Too much of too little: xylitol, an unusual trigger of a chronic metabolic hyperchloremic acidosis

Homeopathic globules are frequently used in children as a first-line treatment. Most of these globules are coated with sugar substitutes like xylitol; these substitutes are known for their laxative effect. Our patient shows that consumption of globules coated with xylitol does not have only laxative effects. It may cause indeed considerable weight loss and life-threatening enteral bicarbonate loss by diarrhea when overdosed in an infan

Impact of Brain Malformations on Neurodevelopmental Outcome in Children with a History of Prenatal Surgery for Open Spina Bifida

INTRODUCTION This retrospective study investigates brain malformations and their impact on neurodevelopmental outcome in children after prenatal surgery for spina bifida (SB). METHODS Sixty-one patients were included. On neonatal MRI, SB-associated brain malformations were assessed. Ventricular size, ventriculo-peritoneal shunt (VPS), and endoscopic third ventriculostomy (ETV) were also documented. Neurodevelopment was assessed with the Bayley-III and correlated with brain malformations, ventricular size, and VPS/ETV placement. RESULTS Chiari II malformation was detected in all patients. Corpus callosum (CC) abnormality was noted in 40%, heterotopies in 35%, and cerebellar parenchymal defects in 11%. 96% had ventriculomegaly; in 46%, VPS/ETV was performed. Cognitive and language testing yielded results in the low-average range (Bayley-III: Cognitive Composite Score 93.6, Language Composite Score 89.7), motor testing was below average (Motor Composite Score 77.4). CC abnormalities, heterotopies, and cerebellar defects were not associated with poorer Bayley-III scores, whereas patients with severe ventriculomegaly performed poorer in all subtests, significantly so for the language composite score. Patients requiring intervention for hydrocephalus had significantly lower scores in motor testing. DISCUSSION/CONCLUSION Additional brain malformations in open SB do not seem to have an impact on cognitive function at 2 years of age. Severe ventriculomegaly is a risk factor for poorer cognitive outcome; hydrocephalus surgery adds an additional risk for delayed motor function

Needs and Research Priorities for Young People with Spinal Cord Lesion or Spina Bifida and Their Caregivers: A National Survey in Switzerland within the PEPSCI Collaboration.

The aim of this study was to describe the needs and research priorities of Swiss children/adolescents and young adults (from here, "young people") with spinal cord injury/disorder (SCI/D) or spina bifida (SB) and their parents in the health and life domains as part of the international Pan-European Pediatric Spinal Cord Injury (PEPSCI) collaboration. Surveys included queries about the satisfaction, importance, research priorities, quality of life (QoL), and characteristics of the young people. Fifty-three surveys with corresponding parent-proxy reports were collected between April and November 2019. The self-report QoL sum scores from young people with SCI/D and SB were 77% and 73%, respectively. Parent-proxy report QoL sum scores were lower, with 70% scores for parents of young people with SCI/D and 64% scores for parents of young people with SB. "Having fun", "relation to family members", and "physical functioning" were found to be highly important for all young people. "Physical functioning", "prevention of pressure injuries", "general health", and "bowel management" received the highest scores for research priority in at least one of the subgroups. As parents tend to underestimate the QoL of their children and young people prioritized research topics differently, both young peoples' and caregivers' perspectives should be included in the selection of research topics

