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MAGIC syndrome

Ryszard Grenda, Wioletta Jarmużek, Jacek Rubik, Barbara Piątosa, Sylwester Prokurat
UNLABELLED: Pediatric patients with end-stage renal failure due to severe drug-resistant nephrotic syndrome are at risk of rapid recurrence after renal transplantation. Treatment options include plasmapheresis, high-dose of cyclosporine A/methylprednisolone and more recently-rituximab (anti-B CD20 monoclonal depleting antibody). We report five patients with immediate (1-2 days) post-transplant recurrence of nephrotic syndrome, treated with this kind of combined therapy including 2-4 weekly doses of 375 mg/m(2) of rituximab...
September 2016: European Journal of Pediatrics
Yuka Kaneko, Noriaki Nakai, Takashi Kida, Yutaka Kawahito, Norito Katoh
Mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome are disease that fulfilled criteria for diagnosis of Behcet's disease (BD) and relapsing polychondritis (RP). We report a 22-year-old Japanese woman presented with MAGIC syndrome and we described the clinicopathological characteristics of MAGIC syndrome based on a review of published cases from July 1985 to December 2015. In our case, the patient with oral aphthae, erythema nodosum, acne-like eruptions, uveitis, and polyarthritis fulfilled criteria for diagnosis of incomplete form of BD...
May 2016: Indian Journal of Dermatology
C Lemos, D Rial, F Q Gonçalves, J Pires, H B Silva, F C Matheus, A C da Silva, J M Marques, R J Rodrigues, I Jarak, R D Prediger, F Reis, R A Carvalho, F C Pereira, R A Cunha
High sugar consumption is a risk factor for metabolic disturbances leading to memory impairment. Thus, rats subject to high sucrose intake (HSu) develop a metabolic syndrome and display memory deficits. We now investigated if these HSu-induced memory deficits were associated with metabolic and electrophysiological alterations in the hippocampus. Male Wistar rats were submitted for 9 weeks to a sucrose-rich diet (35% sucrose solution) and subsequently to a battery of behavioral tests; after sacrifice, their hippocampi were collected for ex vivo high-resolution magic angle spinning (HRMAS) metabolic characterization and electrophysiological extracellular recordings in slices...
February 19, 2016: Neuroscience
Marco G Alves, Ana D Martins, Ivana Jarak, Alberto Barros, Joaquina Silva, Mário Sousa, Pedro F Oliveira
Klinefelter syndrome (KS) is the most common genetic cause of human infertility, but the mechanism(s) responsible for its phenotype remain largely unknown. KS is associated with alterations in body composition and with a higher risk of developing metabolic diseases. We therefore hypothesized that KS men seeking fertility treatment possess an altered testicular metabolism profile that may hamper the nutritional support of spermatogenesis. Testicular biopsies from control (46, XY) (n = 6) and KS (47, XXY) (n = 6) individuals were collected and analyzed by proton high-resolution magic-angle spinning nuclear magnetic resonance spectroscopy...
March 2016: Molecular Reproduction and Development
Boris Ajdinovic, Tamara Dragovic, Zvonko Magic, Nikola Kocev
OBJECTIVE: Diabetic nephropathy (DN) is a clinical syndrome characterized by persistent albuminuria, increasing arterial blood pressure and progressive decline in glomerular filtration rate (GFR). When persistent albuminuria is established, antihypertensive treatment becomes most important factor in slowing the progression of diabetic glomerulopathy. Aim of this study was to examine if renoprotective response to losartan therapy, in patients with diabetic nephropathy, depends on 1166 A/C gene polymorphism for its target receptor, angiotensin II type 1 receptor (AT1R)...
September 2015: Hellenic Journal of Nuclear Medicine
Julie L Huffmyer, Danja S Groves
Pulmonary complications after the use of extracorporeal circulation are common, and they range from transient hypoxemia with altered gas exchange to acute respiratory distress syndrome (ARDS), with variable severity. Similar to other end-organ dysfunction after cardiac surgery with extracorporeal circulation, pulmonary complications are attributed to the inflammatory response, ischemia-reperfusion injury, and reactive oxygen species liberated as a result of cardiopulmonary bypass. Several factors common in cardiac surgery with extracorporeal circulation may worsen the risk of pulmonary complications including atelectasis, transfusion requirement, older age, heart failure, emergency surgery, and prolonged duration of bypass...
