Clinical Characteristics of Disaccharidase Deficiencies Among Children Undergoing Upper Endoscopy.

OBJECTIVES: The epidemiology and clinical significance of disaccharidase deficiencies have not been thoroughly characterized. Recent work suggests at least genetic sucrase-isomaltase deficiency is more prevalent than previously believed. Because lactase deficiency (LD) is well described, the present study focuses on the clinical characteristics of children with disaccharidase deficiencies determined by esophagogastroduodenoscopy. METHODS: Endoscopic records were reviewed from patients undergoing esophagogastroduodenoscopies with biopsies assayed for disaccharidase activity performed by 13 pediatric gastroenterologists during 5 years (2010-2014). Presenting symptoms, clinical and histological diagnosis, treatment, disaccharidase results, and demographic variables were obtained from medical and endoscopic records of those with maltase and sucrase deficiency (SD). RESULTS: Among 963 patients undergoing intestinal disaccharidase testing, 73 (7.6%) had SD on biopsy (enzyme activity <25 µmol ·â€Šmin ·â€Šg). Thirty-four (34/73; 47%) had normal duodenal histology and are the focus of this report. Four patients had SD without LD. Pan-disaccharidase deficiency was observed in 24 patients when maltase and palatinase assays were obtained (n = 646), and 11 had SD + LD when just those 2 enzymes were analyzed (n = 317). Those with SD without LD were younger 4.6 ±â€Š6.1 versus 14.1 ±â€Š3.6 years and uniformly presented with diarrhea. Patients with pan-disaccharidase deficiency or SD + LD primarily reported abdominal pain (33/35; 94%), diarrhea (16/35; 46%), nausea (14/35; 40%); and poor weight gain/weight loss (10/35; 29%); constipation, flatulence, and bloating were also noted. Maltase deficiency is less common (8/963; 0.8%), presenting with similar symptoms. CONCLUSIONS: Genetic sucrase-isomaltase deficiency often occurs together with lactase or pan-disaccharide deficiency. Disaccharidase deficiency should be considered a potential cause of abdominal pain and/or diarrhea in children and adolescents.

Relationships among Dietary Intakes and Persistent Gastrointestinal Symptoms in Patients Receiving Enzyme Treatment for Genetic Sucrase-Isomaltase Deficiency.

BACKGROUND: Sucrose-isomaltase deficiency (SID) remains underdiagnosed. Absent or reduced enzyme activity promotes diarrhea, abdominal bloating, and flatulence from undigested and malabsorbed disaccharides. Frequency and severity of gastrointestinal symptoms may be associated with the type of carbohydrates consumed. OBJECTIVE: To characterize the dietary intakes of patients treated with sacrosidase (Sucraid; QOL Medical) for SID and determine relationships between type of carbohydrates, sacrosidase dose, and gastrointestinal symptoms. DESIGN: A prospective 30-day observational study. PARTICIPANTS/SETTING: Forty-nine patients treated with sacrosidase for ≥3 months were recruited from the enzyme manufacturer's nationwide clinical database between November 2014 and August 2015. MAIN OUTCOME MEASURES: Dietary energy and nutrient intakes reported during 24-hour diet recall interviews, frequency and severity of gastrointestinal (GI) symptoms, and sacrosidase dose. STATISTICAL ANALYSES PERFORMED: Relationships between nutrient intakes, sacrosidase dose, and GI symptoms were evaluated using Spearman ρ correlation coefficients. RESULTS: Sacrosidase dose averaged 5.2±3.1 mL/day. Participants reported 1.3±0.9 bowel movements daily. Having less frequent GI symptoms was associated with higher sacrosidase intake. Energy intakes averaged 1,562.5±411.5 kcal/day in children, 1,964.7±823.6 kcal/day in adolescents, and 1,952.6±546.5 kcal/day in adults. Macronutrient composition averaged 44% carbohydrate, 39% fat, and 17% protein. Average carbohydrate composition was 35% starch, 8% fiber, and 59% sugars. Sucrose and fructose intakes were not associated with GI symptoms. Lactose intake was associated with diarrhea. Maltose intake was associated with nausea, distension, and reflux. CONCLUSIONS: Intakes were lower in carbohydrates and higher in fat compared with the Acceptable Macronutrient Distribution Ranges. Sucrose and fructose intakes were not associated with GI symptoms. Higher maltose and lactose intakes were associated with GI symptom frequency and severity. These findings provide evidence to guide nutrition counseling for patients treated for SID.

The promise of the state space approach to time series analysis for nursing research.

