Ocular Changes in Cystic Fibrosis: A Review.

Cystic fibrosis (CF), also known as mucoviscidosis, is the most common autosomal recessive genetic disease in the Caucasian population, with an estimated frequency of 1:2000-3000 live births. CF results from the mutation of the cystic fibrosis transmembrane conductance regulator (CFTR) gene localized in the long arm of chromosome 7. The product of CFTR gene expression is CFTR protein, an adenosine triphosphate (ATP)-binding cassette (ABC) transporter that regulates the transport of chloride ions (Cl-) across the apical cell membrane. Primary manifestations of CF include chronic lung and pancreas function impairment secondary to the production of thick, sticky mucus resulting from dehydrated secretions. It is well known that CF can cause both anterior and posterior ocular abnormalities. Conjunctival and corneal xerosis and dry eye disease symptoms are the most characteristic manifestations in the anterior segment. In contrast, the most typical anatomical and functional changes relating to the posterior segment of the eye include defects in the retinal nerve fiber layer (RNFL), vascular abnormalities, and visual disturbances, such as reduced contrast sensitivity and abnormal dark adaptation. However, the complete background of ophthalmic manifestations in the course of CF has yet to be discovered. This review summarizes the current knowledge regarding ocular changes in cystic fibrosis.

Body Composition, Anthropometric Indices and Hydration Status of Obstructive Sleep Apnea Patients: Can Cachexia Coexist with Obesity?

The aim of this study was to elucidate body composition, anthropometric indices, and hydration status in obstructive sleep apnea (OSA) patients, taking into account different disease stages, gender, and the possibility of the presence of cachexia. There were 98 OSA patients and 23 control subjects enrolled into the study. All study participants underwent polysomnography examination. Body mass index (BMI), fat mass index (FMI), fat free mass, muscle mass, body cell mass, total body water, and extracellular and intracellular water were evaluated. The neck, abdominal, and waist circumference was measured. We found that overweight and obesity were present in 96% of patients. Cachexia was present in one OSA individual with comorbidities. Apnea-hypopnea index correlated with the neck and waist circumference, and with BMI in OSA patients. All muscle indices and water contents above outlined were significantly higher in severe OSA compared with control subjects. BMI, FMI, neck circumference, and extracellular water were greater in a subset of severe OSA compared with a moderate OSA stage. The female OSA patients had a higher FMI than that present in males at a comparable BMI. We conclude that the most body composition indices differed significantly between severe OSA patients and control subjects. A higher FMI in females at a comparable BMI could be due to a discordance between BMI and FMI. Cachexia occurs rarely in OSA and seems to coexist with comorbidities.

Problems of patients with cystic fibrosis during transition to adulthood.

The proper care of cystic fibrosis patients extends over their lifetime. More than half of the children with the disease die before adulthood. An important element in the patient's care is a time of transition from a paediatric to the care of an internist and the patient's acceptance of this necessity. Transition from paediatric care to an internist should be adequately prepared. It is not only a question of transfer of medical records, but also careful preparation of patients for such transition. The patients expect not only continuity of care but also the introduction to the management with the disease. The creation of a base for specialist hospital treatment for exacerbation of the disease at the adulthood is an important element in the care of these patients. The problem has been solved in the children group, but is still waiting for solution in adults with cystic fibrosis. It has been proven that care in the centres carried out by a specialized team ensures longer life and better quality of life of these patients. The paper is an overview of these two important elements of care of adults with cystic fibrosis.

[Is there a relationship between pregnancy induced hypertension and obstructive sleep apnea? Case report]. / Czy istnieje zwiazek miedzy nadcisnieniem indukowanym ciaza a obturacyjnym bezdechem sennym? Opis przypadku.

