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Liaoran Wang, David K C Cooper, Lars Burdorf, Yi Wang, Hayato Iwase
There has recently been considerable progress in the results of pig organ transplantation in nonhuman primates (NHPs), largely associated with the availability of (i) pigs genetically-engineered to overcome coagulation dysregulation, and (ii) novel immunosuppressive agents. The barriers of thrombotic microangiopathy and/or consumptive coagulation were believed to be associated with (i) activation of the graft vascular endothelial cells (VECs) by a low level of anti-pig antibody binding and/or complement deposition and/or innate immune cell activity, and (ii) molecular incompatibilities between the NHP and pig coagulation-anticoagulation systems...
March 10, 2018: Transplantation
Samuel Romero, Amparo Sempere, Inés Gómez-Seguí, Elena Román, Andrés Moret, Rosa Jannone, Iván Moreno, Santiago Mendizábal, Jordi Espí, Ana Peris, Rafael Carbonell, José Cervera, Javier Pemán, Santiago Bonanad, Javier de la Rubia, Isidro Jarque
BACKGROUND AND AIM: The term thrombotic microangiopathy (TMA) involves a heterogeneous group of diseases that can be overwhelming or invalidating, with an acute development, characterised by microangiopathic haemolytic anaemia and thrombocytopaenia. Its management during its initial hours is essential to improving the prognostic of these patients. The aim of this review is to give recommendations about the optimisation of TMA initial treatment and to accelerate the aetiological diagnosis...
March 10, 2018: Medicina Clínica
Agnieszka E Zawada, Małgorzata Moszak, Dorota Skrzypczak, Marian Grzymisławski
Diabetes mellitus is a systemic disease which affects patients of various age. Hyperglycemia induces damage of vascular endothelium, development of chronic inflammation, organic and functional lesions in several systems and organs. The principal gastroenterological complaints linked to the manifestation of the disease include abdominal pain, diarrhea, nausea, flatulence, and vomiting. However, complications in the alimentary system may manifest exclusively by difficulties in reaching normoglycemia and numerous persistent episodes of hypoglycemia...
March 13, 2018: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
B Handan Özdemir, Alev Ok Atılgan, Eda Yılmaz Akçay, Gökçe Özdemir, Ebru Ayvazoğlu Soy, Aydıncan Akdur, Mehmet Haberal
OBJECTIVES: Thrombotic microangiopathy is a form of renal capillary injury possibly associated with calcineurin inhibitor toxicity, acute humoral rejection, infections, and recurrent diseases. Here, we examined its incidence in patients diagnosed with acute and chronic active humoral rejection, polyomavirus nephropathy, acute cellular rejection, and immunoglobulin A recurrence. MATERIALS AND METHODS: In total, 272 renal allograft recipients who met the inclusion criteria were reevaluated for presence of renal thrombotic microangiopathy...
March 2018: Experimental and Clinical Transplantation
Yuka Matsunaga, Masataka Ishimura, Hazumu Nagata, Kiyoshi Uike, Tadamune Kinjo, Masayuki Ochiai, Kenichiro Yamamura, Hidetoshi Takada, Yoshihisa Tanoue, Masaki Hayakawa, Masanori Matsumoto, Toshiro Hara, Shouichi Ohga
BACKGROUND: Thrombotic microangiopathies (TMA) are microvascular occlusive disorders characterized by systemic or intrarenal platelet aggregation, thrombocytopenia, and red cell fragmentation. Post-operative TMA mostly occurs in adult patients with cardiovascular surgery, with the distinct pathophysiology from classical thrombotic thrombocytopenic purpura (TTP) although the exact pathophysiology remains unclear. CASE PRESENTATION: A one-month-old infant developed TMA after the initial surgery of double outlet right ventricle...
