Diagnosis and treatment of Fabry disease. Expert Opinion of the Polish Cardiac Society and the Polish Forum for Fabry Disease.

Fabry disease (FD) belongs to the group of lysosomal storage diseases (LSD), which are characterised by insufficient activity of enzymes responsible for the intra-lysosomal breakdown of various substrates. The result is an uncontrolled accumulation of by-products of cellular metabolism. Lysosomal storage diseases are inherited diseases, transmitted mainly in an autosomal recessive fashion.

Sleep Efficiency and Neurocognitive Decline in Atopic Dermatitis: A Systematic Review.

Atopic dermatitis is often accompanied by a lack of sleep efficiency, which may impact neurocognitive functions. This review assesses the association between sleep quality in atopic dermatitis patients and neurocognitive decline. Databases searched included PubMed, Scopus, and Web of Science from inception to 8 January 2024, adhering to PRISMA guidelines.

The Impact of Disease Severity on the Serum Levels of Significant Neutrophil Extracellular Trap (NET) Proteins in Patients with Psoriasis.

Psoriasis is an inflammatory skin disease with various symptoms of differing severities and with the reported prominent involvement of neutrophil extracellular traps (NETs). The excitation of neutrophils, e.g.

Fostering social health of people with dementia: evaluation of dementia awareness campaign in Poland.

Background: Due to the need to increase social awareness about dementia and the needs of patients living with dementia in Poland, the (eng. ) campaign was created. The aim of the study was to evaluate its effectiveness.

mRNA expression of caspase 14 in skin epithelial malignancies.

Introduction: The skin is the largest organ in the human body and it is also a complex organ. Its protective function is properly maintained due to its continuous renewal. Malignancies develop when the balance between proliferation and cell death is dysregulated in skin cells.

Satisfaction with Life and Coping Strategies among Patients with Hidradenitis Suppurativa: A Cross-Sectional Study.

Introduction: Hidradenitis suppurativa (HS) is a chronic recurrent inflammatory dermatosis with vast psychosocial burden. The objective of this study is to thoroughly analyze satisfaction with life (SWL) and coping strategies of HS patients in relation to the clinical and psychosocial factors.

Methods: 114 HS patients (53.

Characteristics of pruritus in various clinical variants of psoriasis: Final report of the binational, multicentre, cross-sectional study.

Background: Pruritus, which is the most frequent subjective symptom of psoriasis, may cause significant discomfort, embarrassment and even interfere with patients normal daily activities. However, the perception of itch in various psoriasis subtypes remains unknown.

Objectives: The aim of this study was to investigate and to characterize pruritus in different clinical variants of psoriasis.

The Impact of Pruritus on the Quality of Life and Sleep Disturbances in Patients Suffering from Different Clinical Variants of Psoriasis.

Background: Quality of life (QoL) and sleep, which are essential for well-being in the mental, physical, and socioeconomic domains, are impaired in psoriatic patients. However, the exact role of the clinical subtype of psoriasis in this aspect remains poorly studied.

Objectives: The aim of this study was to investigate differences in QoL impairment and sleeping problems in patients suffering from various clinical subtypes of psoriasis and to evaluate the effects of pruritus on QoL.

Atypical presentation of Sweet syndrome with nodular erythema and oral ulcerations provoked by Ad26.COV2.S SARS-CoV-2 vaccination and review of literature.

The aim of this article is to present the case of acute febrile neutrophilic dermatosis (Sweet syndrome-SS) after Ad26.COV2.S vaccination against SARS-CoV-2.

The role of the clinical and psychological symptoms of mastocytosis in the patient's quality of life.

Introduction: Patients with mastocytosis have various clinical and psychological symptoms, for example, life-threatening anaphylactic reactions or anxiety, resulting in decreased quality of life (QoL).

Aim: To assess the clinical and psychological symptoms (such as depression, anxiety) as well as the quality of life and satisfaction with life in patients with mastocytosis.

Material And Methods: The study group included 85 patients with mastocytosis (57 women and 28 men) treated at the Department of Allergology, Medical University of Gdansk, Poland.

Evaluation of knowledge in the field of proper skin care and exacerbating factors in patients with psoriasis.

Introduction: Psoriasis is one of the most common dermatological diseases. It is characterized by a chronic course with periods of exacerbations and remissions. The recurrent nature of the disease and the influence of environmental factors on its course require the patients not only to be treated but also to have knowledge of prophylaxis and proper skin care.

Venous Leg Ulcers: Advanced Therapies and New Technologies.

The prevalence of venous leg ulcers (VLUs) differs between 1.5% and 3% in the general population. The challenge in treating VLUs is common recurrence.

The microbiome of the human skin and its variability in psoriasis and atopic dermatitis.

The human organism is inhabited by very diverse microorganisms, which constitute the so-called human microbiome and are necessary for the proper functioning of the macroorganism. The correct microbiome ensures homeostasis of the body. A disturbance in its homeostasis leads to dysbiosis.

Ocular manifestations of skin diseases with pathological keratinization abnormalities.

Keratinization means cytodifferentiation of keratinocytes turning into corneocytes in the stratum corneum. Disorders of keratinization (hyperkeratosis, parakeratosis and dyskeratosis) are causing many dermatological diseases, including various types of ichthyoses, pachyonychia congenita, pityriasis rubra pilaris, all subtypes of psoriasis, pityriasis lichenoides, dyskeratosis congenita, leukoplakia and keratosis follicularis, which apart from skin lesions may affect the eye's adnexae causing ectropion, entropion, blepharitis, madarosis, and trichiasis, the ocular surface causing keratitis, conjunctivitis, corneal ulceration and episcleritis, which in turn cause uveitis and various fundoscopic changes (proliferative retinopathy, retinal vasculopathy, macular oedema and birdshot chorioretinopathy). Knowledge of ocular symtoms associated with pathological keratinization is crucial, preventing sight-threatening complications such as corneal perforation, lagophthalmus, phthisis bulbi, retinal neovascularization, retinal vasculopathy and optic nerve atrophy.

Characteristics of Pruritus in Various Clinical Variants of Psoriasis: Results of the Multinational, Multicenter, Cross-Sectional Study.

Psoriasis is a chronic, inflammatory skin disease present in about 3% of the world's population. The clinical symptoms manifest diversely, therefore one can distinguish several subtypes of psoriasis. The majority of patients with psoriasis experience pruritus, which is an unpleasant sensation that decreases patients' quality of life.

Caspase-14-From Biomolecular Basics to Clinical Approach. A Review of Available Data.

Caspase-14 is a unique member of the caspase family-a family of molecules participating in apoptosis. However, it does not affect this process but regulates another form of programmed cell death-cornification, which is characteristic of the epidermis. Therefore, it plays a crucial role in the formation of the skin barrier.