The prevalence of developmental defects of enamel in people with cystic fibrosis: a systematic review.

BACKGROUND: Oral health impacts systemic health, individual well-being, and quality of life. It is important to identify conditions that may exacerbate oral disease to aid public health and policy development and promote targeted patient treatment strategies. Developmental defects can increase an individual's risk of dental caries, hypersensitivity, premature tooth wear, erosion, and poor aesthetics. As part of an ongoing study assessing oral health in adults with cystic fibrosis at Cork University Dental School and Hospital, a systematic review of available literature was conducted to assess the prevalence of enamel defects in people with cystic fibrosis. AIMS: To critically evaluate the literature to determine if the prevalence of developmental defects of enamel is higher in people with cystic fibrosis (PwCF). METHODS: Data Sources: Three online databases were searched Embase, Scopus, and Web of Science Core Collection. Studies that examined an association between cystic fibrosis and developmental defects of enamel were included in this systematic review. RESULTS: The initial search identified 116 publications from the following databases Embase, Web of Science Core Collection, and Scopus. Eleven studies were included for qualitative analysis. Nine studies concluded that PwCF had a higher prevalence of enamel defects than control people and one study found no difference in cystic fibrosis (CF) status. All studies had a risk of bias that may influence study results and their interpretation. CONCLUSIONS: The results of the systematic review show a consistent pattern that PwCF have a higher prevalence of DDE than people without CF. Genetic dysfunction, chronic systemic infections, and long-term antibiotic use are possible aetiological causes. This review highlights the need for future studies to investigate if DDEs are caused by the underlying CFTR mutation or as a consequence of disease manifestations and/or management.

The prevalence of developmental defects of enamel in a cohort of adults with cystic fibrosis - A cross sectional study.

OBJECTIVES: Cystic Fibrosis is an autosomal recessive condition. It is a multisystem disease treated with a broad range of pharmacological therapies, diet and nutrition, and physiotherapy. Previous studies suggest that people with cystic fibrosis have a higher prevalence of developmental defects of enamel which may place this population at a greater risk of developing oral diseases such as caries. The aim of this study was to assess a cohort of people with cystic fibrosis (PwCF) for the presence of developmental defects of enamel and compare the results with a control group of people without cystic fibrosis. METHODS: A cross sectional study involving 92 participants with cystic fibrosis and 92 controls was conducted in Cork University Dental School & Hospital. All participants completed a detailed questionnaire prior to undergoing a full clinical examination. The Developmental Defect of Enamel Index was used as a measurement index. All data was statistically analysed with the help of statisticians from Cystic Fibrosis Registry of Ireland. RESULTS: 64 % (n = 59) of PwCF had enamel defects compared to just 30 % (n = 28) of people without cystic fibrosis. The median number of teeth affected by enamel defects in the study group was 1.5, compared to 0 in the control group. CONCLUSION: In this study the cohort of PwCF had more enamel defects than people without CF. Further research is required to investigate the aetiology of these findings. CLINICAL SIGNIFICANCE: Clinicians should be vigilant after teeth have erupted in PwCF as they may have an increased susceptibility to developmental defects of enamel.

Periodontal disease prevalence and oral hygiene status of adults with cystic fibrosis: A case-control study.

AIM: To investigate the prevalence of gingivitis and periodontitis, and the oral hygiene status of adults with cystic fibrosis (CF) in the Republic of Ireland. MATERIALS AND METHODS: A case-control study in the form of a clinical examination of 92 adults with a diagnosis of CF was carried out in the adult CF unit in Cork University Hospital. A 40-item questionnaire was used to capture socio-demographic variables and medical and dental information. Two calibrated examiners carried out a periodontal assessment on participants, using the WHO-recommended CPI-modified index, and oral hygiene status was measured using the Greene-Vermillion index. The results were compared with a population-based control group of similar socio-demographic profile. RESULTS: Oral hygiene levels (plaque and calculus) were significantly worse in people with CF, with a median plaque index of 0.83 (interquartile range [IQR] 0.333-1.542) in the CF group compared with 0.5 (IQR 0.167-0.667) in the non-CF group. Calculus index in the CF group was 0.33 (IQR 0.17-0.83) compared with 0.33 (IQR 0.125-0.33) in the non-CF group. However, periodontal disease levels were significantly lower in the CF group. Gingivitis (bleeding on probing ≥ 10% sites) was seen in 67.4% of the CF group, compared with 83.7% of the non-CF group, OR 0.365 (95% confidence interval [CI] 0.181-0.736), relative risk (RR) 0.779 (95% CI 0.655-0.928). Mild periodontitis (periodontal probing depth [PPD] < 5 mm) was seen in 15.2% of the CF group, compared with 31.5% of the non-CF group, OR 0.390 (CI 0.190-0.800), RR 0.483 (95% CI 0.273-0.852). Severe periodontitis (PPD ≥ 6 mm) was seen in 0% of the CF group, compared with 9.8% of the non-CF group. There was a tendency, albeit non-significant, towards reduced periodontitis in PWCF who regularly took antibiotics, particularly azithromycin. CONCLUSIONS: In this study, adults with CF had poor oral hygiene practices, with high levels of plaque and calculus. Despite this finding, adults with CF had lower levels of clinical gingivitis and periodontitis than seen in a non-CF control group. Further study is required to examine the causes of this phenomenon.

