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https://www.readbyqxmd.com/read/28340492/hemolysis-elevated-liver-enzymes-low-platelets-syndrome-superimposed-on-hemolytic-uremic-syndrome
#1
Inês Martins, Madalena Gomes Conceição, Paulo Pereira Gomes, Nuno Clode
A pregnancy complicated by typical hemolytic uremic syndrome (HUS) and hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome is reported. At 20 weeks of gestation, a case of HUS was diagnosed, with Shiga toxin-producing Escherichia coli identified. Plasmapheresis allowed continuation of the pregnancy for 5 weeks. Superimposed preeclampsia and HELLP syndrome were diagnosed after the establishment of nephrotic range proteinuria, hypertension and recurrence of hemolysis. This is a singular case, as it demonstrates that HELLP syndrome can superimpose upon HUS, a fact that can impact future research on reproductive immunology...
March 24, 2017: Revista Brasileira de Ginecologia e Obstetrícia
https://www.readbyqxmd.com/read/28339660/eculizumab-in-secondary-atypical-haemolytic-uraemic-syndrome
#2
Teresa Cavero, Cristina Rabasco, Antía López, Elena Román, Ana Ávila, Ángel Sevillano, Ana Huerta, Jorge Rojas-Rivera, Carolina Fuentes, Miquel Blasco, Ana Jarque, Alba García, Santiago Mendizabal, Eva Gavela, Manuel Macía, Luis F Quintana, Ana María Romera, Josefa Borrego, Emi Arjona, Mario Espinosa, José Portolés, Carolina Gracia-Iguacel, Emilio González-Parra, Pedro Aljama, Enrique Morales, Mercedes Cao, Santiago Rodríguez de Córdoba, Manuel Praga
Background.: Complement dysregulation occurs in thrombotic microangiopathies (TMAs) other than primary atypical haemolytic uraemic syndrome (aHUS). A few of these patients have been reported previously to be successfully treated with eculizumab. Methods.: We identified 29 patients with so-called secondary aHUS who had received eculizumab at 11 Spanish nephrology centres. Primary outcome was TMA resolution, defined by a normalization of platelet count (>150 × 10 9 /L) and haemoglobin, disappearance of all the markers of microangiopathic haemolytic anaemia (MAHA), and improvement of renal function, with a ≥25% reduction of serum creatinine from the onset of eculizumab administration...
February 20, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28333416/severe-transplant-associated-thrombotic-microangiopathy-in-patients-with-hemoglobinopathies
#3
Ghada A Abusin, Rolla Abu-Arja, Rajinder P S Bajwa, Edwin M Horwitz, Jeffery J Auletta, Hemalatha G Rangarajan
Incidence and severity of transplant-associated thrombotic microangiopathy (TA-TMA) in patients with hemoglobinopathies receiving hematopoietic cell transplant is unknown. We report the outcomes for two patients with TA-TMA who received eculizumab. A 2.5-year-old male with sickle cell disease developed TA-TMA-associated pericardial tamponade, severe hypertension, and acute kidney injury 2 months after transplant. A 7-year-old female with β-thalassemia major developed TA-TMA-related acute kidney injury, severe hypertension, and seizures at 6 months after transplant...
March 23, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28328331/st2-and-endothelial-injury-as-a-link-between-gvhd-and-microangiopathy
#4
LETTER
Seth J Rotz, Christopher E Dandoy, Stella M Davies
No abstract text is available yet for this article.
March 23, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28326034/the-presence-of-previous-cerebral-microbleeds-has-a-negative-effect-on-hypertensive-intracerebral-hemorrhage-recovery
#5
Kang Yang, Yulan Feng, JinJin Mu, Ningzhen Fu, Shufen Chen, Yi Fu
Background and Purpose: Cerebral microbleeds are an intracerebral microangiopathy with bleeding tendency found in intracerebral hemorrhage patients. However, studies about cerebral microbleed effects on the prognosis of hypertensive intracerebral hemorrhage patients are rare. We performed a prospective study to discuss not only the risk factors of cerebral microbleed incidence in hypertensive intracerebral hemorrhage patients but also the relevance of cerebral microbleeds with silent brain infarction, hemorrhage and prognosis...
