Caregiver Quality of Life with Tyrosinemia Type 1.

Tyrosinemia type I (HT1) is an inborn error of metabolism (IEM). Current management guidelines include lifelong specialized diet and use of the orphan drug, nitisinone. This study explores the quality of life (QOL) of caregivers of children with HT1. Caregivers for 26 children with HT1 completed a questionnaire (TYR-QOL) adapted to this patient population from an existing validated QOL questionnaire (PKU-QOL). Responses were analyzed via domain scores, based on predetermined scoring guidelines. Results suggest HT1 has a moderate overall impact on caregiver QOL, with emotional aspects of the disease having the greatest impact. HT1 diet and specialized formula also had an impact on caregiver QOL, with the vast majority feeling guilt if their child's diet and specialized formula plan were not followed. Management of nitisinone did not impact caregiver QOL. Results were compared to the phenylketonuria (PKU) population. Domain scores for the emotional, practical, social, and overall impact on QOL were higher for HT1 than for mild PKU, indicating a greater impact on QOL. Domain scores for practical and social aspects were similarly higher for HT1 than for classic PKU, though emotional and overall impacts were comparable. This is the first questionnaire to assess QOL in caregivers of children with HT1. Results can be used to better understand psychosocial implications of HT1 and assist healthcare professionals in addressing treatment issues.

A Synthesis of Coping Experiences After Infant Death.

The purpose of this article was to synthesize qualitative research data that examine parental coping strategies following infant death. This qualitative synthesis found that parents who effectively cope with the death of their infant would continue the bond with the deceased child, have differences in the way they manage their emotions about the loss, and have intergenerational support in the form of family being present, acknowledging the death, performing immediate tasks, and providing helpful information. Nurses should be vigilant to ensure parents receive "memories" of their infant after an in-hospital death. Knowledge of the coping process can assist nurses and clinicians to better care and support parents following an infant death and, in turn, facilitate the healing process.

Cytotoxic oplopane sesquiterpenoids from Arnoglossum atriplicifolium.

Pale Indian plantain (Arnoglossum atriplicifolium (L.) H. Rob.) is a plant with traditional medicinal usage among the Cherokee Native American tribe for treating cancer. Two oplopane sesquiterpenoids were isolated from an extract of A. atriplicifolium from Western North Carolina. The compounds were isolated by bioassay-guided fractionation using an MCF-7 breast tumour cell line assay. The known compound (1S,6R,7R,8R)-1-acetoxy-6,7-diangeloxy-8,10-epoxy-2-oxo-oplopa-3,14Z,11,12-dien-13-al (1) had an EC50 value of 9.0 µM against MCF-7 cells, while the new compound (1S,3R,6R,7R,8R,11S)-1-acetoxy-6,7-diangeloxy-8,10,11,13-bisepoxyoplopan-2-one (2) had an EC50 value of 96 µM. The compounds were characterised by 1D and 2D NMR spectroscopy and by comparison with literature values in the case for 1. Based on NOESY analysis, a correction of the relative configuration for 1 is presented. The presence of these compounds may help to explain the folk remedy usage of this plant as an anticancer agent.