June 2015: Best Practice & Research. Clinical Anaesthesiology
Amerigo Vitagliano, Marco Noventa, Michela Quaranta, Salvatore Gizzo
The aim of the study was to analyze all the available evidence from both in vitro and in vivo studies regarding the efficacy of statin therapy in the treatment of endometriosis, evaluating the potential efficacy, side effects, and contraindications of their administration in humans. We focused on defining the potential benefits that the administration of statins may have on patients affected by endometriosis and the possible adverse effects of such a therapy on ovarian function and fertility profile. According to our article selection criteria, we included in the review in vitro and in vivo studies performed on human or animal models...
April 2016: Reproductive Sciences
Rodrigo Badotti Antunes, Anderson Aparecido Camilo, Airton Moreira da Silva, Jorge Vicente Lopes da Silva, Nivaldo Alonso
OBJECTIVES: The objective of this study was to evaluate the variation of the orbital volume and the correlation with the magnitude of craniofacial advances as well as demonstrate its effectiveness through comparisons with normal levels of orbital volumes. INTRODUCTION: Syndromic craniofacial synostosis is characterized by cranial morphological alterations and hypoplasia of the midface that may result in obstructive sleep apnea and exorbitism; these effects are indications of surgical treatment...
May 2015: Journal of Craniofacial Surgery
Yoko Kawakami, Katsuya Endo, Tomonori Ishii, Sho Haneda, Fumiyoshi Fujishima, Yoichi Kakuta, Hisashi Shiga, Yoshitaka Kinouchi, Tooru Shimosegawa
INTRODUCTION: Mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome is a rare overlap syndrome that includes features characteristic of both Behçet's disease (BD) and relapsing polychondritis (RP). CASE DESCRIPTION: A 30-year-old female complained of lower abdominal pain and bloody stools during medical treatment for RP. Total colonoscopy revealed oval-shaped deep ulcers on the terminal ileum similar to those of intestinal BD. After performing the ileocecal resection, both RP and gastrointestinal lesions relapsed, but improved with infliximab treatment...
2014: SpringerPlus
Martin L Pall, Stephen Levine
The transcription factor Nrf2, nuclear factor erythroid-2-related factor 2, activates the transcription of over 500 genes in the human genome, most of which have cytoprotective functions. Nrf2 produces cytoprotection by detoxification mechanisms leading to increased detoxification and excretion of both organic xenobiotics and toxic metals; its action via over two dozen genes increases highly coordinated antioxidant activities; it produces major anti-inflammatory changes; it stimulates mitochondrial biogenesis and otherwise improves mitochondrial function; and it stimulates autophagy, removing toxic protein aggregates and dysfunctional organelles...
February 25, 2015: Sheng Li Xue Bao: [Acta Physiologica Sinica]
Tanja Barkhausen, Ulrike Junius-Walker, Eva Hummers-Pradier, Christiane A Mueller, Gudrun Theile
BACKGROUND: Geriatric assessments are established tools in institutional care since they enable standardized detection of relevant age-related disorders. Geriatric assessments could also be helpful in general practice. However, they are infrequently used in this setting, mainly due to their lengthy administration. The aim of the study was the development of a "manageable geriatric assessment--MAGIC", specially tailored to the requirements of daily primary care. METHODS: MAGIC was developed based on the comprehensive Standardized Assessment for Elderly People in Primary Care (STEP), using four different methodological approaches: We relied on A) the results of the PRISCUS study by assessing the prevalence of health problems uncovered by STEP, the importance of the respective problems rated by patients and general practitioners, as well as the treatment procedures initiated subsequently to the assessment...
2015: BMC Family Practice
Macaulay Amechi C Onuigbo
The consensus conference on cardio-renal syndromes (2008) defined 'cardio-renal syndromes' as 'disorders of the heart and kidneys whereby acute or chronic dysfunction in one organ may induce acute or chronic dysfunction of the other' and identified five subtypes of the syndromes. Various pathophysiologic mechanisms underlie cardiorenal syndrome including hemodynamic derangements, reduced cardiac output leading to impaired renal perfusion, reduced stroke volume, raised atrial filling pressures, elevated atrial pressures, sodium and water retention, venous congestion, right ventricular dysfunction and venous hypertension causing increased renal venous pressure, intra-abdominal hypertension, various neurohormonal adaptations including activation of the renin-angiotensin-aldosterone system, adaptive activation of the sympathetic nervous system, cytokine release and oxidative stress...
