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REVIEW
Quentin Moyon, Alexis Mathian, Matthias Papo, Alain Combes, Zahir Amoura, Marc Pineton de Chambrun
PURPOSE OF THE REVIEW: Antiphospholipid syndrome (APS) is a rare systemic autoimmune disorder that can escalate into a 'thrombotic storm' called the catastrophic antiphospholipid syndrome (CAPS), frequently requiring ICU admission for multiple organ failure. This review aims to offer insight and recent evidence on critically-ill APS patients. RECENT FINDINGS: The CAPS classification criteria define this condition as the involvement of at least three organs/systems/tissues within less than a week, caused by small vessel thrombosis, in patients with elevated antiphospholipid antibodies levels...
April 23, 2024: Current Rheumatology Reports
#2
JOURNAL ARTICLE
Mouhamed Yazan Abou-Ismail, Chong Zhang, Angela P Presson, Shruti Chaturvedi, Ana G Antun, Andrew M Farland, Ryan Woods, Ara Metjian, Yara A Park, Gustaaf de Ridder, Briana Gibson, Raj S Kasthuri, Darla K Liles, Frank Akwaa, Todd Clover, Lisa Baumann Kreuziger, Meera Sridharan, Ronald S Go, Keith R McCrae, Harsh Vardhan Upreti, Radhika Gangaraju, Nicole K Kocher, X Long Zheng, Jay S Raval, Camila Masias, Spero R Cataland, Andrew D Johnson, Elizabeth Davis, Michael D Evans, Marshall Mazepa, Ming Y Lim
BACKGROUND: Mortality due to immune-mediated thrombotic thrombocytopenic purpura (iTTP) remains significant. Predicting mortality risk may potentially help individualize treatment. The French Thrombotic Microangiopathy (TMA) Reference Score has not been externally validated in the United States. Recent advances in machine learning technology can help analyze large numbers of variables with complex interactions for the development of prediction models. OBJECTIVES: To validate the French TMA Reference Score in the United States Thrombotic Microangiopathy (USTMA) iTTP database and subsequently develop a novel mortality prediction tool, the USTMA TTP Mortality Index...
March 2024: Research and Practice in Thrombosis and Haemostasis
#3
JOURNAL ARTICLE
Masaya Iwamuro, Daisuke Ennishi, Nobuharu Fujii, Ken-Ichi Matsuoka, Takehiro Tanaka, Toshihiro Inokuchi, Sakiko Hiraoka, Motoyuki Otsuka
BACKGROUND: Endoscopic features of intestinal transplant-associated microangiopathy (iTAM) have not been comprehensively investigated. This study aimed to examine the endoscopic characteristics of patients diagnosed with iTAM. METHODS: This retrospective analysis included 14 patients pathologically diagnosed with iTAM after stem cell transplantation for hematolymphoid neoplasms (n = 13) or thalassemia (n = 1). The sex, age at diagnosis, endoscopic features, and prognosis of each patient were assessed...
April 22, 2024: BMC Gastroenterology
#4
Lawanya Singh, Daniel Matassa, Sharon Li
There have been rare reports of patients developing nephrotic syndrome and thrombotic microangiopathy (TMA) with tyrosine kinase inhibitors (TKIs). We present the case of a patient with a history of metastatic pancreatic neuroendocrine tumor (pNET), treated with sunitinib, who rapidly developed TMA and acute kidney injury. The patient was successfully treated with cessation of sunitinib and administration of steroids. This case report contributes to the growing body of literature surrounding the rare side effects of TKIs and our experience with the management of these adverse events...
