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Frontiers in Immunology 2022Comèl-Netherton syndrome (NS) is a rare disease caused by pathogenic variants in the gene, leading to severe skin barrier impairment and proinflammatory upregulation....
BACKGROUND
Comèl-Netherton syndrome (NS) is a rare disease caused by pathogenic variants in the gene, leading to severe skin barrier impairment and proinflammatory upregulation. Given the severity of the disease, treatment of NS is challenging. Current treatment regimens are mainly topical and supportive. Although novel systemic treatment options for NS have been suggested in recent literature, little is known about their outcomes.
OBJECTIVE
to provide an overview of systemic treatment options and their outcomes in adults and children with NS.
METHODS
Embase, MEDLINE, Web of Science, Cochrane Central Register of Controlled Trials, and Google Scholar were searched up to July 22, 2021. Empirical studies published in English language mentioning systemic treatment in NS were enrolled. Studies that did not define a treatment period or report at least one outcome were excluded. Methodological quality was evaluated by the Joanna Briggs Institute critical appraisal checklist for case reports or case series. Overall quality of evidence of the primary outcome, skin, was assessed by the GRADE approach.
RESULTS
36 case series and case reports were included. The effects of 15 systemic therapies were described in 48 patients, of which 27 were children. Therapies included retinoids, prednisolone, cyclosporine, immunoglobulins, and biologicals. In retinoids both worsening (4/15 cases) and improvement (6/15 cases) of the skin was observed. Use of prednisolone and cyclosporine was only reported in one patient. Immunoglobulins (13/15 cases) and biologicals (18/21 cases) showed improvement of the skin. Certainty of evidence was rated as very low.
CONCLUSION
NS is a rare disease, which is reflected in the scarce literature on systemic treatment outcomes in children and adults with NS. Studies showed large heterogeneity in outcome measures. Adverse events were scarcely reported. Long-term outcomes were reported in a minority of cases. Nonetheless, a general beneficial effect of systemic treatment was found. Immunoglobulins and biologicals showed the most promising results and should be further explored. Future research should focus on determining a core outcome set and measurement instruments for NS to improve quality of research.
SYSTEMATIC REVIEW REGISTRATION
https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=217933, PROSPERO (ID: 217933).
Topics: Adult; Child; Cyclosporine; Humans; Netherton Syndrome; Prednisolone; Rare Diseases; Retinoids
PubMed: 35464459
DOI: 10.3389/fimmu.2022.864449 -
Frontiers in Pharmacology 2023Chronic urticaria (CU) is a commonly seen skin disorder featured by recurring wheals, with or without angioedema, lasting for at least 6 weeks. Runzao Zhiyang capsule...
Chronic urticaria (CU) is a commonly seen skin disorder featured by recurring wheals, with or without angioedema, lasting for at least 6 weeks. Runzao Zhiyang capsule (RZC) has been widely applied to treat patients with CU. This study is aimed at systematically evaluating the efficacy and safety of RZC in treating CU. Randomized controlled trials (RCTs) of RZC on treating CU from Chinese and English databases were searched. Data were collected by two independent researchers. The Cochrane Collaboration tool was adopted for evaluating the risk of bias. The meta-analysis was performed with Review Manager 5.3 software. Sensitivity analysis and publication bias assessment were conducted by Stata 14.0 software. Totally 27 studies were included in the analysis, involving 2,703 patients. The pooled results showed that compared with second-generation H1-antihistamines (sgAHs) therapy alone, RZC combined with sgAHs is more effective in improving the total effective rate (RR = 1.32, 95% CI: 1.25 to 1.39, < 0.00001), the quality of life measured by Dermatology Life Quality Index (DLQI) (MD = -2.63, 95% CI: -3.68 to -1.58, < 0.00001) and the serum IFN-γ level (SMD = 3.10, 95% CI: 1.58 to 4.62, < 0.0001), and reducing the recurrence rate (RR = 0.39, 95% CI: 0.27 to 0.55, < 0.00001), the serum total IgE level (SMD = -2.44, 95% CI: -3.51 to -1.38, < 0.00001), the serum IL-4 level (SMD = -2.96, 95% CI: -4.10 to -1.83, < 0.00001), and the incidence of adverse events including dizziness, fatigue, dry mouth, and constipation (RR = 0.53, 95% CI: 0.33 to 0.85, = 0.009; RR = 0.46, 95% CI: 0.26 to 0.84, = 0.01; RR = 0.57, 95% CI: 0.34 to 0.95, = 0.03; RR = 0.24, 95% CI: 0.07 to 0.85, = 0.03). The current evidence indicates that RZC may be an efficient therapeutic regimen in patients with CU. Nevertheless, owing to the suboptimal quality of the included studies, more large-scale, well-designed RCTs are required to verify the obtained findings. https://www.crd.york.ac.uk/PROSPERO/; Identifier: CRD42022313177.
