Aquagenic wrinkling of the palms in a cohort of Greek children diagnosed with cystic fibrosis: Associated clinical parameters.

BACKGROUND: Aquagenic wrinkling of the palms (AWP) is an excessive and early palmar wrinkling occurring after brief immersion to water (BIW), and has been reported as a frequent finding among Cystic Fibrosis (CF) patients. OBJECTIVES: To investigate any associations of CF patients presenting AWP with other disease characteristics and explore the pathomechanism of AWP phenomenon. METHODS: We evaluated AWP in CF patients and assessed the AWP parameters of palmar wrinkling, oedema, papules, pruritus and pain at 3, 7 and 11 min after a BIW test with other disease characteristics. Statistical analyses explored the associations of AWP with genotype, lung function, pancreatic insufficiency, hyperhidrosis, personal and family history of atopy and sweat chloride levels. RESULTS: One hundred CF patients (mean age 10.4 years) were included in the analysis. The genotypic distribution was ΔF508/ΔF508: 47%, ΔF508/other: 41% and other/other: 12%. Statistically significant associations of Kaplan-Meier curves of the AWP parameters with various disease characteristics and personal/family history were detected. Wrinkling was associated with history of atopy, hyperhidrosis and levels of sweat chloride test. The time to presentation of oedema and the appearance of papules were associated with history of hyperhidrosis and age at diagnosis. Finally, time to appearance of pruritus was related to history of atopy and of hyperhidrosis. Regarding TEWL regression analysis showed significant associations with age at diagnosis (p = 0.024), sweat chloride test levels (p = 0.005), history of hyperhidrosis (p = 0.033), history of atopy (p = 0.002) and hepatic-pancreatic involvement (p = 0.027). CONCLUSIONS: The existence of a statistically significant association between AWP and the history of hyperhidrosis, atopy, sweat chloride levels and hepatic-pancreatic function in CF patients was detected. A strong association between AWP and CF was detected. AWP after BIW could be elicited easily and possibly can be used as an initial screening tool to diagnose an individual with symptoms and signs that raise the likelihood of CF.

Aquagenic wrinkling of the palms after brief immersion to water test as a screening tool for cystic fibrosis diagnosis.

BACKGROUND: Aquagenic wrinkling of the palms (AWP) is an excessive and early palmar wrinkling occurring after Brief Immersion to Water (BIW), and has been reported as a frequent finding among cystic fibrosis (CF) patients. OBJECTIVES: To evaluate and assess the diagnostic performance of BIW test as an initial screening tool for CF diagnosis. METHODS: We measured AWP in CF patients, CF-heterozygotes (CF-het) and normal controls. The AWP parameters of palmar wrinkling, oedema, papules, pruritus and pain were assessed at 3, 7 and 11 min after a BIW test was performed for all the participants. Statistical analyses explored the progression of AWP in time for the three groups and assessed the diagnostic performance of BIW test as a diagnostic screening tool for CF. RESULTS: A total of 250 individuals (100 CF patients, their 50 CF-het parents, 100 healthy controls) were included in the analysis. The average age in years (mean ± SD) was 10.4 ± 4.0 for CF, 35.9 ± 6.1 for CF-het and 10.5 ± 4.0 for controls. The rate of positives for AWP at 3 min among CF patients, CF-het and controls was 68%, 8% and 0%, respectively (P < 0.01). Kaplan-Meier analysis showed a clear trend towards earlier appearance of all five parameters in the direction controls < hetCF < CF (P values <0.01). The best diagnostic performance in detecting between CF patients and non-CF was achieved by the presence of papules and wrinkling at 7 min (sensitivity/specificity: 94.0%/98.3% and 100.0%/92.0%, respectively). CONCLUSIONS: A strong association between AWP and CF was detected. AWP after BIW could be elicited easily and possibly can be used as an initial screening tool to assess if an individual with symptoms and signs that raise the likelihood of CF is a CF patient.

Bullying victimization: Associated contextual factors in a Greek sample of children and adolescents.

