Use of cystic fibrosis inhaled medication before and after elexacaftor/tezacaftor/ivacaftor initiation.

Elexacator/tezacaftor/ivacaftor (ETI) has improved cystic fibrosis (CF) outcomes. A reduction in use of maintenance medication after its initiation has been reported. Seventy-one adult people with CF (PwCF) who are followed in three CF centers and completed one year of treatment with ETI were included in this study. Their use of inhaled dornase-α, colistin, tobramycin, aztreonam and levofloxacin during this period was compared with the corresponding use during one year without ETI, using the Medication Possession Ratio (MPR). MPR was significantly decreased after ETI initiation for dornase-α (67±35% vs 48±40%, p<0.001) and for all four inhaled antibiotics together (62±33% vs 41±37%, p<0.001). The findings of this multi-center, retrospective, study suggest that the initiation of ETI significantly leads to decrease in use of standard inhaled medication in PwCF. The significance of this finding in the course of the disease is yet to be investigated by larger prospective clinical trials.

Low-frequency oscillometry indices to assess ventilation inhomogeneity in CF patients.

BACKGROUND: The utility of the forced oscillations technique (FOT) in cystic fibrosis (CF) remains uncertain. The aim of this study was to explore the ability of lower-frequency FOT indices, alone and after adjustment for the lung volume, to assess the extent of ventilation inhomogeneity in CF patients with varying disease severity. METHODS: Forty-five children, adolescents, and adults with CF (age 6.9-27 years) underwent spirometry, FOT, and nitrogen multiple-breath washout (N2-MBW) measurements. The respiratory resistance and reactance at 5 Hz (Rrs5 and Xrs5, respectively) were recorded, and a novel FOT index, the specific respiratory conductance (sGrs), was computed as the reciprocal of Rrs5 divided by the functional residual capacity. RESULTS: The sGrs correlated well with the lung clearance index (LCI) (Spearman's r: -.797), whereas the correlation of Rrs5 and Xrs5 with the LCI, albeit significant, was weaker (r: .643 and -.631, respectively). The sGrs emerged as the most robust predictor of LCI regardless of the severity of lung disease, as reflected by patients' age and lung function measurements. Most importantly, the relationship between sGrs and LCI remained unaffected by lung hyperinflation, as opposed to that of the LCI with the spirometric and standard FOT indices. CONCLUSIONS: In CF patients, the FOT indices at 5 Hz and the novel, volume-adjusted parameter sGrs, reflect the extent of lung involvement and the underlying ventilation inhomogeneity in a way comparable to N2-MBW. Future research should explore the role of lower-frequency FOT in assessing the severity and monitoring the progression of CF lung disease.

Linguistic, affective, parental, and educational contributions to the bicultural identity development of Balkan minority adolescents.

This study investigated linguistic, affective, parental, and educational contributions to bicultural identity, in two samples of younger (13- to 14-year-old; N = 95) and older (16- to 17-year-old; N = 67) bilingual adolescents, who were immigrants or belonged to ethnic minority communities in the Balkans. While bicultural identity level was not differentiated as a function of age group, there was an age-related shift in its predictors. Bicultural identity level was significantly predicted by perceived educators' attitudes toward linguistic/cultural diversity in the younger adolescent group, but by personal affective states (motivation and attitudes) toward the mainstream language in the older adolescent group. Implications of the findings are discussed regarding educational and family practices that would facilitate biculturalism in minority adolescents.

Pulmonary medication adherence among children and adults with cystic fibrosis: Is there an association with disease severity?

