Utilisation of goals of care discussions and palliative care prior to image-guided procedures near the end of life.

AIM: To characterise image-guided procedures performed near the end of life and the use of goals of care discussions (GOC) and palliative care consultation (PCC) prior to these procedures. MATERIALS AND METHODS: Retrospective chart review of 3,714 consecutive inpatient procedures performed for 2,351 patients and 8,206 outpatient procedures performed for 5,225 patients within a suburban medical system. Data were collected on demographics, procedures performed, mortality, and use of GOC or PCC prior to the procedures. Procedures near the end of life were classified as emergent, elective, or palliative. Logistic regression was used to assess for demographic disparities in care. RESULTS: Nine percent of inpatients died within 30 days of their procedure, 57% of which were within the same hospitalisation. Of these patients, 59% had a documented GOC and 35% had a PCC. Similarly, 7% of outpatients died within 6 months of their procedure. A minority of these patients had a documented GOC (37%) or PCC (13%). There were few statistically significant demographic disparities in this care and the associated odds ratios were small. CONCLUSION: A wide array of image-guided procedures is performed near the end of life. GOC and PCC are underutilised prior to these procedures. Few demographic disparities exist in this care.

Genetic investigation of equine recurrent uveitis in Appaloosa horses.

Equine recurrent uveitis (ERU) is characterized by intraocular inflammation that often leads to blindness in horses. Appaloosas are more likely than any other breed to develop insidious ERU, distinguished by low-grade chronic intraocular inflammation, suggesting a genetic predisposition. Appaloosas are known for their white coat spotting patterns caused by the leopard complex spotting allele (LP) and the modifier PATN1. A marker linked to LP on ECA1 and markers near MHC on ECA20 were previously associated with increased ERU risk. This study aims to further investigate these loci and identify additional genetic risk factors. A GWAS was performed using the Illumina Equine SNP70 BeadChip in 91 horses. Additive mixed model approaches were used to correct for relatedness. Although they do not reach a strict Bonferroni genome-wide significance threshold, two SNPs on ECA1 and one SNP each on ECA12 and ECA29 were among the highest ranking SNPs and thus warranted further analysis (P = 1.20 × 10-5 , P = 5.91 × 10-6 , P = 4.91 × 10-5 , P = 6.46 × 10-5 ). In a second cohort (n = 98), only an association with the LP allele on ECA1 was replicated (P = 5.33 × 10-5 ). Modeling disease risk with LP, age and additional depigmentation factors (PATN1 genotype and extent of roaning) supports an additive role for LP and suggests an additive role for PATN1. Genotyping for LP and PATN1 may help predict ERU risk (AUC = 0.83). The functional role of LP and PATN1 in ERU development requires further investigation. Testing samples across breeds with leopard complex spotting patterns and a denser set of markers is warranted to further refine the genetic components of ERU.

Widespread correction of central nervous system disease after intracranial gene therapy in a feline model of Sandhoff disease.

Sandhoff disease (SD) is caused by deficiency of N-acetyl-ß-hexosaminidase (Hex) resulting in pathological accumulation of GM2 ganglioside in lysosomes of the central nervous system (CNS) and progressive neurodegeneration. Currently, there is no treatment for SD, which often results in death by the age of five years. Adeno-associated virus (AAV) gene therapy achieved global CNS Hex restoration and widespread normalization of storage in the SD mouse model. Using a similar treatment approach, we sought to translate the outcome in mice to the feline SD model as an important step toward human clinical trials. Sixteen weeks after four intracranial injections of AAVrh8 vectors, Hex activity was restored to above normal levels throughout the entire CNS and in cerebrospinal fluid, despite a humoral immune response to the vector. In accordance with significant normalization of a secondary lysosomal biomarker, ganglioside storage was substantially improved, but not completely cleared. At the study endpoint, 5-month-old AAV-treated SD cats had preserved neurological function and gait compared with untreated animals (humane endpoint, 4.4±0.6 months) demonstrating clinical benefit from AAV treatment. Translation of widespread biochemical disease correction from the mouse to the feline SD model provides optimism for treatment of the larger human CNS with minimal modification of approach.

Development and validation of an instrument to assess child dietary fat intake.