School bullying is increasingly recognized as an important factor affecting both individual's wellbeing and social functioning. Several studies provide evidence for the potential role of contextual factors that relate to bullying victimization such as the socioeconomic status of the parents/ family, the quality of family and home environment, the school climate, structure and ethos, and also various community characteristics. The objectives of this school-based, cross-sectional study were to report the prevalence of the perception of being bullied in a sample of Greek children and adolescents from 6 to 17 years of age and to investigate the relations among the subjective impression of bullying victimization and several sociodemographic and socioeconomic factors. We hypothesized that influences external to individual children and adolescents play a decisive role to their perception of being victimized. Bullying victimization was measured through a simple "yes/no" question, which confirmed or rejected respectively the fact that the child or adolescent has been at some time victimized in the school environment. Also, demographic and socioeconomic data about the families of children and adolescents were collected. A total of 1,588 children (51.8% females, mean age ± SD: 12.9±2.8 years) were assessed. The overall prevalence of victimization was 10.4%. Multiple logistic regression analysis on the probability of being victimized identified that living at a main urban center (Odds Ratio[OR]: 2.63, CI: 1.78-3.87, p<0.001), presence of a person with a chronic illness at home (OR: 1.90, CI: 1.12-3.20, p=0.016), poor family economic status (OR: 1.83, CI: 1.05-3.20, p=0.032),and increased number of adults at home (OR: 2.00, CI: 1.00-3.77, p=0,041) had a positive correlation with the prevalence of reported bullying victimization. Moreover, higher parental educational level related to lower probability of victimization (OR: 0.88, CI: 0.78-0.99, p=0.05). These findings demonstrate that several demographic and socioeconomic factors play a potential role in bullying victimization among schoolchildren. Our results also highlight the need to consider the influence of contextual factors in the design of targeting efforts countering and/or preventing bullying victimization.

Lactobacillus reuteri DSM 17938 and a placebo both significantly reduced symptoms in children with functional abdominal pain.

AIM: Lactobacillus reuteri is a Gram-positive bacterium that naturally inhabits the human intestinal tract. This study assessed how effectively the probiotic L. reuteri DSM 17938 managed childhood functional abdominal pain (FAP). METHODS: We recruited 54 children with a mean age 9.1 ± 3.8 years, who were diagnosed with FAP in the outpatient clinics of three university hospitals in Greece, Slovenia and Poland, according to the Rome III criteria, from January 2013 to December 2015. They were randomly assigned to receive either 2 × 108 colony-forming units of L. reuteri (n = 27) or a placebo (n = 27) for four weeks. RESULTS: Both L. reuteri and the placebo significantly reduced the frequency and intensity of abdominal pain episodes at four and eight weeks compared to baseline (all p < 0.001). L. reuteri decreased the use of pain relieving drugs at four weeks and the number of child school and adult work absences at four and eight weeks, unlike the placebo, which achieved nonsignificant results. However, the difference between the groups did not reach significance. No side effects were recorded. CONCLUSION: Both L. reuteri and the placebo were effective in alleviating pain in children with FAP, but only L. reuteri improved the child's and family's normal activities.

Functional gastrointestinal disorders in Greek Children based on ROME III criteria: identifying the child at risk.

BACKGROUND: Functional gastrointestinal disorders (FGIDs) are a common, diverse group of disorders of unknown etiology, resulting in significant socieconomic burden. In this study, we aimed to assess the prevalence of FGIDs in children aged 6-18 years and examine their association with various demographic and socioeconomic parameters. METHODS: This was a school-based, cross-sectional study approved by the relevant government authorities. Informed consent was obtained by the legal representatives of all children who participated. Diagnoses of FGIDs were based on the Greek official translation of the ROME-III questionnaire. Demographic and socioeconomic information were also collected. KEY RESULTS: A total of 1588 children (51.8% females, mean age: 12.9±2.8 years) were included. The overall prevalence of any-FGID was 23.1% (95% CI: 21.1-25.2). The most common FGIDs were functional constipation, n=231 (13.9%), abdominal migraine, n=84 (5.6%), aerophagia, n=58 (3.5%), and irritable bowel syndrome, n=48 (3.0%). Multiple logistic regression analysis on the probability of any-FGID identified physical exercise, TV-exposure, victimization, gender, parental educational level, number of children at home and number of adults at home as significant covariates for any-FGID in the final model. CONCLUSIONS AND INFERENCES: FGIDs affect approximately 1 in 4 school-aged children in Greece. The following characteristics are associated with a higher probability of any-FGID: female gender, living in a non-nuclear household, victimization, lower parental education level, infrequent physical activity, and high television exposure.

Orofacial granulomatosis as early manifestation of Crohn's disease: report of a case in a paediatric patient.

