Chronic granulomatous disease in the United Arab Emirates: clinical and molecular characteristics in a single center.

Background: Chronic granulomatous disease (CGD) is a genetic disorder caused by defective oxidative burst within phagocytes, manifesting as recurrent, severe infections as well as hyperinflammation. Objective: This is the first report from the United Arab Emirates (UAE) to describe the demographic, clinical, laboratory, radiological, and genetic characteristics of patients with CGD. Methods: This is a retrospective study that was conducted at Tawam Hospital in the UAE on patients with confirmed CGD between 2017 and 2022. Results: A total of 14 patients were diagnosed with CGD, of whom 13 patients had autosomal recessive (AR) CGD due to NCF1 deficiency. Consanguinity was noted in all patients with AR CGD, whereas positive family history was identified in 50% of cases. The median age of onset of symptoms was 24 months, while the median age at diagnosis was 72 months. Lymphadenitis was the most common clinical feature identified in 71% of patients. Other common infectious manifestations included abscess formation (57%), pneumonia (50%), invasive aspergillosis (21%), oral thrush (14%), and sepsis (14%). Disseminated trichosporonosis was reported in one patient. Autoimmune and inflammatory manifestations included celiac disease in two patients, diabetes mellitus and asymptomatic colitis in one patient each. Genetic analysis was performed in all patients; NCF1 deficiency was diagnosed in 13 (93%) patients, with c.579G>A being the most prevalent pathogenic variant identified. The treatment modalities, as well as treatment of acute infections, treatment modalities included antimicrobial prophylaxis in 12 (86%) patients and hematopoietic stem cell transplant in six patients (42%). Conclusion: This is the first report from the UAE describing the clinical and molecular characteristics of patients with CGD. The homozygous variant c.579G>A causing NCF1 deficiency can be considered as a founder mutation for AR CGD in the UAE.

Personal protective equipment-related dermatoses in COVID-19 frontline health workers. A lesson learned from 1-year single center in the UAE.

Since COVID-19 was declared a pandemic in March 2020, frontline health care workers wear personal protective equipment (PPE, surgical masks, N95 or similar respirators, gloves, goggles, face shields, and gowns). Alcohol-based sanitizers and wipes were recommended. Such measures lead to disruption of the natural skin habitat and skin barrier and various cutaneous reactions. The aim was to assess the prevalence and characteristics of PPE-related dermatoses among health care workers in Sheikh Khalifa Medical City (SKMC), a COVID-19 facility, Abu Dhabi, United Arab Emirates. We conducted a voluntary, cross-sectional anonymous survey among first-line health care workers addressing types of PPE used, dermatoses classified as PPE related, and factors that influence them. Facial, nasal, and hand dermatoses were the most prevalent with 40.2%, 19.9%, and 14.1%, respectively. The changes are primarily attributed to surgical masks, N-95 masks, and gloves. The shift duration is a contributing factor correlating with the severity of skin damage. Results of this study encouraged decision makers to recognize PPE-related dermatoses as a continuously growing burden, reorganized the shift duration and PPE exposure, animated the personal to apply preventive measures, and promoted the well-being of medical professionals in new waves of the pandemic.

Interrupting aerosol spread and nasal acquisition of SARS-CoV-2 with topical trichloroacetic acid.

The devastating effects of the coronavirus designated severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) have merited many approaches to combat COVID-19. Since it is transmitted largely by fine droplets and aerosols, air defense with suitable ventilation and mask use might be enhanced by attacking the virus and its likely main point of invasion, the nasal mucosa. We recommend formal clinical trials employing topical trichloroacetic acid (TCA), which successfully blocks the nasal entry point for airborne allergens and to be curative for the early stages of viral infections of the oral mucosa. TCA chemically redesigns the nasal and nasopharyngeal epithelia to in effect remove receptors and cell, allowing SARS-CoV-2 viral attachment. TCA fits the ideal category for medication, being inexpensive, readily available, easy-to-use, and proven to be safe for suggested use. We postulate its potential efficacy in SARS-CoV-2 infection and urge consideration of clinical trials. Local delivery of TCA in the form of contact application directly to the nasal cavity may have a preventive effect, potentially neutralizing virus entry and subsequential virus spread to the lower respiratory tract, especially for those at high risk. In addition, TCA may diminish the local viral load and its replication potential in those newly infected.

COVID-19: Topical agents and therapeutic prevention of nasal viral acquisition.

Since the spread of SARS-CoV-2 became a pandemic, the number of cases has been continuously growing worldwide. Numerous recommendations and suggestions have been published to prevent the acquisition and spread of the SARS-CoV-2, especially to protect health workers and front-line caregivers. SARS-CoV-2 is transmitted by aerosol, rendering air defense with suitable ventilation and adequate mask use pivotal. Recently, locally applied antiseptic, antiviral, or structure competitive receptor blockers were suggested to attack the virus at its main point of invasion, the nasal mucosa and nasopharynx. We discuss the most plausible and safe ideas to reduce viral load at the point of entry, and subsequently the spread of SARS-CoV-2 to the lower respiratory tract, lungs, and other organs. In addition, we analyze the value and recommend clinical trials employing topical trichloroacetic acid (TCA), a substance well known from dermatologic and cosmetic procedures. It has been proven to successfully block the nasal entry for airborne allergens, preventing the development of allergic rhinitis and asthma, and to be curative for early stages of viral infections entering through the oral mucosa. For SARS-CoV-2, TCA in a single, short-time application is expected to remodel the nasal and nasopharyngeal epithelia, eliminating both the receptors and cells responsible for viral entry and subsequent viral spread to the lower respiratory tract. Moreover, this may have therapeutic benefits for those recently infected by reducing local viral replication. Such procedures are cheap, safe, and can be conducted in almost every setting, especially in regions with inadequate financial and logistic resources.