The paper examines the case of a pregnant woman in a twin pregnancy complicated by pre-eclampsia and sleep disordered breathing. The patient was admitted to hospital with high blood pressure, proteinuria and increasing oedema. Laboratory tests revealed proteinuria and reduced total protein concentration in serum. The patient was diagnosed with mild pre-eclampsia. Due to the observed severe daytime sleepiness of the patient, loud snoring and pauses in breathing during sleep, polysomnography was performed. The test revealed a number of episodes of obstructive apnea and hypopnea. The AHI (apnea-hypopnea index) value was 82.1. A number of episodes of desaturation were observed. The lowest saturation had a value of 82%. When the patient was diagnosed with severe obstructive sleep apnea, treatment with CPAP (continuous positive airway pressure) was introduced. The use of CPAP allowed a reduction of the AHI to 1.2, and the blood pressure value normalised. The patient used CPAP between the 33rd and 35th weeks of gestation. At the 35th week of gestation, caesarean section was performed due to life-threatening symptoms of the first foetus in the CTG-recording. Two male infants were delivered in good condition. During the postpartum period, the patient discontinued the use of CPAP. Following this, an increase in AHI to 45.3 and an increase in blood pressure to a maximum of 180/100mmHg were observed. The patient was discharged from hospital in good general condition on the 7th day postpartum with recommendation for further diagnostic and therapy. The conclusion that may be drawn from the case is that sleep disorders adversely affect the health of the mother and the foetus. Also, the mood and quality of life of the pregnant woman deteriorate. It has been proven that there are many relationships between sleep and health. Sleep disorders and disorders of breathing during sleep contribute to the development of various diseases or they degrade the overall phenomena. A disease, on the other hand, additionally negatively affects the quality of sleep. Therefore, it is essential to treat not only the primary disease, but also the accompanying sleep disorders.

Gut microbiota in adults with cystic fibrosis: Implications for the severity of the CFTR gene mutation and nutritional status.

BACKGROUND: Microbial dysbiosis has been linked to cystic fibrosis (CF); however, the composition of gut microbiota in adult CF patients in relation to severity of CF transmembrane conductance regulator (CFTR) gene mutation and nutritional status have not yet been explored. Study aimed to assess the gut microbiota composition in adults with CF, and its relationship with the severity of CFTR mutations, and BMI. METHODS: Gut microbiota of 41 adults with CF, and 26 non-CF controls were compared using whole 16S rRNA gene sequencing. Differences in the microbial community between groups of patients classified according to the severity of CFTR mutations, and BMI were assessed. The alpha diversity, beta diversity, and taxa abundance were identified to reflect gut microbiota composition. RESULTS: Results showed a significant decrease in alpha diversity of bacterial communities in CF compared to non-CF group, but no significant difference between the CF groups distinguished by the severity of CFTR mutations. However, more severe mutations were associated with the higher relative abundance of Bacteroides and Streptococcus and the lower relative abundance of Faecalibacterium and Blautia. Undernourished CF patients showed significantly lower alpha diversity compared to non-CF group and CF patients with BMI within the norm. Significant differences in the structure of the gut microbiota between CF and non-CF groups, as well as between BMI groups were also found. CONCLUSIONS: Our research indicates that CF is associated with alterations in gut microbiota in adults. Additionally, in adult CF patients, the composition of the gut microbiota is also related to BMI.

Blood antioxidant status, dysglycemia and obstructive sleep apnea.

