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Thus, there is an increasing need for the practising dermatologist to become familiar with the concept of gene therapy, fundamental differences between various approaches and their human applications. This review explains the principles of different approaches of gene therapy; summarises its journey and discusses its current and future impact in RDEB.
Cold urticaria (ColdU), that is, the occurrence of wheals or angioedema in response to cold exposure, is classified into typical and atypical forms. The diagnosis of typical ColdU relies on whealing in response to local cold stimulation testing (CST). It can also manifest with cold-induced anaphylaxis (ColdA). We aimed to determine risk factors for ColdA in typical ColdU.
An international, cross-sectional study COLD-CE was carried out at 32 urticaria centers of reference and excellence (UCAREs). Detailed history was taken and CST with an ice cube and/or TempTest
performed. ColdA was defined as an acute cold-induced involvement of the skin and/or visible mucosal tissue and at least one of cardiovascular manifestations, difficulty breathing, or gastrointestinal symptoms.
Of 551 ColdU patients, 75% (n=412) had a positive CST and ColdA occurred in 37% (n=151) of the latter. Cold-induced generalized wheals, angioedema, acral swelling, oropharyngeal/laryngeal symptoms, and itch of earlobes were identified as signs/symptoms of severe disease. ColdA was most commonly provoked by complete cold water immersion and ColdA caused by cold air was more common in countries with a warmer climate. Ten percent (n=40) of typical ColdU patients had a concomitant chronic spontaneous urticaria (CSU). They had a lower frequency of ColdA than those without CSU (4% vs. 39%, p=.003). We identified the following risk factors for cardiovascular manifestations previous systemic reaction to a Hymenoptera sting, angioedema, oropharyngeal/laryngeal symptoms, and itchy earlobes.
ColdA is common in typical ColdU. High-risk patients require education about their condition and how to use an adrenaline autoinjector.
ColdA is common in typical ColdU. High-risk patients require education about their condition and how to use an adrenaline autoinjector.
The interest of consumers and market and scientific research for added-value foods obtained with environmentally sustainable productive chains is increasing. Silver fir (Abies alba Mill.) needles (SFNs), often by-products of forest management and logging, represent an unexploited source of bioactive compounds.
For the first time, SFN aqueous extract obtained through controlled hydrodynamic cavitation was used to enrich whole wheat flour bread. The first trial found that 35% SFNs extract addition was the absolute threshold of taste perception. The second trial investigated dough rheological properties and bread technological and antioxidant properties in samples enriched with 35% and 100% SFNs extract compared with the control (0% SFNs extract). SFNs extract significantly increased bread antioxidant capacity in both 35% and 100% SFN fresh breads by ~42.5% and ~87% respectively and in 100% SFNs bread samples after 72 h of storage by ~76%. Enrichment of 35% showed higher alveograph dough extensibility (~11%) and different bread texture in terms of hardness, springiness, and chewiness. Enrichment with 100% SFNs extract significantly improved dough and bread technological quality it increased alveograph dough extensibility L (~18%), swelling index G (~8%), and flour strength W (~14%) and showed the highest increase in bread specific volume (~0.200 Lkg
).
SFNs aqueous extract produced with controlled hydrodynamic cavitation appeared a valuable technical material for the manufacturing of added-value and functional breads. © 2021 Society of Chemical Industry.
SFNs aqueous extract produced with controlled hydrodynamic cavitation appeared a valuable technical material for the manufacturing of added-value and functional breads. © 2021 Society of Chemical Industry.
The aim of the current study was to determine the clinical characteristics of migraine with aura (MA) as well as the frequency and patterns of perfusion-computed tomography (PCT) alterations, in a series of patients with MA mimicking acute ischemic stroke.
MA is one of the most frequent stroke mimics, following seizures and psychiatric disorders. Previous case reports and short series have reported abnormal PCT patterns in patients with MA.
We conducted a retrospective cohort study including all consecutive patients presenting with focal neurological symptoms during complete multimodal CT including baseline CT, angio-CT, and PCT with a final diagnosis of MA. We collected demographic data and clinical information about MA variables using the hospital electronic database.
We found 25 patients with a final diagnosis of MA among 1761 patients who attended our stroke center with complete multimodal CT (1.4% [95% CI 0.9-2.1]). Among them, 14/25 (56%) were women, average age 38.7years (SD 12.5), and 16/25 (6ing still symptomatic by the time they were scanned. Further research will clarify the presence and type of PCT alterations in this entity.
The General Life Functioning Scale (GLF) was developed to provide a complementary alternative to existing measures of impairment. We examined the psychometric properties of the GLF-Parent version (GLF-P), given the known value of informant ratings.
The GLF-P was administered to parents of adults with attention-deficit/hyperactivity disorder(ADHD) diagnosed in childhood and a nonADHD comparison group in the Pittsburgh ADHD Longitudinal Study. GLF-P ratings described 334 participants (ADHD = 186; comparison = 148) rated at age 25 (M
= 24.80 years, SD
= 0.46, range = 24-26) and 401 participants (ADHD = 237; comparison = 164) rated at age 30 (M
= 29.30, SD
= 0.64, range = 28-33). Exploratory (EFA) and confirmatory (CFA) factor analyses were used.
