The Epidemiology of Pediatric Autoimmune Hepatitis in Scotland: A National Cohort Study.

Autoimmune hepatitis (AIH) is a rare, but potentially severe, cause of liver disease in children. We aimed to summarize how children with AIH in Scotland presented, were investigated and managed in addition to producing novel epidemiological data and outcomes. Methods: All prevalent pediatric patients with AIH cared for in pediatric services between January 2013 and September 2018 were included. Individual patient data were obtained from electronic patient records in the 3-main academic pediatric centers in Scotland covering the entire population. Results: Thirty-eight patients were included (25 female) with median follow-up of 33 months (range, 2-145 mo) and 136 total patient years. The incidence between 2014 and 2017 was 0.49/100 000/y (95% confidence interval, 0.29-0.78) and point prevalence between 2013 and 2018 was 1.75/100 000 (95% confidence interval, 1.42-2.13). Thirty-five (92%) patients were autoantibody positive, most commonly anti-nuclear antibody (63%) and anti-smooth muscle antibody (42%). Thirty-seven (97%) patients had induction therapy with oral corticosteroids, 30 (79%) required maintenance treatment with azathioprine, and 23 (61%) received ursodeoxycholic acid. There were 1.4 disease flares per 10 patient years and 3 patients required liver transplantation with an overall 5-year survival rate without the need for transplantation of 95%. Conclusions: We calculated a novel incidence and prevalence rate for pediatric AIH in Scotland. Nearly all were invariably treated initially with corticosteroids with most placed-on azathioprine as maintenance therapy. Outcomes were generally favorable with low rates of disease flares and the need for transplantation being rare.

Nutritional status, growth and disease management in children with single and dual diagnosis of type 1 diabetes mellitus and coeliac disease.

BACKGROUND: The consequences of subclinical coeliac disease (CD) in Type 1 diabetes mellitus (T1DM) remain unclear. We looked at growth, anthropometry and disease management in children with dual diagnosis (T1DM + CD) before and after CD diagnosis. METHODS: Anthropometry, glycated haemoglobin (HbA1c) and IgA tissue transglutaminase (tTg) were collected prior to, and following CD diagnosis in 23 children with T1DM + CD. This group was matched for demographics, T1DM duration, age at CD diagnosis and at T1DM onset with 23 CD and 44 T1DM controls. RESULTS: No differences in growth or anthropometry were found between children with T1DM + CD and controls at any time point. Children with T1DM + CD, had higher BMI z-score two years prior to, than at CD diagnosis (p < 0.001). BMI z-score change one year prior to CD diagnosis was lower in the T1DM + CD than the T1DM group (p = 0.009). At two years, height velocity and change in BMI z-scores were similar in all groups. No differences were observed in HbA1c between the T1DM + CD and T1DM groups before or after CD diagnosis. More children with T1DM + CD had raised tTg levels one year after CD diagnosis than CD controls (CDx to CDx + 1 yr; T1DM + CD: 100% to 71%, p = 0.180 and CD: 100% to 45%, p < 0.001); by two years there was no difference. CONCLUSIONS: No major nutrition or growth deficits were observed in children with T1DM + CD. CD diagnosis does not impact on T1DM glycaemic control. CD specific serology was comparable to children with single CD, but those with dual diagnosis may need more time to adjust to gluten free diet.

Detection of four major bacterial potato pathogens.

Ring rot, brown rot and blackleg represent major bacterial pathogens of potato. The methods described below are aimed at basic identification of ring rot, brown rot or blackleg in a tuber sample.

How does a standardized care plan facilitate the practice of pharmaceutical care planning in mental health settings across Scotland?