Current practices and attitudes of dental practitioners regarding the provision of dental treatment for patients with solid organ transplants.

BACKGROUND: Solid organ transplant provides a lifeline for people with end stage organ failure. Each year the number of individuals in receipt of a solid organ transplant is increasing. Prevention of post-transplant sepsis and infection are critical for transplant success. The oral cavity contains more than 700 different species of bacteria and is a potential reservoir for disease causing pathogens. Prior to undergoing solid organ transplant, individuals must receive a certification of dental health from a dental practitioner. There are currently no guidelines or protocols for dental practitioners to follow when certifying a patient as dentally fit. This allows for a wide variation of the term 'dentally fit'. This survey was conducted as part of a larger study assessing the oral health of adults with cystic fibrosis ongoing in Cork University Dental School and Hospital. The aim of the study was to ascertain current practices and attitudes of dental practitioners regarding the provision of dental treatment pre and post solid organ transplantation. METHODS: An anonymous cross sectional survey of dental practitioners in Ireland was conducted. RESULTS: The data collected showed a wide variation in the provision of treatment for patient undergoing or in receipt of a solid organ transplant. CONCLUSION: It demonstrates a need for further research to be conducted to ascertain the full impact solid organ transplant has on oral health, so that guidelines can be developed to aid both dental and medical professionals in the treatment of this vulnerable cohort.

Oral care considerations for people with cystic fibrosis: a cross-sectional qualitative study.

OBJECTIVES: To investigate the attitudes of adults with Cystic Fibrosis (CF) towards dental attendance and any perceived barriers to treatment. METHODS: A cross sectional survey in the form of a structured, anonymous questionnaire was used to obtain information regarding adults with CF's feelings towards dentists and dental treatment. The final version of the questionnaire was based on a collaborative effort between researchers at Cork University Dental School and Hospital and Cystic Fibrosis (CF) patient advocates from CF Ireland. Participants were recruited via CF Ireland's mailing list and social media channels. The responses underwent descriptive statistical analysis and inductive thematic analysis. RESULTS: A total of 71 people (33 Male: 38 Female) over the age of 18 living with CF in the Republic of Ireland responded to the survey. 54.9% of respondents were unhappy with their teeth. 63.4% felt that CF had an impact on oral health. 33.8% were anxious about attending their dentist. Respondents believed that CF has impacted on their oral health due to the medications and dietary requirements involved, as well as tiredness and other side effects of CF. Reasons for being anxious about attending the dentist included cross infection concerns, issues with the dentist, with tolerating treatment, and with the teeth themselves. Respondents wanted dentists to be aware of the practicalities of dental treatment for people with CF, especially their discomfort with lying back. They also want the dentist to be aware of the impact that their medication, treatment and diet has on their oral health. CONCLUSIONS: Over one third of adults with CF reported anxiety about attending the dentist. Reasons for this included fear, embarrassment, cross infection concerns and problems with treatment, especially being in the supine position. Adults with CF want dentists to be aware of the impact that CF can have upon dental treatment and oral health care.

Self-reported dental attendance, oral hygiene habits, and dietary habits of adults with cystic fibrosis.