February 7, 2018: Pediatrics and Neonatology
Francesco Saverio Sorrentino, Silvia Matteini, Claudio Bonifazzi, Adolfo Sebastiani, Francesco Parmeggiani
In the face of the global epidemic of diabetes, it is critical that we update our knowledge about the pathogenesis of diabetes and the related micro alterations on the vascular network in the body. This may ultimately lead to early diagnosis and novel treatment options for delaying the progression of diabetic complications. Research has recently revealed the pivotal role of endothelin in the pathogenesis of diabetic complications, particularly in the regulation of the capillary flow, which is affected in the course of retinopathy...
March 9, 2018: Eye
Chisa Fukasawa, Saori Ooishi, Takuma Kumagai, Megumi Koshiisi, Yuki Sueki, Kei Nakajima, Toru Mitsumori, Yoko Yoshida, Hideki Kato, Masaomi Nangaku, Toshiyuki Miyata, Keita Kirito
Herein, we present an elderly onset case of aHUS successfully treated with eculizumab. An 80-year-old woman with severe anemia, thrombocytopenia, and acute renal dysfunction was admitted to our hospital. A laboratory test revealed steep elevation in the LDH level, and the peripheral blood smear showed erythrocyte fragmentations. Accordingly, we diagnosed thrombotic microangiopathy, and treatment with plasma exchange was immediately initiated. In addition, she required hemodialysis because of rapid impairment of the renal function...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Motohiko Okano, Takeshi Matsumoto, Yoshiki Nakamori, Kazuko Ino, Kana Miyazaki, Atsushi Fujieda, Yuka Sugimoto, Isao Tawara, Motoko Yamaguchi, Kohshi Ohishi, Hiroshi Miwa, Masahiro Masuya, Hideo Wada, Naoyuki Katayama
A 23-year-old man from Mie Prefecture, Japan, with past and family history of hematuria was diagnosed with influenza A and admitted to our hospital on the following day because of hemoglobinuria. He was diagnosed with thrombotic microangiopathy and was suspected of having atypical hemolytic uremic syndrome (aHUS). C3 p.I1157T missense mutation, which we had previously reported in eight aHUS patients from six families in Mie Prefecture, was identified. The laboratory findings and symptoms of our patient promptly improved after administering eculizumab...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Christian Patry, Christian Betzen, Farnoosh Fathalizadeh, Alexander Fichtner, Jens H Westhoff, Thomas Fleming, Volker Eckstein, Tom Bruckner, Martina Bielaszewska, Helge Karch, Georg F Hoffmann, Burkhard Tönshoff, Neysan Rafat
Endothelial injury with consecutive microangiopathy and endothelial dysfunction plays a central role in the pathogenesis of the post-enteropathic hemolytic uremic syndrome (D+HUS). To identify new treatment strategies, we examined the regenerative potential of endothelial progenitor cells (EPC) in an in vitro model of Shiga toxin (Stx) 2a-induced glomerular endothelial injury present in D+HUS and the mechanisms of EPC-triggered endothelial regeneration. We simulated the pro-inflammatory milieu present in D+HUS by priming human renal glomerular endothelial cells (HRGEC) with Tumor Necrosis Factor (TNF)-α prior to stimulation with Stx2a...
March 7, 2018: American Journal of Physiology. Renal Physiology
Serkan Yıldız, Fatih Demirkan
Thrombotic microangiopathies (TMAs) are disorders characterized by endothelial cell activation, microangiopathic hemolytic anemia, thrombocytopenia and organ failure of variable intensity. The pathophysiology of various types of TMAs have become an interesting field of study. Alternative complement system activation plays an important role in several pathophysiological conditions. Complement activation is also described in an increasing number of TMAs. Inherited defects in complement regulatory genes and acquired autoantibodies against complement regulatory proteins have been described...
February 21, 2018: Transfusion and Apheresis Science
Tatsuyuki Sato, Masaya Mori, Jiro Aoki, Kengo Tanabe
Pulmonary tumor thrombotic microangiopathy (PTTM) is a fatal cancer-related complication characterized by severe progressive pulmonary hypertension. Antemortem diagnosis is difficult owing to the rapid progression of the condition, especially when the patient has no known malignancies and initially presents with pulmonary hypertension. Here we report a case of PTTM due to occult gastric cancer with metastasis in the left supraclavicular lymph node, also known as Virchow's node. Enlarged Virchow's node is an important indicator of advanced gastric cancer...