Obstructive sleep apnea (OSA) patients present increased cardiovascular morbidity and mortality. Oxidative stress is involved in OSA and cardiovascular pathology. The aim of the study was to assess oxidative stress markers in the blood of OSA males during oral glucose tolerance test (OGTT). The study involved OSA-suspected obese males (BMI ≥ 25 kg/m(2)) aged 35-64, with no acute or chronic disorders, appointed for polysomnography to diagnose OSA (AHI ≥ 5). The results of OGTT allowed to select prediabetic (Pre) subjects and normal glucose tolerance (N), excluding newly diagnosed diabetes. Blood was collected at 0 min (fasting) and 120 min of the test. Plasma glucose, total antioxidant status (TAS), thiobarbituric acid-reacting substances (TBARS), and activity of superoxide dismutase-1 (SOD) in erythrocytes, were determined at the two time points and the difference (D) between the 120 and 0 min time points was calculated for either oxidative stress variable (D-TAS, D-SOD and D-TBARS). Fasting serum insulin and lipids also were measured fasting. There were four groups of subjects, each consisting of 22 individuals N-OSA-neg(ative), N-OSA-pos(itive), Pre-OSA-neg and Pre-OSA-pos. The N-OSA-pos and Pre-OSA-pos subjects demonstrated decreased SOD-0 compared with OSA-negative groups. In N-OSA-neg and N-OSA-pos groups, the positive differences D-SOD and D-TAS were observed, while Pre-OSA subjects presented negative differences. In conclusion, prediabetic OSA patients may consume blood antioxidant factors to counter the effects of oxidative stress, more than individuals with normal glucose tolerance.

Prevalence of oral complications in the course of severe SARS-CoV-2 infection under mechanical non-invasive ventilation.

BACKGROUND: The management of oral health during severe symptoms of Covid-19 is still a challenge, especially in intensive care units under invasive/noninvasive ventilation in hospital. Understanding the cause-and-effect relationships may allow for individual adjustment of oral care recommendations during Covid-19 disease. The study's objective was to assess Covid-19 patients' oral health status under hospital treatment due to pulmonary adverse Covid-19 outcomes. MATERIAL AND METHODS: Covid-19 patients (mean age 74.4 ± 15.4; n = 120, male n = 50/female n = 70) were admitted to hospital in the acute phase of Covid-19 between January and March 2022 who required oxygen therapy due to pneumonia, rapid respiratory failure, low saturation. Blood and radiological tests were taken according to National Health Fund guidelines. The condition of teeth (Decayed, Missing, Filled teeth as DMFT index), dental hygiene (Plaque Control Record as PCR index), periodontal status (probing depth PD, clinical attachment CAL, bleeding on probing BOP) and oral mucosa (BRUSHED and Beck scores) were examined. RESULTS: Charateristics of the teeth (dental caries 35.2%, DMFT Median 22), plaque retention (83.4%), advanced periodontitis (48.3%), xerostomia (74.2%), oral mucosa inflammation (80.8%), angular cheilitis (53.3%), hemorrhagic (21.7%) showed a high incidence of harmful oral conditions. BRUSHED model and Beck score indicated moderate oral dysfunction and need for oral care every 8 h. Spearman's analysis revealed a significant positive correlation between pneumonia and neutrophile, interleukin-6 IL-6, C-reactive protein CRP (p = 0.01, p < 0.001, p < 0.001), negative to lymphocyte count (p < 0.001). Multiple and logistic regressions selected the following risk predictors for pneumonia as IL-6, CRP, obesity and for severe COVID-19 symptoms D-dimer level and a lack of targeted vaccination (p < 0.001). Among oral predictors, the PCR index and Beck score were significant for both outcomes (respectively p < 0.001, p < 0.012). Patients who received oxygen therapy with face masks had more often angular heilitis and debris (p = 0.025, p = 0.035). CONCLUSIONS: COVID-19 hospitalised patients with severe symptoms crossing with poor oral health-related conditions. This may exacerbate a response for COVID infection, and play a role in cytokine storm. For Covid-19 management, to inhibit extraoral/intraoral complications, it is recommended to adjust oral hygiene procedures, including antibacterial, protective, moisturising agents after individual oral health assessment.

The use of non-invasive respiratory assistance to facilitate bronchofiberoscopy performance in patients with hypoxemic (type one) respiratory failure - Study protocol.