AIMS: To evaluate the dental attendance, oral hygiene habits, and dietary habits of adults with Cystic Fibrosis in the Republic of Ireland. METHODS AND RESULTS: A cross-sectional study was carried out using a structured anonymous questionnaire. A total of 71 adults with Cystic Fibrosis responded. While the majority of respondents (66.2%) saw a dentist in the preceding year, 15.5% had not attended a dentist for over 2 years. Smoking and alcohol consumption levels were low. 63.4% brushed twice or more daily, with 70.4% using a Fluoride containing toothpaste. 62% did not use any interdental cleaning aid. 5.6% changed their toothbrush at least once a month, but for 22.5% it was over 6 months. 70.4% used fluoride toothpaste. 38% snacked three or more times daily and 29.5% consumed fizzy drinks at least once daily. CONCLUSIONS: The alcohol and tobacco consumption in this study group was low. However, a large proportion frequently consumed sugar-rich foods, and they did not change their toothbrush, brush their teeth, or attend the dentist as regularly as is advised. More targeted advice may be necessary to improve the oral hygiene habits of adults with Cystic Fibrosis.

Proficiency Based Progression (PBP) training- the future model for dental operative skills training?: A systematic review and meta-analysis of existing literature.

OBJECTIVE: To evaluate the effectiveness of Proficiency-Based Progression (PBP) operative training using validated performance metrics, by comparing this to standard, conventional training methods. DATA: This systematic review was conducted in accordance with the guidelines of the Transparent Reporting of Systematic Reviews and Meta-Analyses (PRISMA). Study quality was assessed using the MERSQI tool and the Cochrane Risk of Bias tool. Results were pooled using biased corrected standardized mean difference and ratio-of-means (ROM). Summary effects were evaluated using a series of fixed and random effects models. The primary outcome was the number of procedural errors performed comparing PBP and non-PBP-based training pathways. In quantitative synthesis testing for procedural errors, a pooled meta-analysis on 87 trainees was conducted using random-effects models. In a ROM analysis, PBP was estimated to reduce the mean rate of errors by 62%, when compared to standard training (ROM 0.38, 95% CI: 0.25; 0.58; p < 0.001) Sources: The electronic databases of PubMed, Embase, Web of Science, MEDLINE and Cochrane library's CENTRAL were searched from inception to 8/11/2021. Filters activated were Randomized Controlled trials, clinical trial. STUDY SELECTION: 13 studies were included for review with 11 included in the quantitative synthesis from 174 potentially relevant publications identified by the search strategy. Main inclusion criteria were studies comparing standard surgical/operative training with proficiency-based simulation training using validated metrics based on expert performance. CONCLUSIONS: Our meta-analysis found that PBP training improved trainees' performances, by decreasing procedural errors. There is sufficient evidence to explore PBP training for use in dental skills training. PBP training was estimated to reduce the mean rate of operative errors by 62%, when compared to standard training. Given that there is a direct correlation between operative skill and patient outcomes, these data suggest that there is sufficient evidence to explore PBP training for use in dental skills training.

"Oral Nutritional Supplements: Sugar Content and Potential Dental Implications".

OBJECTIVES: The objectives of this article are to list the most commonly prescribed Oral Nutritional Supplements in the UK and Ireland and their sugar content; and to raise awareness among the dental profession regarding their uses and potential dental risks involved. BACKGROUND: Many older patients benefit from Oral Nutritional Supplements. Prescribers may not consider dental implications of these. Patients may not think to disclose these medications to their dentist. MATERIALS AND METHODS: A list of commonly prescribed Oral Nutritional Supplements in the UK and Ireland was compiled. Nutritional information was obtained from the manufacturers' website and arranged in order of decreasing sugar content. Potential dental implications are discussed and recommendations made for dental practitioners. RESULTS: Pre-formed Oral Nutritional Supplements can contain between 6.6 and 27.2 g of sugar per serving. Powdered Oral Nutritional Supplements, which are to be mixed with 200 ml whole milk, contain between 16.4 and 35.0 g sugar per serving. The "shot"-type Oral Nutritional Supplements contain less sugar, ranging from 0.0 to 4.0 g per serving. CONCLUSIONS: The sugar content of frequently prescribed Oral Nutritional Supplements can be high. While they are beneficial in assisting the patient to maintain a healthy BMI, they may increase the risk of dental caries. Dental professionals should enquire specifically about Oral Nutritional Supplements during history taking, particularly in groups who are likely to be prescribed such supplements. Consideration should also be given to increasing caries-preventive measures for patients who take these supplements.

Periodontal and oral health status of people with Cystic Fibrosis: a systematic review.

