PEX

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...

The aim of the study was to compare the anterior segment parameters after cataract surgery in pseudoexfoliation syndrome (PEX) and control eyes. We conducted a prospective comparative study of 36 eyes (PEX group), 16 eyes (PEXG group) and 46 eyes (control group) of 98 patients after phacoemulsification with intraocular lens implantation. Before surgery, 1 week, 1 month and 3 months postoperatively, anterior chamber parameters were evaluated by swept source anterior segment optical coherence tomography (AS-OCT)...

BACKGROUND: Elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) has been shown to be safe and efficacious in people with cystic fibrosis (CF) aged ≥2 years. Here, we describe results from an observational study assessing change in burden of illness following initiating ELX/TEZ/IVA in real-world settings. METHODS: This US-based, multicenter, observational study used data from electronic medical records to evaluate real-world burden of illness before and after ELX/TEZ/IVA initiation in people with CF aged ≥12 years heterozygous for F508del and a minimal function mutation ( F /MF) or an uncharacterized CFTR mutation...

The oxygen isotope composition of cellulose (δ18 O values) has been suggested to contain information on stomatal conductance (gs ) responses to rising pCO2 . The extent by which pCO2 affects leaf water and cellulose δ18 O values (δ18 OLW and δ18 OC ) and the isotope processes that determine pCO2 effects on δ18 OLW and δ18 OC are, however, unknown. We tested the effects of pCO2 on gs , δ18 OLW and δ18 OC in a glasshouse experiment, where six plant species were grown under pCO2 ranging from 200 to 500 ppm...

Polymicrobial infection of the airways is a hallmark of obstructive lung diseases such as cystic fibrosis (CF), non-CF bronchiectasis, and chronic obstructive pulmonary disease. Pulmonary exacerbations (PEx) in these conditions are associated with accelerated lung function decline and higher mortality rates. An understanding of the microbial underpinnings of PEx is challenged by high inter-patient variability in airway microbial community profiles. We analyzed bacterial communities in 880 CF sputum samples and developed microbiome descriptors to model community reorganization prior to and during 18 PEx...

Despite being extremely rare, Guillain-Barré syndrome (GBS) has been recognized as a neurological complication of multiple myeloma, with variable responses to plasmapheresis (PEX), intravenous immunoglobulins (IVIG), and anti-myeloma therapies. In this paper, we report a case of a female patient with asymptomatic multiple myeloma (aMM) who initially presented as PEX- and IVIG-refractory GBS. After failure of PEX, IVIG, and anti-myeloma therapy (bortezomib, melphalan, and prednisone), the patient was eventually successfully treated with low-dose rituximab (100 mg/m2 per week in four doses)...

BACKGROUND AND AIMS: Clinicians sometimes encounter papillary thyroid microcarcinoma (PMC) that is less than 10 mm, associated with lymph node metastasis. In this study, we assessed PMC clinicopathologically to clarify risk factors for poor prognosis. PATIENTS AND METHODS: Fifty-one patients who underwent thyroid surgery at Aichi Medical University from September 2009 to October 2016 were included. Patients were divided into two groups, pEX-positive (23 patients) and pEX-negative (28 patients), based on the pathological finding of thyroid capsule invasion...

Digoxin toxicity can be life-threatening. Digoxin-specific antibody (DSA) fragments are used in severe digoxin toxicity, binding to serum-free digoxin and enabling increased renal excretion. In severe renal impairment, clearance of these complexes is prolonged, leading to rebound toxicity. Digoxin and DSA complexes are not dialysable. We present a case of a gentleman with severe digoxin toxicity and acute kidney injury (AKI). Despite receiving DSA doses, his digoxin levels rebounded and symptoms persisted. Based on published case reports, plasma exchange (PEX) after further dosing was arranged...

The 18 O enrichment (Δ18 O) of cellulose (Δ18 OCel ) is recognized as a unique archive of past climate and plant function. However, there is still uncertainty regarding the proportion of oxygen in cellulose (pex ) that exchanges post-photosynthetically with medium water of cellulose synthesis. Particularly, recent research with C3 grasses demonstrated that the Δ18 O of leaf sucrose (Δ18 OSuc , the parent substrate for cellulose synthesis) can be much higher than predicted from daytime Δ18 O of leaf water (Δ18 OLW ), which could alter conclusions on photosynthetic versus post-photosynthetic effects on Δ18 OCel via pex ...

Schwann cells (SCs) undergo phenotypic transformation and then orchestrate nerve repair following a peripheral nervous system injury. The low-density lipoprotein receptor-related protein-1 (LRP1) is significantly upregulated in SCs in response to acute injury, activating cJun and promoting SC survival. Matrix-metalloproteinase-9 (MMP-9) is an LRP1 ligand that binds LRP1 through its hemopexin domain (PEX) and activates SC survival signaling and migration. To identify novel peptide mimetics within the hemopexin domain of MMP-9, we examined the crystal structure of PEX, synthesized four peptides, and examined their potential to bind and activate LRP1...