PURPOSE: Bronchofiberoscopy (FOB) is a procedure routinely performed for: lung cancer, obstruction, interstitial diseases, foreign bodies' removal, airway clearance, and hemoptysis. It causes acute airway narrowing leading to respiratory and cardiovascular stress. Due to increasing number of ill patients with respiratory failure (RF), conventional oxygen therapy (COT) is frequently insufficient to assure accurate oxygenation and prevent RF in patients requiring FOB. In this clinical scenario, patients may be intubated and supported with invasive mechanical ventilation (IMV) with the specific aim of allowing a safe FOB. However, this invasive strategy is associated with an increased risk of IMV-associated complications. MATERIALS AND METHODS: Our study is a planned prospective multicenter three-arm randomized controlled trial (RCT). The target number of 300 patients was calculated based on the intubation risk in RF patients, which is 0.2-2%. The patients will be assigned to each arm based on Horowitz index. In each arm, the patients will be randomly assigned to one out of two dedicated respiratory support methods in each group i.e. COT/high flow nasal cannula (HFNC), HFNC/non-invasive ventilation (NIV) and NIV/IMV. In the manuscript the current state of art in the area of respiratory support is discussed. We have underlined knowledge gaps in medical evidence which we are planning to reveal with our results. RESULTS: The results of our study are clinically crucial, because they address current gaps concerning COT/HFNC/NIV/IMV. CONCLUSION: The expected findings of this study would allow for careful selection of respiratory support method to safely perform FOB in patients with hypoxemic RF.

May noninvasive mechanical ventilation and/ or continuous positive airway pressure increase the bronchoalveolar lavage salvage in patients with pulmonary diseases? Randomized clinical trial - Study protocol.

PURPOSE: Bronchoalveolar lavage (BAL) procedure is a useful tool in the diagnosis of patients with interstitial lung disease (ILD) and is helpful in clinical research of chronic obstructive pulmonary disease (COPD) patients. Still little is known about predictors of poor BAL salvage. The trial aims to find the most efficient way to improve BAL recovery. MATERIAL AND METHODS: Our study is a prospective, multicenter, international, two-arm randomized controlled trial. We aim to obtain BAL samples from a total number of 300 patients: 150 with ILD and 150 with COPD to achieve a statistical power of 80 â€‹%. Patients with initial BAL salvage <60 â€‹% will be randomized into the non-invasive ventilation (NIV) or continuous positive airway pressure (CPAP) arm. The NIV and CPAP will be set according to the study protocol. The influence on BAL salvage will be assessed in terms of BAL volume and content. Multivariable analysis of the additional test results to determine predictors for low BAL recovery will be conducted. In a study subgroup of approximately 20 patients per specific disease, a metabolomic assessment of exhaled air condensate will be performed. All procedures will be assessed in terms of the patient's safety. The trial was registered on clinicaltrials.gov (ID# NCT05631132). Interested experienced centers are invited to join the research group by writing to the corresponding author. CONCLUSION: The results of our prospective study will address the currently unsolved problem of how to increase BAL salvage in patients with pulmonary diseases without increasing the risk of respiratory failure exacerbation.

Efficacy of oral amantadine among patients hospitalised with COVID-19: A randomised, double-blind, placebo-controlled, multicentre study.

BACKGROUND: Amantadine has been proposed as a treatment for COVID-19 because it shows anti-SARS-CoV-2 activity in vitro. However, to date, no controlled study has assessed the safety and efficacy of amantadine in COVID-19. RESEARCH QUESTION: Whether amantadine is effective and safe among patients with different COVID-19 severity classifications. STUDY DESIGN: and Methods: This was multi-centre, randomised, placebo-controlled study.Patients with oxygen saturation ≤94% and no need for high-flow oxygen or ventilatory support were randomly allocated to receive oral amantadine or placebo (1:1) for 10 days in addition to standard care. The primary endpoint was time to recovery assessed over 28 days since randomisation, defined as discharge from hospital or no need for supplemental oxygen. RESULTS: The study was terminated early due to a lack of efficacy after an interim analysis. Final data from 95 patients who received amantadine (mean age, 60.2 years; 65% male; 66% with comorbidities) and 91 patients who received placebo (mean age, 55.8 years; 60% male; 68% with comorbidities) were obtained. The median (95% CI) time to recovery was 10 days both in the amantadine (9-11) and placebo arms (8-11; subhazard ratio = 0.94 [95%CI 0.7-1.3]). The percentage of deaths and percentage of patients who required intensive care at 14 and 28 days did not significantly differ between the amantadine and placebo groups. INTERPRETATION: Adding amantadine to standard care in patients hospitalised with COVID-19 did not increase the likelihood of recovery. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov; No.: NCT04952519; www. CLINICALTRIALS: gov.

