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Raynaud's phenomenon

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https://www.readbyqxmd.com/read/28431994/raynaud-s-phenomenon-revisiting-a-rare-sign-of-pheochromocytoma-paraganglioma
#1
Virendra Ashokrao Patil, Rajiv Kasaliwal, Manjunath R Goroshi, Anurag R Lila, Tushar Bandgar, Nalini S Shah
Paraganglioma (PGL) are rare tumors arising from extraadrenal chromaffin cells and occasionally secret catecholamines. The patient commonly presents with headache, palpitation, anxiety, diaphoresis and episodic or sustained hypertension. Rarely patient can present with Raynaud's phenomenon. We present a case of adolescent girl who presented with isolated Raynaud's phenomenon as only manifestation of metastasis of PGL three years after undergoing surgical excision of normetanephrine secreting abdominal PGL.
April 18, 2017: Urology
https://www.readbyqxmd.com/read/28429690/-our-experience-in-the-diagnosis-and-treatment-of-postural-orthostatic-tachycardia-syndrome-vasovagal-syncope-and-inappropriate-sinus-tachycardia-in-children
#2
Sezen Ugan Atik, Reyhan Dedeoğlu, Aida Koka, Funda Öztunç
OBJECTIVES: The aim of this study was to share our experience in the diagnosis and treatment of patients who presented at our clinic with syncope, pre-syncope, dizziness, and palpitations. STUDY DESIGN: Patients who were treated at pediatric cardiology clinic for complaints of syncope, dizziness, and palpitations between 2014 and 2016 were enrolled in the study. Detailed history of the patients, physical examination findings, laboratory and electrocardiogram results were recorded...
April 2017: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/28426903/the-therapeutic-efficacy-of-botulinum-toxin-in-treating-scleroderma-associated-raynaud-s-phenomenon-a-randomized-double-blind-placebo-controlled-clinical-trial-assessing
#3
Ricardo J Bello, Carisa M Cooney, Eitan Melamed, Keith Follmar, Gayane Yenokyan, Gwendolyn Leatherman, Ami A Shah, Fredrick M Wigley, Laura K Hummers, Scott D Lifchez
Objective To assess the therapeutic efficacy of local injections with Botulinum Toxin Type-A (Btx-A) in improving blood flow to the hands of patients with Raynaud's Phenomenon (RP) secondary to scleroderma. Methods In this randomized, double-blind, placebo-controlled clinical trial (http://ClinicalTrials.gov #NCT02165111), patients with scleroderma-associated RP received Btx-A (50 units in 2.5 mL) in one randomly selected hand and sterile saline (2.5 mL) in the opposite hand. Follow-up at 1 and 4 months post-injection included Laser Doppler Imaging (LDI) of hands, patient-reported outcomes, and physical exam...
April 20, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28420068/pentraxin-3-levels-are-associated-with-vasculitis-and-disease-activity-in-childhood-onset-systemic-lupus-erythematosus
#4
S Sahin, A Adrovic, K Barut, S Durmus, R Gelisgen, H Uzun, O Kasapcopur
Objectives Childhood-onset systemic lupus erythematosus (cSLE) is a multisystemic autoimmune disease characterized by inflammatory organ damage by means of vasculitis. Pentraxin-3 (PTX3) is expressed locally at the sites of inflammatory processes, predominantly from endothelial cells. In adult studies, PTX3 has shown to be an indicator of active vasculitis both in large-vessel and small-vessel vasculitides, as well as in SLE. Moreover, in SLE it has found to be correlated with disease activity, and with some of the clinical manifestations and laboratory parameters...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28414153/inflammatory-myopathy-associated-with-myasthenia-gravis-with-and-without-thymic-pathology-report-of-four-cases-and-literature-review
#5
REVIEW
Ernestina Santos, Ester Coutinho, Ana Martins da Silva, António Marinho, Carlos Vasconcelos, Ricardo Taipa, Manuel Melo Pires, Guilherme Gonçalves, Carlos Lopes, Maria Isabel Leite
INTRODUCTION: the association of myasthenia gravis (MG) and inflammatory myopathy is rare and often only one of the diseases is diagnosed. Thymus pathology may be in the origin of such disease association. METHODS: we described four patients with both MG and inflammatory myopathy. RESULTS: these cases correspond to 2.3% of our MG cohort. Case 1: MG, polymyositis and thymolipoma; case 2: MG and necrotizing myopathy without thymic pathology on a background of scleroderma, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia (CREST); case 3: MG and dermatomyositis without thymic pathology; case 4: MG and dermatomyositis with type C thymoma...
