Long-Term Dietary Changes in Subjects with Glucose Galactose Malabsorption Secondary to Biallelic Mutations of SLC5A1.

BACKGROUND: Glucose galactose malabsorption (GGM) is a congenital diarrheal disorder of intestinal Na+/glucose cotransport (SGLT1/SLC5A1). The required glucose and galactose-restricted diet has been well described in infancy, but long-term nutrition follow-up is limited. AIM: To perform a comprehensive nutritional assessment on a cohort of patients with GGM to gain insights into the consumption patterns within the population. METHODS: A cross-sectional study examining dietary intake of a GGM cohort using prospective food records. The calories and nutrients of all foods, beverages, and condiments were analyzed with descriptive statistics and compared to intake patterns of age- and sex-matched NHANES groups. RESULTS: The six patients were 0.7-26 years old. Whole foods and vegetable fats were major parts of the diet, while dairy and added sweeteners were restricted. Compared to typical US intakes, mean macronutrient distribution was 88th percentile from fat, 18th percentile from carbohydrates, and 78th percentile from protein. Fructose consumption, as a proportion of total sugar intake, decreased with age, from 86.1 to 50.4%. Meanwhile, glucose consumption increased with age, from 13.8 to 48.6% of sugar intake. However, the actual amount of glucose consumed remained low, equivalent to 4th percentile of US consumption level. Galactose intake was marginal throughout life. CONCLUSIONS: A GGM diet is a high-fat and high-protein/low-carbohydrate diet that is rich in fruits and vegetables but limited in dairy and added sugar. Relatively less fructose but more glucose is incorporated into the diet with age. Future studies should investigate the effects of the GGM diet on gut microbiome and long-term health.

Quality Initiative to Improve time to Antibiotics for Febrile Pediatric Patients with Potential Neutropenia.

INTRODUCTION: In patients who are immunocompromised, fever may indicate a life-threatening infection. Prompt time to antibiotic administration in febrile patients at risk for neutropenia has been identified by national and international panels as a key benchmark of quality care in emergent situations. A quality improvement initiative to improve health care provided in a pediatric emergency department (ED) is described. METHODS: A clinical pathway was previously initiated in a pediatric ED with a goal of improving time to antibiotics for febrile neutropenia patients. An agreed upon pathway and order set being initiated. Improvements were seen but not to the desired level. This project involved an improvement cycle that focused on nonvalue added time in the workflow. RESULTS: Percent of patients receiving antibiotics within the goal time of 1 hour increased from 40% to 80% with the intervention. Process measures including arrival to ED bed time, ED bed to antibiotic order time and antibiotic order time to delivery time were followed. CONCLUSION: Clinical guidelines, order sets and detailed understanding of the actual workflow at the point of care delivery can be instrumental in achieving the goals of reducing time to antibiotics.

A comparison of parental and nursing assessments of level of illness or injury in a pediatric emergency department.

BACKGROUND: The 5-tier Emergency Severity Index (ESI) score is a well-accepted, validated triage tool with good interrater reliability. Parental perception of illness severity has not been compared to ESI score. OBJECTIVE: This study compares parental assessment of severity of illness to triage nurse acuity. DESIGN: Prospective and descriptive. SETTING: Large, urban pediatric emergency department (ED). PARTICIPANTS: Parents/guardians of patients younger than 18 years. INTERVENTION: The triage nurse assigned an ESI score, and the parent/ guardian assigned all patients a severity score on a scale of 1 to 5 (1, most sick and 5, least sick). Mean severity scores were compared between the groups. RESULTS: There were 142 participants with a mean patient age of 6.15 years. The mean participant and nurse severity scores were 3.01 and 3.35, respectively, with an intraclass correlation coefficient of 0.203 (P = 0.008). Most frequently, the parent/guardian and triage nurse assigned the same score (n = 44, 31%). Seventy-six percent of the parent/ guardian scores were within 1 point of the triage nurse score. CONCLUSIONS: Close agreement exists between parent/guardian and nurse ESI scores, illustrating objectivity in parent/guardian assessments. This study provides a springboard for future studies regarding ED use after educating families on ED triage.

The effects of very early Alzheimer's disease on the characteristics of writing by a renowned author.

Iris Murdoch (I.M.) was among the most celebrated British writers of the post-war era. Her final novel, however, received a less than enthusiastic critical response on its publication in 1995. Not long afterwards, I.M. began to show signs of insidious cognitive decline, and received a diagnosis of Alzheimer's disease, which was confirmed histologically after her death in 1999. Anecdotal evidence, as well as the natural history of the condition, would suggest that the changes of Alzheimer's disease were already established in I.M. while she was writing her final work. The end product was unlikely, however, to have been influenced by the compensatory use of dictionaries or thesauri, let alone by later editorial interference. These facts present a unique opportunity to examine the effects of the early stages of Alzheimer's disease on spontaneous written output from an individual with exceptional expertise in this area. Techniques of automated textual analysis were used to obtain detailed comparisons among three of her novels: her first published work, a work written during the prime of her creative life and the final novel. Whilst there were few disparities at the levels of overall structure and syntax, measures of lexical diversity and the lexical characteristics of these three texts varied markedly and in a consistent fashion. This unique set of findings is discussed in the context of the debate as to whether syntax and semantics decline separately or in parallel in patients with Alzheimer's disease.