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Objectives: The success of cochlear implantation in postlingually deafened adults is usually measured by puretone and speech audiometry. However, the WHO states in the ICF that rehabilitation should also be assessed according to individual communication skills, social participation and quality of life. We developed and implemented a questionnaire that focuses on everyday communication and allows comparison between people with normal and impaired hearing. A parallel version for assessment of the CI-users' communication by their significant other was also developed. Additionally, we assessed social participation and quality of life of CI-users before implantation and with 4 months CI experience Sources and methods: In a prospective pilot study, five post-lingually and progressively deafened participants (55-80 yrs) and their significant others answered questionnaires concerning communication behaviour (questionnaire developed by the authors), social and emotional handicap (HHIA, HHIE, HII-SOP) [1-3], and quality of life (AQoL-6D) [4] at two timepoints: before implantation and with 4 months CI experience. Hearing ability was measured using pure-tone and speech audiometry and the SSQ [5]. Five matched normal-hearing participants served as controls. Results: Participants with 4 months CI experience reported fewer misunderstandings, higher communicative success and less communicative effort than before implantation, though they did not reach the level of normal-hearing controls on these measures. CI users still often pretend that they have understood spoken communication and are still dependent on their significant other. They wish for further communicative success and greater independence. Conclusion: Cochlear implantation alone does not lead to normal communication behaviour and social participation. These issues and the role of the significant other should also be focused on in rehabilitation alongside auditory training. Apart from the AQoL-6D the questionnaires used in this study proved to be suitable instruments for documenting the course of rehabilitation. [ABSTRACT FROM AUTHOR]

Background: Dialysis is lifesaving for acute kidney injury (AKI), but access is poor in less resourced settings. A "peritoneal dialysis (PD) first" policy for paediatric AKI is more feasible than haemodialysis in low-resource settings., Methods: Retrospective review of modalities and outcomes of children dialysed acutely at Red Cross War Memorial Children's Hospital between 1998 and 2020., Results: Of the 593 children with AKI who received dialysis, 463 (78.1%) received PD first. Median age was 9.0 (range 0.03-219.3; IQR 13.0-69.6) months; 57.6% were < 1 year old. Weights ranged from 0.9 to 2.0 kg (median 7.0 kg, IQR 3.0-16.0 kg); 38.6% were < 5 kg. PD was used more in younger children compared to extracorporeal dialysis (ECD), with median ages 6.4 (IQR 0.9-30.4) vs. 73.9 (IQR 17.5-113.9) months, respectively (p = 0.001). PD was performed with Seldinger soft catheters (n = 480/578, 83%), predominantly inserted by paediatricians at the bedside (n = 412/490, 84.1%). Complications occurred in 127/560 (22.7%) children receiving PD. Overall, 314/542 (57.8%) children survived. Survival was significantly lower in neonates (< 1 month old, 47.5%) and infants (1-12 months old, 49.2%) compared with older children (> 1 year old, 70.4%, p < 0.0001). Survival was superior in the ECD (75.4%) than in the PD group (55.6%, p = 0.002)., Conclusions: "PD First for Paediatric AKI" is a valuable therapeutic approach for children with AKI. It is feasible in low-resourced settings where bedside PD catheter insertion can be safely taught and is an acceptable dialysis modality, especially in settings where children with AKI would otherwise not survive., (© 2024. The Author(s).)

Confirming the aetiology of pleural effusion in children may be difficult in TB-endemic settings. We investigated the role of polymerase chain reaction (PCR) and routine biochemical tests in discriminating pleural effusion caused by bacteria from other aetiologies.This is a cross-sectional post-hoc analysis among children with pleural effusion in a tertiary hospital in South Africa, incorporating new data from PCR testing of stored pleural fluid. Aetiological classification was defined by microbiological confirmation.Ninety-one children were enrolled; the median age 31 months (IQR 12-102). The aetiology of pleural effusion was 40% (36/91) bacteria, 11% (10/91) TB, 3% (3/91) viruses, 11% (10/91) polymicrobial and 35% (32/91) had no pathogen identified. The most common pathogen was Staphylococcus aureus (27/91, 30%) with similar yields on culture and PCR, followed by Streptococcus pneumoniae (12/91, 13%), detected more commonly by PCR. PCR reduced the number of children with unconfirmed aetiologies from 48 to 32. Characteristics of children with no pathogen most resembled those with TB. Pleural fluid lactate dehydrogenase ≥1,716 U/L best discriminated bacterial pleural effusion from other aetiologies (sensitivity of 86%; specificity 95%).PCR improved detection of pathogens and reduced number of children with unconfirmed aetiologies in presumed exudative pleural effusion..

