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W Landon Jackson
The alpha-gal allergy is an emerging IgE-mediated reaction against the galactose-alpha-1,3-galactose carbohydrate found in mammalian meats. Patients with this condition will develop anaphylactic symptoms 3-6 h after the ingestion of mammalian meat food products such as beef, pork or lamb. The prevalence of this allergy is drastically increasing and severe reactions including anaphylactic shock have been reported, yet many patients experience symptoms for years before a diagnosis is made. We describe the presentation, diagnosis and management of a patient with the alpha-gal allergy in attempt to improve early recognition and management of patients with this condition...
February 2018: Oxford Medical Case Reports
Anukul Taweechaipaisankul, Geon A Kim, Jun-Xue Jin, Su Cheong Yeom, Byeong Chun Lee
Due to their close similarities with humans in anatomy, physiology and genetics and handling advantages, miniature pigs are becoming a very attractive model for biomedical research. The purpose of this study was to establish and evaluate blood type O cells derived from Korean native pig (KNP), a typical miniature pig breed in Korea. Total 10 cell lines derived from 8 KNP piglets, adult KNP female pig (kidney and ear tissues) were established. To confirm the blood type O, genomic DNA, fucosyltransferase (FUT) expression and immunofluorescence staining were examined...
February 27, 2018: Journal of Veterinary Science
Aila Daugs, Nadine Lehmann, Dilem Eroglu, Martina Meinke, Annina Markhoff, Oliver Bloch
Tissue antigenicity represents the main limitation for the use of xenografts in clinical practice. To eliminate xenoantigens and avoid graft rejection in human, decellularization is often used to remove all immunoreactive components from the extracellular matrix (ECM). After decellularization, acellular scaffolds are required to be investigated regarding the presence of antigens, but commonly used detection methods solely focus on known xenoantigens such as alpha Gal (Galα1,3-Galβ1-4GlcNAc-R) or major histocompatibility complex-I (MHC-I) However, there are unknown xenoantigens, which escape the standard methods...
February 24, 2018: Tissue Engineering. Part C, Methods
Lorenzo Ferri, Duccio Malesci, Antonella Fioravanti, Gaia Bagordo, Armando Filippini, Anna Ficcadenti, Raffaele Manna, Daniela Antuzzi, Elena Verrecchia, Ilaria Donati, Renzo Mignani, Catia Cavicchi, Renzo Guerrini, Amelia Morrone
BACKGROUND: Allelic heterogeneity is an important feature of the GLA gene for which almost 900 known genetic variants have been discovered so far. Pathogenetic GLA variants cause alpha-galactosidase A (α-Gal A) enzyme deficiency leading to the X-linked lysosomal storage disorder Fabry disease (FD). Benign GLA intronic and exonic variants (e.g. pseudodeficient p.Asp313Tyr) have also been described. Some GLA missense variants, previously deemed to be pathogenetic (e.g. p.Glu66Gln and p...
February 21, 2018: Clinica Chimica Acta; International Journal of Clinical Chemistry
Yasmin S Abulfadl, Nabila N El-Maraghy, Amany Ali Eissa Ahmed, Shahira Nofal, Osama A Badary
OBJECTIVES: Thymoquinone (TQ), the main active ingredient in Nigella sativa oil, exhibits various bioactivities. This study aimed to investigate the effect of TQ on neurobehavioral and neuropathological alterations induced by aluminum trichloride (AlCl3 ) and D-galactose (D-gal)-in male rats and to explore the related mechanisms. METHODS: D-gal (60 mg/kg day) and AlCl3 (10 mg/kg day) were given intraperitoneally (i.p.) once daily for 42 days and after 4 weeks TQ was concomitantly administered intragastrically (i...
February 21, 2018: Neurological Research
Y S Abulfadl, N N El-Maraghy, Aa Eissa Ahmed, S Nofal, Y Abdel-Mottaleb, O A Badary
Alzheimer's disease (AD) is characterized by a robust inflammatory response elicited by the accumulation and deposition of amyloid-β (Aβ) within the brain. Aβ induces detrimental inflammatory responses through toll-like receptors (TLRs) signaling pathway. Thymoquinone (TQ), the main active constituent of Nigella sativa oil, has been reported by several previous studies for its potent anti-inflammatory effect. The aim of this study is to elucidate the effect of TQ in improving learning and memory, using a rat model of AD induced by a combination of aluminum chloride (AlCl 3 ) and d-galactose (d-Gal)...
January 1, 2018: Human & Experimental Toxicology
Ahmed Atwa, Rehab Hegazy, Rania Mohsen, Neamat Yassin, Sanaa Kenawy
BACKGROUND: Renal dysfunction is very common in patients with advanced liver cirrhosis and portal hypertension. The development of renal failure in the absence of clinical, anatomical or pathological causes renal of failure is termed hepatorenal syndrome (HRS). AIM: The present study was constructed to investigate the possible protective effects of nebivolol (Nebi) against D-galactosamine (Gal)-induced HRS in rats. MATERIAL AND METHODS: Rats were treated with Nebi for ten successive days...