2017: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/28325251/interferon-induced-thrombotic-microangiopathy-tma-analysis-and-concise-review
#6
REVIEW
Ajay Kundra, Jen Chin Wang
Interferon (IFN) has been associated with development of thrombotic microangiopathy including thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). We reviewed literature from the earliest reported association in 1993, to July 2016 and found 68 cases. Analysis of this data shows: (1) Mean age at diagnosis was 47 years (95% CI, 44-50). (2) Majority of cases were seen where IFN was used for the treatment of chronic myelogenous leukemia (CML), multiple sclerosis (MS), chronic hepatitis C virus infection (HCV) and one case each for hairy cell leukemia (HCL) and Sezary syndrome...
April 2017: Critical Reviews in Oncology/hematology
https://www.readbyqxmd.com/read/28321601/late-onset-melas-with-m-3243a%C3%A2-%C3%A2-g-mutation-and-its-association-with-aneurysm-formation
#7
Kun Zhu, Shuang Li, Huan Chen, Yao Wang, Miao Yu, Hongyan Wang, Weijie Zhao, Yunpeng Cao
We reported a 53-year-old with late-onset mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) accompanied by aneurysm and large vessel dilations. Most studies have focused on microangiopathy causing stroke-like episodes. We report a case to describe large vessel involvement in clinical considerations, and possible mechanisms of aneurysm formation. We recommended regular angiographic examination for patients with MELAS.
March 21, 2017: Metabolic Brain Disease
https://www.readbyqxmd.com/read/28303689/non-healing-ischaemic-digital-ulcer-in-a-systemic-sclerosis-patient-a-challenging-clinical-case
#8
Sophie Blaise, Matthieu Roustit, Alexandra Forli, Bernard Imbert, Jean-Luc Cracowski
Ischaemic digital ulcers (DUs) are an indicator of the severity of the microangiopathy in patients with systemic sclerosis (SSc). DUs are a frequent complication, affecting about 50% of patients with SSc, and are often recurrent. In cross-sectional studies involving patients with SSc, the frequency of ischaemic DUs was 12-16% with a major impact on hand function and quality of life. Effective therapy for DUs remains elusive. Intravenous iloprost has been demonstrated to have a positive effect on healing of active DUs...
March 16, 2017: International Wound Journal
https://www.readbyqxmd.com/read/28303518/potentially-life-threatening-coagulopathy-associated-with-simultaneous-reduction-in-coagulation-and-fibrinolytic-function-in-pediatric-acute-leukemia-after-hematopoietic-stem-cell-transplantation
#9
Takashi Ishihara, Keiji Nogami, Tomoko Matsumoto, Akitaka Nomura, Yasufumi Takeshita, Satoshi Ochi, Midori Shima
The pathogenesis of sinusoidal obstruction syndrome (SOS) and thrombotic microangiopathy (TMA) after hematopoietic stem cell transplantation (HSCT) is poorly understood, and limited information is available on global hemostatic function in HSCT. We assessed changes in coagulation and fibrinolysis using a simultaneous thrombin and plasmin generation assay (T/P-GA) during HSCT. Measurements of endogenous thrombin potential (T-EP) and plasmin peak height (P-Peak) using T/P-GA in six pediatric acute leukemia patients treated with HSCT were compared to normal plasma...
March 16, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28297006/microangiopathy-arterial-stiffness-and-risk-stratification-in-patients-with-type-2-diabetes
#10
Alexios S Antonopoulos, Gerasimos Siasos, Dimitris Tousoulis
No abstract text is available yet for this article.
March 15, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/28296995/microangiopathy-arterial-stiffness-and-risk-stratification-in-patients-with-type-2-diabetes-reply
#11
Benjamin M Scirica, Eugene Braunwald, Deepak L Bhatt
No abstract text is available yet for this article.
