X-linked ichthyosis is a relatively common syndromic form of ichthyosis most often due to deletions in the gene encoding the microsomal enzyme, steroid sulfatase, located on the short area of the X ...chromosome. Syndromic features are mild or unapparent unless contiguous genes are affected. In normal epidermis, cholesterol sulfate is generated by cholesterol sulfotransferase (SULT2B1b), but desulfated in the outer epidermis, together forming a ‘cholesterol sulfate cycle’ that potently regulates epidermal differentiation, barrier function and desquamation. In XLI, cholesterol sulfate levels my exceed 10% of total lipid mass (≈1% of total weight). Multiple cellular and biochemical processes contribute to the pathogenesis of the barrier abnormality and scaling phenotype in XLI. This article is part of a Special Issue entitled The Important Role of Lipids in the Epidermis and their Role in the Formation and Maintenance of the Cutaneous Barrier. Guest Editors: Kenneth R. Feingold and Peter Elias.
•X-linked ichthyosis (XLI) is due to loss-of-function mutations in steroid sulfatase (SSase; STS).•Cholesterol sulfotransferase (SULT2B1b) generates CSO4, while SSase degrades CSO4 in the epidermis.•Sulfation of cholesterol is linked to epidermal differentiation and corneocyte formation.•The phenotype in XLI results from persistence of “pristine” corneodesmosomes in the outer SC.•Disruption of lamellar bilayers creates a pathway for excess water loss in XLI.
Aging of the skin barrier Choi, Eung Ho, MD, PhD
Clinics in dermatology,
07/2019, Letnik:
37, Številka:
4
Journal Article
Recenzirano
AbstractThe skin barrier is mainly present in the stratum corneum (SC), composed of corneocytes surrounded by intercellular lipid lamellae, and attached by corneodesmosome. The tight junction ...attached to the lateral walls of keratinocytes in the upper part of the stratum granulosum is also included in the skin barrier. During aging, the following structures and functions of the skin barrier are changed or disturbed: (1) skin barrier structure, (2) permeability barrier function, (3) epidermal calcium gradient, (4) epidermal lipid synthesis and SC lipid processing, (5) cytokine production and response after insults, (6) SC acidity, (7) SC hydration, and (8) antimicrobial barrier. Patients with diabetes also show changes in the skin barrier similar to those in aged skin, and the characteristics of the skin barrier are very similar. Understanding the pathogenic mechanisms of the skin barrier in aging will permit us to develop therapeutic strategies for aged or diabetic skin.
We tried to investigate the effect of non-persistence with antiplatelets after ischemic stroke on long-term all-cause mortality (ACM).
We selected newly diagnosed ischemic stroke patients aged ...≥20years who were newly treated with aspirin or clopidogrel from 2003-2010 Korean National Health Insurance Service-National Sample Cohort, a random sample of 2.2% of total population. Subjects were divided into two pairs of groups according to persistence with antiplatelets at 6 and 12 months: those who discontinued antiplatelets within 6 months (DA6M) and those who continued them for 6 or months or more (CA6M); and those who discontinued antiplatelets within 12 months (DA12M) and those who continued them for 12 months or more (CA12M). Those who died within 6 months among DA6M and those who died within 12 months among DA12M were excluded along with those with medication possession ratio<80% among CA6M and CA12M. Subjects were followed-up until death or December 31, 2013. Among 3,559 total subjects, DA6M were 1,080 and CA6M were 2,479 while, out of 3,628 total patients, DA12M were 1,434 and CA12M were 2,194. The risks of ACM adjusted hazard ratio (aHR), 2.25; 95% confidence interval (CI), 1.94-2.61, cerebro-cardiovascular disease (CVD) death (aHR, 2.52; 95% CI, 1.96-3.24) and non-CVD death (aHR, 2.11; 95% CI, 1.76-2.64) of DA6M were all significantly increased compared to CA6M. DA12M also had significantly higher risks of ACM (aHR, 1.93; 95% CI, 1.65-2.25), CVD mortality (aHR, 2.13; 95% CI; 1.63-2.77) and non-CVD mortality (aHR, 1.83;95% CI 1.51-2.22) than DA12M but aHRs were lower than that between DA6M and CA6M. The difference rates of ACM, CVD death, and non-CVD death between non-persistent and persistent groups all continuously widened over time but the degree of difference was gradually decreased.