INTRODUCTION AND OBJECTIVES: People with Cystic Fibrosis (PWCF) may be presumed to be at lower risk of periodontal disease due to long term antibiotic use but this has not been comprehensively investigated. The oral hygiene and periodontal status of PWCF in comparison to the general population is not well established. The objective of this systematic review was to critically evaluate the literature on periodontal and oral hygiene status in PWCF to see if this group are at increased risk of periodontal disease (gingivitis or periodontitis). DATA SOURCES: 5 databases were searched: Scopus, MEDLINE, Embase, Cochrane Library and Web of Science. STUDY SELECTION: The search resulted in 614 publications from databases with one more publication identified by searching bibliographies. 13 studies were included in the qualitative analysis. CONCLUSIONS: The majority of studies showed better oral hygiene, with lower levels of gingivitis and plaque among people with Cystic Fibrosis (PWCF) than controls. Interestingly, despite this, many studies showed that PWCF had higher levels of dental calculus. Three studies found there was no difference in Oral Hygiene between PWCF and controls. One study found that PWCF aged between 6 and 9.5 years had increased levels of clinical gingivitis, and one study showed that PWCF with gingivitis had more bleeding on probing than people without CF. The vast majority of PWCF examined were children- only five studies included people over 18 years, and only one looked exclusively at adults. There is a need for further study into the periodontal health of PWCF- particularly those over the age of 18. CLINICAL SIGNIFICANCE: There are currently no guidelines referring to oral care in PWCF. Studies have suggested that the oral cavity acts as a reservoir of bacteria which may colonise the lungs. If PWCF are at increased risk of periodontal disease, they should attend for regular screenings to facilitate early detection.

Modified Inside-Out Technique for Continued Use of Chronically Occluded Upper Central Veins.

This report describes a 2-step, inside-out procedure for upper body central venous access in patients with chronic central venous occlusions. Blunt cephalad dissection through the mediastinum was achieved with a curved metal cannula and guide wire followed by percutaneous puncture of an open snare from a right supraclavicular approach and dilation of the tract for a tunneled central venous catheter insertion. Of 9 patients, all had a successful placement of a tunneled central venous catheter using this method. Although this 2-step, inside-out technique is effective for creating access in the presence of central venous occlusion, further clinical evaluation is warranted.

Practical applications of balanced steady-state free-precession (bSSFP) imaging in the abdomen and pelvis.

Balanced steady-state free-precession (bSSFP) is an important pulse sequence that may be underutilized in abdominal and pelvic magnetic resonance imaging (MRI). bSSFP offers several advantages for abdominal and pelvic MRI that include: bright blood effects, a relative insensitivity to the dephasing effects which occur in structures with linear movement, low specific absorption rate (SAR), high signal-to-noise ratio (SNR), high spatial resolution, and rapid acquisition times. Bright blood effects can be exploited to diagnose or confirm vascular pathologies when gadolinium-enhanced imaging cannot be performed, is indeterminate, or is degraded by artifact. The relative insensitivity to dephasing artifact in areas of linear movement is useful when imaging the biliary, urinary, and gastrointestinal tracts where dephasing artifacts may mimic filling defects such as calculi or polyps. Low SAR imaging is important in pediatric and pregnant patients and may be useful in patients with medical devices that restrict SAR levels. Rapid acquisition times and high SNR are extremely valuable assets in abdominal and pelvic MRI and bSSFP (which can be performed as static or cine acquisitions) and can be added to most existing abdominal and pelvic protocols when deemed suitable without significantly prolonging examination times. This article reviews the fundamentals of bSSFP imaging, presents vascular and nonvascular applications of bSSFP in abdominal and pelvic MRI, and discusses potential limitations (including imaging artifacts) of bSSFP. LEVEL OF EVIDENCE: 5 J. Magn. Reson. Imaging 2017;45:11-20.

Recurrent spontaneous pneumothoraces and bullous emphysema. A novel mutation causing Birt-Hogg-Dube syndrome.

Birt-Hogg-Dube syndrome (BHDS) is a rare form of classically cystic lung disease that may present with spontaneous pneumothorax. The associated skin manifestations (fibrofolliculomas) are not always present. This article describes a case of spontaneous pneumothorax secondary to bullous emphysema in an otherwise healthy gentleman caused by a novel mutation in the folliculin (FLCN) gene.

Multi-parametric (mp) MRI of prostatic ductal adenocarcinoma.