Introduction: Anaphylaxis is a severe, life-threatening generalized hypersensitivity reaction. While guidelines to reduce the morbidity, risk, and mortality of anaphylaxis are widely available, adherence to these is often suboptimal. We aimed to audit paediatric anaphylaxis at a South African tertiary allergy referral centre, comparing our data to those of the large Network of Severe Allergic Reactions (NORA) registry., Methods: Children treated for severe allergic reactions between January 2014 and August 2016 were identified for screening using ICD-10 coding of all admissions and discharges, pharmacy records of adrenaline autoinjector dispensing, and additional referrals from the allergy department to the study. Screened participants not meeting the inclusion criteria after preliminary questioning and/or folder review were excluded. Data were collected via a standardized questionnaire using direct interviews, and captured on a local web-based registry., Results: Of the 156 episodes analysed, >40% were graded as severe and nearly two-thirds of patients were seen for a recurrent episode. Males, younger children, and individuals of mixed-race ethnicity were more frequently affected. Skin and mucosa were most commonly involved, followed by respiratory and gastrointestinal involvement; cardiovascular and other systemic involvement occurred infrequently. Specific IgE assay was the most frequently requested test. Food-related triggers (peanut, hen's egg, fish, cashew nuts and cows' milk) predominated and decreased with age. Anaphylaxis was strongly correlated with atopic conditions. While prophylactic measures were almost universally instituted, adrenaline was rarely used, by both lay persons and healthcare professionals. Hospital admissions were infrequent, and no deaths were recorded., Conclusion: Management of anaphylaxis can be improved. Specifically, the use of adrenaline prior to hospital arrival remains suboptimal. Ongoing education and training of patients, parents, teachers, and healthcare workers is identified as an area requiring intensification., Competing Interests: None to declare., (© 2022 The Authors.)

One risk factor of neovascular age-related macular degeneration is systemic hypertension; hypertension is mainly caused by extracellular hyperosmolarity after consumption of dietary salt. In retinal pigment epithelial (RPE) cells, high extracellular osmolarity induces vascular endothelial growth factor (VEGF)-A (Hollborn et al. in Mol Vis 21:360-377, 2015). The aim of the present study was to determine whether extracellular hyperosmolarity and chemical hypoxia trigger the expression of further VEGF family members including placental growth factor (PlGF) in human RPE cells. Hyperosmotic media were made up by addition of 100 mM NaCl or sucrose. Chemical hypoxia was induced by CoCl2. Gene expression was quantified by real-time RT-PCR, and secretion of PlGF-2 was investigated with ELISA. Nuclear factor of activated T cell 5 (NFAT5) was depleted using siRNA. Extracellular hyperosmolarity triggered expression of VEGF-A, VEGF-D, and PlGF genes, and secretion of PlGF-2. Hypoosmolarity decreased PlGF gene expression. Hypoxia induced expression of VEGF-A, VEGF-B, VEGF-D, and PlGF genes. Extracellular hyperosmolarity and hypoxia produced additive PlGF gene expression. Both hyperosmolarity and hypoxia induced expression of KDR and FLT-4 receptor genes, while hyperosmolarity caused neuropilin-2 and hypoxia neuropilin-1 gene expression. The hyperosmotic, but not the hypoxic, PlGF gene expression was in part mediated by NFAT5. The expression of PlGF in RPE cells depends on the extracellular osmolarity. The data suggest that high consumption of dietary salt may exacerbate the angiogenic response of RPE cells in the hypoxic retina via transcriptional activation of various VEGF family member genes.

Background: Parents of newborns with hearing loss (HL) identified by Universal Newborn Hearing Screening (UNHS) programmes wish for educational support soon after confirmation and for contact with other affected families. Besides pedaudiological care, a high level of family involvement and an early start of educational intervention are the best predictors for successful oral language development in children with HL. The implementation of UNHS has made it necessary to adapt existing intervention concepts for families of children with HL to the needs of preverbal infants. In particular, responsiveness has proven to be a crucial skill of intuitive parental behaviour in early communication between parents and their child. Since infants with HL are being fitted earlier with hearing devices, their chances of learning oral language naturally in daily communication with family members have noticeably improved., Objectives: The Muenster Parental Programme (MPP) aims at empowering parents in communicating with their preverbal child with HL and in (re-)building confidence in their own parental resources. Additionally, it supplies specific information about auditory and language development and enables exchange with other affected parents shortly after the diagnosis., Concept: The MPP is a responsive parenting intervention specific to the needs of parents of infants with HL identified by UNHS or through other indices and testing within the first 18 months of life. It is based on the communication-oriented Natural Auditory Oral Approach and trains parental responsiveness to preverbal (3-18 months) infants with HL. The MPP has been developed for groups of 4-6 families and comprises six group sessions (without infants), two single training sessions with video feedback, and two individual counselling sessions. At the age of 24-30 months, an individual refresher training session is offered to the parents for adapting their responsiveness to the current verbal level of the child via dialogic book reading. The programme also benefits parents of paediatric cochlear implant (CI) candidates preimplantation and postimplantation., Conclusions: The MPP is evidence-based (see Glanemann et al., this volume) and meets the current need for effective family-centred educational intervention after UNHS., (Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.)