[Embolization of the bronchial artery as a treatment of reccurent hemoptysis in patient with cystic fibrosis]. / Embolizacja tetnicy oskrzelowej w leczeniu nawracajacego krwioplucia u pacjenta z mukowiscydoza.

Hemoptisis is one of the possible complication in patients with cystic fibrosis, which has great influence on patients condidtion and their quality of life. There are few methods available for treatment of hemoptisis. Authors have reported case of 30-year-old patient with cystic fibrosis and hemoptysis, who was successful treated with right bronchial artery embolization.

Oral Care Recommendation for Cystic Fibrosis Patients-Recommendation for Dentists.

Cystic fibrosis (CF) is a genetic disease that is caused by a defect in the gene coding for the transmembrane cystic fibrosis transmembrane conductance regulator (CFTR). Research papers published so far point out that despite the numerous dental treatment needs of CF patients, there are no oral care guidelines for this group of patients. The aim of the article is to propose standards of dental prophylactic and therapeutic procedures for CF patients in different age groups. Regardless of the CF patient's age, dental check-ups should be scheduled at least every 6 months. However, taking into account the actual condition of the individual CF patients, therapeutic visits may be scheduled for earlier dates, to provide well-fitting treatment, considering the level of risk of oral diseases. The described management standards may be helpful and may improve the quality of dental care provided to CF patients.

Clinical significance of basic laboratory parameters in predicting the use of various methods of oxygen supplementation in COVID-19.

INTRODUCTION: The severe acute respiratory syndrome coronavirus type 2 (SARS-CoV-2) infection resulted in significant worldwide morbidity and mortality. The aim of our study was to evaluate the results of laboratory tests performed on patients on admission to the hospital between groups of patients requiring and not requiring oxygen supplementation, and to find predictive laboratory indicators for the use of high-flow nasal oxygen therapy (HFNOT)/continuous positive airway pressure (CPAP)/bilevel positive airway pressure (BPAP). MATERIALS AND METHODS: We retrospectively analysed the data of consecutive patients hospitalised in the Pulmonology Department of the Temporary COVID Hospital in Poznan from February to May 2021. On admission to the department, the patients had a panel of laboratory blood tests. RESULTS: The study group consisted of 207 patients with a mean age of 59.2 ± 15.0 years of whom 179 (72%) were male. During hospitalisation, oxygen supplementation was required by 87% of patients. Patients requiring oxygen supplementation and/or the use of HFNOT/CPAP/BPAP had lower lymphocyte counts and higher levels of urea, C-reactive protein, D-dimer, troponin, glucose, lactate dehydrogenase (LDH) as well as higher white blood cell and neutrophil counts, The parameter that obtained the highest area under curve value in the receiver operator curve analysis for the necessary use of HFNOT/CPAP/BPAP or CPAP/BPAP was LDH activity. CONCLUSIONS: Among the basic parameters assessed on admission to the temporary hospital, LDH activity turned out to be the most useful for assessing the need for CPAP/BPAP active oxygen therapy. Other parameters that may be helpful for predicting the need for HFNOT/CPAP/BPAP are serum levels of urea, D-dimer and troponin.

Effect of Face Masks on Blood Saturation, Heart Rate, and Well-Being Indicators in Health Care Providers Working in Specialized COVID-19 Center.

