Dental caries and associated salivary biomarkers in patients with cystic fibrosis.

PURPOSE: To evaluate the caries status of the Cystic fibrosis (CF) children and adolescents with the comparation of some biochemical markers, secretory-immunoglobulin-A (sIgA), and antimicrobial peptides in the saliva. METHODS: In this cross-sectional descriptive study, the approval Ethics Board was obtained. Unstimulated saliva samples were collected from CF and healthy control children (non-CF) patients. Both groups underwent the same dental and periodontal evaluation scheme of the assessment. Human beta defensin (HBD1), human alpha defensin (HNP-1), cathelicidin (LL-37), sIgA in saliva were evaluated by enzyme-linked immunoassay method. A general biochemical analysis was performed. Statistical analysis was performed by using Statistical Package for the Social Sciences Version 20.0 (SPSS Inc.). RESULTS: A total of 21 (9 male, 12 female) CF and 23 (11 male, 12 female) control patients were participated with the mean age of 10.17 ± 3.38 and 9.52 ± 2.15 years, respectively. In control children, DMFT/S (decayed-missing-filled-tooth/surface-in-permanent-dentition), dmft/s (decayed-missing-filled-tooth/surface-in-primary-dentition) values were higher; DT (decayed-tooth in permanent dentition), ft (filled-tooth in primary dentition) and plaque index values were statistically significantly higher (p = 0.042, p = 0.005, p = 0.038, respectively) than CF patients. Bicarbonate was higher in control group; sodium, chloride, and total protein were higher in CF group; magnesium, calcium and phosphate levels were similar in each group (p > 0.05). Alpha and beta defensin-1 levels in control group was statistically significantly higher (p = 0.037 and p = 0.020, respectively), while LL37 and sIgA were not statistically significantly higher (p > 0.05) than CF group. CONCLUSIONS: Children with CF had lower caries in permanent teeth, filling in primary teeth, and an altered salivary biomarker profile, especially in HNB1, HNP1. Therefore, it is important to conduct periodic oral-dental controls among CF patients during their childhood.

Identifying undiagnosed or undertreated patients with familial hypercholesterolemia from the laboratory records of a tertiary medical center.

OBJECTIVE: Familial hypercholesterolemia (FH) is a life-threatening genetic disease associated with elevated low-density lipoprotein cholesterol (LDL-C) and premature coronary heart disease that is undiagnosed and undertreated around the world. This study aimed to examine the demographic characteristics, awareness, and treatment adherence of undiagnosed or undertreated FH patients based on laboratory records. METHODS: In a 16-month retrospective survey using laboratory records, patients with elevated LDL-C (>250 mg/dL) were identified (n=395). Patients younger than 18 years of age or with secondary causes of dyslipidemia were excluded (n=98). In all, 297 patients were called and asked to participate in a phone interview regarding their demographic characteristics, awareness of dyslipidemia, and treatment adherence. RESULTS: A total of 147 patients (mean age: 51.7±16.6 years; 59.2% female) completed the interview. The mean LDL-C level of the patients was 292.8±49.9 mg/dL. According to the Dutch Lipid Clinic Network criteria, 18.4% of the patients had definite FH, 66.0% had probable FH, and 15.6% had possible FH. Although the majority of the patients (93.9%) were aware of their high LDL-C level, only about half of them (n=75; 51.0%) were in treatment. Of all the patients who were interviewed, 21% (n=31) had never taken medication to lower their LDL-C, and 28% (n=41) had stopped taking a lipid-lowering drug. CONCLUSION: This pilot study revealed that a significant number of FH patients were not taking statins despite having a very high LDL-C level. Nationwide detection of likely FH patients using hospital records and interviewing them via a phone survey may help to better understand and manage these high-risk patients.

Six minute walk test versus incremental shuttle walk test in cystic fibrosis.

BACKGROUND: Although both self-paced and externally paced field tests are widely used in cystic fibrosis (CF), it is still unclear whether they induce clinically relevant and similar cardiorespiratory responses. The aim of this study was therefore to compare the incremental shuttle walk test (ISWT) and 6 min walk test (6MWT), and to determine the factors influencing exercise capacity in CF. METHODS: Fifty clinically stable CF patients were included in the study. Pulmonary function, peripheral and respiratory muscle strength were assessed, anthropometric measurements were recorded, and 6MWT and ISWT carried out. RESULTS: The CF patients covered significantly more distance in the ISWT than 6MWT (P < 0.001). Heart rate response and dyspnea score at the end of the tests and during the recovery phase were significantly higher in the ISWT compared with the 6MWT (P < 0.05). The 6MWT and ISWT had similar moderate-strong correlations with age, height, weight, pulmonary function, respiratory and peripheral muscle strength (P < 0.05). Forty-nine percent of the variance in 6MWT distance was explained by age and forced expiratory volume in 1 s (FEV1 ; R(2)  = 0.49, F(2-48) = 22.033, P < 0.001). The variables contributing to ISWT distance were FEV1 , inspiratory muscle strength, and body mass index (R(2)  = 0.596, F(3-44) = 20.176, P < 0.001). CONCLUSIONS: The ISWT is a better reflection of exercise tolerance in CF than 6MWT. ISWT is a preferable alternative assessment of exercise tolerance in terms of cardiorespiratory response.