December 15, 2017: Open Access Macedonian Journal of Medical Sciences
C G Uasuf, A Torina, V Ferrantelli, I Brusca
We report the case of a 38-year-old man who was bitten several times during his life by a tick. He didn't report any previous history of anaphylaxis after the ingestion of red meat. The serum specific IgE showed positivity to α-Gal. The proximity of the bits didn't increase the titer of IgE antibodies to alpha-gal. We could hypothesize that the frequency of the exposure to the tick Corresponding author bites and the amount of tick bites during his lifetime induced a sort of tolerance in this patient.
January 2018: European Annals of Allergy and Clinical Immunology
Hideo Hashizume, Toshiharu Fujiyama, Takatsune Umayahara, Reiko Kageyama, Andrew F Walls, Takahiro Satoh
BACKGROUND: Alpha gal syndrome is a hypersensitivity reaction to red meat mediated by specific IgE antibody to galactose-α-1,3-galactose carbohydrate (alpha gal). Amblyomma tick bites are associated with this condition although the pathophysiology is not understood. OBJECTIVE: To clarify the mechanism of development of alpha gal syndrome after tick bites. METHODS: We compared alpha gal antibodies between patients with and without a history of tick bites and examined the histology of tick bite lesions between patients with and without detectable anti-alpha-gal IgE antibody...
December 19, 2017: Journal of the American Academy of Dermatology
M Venturini, T Lobera, A Sebastián, A Portillo, J A Oteo
BACKGROUND AND OBJECTIVE: To investigate the prevalence and associated factors to the presence of alpha-gal-specific IgE in a risk group of foresters and forest workers from La Rioja and in a control group. METHODS: A total of 169 workers and 100 individuals who did not recall tick-bites were selected. A questionnaire including demographic data and number of tick bites per year was administered by a physician. Alpha-gal sIgE was assessed with serum samples that had been taken in 2010 using ImmunoCAP®...
December 13, 2017: Journal of Investigational Allergology & Clinical Immunology
Bojana Zupan, Bingfang Liu, Faten Taki, Judit Gal Toth, Miklos Toth
Tumor necrosis factor alpha (TNF-α) is a cytokine that not only coordinates local and systemic immune responses [1, 2] but also regulates neuronal functions. Most prominently, glia-derived TNF-α has been shown to regulate homeostatic synaptic scaling [3-6], but TNF-α-null mice exhibited no apparent cognitive or emotional abnormalities. Instead, we found a TNF-α-dependent intergenerational effect, as mothers with a deficit in TNF-α programmed their offspring to exhibit low innate fear. Cross-fostering and conditional knockout experiments indicated that a TNF-α deficit in the maternal brain, rather than in the hematopoietic system, and during gestation was responsible for the low-fear offspring phenotype...
December 18, 2017: Current Biology: CB
Michael P Nelson, Michel Boutin, Tonia E Tse, Hailin Lu, Emily D Haley, Xiaosen Ouyang, Jianhua Zhang, Christiane Auray-Blais, John J Shacka
The aberrant accumulation of alpha-synuclein (α-syn) is believed to contribute to the onset and pathogenesis of Parkinson's disease (PD). The autophagy-lysosome pathway (ALP) is responsible for the high capacity clearance of α-syn. ALP dysfunction is documented in PD and pre-clinical evidence suggests that inhibiting the ALP promotes the pathological accumulation of α-syn. We previously identified the pathological accumulation of α-syn in the brains of mice deficient for the soluble lysosomal enzyme alpha-Galactosidase A (α-Gal A), a member of the glycosphingolipid metabolism pathway...
February 2018: Neurobiology of Disease
Renuka Kandhaya-Pillai, Francesc Miro-Mur, Jaume Alijotas-Reig, Tamara Tchkonia, James L Kirkland, Simo Schwartz
Cellular senescence is a cell fate program that entails essentially irreversible proliferative arrest in response to damage signals. Tumor necrosis factor-alpha (TNFα), an important pro-inflammatory cytokine secreted by some types of senescent cells, can induce senescence in mouse and human cells. However, downstream signaling pathways linking TNFα-related inflammation to senescence are not fully characterized. Using human umbilical vein endothelial cells (HUVECs) as a model, we show that TNFα induces permanent growth arrest and increases p21CIP1, p16INK4A, and SA-β-gal, accompanied by persistent DNA damage and ROS production...
November 22, 2017: Aging
M C Carter, K N Ruiz-Esteves, L Workman, P Lieberman, T A E Platts-Mills, D D Metcalfe
IgE antibodies (Ab) specific to galactose-α-1,3-galactose (alpha-gal) are responsible for a delayed form of anaphylaxis that occurs 3-6 hours after red meat ingestion. In a unique prospective study of seventy participants referred with a diagnosis of idiopathic anaphylaxis (IA), six (9%) were found to have IgE to alpha-gal. Upon institution of a diet free of red meat, all patients had no further episodes of anaphylaxis. Two of these individuals had indolent systemic mastocytosis (ISM). Those with ISM had more severe clinical reactions but lower specific IgE to alpha-gal and higher serum tryptase levels, reflective of the mast cell burden...
