Matthias Kieslich

Purpose: The impact of pediatric traumatic brain injury (pTBI) on health-related quality of life (HRQoL) in children and adolescents remains understudied. Short scales have some advantages in terms of economy and administration over longer scales, especially in younger children. The aim of the present study is to psychometrically evaluate the six-item German version of the QOLIBRI-OS-KID/ADO scale for children and adolescents. In addition, reference values from a general German pediatric population are obtained to assist clinicians and researchers in the interpretation of HRQoL after pTBI. Methods: A total of 297 individuals after TBI and 1997 from a general population sample completed the questionnaire. Reliability, validity, and comparability of the assessed construct were examined. Results: The questionnaire showed satisfactory reliability (α = 0.75 and ω = 0.81 and α = 0.85 and ω = 0.86 for the TBI and general population samples, respectively). The QOLIBRI-OS-KID/ADO was highly correlated with its long version (R² = 67%) and showed an overlap with generic HRQoL (R² = 55%) in the TBI sample. The one-dimensional factorial structure could be replicated and tested for measurement invariance between samples, indicating a comparable HRQoL construct assessment. Therefore, reference values and cut-offs indicating clinically relevant impairment could be provided using percentiles stratified by factors significantly associated with the total score in the regression analyses (i.e., age group and gender). Conclusion: In combination with the cut-offs, the QOLIBRI-OS-KID/ADO provides a cost-effective screening tool, complemented by interpretation guidelines, which may help to draw clinical conclusions and indications such as further administration of a longer version of the instrument to gain more detailed insight into impaired HRQoL domains or omission of further steps in the absence of an indication.

Until recently, no disease-specific health-related quality of life (HRQoL) questionnaire existed for pediatric traumatic brain injuries (TBIs). In this revalidation study, the psychometric properties and the validity of the 35-item QOLIBRI-KID/ADO questionnaire in its final German version were examined in 300 children and adolescents. It is the first self-reported TBI-specific tool for measuring pediatric HRQoL in individuals aged between 8 and 17 years. The six-factor model fits the data adequately. The questionnaire’s internal consistency was excellent for the total score and satisfactory to excellent for the scale scores. Intraclass correlations indicated good test–retest reliability, and the measure’s construct validity was supported by the overlap between the QOLBRI-KID/ADO and the PedsQL, which measures generic HRQoL. The discriminant validity tests showed that older children and girls reported a significantly lower HRQoL than comparison groups, and this was also true of children who were anxious or depressed, or who suffered from post-concussion symptoms, replicating the results of the questionnaire’s first developmental study. Our results suggest that the QOLIBRI-KID/ADO is a reliable and valid multidimensional tool that can be used together with the adult version in clinical contexts and research to measure disease-specific HRQoL after pediatric TBI throughout a person’s life. This may help improve care, treatment, daily functioning, and HRQoL after TBI.

Until recently, no disease-specific health-related quality of life (HRQoL) instrument existed for pediatric traumatic brain injury (TBI). In this revalidation study, the psychometric properties and the validity of the final German version of the 35-item QOLIBRI-KID/ADO questionnaire were examined in 300 children and adolescents. It is the first self-reported TBI-specific instrument for measuring pediatric HRQoL in individuals aged 8–17 years. The six-factor model fits the data adequately. Internal consistencies were excellent for the total score and satisfactory to excellent for the scale scores. Intraclass correlations indicated good test-retest reliability. Construct validity was supported by overlaps between the QOLBRI-KID/ADO and the PedsQL, which measures generic HRQoL. Discriminant validity indicated that HRQoL was significantly lower in older children and girls, and in those with anxiety, depression, and post-concussion symptoms than in comparison groups, replicating the results of the questionnaire’s first development study. Our results suggest that the QOLIBRI-KID/ADO is a valid and reliable multidimensional instrument for assessing disease-specific HRQoL after pediatric TBI in clinical and research settings, which can additionally be used across the lifespan in combination with the adult version. This may help to improve care, treatment, daily functioning and HRQoL after TBI.

