Gastrointestinal bleeding in children with familial dysautonomia: a case-control study.

OBJECTIVE: Familial dysautonomia (FD) is a rare inherited autosomal recessive disorder with abnormal somatosensory, enteric, and afferent autonomic neurons. We aimed to define the incidence of gastrointestinal bleeding and its associated risk factors in patients with FD. METHODS: In this retrospective case-control study, we identified all episodes of gastrointestinal bleeding in patients with FD, occurring over four decades (January 1980-December 2017), using the New York University FD registry. RESULTS: We identified 104 episodes of gastrointestinal bleeding occurring in 60 patients with FD. The estimated incidence rate of gastrointestinal bleeds in the FD population rate was 4.20 episodes per 1000 person-years. We compared the 60 cases with 94 age-matched controls. Bleeding in the upper gastrointestinal tract from gastric and duodenal ulcers occurred most frequently (64 bleeds, 75.6%). Patients were more likely to have a gastrostomy (G)-tube and a Nissen fundoplication [odds ratio (OR) 3.73, 95% confidence interval (CI) 1.303-13.565] than controls. The mean time from G-tube placement to first gastrointestinal bleed was 7.01 years. The mean time from Nissen fundoplication to bleed was 7.01 years. Cases and controls had similar frequency of intake of nonsteroidal antiinflammatory drugs (NSAID) and selective serotonin reuptake inhibitors (SSRI). CONCLUSION: The incidence of gastrointestinal bleeding in the pediatric FD population was estimated to be 4.20 per 1000 person-years, 21 times higher than in the general pediatric population (0.2 per 1000 person-years). Patients with FD with a G-tube and a Nissen fundoplication had a higher risk of a subsequent gastrointestinal bleeding.

Frequency and burden of gastrointestinal symptoms in familial dysautonomia.

PURPOSE: Familial dysautonomia (FD) is a rare hereditary sensory and autonomic neuropathy (HSAN-3) that is clinically characterized by impaired pain and temperature perception and abnormal autonomic function. Patients with FD have gastrointestinal dysmotility and report a range of gastrointestinal symptoms that have yet to be systematically evaluated. The aim of this study was to establish the frequency and severity of gastrointestinal symptoms in patients with FD. METHODS: The validated National Institutes of Health Patient-Reported Outcomes Measurement Information System (PROMIS) survey questionnaire, together with additional FD-specific questions, were distributed to 202 living patients with genetically confirmed FD who had been identified from the New York University FD Patient Registry or, when relevant, to their respective caretaker. As a comparison group, we used a general US adult population for whom PROMIS scores were available (N = 71,812). RESULTS: Of the 202 questionnaires distributed, 77 (38%) were returned, of which 53% were completed by the patient. Median age of the respondents was 25 years, and 44% were male. Gastrostomy tube was the sole nutrition route for 25% of the patients, while 53% were reliant on the gastrostomy tube only for liquid intake. The prevalence of gastrointestinal symptoms was significantly higher in each of the eight domains of PROMIS in patients with FD than in the controls. Gastrointestinal symptoms as measured by raw scores on the PROMIS scale were significantly less severe in the FD patient group than in the control population in all domains with the exception of the abdominal pain domain. The surveys completed by caregivers reported the same burden of symptoms as those completed only by patients. CONCLUSION: Gastrointestinal symptoms affect nearly all patients with FD. Gastrointestinal symptoms are more prevalent in adult patients with FD than in the average US adult population but are less severe in the former.

Intervention and Mechanisms of Alanyl-glutamine for Inflammation, Nutrition, and Enteropathy: A Randomized Controlled Trial.

OBJECTIVE: Determine the minimum dosage of alanyl-glutamine (Ala-Gln) required to improve gut integrity and growth in children at risk of environmental enteropathy (EE). METHODS: This was a double-blinded randomized placebo-controlled dose-response trial. We enrolled 140 children residing in a low-income community in Fortaleza, Brazil. Participants were 2 to 60 months old and had weight-for-age (WAZ), height-for-age (HAZ), or weight-for-height (WHZ) z-scores less than -1. We randomized children to 10 days of nutritional supplementation: Ala-Gln at 3 g/day, Ala-Gln at 6 g/day, Ala-Gln at 12 g/day, or an isonitrogenous dose of glycine (Gly) placebo at 12.5 g/day. Our primary outcome was urinary lactulose-mannitol excretion testing. Secondary outcomes were anthropometry, fecal markers of inflammation, urine metabolic profiles, and malabsorption (spot fecal energy). RESULTS: Of 140 children, 103 completed 120 days of follow-up (24% dropout). In the group receiving the highest dose of Ala-Gln, we detected a modest improvement in urinary lactulose excretion from 0.19% on day 1 to 0.17% on day 10 (P = 0.05). We observed significant but transient improvements in WHZ at day 10 in 2 Ala-Gln groups, and in WHZ and WAZ in all Ala-Gln groups at day 30. We detected no effects on fecal inflammatory markers, diarrheal morbidity, or urine metabolic profiles; but did observe modest reductions in fecal energy and fecal lactoferrin in participants receiving Ala-Gln. CONCLUSIONS: Intermediate dose Ala-Gln promotes short-term improvement in gut integrity and ponderal growth in children at risk of EE. Lower doses produced improvements in ponderal growth in the absence of enhanced gut integrity.

