Can EGCG Alleviate Symptoms of Down Syndrome by Altering Proteolytic Activity?

Down syndrome (DS), also known as "trisomy 21", is a genetic disorder caused by the presence of all or part of a third copy of chromosome 21. Silencing these extra genes is beyond existing technology and seems to be impractical. A number of pharmacologic options have been proposed to change the quality of life and lifespan of individuals with DS. It was reported that treatment with epigallocatechin gallate (EGCG) improves cognitive performance in animal models and in humans, suggesting that EGCG may alleviate symptoms of DS. Traditionally, EGCG has been associated with the ability to reduce dual specificity tyrosine phosphorylation regulated kinase 1A activity, which is overexpressed in trisomy 21. Based on the data available in the literature, we propose an additional way in which EGCG might affect trisomy 21-namely by modifying the proteolytic activity of the enzymes involved. It is known that, in Down syndrome, the nerve growth factor (NGF) metabolic pathway is altered: first by downregulating tissue plasminogen activator (tPA) that activates plasminogen to plasmin, an enzyme converting proNGF to mature NGF; secondly, overexpression of metalloproteinase 9 (MMP-9) further degrades NGF, lowering the amount of mature NGF. EGCG inhibits MMP-9, thus protecting NGF. Urokinase (uPA) and tPA are activators of plasminogen, and uPA is inhibited by EGCG, but regardless of their structural similarity tPA is not inhibited. In this review, we describe mechanisms of proteolytic enzymes (MMP-9 and plasminogen activation system), their role in Down syndrome, their inhibition by EGCG, possible degradation of this polyphenol and the ability of EGCG and its degradation products to cross the blood-brain barrier. We conclude that known data accumulated so far provide promising evidence of MMP-9 inhibition by EGCG in the brain, which could slow down the abnormal degradation of NGF.

Impact of incorrect oral habits on mastication anomalies in children and adolescents - literature review and own observations.

Incorrect oral habits, such as dysfunctions and parafunctions, may lead to the development of mastication anomalies. The present analysis covered 15 publications from the years 2005-2015, examining the impact of harmful oral habits and dysfunctions on the development of mastication anomalies. Based on the literature review, it can be stated that malocclusion is common in populations worldwide, irrespective of the racial background. The malocclusion severity is related to harmful habits and dysfunctions co-occurring during individual development. The prevalence of malocclusion and dental anomalies related to parafunctions and dysfunctions has been observed to have a tendency to grow.

Occluso-facial manifestations in a child with the Rasmussen syndrome and intercurrent Lyme disease--a case report.

The Rasmussen Syndrome (RS) is a rare neurological disease, usually diagnosed in the 1st decade of life, leading to damage of the central nervous system. It is characterised by sudden seizures in previously healthy children. In this paper we present a case of a 14-year-old female patient with malocclusion who reported to the orthodontic clinic to start orthodontic treatment. The first symptoms of nervous system disorders manifested when the patient was 8 years old. Until then, the patient had been developing correctly. The interview revealed that at the age of 9 the patient was diagnosed with Lyme disease and a long-term antibiotics therapy was started. At the age of 10, the Rasmussen syndrome was diagnosed (the antibiotics therapy was discontinued) and confirmed by magnetic resonance imaging (MRI) and histopathological examination after brain biopsy. The study focuses in particular on the changes in the craniofacial skeleton and oral cavity, taking into account dynamically developing and increasing occlusion defects. The ethical aspect of the treatment has also been considered.

Holistic approach to treatment in the context of bioethics.

Hippocrates saw the man as the unity of the body, mind and soul, and the disease as the absence of balance between the internal and external world. The most important concept of the holistic treatment is to take the interest in the entire patient and not only in the illness affecting negatively his or her health. That approach regards the patient as the subject, defines the patient's needs and provides multidisciplinary treatment, taking into account not only the physical but also emotional, psychological, spiritual and social needs of the sick person. Such an approach allows to obtain better therapeutic effects.

The use of botulinum toxin for grinding in patients with Rett syndrome-Case report.

AIMS: Rett syndrome (RTT) is a neurodevelopmental disorder caused by a mutation in the methyl-CpG-binding protein 2 (MECP2) gene. It is characterized by profound and severe motor disability. Patients with RTT grind their teeth, causing damage to their permanent teeth. This article aims to describe the case of an 8-year-old child with RTT and the methods of botulinum toxin-based treatment for teeth grinding. METHODS AND RESULTS: Surface electromyography (sEMG) was used to monitor the effects of treatment. The masseter and temporal muscles, responsible for the elevation of the mandible, were injected with botulinum toxin. The results of the sEMG examination performed 1 week, 1 month and 4 months after toxin administration were compared to the sEMG conducted before treatment. The research demonstrates a significant reduction in both grinding frequency and tension of the masseter and temporal muscles. CONCLUSION: Possible adverse effects after chronic use of botulinum toxin were discussed. The article also emphasizes the need for masticatory muscle rehabilitation during botulinum toxin therapy. However, it is necessary to repeat this procedure on a larger group of children with RTT to establish our efforts' efficacy.