Exile Vol. XXXIX No. 1

Title Page by Ellen Gurley \u2793 i Epigraph by Ezra Poind ii Table of Contents iii-iv Remaining a Soldier by Kristin Kruse \u2793 1-2 Vietnam War Memorial by Brooke MacKaye 3 We both ride in back by Chris Macaluso \u2793 4 Artwork by Jamie Oliver \u2794 5 Liberal Dirge #1 by Charis Brummitt \u2796 6-7 Artwork (anonymous) 7 Two ex-lovers and a dirty glass door by Chris Macaluso \u2793 8 The Salt of the Air by Kristen Padden \u2793 9-12 Artwork (anonymous) 13 Artwork by Ellen Gurley \u2793 14 Sun-Child by Jen Rudgers \u2796 15 Crazy Horse by Kevin Nix \u2794 16 The Fall of the Western Field by Rich Croft \u2793 17 In the Closet by Beth Widmaier \u2795 18 Winter Strawberries by Katy Rudder \u2793 19 Still Life (anonymous) 19 For This and Much Beyond This Poem by Matt Wanat \u2795 20-21 Artwork by Peggy Ryan \u2793 22 The Cycle Repeats: Apathy by Ishak Kang \u2793 23 The Judge by Ellen Gurley \u2793 24 Pear Colored by Erin Dempsey \u2793 25-26 4-Square by Trey Dunham \u2794 27 Artwork by Jamie Oliver \u2794 28 Ink & Heroine by Rich Croft \u2793 29 Figments by Craig Bowers \u2793 30-31 Malfi Coast (anonymous) 31 Suzanne (anonymous) 32 Hey Stella by Carey Chistie \u2795 33 Turning Leaves by Erin Lott \u2796 34-35 Reclining Nude (anonymous) 35 Blazon by Matt Wanat \u2795 36-37 Artwork by Holly Aikens \u2793 38 Awake by A. Fair \u2796 39 Dell the Barber by Kevin Nix \u2795 40 Artwork by Holly Aikens \u2793 40 Tree House by Katy Rudder \u2793 41-46 Jailbait by Ellison J. Stind \u2795 47 Mother by Charis Brummitt \u2796 48-49 Artwork by Bess Hammer \u2795 49 Private Origami by Trey Dunham \u2794 50 Among the Tendrils of Sleep by J. Trevett Allen \u2795 51 Poet of the Unforgiven by Carey Christie \u2795 52 Stuntman Steve by Andrew Zobay \u2793 53 sculpture by Lily Streett \u2794 53 Wonderings of an Adopted Son by Andy Heckert \u2793 54-55 Artwork by Holly Aikens \u2793 55 Odd Binge by C. N. Polumbus \u2793 56-57 Artwork by Holly Aikens \u2793 57 Artwork by Peggy Ryan \u2793; untitled by Jennifer Wendell \u2794 (superimposed) 58 Shadows of Pearl by Travis Brady \u2793 59-60 October/Rt. 161 by Annette Gallagher 61 Artwork by Jamie Oliver \u2794 61 The Influx by Craig Bowers \u2793 62 Artwork by Michael Norpell \u2794 63 editorial board 64 Editorial decision is shared equally among the Editorial Board. -64 Cover: Jamie Oliver -64 NOTE: With the exeption of Malfi Coast , all artwork listed as anonymous in the published table of contents appears to be signed by Ellen Gurley. 37th Yea

Does amplitude-integrated electroencephalogram background pattern correlate with cerebral injury in neonates with hypoxic-ischaemic encephalopathy?

AIMS To determine the correlation between amplitude-integrated electroencephalogram (aEEG) background pattern and cerebral magnetic resonance imaging (MRI) in infants with hypoxic-ischaemic encephalopathy (HIE) and to examine whether the correlation changes with therapeutic hypothermia. METHODS We included 38 term-born infants with HIE of whom 17 were cooled. All were continuously monitored with aEEG. Background pattern was scored at the beginning and the end of the recording. Cerebral MRI was obtained on median day 5 (2-11 days). Abnormalities were classified using a predefined scoring system for basal ganglia, watershed and overall injury, and then grouped into mild-moderate and severe. RESULTS Abnormal aEEG background pattern correlated with more severe cerebral injury on MRI in the non-cooled infants (P < 0.01). In addition, cooled infants had less severe cerebral injury than non-cooled infants, in particular on T2-weighted images (watershed P = 0.04 and total injury score = 0.07). CONCLUSIONS Abnormal aEEG background pattern is predictive of abnormal MRI, but therapeutic hypothermia seems to reduce this association. Thus, when cooling is applied in a clinical setting, the predictive value of aEEG may be limited

Acid-base parameters for predicting magnetic resonance imaging measures of neurologic outcome after perinatal hypoxia-ischemia: is the strong ion gap superior to base excess and lactate?