The impact of pediatric traumatic brain injury (pTBI) on health-related quality of life (HRQoL) in children and adolescents remains understudied. Short scales have some advantages in terms of economy and administration over longer scales, especially in younger children. The aim of the present study is to psychometrically evaluate the six-item German version of the QOLIBRI-OS-KID/ADO scale for children and adolescents. In addition, reference values from a general German pediatric population are obtained to assist clinicians and researchers in the interpretation of HRQoL after pTBI. A total of 297 individuals after TBI and 1997 from a general population sample completed the questionnaire. Reliability, validity, and comparability of the assessed construct were examined. The questionnaire showed satisfactory reliability (α = 0.75 and ω = 0.81 and α = 0.85 and ω = 0.86 for the TBI and general population samples, respectively). The QOLIBRI-OS-KID/ADO was highly correlated with its long version (R² = 67%) and showed an overlap with disease-specific HRQoL (R² = 55%) in the TBI sample. The one-dimensional factorial structure could be replicated and tested for measurement invariance between samples, indicating a comparable HRQoL construct assessment. Therefore, reference values and cut-offs indicating clinically relevant impairment could be provided using percentiles stratified by factors significantly associated with the total score in the regression analyses (i.e., age group and gender). In combination with the cut-offs, the QOLIBRI-OS-KID/ADO provides a cost-effective screening tool, complemented by interpretation guidelines, which may help to draw clinical conclusions and indications for further diagnostics and therapy.

Background: Ataxia telangiectasia (A-T) is a rare autosomal recessively inherited neurodegenerative multisystemic disease that is characterized by cerebellar ataxia, immunodeficiency, and chromosomal instability. However, extrapyramidal movement disorders und peripheral neuropathy may also be present.[1] [2] Currently, there are no curative therapy options. Various systemic therapies, stem cell transplantation, gene therapy, or immunomodulators are being tested. The neurodegenerative process of A-T is well described.[3] However, knowledge about the affection of the peripheral nervous system is still very limited. The goal of this study was to examine patients with genetically proven A-T via nerve conduction studies (NCS) in order to assess the course of peripheral neuropathy in A-T. Methods: Study design: prospective cohort study. Patients: 43 A-T patients (39 classical, 1 patient in the conditioning phase prior to stem cell transplantation, 1 patient after allogenic stem cell transplantation, 2 variant). Age range: 1–37 years. Control Group: age- and gender-matched. Clinical Evaluation: neurostatus, ataxia scores (SARA, Klockgether, ESFAT-C). Electrophysiology: motor and sensory nerve conduction studies (median + tibial nerves). Results: In comparison to the control group decline of sensory nerve amplitudes (median + tibial nerves) could be detected as early as with 2 years old. Delayed sensory nerve conduction velocities were detected with 3 years old (tibial nerve) and with 7 years old (median nerve). Decline in motor amplitudes were detected with 5 years old (tibial nerve) and 8 years old (median nerve). Delayed motor nerve conduction velocities were detected with 6 years old (tibial nerve) and 10 years old (median nerve). Peripheral neuropathy in A-T correlates with increasing age and increasing severity of the ataxia. Conclusions: The affection of the peripheral nervous system in A-T has been described mostly in older children. However, through electrophysiological examination peripheral neuropathy can be detected at very young age. Due to lack of causal therapy conservative & supportive treatments are at the foreground currently. On the other hand, the affection of the peripheral nervous system should be taken as much into consideration as it is the case with the cerebellar, cerebral and spinal affection of A-T regarding the development of therapeutic options. Publication Date: 28 October 2021 (online) © 2021. Thieme. All rights reserved. Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany

Hintergrund: Rezidivierend kommt es weltweit, insbesondere im Sommer, zu Ausbrüchen von Enterovirusinfektionen. Die Übertragung der Enteroviren erfolgt fäkal-oral oder direkt von Mensch zu Mensch. Das gehäufte Auftreten von serösen Meningitiden ist ein Indikator für eine intensive Enteroviruszirkulation. Die häufigsten Erreger sind Coxsackie-B- und ECHO-Viren (Enteric Cytopathogenic Human Orphan viruses). Letztere umfassen 34 Serotypen, welche vor allem grippale Erkrankungen („Sommergrippe”) aber auch seröse Meningitiden und Meningoenzephalitiden bei Kindern und Jugendlichen verursachen können. Der Typ 30 ist in Deutschland schon seit Jahrzehnten prävalent und führte im Sommer 1997 im Rhein-Main-Gebiet zu einem Ausbruch. Insgesamt wurden in 5 Monaten 63 Infektionen bei Kindern unter 16 Jahren gemeldet. Patienten und Methodik: In der Universitätskinderklinik Frankfurt am Main wurden in diesem Zeitraum 18 Kinder mit nachgewiesenen zentralen Enterovirusinfektionen behandelt und standardisiert infektiologisch untersucht (Liquor, Stuhl, Serum). Ferner wurden Risikofaktoren, klinische Symptomatologie, Entzündungsparameter, neurophysiologische Befunde (Elektroenzephalographie und evozierte Potenziale) sowie der klinische Verlauf analysiert. Ergebnisse: Die erkrankten Kinder waren zwischen 3 und 11 Jahre alt. Klinisch imponierten Kopfschmerzen, Nausea, Erbrechen, Meningismus und Krampfanfälle bei Fieber. In den meisten Fällen konnte ECHO-30-Virus nachgewiesen werden. Die Virus-Isolation aus Fäzes und Liquor sowie die entsprechenden Polymerasekettenreaktionen (PCR) zeigten sich den serologischen Methoden deutlich überlegen. Die Blutsenkungsgeschwindigkeit (BSG) zeigte deutlichere Veränderungen als die Werte des C-reaktiven Proteins (CRP) und die Leukozytenzahlen im Blut. Die Liquorpleozytosen zeigten eine hohe Varianz. Der klinische Verlauf gestaltete sich in den meisten Fällen unkompliziert, die Prognose war günstig. Schlussfolgerung: Zum Nachweis einer zerebralen Enterovirusinfektion ist die Virusisolation aus Fäzes und Liquor am Erfolg versprechendsten. Der klinische Verlauf und die Prognose sind in der Regel als günstig einzustufen. Komplizierend kann sich jedoch eine Enzephalitis entwickeln. Background: Enterovirus infections are among the most common causes of aseptic meningitis. Worldwide there are reports about recurring outbreaks, especially during the summer. They are favoured by conditions of bad hygiene and contaminated water, transmission is predominantly through the faeco-oral route or by droplet infection. The most common species are Coxsackie B and ECHO (Enteric Cytopathogenic Human Orphan) virus. ECHO viruses have a worldwide distribution and usually occur as “summer flu” or aseptic meningitis and meningoencephalitis in toddlers and infants. Type 30 caused an outbreak of aseptic meningitis in the Rhein-Main region in summer 1997. During five months 63 children younger than 16 years were reported. Patients and Methods: During this outbreak 18 children with prooved enterovirus infections were treated at the Frankfurt/Main University Children's Hospital. Standardized infectiological diagnostic procedures were performed and risc factors, clinical symptoms, inflammatory marker, neurophysiological findings (electroencephalography, evoked potentials) and outcome were assessed. Results: The affected children were between 3 and 11 years old. Clinical symptoms were cephalgia, nausea, vomiting, meningism and seizures with fever. Virus isolation from faecal and cerebrospinal fluid (CSF) samples and the use of polymerase chain reaction (PCR) was superior to serological methods. Erythrocyte sedimentation rates showed more significant increase than C-reactive protein (CRP) and blood leukocytes. CSF pleocytosis showed high variation. Clinical course as well as prognosis and outcome were favourable. Conclusion: Virusisolation in stool and CSF is most promising in the diagnostic of cerebral enterovirus infections. Usually the outcome is favourable, encephalitis can occur as serious complication.