Impact of Socioeconomic Status on the Perception of Accessibility to and Quality of Healthcare Services during the COVID-19 Pandemic among Poles-Pilot Study.

This pilot study was conducted on the reported hypothesis that the COVID-19 pandemic outbreak had an impact on the accessibility and quality of healthcare services and exacerbated socioeconomic inequalities. The aim was to determine whether economic status and education had an impact on the perception of access and quality to healthcare services during the COVID-19 pandemic and whether, according to patients, accessibility and quality had changed significantly compared to the pre-pandemic period in Poland. The study was based on the authors' questionnaire and the results were statistically analyzed. Two hundred forty-seven feedback responses were received with a responsiveness rate of 93 percent. Statistically significant differences were found when comparing education level and utilization of healthcare services during the COVID-19 pandemic. A comparison of gender and economic situation, and average monthly income found no statistically significant differences. The outbreak of the COVID-19 pandemic has undoubtedly affected the provision of health services in many countries around the world. One result of the pandemic crisis has been widening socioeconomic inequalities among patients.

Correlation between religion, spirituality and perception of healthcare services utilisation in Poland during COVID-19 pandemic.

Background: The worldwide transmission of SARS CoV-2 caused the COVID-19 pandemic and had an impact on healthcare provision. The disruption of reliance on the health system during the COVID-19 pandemic posed a clear threat to public trust. Religiosity, like spirituality, is believed to have a positive influence on people's lives, enabling them to cope with illness, stress, and sudden life changes. In practice, although the terms religiosity and spirituality have similar meanings and are related, they are not identical concepts. The aim of this study is to compare the perceptions of the accessibility and quality of healthcare services provided before and during the COVID-19 pandemic in Poland by religious/spiritual people compared to those for whom religion and spirituality have little or no importance in their lives. Methodology: This cross-sectional study was based on the authors' questionnaire, carried out during the third wave of the COVID-19 pandemic in Poland. Participants in the study were people living in various regions of Poland over 18 who were willing to complete the questionnaire voluntarily. The number of people sampled was two hundred and sixty-four. Convenience sampling method was used for this study. Statistical calculations were performed using Statistica 13 software from TIBCO and PQStat from PQStat Software and were based on the Kruskal-Wallis test, multiple regression model, the chi2 test of independence or the Mann-Whitney test. The result was considered statistically significant when p < α. The significance level was taken as α = 0.05. Results: Two hundred and sixty-three respondents answered the questionnaire. Among them, 181 (69%) were women, and 82 (31%) were men. It was shown that religion is more important for women than for men and women who report a high role of religion in their lives rated the quality of healthcare services better before and during the COVID-19 pandemic. It was also concluded that religious people for whom religion played a major role and those who were helped by spiritual life rated accessibility and quality higher both before and during the pandemic. Conclusions: Religious/spiritual people, through their more positive attitudes towards the world, were thought to rate access and quality of healthcare services better. Regardless of religious affiliation, the help of spiritual life during the pandemic or the importance of religion in life in all respondents, perception of healthcare services utilisation were decreased by the pandemic. This prompts thoughts on the implementation of spiritual assistance as a supportive measure to mitigate the effects of the pandemic.

Quality of the Healthcare Services During COVID-19 Pandemic in Selected European Countries.

Background: There are several definitions of the quality of healthcare services. It may be defined as a level of value provided by any health care resource, as determined by some measurement. Scientists use a variety of quality measures to attempt to determine health care quality. They use special indicators or based on a patients' or healthcare professional's perception. This article aims to provide a short review of the available data on the quality of healthcare services in selected European countries during the COVID-19 pandemic. Methodology: The research was done by the use of online databases such as PubMed, Google Scholar, and Science Direct. All the studies focused on the quality of healthcare services, yet the studies used different methods to measure this quality. In addition, the results of the authors' survey on the assessment of the quality of healthcare services before and during the COVID-19 pandemic were presented. Results: Among twelve studies, four were from the United Kingdom and one each of Catalonia, Italy, Sweden, Poland, Netherlands, France, Germany, Belgium. Patients in the United Kingdom felt that the quality of services was good during the pandemic, whereas the quality declined in the other studies cited. The results of our research also revealed a decrease in the quality of healthcare services provided. Conclusions: Nevertheless the development of telemedicine has had a positive impact on the quality of healthcare services. The COVID-19 pandemic has undoubtedly affected most European countries' quality of healthcare services.