April 13, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28401298/neurovascular-hand-symptoms-in-relation-to-cold-exposure-in-northern-sweden-a-population-based-study
#6
Albin Stjernbrandt, Bodil Björ, Martin Andersson, Lage Burström, Ingrid Liljelind, Tohr Nilsson, Ronnie Lundström, Jens Wahlström
PURPOSE: To describe the self-reported ambient cold exposure in northern Sweden and to relate the level of cumulative cold exposure to the occurrence of sensory and vascular hand symptoms. We hypothesize that cold exposure is positively related to reporting such symptoms. METHODS: A questionnaire about cold exposure and related symptoms was sent out to 35,144 subjects aged 18-70 years and living in northern Sweden. RESULTS: A total of 12,627 out of 35,144 subjects returned the questionnaire (response rate 35...
April 11, 2017: International Archives of Occupational and Environmental Health
https://www.readbyqxmd.com/read/28391171/a-two-scale-modeling-and-computational-framework-for-vibration-induced-raynaud-syndrome
#7
Yue Hua, Pierre Lemerle, Jean-François Ganghoffer
Hand-Arm Vibration syndrome (HAVS), usually caused by long-term use of hand-held power tools, can in certain manifestations alter the peripheral blood circulation in the hand-arm region. HAVS typically occurs after exposure to cold, causing an abnormally strong vasoconstriction of blood vessels. A pathoanatomical mechanism suggests that a reduction of the lumen of the blood vessels in VWF (Vibration White Finger) subjects, due to either hypertrophy or thickening of the vessel wall, may be at the origin of the disease...
March 28, 2017: Journal of the Mechanical Behavior of Biomedical Materials
https://www.readbyqxmd.com/read/28386141/infrared-thermal-imaging-in-connective-tissue-diseases
#8
REVIEW
Marek Chojnowski
Infrared thermal imaging (IRT) is a non-invasive, non-contact technique which allows one to measure and visualize infrared radiation. In medicine, thermal imaging has been used for more than 50 years in various clinical settings, including Raynaud's phenomenon and systemic sclerosis. Imaging and quantification of surface body temperature provides an indirect measure of the microcirculation's overall performance. As such, IRT is capable of confirming the diagnosis of Raynaud's phenomenon, and, with additional cold or heat challenge, of differentiating between the primary and secondary condition...
2017: Reumatologia
https://www.readbyqxmd.com/read/28382775/dermoscopic-features-of-periungual-papules-in-multicentic-reticulohistiocytosis-dermoscopy-in-multicentic-reticulohistiocytosis
#9
M Sobjanek, M Sławińska, A Romaszkiewicz, M Sokołowska-Wojdyło, E Jasiel-Walikowska, R Nowicki
A 66-year-old woman was diagnosed with MRH based on clinical and histopathological findings. The disease had started 6 months before with arthralgia of the right shoulder joint and symmetric xanthelasma on the palpebrae. One month later symmetric, bilateral arthralgia affecting most of the proximal and distal intraphalangeal joints of both hands have occurred. The multiple, asymptomatic, 2-3 mm in size, reddish -NDASH- brown papules adjacent to the proximal nail fold and two larger asymptomatic flesh-coloured nodules located at the extensor surface of the right antebrachium and the dorsal surface of the third right finger have appeared at the same time...
April 6, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28370486/clinical-presentation-and-outcomes-of-patients-with-type-1-monoclonal-cryoglobulinemia
#10
Surbhi Sidana, S Vincent Rajkumar, Angela Dispenzieri, Martha Q Lacy, Morie A Gertz, Francis K Buadi, Suzanne R Hayman, David Dingli, Prashant Kapoor, Wilson I Gonsalves, Ronald S Go, Yi Lisa Hwa, Nelson Leung, Amie L Fonder, Miriam A Hobbs, Steven R Zeldenrust, Stephen J Russell, John A Lust, Robert A Kyle, Shaji K Kumar
We describe a series of 102 patients diagnosed from January 1, 1990 to December 31, 2015 with Type 1 monoclonal cryoglobulinemia (MoC). Symptoms were seen in 89 (87%) patients, including: cutaneous symptoms in 64 (63%) patients, with purpura (n=43, 42%) and ulcers/gangrene (n=35, 34%) being most common; neurological findings in 33 (32%) patients, most frequently sensory neuropathy (n=24, 24%); vasomotor symptoms, mainly Raynaud's phenomenon in 25 (25%); arthralgias in 24 (24%); and renal manifestations, primarily glomerulonephritis in 14 (14%) patients...