Objective: With the implementation of the Universal Newborn Hearing Screening (UNHS), the age of diagnosis of children with hearing loss (HL) has been steadily declining in the past years. Consequently, there is a need for early educational intervention methods that are suitable for infants at the preverbal level. To meet this need we have developed and evaluated the Muenster Parental Programme (MPP), a responsive parenting programme for parents of children with HL aged 3-18 months. It aims at enhancing the parents' communicative skills towards their child., Methods: The MPP is introduced following confirmation of a HL. Flanked by two individual counselling sessions, the programme comprises six group sessions and two single training sessions with video feedback. The focus of the programme lies in enhancing parents' responsive behaviour and in reducing inappropriate initiative behaviour. The present study involved 29 parents of 24 children aged 6.6 (mean, range: 3-12) months at the outset of the MPP. The children's degree of HL ranged from moderate to profound. Parents of children with unilateral HL and/or risk for an additional developmental delay were included. The prospective study compared parent communication skills of a trained (N = 15) versus a control group (N = 14) before and after the MPP. For this purpose, instances of responsive behaviour to the signals of the child and total time of initiative behaviour within a 4-min video-sample were measured before and after completion of the study in both groups., Results: Trained parents could enhance their responsiveness to vocal and preverbal signals of the child (Wilcoxon test, p = .002) and also their responsiveness to non-verbal signals (Wilcoxon test, p < .001). Moreover, parents reduced their inappropriate initiative behaviour (related t-test, p < .001). Pre-post comparisons in the control group were non-significant., Conclusions: The increased parental responsiveness to infants with HL is of great importance as these early behaviours underlie later acquisition of speech, language, hearing and social communication skills. The MPP constitutes the first evaluated group concept for parents of infants with HL in the German-speaking countries and equally meets the needs of parents and professionals., (Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.)

Rifampin-based tuberculosis treatment can cause subtherapeutic concentrations of protease inhibitors and virologic failure in children receiving antiretroviral therapy. Among 217 children on antiretroviral therapy, tuberculosis cotreatment (in 78) was associated with virologic failure. Ritonavir-based single protease inhibitor antiretroviral therapy regimen predicted virologic failure (adjusted odds ratio 3.7, 95% confidence interval 1.5-8.9, P = 0.004) on multivariate analysis.

Background: The parent questionnaire ELFRA-2 is considered a valid tool for early detection of delayed language development in 2-year-old children. Applicability for children treated with cochlear implants (CI) is to be investigated., Methods: By means of the ELFRA-2 we documented longitudinally for up to 24 months post implantation language development in 27 children treated before 3 years of age. The critical developmental criteria (related to age) were applied to CI children (related to duration of CI use) and gender-related normative data were taken as a reference., Results: Only two boys were identified as showing a language delay after 2 years of CI use. However, using normative data 11-44% of the children performed below average. Development in girls was faster than in boys. The influence of preoperative hearing experience declined over time., Conclusion: The critical developmental criteria of ELFRA-2 have proved to be unreliable for the identification of varying development after CI. Modified and gender-related evaluation is necessary.