2014: Current Hypertension Reviews
Daniel J DiBardino
PURPOSE OF REVIEW: Much data exist concerning Norwood discharge mortality. Less is known about late survival. Examining the available data in light of the Single Ventricle Reconstruction trial is insightful as focus shifts toward long-term survival. RECENT FINDINGS: Data from 2000 to 2001 demonstrated approximately 40-50% 10-year survival, 30-40% or less between 10 and 15 years. The shape of the curves was characteristic; the majority of deaths within the first year, followed by a late constant phase...
January 2015: Current Opinion in Cardiology
Marianna Parlato, Jean-Marc Cavaillon
Critically ill patients who display a systemic inflammatory response syndrome (SIRS) are prone to develop nosocomial infections. The challenge remains to distinguish as early as possible among SIRS patients those who are developing sepsis. Following a sterile insult, damage-associated molecular patterns (DAMPs) released by damaged tissues and necrotic cells initiate an inflammatory response close to that observed during sepsis. During sepsis, pathogen-associated molecular patterns (PAMPs) trigger the release of host mediators involved in innate immunity and inflammation through identical receptors as DAMPs...
2015: Methods in Molecular Biology
Benjamin C Haller, Jurriaan M de Vos, Barbara Keller, Andrew P Hendry, Elena Conti
The evolution of the flower is commonly thought to have spurred angiosperm diversification. Similarly, particular floral traits might have promoted diversification within specific angiosperm clades. We hypothesize that traits promoting the precise positional transfer of pollen between flowers might promote diversification. In particular, precise pollen transfer might produce partial reproductive isolation that facilitates adaptive divergence between parapatric populations differing in their reproductive-organ positions...
2014: PloS One
Claire Masterson, Daniel O'Toole
Rojas and colleagues have presented an exciting paper demonstrating yet another relevant preclinical setting in which the mesenchymal stromal cell has a potential therapeutic application. What is particularly interesting about this study is that it addresses a disease, blood-borne systemic sepsis, which has multiple complex host responses and involves a variety of disparate organs and immune cell types. Here, the authors focus on how this injury relates more specifically to the lung, with quite dramatic improvements in several assessed injury parameters...
2014: Stem Cell Research & Therapy
Tracy R McKnight, Hikari A I Yoshihara, Lungile J Sitole, Jeffery N Martin, Francois Steffens, Debra Meyer
Individuals infected with the human immunodeficiency virus (HIV) often suffer from concomitant metabolic complications. Treatment with antiretroviral therapy has also been shown to alter the metabolism of patients. Although chemometric analysis of nuclear magnetic resonance (NMR) spectra of human sera can distinguish normal sera (HIVneg) from HIV-infected sera (HIVpos) and sera from HIV-infected patients on antiretroviral therapy (ART), quantitative analysis of the discriminating metabolites and their relationship to disease status has yet to be determined...
November 2014: Molecular BioSystems
Annette Joosten, Sonya Girdler, Matthew A Albrecht, Chiara Horlin, Marita Falkmer, Denise Leung, Anna Ordqvist, Håkan Fleischer, Torbjörn Falkmer
OBJECTIVE: To examine visual search patterns and strategies used by children with and without Asperger syndrome/high functioning autism (AS/HFA) while watching a magic trick. Limited responsivity to gaze cues is hypothesised to contribute to social deficits in children with AS/HFA. METHODS: Twenty-one children with AS/HFA and 31 matched peers viewed a video of a gaze-cued magic trick twice. Between the viewings, they were informed about how the trick was performed...
2016: Developmental Neurorehabilitation
David C Cicero, Elizabeth A Martin, Theresa M Becker, Anna R Docherty, John G Kerns
Despite the common use of either psychometric or clinical methods for identifying individuals at risk for psychosis, previous research has not examined the correspondence and extent of convergence of these 2 approaches. Undergraduates (n = 160), selected from a larger pool, completed 3 self-report schizotypy scales: the Magical Ideation Scale, the Perceptual Aberration Scale, and the Revised Social Anhedonia Scale. They were administered the Structured Interview for Prodromal Syndromes. First, high correlations were observed for self-report and interview-rated psychotic-like experiences (rs between ...
September 2014: Psychological Assessment
Ana Cláudia Mendes do Nascimento, Daniela Barros Cortez Gaspardo, Tatiana Mimura Cortez, Hélio Amante Miot
The authors present a male 40-year-old patient with established diagnosis of Behçet's disease which had evolved to recurrent bilateral auricular polychondritis crises. MAGIC syndrome (mouth and genital ulcers with inflamed cartilage) is rare and groups together patients with this clinical picture without necessarily fulfilling the clinical criteria for Behçet's disease or relapsing polychondritis, demonstrating an independent disorder.
January 2014: Anais Brasileiros de Dermatologia
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