Ocular Changes in Cystic Fibrosis: A Review Liberski, Slawomir; Confalonieri, Filippo; Cofta, Szczepan ...
International journal of molecular sciences,
06/2024, Letnik:
25, Številka:
12
Journal Article
Recenzirano
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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 (Clsup.−) 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.
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 (
= 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 (
< 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 (
< 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.
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.
Background/Objectives: Cystic fibrosis is a genetically determined disease that significantly influences and shortens life. Treatment with CFTR modulators (CFTR-T) is a new hope for patients. It can ...change the predictive values of a poor prognosis (e.g., exacerbation rate and FEV1 value). The aim of the study was to analyse exacerbation incidence and spirometry data before and after one year (+/− 2 weeks) of CFTR-T in 85 CF patients at the CF Centre in Poznań. To our knowledge, this is the first analysis of CFTR-T efficiency in the Central–Eastern Europe population. Methods: We retrospectively analysed the spirometry and exacerbation data of 85 CF adult patients (both men and women), who in the middle of 2022 began treatment with CFTR modulators. Results: The one-year ratio of hospitalisation caused by severe exacerbations lowered from 1.25 to 0.21 per patient per year. We also saw a 66% decline in ambulatory exacerbations. The median FEV1% increased by 9.60% in absolute values and by 460 mL. Even in the group with very severe obstruction (FEV1 < 35%), there was an increase in median FEV1% of 5.9 in absolute values. We also proved the increase in FVC% (median 17.10% in absolute value and 600 mL) in the study group. Conclusions: After one year of treatment, an impressive improvement was observed in two important predictive values of poor prognosis: exacerbation rate and FEV1 values. Further observation is needed to determine how long the improvement will be present and its influence on quality of life and life expectancy.
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 + or - 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. Keywords: Covid-19, Hospital treatment, Oral health, Pneumonia
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.
The obstructive sleep apnea (OSA) is highly associated with various significant cardiovascular outcomes such as resistant hypertension (RAH). Despite this, as of now the relationship between high ...night-time blood pressure (BP) and left ventricular hypertrophy (LVH) in patients with OSA and RAH is not fully understood. The aim of this study was to assess the influence of the addition of eplerenone to a standard antihypertensive therapy on parameters of 24-h ambulatory blood pressure measurement (ABPM) as well as on the results of echocardiography and polysomnography in patients with OSA and RAH. The patients were randomly assigned to one of the two study groups: the treatment group, receiving 50 mg/d eplerenone orally for 6 months (n = 51) and the control group, remaining on their standard antihypertensive therapy (n = 51). After that period, a significant reduction in the night-time BP parameters in the treatment group including an increased night blood pressure fall from 4.6 to 8.9% was noted. Additionally, the number of non-dipper patients was reduced by 45.1%. The treatment group also revealed a decrease in left ventricular hypertrophy and in the apnea–hypopnea index (AHI) with a positive correlation being observed between these two parameters. This study is the first to report the improvement of the circadian BP profile and the improvement of the left ventricle geometry in patients with OSA and RAH following the addition of selective mineralocorticoid receptor antagonists to antihypertensive therapy.
The aim of this study was to assess the periodontal status of cystic fibrosis (CF) adult patients and to evaluate whether there is a correlation between the bacterial population of the subgingival ...biofilm and the health status of the periodontal tissues in this group of adults. The study involved 22 cystic fibrosis adult patients. The periodontal condition was assessed using Plaque Index (PLI), Gingival Index (GI), and Probing Pocket Depth (PPD). The gingival sulcus samples were analyzed by the Real-Time PCR assay (RT-PCR). Majority of patients showed moderate or severe bacterial dental plaque accumulation, but none of them had clinical symptoms of periodontal diseases. RT-PCR showed the presence of periopathogens in 50% of patients. Red complex microorganisms were detected in 9.09%, orange complex in 27.27%, and green complex in 31.82% of the samples analyzed. In cystic fibrosis patients colonized by periopathogens, the periodontal markers were significantly higher in comparison to not colonized by periopathogens patients. Despite the widespread presence of bacterial dental deposits in the cystic fibrosis adult patients examined, none of them has clinical symptoms of periodontal disease; however, the presence of periodontal pathogens in subgingival biofilm may represent a possible risk factor of this disease in the future. An unsatisfactory level of oral hygiene in any patient with cystic fibrosis indicates a need to focus on standards of dental care for such patients.
The aim of this study was to assess the periodontal status of cystic fibrosis (CF) adult patients and to evaluate whether there is a correlation between the bacterial population of the subgingival biofilm and the health status of the periodontal tissues in this group of adults. The study involved 22 cystic fibrosis adult patients. The periodontal condition was assessed using Plaque Index (PLI), Gingival Index (GI), and Probing Pocket Depth (PPD). The gingival sulcus samples were analyzed by the Real-Time PCR assay (RT-PCR). Majority of patients showed moderate or severe bacterial dental plaque accumulation, but none of them had clinical symptoms of periodontal diseases. RT-PCR showed the presence of periopathogens in 50% of patients. Red complex microorganisms were detected in 9.09%, orange complex in 27.27%, and green complex in 31.82% of the samples analyzed. In cystic fibrosis patients colonized by periopathogens, the periodontal markers were significantly higher in comparison to not colonized by periopathogens patients. Despite the widespread presence of bacterial dental deposits in the cystic fibrosis adult patients examined, none of them has clinical symptoms of periodontal disease; however, the presence of periodontal pathogens in subgingival biofilm may represent a possible risk factor of this disease in the future. An unsatisfactory level of oral hygiene in any patient with cystic fibrosis indicates a need to focus on standards of dental care for such patients.
Several studies have shown that plasma copper concentrations are increased in various carcinomas. Zinc acts as a cellular growth protector, including growth of neoplastic cells, and its deficiency ...was demonstrated to be involved in several stages of malignant transformation. However, the usefulness of the serum zinc and copper determinations in cancer prevention, detection, monitoring treatment, and prognosis requires further investigations. The aim of the present study was to compare the serum copper and zinc levels in patients with cancer of the lung (PC), breast (BC), gastrointestinal tract (GIC), and gynecological (GYNC) malignancy with progress of the disease. The results of the study have shown a significant increase in the mean total serum Cu levels and the serum Cu/Zn ratio in all patient groups with cancer compared to a control group. Increased mean serum concentrations and Cu/Zn ratios were found in the whole group (ALLC), and for the GIC and GYNC groups with local as well as metastasized (Meta) disease in comparison with the control group. The mean serum concentrations of Zn were decreased only in metastasized ALLC and GYNC groups.
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