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1.

Introduction

The purpose of this study was to explore the communication behaviors demonstrated by parents of children with chronic conditions and provider team members when communicating about the child's care in outpatient clinics using concepts from the Theory of Shared Communication (TSC).

Methods

This was a secondary data analysis of 30 previously recorded pediatric clinic visits. Communication among parents and provider team members was analyzed using a researcher-developed coding scheme based on the TSC.

Results

Provider team members dominated communication during clinic visits, showing more frequent use of asking, explaining, advocating, and negotiating behaviors than parents. Parents were engaged in communication with frequent asking, explaining, and advocating behaviors.

Discussion

Parents of children with chronic conditions and multidisciplinary providers demonstrated the communication behaviors of the TSC in an outpatient clinic setting. Provider dominance of communication in the clinic setting may disempower parents and impair relationships.  相似文献   

2.

Background and purpose

Safety and efficacy of intravenous (IV) thrombolysis and endovascular therapy in children with acute ischemic stroke (AIS) are unknown to date. We aimed to review and synthesize currently available evidence on these acute recanalization therapies in pediatric stroke patients.

Methods

We performed a systematic review and meta-analysis of all available data on safety and efficacy of acute treatment including thrombolysis and endovascular therapy in pediatric AIS patients aged <18 years. We searched the electronic databases Medline and Cochrane Library for eligible studies published from the earliest date available until August 31, 2016. Safety outcomes included intracerebral hemorrhage (ICH) post-treatment and in-hospital mortality. Efficacy outcomes included functional outcome 3–6 months after index stroke.

Results

We identified 222 records, of which 3 studies with a total of 16,987 pediatric stroke patients met our eligibility criteria of whom 181 received IV thrombolysis. No data exists from randomized trials and no data is available on endovascular thrombectomy. Risk of any ICH was increased in children receiving thrombolysis (risk ratio = 3.48, 95%CI: 1.66–7.29; p = 0.001) compared with controls, with no evidence of heterogeneity (I2 = 0%). None of the included studies reported complete data on symptomatic ICH. In-hospital mortality was similar between pediatric stroke patients treated with thrombolysis and controls (risk ratio = 1.44, 95%CI: 0.39–5.40; p = 0.586), with evidence of heterogeneity (I2 = 62%). Efficacy of revascularization therapies could not be analyzed due to lack of outcome data.

Conclusions

Our analyses demonstrate a substantial lack of data on efficacy and safety of acute recanalization therapies in children with AIS.

PROSPERO Registration Information

URL: http://www.crd.york.ac.uk/PROSPERO. Unique identifier: CRD42016047140.  相似文献   

3.
4.

Introduction

Surgery in children can be difficult for patients and parents. We aimed to summarize pediatric patients' and parents' psychosocial experiences and needs in surgery.

Method

We used the Ovid search engine and screened 877 abstracts across three databases to extract data on pediatric patients' and parents' surgical experiences.

Results

Our search yielded 11 eligible studies representing 1,307 children undergoing surgery and their parents. Children's adverse experiences included psychological and behavioral changes before, during, and after surgery (e.g., anxiety, eating disturbances). Parents commonly experienced psychological distress. Children's needs related to medical and health care services, whereas parents had high information needs.

Discussion

Children's adverse experiences can negatively affect medical outcomes. Children's experiences are inextricably linked to their parents' and can become negatively affected by their parents' adverse experiences. Patients and parents with previous hospitalizations and surgeries had worse surgical experiences, highlighting further research in the context of chronic illness.  相似文献   

5.

Introduction

The purposes of this project were (a) to examine criteria derived from evidence-based pediatric acute asthma exacerbation assessment tools, asthma scores, and the acute asthma prediction rule validated and used in the emergency department and (b) to adapt these criteria for pediatric primary care.

Method

The three stages of the project included (a) identification of criteria in a literature review, (b) validation of the criteria by an expert panel, and (c) adaptation of the criteria in the design of an assessment tool.

Results

The criteria were validated and adapted in the design of The Pediatric Acute Asthma Exacerbation Severity Assessment and Disposition Decision-Making Tool for Pediatric Primary Care.

Discussion

The adaptation of criteria derived from the evidence and validated by an expert panel will inform and guide clinicians in assessing severity and support decision making in determining disposition of pediatric patients presenting with an acute asthma exacerbation in primary care.  相似文献   

6.

Background

The quality of general movements (GMs) has proven to have predictive value for the developmental outcome, but this has not yet been studied in twins.

Aims

Our aim was to analyse the quality of GMs and neurological and developmental outcome in relation to the gestational age (GA), mode of conception and other perinatal risk factors in a group of twins.

Study design

The documentation of twins referred for follow-up in the period from 1998 to 2016 was studied retrospectively. Data concerning the quality of GMs, perinatal risk factors and developmental outcome were analysed.

Subjects

Eighty-nine twin pairs, GA from 24 to 38 weeks (median 35.0; IQR 3), birth weight 670 g–3820 g (median 2323; IQR 645) were included.

Outcome measures

Results of neurological, psychological and speech/language development and school outcome were analysed.

Results

GMs at term age and at three to four months postterm age did not differ with regard to the mode of conception. Preterm birth was significantly related to GMs at three to four months postterm age. At term age, GMs were significantly related to neurological outcome, while at three to four months postterm age, GMs were related to both the neurological and psychological outcome. Difficulties in speech/language development were diagnosed in almost half of the children, more frequently in boys and children with lower Apgar scores.