PubMed: 37693898
DOI: 10.3389/fphar.2023.1200252 -
Frontiers in Medicine 2021The skin is a flexible organ that forms a barrier between the environment and the body's interior; it is involved in the immune response, in protection and regulation,...
The skin is a flexible organ that forms a barrier between the environment and the body's interior; it is involved in the immune response, in protection and regulation, and is a dynamic environment in which skin lipids play an important role in maintaining homeostasis. The different layers of the skin differ in both the composition and amount of lipids. The epidermis displays the best characteristics in this respect. The main lipids in this layer are cholesterol, fatty acids (FAs) and ceramides. FAs can occur in free form and as components of complex molecules. The most poorly characterized FAs are very long-chain fatty acids (VLCFAs) and ultra long-chain fatty acids (ULCFAs). VLCFAs and ULCFAs are among the main components of ceramides and are part of the free fatty acid (FFA) fraction. They are most abundant in the brain, liver, kidneys, and skin. VLCFAs and ULCFAs are responsible for the rigidity and impermeability of membranes, forming the mechanically and chemically strong outer layer of cell membranes. Any changes in the composition and length of the carbon chains of FAs result in a change in their melting point and therefore a change in membrane permeability. One of the factors causing a decrease in the amount of VLCFAs and ULCFAs is an improper diet. Another much more important factor is mutations in the genes which code proteins involved in the metabolism of VLCFAs and ULCFAs-regarding their elongation, their attachment to ceramides and their transformation. These mutations have their clinical consequences in the form of inborn errors in metabolism and neurodegenerative disorders, among others. Some of them are accompanied by skin symptoms such as ichthyosis and ichthyosiform erythroderma. In the following review, the structure of the skin is briefly characterized and the most important lipid components of the skin are presented. The focus is also on providing an overview of selected proteins involved in the metabolism of VLCFAs and ULCFAs in the skin.
PubMed: 34497816
DOI: 10.3389/fmed.2021.730855 -
Indian Journal of Occupational and... 2022The COVID-19 pandemic has necessitated the use of personal protective equipment (PPE) among the frontline health care workers (HCWs). Even though PPE helps in preventing...
BACKGROUND
The COVID-19 pandemic has necessitated the use of personal protective equipment (PPE) among the frontline health care workers (HCWs). Even though PPE helps in preventing infection, it poses significant physical and psychological impacts at varying levels. Correspondingly, multiple independent studies have brought out the PPE-associated problems. However, there exists a lacuna on comprehensive information of global prevalence related to the same.
AIM
To estimate the prevalence and risk factors of PPE among HCWs during COVID-19 across the globe.
DESIGN
Systematic review and meta-analysis.
METHOD
The review was undertaken as per the protocol registered in PROSPERO CRD42021272216 following Preferred Reporting Items for Systematic Reviews and Meta-Analysis(PRISMA) guidelines. Two independent reviewers have undertaken the search strategy, study selection, and methodological quality assessment. Discrepancies were addressed by the third reviewer. Heterogeneity was addressed through I2 statistics and forest plots generated by open meta-software.
RESULTS
A total of 16 articles conducted across 6 different countries among 10,182 HCWs were included in the review. The pooled prevalence of skin lesions, headache, sweating, breathing difficulty, vision difficulty, thirst/dry mouth, fatigue, and communication difficulty, anxiety, fear were 57 (47-66%), 51 (37-64%), 75 (56-90%), 44 (23-68%), 61 (21-94%), 54 (30-77%), 67 (58-76%), 74 (47-94%), 28 (24-33%), 14 (10-17%), respectively. Moreover, the various risk factors included are the use of PPE for >6 h and young females. In addition, the medical management of new-onset problems created an additional burden on the frontline health care personnel (HCP).
CONCLUSION
The frontline HCWs encountered physical and psychological problems at varying levels as a result of wearing PPE which needs to be addressed to prevent the inadequate use of PPE leading to infections.
PubMed: 36408432
DOI: 10.4103/ijoem.ijoem_32_22 -
PloS One 2021The pathophysiology of xerosis depends on extrinsic and intrinsic exposures. Residential hard water may constitute such an exposure.
BACKGROUND
The pathophysiology of xerosis depends on extrinsic and intrinsic exposures. Residential hard water may constitute such an exposure.
OBJECTIVES
To estimate the prevalence of xerosis and to compare water hardness exposure in blood donors with and without xerosis.