BACKGROUND: Prostatic ductal adenocarcinoma (DCa) is an aggressive variant of conventional adenocarcinoma (CCa) with mixed DCa and CCa tumors comprising up to 5% of all prostate cancers. DCa may be underestimated on T2-weighted (T2W) MRI. This study assessed the mp-MRI appearance of DCa as compared with CCa. METHODS: With research ethics board approval, we identified 38 patients who underwent mp-MRI (T2W, DWI, and DCE) and radical prostatectomy (RP) between 2012 and 2014. Eight DCa in 8 patients and 39 CCa tumor foci in 30 consecutive patients were identified. Tumor volume, apparent diffusion coefficient (ADC;10(-3) mm(2) /s), and time-signal intensity (SI) curves were calculated. Parametric data were compared using the Kruskal-Wallis test and univariate regression. Time-SI curves were compared using the chi-square test. RESULTS: Tumor volumes were: 1.62(±1.02) for DCa, 1.03(±0.54) for Gleason 9, 0.88(±0.93) for Gleason 7/8, and 0.26(±0.14) mL for Gleason 6. There was no difference in size between DCa and Gleason 9 (P = 0.22); however, DCa were larger than Gleason 7/8 (P = 0.03) and Gleason 6 (P = 0.003) tumors. ADC values were: 0.789(±0.22) for DCa, 1.01(±0.19) for Gleason 9, 0.992(±0.23) for Gleason 7/8 and 1.389(±0.41) 10(-3) mm(2) /s for Gleason 6 tumors. There was no difference in ADC between DCa and Gleason 9 (P = 0.14) or Gleason 7/8 (P = 0.055) tumors. There was a difference in ADC for DCa and Gleason ≥7 CCa compared to Gleason 6 tumors, (P < 0.001 and P = 0.012). All DCa demonstrated type III time-SI curves. Gleason ≥ 7 tumors demonstrated type II/III curves. Gleason 6 tumors demonstrated Type I/II time-SI curves. There was no difference in curve type between groups, (P = 0.18). CONCLUSION: Although DCa mimics Gleason score 3 + 3 = 6 tumor at T2W MRI; DCa resembles Gleason ≥7 CCa on mp-MRI.

Prostatic ductal adenocarcinoma: an aggressive tumour variant unrecognized on T2 weighted magnetic resonance imaging (MRI).

INTRODUCTION: Prostatic ductal adenocarcinoma (DCa) is an aggressive variant. The purpose of this study was to determine if T2 signal intensity (SI) differs from conventional adenocarcinoma (CCa). MATERIALS AND METHODS: A retrospective study of patients who underwent preoperative MRI and prostatectomy between 2009 and 2012 was performed. T2 SI ratios (SIR) for tumour (T) to obturator internus muscle (M) and normal peripheral zone (PZ) were compared. Two radiologists evaluated the central gland/PZ to detect tumours and compared diagnostic accuracy. RESULTS: T2 SIR for DCa were 3.60 (T/M), 0.66 (T/PZ); 2.68 (T/M), 0.47 (T/PZ) for Gleason 9; 2.50 (T/M), 0.47 (T/PZ) for Gleason 7/8 and 3.95 (T/M), 0.73 (T/PZ) for Gleason 6 tumours. There was a difference in T2 T/M and T/PZ SIR between DCa and Gleason 9 (p = 0.003, p = 0.004) and Gleason 7/8 (p = 0.006, p = 0.002), but no difference in SIR between DCa and Gleason 6 tumours. The sensitivity for tumour detection was 0-27 % for DCa, 64-82 % for Gleason 9, 44-88 % for Gleason 7-8 and 0-20 % for Gleason 6. There was a difference in the sensitivity of detecting Gleason 9 and 7/8 tumours when compared to DCa (p = 0.004, p = 0.001). CONCLUSIONS: DCa resembles Gleason score 6 tumour at T2-weighted MRI, which underestimates tumour grade and renders the tumour occult. KEY POINTS: Prostatic ductal adenocarcinoma is aggressive, resembling endometrial carcinoma at histopathology. Prostatic ductal adenocarcinoma resembles Gleason score 6 tumour at T2-weighted MRI. MRI grading may underestimate ductal adenocarcinoma based on increased T2 signal.

Diffuse pulmonary ossification as a rare cause of interstitial lung disease.

Diffuse pulmonary ossification (DPO) is a rare form of interstitial lung disease. The present article describes a case of DPO in an elderly man who presented with progressive dyspnea on exertion and an isolated reduction in diffusing capacity for carbon monoxide. DPO may occur as sequelae of mitral stenosis, left heart failure, idiopathic pulmonary fibrosis, recurrent aspiration pneumonia, solid organ transplant, adult respiratory distress syndrome or may arise idiopathically. In the absence of other findings of interstitial lung disease, a lung biopsy is unlikely to be helpful in the management of these patients.