March 2024: Curēus
#5
Janmejay Ashvinkumar Kunpara, Bhavya Prakash Darji, Himanshu Arvindbhai Patel, Devang Patwari, Sujit Shaileshbhai Patel, Prakash Ishwarlal Darji
Proliferative glomerulonephritis in myelofibrosis is a very rare. Mesangial proliferation and sclerosis with changes of chronic thrombotic microangiopathy have been reported, but pauci-immune focal crescentic glomerulonephritis has not been described so far. Herein, we present a 68-year-old male who was a known case of myelofibrosis and presented with rapidly progressive glomerulonephritis and nephrotic range proteinuria. He was diagnosed as anti-neutrophil cytoplasmic antibody (ANCA)-negative focal crescentic glomerulonephritis, and he responded well to a course of intravenous methylprednisolone and cyclophosphamide...
2024: Indian Journal of Nephrology
#6
MULTICENTER STUDY
Michael D Keller, Patrick J Hanley, Yueh-Yun Chi, Paibel Aguayo-Hiraldo, Christopher C Dvorak, Michael R Verneris, Donald B Kohn, Sung-Yun Pai, Blachy J Dávila Saldaña, Benjamin Hanisch, Troy C Quigg, Roberta H Adams, Ann Dahlberg, Shanmuganathan Chandrakasan, Hasibul Hasan, Jemily Malvar, Mariah A Jensen-Wachspress, Christopher A Lazarski, Gelina Sani, John M Idso, Haili Lang, Pamela Chansky, Chase D McCann, Jay Tanna, Allistair A Abraham, Jennifer L Webb, Abeer Shibli, Amy K Keating, Prakash Satwani, Pawel Muranski, Erin Hall, Michael J Eckrich, Evan Shereck, Holly Miller, Ewelina Mamcarz, Rajni Agarwal, Satiro N De Oliveira, Mark T Vander Lugt, Christen L Ebens, Victor M Aquino, Jeffrey J Bednarski, Julia Chu, Suhag Parikh, Jennifer Whangbo, Michail Lionakis, Elias T Zambidis, Elizabeth Gourdine, Catherine M Bollard, Michael A Pulsipher
Viral infections remain a major risk in immunocompromised pediatric patients, and virus-specific T cell (VST) therapy has been successful for treatment of refractory viral infections in prior studies. We performed a phase II multicenter study (NCT03475212) for the treatment of pediatric patients with inborn errors of immunity and/or post allogeneic hematopoietic stem cell transplant with refractory viral infections using partially-HLA matched VSTs targeting cytomegalovirus, Epstein-Barr virus, or adenovirus...
April 18, 2024: Nature Communications
#7
JOURNAL ARTICLE
Soumya Pandey, Akul Shrivastava, Yanping Izak Harville, Michele Cottler-Fox, Terry O Harville
BACKGROUND: Thrombotic microangiopathies (TMA) are a group of disorders with overlapping clinical features that require urgent intervention. Treatment is based on the recognition of the TMA type, which is often challenging. The aim of this study was to identify specific HLA associations with different TMA types to aid rapid diagnosis and appropriate treatment, since the HLA assay can be completed within five hours. METHODS: All 86 consecutive patients who presented to the University of Arkansas for Medical Sciences between May 2013 and January 2021 with a presumptive diagnosis of TMA were included in this study...
February 8, 2024: Hematology, Transfusion and Cell Therapy
#8
P V Akhila Arya, Erica Marnet, Madhumita Rondla, Jia Wei Tan, Dileep Unnikrishnan, Gregory Buller
OBJECTIVE: We aimed to review the literature on the clinical presentation, renal pathology, treatment, and outcome of renal manifestations in adult-onset Still's disease (AOSD). METHODS: We used PRISMA guidelines for our systematic review and included all English-language original articles from inception till September 15, 2023, on AOSD and kidney involvement in any form. Data on patient demographics, diagnostic criteria, clinical presentation, renal pathology, treatment employed including dialysis, outcome, cause of death were collected and analyzed...