BACKGROUND: Adherence to pulmonary medication is pivotal in delaying the progression of lung disease in cystic fibrosis (CF). Further exploring the consequences of poor adherence and its impact on disease severity may be valuable to personalize CF treatment strategy. AIM: To evaluate indicators of disease severity among children and adults with CF and investigate which of them are related to pulmonary medication adherence. METHODS: This is a retrospective cohort study. Data of children and adults followed up in one pediatric and one adult CF Unit were evaluated over 4 years. Disease severity was assessed by measuring body mass index (BMI), lung function, history of pulmonary exacerbations, and medication complexity. Adherence was assessed by calculating a 12-month medication possession ratio (MPR) for each pulmonary medication and then averaged for a composite MPR (cMPR) for each patient. Regression analysis was performed to explore the association of adherence with disease severity. RESULTS: Ninety-five patients were included in our study, 52 children and 43 adults. The overall cMPR was 0.74 (SD = 0.25); 0.68 (SD = 0.24) for children and 0.81 (SD = 0.24) for adults. Adults presented higher adherence, with overall mean cMPR 0.81 (SD = 0.24) compared to children with mean cMPR 0.68 (SD = 0.24) (p < 0.05, 95% CI = -0.27 to -0.03). Adherence was inversely related to FEV1 % predicted (ß = -0.002, 95% CI = -0.004 to 0, p = 0.023) and FVC% predicted (ß = -0.003, 95% CI = -0.005 to -0.001, p = 0.006) in regression analysis. Adherence was not found to be associated with BMI, history of exacerbations and medication complexity. The analysis of each medication showed that adherence to Dornase-alpha, Tobramycin and Colomycin was significantly related to specific disease severity indicators. CONCLUSION: An overall moderate to high level of adherence was found among our study population. Adults presented higher adherence compared to children. FEV1% and FVC% predicted were related to a significant decrease in adherence. Among our group of CF patients with an overall moderate to high level of adherence, adherence to pulmonary medication was inversely related to disease severity.

Executive Function, Working Memory, and Verbal Fluency in Relation to Non-Verbal Intelligence in Greek-Speaking School-Age Children with Developmental Language Disorder.

Developmental Language Disorder (DLD) is often associated with impairments in working memory (WM), executive functions (EF), and verbal fluency. Moreover, increasing evidence shows poorer performance of children with DLD on non-verbal intelligence tests relative to their typically developing (TD) peers. Yet, the degree and generality of relevant difficulties remain unclear. The present study aimed at investigating WM capacity, key EFs and verbal fluency in relation to non-verbal intelligence in Greek-speaking school-age children with DLD, compared to TD peers (8-9 years). To our knowledge, the present study is the first to attempt a systematic relevant assessment with Greek-speaking school-age children, complementing previous studies mostly involving English-speaking participants. The results showed that children with DLD scored lower than TD peers on the non-verbal intelligence measure. Groups did not differ in the inhibition measures obtained (tapping resistance to either distractor or proactive interference), but children with DLD were outperformed by TD peers in the WM capacity, updating, monitoring (mixing cost), and verbal fluency (phonological and semantic) measures. The effects showed limited (in the case of backward digit recall) or no dependence on non-verbal intelligence. Findings are discussed in terms of their theoretical and practical implications as well as in relation to future lines of research.

Biculturalism, linguistic distance, and bilingual profile effects on the bilingual influence on cognition: A comprehensive multipopulation approach.

The idea that being bilingual benefits one's cognitive development and performance has been greatly challenged over the last years. If such an effect exists, as some studies continue to show, it might actually be restricted to particular contexts and bilingual profiles; not unlikely, considering the enormous diversity in the latter across the world. In this study, we assessed 4 different bilingual populations (N = 201) and 2 monolingual populations (N = 105), in the Balkan region. We formed bilingual groups based on (a) acculturation strategy (bicultural vs. monocultural), (b) linguistic distance, as well as (c) bilingual profile (balanced vs. unbalanced), based on linguistic, affective, and acculturation measures and cluster analysis. Beyond prior work, this allowed us to explore the specific conditions under which any cognitive advantage may be observed in bilinguals. We did not find systematic evidence for positive effects of bilingualism, biculturalism, or a balanced bilingual profile on inhibitory control, cognitive flexibility, monitoring, and proactive-reactive control management. The only evidence pointing to an advantage was restricted to Bosnian-Albanian bilinguals (linguistic distance analyses) and their general monitoring capacity. Acculturation strategy though, played an important role in shaping the bilinguals' language profile, and appeared to have independent effects on cognition from bilingualism. On this basis, acculturation should be considered in future explorations of bilingual cognitive development. (PsycInfo Database Record (c) 2022 APA, all rights reserved).