Background: Despite the fact that literacy acquisition in hearing impaired children is frequently hampered, reading and writing competences continue not to be regularly evaluated and documented in children fitted with cochlear implants (CI). In this 2-year longitudinal study literacy acquisition in children fitted with CI was investigated., Patients and Methods: In total, 18 pre- and primary school children fitted with CI who had suffered prelingual deafness were examined. Subjects' ages at CI fitting ranged from 0.9 to 5.9 years; they were raised orally and monolingual German and showed normal intellectual achievement. Familial risk of developing dyslexia was ruled out. To assess subjects' literacy acquisition precursor and partial abilities in reading and writing according to dual route and developmental models were examined three times within 2 years. Precursor abilities included development of vocabulary and phonological awareness. Partial abilities were mastery in sublexical and lexical word processing in reading and writing as well as auditory and visual working memory., Results: Subjects showed a broad range in performance regarding vocabulary development as well as literacy. Discrepant results in terms of age equivalent visual and underachievement in auditory working memory as well as good achievement in implicit phonological awareness and weakness in explicit demands on phoneme analysis and manipulation of phonemes can be described. Indications were that subjects tended towards lip reading the instructor's item presentation. Performance in the administered writing test reveals a preference for lexical word processing, whereas sublexical word processing seems to make relatively higher demands on subjects., Conclusions: Easier processing of visual information in partial and precursor abilities are consistent with a tendency to prefer a visual-lexical processing strategy. The presented study stresses the importance of generally assessing reading and writing skills when evaluating language development in children supplied with cochlear implants. Partial and precursor abilities should be included in order to identify any delay in development promptly. Thus, individual qualitative characteristics can be addressed in therapeutic intervention.

Our previous work showed a diminished cerebral blood flow (CBF) response to changes in Pa(CO(2)) in congestive heart failure patients with central sleep apnea compared with those without apnea. Since the regulation of CBF serves to minimize oscillations in H(+) and Pco(2) at the site of the central chemoreceptors, it may play an important role in maintaining breathing stability. We hypothesized that an attenuated cerebrovascular reactivity to changes in Pa(CO(2)) would narrow the difference between the eupneic Pa(CO(2)) and the apneic threshold Pa(CO(2)) (DeltaPa(CO(2))), known as the CO(2) reserve, thereby making the subjects more susceptible to apnea. Accordingly, in seven normal subjects, we used indomethacin (Indo; 100 mg by mouth) sufficient to reduce the CBF response to CO(2) by approximately 25% below control. The CO(2) reserve was estimated during non-rapid eye movement (NREM) sleep. The apnea threshold was determined, both with and without Indo, in NREM sleep, in a random order using a ventilator in pressure support mode to gradually reduce Pa(CO(2)) until apnea occurred. results: Indo significantly reduced the CO(2) reserve required to produce apnea from 6.3 +/- 0.5 to 4.4 +/- 0.7 mmHg (P = 0.01) and increased the slope of the ventilation decrease in response to hypocapnic inhibition below eupnea (control vs. Indo: 1.06 +/- 0.10 vs. 1.61 +/- 0.27 l x min(-1) x mmHg(-1), P < 0.05). We conclude that reductions in the normal cerebral vascular response to hypocapnia will increase the susceptibility to apneas and breathing instability during sleep.

Background: Communication disorders are not taught as a self-contained topic in medical education, despite their high incidence and the similarities in the way in which they present clinically., Aims: This article describes the development of an elective subject 'Phoniatrics and Pediatric Audiology' covering five topics: hearing, language, and voice as well as psychometric tests and swallowing, with the objective of teaching a basic knowledge of the anatomy, physiology, and pathophysiology of the hearing and language system as well as symptoms, diagnostics, examination, and therapy of communication disorders. It contains theoretical background, practical exercises, and demonstrations of patient examinations., Methods: After initial modifications to the course, a statistical evaluation of the last two half-years in 2006 was performed., Results: The majority of students believe that the subject will be useful in their subsequent studies (94.2%) and medical practice (51.9%). All students affirmed that their expectations had been met by the course and they would choose it again., Conclusions: Uniting several communication disorders within a self-contained topic provides the opportunity to understand pathophysiological principles, similarities, and differences between normal and impaired function of the hearing and language system and voice production. In the authors' opinion, it is a reasonable inclusion in medical training recognizing the importance of communication in today's service society.

An important determinant of [H(+)] in the environment of the central chemoreceptors is cerebral blood flow. Accordingly we hypothesized that a reduction of brain perfusion or a reduced cerebrovascular reactivity to CO(2) would lead to hyperventilation and an increased ventilatory responsiveness to CO(2). We used oral indomethacin to reduce the cerebrovascular reactivity to CO(2) and tested the steady-state hypercapnic ventilatory response to CO(2) in nine normal awake human subjects under normoxia and hyperoxia (50% O(2)). Ninety minutes after indomethacin ingestion, cerebral blood flow velocity (CBFV) in the middle cerebral artery decreased to 77 +/- 5% of the initial value and the average slope of CBFV response to hypercapnia was reduced to 31% of control in normoxia (1.92 versus 0.59 cm(-1) s(-1) mmHg(-1), P < 0.05) and 37% of control in hyperoxia (1.58 versus 0.59 cm(-1) s(-1) mmHg(-1), P < 0.05). Concomitantly, indomethacin administration also caused 40-60% increases in the slope of the mean ventilatory response to CO(2) in both normoxia (1.27 +/- 0.31 versus 1.76 +/- 0.37 l min(-1) mmHg(-1), P < 0.05) and hyperoxia (1.08 +/- 0.22 versus 1.79 +/- 0.37 l min(-1) mmHg(-1), P < 0.05). These correlative findings are consistent with the conclusion that cerebrovascular responsiveness to CO(2) is an important determinant of eupnoeic ventilation and of hypercapnic ventilatory responsiveness in humans, primarily via its effects at the level of the central chemoreceptors.