March 29, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28358168/efficacy-of-botulinum-toxin-b-injection-for-raynaud-s-phenomenon-and-digital-ulcers-in-patients-with-systemic-sclerosis
#11
Sei-Ichiro Motegi, Akihiko Uehara, Kazuya Yamada, Akiko Sekiguchi, Chisako Fujiwara, Sayaka Toki, Yuki Date, Tetsuya Nakamura, Osamu Ishikawa
The efficacy and safety of botulinum toxin B (BTX-B) for treatment of Raynaud's phenomenon and digital ulcers in patients with systemic sclerosis was assessed. A total of 45 patients with systemic sclerosis who had Raynaud's phenomenon were blinded and divided randomly into 4 groups: a no-treatment control group, and 3 treatment groups, using 250, 1,000 or 2,000 U of BTX-B injections in the hand with more severe symptoms. Four weeks after injection, pain/numbness visual analogue scale score and Raynaud's score in the groups treated with 1,000 and 2,000 U BTX-B were significantly lower than in the control group and the group treated with 250 U BTX-B...
March 30, 2017: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/28357090/raynaud-s-of-the-tongue-following-chemoradiation-for-squamous-cell-carcinoma-of-the-oropharynx
#12
Amit Mittal, Bharat B Mittal
Raynaud's of the tongue following radiation alone or chemoradiotherapy (CRT) is a rare occurrence. The present study reports a case where typical symptoms and signs of Raynaud's phenomenon involving the tongue occurred ~18 months following CRT treatment in a 53-year-old female, who was a smoker prior to CRT with stage T2N2cM0 local-regional advanced stage IV oropharyngeal cancer. The patient was treated using cisplatin chemotherapy and intensity-modulated radiation with a dose of 70 Gray (Gy). The intermittent episodes of painful discoloration of the tongue were exacerbated due to the cold and emotional stress...
February 2017: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/28350093/inhibitory-effect-of-angelica%C3%A2-gigas-on-cold%C3%A2-induced-rhoa-activation-in-vascular-cells
#13
Kangwook Lee, Min Soo Chae, Sung-Gook Cho, Ho Yeon Go, Seung-Ho Sun, Junbock Jang, Ki-Yong Jung, You-Kyung Choi, Yun-Kyung Song, Sung Yong Sim, Hye Lim Lee, Mi Suk Kang, Chan-Yong Jeon, Seong Gyu Ko
The herbal extract Angelica gigas (AG) has been applied as a vasodilating agent for patients suffering from vascular diseases for many years; however, the underlying mechanism has not been fully elucidated. The present study hypothesized that the anti‑vasoconstrictive effect of AG may be effective in the treatment of abnormal cold‑mediated vasospasms that occur in Raynaud's phenomenon (RP). The effect of AG on the activity of ras homolog gene family member A (RhoA) was investigated in cold‑exposed vascular cells...
March 28, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28344932/mutation-in-tnxb-gene-causes-moderate-to-severe-ehlers-danlos-syndrome
#14
Carolyn S Kaufman, Merlin G Butler
We report a 28-year-old female who presented with severe joint pain, chronic muscle weakness, Raynaud's phenomenon, and hypermobility. She was found to have a 6074A > T nucleotide transition in the TNXB gene causing an amino acid protein change at Asp2025Val classified as likely pathogenic. We add this clinical report to the literature and classical human disease gene catalogs to identify this specific mutation as disease-causing. This gene variant was reported previously in a different 36-year-old patient who shared our patient's symptoms of joint hypermobility, skeletal and joint pain, skin elasticity and musculoskeletal problems, thereby causing a more severe presentation than seen in the hypermobility type of Ehlers-Danlos syndrome (EDS)...
May 27, 2016: World Journal of Medical Genetics
https://www.readbyqxmd.com/read/28343718/-comparative-evaluation-of-dermoscopy-and-capillaroscopy-in-raynaud-s-phenomenon
#15
J Moreau, A-S Dupond, N Dan, T Untereiner, C Vidal, F Aubin
OBJECTIVE: Raynaud's phenomenon (RP) is a common cause for consultation. Capillaroscopy is a well-established technique to detect capillary abnormalities suggestive of a connective tissue disease, but it is sometimes unavailable. The aim of this study was to compare dermoscopy and capillaroscopy in the assessment of RP. METHODS: This was a prospective single-centre observational study in adult patients consulting for RP at the Hôpital Nord Franche-Comté between January 2014 and June 2015...
