Symptomatic palliative care for children with neurodisability

Paediatric palliative care and neurodisability are two relatively new, evolving paediatric sub-specialities that have increasing relevance in the current paediatric landscape. For many people palliative care has been synonymous with end of life care, but in paediatrics it encompasses much more and is for all children with life-threatening or life-limiting conditions, from the point of diagnosis. This breadth of focus is demonstrated well through the interface between paediatric palliative care and paediatric neurodisability. In this article we explore this unique interface through the three domains of complex symptom management, advanced care planning and end of life care. We describe the practicalities involved in all three areas and highlight the importance of early referral and the process of “dual” or “parallel” planning. We cover in more depth the specific management of the symptoms: dystonia/abnormalities of muscle tone, seizures, pain, agitation, secretions, respiratory failure, and gut failure.     

Comparison of three screening tools to detect psoriatic arthritis in patients with psoriasis (CONTEST study)

Background Multiple questionnaires to screen for psoriatic arthritis (PsA) have been developed but the optimal screening questionnaire is unknown. Objectives To compare three PsA screening questionnaires in a head‐to‐head study using CASPAR (the Classification Criteria for Psoriatic Arthritis) as the gold standard. Methods This study recruited from 10 U.K. secondary care dermatology clinics. Patients with a diagnosis of psoriasis, not previously diagnosed with PsA, were given all three questionnaires. All patients who were positive on any questionnaire were invited for a rheumatological assessment. Receiver operating characteristic (ROC) curves were used to compare the sensitivity, specificity and area under the curve of the three questionnaires according to CASPAR criteria. Results In total, 938 patients with psoriasis were invited to participate and 657 (70%) patients returned the questionnaires. One or more questionnaires were positive in 314 patients (48%) and 195 (62%) of these patients attended for assessment. Of these, 47 patients (24%) were diagnosed with PsA according to the CASPAR criteria. The proportion of patients with PsA increased with the number of positive questionnaires (one questionnaire, 19·1%; two, 34·0%; three, 46·8%). Sensitivities and specificities for the three questionnaires, and areas under the ROC curve were, respectively: Psoriatic Arthritis Screening Evaluation (PASE), 74·5%, 38·5%, 0·594; Psoriasis Epidemiology Screening Tool (PEST), 76·6%, 37·2%, 0·610; Toronto Psoriatic Arthritis Screen (ToPAS), 76·6%, 29·7%, 0·554. The majority of patients with a false positive response had degenerative or osteoarthritis. Conclusion Although the PEST and ToPAS questionnaires performed slightly better than the PASE questionnaire at identifying PsA, there is little difference between these instruments. These screening tools identify many cases of musculoskeletal disease other than PsA.     

Low frequency of α-synuclein mutations in familial Parkinson's disease

A mutation in exon 4 of the α-synuclein (NACP) gene has been reported to explain the chromosome 4 linkage to autosomal dominant Parkinson's disease. We developed primers and methods for exonic sequencing of this gene and sequenced the entire coding region of the gene in 6 families with autosomal dominant disease and in 2 cases of lytico and bodig from Guam. In addition, we have sequenced exon 4 of this gene in 5 cases of familial disease and have screened for the specific mutation (A53T) in a 40 cases of idiopathic Parkinson's disease, 3 cases of multisystem atrophy, and 15 cases of Lewy body dementia. We have found no genetic variation in the gene. We discuss these findings with respect to both the epidemiology of Parkinson's disease and the possibility that NACP is not the chromosome 4 locus for disease.     

Helicobacter pylori-like Microorganisms and Chronic Active Gastritis in Ferrets

To determine the prevalence and histology of Helicobacter pylori (HP) associated gastritis in young ferrets, we examined 36 normal 2- to 4-month old ferrets. Identification of HP-like microorganisms included Warthin Starry stains of tissue sections, rapid urease test on fresh tissue, and culture. HP-like microorganisms were found in the stomachs of 35/36 ferrets. The highest density of microorganisms was seen in the antrum, where HP-like microorganisms were present in the pits and in deep glands. HP-like microorganisms were also seen in the cardia and on the foveolar epithelium of the fundus, but not in fundic glands. Chronic active gastritis was seen in all animals with HP-like microorganisms, but involved only the antrum. The distal antrum was most severely involved. One animal had no evidence of HP-like microorganisms on tissue sections or by rapid urease test. Gastric tissue sections from this animal showed only minimal infiltration of the lamina propria by polymorphonuclear leukocytes and lymphocytes. Gastritis associated with HP-like microorganisms is common in ferrets and is acquired at a young age. It is associated with chronic active antral gastritis similar to that seen in humans, suggesting that ferrets should provide a useful experimental model for HP-associated gastritis and peptic ulcer.     

How effective are interventions in improving dietary behaviour in low- and middle-income countries? A systematic review and meta-analysis

Several interventions encouraging people to change their diet have been tested in low- and middle-income countries (LMICs) but these have not been meta-synthesised and it is not known which elements of these interventions contribute to their effectiveness. The current review addressed these issues. Randomised controlled trials of dietary interventions in LMICs were eligible and identified via eight publication databases. Elements of both the intervention and comparison groups (e.g., behaviour change techniques (BCTs), delivery mode), participant characteristics and risk of bias were coded. Random effects meta-analysis of 76 randomised controlled trials found, on average, small- to medium-sized but highly heterogeneous improvement in dietary behaviour following an intervention. Small and homogeneous improvements were found for BMI/weight, waist- and hip-circumference, with medium-sized, but heterogeneous, improvements in blood pressure and cholesterol. Although many BCTs have yet to be tested in this context, meta-regressions suggested some BCTs (action planning, self-monitoring of outcome(s) of behaviour; demonstration of behaviour) as well as individually randomised trials, adult- or hypertensive-samples and lack of blinding were associated with larger dietary behaviour effect sizes. Interventions to encourage people from LMICs to change their diet produce, on average, small-to-medium-sized effects. These effects may possibly be increased through the inclusion of specific BCTs and other study elements.     

Intergenerational participatory discussion groups foster knowledge exchange to improve child nutrition and food security in northern Malawi

This article assesses the effectiveness of a participatory, intergenerational, dialogue approach in addressing gender and generational conflicts related to both child nutrition and agriculture. Analysis of 46 interviews and 3 focus groups with smallholder farmers in rural agrarian communities with high rates of child malnutrition in northern Malawi suggested that participatory discussion can lead to positive change, including increasing child feeding frequency and dietary diversity. An intergenerational, transformative, and holistic approach to nutrition education which integrates agricultural and gender issues can effectively address sensitive conflicts within households and communities that affect child nutrition, and come up with local solutions.