Toward the Establishment of New Clinical Endpoints for Cystic Fibrosis: The Role of Lung Clearance Index and Cardiopulmonary Exercise Testing.

As Cystic Fibrosis (CF) treatment advances, research evidence has highlighted the value and applicability of Lung Clearance Index and Cardiopulmonary Exercise Testing as endpoints for clinical trials. In the context of these new endpoints for CF trials, we have explored the use of these two test outcomes for routine CF care. In this review we have presented the use of these methods in assessing disease severity, disease progression, and the efficacy of new interventions with considerations for future research.

Emotional devaluation in ignoring and forgetting as a function of adolescent development.

We know that emotion and cognition interact to guide goal-directed behavior. Accordingly, it has recently been shown that distracting stimuli (Raymond, Fenske, & Tavassoli, 2003) and instructed to-be-forgotten items (Vivas, Marful, Panagiotidou, & Bajo, 2016) are emotionally devaluated. The devaluation by inhibition hypothesis (Raymond, Fenske, & Tavassoli, 2003) is the main theoretical explanation of these effects. However, we know little about how the cognition-emotion interplay is further modulated by development, and particularly, by changes in inhibitory control and affective processing within the adolescence period. In the present study we combined a selective attention task with faces, and a selective memory (directed forgetting paradigm) task with words, with a pleasantness evaluation task to address this question in three age groups; younger adolescents, older adolescents and young adults. Younger adolescents exhibited worse accuracy in the attention task, lower overall recognition of words in the memory task, and a smaller in magnitude directed forgetting effect in the latter, relative to the two older groups. That is, they showed less efficient inhibitory control in attention and memory selection. Despite this, all groups showed similar devaluation effects of the distractor faces and the to-be-forgotten words. Our findings do not fully support an inhibition account of such effects. Yet, they support the robustness of the forgetting devaluation effect, replicating the findings of Vivas, Marful, Panagiotidou, and Bajo (2016) with a Greek version of the task and in a bigger sample of participants.

Validation study of the pediatric allergic rhinitis quality of life questionnaire.

BACKGROUND: The Paediatric Allergic Rhinitis Quality of Life Questionnaire (Ped-AR-QoL) is the first tool developed for the assessment of health-related quality of life (QoL) in Greek children with allergic rhinitis (AR). OBJECTIVE: The aim of the current study was to validate the child and parent forms of the Ped-AR-QoL in children aged 6-14 years-old who suffered from AR and were followed in a pediatric allergy clinic. METHODS: The Ped-AR-QoL, which was completed by 112 children and their parents, was correlated to the generic QoL questionnaire (Disabkids), which is already valid in Greece for children with chronic disorders, as well as with expert opinions on the severity of disease. RESULTS: The Ped-AR-QoL child and parent forms had very good internal consistency (α values of 0.797 and 0.872, respectively), while there was a moderate positive correlation of the disease-specific questionnaire with most of the subscales of the generic questionnaire. There has been a statistically significant association between the Ped-AR-QoL and the expert perception of disease severity. CONCLUSIONS: The Ped-AR-QoL had very good reliability and convergent validity when compared with the generic Disabkids QoL. The significance of the association between the disease-specific questionnaire and the expert opinion is an important finding validating the questionnaire. The Ped-AR-QoL may become a helpful tool which can be used in everyday clinical practice by clinicians and it may also be used for assessing therapeutic interventions in clinical trials.

A Rare Case of Clavicle Osteomyelitis in a Child and Literature Review.