Maintaining antiplatelets for the first 12 months after ischemic stroke reduces long-term risks of both CVD death and non-CVD death.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
Background Thoracoscopic wedge resection is generally accepted as a standard surgical procedure for primary spontaneous pneumothorax. Because of the relatively high recurrence rate after surgery, ...additional procedures such as mechanical pleurodesis or visceral pleural coverage are usually applied to minimize recurrence, although mechanical pleurodesis has some potential disadvantages. The aim of this study was to clarify whether an additional coverage procedure on the staple line after thoracoscopic bullectomy prevents postoperative recurrence compared with additional pleurodesis. Methods A total of 1,414 patients in 11 hospitals with primary spontaneous pneumothorax undergoing thoracoscopic bullectomy were enrolled. After bullectomy with staplers, patients were randomly assigned to either the coverage group (n = 757) or the pleurodesis group (n = 657). In the coverage group, the staple line was covered with absorbable cellulose mesh and fibrin glue. The pleurodesis group underwent additional mechanical abrasion on the parietal pleura. Results The coverage group and the pleurodesis group showed comparable surgical outcomes. After a median follow-up of 19.5 months, the postoperative 1-year recurrence rate was 9.5% in the coverage group and 10.7% in the pleurodesis group. The 1-year recurrence rate requiring intervention was 5.8% in the coverage group and 7.8% in the pleurodesis group. The coverage group showed better recovery from pain. Conclusions In terms of postoperative recurrence rate, visceral pleural coverage after thoracoscopic bullectomy was not inferior to mechanical pleurodesis. Visceral pleural coverage may potentially replace mechanical pleurodesis, which has potential disadvantages such as disturbed normal pleural physiology.
: Stem cells are undifferentiated cells, which have the important properties of self‐renewal and differentiation. Adipose‐derived stem cells (ADSC) have relative advantages in accessibility and ...abundance compared to other kinds of stem cells. Regeneration therapy using ADSC has received attention in the treatment of various dermatologic diseases. In previous studies, ADSC were shown to have antioxidant, whitening and wound‐healing effects in the skin through secretion of growth factors and by activating fibroblasts. In this study, we investigated whether ADSC could be used as an anti‐ageing therapy, especially by dermal collagen synthesis and angiogenesis. Subcutaneous injection of ADSC significantly increased collagen synthesis in hairless mice, and dermal thickness, collagen density and fibroblast number also increased. In addition, procollagen type I protein and mRNA expression increased, which accounts for the increased dermal collagen density. Angiogenesis, which was visualized by CD31 and NG2 immunofluorescence stains, also increased in ADSC‐treated skin. Our results suggest that ADSC therapy may be useful in ageing skin. Its effects are mainly mediated by stimulating collagen synthesis in dermal fibroblasts and increasing angiogenesis.
Human epidermis elaborates two small cationic, highly hydrophobic antimicrobial peptides (AMP), β-defensin 2 (hBD2), and the carboxypeptide cleavage product of human cathelicidin (hCAP18), LL-37, ...which are co-packaged along with lipids within epidermal lamellar bodies (LBs) before their secretion. Because of their colocalization, we hypothesized that AMP and barrier lipid production could be coregulated by altered permeability barrier requirements. mRNA and immunostainable protein levels for mBD3 and cathelin-related antimicrobial peptide (CRAMP) (murine homologues of hBD2 and LL-37, respectively) increase 1–8hours after acute permeability barrier disruption and normalize by 24hours, kinetics that mirror the lipid metabolic response to permeability barrier disruption. Artificial permeability barrier restoration, which inhibits the lipid-synthetic response leading to barrier recovery, blocks the increase in AMP mRNA/protein expression, further evidence that AMP expression is linked to permeability barrier function. Conversely, LB-derived AMPs are also important for permeability barrier homeostasis. Despite an apparent increase in mBD3 protein, CRAMP−/− mice delayed permeability barrier recovery, attributable to defective LB contents and abnormalities in the structure of the lamellar membranes that regulate permeability barrier function. These studies demonstrate that (1) the permeability and antimicrobial barriers are coordinately regulated by permeability barrier requirements and (2) CRAMP is required for permeability barrier homeostasis.