BACKGROUND: brief dietary assessment instrument to assess dietary intakes of total fat, saturated fatty acids (SFA), and cholesterol in young children was developed and validated. METHODS: Young children and their parent or primary caretaker were recruited from a general primary care health center and local Head Start programs. Dietary records, entered and analyzed using the Minnesota Nutrition Data System, were used to calculate children's mean dietary intakes. Stepwise linear regression analysis was used to select questionnaire items that best predicted total fat, SFA, and dietary cholesterol intakes. RESULTS: This yielded a 17-item Child Dietary Fat Questionnaire (CDFQ); 9 questions correlated with total fat intake (r = 0.68, P < 0.0001); 15 questions correlated with SFA intake (r = 0.75, P < 0.0001); and 4 questions correlated with dietary cholesterol intake (r = 0.57, P < 0.0001). The test-retest reliabilities of the CDFQ in predicting children's dietary intakes of total fat, SFA, and cholesterol were 0.41, 0.66, and 0.64, respectively. The criterion-based validity of the CDFQ, evaluated against 4 days of dietary records, yielded correlations of 0.54 (P < 0.0001) for total fat, 0.36 (P < 0.01) for SFA, and 0.55 (P < 0. 0001) for dietary cholesterol intake. CONCLUSIONS: The 17-item CDFQ is a brief, easy-to-use dietary assessment instrument that could be used to identify children with high, as well as low, dietary intakes of total fat, SFA, and/or cholesterol.

Children's growth parameters vary by type of fruit juice consumed.

BACKGROUND: Excessive fruit juice consumption in young children has been associated with nonorganic failure to thrive and short stature in some children and with obesity in others. OBJECTIVE: To evaluate, in a sample of healthy young children, whether the associations between fruit juice intakes and growth parameters differ by the type of fruit juice consumed. DESIGN: Cross-sectional study. SETTING: General primary care health center in upstate New York. PARTICIPANTS: One hundred sixteen two-year-old children and one hundred seven five-year-old children, who were scheduled for a nonacute visit, and their primary care-takers or parents were recruited over a two-year period. METHODS: For 163 children (73% of total), 14 days of dietary records were available. The dietary records were entered and analyzed using the Nutrition Data System (NDS). Type of fruit juice was classified according to Nutrition Coordinating Center food codes. Height was measured using a Harpenden Stadiometer. Weight was measured using a standard balance beam scale. RESULTS: The children consumed, on average, 5.5 fluid oz/day of fruit juices, which were classified by the NDS software as 35% apple juice, 31% orange juice, 25% grape juice and 9% other types and/or mixtures of fruit juice. Children with higher fruit juice intakes had lower total fat, saturated fat and cholesterol intakes. Child height was inversely related to apple juice intake (p=0.007) and grape juice intake (p=0.02), after adjustment for child age, gender and energy intake (excluding fruit juice) and maternal height. Apple juice intake was correlated with child body mass index (p<0.05) and ponderal index (p<0.005), after adjustment for the above covariates. Total cholesterol, LDL-cholesterol, triglyceride and lipoprotein(a) levels were not related to intakes of any of the fruit juices examined. The children's ratios of total cholesterol to HDL cholesterol were correlated with grape juice intakes, while HDL-cholesterol levels were inversely related to grape juice intakes. There were no significant relationships between fruit juice intake and measures of anemia (hematocrit or mean corpuscular volume). CONCLUSIONS: The previously reported associations between short stature and high intakes of fruit juice were observed for intakes of both apple juice and grape juice. The associations between high fruit juice intakes and obesity were observed with apple juice intakes only. Because most of the fruit juice mixtures were classified as single fruit juices, the findings, especially those with grape juice, need to be cautiously interpreted. High intakes of fruit juice, however, appear to be associated with growth extremes in young children. Thus, it would seem prudent for parents and caretakers to moderate the fruit juice intakes of their young children.

Fruit and vegetable intake in young children.

