moonen, Jeroen van Bavel
Gluteal abscesses and fistula are rare complications after release of sacrospinous fixation sutures. The entire suture should be removed to prevent complications. Sacrospinous fixation (SSF) is a safe and effective procedure for the treatment of uterovaginal prolapse. The present report describes an abnormal complication of a gluteal abscess and fistula after release of SSF sutures. The patient provided written informed consent. This article is protected by copyright. All rights reserved.
March 20, 2017: International Journal of Gynaecology and Obstetrics
https://www.readbyqxmd.com/read/28319177/radiological-features-of-primitive-neuroectodermal-tumors-in-intra-abdominal-and-retroperitoneal-regions-a-series-of-18-cases
#13
Xiaoping Yi, Wenguang Liu, Youming Zhang, Desheng Xiao, Hongling Yin, Xueying Long, Li Li, Hongyan Zai, Minfeng Chen, Wenzheng Li, Lunquan Sun
OBJECTIVES: To characterize the imaging and clinicopathological features of primitive neuroectodermal tumors (PNETs) arising in intra-abdominal and retroperitoneal regions. METHODS: Eighteen patients with histopathologically proven intra-abdominal and retroperitoneal PNET were enrolled; computed tomography was performed for all cases, and magnetic resonance imaging was performed for a single case. Typical computed tomography and magnetic resonance imaging findings, including morphology, texture and enhancement features, as well as clinicopathological characteristics and prognosis data were retrospectively analyzed...
2017: PloS One
https://www.readbyqxmd.com/read/28319067/the-histone-demethylase-kdm3a-and-its-downstream-target-mcam-promote-ewing-sarcoma-cell-migration-and-metastasis
#14
M Sechler, J K Parrish, D K Birks, P Jedlicka
Ewing Sarcoma is the second most common solid pediatric malignant neoplasm of bone and soft tissue. Driven by EWS/Ets, or rarely variant, oncogenic fusions, Ewing Sarcoma is a biologically and clinically aggressive disease with a high propensity for metastasis. However, the mechanisms underpinning Ewing Sarcoma metastasis are currently not well understood. In the present study, we identify and characterize a novel metastasis-promotional pathway in Ewing Sarcoma, involving the histone demethylase KDM3A, previously identified by our laboratory as a new cancer-promoting gene in this disease...
March 20, 2017: Oncogene
https://www.readbyqxmd.com/read/28319023/isolated-bilateral-simplex-ureteric-ectopia-bladder-capacity-as-an-indicator-of-continence-outcome
#15
Vasilis Stavrinides, Paul Charlesworth, Dan Wood, Divyesh Desai, Abraham Cherian, Imran Mushtaq, Peter Cuckow, Naima Smeulders
INTRODUCTION: Isolated bilateral simplex ectopic ureters (BSEUs) are rare but pose a therapeutic challenge: ureteric reimplantation alone does not accomplish continence in all. Identifying the patients needing additional procedures for continence early could prevent multiple operations. OBJECTIVE: Potential preoperative indicators for postoperative continence are explored in eight BSEU girls without cloacal, anorectal, or spinal anomalies. STUDY DESIGN: With institutional approval, all patients with BSEU between 1985 and 2012 were retrospectively reviewed...
March 6, 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28318970/transcricothyroid-endoscopic-subglottic-surgery-for-posterior-glottic-stenosis-a-case-report
#16
Koji Matsushima, Kentaro Matsuura, Teppei Takeda, Yuko Sasaki, Akira Fukuo, Kota Wada, Yusuke Watanabe
Posterior glottic stenosis (PGS) is a rare but life-threatening condition mostly caused by damage to the interarytenoid mucosa by an endotracheal tube. In surgical treatment of PGS, airway patency is prioritized, and the laryngeal functions involved in swallowing and phonation are considerably sacrificed. In the majority of cases, lateralization of a vocal fold or partial excision of a vocal fold and arytenoid cartilage results in glottal closure insufficiency and deterioration of phonatory function. We present the first report of transcricothyroid endoscopic subglottic surgery to treat a 46-year-old man with PGS who was intubated for 10 days...
March 16, 2017: Journal of Voice: Official Journal of the Voice Foundation
https://www.readbyqxmd.com/read/28318968/improved-glottic-exposure-for-robotic-microlaryngeal-surgery-a-case-series
#17
Dehra A McGuire, Jennifer P Rodney, Nilesh R Vasan
Robotic surgery has become the standard of care for many procedures outside of otolaryngology and now is gaining momentum within our specialty. The da Vinci (Intuitive Surgical, Sunnyvale, CA) robot has several advantages to human hands, including removal of tremor and better access to lesions because of increased degree of movement of the articulated instruments. The glottis has rarely been addressed using robotic surgery because access was previously thought to be difficult because of the limitations of currently used retractors, which include poor base of tongue and oral commissure retraction resulting in lack of exposure of the glottis in many patients and lack of space for the robotic instruments to occupy...
March 17, 2017: Journal of Voice: Official Journal of the Voice Foundation
https://www.readbyqxmd.com/read/28318950/cholesteryl-ester-storage-disease-an-underdiagnosed-cause-of-cirrhosis-in-adults
#18
REVIEW
Mamta Pant, Kiyoko Oshima
Cholesteryl Ester Storage Disease (CESD), is a rare multisystem autosomal recessive disorder and belongs to the broad family of lysosomal storage disorders. It can present anytime from infancy and childhood to even adulthood. The clinical manifestations are generally severe in infants and with milder forms in adults. One of the prominent sites of involvement is liver. Due to low awareness of this condition among physicians including surgical pathologists, majority of the liver biopsies, especially from the adults are often misdiagnosed as non-alcoholic fatty liver disease/non-alcoholic steatohepatitis or cryptogenic cirrhosis...
February 9, 2017: Annals of Diagnostic Pathology
https://www.readbyqxmd.com/read/28318868/management-of-periprosthetic-joint-infection-the-more-we-learn-the-less-we-know
#19
Robert A Henderson, Matthew S Austin
BACKGROUND: Despite the tremendous and long-standing success of total hip arthroplasty and total knee arthroplasty as treatments for end-stage arthritis, periprosthetic joint infection (PJI) remains a rare but feared complication of these procedures. METHODS: This review highlights some of the difficulties inherent to studying PJI. These include the difficulty in powering studies to capture this relatively uncommon complication, as well as the heterogeneity in clinical presentation and manifestations associated with the diagnosis and treatment of PJI...
February 23, 2017: Journal of Arthroplasty
https://www.readbyqxmd.com/read/28318810/incomplete-partition-type-iii-a-rare-and-difficult-cochlear-implant-surgical-indication
#20
Levent Sennaroğlu, Münir Demir Bajin
OBJECTIVE: Presenting the clinical features and treatment options for incomplete partition type-III. METHODS: Nine primary and 1 revision incomplete partition type-III cochlear implant cases treated between 2004 and 2015 in Hacettepe University Department of Otolaryngology were included in the study. Treatment options and particularly cochlear implantation tecnique were described. RESULTS: Nine primary and 1 revison cases were all succesfully implanted...
March 16, 2017: Auris, Nasus, Larynx
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