April 16, 2024: Rheumatology International
#9
Pilar Musalem, Cristian Pedreros-Rosales, Hans Müller-Ortiz, Carlos Gutierrez-Navarro, J Daniel Carpio
Complement-mediated thrombotic microangiopathy (CM-TMA) is a rare and life-threatening complication that can occur in kidney transplant recipients, with various potential triggers including immunosuppressive medications. The optimal management and duration of treatment with C5 inhibitors (C5i) for CM-TMA in this patient population remain areas of ongoing investigation. We present the case of a 38-year-old female with a history of IgA nephropathy who underwent preemptive living-related kidney transplantation and subsequently developed CM-TMA seven years post-transplant...
April 14, 2024: Nephron
#10
Yang Song, Shi Yan Lee, Yen-Lin Chee, Wei-Ying Jen
Thrombotic microangiopathy (TMA) is associated with both hypertensive emergency and primary thrombocytopenia purpura (TTP). However, their clinical management is vastly different, with the latter necessitating urgent plasma exchange (PEX). We report two cases of hypertension-associated TMA (HTN-TMA) and a literature review of the clinical management of malignant hypertension. We suggest that in patients presenting with hypertensive emergency associated with TMA, a clinical diagnosis of HTN-TMA should be made, with emergent treatment to lower blood pressure started immediately...
March 25, 2024: Journal of Clinical Medicine
#11
JOURNAL ARTICLE
Christoph Licht, Imad Al-Dakkak, Katerina Anokhina, Nicole Isbel, Véronique Frémeaux-Bacchi, Rodney D Gilbert, Larry A Greenbaum, Gema Ariceta, Gianluigi Ardissino, Franz Schaefer, Eric Rondeau
INTRODUCTION: Atypical haemolytic uremic syndrome (aHUS) is a rare form of thrombotic microangiopathy (TMA) associated with complement dysregulation; aHUS may be associated with other 'triggers' or 'clinical conditions'. This study aimed to characterize this patient population using data from the Global aHUS Registry, the largest collection of real-world data on patients with aHUS. METHODS: Patients enrolled in the Global aHUS Registry between April 2012 and June 2021 and with recorded aHUS-associated triggers or clinical conditions prior/up to aHUS onset were analysed...
April 11, 2024: Nephrology
#12
Craig Peter Coorey, Theo de Malmanche, Angela Chou, Martin Feddersen
Here we present a case of complement-mediated thrombotic microangiopathy (TMA) in a patient who has a background of Stage 5 chronic kidney disease secondary to Alport syndrome. We explain our approach to the diagnosis of TMA, especially the reliance on non-renal manifestations of TMA and the role of kidney biopsy given there was a background of advanced kidney impairment at baseline.
April 11, 2024: Nephrology
#13
William Hughes, Susan Blair, Hannah Rose
This report details the case of a 51-year-old man with a Tiger snake bite who developed systemic envenomation, coagulopathy and thrombotic microangiopathy (TMA) requiring renal replacement therapy. He received plasma exchange as additional therapy while awaiting confirmation of the cause of the TMA. We discuss clinical decision making in detection of systemic envenomation and management of the rare complication of TMA, as well as current Australian guidelines around antivenom administration. This is the fourth known documented case of TMA from a Tiger snake bite in Australia...
April 10, 2024: Nephrology
#14
REVIEW
Maike Büttner-Herold, Kerstin Amann, Joachim Velden
Infections can affect the kidney via different pathways. Urinary tract infections can directly involve the renal tissue by spreading along pre-existing canalicular structures. Such an ascending infection can manifest as a highly active and purulent or even abscessing interstitial nephritis or as a chronic-fibrosing process in recurrent pyelonephritis. Viral infections can also use the canalicular route as in polyomavirus nephropathy or spread via the blood stream in a hematogenous manner as in the case of cytomegalovirus or hantavirus infections...