Background: Although most hearing-impaired children lag behind normally hearing children in literacy acquisition, this aspect has hardly been addressed in the evaluation of language acquisition after cochlear implantation. The present study investigated written language abilities in 8 school-age children with cochlear implants. Neurolinguistic dual-route-models of written language processing indicate that literacy acquisition leads to the establishment of two distinct reading and writing strategies: a lexical one for the quick processing of known words and a sublexical one for decoding unfamiliar words or nonwords letter by letter., Patients: 8 school-aged children were investigated, a very heterogeneous group concerning age of onset of hearing impairment, educational placement, and competences in sign language. However, this range is typical of the group of CI-children., Methods: The aim was to investigate if children with cochlear implants are able to establish both strategies or if they need to find a differential and individual access to written language. Performance within the Salzburger Lese-Rechtschreib-Test was evaluated. Individual performance of each subject was analysed., Results: Performance varied substantially ranging from only rudimentary spoken and written language abilities in two children to age-equivalent performance in three of them. Severe qualitative differences in written language processing were shown in the remaining three subjects. Suggestions for remediation were made and a re-test was carried out after 12 months. Their individual profiles of performance are described in detail., Conclusions: The present study stresses the importance of a thorough investigation of written language performance in the evaluation of language acquisition after cochlear implantation. The results draw a very heterogeneous picture of performance. Model-oriented testing and analysis of performance prove to be sensible in at least a subpopulation of children with cochlear implants. Based on a better understanding of their acquired word-processing strategies, remediation programs meeting the needs of each individual child can be derived.

We measured ventilation, arterial O2 saturation, end-tidal CO2 (PET,CO2), blood pressure (intra-arterial catheter or photoelectric plethysmograph), and flow velocity in the middle cerebral artery (CFV) (pulsed Doppler ultrasound) in 17 healthy awake subjects while they performed 20 s breath holds under control conditions and during ganglionic blockade (intravenous trimethaphan, 4.4 +/- 1.1 mg min-1 (mean +/- S.D.)). Under control conditions, breath holding caused increases in PET,CO2 (7 +/- 1 mmHg) and in mean arterial pressure (MAP) (15 +/- 2 mmHg). A transient hyperventilation (PET,CO2 -7 +/- 1 mmHg vs. baseline) occurred post-apnoea. CFV increased during apnoeas (by 42 +/- 3 %) and decreased below baseline (by 20 +/- 2 %) during post-apnoea hyperventilation. In the post-apnoea recovery period, CFV returned to baseline in 45 +/- 4 s. The post-apnoea decrease in CFV did not occur when hyperventilation was prevented. During ganglionic blockade, which abolished the increase in MAP, apnoea-induced increases in CFV were partially attenuated (by 26 +/- 2 %). Increases in PET,CO2 and decreases in oxyhaemoglobin saturation (Sa,O2) (by 2 +/- 1 %) during breath holds were identical in the intact and blocked conditions. Ganglionic blockade had no effect on the slope of the CFV response to hypocapnia but it reduced the CFV response to hypercapnia (by 17 +/- 5 %). We attribute this effect to abolition of the hypercapnia-induced increase in MAP. Peak increases in CFV during 20 s Mueller manoeuvres (40 +/- 3 %) were the same as control breath holds, despite a 15 mmHg initial, transient decrease in MAP. Hyperoxia also had no effect on the apnoea-induced increase in CFV (40 +/- 4 %). We conclude that apnoea-induced fluctuations in CFV were caused primarily by increases and decreases in arterial partial pressure of CO2 (Pa,CO2) and that sympathetic nervous system activity was not required for either the initiation or the maintenance of the cerebrovascular response to hyper- and hypocapnia. Increased MAP or other unknown influences of autonomic activation on the cerebral circulation played a smaller but significant role in the apnoea-induced increase in CFV; however, negative intrathoracic pressure and the small amount of oxyhaemoglobin desaturation caused by 20 s apnoea did not affect CFV.