March 15, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/28292995/-a-case-of-stage-iv-breast-cancer-with-long-term-survival-by-chemotherapy-developing-pulmonary-tumor-thrombotic-microangiopathy-during-the-treatment-course
#12
Kana Kobayashi, Akari Murakami, Saaya Komatsu, Kanako Nishiyama, Michiko Yamashita, Wakana Sugimori, Yoshiaki Kamei, Hiroaki Asai, Akiyoshi Ogimoto, Kazuo Honda, Yasutsugu Takada
Pulmonary tumor thrombotic microangiopathy(PTTM)caused by pulmonary artery microscopic tumor emboli and fibrocellular and/or fibromuscular proliferation leads to progressive pulmonary hypertension and respiratory failure.The prognosis is extremely poor and most patients die shortly after onset.We report a patient with Stage IV breast cancer and long-term survival who developed PTTM during chemotherapy treatment.A 63-year-old woman with multiple metastases in her cerebellum, bone, lung, and lymph node after left breast conserving surgery started to experience dyspnea and malaise 7 years after the surgery...
March 2017: Gan to Kagaku Ryoho. Cancer & Chemotherapy
https://www.readbyqxmd.com/read/28291506/immunotactoid-glomerulopathy-leading-to-the-discovery-of-poems-syndrome%C3%A2
#13
Carole Philipponnet, Jean-Louis Kemeny, Cyril Garrouste, Martin Soubrier, Anne-Elisabeth Heng
Monoclonal gammopathy of renal significance (MGRS) can manifest in many different ways depending on the nature of the immunoglobulin and its physicochemical properties. MGRS can lead to the discovery of a hematological malignancy. We report the case of a 32-year-old female patient who underwent renal biopsy on account of an impure nephrotic syndrome associated with immunoglobulin (Ig)G κ monoclonal gammopathy. Histological analysis revealed membranoproliferative glomerulonephritis with IgG, IgM, κ, λ, and C3 deposits...
March 14, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28287636/transplant-associated-thrombotic-microangiopathy-opening-pandora-s-box
#14
REVIEW
E Gavriilaki, I Sakellari, A Anagnostopoulos, R A Brodsky
Transplant-associated thrombotic microangiopathy (TA-TMA) is an early complication of hematopoietic cell transplantation (HCT). A high mortality rate is documented in patients who are refractory to calcineurin inhibitor cessation. Estimates of TA-TMA prevalence vary significantly and are higher in allogeneic compared with autologous HCT. Furthermore, our understanding of the pathophysiology that is strongly related to diagnosis and treatment options is limited. Recent evidence has linked TA-TMA with atypical hemolytic uremic syndrome, a disease of excessive activation of the alternative pathway of complement, opening the Pandora's box in treatment options...
March 13, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28275964/reversal-of-threatening-blindness-after-initiation-of-eculizumab-in-purtscher-like-retinopathy-secondary-to-atypical-hemolytic-uremic-syndrome
#15
J E Ramos de Carvalho, R O Schlingemann, M Oranje, F J Bemelman, M J van Schooneveld
Purtscher-like retinopathy, a rare manifestation of systemic thrombotic microangiopathy, is a potentially visually debilitating condition with no effective proven treatment. Distinct pathogenic pathways have been proposed as etiological factors. We revisit the etiology of Purtscher-like retinopathy based on the rapid response and profound visual improvement after initiation of systemic intravenous eculizumab, an inhibitor of the complement cascade, in a patient with Purtscher-like retinopathy secondary to familial atypical hemolytic uremic syndrome (aHUS) due to a mutation in complement factor H...
March 8, 2017: International Ophthalmology
https://www.readbyqxmd.com/read/28271596/team-based-approach-to-identify-cardiac-toxicity-in-critically-ill-hematopoietic-stem-cell-transplant-recipients
#16
Christopher E Dandoy, Sonata Jodele, Zachary Paff, Russel Hirsch, Thomas D Ryan, John L Jefferies, Michelle Cash, Seth Rotz, Abigail Pate, Michael D Taylor, Javier El-Bietar, Kasiani C Myers, Gregory Wallace, Adam Nelson, Michael Grimley, Thomas Pfeiffer, Adam Lane, Stella M Davies, Ranjit S Chima
INTRODUCTION: We observed pulmonary hypertension (PH), pericardial effusions, and left ventricular systolic dysfunction (LVSD) in multiple critically ill hematopoietic stem cell transplant (HSCT) recipients. We implemented routine structured echocardiography screening for HSCT recipients admitted to the pediatric intensive care unit (PICU) using a standardized multidisciplinary process. METHODS: HSCT recipients admitted to the PICU with respiratory distress, hypoxia, shock, and complications related to transplant-associated thrombotic microangiopathy were screened on admission and every 1-2 weeks thereafter...