BACKGROUND: Current recommendations call for most Americans, 2 years of age and over, to ent more fruits and vegetables. OBJECTIVE: To determine, in a sample of healthy children, the extent to which young children's diets include the recommended numbers of fruit and vegetable servings per day. DESIGN: Cross-sectional study. SETTING: A general primary care health center in upstate New York. PARTICIPANTS: One-hundred-sixteen 2-year-old children and 107 5-year-old children, who were scheduled for a non-acute visit, and their parent/primary caretaker (PPC) were recruited between 1992 and 1993. MEASUREMENTS: For 168 children (94 2-year-old children and 74 5-year-old children), mean dietary intakes were calculated from 7 days of written dietary records, entered and analyzed using the Minnesota Nutrition Data System. The numbers of fruit and vegetable servings/day were calculated according to USDA definitions of serving sizes. RESULTS: The 2-year-old children consumed the same amounts of fruits, 100% fruit juice, and total fruits and vegetables as the 5-year-old children (0.8 and 0.7 fruit servings/day, 1.0 and 0.8 juice servings/day, and 2.2 and 2.1 total fruit and vegetable servings/day, respectively). Fruit juice accounted for 54% of all fruit servings consumed and 42% of all fruit and vegetable servings consumed. Total fruit consumption (fruits plus juice) was correlated with carbohydrate intake (R = 0.46), and inversely correlated with total fat and saturated fat intakes (R = -0.48 and R = -0.36, respectively, both p < 0.0001) and with cholesterol intake (R = -0.21, p < 0.01). Citrus fruit and juice consumption was strongly correlated with vitamin C intake (R = 0.56, p < 0.0001). Total vegetable consumption was strongly correlated with beta-carotene and vitamin A intakes (R = 0.63 and R = 0.32, respectively, both p < 0.0001). Total fruit and vegetable consumption correlated with intakes of beta-carotene, vitamin A, vitamin C, fiber, and potassium (R = 0.55, R = 0.31, R = 0.56, R = 0.58, and R = 0.66, respectively, all p < 0.0001). Forty percent of 2-year-old children and 50% of 5-year-old children consumed < 2 servings/day of fruits and vegetables. Ninety-five percent of children consuming > or = 2 servings/day of fruits and vegetables met the RDA for vitamin C vs. 50% of those consuming < 2 servings/day (p < 0.001). CONCLUSIONS: In this study, preschool-aged children consumed, on average, about 80% of the recommended fruit servings/day, but only 25% of the recommended vegetable servings/day. Low intakes of fruits and vegetables were associated with inadequate intakes of vitamin A, vitamin C, and dietary fiber, in addition to high intakes of total fat and saturated fat.

Excess fruit juice consumption by preschool-aged children is associated with short stature and obesity.

BACKGROUND: In a referral population of young children, excessive fruit juice consumption has been reported to be a contributing factor in nonorganic failure to thrive. OBJECTIVE: To evaluate, in a population-based sample of healthy children, fruit juice consumption and its effects on growth parameters during early childhood. DESIGN: Cross-sectional study. SETTING: General primary care health center in upstate New York. PARTICIPANTS: One hundred sixteen 2-year-old children and one hundred seven 5-year-old children, who were scheduled for a nonacute visit, and their primary care taker/parent were recruited over a 2-year period. MEASUREMENTS: For 168 children (ninety-four 2-year-old children and seventy-four 5-year-old children), mean dietary intake was calculated from 7 days of written dietary records, entered, and analyzed using the Minnesota Nutrition Data System. Height was measured using a Harpenden Stadiometer. Weight was measured using a standard balance beam scale. RESULTS: The 2-year-old and 5-year-old children consumed, on average, 5.9 and 5.0 fl oz/day of fruit juice and 9.8 and 11.0 fl oz/day of milk, respectively. Nineteen children (11%) consumed > or = 12 fl oz/day of juice. Forty-two percent of children consuming > or = 12 fl oz/day of juice had short stature (height less than 20th sex-specific percentile for age) vs 14% of children drinking less than 12 fl oz/day of juice. Obesity was more common among children drinking > or = 12 fl oz/day of juice compared with those drinking less juice: 53% vs 32% had a body mass index > or = 75th age- and sex-specific percentile; 32% vs 9% had a body mass index > or = 90th age- and sex-specific percentile; and 32% vs 5% had a ponderal index > or = 90th age-specific percentile. After adjustment for maternal height, child age, child sex, and child age-sex interaction, children consuming > or = 12 fl oz/day of juice, compared with those drinking less than 12 fl oz/day of juice, were shorter (86.5 vs 89.3 cm and 106.5 vs 111.2 cm for the 2-year-old and 5-year-old children, respectively) and more overweight (body mass index = 17.2 vs 16.3 kg/m2 and ponderal index = 18.4 vs 16.8 kg/m3). CONCLUSIONS: Consumption of > or = 12 fl oz/day of fruit juice by young children was associated with short stature and with obesity. Parents and care takers should limit young children's consumption of fruit juice to less than 12 fl oz/day.