METHODS
In this retrospective cohort study in 2018-2019, blood donors with self-reported moderately or severely dry skin were compared to blood donors without dry skin. Blood donors with ichthyosis, lichen planus and psoriasis were excluded. Water hardness data was collected from the Geology Survey of Denmark and Greenland.
RESULTS
Overall, 4,748 of 30,721 (15.5%; 95% confidence interval 15.1-15.9%) blood donors had xerosis. After excluding blood donors with ichthyosis, lichen planus and psoriasis, 4,416 blood donors (2,559 females; median age 38.4 years [interquartile range 28.0-49.8]; 700 smokers) remained in this study. Water softer than 12-24 degrees Deutsche härte was associated with decreased probability of xerosis (odds ratio 0.83; 95% confidence interval 0.74-0.94) and water harder than 12-24 degrees Deutsche härte was associated with increased probability of xerosis (odds ratio 1.22; 95% confidence interval 1.03-1.45). The association between water hardness and xerosis remained significant after excluding blood donors with dermatitis.
CONCLUSIONS
Water hardness is associated with xerosis independent of other dermatoses.
Topics: Blood Donors; Cohort Studies; Databases as Topic; Denmark; Humans; Psoriasis
PubMed: 34077478
DOI: 10.1371/journal.pone.0252462 -
Cell Journal Feb 2024Exposure to phosgene, a colourless poisonous gas, can lead to various health issues including eye irritation, a dry and burning throat, vomiting, coughing, the...
Exposure to phosgene, a colourless poisonous gas, can lead to various health issues including eye irritation, a dry and burning throat, vomiting, coughing, the production of foamy sputum, difficulty in breathing, and chest pain. This systematic review aims to provide a comprehensive overview of the clinical manifestations and treatment of phosgene toxicity by systematically analyzing available literature. The search was carried out on various scientific online databases to include related studies based on inclusion and exclusion criteria with the use of PRISMA guidelines. The quality of the studies was assessed using the Mixed Methods Appraisal Tool (MMAT). Thirteen articles were included in this study after the screening process. Inhalation was found to be the primary health problem of phosgene exposure with respiratory symptoms such as coughing and dyspnea. Chest pain and pulmonary oedema were also observed in some cases. Furthermore, pulmonary crackle was the most common reported physical examination. Beyond respiratory tract health issues, other organs involvements such as cardiac, skin, eye, and renal were also reported in some studies. The symptoms can occur within minutes to hours after exposure, and the severity of symptoms depends on the amount of inhaled phosgene. The findings showed that bronchodilators can alleviate symptoms of bronchoconstriction caused by phosgene. Oxygen therapy is essential for restoring oxygen levels and improving respiratory function in cases of hypoxemia. In severe cases, endotracheal intubation and invasive mechanical ventilation are used for artificial respiration, along with the removal of tracheal secretions and pulmonary oedema fluid through suctioning as crucial components of supportive therapy.
PubMed: 38459726
DOI: 10.22074/cellj.2024.2011864.1405 -
Frontiers in Veterinary Science 2022Breeder animals are an important focus in farm animal welfare assessments as they typically live the longest lives and are at the greatest risk for suffering due to...
Breeder animals are an important focus in farm animal welfare assessments as they typically live the longest lives and are at the greatest risk for suffering due to their longevity. For breeding pigs, the time between the end of lactation (post-weaning) and the implantation of embryos (early gestation) is very dynamic from both a physiological and husbandry perspective. However, research to date is limited on how best to house and manage sows during this critical period of their production cycle from a welfare perspective. Previous animal-based welfare outcome measures were restricted to certain health, behavioral and physiological indicators. This systematic review used Web of Science to make in-depth comparisons among welfare-based studies that focus on sow housing during the post-weaning and early pregnancy period to identify important knowledge gaps. Only a small number of studies ( = 27) were found that met our systematic search criteria. Compared to stalls, group housing requires mixing of animals and always triggers more aggression and skin lesions at the time of mixing. The predominant use of health and physiological indicators constrained the animal-based welfare outcomes in these studies. Thus, what type of housing yields the best overall welfare outcome remains to be elucidated as none of the studies found explored the mental wellbeing of sows during this period. This systematic review defines a critical knowledge gap regarding the full impact of housing on the welfare of post-weaning and early gestation sows. This gap, and thus the true welfare impact of sow housing, will only be addressed by the use of novel, more holistic assessment methods that also capture the psychological state of the sow.