OBJECTIVES: The aim of the project was to understand how a standardized generic pharmaceutical care plan could facilitate the practice of mental health pharmacy care planning across Scotland. Specifically, the objectives were to examine whether the plan was easy to use, collected appropriate information and enabled communication on discharge; when the plan was used in relation to multidisciplinary meetings; how useful it was to other team members; whether it changed the way pharmacists worked; and who wrote in the plan. A further objective was to examine whether there were barriers to implementing the plan and how these may be overcome. METHODS: A piloted questionnaire and diary were completed by practising pharmacists, asking them to describe their use of the generic care plan. Mental health pharmacists throughout Scotland used the care plans with adult, elderly and forensic mental health inpatients over the 2 month period. KEY FINDINGS: Forty-four mental health pharmacists participated (69.84% of practising mental health pharmacists across Scotland). They used the plan with 241 adult, 70 elderly and 16 forensic mental health patients. Many sections of the plan received positive appraisal, but changes were also widely recommended. Changes were on three levels: (1) additions or deletions to individual sections of the care plan, some of which may reflect local circumstances, (2) more radical deletions or changes to entire sections or pages and (3) to take into account the wider social and political context in which pharmacists work, with the potential for local rates of progress towards national imperatives, such as multidisciplinary working. CONCLUSIONS: The development of a standardized care plan for mental health pharmacists should address policy issues and could enhance the role of the pharmacist within multidisciplinary teams.

State Behavioral Scale: a sedation assessment instrument for infants and young children supported on mechanical ventilation.

OBJECTIVE: To develop and test the reliability and validity of the State Behavioral Scale for use in describing sedation/agitation levels in young intubated patients supported on mechanical ventilation. DESIGN: : In this prospective, psychometric evaluation, pairs of trained pediatric critical care nurse evaluators simultaneously and independently assessed a convenience sample of pediatric intensive care unit patients along eight state/behavioral dimensions and a numeric rating scale (NRS) of 0 (extremely sedated) to 10 (extremely agitated). The eight dimensions were derived from the sedation/agitation literature and expert opinion and included respiratory drive, response to ventilation, coughing, best response to stimulation, attentiveness to careprovider, tolerance to care, consolability, and movement after consoled, each with 3-5 levels. SETTING: An 18-bed pediatric medical-surgical intensive care unit and 26-bed pediatric cardiovascular intensive care unit in a university-affiliated academic children's hospital. PATIENTS: A total of 91 intubated mechanically ventilated patients 6 wks to 6 yrs of age provided a median of two observations (interquartile range, 1-3) for a total of 198 sets of observations. Excluded were postoperative patients or those receiving neuromuscular blockade. INTERVENTIONS: Patients were observed for 1 min, and then incremental levels of stimulation were applied until patient response. After 2 mins of consoling, the state behavioral assessment and NRS were completed. MEASUREMENTS: Weighted kappa and intraclass coefficients were generated to assess interrater reliability of the eight dimension and NRS ratings. Distinct state behavior profiles were empirically identified from the dimension ratings using hierarchical cluster analysis using a squared Euclidean distance measure and between-groups linkage. Construct validity of these profiles was assessed by comparing group mean NRS scores using one-way analysis of variance. MAIN RESULTS: Weighted kappa scores for all 198 dimension ratings ranged from .44 to .76, indicating moderate to good interrater reliability. The intraclass coefficient of .79 was high for NRS ratings. Cluster analysis revealed five distinct state profiles, with mean NRS ratings of 1.1, 2.5, 4.0, 5.3, and 7.6, all of which differed significantly from each other (F = 75.8, p < .001), supporting the profiles' construct validity. CONCLUSIONS: Based on empirically derived state behavior profiles, we have constructed the State Behavioral Scale to allow systematic description of the sedation-agitation continuum in young pediatric patients supported on mechanical ventilation. Further studies including prospective validation and describing the effect of State Behavioral Scale implementation on clinical outcomes, including the quality of sedation and length of mechanical ventilation, are warranted.

NK cells developing in vitro from fetal mouse progenitors express at least one member of the Ly49 family that is acquired in a time-dependent and stochastic manner independently of CD94 and NKG2.

NK cells developing in vitro from fetal progenitors in the presence of IL-2 are phenotypically and functionally indistinguishable from mature adult NK cells, with the exception that they generally lack surface expression of any of the Ly49 molecules that have previously been examined. Using two recently developed anti-Ly49 mAb, we show here that most of these NK cells in fact express high levels of at least one previously uncharacterized member of the Ly49 family, most likely Ly49E. Detailed kinetic and clonal analysis revealed that these Ly49 molecules were acquired in a progressive and stochastic manner independently of CD94 and NKG2. CD94 and NKG2 were both expressed early in NK cell development, sometimes in the absence of NK1.1, with CD94 invariably being expressed at two different levels. IL-4 differentially inhibited the expression of CD94 and Ly49 receptors, but had little or no effect on the expression of NKRP1 molecules.