We conducted this study to compare the strong ion gap (SIG) with base excess (BE) and lactate for predicting neurologic outcome measured by magnetic resonance imaging (MRI) in newborns with hypoxic-ischemic encephalopathy (HIE). In a retrospective cohort of 39 newborns with HIE treated with whole-body surface cooling (n = 17) and no cooling (n = 22), we measured blood SIG, BE, and lactate at 4, 24, and 48 hours after birth, and determined cerebral injury severity by T1-, T2-, and diffusion-weighted MRI scores at age 5 days. Lower SIG levels correlated with better neurologic outcome. The highest correlation coefficient (0.63) was in the "no cooling" subcohort between diffusion-weighted scores and SIG levels at 24 hours; the latter also had the highest area under the receiver operating characteristic curve (AUC), 0.90, with positive and negative predictive values of 84 and 90%. SIG outperformed lactate in the "no cooling" subcohort, and vice-versa in the "cooling" subcohort. All BE AUCs were <0.6. Overall, the SIG is similar to lactate as a prognostic parameter. BE levels at 4, 24, and 48 hours after birth do not predict neurologic outcome. While not displacing lactate the SIG is an additional prognostic parameter for newborns in the first 2 days after hypoxia-ischemia

Verbesserung der Sprechstunde mit chronisch kranken Kindern: Gesundheits-, Funktions- und Wohlergehensampel

Improving Communication Quality Caring for Children with Chronic Conditions: Health, Functioning and Wellbeing Traffic-Light Tool Abstract. Effective patient-doctor communication is a crucial aspect while caring for children with chronic conditions or disabilities. The Health, Functioning and Wellbeing Summary Traffic Light has been developed as a communication tool especially for these patients. In a two-month pilot phase the German version was evaluated by parents and physicians in a rehabilitation out-patient clinic setting. 71 % (n = 35/49) returned the evaluation form. The traffic-light tool was rated positive by 80 % of participants and physicians. It can be recommended as a useful tool for improved communication. The simple language version as well as translations into other languages and the use of a mobile App will facilitate its use. Its use is not limited to paediatrics and could be adapted for other disciplines

Early childhood neurodevelopmental outcome after open prenatal spina bifida aperta repair

Aim: To investigate neurodevelopmental outcome of children with open prenatal spina bifida aperta (SBA) repair. Method: Prenatal SBA repair was performed in 130 fetuses at the Zurich Center between 2010 and 2019. Seventy-seven children underwent 1 year assessment with the Griffiths Mental Developmental Scales (Griffiths) and 65 with the Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-III) at 2 years. Anatomical and functional level and ambulation status were assessed. Descriptive statistics and multiple linear regression analyses for risk factors were performed. Results: The Bayley-III cognition composite score in children with prenatal SBA repair was within normal limits but lower compared to population norms (mean=95.15, SD=14.683 vs norm=100, SD=15, p=0.01). Fine motor development (mean=9.58, SD=2.744, p=0.227) was typical while gross motor development was lower than the norm (mean=3.02, SD=2.758 vs norm=10, SD=3, p<0.001). Griffiths developmental quotient subscales correlated significantly with corresponding Bayley-III scores (all p<0.001, r=0.519-0.594). At 2 years, 50.8% could walk. Interpretation: Children with non-trial open prenatal SBA repair show favourable cognitive outcome in the low-average range at 1 and 2 years of age. While gross motor function remained delayed, fine motor function was age appropriate. The correlation between Griffiths and Bayley-III allows a prediction about neurodevelopmental outcome at the age of 1 year. What this paper adds Children with non-trial open prenatal spina bifida repair show favourable cognitive outcome. Gross motor function remains impaired, while fine motor function is age appropriate. At 2 years of age, 50.8% of children were walking. Neurodevelopmental testing correlated between 1 (Griffiths Mental Developmental Scales) and 2 (Bayley Scales of Infant and Toddler Development, Third Edition) years

Too much of too little: xylitol, an unusual trigger of a chronic metabolic hyperchloremic acidosis

Homeopathic globules are frequently used in children as a first-line treatment. Most of these globules are coated with sugar substitutes like xylitol; these substitutes are known for their laxative effect. Our patient shows that consumption of globules coated with xylitol does not have only laxative effects. It may cause indeed considerable weight loss and life-threatening enteral bicarbonate loss by diarrhea when overdosed in an infant