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For many years after its first description in 1924 thrombotic thrombocytopenic purpura was an intriguing puzzle for clinicians and researchers, not only for its unique pathology, perplexing changes in von Willebrand factor multimers and high rate of rapid fatality but also for its dramatic response to plasma infusion or exchange. The discovery of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeat member 13) and its deficiency in thrombotic thrombocytopenic purpura, due to inhibitory autoantibodies or genetic mutations, provides a mechanistic scheme for understanding its pathogenesis. This new knowledge quickly led to the use of rituximab to promote its remission and prevent recurrence. Recombinant ADAMTS13 is also under development to replace plasma infusion as the therapy for the hereditary thrombotic thrombocytopenic purpura. Recently, caplacizumab, a bivalent nanobody targeting the glycoprotein 1b binding epitope of von Willebrand factor A1 domain, was approved as an addition to the current regimen of plasma exchange and immunomodulation for adult patients of acquired thrombotic thrombocytopenic purpura. This review discusses how the new treatment may improve patient outcomes and its potential pitfalls.
This article was published in the following journal.
Name: The American journal of medicine
To investigate the association between sleep-related characteristics and cognitive change over 3 years of follow up in an aged population.
To identify the role of end-tidal carbon dioxide (EtCO2) monitoring during polysomnography in evaluation of children with obstructive sleep apnea syndrome (OSAS), including the correlation of EtCO2 wi...
Emerging evidence links obstructive sleep apnea syndrome (OSA) with increased cancer incidence and mortality. Invariant natural killer T (iNKT) cells play an important role in cancer immunity. We hypo...
We examined whether interindividual differences in habitual sleep patterns, quantified as the cumulative habitual total sleep time (cTST) over a 2-w period, were reflected in waking measurements of in...
To evaluate vitamin D (25(OH)D) levels in obstructive sleep apnea syndrome (OSAS) and possible relationships to OSAS severity, sleepiness, lung function, nocturnal heart rate (HR), and body compositio...
Arnica montana is a homeopathic drug that is made in accordance with the official methods of the Homeopathic Pharmacopoeia of the United States (HPUS) and is commonly used by cosmetic surg...
Enlarged tonsils and malocclusion has relationship with sleep disturbance in children. The consequences of these features can include aggravation of craniofacial growth such that the devel...
The purpose of this study is to assess the effects of repeated RIPC and exercise, on exercise performance, skeletal muscle responses and circulating cellular and humoral biology in humans
The substitution of raltegravir for the NRTIs will result in some reversal of the long term adverse effect of lipodystrophy (specifically peripheral lipoatrophy) that is associated with th...
Brief Summary: RATIONALE: Comparing results of diagnostic procedures, such as esophagoscopy, done before and after radiation therapy and/or chemotherapy may help doctors predict a patient...
An ionophorous, polyether antibiotic from Streptomyces chartreusensis. It binds and transports cations across membranes and uncouples oxidative phosphorylation while inhibiting ATPase of rat liver mitochondria. The substance is used mostly as a biochemical tool to study the role of divalent cations in various biological systems.
An organothiophosphate insecticide.
Places where animals are slaughtered and dressed for market.
Shortened forms of written words or phrases used for brevity.
That portion of the body that lies between the THORAX and the PELVIS.
Blood is a specialized bodily fluid that delivers necessary substances to the body's cells (in animals) – such as nutrients and oxygen – and transports waste products away from those same cells. In vertebrates, it is composed of blo...
An antibody is a protein produced by the body's immune system when it detects harmful substances, called antigens. Examples of antigens include microorganisms (such as bacteria, fungi, parasites, and viruses) and chemicals. Antibodies may be produc...
Recombinant DNA is the formation of a novel DNA sequence by the formation of two DNA strands. These are taken from two different organisms. These recombinant DNA molecules can be made with recombinant DNA technology. The procedure is to cut the DNA of ...