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Papillon-Lefëvre syndrome (PLS), classified as ectodermal dysplasia, is an autosomal recessive condition related to the cathepsin C (CTSC) gene mutation. The first clinical symptoms, occurring most commonly between the ages of 1 and 4, are palmoplantar hyperkeratosis and also periodontitis resulting in the loss of most or all teeth in the same sequence in which they erupted. Most often the redness of palms and soles precede the occurrence of keratoderma. Moreover, excessive sweating, moderate mental retardation, the tendency to purulent skin and internal organs infection may occur. Lack of cathepsin seems to have a crucial role in the intensity of symptoms. In most of the patients, there can be observed impairment of phagocytosis and chemotaxis of neutrophils, granulocytes, leukocytes and cytotoxic lesion of fibroblasts and macrophages. Also, functional impairment of lymphocytes, neutrophils, and monocytes is observed. The study, using flow cytometry, showed a decreased percentage of T cells CD8+ and increased CD4:CD8 ratio., Competing Interests: The authors declare no conflict of interest., (Copyright © 2020 Termedia.)

Introduction: The Williams syndrome (WS) is a rare genetic disease. The disorder is characterized by symptoms, such as facial dysmorphism, defects within the masticatory organ and cardiovascular system as well as intellectual and physical disability. Moreover, the disorder may also affect vision or hearing, gastrointestinal and urogenital system and skin. Limited cooperation with children suffering from WS is related not only from their young age and fear of dental treatment but also with hypersensitivity to sounds, which is one of the symptoms of this disease. Therefore, there is a need to carry out special dental treatment of such patients., Aim: The aim of the paper is to describe the case of a patient with Williams syndrome., Case Report: The girl showed characteristic findings of the disease such as heart defect and typical facial features (known as “ elfin face” with full lips, wide mouth, long and smooth philtrum, prominent forehead and malocclusion). A radiological examination revealed lack of few permanent premolars and incisor. In addition, the patient had many teeth with active caries process and its complications. Therefore, comprehensive dental therapeutic procedures were carried out. At the beginning of the treatment the sensitivity to high-pitched sounds as well as the significant anxiety of the child before dental intervention were the problem., Conclusions: In individuals with congenital syndromes it is necessary to prepare the patient for dental prophylaxis and therapeutic approach with regard to a variety of physical and intellectual limitations. In addition, there is a need for systematic dental care from their infancy.

The authors discuss the problem of joint inflammation based on the case of a 3 years old girl in whom the changes occurred in the lower limbs. The detailed diagnostic investigations confirmed the existence of the active focus in the oral cavity. The paper presents the case of a 3-year old girl with lower limbs joints inflamation. After their extraction a complete remission of inflammatory markers was achieved, which confirmed the correctness existence of an active focus. Furthermore, the presented case report demonstrates that oral health is a very important factor, which should be considered in the diagnosis and treatment of systemic diseases, including rheumathoid arthritis. It proves also that prevention of dental caries and oral hygiene should be implemented from early childhood.

Non carious lesions, including erosion changes, are becoming increasingly apparent. There are multiple factors involved in the etiology of dental erosion i.a. acids in commercially available drinks. The aim of this study was to determine the frequency of soft drink consumption that promote dental erosion among young adults. The 266 subjects were asked to fill in a questionnaire. The questionnaire inquired questions about consumption of drinks favouring tooth erosion. The students declared frequent drinking of isotonic drinks, energetic drinks, fizzy drinks and coca-cola type drinks. On the basis of a survey of Poznań University of Medical Sciences students it can be determined, that they have relatively high risk of dental erosion. To minimize the risk of dental erosion occurrence in young population there is a need to disseminate knowledge about the etiology.