Background
In superficial fungal infections, prompt diagnosis and treatment are essential to prevent the spread of infection and minimise the impact on patients' quality of life. Traditional ...diagnostic methods, such as KOH smear and fungal culture, have limitations in terms of sensitivity and turnaround time. Recently, the PCR‐reverse blot hybridization assay (PCR‐REBA) has been developed for the direct detection of dermatophyte DNA. However, there is a lack of information assessing the diagnostic accuracy of PCR‐REBA.
Objectives
This systematic review aimed to evaluate the diagnostic accuracy of PCR‐REBA in superficial fungal infections compared to conventional and molecular methods.
Methods
The comprehensive search containing Ovid MEDLINE and Embase databases was conducted on 7 August 2022. Two reviewers independently reviewed the included articles. Quality assessment was performed using the Newcastle‐Ottawa Scale tool.
Results
The included studies were conducted in Korea (five studies) and the Netherlands (two studies), all of which were conducted in a single institution. The quality assessment of these studies indicated low risk of bias. When compared to the potassium hydroxide (KOH) smear and fungus culture, the sensitivity of PCR‐REBA ranged from 85% to 100%, and the positive predictive values ranged from 58.9% to 100%. When compared to the RT‐PCR, the sensitivity of PCR‐REBA ranged from 93.3% to 100%, and the positive and negative predictive values were 91.6%–99.6% and 81.0%–89.1%, respectively.
Conclusions
The PCR‐REBA shows promise as a valuable diagnostic tool for dermatophytosis, offering practical and cost‐effective benefits.
Although systemic inflammatory responses are common after endovascular aortic repair (EVAR), its etiology remains uncertain. It is normally well tolerated and has a benign course. This study was ...undertaken to investigate the possible etiology of post-EVAR inflammation by measuring volumes of chronic mural thrombus and fresh thrombus.
The subjects of this study included 34 patients who underwent EVAR from February 2012 to July 2017. Inflammatory markers in all the patients were evaluated before surgery, using the highest value among the laboratory data up to 5 days after surgery, and postoperative computed tomographic angiography (CTA) was taken for all of them before their discharging. Volumes of mural thrombus and fresh thrombus were calculated by CTA. The mean interval from surgery to immediate postoperative CTA was estimated as 6.8 ± 4.0 days.
After undergoing EVAR, white blood cell (WBC) (p < 0.01), C-reactive protein (CRP) (p < 0.01) and erythrocyte sedimentation rate (ESR) (p = 0.01) were significantly elevated. Two groups were defined according to the post-implantation syndrome (PIS) by the criteria of systemic inflammatory response syndrome (SIRS);no significant differences were observed in any factors between the two groups. Classification of two groups by the criteria of increasing WBC and CRP revealed that inflammatory markers were significantly enhanced as the volume of mural thrombus increased (p = 0.03). However, no significant risk factor was found in view of aneurysmal growth after EVAR.
Volume of mural thrombus is an important risk factor for the elevation of inflammatory markers after EVAR.
We encountered a 35-year-old male patient with scimitar syndrome, acute type A aortic dissection, and severe iliac malperfusion. He presented with pain and weakness of the left lower extremity and ...underwent ascending aorta replacement through sternotomy and right anterior thoracotomy. Left iliac artery flow was recovered after the operation and the patient was discharged on the tenth day without any complication. The purpose of this report is to discuss the considerations in aortic surgery in patients with scimitar syndrome, and the surgical principles for treatment of aortic dissection with critical limb ischemia.
Psychological stress (PS) increases endogenous glucocorticoids (GC) by activating the hypothalamic-pituitary-adrenal axis. The negative effects of GC on skin barrier function under PS have been ...well-established. However, endogenous GC can also be active when cortisone (inactive form) is converted to cortisol (active form) by 11β-hydroxysteroid dehydrogenase type I (11ß-HSD1) in the peripheral tissue. Here, we evaluated the changes in 11ß-HSD1 and barrier function under PS. Elevated 11ß-HSD1 in oral mucosa correlated with increased cortisol in the stratum corneum and deteriorated barrier function. Expression of 11ß-HSD1 in the oral mucosa correlated with that in the epidermal keratinocytes. We further investigated whether barrier function improved when PS was relieved using a selective serotonin reuptake inhibitor (SSRI) in patients with anxiety. Decreased 11ß-HSD1 and improved barrier function were observed after SSRI treatment. The collective findings suggest that elevated 11ß-HSD1 under PS increases the level of cutaneous GC and eventually impairs barrier function. PS-alleviating drugs, such as SSRI, may help to treat PS-aggravated skin diseases.
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