BACKGROUND: Nursing research, particularly related to physiological development, often depends on the collection of time series data. The state space approach to time series analysis has great potential to answer exploratory questions relevant to physiological development but has not been used extensively in nursing. OBJECTIVES: The aim of the study was to introduce the state space approach to time series analysis and demonstrate potential applicability to neonatal monitoring and physiology. METHODS: We present a set of univariate state space models; each one describing a process that generates a variable of interest over time. Each model is presented algebraically and a realization of the process is presented graphically from simulated data. This is followed by a discussion of how the model has been or may be used in two nursing projects on neonatal physiological development. RESULTS: The defining feature of the state space approach is the decomposition of the series into components that are functions of time; specifically, slowly varying level, faster varying periodic, and irregular components. State space models potentially simulate developmental processes where a phenomenon emerges and disappears before stabilizing, where the periodic component may become more regular with time, or where the developmental trajectory of a phenomenon is irregular. DISCUSSION: The ultimate contribution of this approach to nursing science will require close collaboration and cross-disciplinary education between nurses and statisticians.

The effects of environmental noise and infant position on cerebral oxygenation.

PURPOSE: To assess how different infant positions and peak sound levels affected cerebral oxygen saturation over time. SUBJECTS: Twenty-four premature infants who were born less than 32 weeks' gestational age without congenital cardiac, neurologic, and gastrointestinal anomalies. DESIGN: Repeated-measures design with the first observation between 2 and 48 hours of life; once again between 49 and 96 hours of life; on day of life 7; and every 7 days thereafter until discharge home, transfer to another hospital, or 40 weeks postmenstrual age, whichever came first. METHODS: Continuous sound levels (decibels) were obtained and 2 infant positions were performed while measuring cerebral oxygen saturation during 40-minute observation periods. MAIN OUTCOME MEASURES: Effect of peak sound and differences in infant position on cerebral oxygen saturation. RESULTS: Peak sound levels 5 dB above the average ambient sound level did not significantly change cerebral oxygen saturation values. Differences in cerebral oxygenation were significantly less when infants were changed from a supine, head midline position to a right lateral, 15° head elevation compared with a left lateral, 0° elevation position. CONCLUSIONS: Aspects of the current neonatal intensive care unit environment do not appear to affect cerebral oxygen saturation.

Positioning after feedings: what is the evidence to reduce feeding intolerances?

Nursing's goal is to be the least invasive as the patient's condition allows. As a NICU nurse, each feeding for our patients is a priority for them to grow and develop with the idea to speed discharge to home. When infants develop reflux or have increased gastric aspirates, we are concerned that their weight will taper and their length of stay will increase. Positioning provides nurses with a noninvasive option to care for these patients. The that the right-side position is best for infants to enhance digestion stems from theories of anatomy and physics. Research strengthens this supposition for those infants needing help with decreased gastric motility. Other research supports the left lateral and prone positions for those patients with GER. In practice, a patient does not necessarily have one or the other and in fact may have both GER and slowed gastric motility at any given time. The literature supports the right lateral position for enhancing gastric emptying or motility and left lateral position for GER in the uncomplicated patient with one gastrointestinal concern. The knowledge the research provides is encouraging to provide a solution, but it does not clarify the true issues of a complex patient who can have decreased gastric motility needing the right lateral position and also suffer from symptoms of GER requiring the left lateral position (see Table). For those more complicated infants, the solution might best be choosing the prone position. The prone position should not be forgotten as the findings of many studies, although not often the first choice (best results) showed it to be consistently the second best for digestive problems. In any case, the dominant positions appear to be the right or left lateral side with the prone position considered a reasonable compromise. Further research is needed to provide a clear choice for correct positioning in the NICU population. The reality for nurses is that neonatal patients are often fed every 3 hours and their lives depend on each feeding to provide nutrients for growth. It is ideal for these patients to receive every prescribed feeding and be comfortably placed in a variety of positions. Nurses' assumption that the right lateral position is best is considered, in most cases, to be a true statement for those infants with increased gastric aspirates. For those patients with GER, the left lateral position is more highly preferred. It is important for nurses to be aware of the literature but also guide their practice based on the patient assessment and presentation of symptoms. Future knowledge may provide nurses with the data needed to perfect positioning methods for infants with feeding intolerances.

Cerebral Oxygenation Monitoring: A Strategy to Detect IVH and PVL.

Premature infants are at risk for intraventricular hemorrhage (IVH) and periventricular leukomalacia (PVL) theorized to be a result from fluctuations in cerebral blood flow. Monitoring cerebral oxygenation offers a method to observe changes in cerebral blood flow that may be beneficial for detecting and preventing IVH and PVL. This article explains the potential for cerebral oxygenation monitoring in detecting IVH and PVL using cerebral oximetry, reviews current knowledge known about cerebral oxygenation, and describes current challenges for cerebral oxygenation to be the next neuroprotective vital sign.