Caregiver burden in patients with behavioural variant frontotemporal dementia and non-fluent variant and semantic variant primary progressive aphasia.

Studies on caregiver burden in patients with frontotemporal lobar degeneration are rare, differ methodologically and show variable results. Single center longitudinal pilot study on caregiver burden and potential risk factors in patients with behavioural variant frontotemporal dementia (bvFTD) and semantic (svPPA) and non-fluent variants (nfvPPA) primary progressive aphasia. Forty-six bvFTD, nine svPPA, and six nfvPPA patients and caring relatives were analysed for up to 2 years using the Mini-Mental State Examination as global measure for cognitive performance, Frontal Assessment Battery (frontal lobe functions), Frontal Behavioural Inventory (personality and behaviour), Neuropsychiatric Inventory (dementia-related neuropsychiatric symptoms), Barthel Index and Lawton IADL Scale (basic and instrumental activities of daily living), the Caregiver Strain Index (CSI), and in most participants also the Zarit Burden Interview (ZBI). CSI baseline sum scores were highest in bvFTD (mean ± SD 5.5 ± 3.4, median 5, IQR 6), intermediate in svPPA (2.9 ± 2.3; 3; 3.5) and low in nfvPPA (1.6 ± 2.1; 1; 2). Similar differences of caregiver burden were found using the ZBI. During follow-up, CSI and ZBI sum scores deteriorated in svPPA, not in bvFTD and nfvPPA, and correlated significantly with personality and behaviour, neuropsychiatric symptoms, caregiver age, and instrumental, but not basic activities of daily living, Mini-Mental State Examination scores or frontal lobe functions. This study reveals differences in caregiver burden in variants of frontotemporal lobar degeneration. Caregivers should be systematically asked for caregiver burden from the time of the diagnosis to provide comprehensive support in time.

Fermentation of Whole Grain Sorghum (Sorghum bicolor (L.) Moench) with Different Dry Matter Concentrations: Effect on the Apparent Total Tract Digestibility of Energy, Crude Nutrients and Minerals in Growing Pigs.

This study investigated the effects of sorghum ensiled as whole grains with different dry matter concentrations on the apparent total tract digestibility (ATTD) of energy, crude nutrients and minerals in growing pigs. Whole grain sorghum batches with varying dry matter (DM) concentrations of 701 (S1), 738 (S2) and 809 g kg-1 (S3) due to different dates of harvest from the same arable plot, were stored in air-tight kegs (6 L) for 6 months to ensure complete fermentation. Subsequently, 9 crossbred barrows (34.6 ± 1.8 kg; (Duroc x Landrace) × Piétrain)) were used in a 3 × 3 Latin square feeding experiment. Diets were based on the respective sorghum grain silage and were supplemented with additional amino acids, minerals and vitamins to meet or exceed published feeding recommendations for growing pigs. The ATTD of gross energy, dry matter, organic matter, nitrogen-free extracts, and crude ash were higher in S1 compared to S3 treatments (p ≤ 0.05), while S2 was intermediate. Pigs fed S1 showed significantly higher ATTD of phosphorus (P) compared to all other groups while ATTD of calcium was unaffected irrespective of the feeding regime. In conclusion, growing pigs used whole grain sorghum fermented with a DM concentration of 701 g kg-1 (S1) most efficiently. In particular, the addition of inorganic P could have been reduced by 0.39 g kg-1 DM when using this silage compared to the variant with the highest DM value (809 g kg-1).

Relevance and management of methylation data in electronic health records.

In this paper we give an overview of challenges and chances of the integration of methylation data into Electronic Health Records. Perspectives of methylation data in terms of clinical relevance and characteristics of these data are shown. Among several standards OpenEHR, HL7 CG and LOINC are identified as starting point for the representation and communication of methylation data within the clinical context.

Low tetrahydrobiopterin biosynthetic capacity of human monocytes is caused by exon skipping in 6-pyruvoyl tetrahydropterin synthase.

Biosynthesis of (6 R )-5,6,7,8-tetrahydro-L-biopterin (H(4)-biopterin), an essential cofactor for aromatic amino acid hydroxylases and NO synthases, is effectively induced by cytokines in most of the cell types. However, human monocytes/macrophages form only a little H(4)-biopterin, but release neopterin/7,8-dihydroneopterin instead. Whereas 6-pyruvoyl tetrahydropterin synthase (PTPS) activity, the second enzyme of H(4)-biopterin biosynthesis, is hardly detectable in these cells, PTPS mRNA levels were comparable with those of cell types containing intact PTPS activity. By screening a THP-1 cDNA library, we identified clones encoding the entire open reading frame (642 bp) as well as clones lacking the 23 bp exon 3, which results in a premature stop codon. Quantification of the two mRNA species in different cell types (blood-derived cells, fibroblasts and endothelial cells) and cell lines showed that the amount of exon-3-containing mRNA is correlated closely to PTPS activity. The ratio of exon-3-containing to exon-3-lacking PTPS mRNA is not affected by differential mRNA stability or nonsense-mediated mRNA decay. THP-1 cells transduced with wild-type PTPS cDNA produced H(4)-biopterin levels and expressed PTPS activities and protein amounts comparable with those of fibroblasts. We therefore conclude that exon 3 skipping in transcription rather than post-transcriptional mechanisms is a major cause of the low PTPS protein expression observed in human macrophages and related cell types.