What is cystic fibrosis screen positive inconclusive diagnosis? And what is it not?

Since screening for cystic fibrosis (CF) was incorporated into the newborn screening program, the number of recognised variants in the CF transmembrane conductance regulator (CFTR) gene has significantly increased. This has led to the discovery of combinations of gene variants with an uncertain prognosis. One outcome is the designation of 'cystic fibrosis screen positive inconclusive diagnosis' (CFSPID). While the majority of these children are expected to be unaffected by their CFTR variants, a small proportion have been seen to develop symptoms or increasing sweat chloride levels over time, which may reflect dysfunction of the CFTR protein.As the number of children with CFSPID increases, paediatricians and those working in primary care are more likely to encounter them in their practice. It is important that professionals have an understanding of CFSPID: what it is and, importantly, what it is not (ie, they do not have CF). In this article, we hope to explore this using some example cases, illustrating the ways in which these children may present symptomatically and how to manage them.

Physical Activity Message Framing and Ethnicity Before and During COVID-19.

People of Black ethnicities are well known to be disproportionately burdened by coronavirus and have poorer health outcomes. Public health messages encouraged physical activity during the pandemic as it is evidenced to positively affect the immune system, however people of Black ethnicities are often reported as failing to achieve the recommended daily amount. Health message framing during COVID-19 specifically in relation to ethnicity and physical activity motivation has yet to be investigated. Two studies examined message frame effect on physical activity motivation prior to and at the onset of the pandemic and how this differed by ethnicity. Gain framed messages were found to positively affect physical activity motivation pre-COVID-19 and during the pandemic fear framed messages were found to positively affect physical activity motivation. Neither of these effects differed by ethnicity. Implications for future physical activity health message framing are discussed.

Examining Emotional Literacy Development Using a Brief On-Line Positive Psychology Intervention with Primary School Children.

Wellbeing literacy (WL) may be the missing ingredient required to optimally enhance or enable positive psychology intervention (PPI) effectiveness. This study involved Victorian government funded primary schools, including two rural, two regional, and two city schools; participants included 20 classroom teachers and 131 grade five and six primary school students. A brief online PPI was implemented by teachers for 10-15 min, three times per week, for six weeks. This paper examines quantitative data collected pre and post the six week intervention, and qualitative data gathered in week one of the intervention regarding intervention effectiveness. The aim is to examine if a brief online PPI effectively builds intentional emotional vocabulary use, and to discuss how on-line PPIs can be used in public health to improve young people's WL. Considering evaluations of process effectiveness and outcome measures related to student emotional vocabulary use, results tentatively suggest that online PPIs can positively impact emotional vocabulary capability and intentionality. Multimodal communication was exercised during the PPI, suggesting that the brief online PPI format may provide a valuable tool to promote student WL.

Alterations of glucose-dependent insulinotropic polypeptide and expression of genes involved in mammary gland and adipose tissue lipid metabolism during pregnancy and lactation.

Gastric inhibitory polypeptide (GIP) is a gut derived peptide with multiple emerging physiological actions. Effects of pregnancy and lactation on GIP secretion and related gene expression were studied in Wistar rats. Pregnancy moderately increased feeding (p<0.05), whilst lactation substantially increased food intake (p<0.01 to p<0.001). Circulating GIP was unchanged during pregnancy, but non-fasting plasma glucose was significantly (p<0.01) decreased and insulin increased (p<0.05). Lactation was associated with elevated circulating GIP concentrations (p<0.001) without change of glucose or insulin. Oral glucose resulted in a significantly (p<0.001) decreased glycaemic excursion despite similar glucose-induced GIP and insulin concentrations in lactating rats. Pregnant rats had a similar glycaemic excursion but exhibited significantly lowered (p<0.05) GIP accompanied by elevated (p<0.001) insulin levels. Pregnant rats exhibited increased (p<0.001) islet numbers and individual islet areas were enlarged (p<0.05). There were no significant differences in islet alpha-cell areas, but all groups of rats displayed co-expression of glucagon and GIP in alpha-cells. Lactating rats exhibited significantly (p<0.01) increased intestinal weight, whereas intestinal GIP stores were significantly (p<0.01) elevated only in pregnant rats. Gene expression studies in lactating rats revealed prominent (p<0.01 to p<0.001) increases in mammary gland expression of genes involved in energy turnover, including GIP-R. GIP was present in intestines and plasma of 17 day old foetal rats, with substantially raised circulating concentrations in neonates throughout the period of lactation/suckling. These data indicate that changes in the secretion and action of GIP play an important role in metabolic adaptations during pregnancy and especially lactation.

FDA working to ensure the safety of medical devices used in the pediatric population.