This study aims to investigate whether wearing face masks (filtering facepieces, FFP class 2) with personal protective equipment (FPP2/PPE), while working a 12-h shift in a COVID-19 referral center, affects the blood saturation, heart rate (HR), and well-being of health care providers (HCPs). The study included a group of 37 HCPs. To perform continuous recordings of the SpO2 and heart rate (HR) in real time, we used a Nellcor PM10N (Covidien, Mansfield, MA, USA) portable monitoring system. SpO2, HR, and HCP well-being scales were measured during two 3-h shifts, while HCPs worked during a 12-h period. Additionally, each subject completed a questionnaire concerning their well-being. The difference in the SpO2 level between the 1st and 2nd working shifts while wearing an FFP2/PPE was small, with a median decrease in SpO2 of -1%. The scales of the well-being indicators increased within the shift. They were mainly fatigue and thirst with median scores of 2 out of 6 (range 0-4). We assume that during a 12-h period, a work scheme that consists of a 3-h shift in FFP2/PPE and a 3-h rest period (working without FPP2/PPE) is a reliable and safe solution for HCPs working in specialized COVID-19 referral hospitals.

Spiritual Distress and Spiritual Needs of Chronically Ill Patients in Poland: A Cross-Sectional Study.

INTRODUCTION: Spiritual care is needed in a clinical setting to improve the patients' quality of life. Deep connection with another person and delight with the beauty of nature or art and (in some cases) with God are all transcendental experiences. They may enable patients to ascribe meaning to their life with a chronic illness, find hope and well-being despite burdening symptoms. The opposite situation: lack of inner peace, inability to accept what is happening, feeling disconnected from others is called spiritual distress. OBJECTIVES: The aim of this research is to assess spiritual distress and spiritual needs of a group of Polish chronically ill patients and find associations with independent variables in order to provide data for recommendations on spiritual care in Poland. PATIENTS AND METHODS: 204 patients treated at the University Hospital and the Cystic Fibrosis Clinic in Poznan were surveyed in 2017 and 2018 with an original questionnaire. RESULTS: Over half of the patients felt that their illness was life-threatening. A little more than half reported that faith was a resource to cope with suffering. Almost all patients showed signs of spiritual distress, and more than half expressed spiritual needs. The intensity of distress correlated only with the severity of the disease. The most important predictor of having spiritual needs was recognizing faith as a resource. CONCLUSIONS: Spiritual needs are associated with personal beliefs; however, spirituality spans beyond the religious context since spiritual distress is unrelated to the level of religious devotion. Therefore, any patient with a severe chronic disease needs basic spiritual care, which includes being treated with compassion.

Self-Reported Dietary Choices and Oral Health Care Needs during COVID-19 Quarantine: A Cross-Sectional Study.

The COVID-19 pandemic significantly impacted the healthcare system, including dentistry. However, it is not entirely clear whether affected patients' willingness for regular dental visits and preventive behaviors with regards oral hygiene and diet. This is essential to understanding the potential effects of the COVID-19 pandemic on the acceleration of dental issues in the future. It was aimed at checking the level of dental visit avoidance, self-reported oral health needs, and dietary changes. This cross-sectional questionnaire study conducted in Poland (n = 2574; mean age 44.4 ± 15.6; female 56.3%) assessed nutritional habits and dental care changes during the COVID-19 pandemic. As demonstrated, nearly half of the responders (47.1%) avoided regular dental visits, while only 0.5% used online consultations. Fears related to potential cross-contamination in dental offices dropped from 25% to 11.4% and were associated with increased BMI and age (p < 0.05). Sweet snacking/drinking confirmed 19.1%/33.2% subjects. Self-reported oral health care needs (tooth stain, calculus, gingivitis, loss of fillings) were related to frequent snacking and poor oral hygiene (p < 0.05). The study highlights that pandemic periods are covered by eating and drinking changes combined with inadequate hygiene and dental care impose health complaints in the oral cavity. This can magnify both nutritional and interrelated oral health issues, highlighting the need to implement preventive and mitigation measures.

Implementation of the Patient Counselling Service at the Cancer Hospital in Radom, Poland.