March 8, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28265287/pernicious-anemia-associated-cobalamin-deficiency-and-thrombotic-microangiopathy-case-report-and-review-of-the-literature
#17
Farhanah Yousaf, Bruce Spinowitz, Chaim Charytan, Marilyn Galler
A 43-year-old Hispanic male without significant previous medical history was brought to emergency department for syncope following a blood draw to investigate a 40 lbs weight loss during the past 6 months associated with decreased appetite and progressive fatigue. The patient also reported a 1-month history of jaundice. On examination, he was hemodynamically stable and afebrile with pallor and diffuse jaundice but without skin rash or palpable purpura. Normal sensations and power in all extremities were evident on neurological exam...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28259520/derivation-and-external-validation-of-the-plasmic-score-for-rapid-assessment-of-adults-with-thrombotic-microangiopathies-a-cohort-study
#18
Pavan K Bendapudi, Shelley Hurwitz, Ashley Fry, Marisa B Marques, Stephen W Waldo, Ang Li, Lova Sun, Vivek Upadhyay, Ayad Hamdan, Andrew M Brunner, John M Gansner, Srinivas Viswanathan, Richard M Kaufman, Lynne Uhl, Christopher P Stowell, Walter H Dzik, Robert S Makar
BACKGROUND: Among the syndromes characterised by thrombotic microangiopathy, thrombotic thrombocytopenic purpura is distinguished by a severe deficiency in the ADAMTS13 enzyme. Patients with this disorder need urgent treatment with plasma exchange. Because ADAMTS13 activity testing typically requires prolonged turnaround times and might be unavailable in resource-poor settings, a method to rapidly assess the likelihood of severe ADAMTS13 deficiency is needed. METHODS: All consecutive adult patients presenting to three large academic medical centres in Boston, MA, USA, with thrombotic microangiopathy and a possible diagnosis of thrombotic thrombocytopenic purpura between Jan 8, 2004, and Dec 6, 2015, were included in an ongoing multi-institutional registry (the Harvard TMA Research Collaborative)...
March 1, 2017: Lancet Haematology
https://www.readbyqxmd.com/read/28258475/expanding-the-therapeutic-options-for-renal-involvement-in-lupus-eculizumab-available-evidence
#19
REVIEW
Savino Sciascia, Massimo Radin, Jinoos Yazdany, Maria Tektonidou, Irene Cecchi, Dario Roccatello, Maria Dall'Era
In this study, we aimed to systematically review available literature on the efficacy of eculizumab for the treatment of renal involvement in patients with systemic lupus erythematosus (SLE). We conducted a literature search developed a priori, to identify articles reporting clinical experience with the use of eculizumab in SLE patients, focusing on renal involvement. The search strategy was applied to Ovid MEDLINE, EMBASE, In-Process and Other Non-Indexed Citation, Cochrane Central Register of Controlled Trials and Scopus from 2006 to present...
March 3, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28254515/hereditary-cerebral-small-vessel-disease-and-stroke
#20
REVIEW
Christian Baastrup Søndergaard, Jørgen Erik Nielsen, Christine Krarup Hansen, Hanne Christensen
Cerebral small vessel disease is considered hereditary in about 5% of patients and is characterized by lacunar infarcts and white matter hyperintensities on MRI. Several monogenic hereditary diseases causing cerebral small vessel disease and stroke have been identified. The purpose of this systematic review is to provide a guide for determining when to consider molecular genetic testing in patients presenting with small vessel disease and stroke. CADASIL, CARASIL, collagen type IV mutations (including PADMAL), retinal vasculopathy with cerebral leukodystrophy, Fabry disease, hereditary cerebral hemorrhage with amyloidosis, and forkhead box C1 mutations are described in terms of genetics, pathology, clinical manifestation, imaging, and diagnosis...
February 22, 2017: Clinical Neurology and Neurosurgery
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