April 10, 2024: Pathologie (Heidelb)
#15
Reina Komatsu, Kazuya Mimura, Tatsuya Matsuyama, Yoko Kawanishi, Hitomi Nakamura, Takuji Tomimatsu, Masayuki Endo, Tadashi Kimura
Severe cases of hemolysis, elevated liver enzymes, and low platelet (HELLP) syndrome requiring plasma exchange or dialysis should be differentiated from other thrombotic microangiopathy (TMA) and treated appropriately. To evaluate the prevalence and clinical characteristics of such cases in Japan, a questionnaire-based survey was conducted among obstetricians who are members of the Perinatal Research Network Group in Japan. There were a total of 335 cases of HELLP syndrome over a 3-year period in the 48 facilities that responded to the survey...
April 8, 2024: Journal of Obstetrics and Gynaecology Research
#16
REVIEW
Venu M Ganipisetti, Babu Sriram Maringanti, Elvina C Lingas, Kushal Naha
Cobalamin-deficient thrombotic microangiopathy or vitamin B12 deficiency presenting as pseudo-thrombotic microangiopathy is a rare disorder that can be misdiagnosed as thrombotic thrombocytopenic purpura. Patients with this condition are at risk of receiving unnecessary plasmapheresis with a potential delay in appropriate therapy with vitamin B12 supplementation. There are no established diagnostic criteria for this condition in clinical practice. We performed a systematic review of case reports published between January 2018 and January 2023 to analyze the clinical characteristics, risk factors, and patterns of laboratory markers to improve the diagnostic criteria for this condition...
March 2024: Curēus
#17
REVIEW
Muhammed Mubarak, Amber Raza, Rahma Rashid, Fnu Sapna, Shaheera Shakeel
Thrombotic microangiopathy (TMA) is an uncommon but serious complication that not only affects native kidneys but also transplanted kidneys. This review is specifically focused on post-transplant TMA (PT-TMA) involving kidney transplant recipients. Its reported prevalence in the latter population varies from 0.8% to 14% with adverse impacts on both graft and patient survival. It has many causes and associations, and the list of etiologic agents and associations is growing constantly. The pathogenesis is equally varied and a variety of patho genetic pathways lead to the development of microvascular injury as the final common pathway...
March 18, 2024: World Journal of Transplantation
#18
JOURNAL ARTICLE
Wen-Fei Zhang, Rong-Ping Dai, You-Xin Chen
No abstract text is available yet for this article.
April 2, 2024: Ophthalmology Retina
#19
REVIEW
Jiang-Hui Wang, Dominic J Gessler, Wei Zhan, Thomas L Gallagher, Guangping Gao
Adeno-associated virus (AAV) has emerged as a pivotal delivery tool in clinical gene therapy owing to its minimal pathogenicity and ability to establish long-term gene expression in different tissues. Recombinant AAV (rAAV) has been engineered for enhanced specificity and developed as a tool for treating various diseases. However, as rAAV is being more widely used as a therapy, the increased demand has created challenges for the existing manufacturing methods. Seven rAAV-based gene therapy products have received regulatory approval, but there continue to be concerns about safely using high-dose viral therapies in humans, including immune responses and adverse effects such as genotoxicity, hepatotoxicity, thrombotic microangiopathy, and neurotoxicity...
April 3, 2024: Signal Transduction and Targeted Therapy
#20
JOURNAL ARTICLE
Huimin He, Zheng Wang, Jiayun Xu, Yun Liu, Yeqing Shao, Yulong Hou, Jinping Gu, Ruimin Hu, Guolan Xing
Renal involvement is common in monoclonal gammopathy (MG); however, the same patient may have both MG and non-paraprotein-associated renal damage. Accordingly, distinguishing the cause of renal damage is necessary because of the different clinical characteristics and associated treatments. In this multicenter retrospective cohort study, we described the clinicopathological characteristics and prognosis of 703 patients with MG and renal damage in central China. Patients were classified as having MG of renal significance (MGRS), MG of undetermined significance (MGUS), or hematological malignancy...
April 1, 2024: Scientific Reports
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