EFAs suggested and CFAs confirmed a five-factor solution. We found measurement invariance across diagnostic and age groups, satisfactory internal consistency, construct validity, and known-group validity.
Psychometric results suggest the GLF-P as a helpful adjunctive measure of functioning. Further research is needed to determine the utility of the GLF across diverse settings.
Psychometric results suggest the GLF-P as a helpful adjunctive measure of functioning. Further research is needed to determine the utility of the GLF across diverse settings.Red blood cell (RBC) alloimmunisation with anti-D and anti-K comprise the majority of cases of fetal haemolytic disease requiring intrauterine red cell transfusion (IUT). Few studies have investigated which haematological parameters can predict adverse fetal or neonatal outcomes. The aim of the present study was to identify predictors of adverse outcome, including preterm birth, intrauterine fetal demise (IUFD), neonatal death (NND) and/or neonatal transfusion. We reviewed the records of all pregnancies alloimmunised with anti-K and anti-D, requiring IUT over 27 years at a quaternary fetal centre. We reviewed data for 128 pregnancies in 116 women undergoing 425 IUTs. The median gestational age (GA) at first IUT was significantly earlier for anti-K than for anti-D (24·3 vs. 28·7 weeks, P = 0·004). Selleckchem CDK inhibitor Women with anti-K required more IUTs than women with anti-D (3·84 vs. 3·12 mean IUTs, P = 0·036) and the fetal haemoglobin (Hb) at first IUT was significantly lower (51.0 vs. 70.5 g/l, P = 0·001). The mean estimated stics, in addition to laboratory markers, to further identify and improve the outcomes of these pregnancies.Intra-articular administration of sustained-release anti-inflammatory drugs is indicated in horses suffering from joint inflammation, but no such drugs are labelled for veterinary use. To obtain initial data on synovial disposition and safety of a new sustained-release formulation of diclofenac (SYN321) in the joints of horses, an experimental interventional study of elimination and side effects of intra-articular administration of SYN321 was conducted. Nine clinically sound horses were included in the study, and SYN321 was administered by the intra-articular route. Dose ranges and sampling intervals were established in a pilot study with two horses, and then applied in a main study involving seven horses treated in the fetlock joint. Diclofenac was detected above lower limit of quantification (LOQ 0.5 ng/ml) in synovial fluid throughout the study period (14 days), and below LOQ (0.1 ng/ml) in plasma after 4 days and in urine after 14 days. No obvious clinical side effects were detected. Clinical examination and objective lameness evaluation suggested that SYN321 has potential as a local joint NSAID treatment with sustained release in horses, but further studies on synovial fluid exposure, safety and clinical efficacy are warranted.
Non-alcoholic fatty liver disease (NAFLD) represents a severe public health problem. It often coexists with hypertension in the context of metabolic syndrome. We investigated the effects of amlodipine on NAFLD combined with hypertension and investigated the underlying mechanism/s.
Mice were fed with high-fat diet (HFD) and 0.05% N-nitro-L-arginine methylester sterile water to induce NAFLD with hypertension. Gut microbiota composition and function were assessed by 16S ribosomal DNA and metagenomic sequencing. Untargeted metabolome profiles were applied to identify differential metabolites in mice caecum.
Amlodipine besylate and amlodipine aspartate significantly decreased liver injury and hepatic steatosis, and improved lipid metabolism with a concomitant reduction in the expression of lipogenic genes in mice with NAFLD and hypertension. Mechanistically, amlodipine besylate and amlodipine aspartate have potential to restore intestinal barrier integrity and improve antimicrobial defence, along with the elsing therapeutic agents for treating these diseases.
Cutaneous squamous cell carcinoma (cSCC) is the second most common cancer worldwide with relatively low metastatic potential (2-5%). Developments in therapeutic options have highlighted the need to better identify high-risk patients who could benefit from closer surveillance, adjuvant therapies, and baseline/follow-up imaging, while at the same time safely omitting low-risk patients from further follow-up. Controversy remains regarding the predictive performance of current cSCC staging systems and which methodology to adopt.
To validate the performance of four cSCC staging systems (American Joint Committee on Cancer 8
edition (AJCC8), Brigham and Women's Hospital (BWH), Tübingen, and Salamanca T3 refinement) in predicting metastasis using a nationwide cohort.
A nested case-control study using data from the National Disease Registration Service, England, 2013-2015 was conducted. Metastatic cSCC cases were identified using an algorithm to identify all potential cases for manual review. These were 11 matstaging systems.
We validated four cSCC staging systems using the largest nationwide dataset of metastatic cSCC so far. Although BWH showed the highest overall discriminative ability, PPV was low for all staging systems, which shows the need for further improvement and refining of current cSCC staging systems.
My Website: https://www.selleckchem.com/CDK.html
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