Acute clavicle osteomyelitis in children is rare representing <3% of osteomyelitis cases. We treated a 12-year-old boy who presented with acute pain in the right clavicle and high fever for 4 days. MRI showed abnormal signal in the right clavicle with periosteal reaction. Staphylococcus aureus isolated from blood was susceptible to methicillin, clindamycin, and macrolides. Clindamycin was given intravenously for 3 wks and orally for another 3 wks with no recurrence. We reviewed clavicle osteomyelitis cases in children searching PubMed English literature. From a total of 89 studies retrieved, only 6 fulfilled the criteria and were analyzed. Sixteen patients (56% female) were included with a median age of 9 yrs (range 2 wks-16 yrs). Osteomyelitis was hematogenous in most cases, with S. aureus being the most frequent cause, isolated from either blood or tissue. Symptoms included fever, swelling, and localized bone tenderness. Antimicrobial therapy lasted for 4-12 weeks (median 7.5). Three patients required drainage or curettage. Recurrence occurred in 1/16 cases (6.2%) and persistence of symptoms occurred to 2/16 cases (12.5%) reported before 90s with unknown antimicrobial susceptibility of the pathogen. Acute clavicle osteomyelitis mainly affects older children and has generally good prognosis. Staphylococcus aureus is most commonly implicated and surgery may be needed.

Upset in Response to a Sibling's Partner's Infidelity : A Study With Siblings of Gays and Lesbians, From an Evolutionary Perspective.

Existing evidence suggests that the psychological design of romantic jealousy differs for men and women: Men are more likely than women to report greater upset in response to a partner's sexual than emotional infidelity, whereas women are more likely than men to report greater upset in response to a partner's emotional than sexual infidelity. However, the observed sex difference can be explained after the fact by both an evolutionary analysis of past reproductive costs and a social constructionist analysis of social and gender role training. Attempting to disentangle these competing perspectives, researchers have measured participants' upset in response to a sibling's or a child's partner's infidelities. In contrast to what a socialization perspective would predict, participants' sex did not seem to affect their responses; the key variable was the sex of the sibling or the child, in line with a heuristic application of the evolutionary perspective. The present study attempted not only to test these competing hypotheses but also to extend previous work by involving participants with a gay or lesbian sibling and examining whether participants' responses are triggered by their sibling's or sibling's partner's sex. In line with an evolutionary perspective, participants' sex did not assert an effect on their responses. The key variable seemed to be the sex of the sibling (rather than the sex of the sibling's partner), with participants reporting greater levels of upset in response to the sexual than emotional infidelity of a gay brother's partner and to the emotional than sexual infidelity of a lesbian sister's partner. The ensuing discussion offers suggestions for future work on sex-specific triggers of jealousy.

Working memory contributions to reading comprehension components in middle childhood children.

This study examined working memory contributions to reading comprehension subskills in Greek children (mean age 9 years, 1 month). The phonological loop of the Baddeley and Hitch working memory model was assessed with 3 recall tasks (words, nonwords, and digits) and a word list matching task. The central executive (CE) was assessed with 3 tasks (listening, counting, and backward digit recall). Participants were also given a receptive vocabulary task, a reading fluency task, and written stories accompanied by comprehension questions. Canonical correlation analyses showed that the comprehension variables were related to the CE rather than the phonological loop measures. CE functions were more strongly associated with elaborative inference generation (involving significant offline processing) and comprehension control (involving metacognitive monitoring). Smaller yet significant associations were observed between the CE and the necessary inference and literal comprehension measures, whereas a moderate relationship was found in the case of the simile comprehension variable. Among the CE variables, listening recall demonstrated the highest loading on the canonical function, followed by moderate yet significant counting and backward digit recall loadings. Vocabulary was found to fully mediate several associations between working memory and comprehension measures; however, the relationship between listening recall and elaborative inferences was partly mediated. Reading fluency and, on several occasions, Greek vocabulary knowledge did not mediate the relationships between CE measures and comprehension skills assessed. This study demonstrates the usefulness of CE measures for identifying young children's possible difficulties in carrying out specific reading comprehension processes.