Update on dermal substitutes.

Tissue-engineered biological dressings offer promise in the treatment of burns, chronic ulcers, donor site and other surgical wounds, and a variety of dermatologic conditions. Despite this promise, cellular tissue-engineered products such a Dermagraft and Apligraf have suffered setbacks in recent years with a lower market share than the commercial promoters of these products anticipated. AlloDerm acellular dermal matrix, an older technology than these cell-based products, has made strong progress in winning over clinicians in various disciplines. Similarly, Integra Bilayer Matrix Wound Dressing (BMWD) continues to gain acceptance beyond its original burn audience. A review on the products is offered.

Tissue-engineered temporary wound coverings. Important options for the clinician.

Finding an ideal temporary wound dressing is challenging. Although TransCyte, built on Biobrane, offers many of the characteristics of the ideal wound dressing, and may have added benefits from the delivery of extra-cellular matrix (ECM) and growth factors to the wound, the downside of high cost and less convenient storage and usage are considerable barriers to its broader adoption. The low cost and established clinical utility of Biobrane sets the bar high for new products.

Influence of different collagen species on physico-chemical properties of crosslinked collagen matrices.

Collagen-based scaffolds are appealing products for the repair of cartilage defects using tissue engineering strategies. The present study investigated the species-related differences of collagen scaffolds with and without 1-ethyl-3-(3-dimethyl aminopropyl) carbodiimide (EDC)/N-hydroxysuccinimide (NHS)-crosslinking. Resistance against collagenase digestion, swelling ratio, amino acid sequence, shrinkage temperature, ultrastructural matrix morphology, crosslinking density and stress-strain characteristics were determined to evaluate the physico-chemical properties of equine- and bovine-collagen-based scaffolds. Three-factor ANOVA analysis revealed a highly significant effect of collagen type (p=0.0001), crosslinking (p=0.0001) and time (p=0.0001) on degradation of the collagen samples by collagenase treatment. Crosslinked equine collagen samples showed a significantly reduced swelling ratio compared to bovine collagen samples (p< 0.0001). The amino acid composition of equine collagen revealed a higher amount of hydroxylysine and lysine. Shrinkage temperatures of non-crosslinked samples showed a significant difference between equine (60 degrees C) and bovine collagen (57 degrees C). Three-factor ANOVA analysis revealed a highly significant effect of collagen type (p=0.0001), crosslinking (p=0.0001) and matrix condition (p=0.0001) on rupture strength measured by stress-strain analysis. The ultrastructure, the crosslinking density and the strain at rupture between collagen matrices of both species showed no significant differences. For tissue engineering purposes, the higher enzymatic stability, the higher form stability, as well as the lower risk of transmissible disease make the case for considering equine-based collagen. This study also indicates that results obtained for scaffolds based on a certain collagen species may not be transferable to scaffolds based on another, because of the differing physico-chemical properties.

Effect of collagen matrices on dermal wound healing.

Dermal substitution and wound healing are areas of medicine in which there have been many recent advances, but neither the commercially available products nor the products currently described in experimental studies are able to fully substitute for natural living skin. There is an overall consensus that to heal wounds, the substitution of connective tissue matrix, the main component of each wound, is necessary. Both artificial and natural polymers have been used to reconstitute dermis. Nowadays, collagen has been discovered again. Collagen is a natural substrate for cellular attachment, growth and differentiation, and promotes cellular proliferation and differentiation. Once dermis reconstruction is done, the covering of the wound surface with both in vitro expanded epidermis and autologous split-skin transplants is significantly easier and has an improved chance of success. Nowadays, many commercial and experimental products have been introduced to improve cutaneous wound healing. This review discusses some of both acellular and cell-containing products used in the treatment of skin wounds.

Collagen as a carrier for on-site delivery of antibacterial drugs.

Due to its biocompatibility and well-established safety profile, collagen represents a favourable matrix for on-site drug delivery. In this review, we summarize some of the recent developments and applications of collagen as a biomaterial in drug delivery systems for antibiotics, especially gentamicin. The main clinical and experimental applications covered include: treatment and prophylaxis of bone and soft tissue infections, wound healing, as well as ophthalmic and periodontal treatment. Advantages of local drug application and the rationale of use local drug delivery systems for adjuvant (ancillary) therapy are discussed. Recent efforts in the use of collagen and collagen-synthetic polymer composites for controlled drug delivery as well as collagen-based diffusion membranes for prolonged drug release have also been included in this review.