March 5, 2018: International Heart Journal
Emre Tekgündüz, Mehmet Yılmaz, Mehmet Ali Erkurt, Ilhami Kiki, Ali Hakan Kaya, Leylagul Kaynar, Inci Alacacioglu, Guven Cetin, Ibrahim Ozarslan, Irfan Kuku, Gulden Sincan, Ozan Salim, Sinem Namdaroglu, Abdullah Karakus, Volkan Karakus, Fevzi Altuntas, Ismail Sari, Gulsum Ozet, Ismet Aydogdu, Vahap Okan, Emin Kaya, Rahsan Yildirim, Esra Yildizhan, Gokhan Ozgur, Osman Ilhami Ozcebe, Bahriye Payzin, Seval Akpinar, Fatih Demirkan
Thrombotic microangiopathies (TMAs) are rare, but life-threatening disorders characterized by microangiopathic hemolytic anemia and thrombocytopenia (MAHAT) associated with multiorgan dysfunction as a result of microvascular thrombosis and tissue ischemia. The differentiation of the etiology is of utmost importance as the pathophysiological basis will dictate the choice of appropriate treatment. We retrospectively evaluated 154 (99 females and 55 males) patients who received therapeutic plasma exchange (TPE) due to a presumptive diagnosis of TMA, who had serum ADAMTS13 activity/anti-ADAMTS13 antibody analysis at the time of hospital admission...
February 20, 2018: Transfusion and Apheresis Science
Lauren J Lee, Kristine J Roland, Gayatri M Sreenivasan, Leslie N Zypchen, Kimberley L S Ambler, Paul R Yenson
Solvent detergent-treated plasma (SDP) is a pathogen-inactivated blood plasma, which in comparison to frozen plasma is associated with lower rates of allergic reaction, transfusion-associated lung injury, and viral transmission. SDP has been available in Canada since 2012. Data on SDP use in Canada remains limited. We present a review of subjects receiving SDP at a large tertiary care centre primarily for thrombotic thrombocytopenic purpura and atypical hemolytic uremic syndrome, demonstrating the tolerability and safety of SDP...
February 15, 2018: Transfusion and Apheresis Science
Sonja G Karst, Gabor G Deak, Bianca S Gerendas, Sebastian M Waldstein, Jan Lammer, Christian Simader, Tadhg Guerin, Ursula M Schmidt-Erfurth
Importance: Anti-vascular endothelial growth factor treatment is the first-line therapy in the treatment of center-involving diabetic macular edema. Data on capillary perfusion changes under repeated treatment in a possibly compromised vascular network are limited. Objective: To evaluate the association of repeated ranibizumab injections on macular perfusion in patients with diabetic macular edema. Design, Setting, and Participants: This study analyzed prospectively collected data from the 12-month RESTORE core study and the 24-month open label RESTORE extension study, which assessed the efficacy and safety of ranibizumab in patients with visual impairment due to diabetic macular edema...
March 1, 2018: JAMA Ophthalmology
Kristin Samuelsson, Rayomand Press
Chronic idiopathic axonal polyneuropathy (CIAP) is a slowly progressive predominantly sensory axonal polyneuropathy. The prevalence of CIAP increases with age. The pathogenic cause of CIAP is unknown although there are several prevailing etiological hypotheses. In this mini review, we focus on the hypothesis of disturbed microcirculation in the vasa nervorum of peripheral nerves as a pathogenic cause of CIAP. There is an association between CIAP and metabolic risk factors. Furthermore, the phenotype of CIAP resembles diabetic neuropathy both clinically and electrophysiologically...