The Influence of Parent Education on the Neurobehavior and Sucking Reflexes of Very Preterm Infants.

The diagnosis of neurobehavioral problems in very preterm neonates helps with planning and applying proper and direct therapeutic interventions. (1) Background: The aim of this study was to determine the direct impact of neurobehavior on the sucking reflex and eating abilities of neonates. (2) Methods: We assessed 18 preterm neonates twice hospitalized at the Gynecology and Obstetrics Clinical Hospital through the use of the Neonatal Behavioral Assessment Scale (NBAS). (3) Results: We found that that a neonate's sucking ability positively correlated with the activity level item from the motor system cluster of the NBAS. (4) Conclusions: Neurobehavior should be closely assessed in very preterm neonates. Firstly, because assessments can detect fundamental problems and help a practitioner plan for early intervention. Secondly, the education of parents regarding the neurobehavior of their child can help in the facilitation of feeding skills and the planning of early rehabilitation.

The Importance of Monitoring Neurodevelopmental Outcomes for Preterm Infants: A Comparison of the AIMS, GMA, Pull to Sit Maneuver and ASQ-3.

BACKGROUND: Clinicians and parents should closely monitor the neurodevelopment of very preterm infants. The aim of our study was to compare whether neurodevelopmental assessments completed by parents and those done by specialists yielded similar outcomes. We wanted to check whether the assessments completed by specialists and parents were comparable in outcomes to emphasize the important roles of early assessment of a child and of the parents in their child's treatment and medical care. Another aim was to check whether or not the pull to sit maneuver from the Neonatal Behavioral Assessment Scale (NBAS) is still a parable item in well-known scales of neurodevelopment. METHODS: We assessed 18 preterm neonates in the fourth month of corrected age with scales such as the General Movement Assessment (GMA), the Alberta Infant Motor Scale (AIMS), and the pull to sit maneuver from the NBAS. Finally, we asked parents to complete the Ages and Stages Questionnaire, Third Edition (ASQ-3). RESULTS: We found that the respective assessments completed by specialists and parents are comparable in outcomes. We also found that the pull to sit item from the NBAS was still a valid test since it showed similar findings to those from the AIMS, the GMA, and the ASQ-3. CONCLUSIONS: The pull to sit item from the NBAS is an important item for assessment of very preterm infants. Specialists should also take into consideration the input and concerns of parents when planning for treatment and intervention.

The Evaluation of Facial Muscles by Surface Electromyography in Very Preterm Infants.

BACKGROUND: It is reported that 40% of preterm infants have problems with eating. Neonatal feeding disorders may be one of the factors increasing neonatal mortality. The aim of our study was to evaluate the muscles involved in suckling and swallowing in premature newborns using surface electromyography (sEMG). We would like to objectively describe the tension of muscles engaged in feeding in order to properly plan the therapy. Another aim was to compare sEMG measurements to gestational age, birth weight, and umbilical blood pH to show which parameters put children at risk of feeding problems. METHODS: Sixteen preterm neonates with gestational age less than 32 weeks, birth weight less than 1500 g, and oral feeding difficulties were analyzed for muscle response and electrical activity of nerves using sEMG (surface electromyography). RESULTS: We found a negative correlation indicating that preterm infants with a younger gestational age had higher suprahyoid muscle tension, and a positive correlation was found between pH value and suprahyoid muscles. The lower the pH value, the lower the tension in the suprahyoid muscles. CONCLUSIONS: sEMG may be a helpful diagnostic tool in the evaluation of the masticatory system of premature infants. Due to the abnormal tone of the muscles responsible for swallowing, it is advisable to rehabilitate as early as possible.

Accessibility to Non-COVID Health Services in the World During the COVID-19 Pandemic: Review.

Background: COVID-19 pandemic caused by SARS-CoV2 has seriously impacted the global economy. Medical facilities around the world were not prepared for the enormous challenges posed by the growing number of patients each day, the shortage of personal protective equipment, and insufficient numbers of medical staff. Governments have tried to counteract the impact of the pandemic, but the measures taken have not always been sufficient to maintain access to and quality of health services at the same level as before the pandemic. The disruption of health services has resulted in more and more research reports from different parts of the world on the accessibility of health services during the COVID-19 pandemic. Methodology: This review article presents 21 selected scientific studies on access to health services in different regions of the world. Articles were found in PubMed, GoogleScholar, Medline, and ScienceDirect databases, then grouped, and significant data were extracted from each article. The results were summarized in a table. Results: The range of limited health services included a variety of specialties, including primary care, psychiatry, orthopedics, cardiology, neurosurgery, and more. Methods used in the studies were based on retrospective analysis or on the subjective assessment of patients in the form of a questionnaire or interview. Most authors claimed a decrease in accessibility to health services during the COVID-19 pandemic compared to the pre-pandemic period, including a decrease in planned surgeries, doctor appointments, patient admission to hospital or ER, and access to medicines. Additionally, some authors observed an increase in the mortality rate. One of the few medical services that have expanded rapidly during the pandemic was online appointments. Conclusions: The COVID-19 pandemic has most certainly affected the accessibility of health services worldwide. Lessons should be learned to prevent inaccessibility to medical services, especially as experts predict another wave of COVID-19 cases.