Low-residue diet for colonoscopy in veterans: Risk factors for inadequate bowel preparation and patient satisfaction and compliance.

Bowel preparation with low-residue diet (LRD) has resulted in higher patient satisfaction and similar polyp detection rates compared to conventional clear liquid diet. However, there is limited experience with LRD in veterans, in whom conditions associated with poor bowel preparation are more prevalent than the general population. To examine risk factors associated with inadequate bowel preparation, we conducted a chart review of outpatient colonoscopies at the Manhattan VA Medical Center from February 2017 to April 2018. To examine patient satisfaction and compliance, we administered an anonymous questionnaire to patients undergoing outpatient colonoscopy from March to August 2018. Patients assessed by chart review (n = 660) were 92% male with a mean age of 64 years. An adequate Boston Bowel Preparation Scale score ≥2 in each colonic segment was achieved in 94% of procedures. Higher BMI, diabetes, prior inadequate bowel preparation, bowel preparation duration of two days, and opioid use were associated with inadequate bowel preparation on univariable analysis. On multiple logistic regression, only higher BMI remained a predictor, with every one-unit increase associated with a 6% increased odds of poor bowel preparation. Questionnaire responses showed 84% of patients were willing to repeat LRD bowel preparation, 85% found the process easy or acceptable, and 78% reported full adherence to LRD. These findings demonstrate that bowel preparation quality, patient satisfaction, and compliance were all high among veterans using LRD.

The Use of Tablet Technology by Older Adults in Health Care Settings-Is It Effective and Satisfying? A Systematic Review and Meta Analysis.

OBJECTIVES: This systematic review/meta-analysis examines the potential for older people to accept and use tablet technology in clinical settings by assessing satisfaction and effectiveness. METHODS: A comprehensive literature search was conducted of PubMed, SCOPUS, and CINAHL through March 2017. Inclusion criteria included studies with any clinical use of a tablet technology with a median patient age above 65 years. RESULTS: We included a total of 12 studies (4 randomized controlled trials, 4 cross-sectional studies, and 4 pre/post studies). Interventions included the use of tablet technology for medication self-management, post-surgery education, memory retention, cognitive rehabilitation, and exercise promotion. The use of tablet technology by older people in clinical settings was associated with high satisfaction with a pooled prevalence of satisfaction of 78%; 95% CI 27-100. We did not find evidence for effectiveness in improving clinical or behavioral outcomes. CONCLUSIONS: Older people can use and are satisfied with table technology in clinical settings. More studies are needed to evaluate the effectiveness of tablet technology at promoting health outcomes. CLINICAL IMPLICATIONS: Clinicians should be encouraged to utilize tablet technology in the care of older patients.

Parkinson's Disease and Current Treatments for Its Gastrointestinal Neurogastromotility Effects.

PURPOSE OF REVIEW: Gastrointestinal disturbances are seen in nearly all patients with Parkinson's disease and lead to impaired quality of life, affect drug pharmacodynamics, and potentially worsen patient's existing motor fluctuations, leading to further disability. Recent evidence links abnormal accumulations of α-synuclein aggregates in the periphery (gut) as seen in the cortex which causes dysfunctions impacting every level of the gastrointestinal tract from the esophagus, to the stomach, small bowel, colon, and rectum and can even predate the onset of the central neurologic disorder itself. Many treatments exist for the clinical phenotypes that result from the autonomic dysfunction and neuropathy involved in this neurodegenerative disorder. The treatments for the gut dysfunction seen in Parkinson's disease (PD) depend on the specific area of the gastrointestinal tract affected. For dysphagia, behavioral therapies with speech pathology, neuromuscular electrical stimulation, or botulinum toxin injection may be helpful. For gastroparesis, domperidone may serve as an antiemetic while also blunting the hypotensive potential of Levodopa while new treatments such as ghrelin agonists may prove beneficial to help appetite, satiety, gastric emptying in those with constipation, and even improve constipation. Antibiotics such as rifaximin with poor systemic absorption may be used to treat small bacterial overgrowth also found in those with PD while the benefits of probiotics is yet to be determined. Finally, constipation in PD can be a reflection of pelvic floor dyssynergia, slow transit constipation, or both, thus treatments targeting the specific anorectal dysfunction is necessary for better outcomes.