Special initiatives exist in FDA's Center for Devices and Radiological Health (CDRH), the Center for Drug Evaluation and Research, and the Center for Biologics Evaluation and Research to ensure the safety and effectiveness of medical products used in the vulnerable pediatric population. This article focuses on the special programs, projects, and special studies implemented by CDRH to ensure this safety and effectiveness in devices used in pediatric patients throughout the devices' total product life-cycles. Pediatricians play a major role in keeping medical devices safe for use in children by reporting device problems to FDA.

Insulin modulates glucose-dependent insulinotropic polypeptide (GIP) secretion from enteroendocrine K cells in rats.

Effects of insulin excess and deficiency on glucose-dependent insulinotropic polypeptide (GIP) was examined in rats following insulinoma transplantation or streptozotocin (STZ) administration. Over 14 days, food intake was increased (p < 0.001) in both groups of rats, with decreased body weight (p < 0.01) in STZ rats. Non-fasting plasma glucose levels were decreased (p < 0.01) and plasma insulin levels increased (p < 0.001) in insulinoma-bearing rats, whereas STZ treatment elevated glucose (p < 0.001) and decreased insulin (p < 0.01). Circulating GIP concentrations were elevated (p < 0.01) in both animal models. At 14 days, oral glucose resulted in a decreased glycaemic excursion (p < 0.05) with concomitant elevations in insulin release (p < 0.001) in insulinoma-bearing rats, whereas STZ-treated rats displayed similar glucose-lowering effects but reduced insulin levels (p < 0.01). GIP concentrations were augmented in STZ rats (p < 0.05) following oral glucose. Plasma glucose and insulin concentrations were not affected by oral fat, but fat-induced GIP secretion was particularly (p < 0.05) increased in insulinoma-bearing rats. Exogenous GIP enhanced (p < 0.05) glucose-lowering in all groups of rats accompanied by insulin releasing (p < 0.001) effects in insulinoma-bearing and control rats. Both rat models exhibited increased (p < 0.001) intestinal weight but decreased intestinal GIP concentrations. These data suggest that circulating insulin has direct and indirect effects on the synthesis and secretion of GIP.

Bronchoscopy following diagnosis with cystic fibrosis.

We recently changed our practice to perform bronchoscopy following diagnosis with cystic fibrosis. On a retrospective review of 25 children, Pseudomonas aeruginosa was detected in bronchoalveolar lavage for the first time in five children (20%) and Staphylococcus aureus in four (16%). Lavage culture was positive in eight of 18 children without respiratory symptoms. This highlights the potential of bronchoscopy following diagnosis, even in asymptomatic children.

Voriconazole therapy in children with cystic fibrosis.

BACKGROUND: There is increasing evidence for the efficacy of the antifungal voriconazole, particularly in immunosuppression. We describe our experience of using voriconazole in children with CF. METHODS: We performed a retrospective case note review of children with CF treated with voriconazole in a single centre over an 18 month period. RESULTS: A total of 21 children aged 5 to 16 years (median 11.3) received voriconazole for between 1 and 50 (22) weeks. Voriconazole was used as monotherapy in 2 children with recurrent allergic bronchopulmonary aspergillosis (ABPA); significant and sustained improvements in clinical and serological parameters for up to 13 months were observed, without recourse to oral steroids. Voriconazole was used in combination with an immunomodulatory agent in a further 11 children with ABPA, with significant improvement in pulmonary function and serology. 8 children without ABPA but who had recurrent Aspergillus fumigatus isolates and increased symptoms also received voriconazole; this group did not improve with treatment. Adverse effects occurred in 7 children (33%: photosensitivity reaction 3, nausea 2, rise in hepatic enzymes 1, hair loss 1). CONCLUSIONS: Voriconazole may be a useful adjunctive therapy for ABPA in CF. Voriconazole monotherapy appears to be an alternative treatment strategy when oral corticosteroids may not be suitable.

The development of a scoring system for the Gerontological Apperception Test.

To explore the viability of a revised and more differentiated scoring system for use with the Gerontological Apperception Test (GAT; Wolk & Wolk, 1971), 102 older adults (mean age = 68.3 years) were administered the GAT. Scoring criteria were developed to reflect a variety of constructs speaking to the interpersonal, health-related, and intrapsychic dimensions of the experience of later life. For each of these 20 GAT variables, indices of interscorer agreement exceeded 80%. Bivariate correlations suggested that 12 of 20 GAT dimension scores were related to chronological age as well as to scores derived from measures of crystallized and fluid ability. This also was true for 2 of 5 GAT factor scores. Not only do these data suggest that newly developed GAT variables can be reliably scored, but they also indicate that these more carefully differentiated variables relate to measures of intellectual functioning in theoretically meaningful ways. These findings therefore speak to the utility of the GAT using more carefully defined scoring criteria.