Acquired thrombotic thrombocytopenic (aTTP) purpura is a life-threatening condition that can lead to devastating thromboembolic events. Recently, caplacizumab has been shown to rapidly restore platelet numbers and reduce the risk of severe end-organ damage when added to plasma exchanges (PEXs) and immunosuppression (IST). Here, we report the outcomes in 3 children with aTTP who were treated with caplacizumab in combination with PEXs and IST. In all 3 patients, platelet count increased to >15,000/mm3 in 24 h and normalized on day 4, whereas normalization of ADAMTS13 activity >50% and elimination of the inhibitor was achieved after 18 to 89 days...

Therapeutic apheresis is an important hematological and nephrological method for conditions with altered plasma composition. It is also indicated for the removal of protein-bound molecules, such as bilirubin. Several techniques can remove these compounds, such as the extracorporeal circulation molecular adsorption system (MARS), plasma exchange (PEX), and plasma adsorption and perfusion (PAP). Here we report our experience in the comparison between MARS, PEX and PAP, since current guidelines do not specify which method is the most appropriate and under which circumstances it should be used...

BACKGROUND: Short palate, lung, and nasal epithelium clone 1 (SPLUNC1) is an innate defence protein that acts as an anti-microbial agent and regulates airway surface liquid volume through inhibition of the epithelial sodium channel (ENaC). SPLUNC1 levels were found to be reduced in airway secretions of adults with cystic fibrosis (CF). The potential of SPLUNC1 as a biomarker in children with CF is unknown. METHODS: We quantified SPLUNC1, interleukin-8 (IL-8) and neutrophil elastase (NE) in sputum of CF children treated with either intravenous antibiotics or oral antibiotics for a pulmonary exacerbation (PEx)s, and in participants of a prospective cohort of CF children with preserved lung function on spirometry, followed over a period of two years...

UNLABELLED: Zellweger syndrome is an autosomal recessive disease within the spectrum of peroxisome biogenesis disorder manifesting in the neonatal period with profound dysfunction of the central nervous system, liver and kidney. Common clinical presentations include hypotonia, seizure, hepatomegaly, craniofacial dysmorphism and early death. Mutation in one of the PEX genes coding for a peroxisome assembly protein creates a functionally incompetent organelle causing accumulation of very long chain fatty acids in various organs...

BACKGROUND: Participation restrictions following traumatic brain injury are associated with executive function (EF) deficits (EFDs). The subacute recovery phase's specific characteristics (enhanced brain plasticity and impaired self-awareness) and contextual factors (inpatient setting) warrant adjusting cognitive rehabilitation protocols. The Intervention of Participation and Executive Functions (I-PEX) was designed to improve EFDs during subacute inpatient rehabilitation. OBJECTIVE: To investigate the I-PEX's preliminary efficacy to improve EFDs during the performance of complex daily activities and enhance self-awareness, cognitive self-efficacy, participation, and quality of life postdischarge...

Pseudoexfoliation syndrome (PEX) is characterized by the accumulation of abnormal extracellular matrix material in ocular and non-ocular tissues, including blood vessel walls. Clot-forming dysfunction might be responsible for venous thrombosis in PEX. We investigated global coagulation, the proteome, and functions of platelets in PEX patients and aimed to determine prognostic biomarkers for thrombosis risk in PEX. Peripheral blood was collected from PEX and retinal vein occlusion (RVO) patients, and age-sex matched controls...

BACKGROUND: The increasing prevalence of DH applications has outpaced research and practice in digital health (DH) evaluations. Patient experience (PEx) was reported as one of the challenges facing the health system by the World Health Organization. To generate evidence on DH and promote the appropriate integration and use of technologies, a standard evaluation of PEx in DH is required. OBJECTIVE: This study aims to systematically identify evaluation timing considerations (ie, when to measure), evaluation indicators (ie, what to measure), and evaluation approaches (ie, how to measure) with regard to digital PEx...

Pseudomonas aeruginosa is an opportunistic Gram-negative bacterium with adaptive metabolic abilities. It can cause hospital-acquired infections with significant mortality rates, particularly in people with already existing medical conditions. Its ability to develop resistance to common antibiotics makes managing this type of infections very challenging. Furthermore, oxidative stress is a common consequence of bacterial infection and antibiotic therapy, due to formation of reactive oxygen species (ROS) during their mode of action...

Pseudoexfoliation syndrome (PEX) is a long-term, age-related extracellular matrix condition that causes aberrant fibrillary pseudoexfoliative material (PXM) to accumulate in various body tissues. The anterior portion of the eye is where this disorder most frequently presents. It affects the entire body. Most frequently, it is seen in older people, usually those over 50. Fibrillar deposits are a symptom of the pseudoexfoliation syndrome and are found in the anterior part of the eye. Deposition of fibrillary white flaky material is seen...

Some acute exercise effects are influenced by postexercise (PEX) diet, and these diet-effects are attributed to differential glycogen resynthesis. However, this idea is challenging to test rigorously. Therefore, we devised a novel genetic model to modify muscle glycogen synthase 1 (GS1) expression in rat skeletal muscle with an adeno-associated virus (AAV) short hairpin RNA knockdown vector targeting GS1 (shRNA-GS1). Contralateral muscles were injected with scrambled shRNA (shRNA-Scr). Muscles from exercised (2-hour-swim) and time-matched sedentary (Sed) rats were collected immediately postexercise (IPEX), 5-hours-PEX (5hPEX), or 9-hours-PEX (9hPEX)...