Parental attitudes towards influenza vaccination for children in South India.

BACKGROUND: The rate of influenza vaccination is low for children in India. The purpose of this study is to assess parental attitudes towards influenza vaccination in South India. METHODS: Participants were parents who brought their children to the Well Baby Clinic of Christian Medical College Hospital, Vellore, India for routine immunization. Participants answered questions by written survey while waiting for their children's vaccination. RESULTS: A total of 456 surveys were completed (403 parents did not opt for trivalent influenza vaccination and 53 opted for influenza vaccination). The majority (53.60%) of those parents who did not accept influenza vaccination identified the lack of a doctor's recommendation as the main reason. When asked separately, many non-acceptors (44.91%) indicated that they did not believe or were not sure that the influenza vaccine was effective. Nearly all non-acceptors (92.56%) stated that they would opt for influenza vaccination if a doctor recommended it. CONCLUSIONS: The most common reason that parents not opting for influenza vaccination for their children was the lack of recommendation by a doctor. The results of this study suggest that recommendation by a doctor is a more important factor than belief in efficacy, cost, or convenience in parental decision-making regarding childhood influenza vaccination in India, unlike the United States where parents are less likely to follow recommendations.

Diagnosing Postpartum Hemorrhage: A New Way to Assess Blood Loss in a Low-Resource Setting.

Introduction Postpartum hemorrhage (PPH) is the leading cause of maternal mortality worldwide. The largest barriers to treating PPH are symptom recognition and timely diagnosis. The SAPHE (Signaling a Postpartum Hemorrhage Emergency) Mat was constructed so that each square on the Mat absorbs up to 50 mL of blood. The objective of this study was to evaluate the correlation of visually estimated blood loss (EBL) using the SAPHE Mat with actual blood loss. Methods Thirty-six patients gave birth via vaginal delivery using the SAPHE Mat. Visual estimation of blood loss using the SAPHE Mat was calculated by multiplying the number of blood- saturated squares or partial squares by 50 mL. The visual EBL was compared with the actual blood loss calculated based on Mat weight before and after use (volume blood loss). Results Visual blood loss estimations were within 100 mL of the volume blood loss 69 % of the time and within 200 mL 97 % of the time. The mean difference between the visual EBL and volume blood loss (Mat weight change) was 80.91 mL. The Pearson correlation coefficient for visual EBL and volume blood loss was positive at 0.96 (p < 0.001). Discussion The SAPHE Mat is able to provide a visual estimate of blood loss that is highly correlated with the actual blood loss on the mat. Future studies will assess the ability to deploy the SAPHE Mat in low-resource settings as a potential guide for estimating blood loss to assist in improved management of PPH.

Increasing colonoscopies? A psychological perspective on opting in versus opting out.

OBJECTIVE: A growing strategy to enhancing healthy decision making is to modify behavioral options to an "opt out" (i.e., if you do not want to participate, you must act) as opposed to an "opt in" (i.e., if you want to participate, you must act) default system (Choi, Laibson, Madrian, & Metrick). The current study, however, proposes that this growing trend may not always be strategic and examines these default systems in colonoscopy appointment show-up rates. METHODS: Participants in an opt-in condition received instructions to call and schedule their colonoscopy appointment, whereas participants in an opt-out condition were mailed the date and time of an already scheduled appointment. We then assessed colonoscopy appointment show-up rates. RESULTS: As predicted, results revealed significant decrements in the show-up rates of those in the opt-out (63%) versus opt-in condition, 85%; χ² (1) = 5.51, p = .02. Furthermore, when looking at patients who confirmed their appointment in both conditions, only 3% of opt-in (vs. 21% of opt-outpatients) no-showed, χ² (1) = 4.51, p = .03. CONCLUSION: Although modifying the default to an opt-out system has been shown to be effective in some health care procedures, the current results reveal this system is not always effective in producing the healthiest medical behaviors. Our research, then, suggests that opt-out paradigms are not an overall panacea for increasing participation in preventive health. (PsycINFO Database Record (c) 2014 APA, all rights reserved).