2018: Frontiers in Neurology
Adrien Picod, Agnès Bonnin, Giorgia Battipaglia, Federica Giannotti, Annalisa Ruggeri, Eolia Brissot, Florent Malard, Clémence Médiavilla, Ramdane Belhocine, Anne Vekhoff, Mor Sény Gueye, Simona Lapusan, Rosa Adaeva, Françoise Isnard, Ollivier Legrand, Minh-Tam Baylatry, Anne-Christine Joly, Myriam Labopin, Rémy Duléry, Mohamad Mohty
Sinusoidal obstruction syndrome (SOS), also known as hepatic veno-occlusive disease (VOD) is a serious complication after hematopoietic stem cell transplantation (HSCT). SOS/VOD usually occurs within 3 weeks of HSCT but the 2016 EBMT diagnosis criteria have been revised to include late forms. Prophylactic use of defibrotide is recommended in the pediatric setting but its value remains uncertain in the adult population. We report here a single center series of 63 adult patients considered at high risk of SOS/VOD, receiving defibrotide prophylaxis in combination with ursodeoxycholic acid between May 2012 and August 2016...
February 22, 2018: Biology of Blood and Marrow Transplantation
Esra Terzi Demirsoy, Ozgur Mehtap, Elif Birtas Atesoglu, Pinar Tarkun, Necmi Eren, Ayfer Gedük, Abdullah Hacihanefioglu
Most commonly seen side effects with Tyrosine kinase inhibitors (TKI's) are hematologic toxicities. Besides, with dasatinib autoimmune side effects can be seen. Thrombotic thrombocytopenic purpura (TTP) is a life- threatening disease that can be related to various causes mainly autoimmune disorders or antineoplastic drugs. Few cases of TKI associated secondary thrombotic microangiopathies (TMA) have been reported in literature. Most of cases were diagnosed as hemolytic uremic syndrome (HUS) rather than TTP...
February 9, 2018: Transfusion and Apheresis Science
Sidse Kjærhus Nørgaard, Marianne Jenlev Vestgaard, Isabella Lindegaard Jørgensen, Björg Ásbjörnsdóttir, Lene Ringholm, Harold David McIntyre, Peter Damm, Elisabeth Reinhardt Mathiesen
AIMS: To identify early clinical, modifiable risk factors for preeclampsia present at first antenatal visit and assess the prevalence of pregnancy-related hypertensive disorders in women with pre-existing diabetes treated with tight glycemic and blood pressure (BP) control. METHODS: A population-based cohort study of 494 women with pre-existing diabetes (307 and 187 women with type 1 and type 2 diabetes, respectively), included at their first antenatal visit from 2012-16...
February 20, 2018: Diabetes Research and Clinical Practice
Kevin Miller, Jorge A Fortun
Diabetic retinopathy and diabetic macular edema comprise a major source of visual disability throughout the developed world. The etiology and pathogenesis of macular edema is intricate and multifactorial, in which the hyperglycemic state in diabetes induces a microangiopathy. Through several inflammatory and vasogenic mediators, including vascular endothelial growth factor (VEGF) upregulation and inflammatory cytokines and chemokines, pathologic changes are induced in the vascular endothelium triggering breakdown of the blood retinal barrier, causing extravasation of fluid into the extracellular space and manifesting clinically as macular edema, resulting in visual loss...
January 2018: Asia-Pacific Journal of Ophthalmology
Muhammad Bilal, Muhammad Sarfaraz Iqbal, Syed Bilal Shah, Tahir Rasheed, Hafiz M N Iqbal
BACKGROUND: The naturally inspired treatment options for several disease conditions and human-health related disorders such as diabetes mellitus have gained considerable research interest. In this context, naturally occurring plants and herbs with medicinal functionalities have gained special place than ever before in the current medicinal world. OBJECTIVE: The objective of this review is to extend the current knowledge in the clinical field related to the diabetic complications...
February 21, 2018: Recent Patents on Inflammation & Allergy Drug Discovery
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