March 23, 2017: Annales de Dermatologie et de Vénéréologie
https://www.readbyqxmd.com/read/28336160/unexpected-drug-induced-raynaud-phenomenon-analysis-from-the-french-national-pharmacovigilance-database
#16
Émilie Bouquet, Geoffrey Urbanski, Christian Lavigne, Pascale Lainé-Cessac
OBJECTIVE: To estimate the association between exposure to medicinal products and Raynaud phenomenon. METHODS: The study used the data of all adverse drug reactions notified to the French national pharmacovigilance database. All cases reported between 1st January 1995 and 10th December 2012 were selected. A case/non-case method was used to measure disproportionality of the association between drug exposure and Raynaud phenomenon. The cases concerned all observations involving Raynaud phenomenon...
February 22, 2017: Thérapie
https://www.readbyqxmd.com/read/28329526/chronic-granulomatous-disease-as-a-risk-factor-for-cutaneous-lupus-in-childhood
#17
Sandrina Carvalho, Susana Machado, Rita Sampaio, Margarida Guedes, Júlia Vasconcelos, Diogo Semedo, Manuela Selores
Chronic granulomatous disease (CGD) is a primaryimmunodeficiency disorder that affects the phagocyticcells of the innate immune system. It is characterizedby recurrent or persistent infections with granulomaformation. Lupus-like lesions have been reported incarriers of CGD and less frequently, in patients withCGD. Immunological study in these patients areusually negative. We describe the case of an 8-yearoldboy with CGD who developed chronic and acutecutaneous lupus erythematous with angular cheilitis,oral ulcers, Raynaud phenomenon, and positiveserologies for antinuclear, anticentromere, and anti-Saccharomyces cerevisiae antibodies...
March 15, 2017: Dermatology Online Journal
https://www.readbyqxmd.com/read/28321017/a-non-invasive-technique-for-the-evaluation-of-peripheral-circulatory-functions-in-female-subjects-with-raynaud-s-phenomenon
#18
Seyed Mohammad Mirbod, Haruo Sugiura
Japanese women now account for 43 percent of the labor force. A number of them are involved in construction, agricultural and forestry jobs. The aim of this study was to establish a non-invasive technique for the evaluation of peripheral circulatory functions in women with Raynaud's phenomenon (RP); and introduce a specific method for the assessment of vascular disturbances in females exposed to hand-transmitted vibration. The subjects of this study were 10 women with primary RP, 7 women with progressive systemic sclerosis (PSS) secondary to RP, and 17 females who were included as the control group...
March 17, 2017: Industrial Health
https://www.readbyqxmd.com/read/28316854/a-marked-response-to-immunosuppressive-intervention-for-abruptly-occurring-cardiac-complications-in-a-case-of-juvenile-systemic-sclerosis-overlapped-with-dermatomyositis
#19
Tsunehisa Nagamori, Yoichiro Yoshida, Hironori Takahashi, Hideharu Oka, Aya Kajihama, Koichi Nakau, Masaya Sugimoto, Masako Minami-Hori, Hiroshi Azuma
Juvenile-onset systemic sclerosis (jSSc) is a rare condition, having unique characteristic features compared to adult-onset SSc. Although cardiac involvement (CI) is known as a leading cause of mortality overall in SSc, the importance of CI in jSSc has not been emphasized. Here we present a 13-year-old female with jSSc overlapped with dermatomyositis (DM) complicated CI. She developed skin thickness and induration, Raynaud's phenomenon, digital pitting scars in fingertips, and skeletal myositis. Oral prednisolone and pulse methotrexate treatment led to the improvement of skin findings; however two weeks after the initiation she suddenly presented with muscle pain and dyspnea within a few days...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28303481/morphea-and-eosinophilic-fasciitis-an-update
#20
REVIEW
Jorre S Mertens, Marieke M B Seyger, Rogier M Thurlings, Timothy R D J Radstake, Elke M G J de Jong
Morphea, also known as localized scleroderma, encompasses a group of idiopathic sclerotic skin diseases. The spectrum ranges from relatively mild phenotypes, which generally cause few problems besides local discomfort and visible disfigurement, to subtypes with severe complications such as joint contractures and limb length discrepancies. Eosinophilic fasciitis (EF, Shulman syndrome) is often regarded as belonging to the severe end of the morphea spectrum. The exact driving mechanisms behind morphea and EF pathogenesis remain to be elucidated...
March 16, 2017: American Journal of Clinical Dermatology
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