PubMed: 36082213
DOI: 10.3389/fvets.2022.903822 -
Special Care in Dentistry : Official... May 2022Multisystemic inflammatory syndrome in children (MIS-C) is a condition noted in some children asymptomatic but positive to Sars-cov-2 antibody and it presents clinical... (Review)
Review
AIMS
Multisystemic inflammatory syndrome in children (MIS-C) is a condition noted in some children asymptomatic but positive to Sars-cov-2 antibody and it presents clinical and laboratory changes similar to Kawasaki disease (KD). Oral changes have also been observed. This systematic review evaluated oral manifestations detected in children with MIS-C and KD associated to COVID-19.
METHODS AND RESULTS
This work was registered at PROSPERO (#CRD42020225909), following PRISMA guidelines. A comprehensive research was conducted in MEDLINE, Web of Science, EMBASE, LILACS, Scopus, and Grey Literature through August 2021, based on original research evaluating children diagnosed with MIS-C or KD related to COVID-19. Two authors independently screened all retrieved references. Twenty five selected studies evaluated 624 children, mean age 8.78 years. The assessment of the risk of bias (ROB) showed that most of them presented low ROB. Oral manifestations were erythematous mucous membrane, oral ulcers lesions, dry, swollen and cracked lips, and strawberry tongue.
CONCLUSION
MIS-C and KD share the same oral manifestations and their identification may lead to an early diagnosis.
Topics: Abnormalities, Multiple; COVID-19; Child; Genetic Diseases, X-Linked; Humans; Ichthyosiform Erythroderma, Congenital; Limb Deformities, Congenital; Mucocutaneous Lymph Node Syndrome; SARS-CoV-2; Systemic Inflammatory Response Syndrome
PubMed: 34792813
DOI: 10.1111/scd.12669 -
Chinese Medicine 2019In China, the medical use of fecal matter (fresh fecal suspension or dry feces) can be dated back to the fourth century, approximately 1700 years ago. In long-term... (Review)
Review
In China, the medical use of fecal matter (fresh fecal suspension or dry feces) can be dated back to the fourth century, approximately 1700 years ago. In long-term clinical practice, Chinese doctors have accumulated unique and invaluable medical experience in the use of fecal materials. In view of their good curative effect and medicinal potential, fecal medicines should be paid much attention. This study aimed to provide the first comprehensive data compilation of fecal medicines used in various Chinese traditional medical systems by bibliographic investigation of 31 medicine monographs and standards. A total of 54 fecal medicines were found to be used in 14 traditional Chinese medical systems. Their names, original species, medicinal forms, and traditional uses were described in detail. These fecal medicines were commonly used to treat gastrointestinal, nervous system, skin, and gynecological diseases. Commonly used fecal medicines include Wu-Ling-Zhi, Jiu-Fen and Hei-Bing-Pian. The information summarized in this study can provide a good reference for the development and utilization of fecal medicines. Further studies are necessary to prove their medicinal value, identify their active ingredients, and elucidate their mechanisms of action so that more people can accept these special medicines.
PubMed: 31528199
DOI: 10.1186/s13020-019-0253-x -
Skin Health and Disease Dec 2023Immunotherapy has become a mainstay of treatment for many cancers. Multiple immune checkpoint inhibitors have been used to treat malignancies, including anti-programed... (Review)
Review
Immunotherapy has become a mainstay of treatment for many cancers. Multiple immune checkpoint inhibitors have been used to treat malignancies, including anti-programed death-1 (PD1) and anti-cytotoxic T-lymphocyte-associated protein (anti-CTLA4). However, a significant percentage of patients develop resistance to these immunotherapy drugs. Therefore, novel strategies were developed to target other aspects of the immune response. Lymphocyte activation gene-3 (LAG-3) is a cell-surface molecule found on natural killer cells and activated T-cells which negatively regulates T-cell proliferation and function. LAG-3 inhibitors interact with LAG-3 ligands on the surface of T-cells to block T-regulatory (Treg) cell activity, suppress cytokine secretion and restore dysfunctional effector T-cells which subsequently attack and destroy cancer cells. This review reports the dermatologic side effects associated with LAG-3 inhibitors used in the treatment of melanomas. Using PRISMA 2022 guidelines, a comprehensive literature review of PubMed, Google Scholar, Embase, Cochrane, and Web of Science databases was conducted. Three studies were identified that demonstrated that the use of LAG-3 inhibitors, whether as a single agent or in combination with other immune checkpoint inhibitors, resulted in stomatitis, pruritus, rash, dry skin, erythema, and vitiligo. Further research is warranted to assess the cutaneous adverse events observed with LAG-3 inhibitors in treating melanoma and to identify populations most vulnerable to such side effects.
PubMed: 38047262
DOI: 10.1002/ski2.296