BACKGROUND: Orofacial granulomatosis (OFG) is a controversial entity mainly characterised by recurrent or permanent soft tissue swelling of sudden onset in the orofacial area with a histologic appearance of granulomatous inflammation. Differential diagnosis includes local diseases and systemic conditions, such as Crohn's disease (CD). A case of OFG in a paediatric patient is reported here, focusing on the clinical features, diagnostic procedures, treatment and long-term outcome. CASE REPORT: A 7 year-old boy presented with persistent and prominent lip swelling and painful oral mucosa lesions of six months duration. A biopsy of the lower labial mucosa revealed granulomatous inflammation consistent with OFG. The oral manifestations were managed with topical and intralesional corticosteroids. His medical history included gastrointestinal disturbances, perianal skin folds and bloody stools that raised the suspicion of CD. Colonoscopy showed inflammation without clear evidence of CD. One year later, repeated bowel investigation provided evidence suggestive of CD and the patient was placed under systemic treatment. Two years after the initial diagnosis the patient is free of oral or other pathological findings. CONCLUSION: The differential diagnosis in cases of orofacial swellings with histological granulomatous inflammation includes a variety of local and systemic diseases, diagnosis and management of which require full investigation and cooperation by a team of healthcare providers.

Determinants of adherence to gluten-free diet in Greek children with coeliac disease: a cross-sectional study.

BACKGROUND/OBJECTIVE: A strict and lifelong commitment to a gluten-free diet (GFD) remains one of the most challenging issues in children with coeliac disease. The present study aimed to record compliance rates and investigate the connection between dietary compliance and demographics, disease-related factors and parental knowledge. SUBJECTS/METHODS: Parents of 90 Greek children diagnosed with coeliac disease were recruited from the outpatient gastroenterology clinic of a children's hospital in Athens, Greece. Dietary compliance and a range of demographic and clinical data were obtained from parents through a specially constructed questionnaire. Further data included parental perceived and actual knowledge about coeliac disease and GFD. Compliant and noncompliant groups were compared for measured factors and a multivariate approach was followed to elicit independent effects of compliance determinants. RESULTS: Overall, 44.4% of children with coeliac disease were reported to be compliant to a strict GFD. A 1-year increase in the age of the child was associated with 15% lower odds of adhering to a strict diet after adjusting for other variables (odds ratio (OR)=0.85, 95% CI: 0.75-0.96). Parental perceived knowledge was also independently and significantly associated with dietary compliance (OR=3.3, 95% CI=1.1-9.8). No statistically significant correlation emerged between dietary compliance and other clinical or demographic variables. DISCUSSION: Low compliance rates to GFD were observed in children with coeliac disease. Information based on children's age and perceived parental knowledge can be used to develop risk profiles that health care professionals can utilise to identify children likely to be noncompliant and thus adjust their counselling strategy accordingly.

Biological agents in paediatric inflammatory bowel disease: a clinical observation study from Greece.

OBJECTIVES: Biological agents have contributed significantly in controlling inflammatory bowel disease during the last 15 years. This study aimed at recording and evaluating paediatric data regarding the efficacy and safety of infliximab and adalimumab during the last decade. PATIENTS AND METHODS: A total of 31 patients (43% males) with a mean age of 13.5 +/- 3.0 years were included and the majority (74%) had Crohn's disease (CD). Failure of previous treatment and steroid dependency were the main reasons for initiating anti-TNF-alpha therapy. Mean age at the first infusion was 11.0 +/- 2.8 years, while the mean disease duration at the introduction of infliximab was 2.6 +/- 2.7 years. The number of infusions per patient ranged from 1-25 (median 7, IQR: 4-13). RESULTS: Initial response was achieved in 82.8% of patients. After one year of treatment the estimated rate of remission was 53%. The rate of surgery-free disease at 12, 36 and 60 months, after the first dose of infliximab, was 89.6%, 89.6% and 74.7% respectively. The incidence of serious anaphylaxis was 4/268 infusions (1.5%) or 4/31 patients (12.3%). At three months after the first infusion only 2 children were on steroids. Adalimumab was administered to 5 patients for a mean duration of 7.4 months, as a second option after infliximab failure or infusion reaction. Two out of five patients failed to achieve remission with adalimumab and these two patients were also infliximab failures. CONCLUSION: Biological agents are valuable and safe options for children with refractory IBD. The results, so far, have been satisfactory, although, long-term outcomes remain yet to be determined.

Asporin and knee osteoarthritis in patients of Greek origin.

Ostearthritis (OA) is characterized by focal areas of loss of the articular cartilage in synovial joints, associated with varying degrees of osteophyte formation, subchondral bone change and synovitis. The Asporin (ASPN) gene which encodes a protein of the extracellular cartilage matrix contains a triplet repeat encoding for aspartic acid (D) within exon 2 The D14 allele was found associated with knee and hip osteoarthritis in case-control study in the Japanese population. Genotyping Greek knee OA patients for the D repeats we determined that the D15 allele could be considered a risk allele for our population.