Background: Non-adherence occurs in various groups of patients, including those with chronic diseases. One strategy to increase adherence among oncological patients is to individualise treatment and expand pharmaceutical care. Pharmaceutical labels that remind patients how they should take their medications are of great importance in this respect. Objective: The main objective of this study was to evaluate medication adherence in oncological patients, and to gather their opinions on the individual medication labelling system as an element of effective treatment. Methods: The study was conducted in 2021 among 82 patients of the oncological department of the Centre of Oncology in Radom. The research tool was a questionnaire consisting of personal data and two parts relating to the patient's disease and the medication labelling system. Results: Nearly half of the respondents reported that they forget to take medications and how they should take them. These problems increased with the age of the patient and the number of administered medications. Of the respondents, 89% stated that the labels with dosing information are helpful. Over 67% agreed that these labels should be affixed to all medications. Nearly 90% of the respondents believed the labels should be available in all pharmacies. Conclusions: Non-adherence is a common phenomenon among oncological patients. Pharmacists providing a labelling service for medicinal products can play a significant role in reducing this phenomenon.

Nebulisation therapy in patients with cystic fibrosis - consensus of the Polish Cystic Fibrosis Society.

INTRODUCTION: Nebulisation therapy plays a key role in the treatment of cystic fibrosis (CF). Its effectiveness depends on obtaining a high concentration of drugs in the respiratory tract. Particle deposition is determined by many factors resulting, inter alia, from the essence of the lung disease (mucus, structural changes such as bronchiectasis, fibrous changes, cirrhosis) and the quality of the aerosol and breathing techniques during the procedure. AIM OF THE GUIDELINES: A large variety of available drugs that can be used in the form of aerosols (bronchodilators, mucolytics, antibiotics), a wide range of devices for their delivery, and a different approach to the practical aspect related to the use of inhalation, makes it necessary to systematize knowledge in order to optimize nebulisation therapy. The paper presents an overview of inhaled drugs used in cystic fibrosis and their administration devices. RESULTS: The principles of inhalation antibiotic therapy, which constitute the basis for the treatment of primary and chronic respiratory tract infections of Pseudomonas aeruginosa etiology, are discussed in detail. A very important issue was raised related to the proper selection of devices and their proper operation. In the context of the key role of nebulisation therapy in cystic fibrosis, a huge problem is the limited availability of inhaled antibiotics in Poland. CONCLUSIONS: The possibility of choosing an antibiotic and using alternating therapy increases the effectiveness of inhalation treatment, which results in slowing down the progress of bronchopulmonary disease and extending the life of patients.

Dental Anomalies in Rare, Genetic Ciliopathic Disorder-A Case Report and Review of Literature.

BACKGROUND: Primary ciliary dyskinesia (PCD) is a rare, ciliopathic disorder. In many ciliopathies, dental anomalies are observed alongside other symptoms of the disease. To date, there are no published reports concerning the dental developmental problems that are associated with ciliary defects in PCD patients. METHODS: Patients suffering from PCD underwent dental clinical examination, which included the assessment of developmental disorders regarding the number and morphological structure of the teeth (size and shape) as well as developmental disorders of mineralised dental tissues. Then, three-dimensional radiographic examination was performed utilising Cone Beam Computed Tomography (CBCT). RESULTS: Four PCD patients, aged 31-54, agreed to enter the study. Dental examinations showed the presence of dental developmental disorders in three of them. Additionally, CBCT showed abnormalities in those patients. CONCLUSIONS: 1. The dental phenotype in PCD patients seems to be heterogeneous. Tooth developmental disorders resulting from abnormal odontogenesis may be a symptom of PCD that is concomitant with other developmental abnormalities resulting from malfunctioning primary cilia. 2. Patients with ciliopathies are likely to develop dental developmental defects. Therefore, beginning in early childhood, they should be included in a targeted specialised dental programme to enable early diagnosis and to ensure dedicated preventive and therapeutic measures.