Instrumental diagnosis of facial skin-A necessity or a pretreatment recommendation in esthetic medicine.

BACKGROUND: This paper presents the possibilities of pretreatment instrumental diagnosis for assessing the condition of various facial skin areas. AIM: Tests which aimed at determining the firmness and elasticity of facial skin were conducted. METHODS: Analysis of skin parameters in selected facial areas in adults over a wide age range was performed using the Cutometer Dual MPA 580 probe. Parameters R0, R2, R5, R7, R8, and R9 were analyzed. RESULTS: The following results were obtained: R0 (0.20-0.33) mm; R2 (0.66-0.82) a.u.; R5 (0.60-0.92) a.u.; R7 (0.37-0.52) a.u.; R8 (0.10-0.27) mm; and R9 (0.03-0.05) mm. The R0 parameter showed no statistically significant correlation between skin firmness and age. On the basis of the other parameters, it can be concluded that skin elasticity and an ability to return to its original shape around the eyes, on the cheeks and around the lips, are greater in younger patients than in older groups. There were no statistically significant differences within each age group between the right and left sides of the face. CONCLUSION: Skin firmness described by the R0 parameter is constant in the studied groups and independent of age. Skin elasticity, and an ability to return to its original shape around the eyes, on the cheeks and around the lips, is greater in younger patients. The aging process simultaneously affects the entire facial skin.

Ototoxicity after platinum-based chemotherapy in the treatment of melanotic neuroectodermal tumour of infancy.

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare infantile tumor that originates from mesenchymal-neuroectodermal cells, the treatment of which uses platinum derivatives that can affect hearing loss. The present study evaluated the long-term effects of ototoxicity following chemotherapy with cisplatin, vincristine, cyclophosphamide, teniposide and adriamycin in a 10-year-old patient after surgical removal of a MNTI tumor at the age of 8 months. Audiometric tests (high-frequency tonal audiometry, speech audiometry, speech acoustics, tympanometry and absorbance measurements) were performed during a 10-year follow-up after receiving chemotherapy. Hearing disorders in the high-frequency range (6,000 to 16,000 Hz range) were demonstrated for both ears, indicating that these may be the long-term effects of chemotherapy with use of platinum compounds during the treatment of infants.

The impact of the long-term playing of musical instruments on the stomatognathic system - review.

In this article, we have made a review of the influence of playing musical instruments on the formation of malocclusion and TMJ disorders in musicians. Primary attention was paid to the effects of wind and stringed instruments. The aim of the article was the presentation of research and opinions about this problem in the last 25 years. It is reported that long-term and repetitive playing of musical instruments, particularly stringed (violin and viola) and wind instruments can cause dysfunctions of the stomatognathic system. The impact of wind instruments was assessed in terms of the type of mouthpiece. We studied the possibility of repositioning the front teeth and reducing the width of the upper dental arch and overbite. There were also reports on the use of a specific instrument to improve the child's occlusion. Studies have also been performed on the usefulness of relaxation plates in order to improve, and even prevent, dysfunction caused by the constant stress on the same parts of the stomatognathic system. The experiments were mainly based on interviews, dental cast analyses and cephalometric analyses. Additional methods were dynamometer tests and muscle tension palpation.

Smith-Lemli-Opitz syndrome - clinical consequences for dental care.

The Smith-Lemli-Opitz syndrome (SLOS) is a congenital, genetically conditioned, metabolic disorder with autosomal recessive inheritance. The syndrome is caused by high levels of cholesterol precursors, i.e. 7-dyhdrocholesterol (DHCR7) and 8-dehydrocholesterol (DHCR8), which results in cholesterol synthesis disorders. Cholesterol deficiency leads to a series of developmental disorders in the foetus and in extreme cases even death. The diagnosis of that congenital metabolic disorder is difficult due to a diversity of clinical symptoms (there are many mutations of the DHCR7 and DHCR8 genes), which can be exemplified by such cases as that of the 5-year-old boy with SLOS described below. In the presentation the authors have emphasized the problems related to hygienic procedures of the oral cavity and dental treatment, as well as the importance of the early diagnosis of the syndrome and of cooperation with parents. The publication aims at drawing the attention of clinicians to the need of taking SLOS into account in the differential diagnostics in children.