Are Angelman and Prader-Willi syndromes more similar than we thought? Food-related behavior problems in Angelman, Cornelia de Lange, fragile X, Prader-Willi and 1p36 deletion syndromes.

Food-related behavior problems are well documented in Prader-Willi syndrome (PWS), with impaired satiety, preoccupation with food and negative food-related behaviors (such as taking and storing food) frequently reported as part of the behavioral phenotype of older children and adults. Food-related behavior problems in other genetic neurodevelopmental syndromes remain less well studied, including those seen in Angelman Syndrome (AS), the 'sister imprinted disorder' of PWS. Food-related behavior problems were assessed in 152 participants each with one of five genetic neurodevelopmental syndromes ­ PWS, AS, 1p36 deletion, Cornelia de Lange, and fragile X. Predictably, levels of food-related behavior problems reported in participants with PWS significantly exceeded those of at least one other groups in most areas (impaired satiety; preoccupation with food; taking and storing food; composite negative behavior). However, in some areas people with AS were reported to display food-related problems at least as severe as those with PWS, with the AS group reported to display significantly more food-related behavior problems than at least one comparison group on measures of taking and storing food, composite negative behaviors, impaired satiety and preoccupation with food. Over 50% of participants in the AS group scored above the median point of the distribution of PWS scores on a measure of taking and storing food. These findings indicate further investigation of eating problems in AS are warranted and have implications for current theoretical interpretations of the behavioral differences between AS and PWS.

Comparison between lemonade and potassium citrate and impact on urine pH and 24-hour urine parameters in patients with kidney stone formation.

OBJECTIVES: The past few decades have been notable for advances in minimally invasive techniques to treat kidney stones. Despite successful surgical management of calculi, the impact of diet on stone disease prevention remains an important factor. Dietary modifications can be applied to prevent stone recurrence in an economical manner that is acceptable to patients. Lemonade has been studied in the past and found to increase urinary citrate levels in small groups of patients. The present study examined the effect of lemonade intake on a change in urinary pH and improvement of urinary stone risk factors compared with potassium citrate treatment. METHODS: A total of 21 stone-forming patients were treated with lemonade therapy and potassium citrate supplementation in a prospective cross-over trial. we performed 24-hour urine collections at baseline and with each treatment arm. the results between the two groups were examined to determine whether differences existed in the urinary stone risk factors. RESULTS: Potassium citrate, but not lemonade, improved citrate levels and urinary ph to a significant degree. patients taking potassium citrate had a significantly decreased urine volume compared with their urine volume drinking lemonade. neither lemonade nor potassium citrate affected the uric acid levels to a significant degree. CONCLUSIONS: The results of our study have shown that lemonade did not provide improvements in urinary citrate or pH levels but did assist patients in maintaining urine output compared with potassium citrate therapy.