November 21, 2017: Allergy
Eduardo Sucupira, Miguel Sabino, Edson Luiz de Lima, Gal Moreira Dini, Maria José Azevedo de Brito, Lydia Masako Ferreira
CONTEXT AND OBJECTIVE: Patient-reported outcome measurements assessing the emotional state of children and adolescents who seek plastic surgery are important for determining whether the intervention is indicated or not. The aim of this study was to cross-culturally adapt and validate the Short Mood and Feelings Questionnaire (child/adolescent and parent versions) for Brazilian Portuguese, test its psychometric properties and assess the emotional state of children and adolescents who seek plastic surgery...
November 6, 2017: São Paulo Medical Journal, Revista Paulista de Medicina
Michel Boutin, Iskren Menkovic, Tristan Martineau, Vanessa Vaillancourt-Lavigueur, Amanda Toupin, Christiane Auray-Blais
Fabry disease is an X-linked lysosomal storage disorder caused by α-galactosidase A (α-GAL A) deficiency. This enzyme contributes to the cellular recycling of glycosphingolipids such as galabiosylceramide (Ga2 ), globotriaosylceramide (Gb3 ), and globotriaosylsphingosine (lyso-Gb3 ) by hydrolyzing the terminal α-galactosyl moiety. Urine and plasma α-GAL A substrates are currently analyzed as biomarkers for the detection, monitoring, and follow-up of Fabry disease patients. The sensitivity of the analysis of Ga2 is decreased by the co-analysis of its structural isomer, lactosylceramide (LacCer), which is not an α-GAL A substrate...
December 19, 2017: Analytical Chemistry
Joe Kevin Khoury, Neil Christian Khoury, Deborah Schaefer, Anup Chitnis, Getaw Worku Hassen
Allergic reaction is a common clinical picture in the Emergency Department (ED). Most allergic reactions are from food or drugs. A detailed history is an integral aspect of determining the causative agent of an allergy. Galactose-alpha-1,3-galactose (alpha-gal) allergy is a tick-acquired red meat allergy that causes delayed-onset allergic reaction or anaphylaxis due to molecular mimicry. Alpha-gal allergy may not be widely known as a cause of allergic reactions. Lack of universal awareness of this phenomenon in the ED and Urgent Care setting could lead to misdiagnosis, or delayed diagnosis...
October 16, 2017: American Journal of Emergency Medicine
Filippo Naso, Alessandro Gandaglia
Xenogeneic decellularized heart valve scaffolds have the potential to overcome the limitations of existing bioprosthetic heart valves that have limited duration due to calcification and tissue degeneration phenomena. This article presents a review of 30 years of decellularization approaches adopted in cardiovascular tissue engineering, with a focus on the use, either individually or in combination, of different detergents. The safety and efficacy of cell-removal procedures are specifically reported and discussed, as well as the structure and biomechanics of the treated extracellular matrix (ECM)...
October 22, 2017: Xenotransplantation
Filippo Naso, Ugo Stefanelli, Edward Buratto, Giovanna Lazzari, Andrea Perota, Cesare Galli, Alessandro Gandaglia
BACKGROUND: Glutaraldehyde (GLA) has been used to crosslink bioprosthetic heart valve (BHVs) tissues to enhance their stability, besides ensuring a satisfactory degree of immunological tolerance. Unfortunately, GLA fixation does not guarantee a complete tissue biocompatibility of BHVs in currently used devices. The interaction between preformed human anti-alpha-Gal antibody and alpha-Gal antigens promotes the calcification of GLA-treated alpha-Gal-positive tissue. Recently, an alarming correlation between the presence of the alpha-Gal epitope and a premature BHVs degeneracy was reported...
October 2017: Tissue Engineering. Part A
Gilson Biagini, Ana Clara Simões Flórido Almeida, Tammy Vernalha Rocha Almeida, Cassiano Augusto Braga Silva, Bruna Fernanda de Castro, Tais Cristina Reche, Ana Cláudia Dabinski, Fellype Carvalho Barreto
Fabry disease is an X-linked lysosomal storage disease due to alpha-galactosidase A (α-Gal A) deficient activity which leads to the accumulation of glucoesphingolipids, such as globotriaosilceramide. There are over 700 known mutations of the enzyme gene, and most of them cause Fabry Disease. This case report describes a hemodialysis patient with a rare and controversial GLA gene mutation, the D313Y. The medecial investigation confirmed that D313Y is an alpha-galactosidase A sequence variant that causes pseudo deficient enzyme activity in plasma but not Fabry disease...
July 2017: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
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