Conclusion

The study highlights the value of GMs for predicting the developmental outcome in twins and indicates the importance of developmental, especially speech/language, follow-up in twins.  相似文献   

7.

Background

The ALDH7A1 gene is known to be responsible for autosomal recessive pyridoxine-dependent epilepsy (OMIM 266100). The phenotypic spectrum of ALDH7A1 mutations is very heterogeneous ranging from refractory epilepsy and neurodevelopmental delay, to multisystem neonatal disorder.

Aim

The present study aims at describing the phenotype associated with a novel homozygous ALDH7A1 mutation and the spectrum of brain malformations associated with pyridoxine-dependent epilepsy.

Methods

We conducted a literature review on the Internet database Pubmed (up to November 2017) searching for ALDH7A1 mutations associated with brain malformations and brain MRI findings.

Results

We present the case of two siblings, children of related parents. The proband presented neonatal focal seizures not responding to conventional antiepileptic drugs. Electroencephalography showed a suppression burst pattern and several multifocal ictal patterns, responsive to pyridoxine. Brain MRI was normal. Molecular analysis by targeted next-generation sequencing panel for epileptic encephalopathy disclosed a homozygous missense mutation of ALDH7A1. The same mutation was then found in a stored sample of DNA from peripheral blood of an older sister dead 3 years earlier. This girl presented a complex brain malformation diagnosed with a foetal MRI and had neonatal refractory seizures with suppression burst pattern. She died at 6 months of age.

Literature review

The brain abnormalities most frequently reported in pyridoxine-dependent epilepsy include: agenesia/hypoplasia of the corpus callosum, not specific white matter abnormalities, large cisterna magna, ventriculomegaly, haemorrhages, cerebellum hypoplasia/dysplasia, and, more rarely, dysplasia of the brainstem and hydrocephalus.

Discussion and conclusions

ALDH7A1 mutations have been associated to different brain abnormalities, documented by MRI only in few cases. The study cases expand the clinical spectrum of ALDH7A1 associated conditions, suggesting to look for ALDH7A1 mutations not only in classical phenotypes but also in patients with brain malformations, mainly if there is a response to a pyridoxine trial.  相似文献   

8.

Background and Objectives

Children with cerebral palsy (CP) are routinely treated with botulinum toxin A (BoNT-A). Two non dose-equivalent and differently priced products, Botox and Dysport are used. Depending on the conversion one of the products is considerably cheaper. However, the dose conversion factors studied to date have varied widely and relevant studies have not included children. Our objective here was to compare the efficacy and health economics of the switch from Botox to Dysport in children with CP when conversion was set to 1:2. Specifically were these treatments perceived as equivalent in terms of efficacy, duration and side-effects and were the drug cost lowered by using Dysport.

Methods

This prospective, real-world, cost-effectiveness population-based observational study included all children with CP, (n = 159) mean age 9.4 years (SD, 4.3), in the larger Stockholm area who received BoNT-A between September 1, 2014, and December 31, 2015. Parents reported the efficacy, duration and side-effects of previous treatment while physicians reported doses and goals set by children and parents for the present treatment. Drug acquisition costs were provided by county administrators.

Results

In connection with 341 visits caregivers reported comparable effects of similar duration with these products, with few, similar and transient side-effects. The drug-cost per treatment was 4029 SEK for Botox and 2380 SEK in the case of Dysport.

Conclusion

When Botox was replaced by a two-fold higher Unit dose of Dysport (conversion 1:2) parents perceived the treatment of their children with CP to be equally effective while the cost was 41% lower according to procured prices.  相似文献   

9.

Objective

Recreational trampoline use is increasing in popularity, with a resulting increase in significant trampoline-related injuries in children. Parents are the best advocates for the safety of their children during recreational trampoline use. Our primary objective was to determine the proportion of parents who were aware of 5 key recommendations for safer recreational trampoline use in children.

Methods

This was a cross-sectional survey of parents whose children presented to a tertiary care pediatric emergency department with an extremity injury. Survey questions were derived and validated using expert opinion, available literature, and pre- and pilot testing of questions on the target audience.

Results

Of the 1415 enrolled parents, 654 (46.2%) had regular access to a trampoline and 125 (8.8%) of their children had a history of trampoline injury. A total of 116 (8.2%; 95% confidence interval, 6.8–9.6) parents were aware of all 5 key safety recommendations for home trampoline use. Specifically, the proportion of parents who reported knowledge of the requirement for active supervision, regularly inspected safety equipment, avoiding stunts, multiple jumpers, and use by children 6 years of age and older was 89.0%, 77.2%, 44.3%, 41.6%, and 18.3%, respectively.

Conclusions

Trampoline safety knowledge of the 5 key recommendations among parents was low, specifically with respect to recommended age, number of jumpers, and stunts. Because it is unlikely that use of recreational trampolines will decrease, a harm reduction approach that includes a public knowledge translation strategy of recommendations for safer home use of trampolines is necessary.  相似文献   

10.

Objectives

To elaborate the imaging phenotype associated with a homozygous c.743C > del frameshift mutation in DAG1 leading to complete absence of both α- and β-dystroglycan previously reported in a consanguineous Israeli-Arab family.

Methods

We analyzed prenatal and postnatal imaging data of patients from a consanguineous Israeli-Arab kindred harboring the DAG1 mutation.

Results

The imaging studies (fetal ultrasound, CT scan and postnatal MRI) demonstrated: flat cortex (abnormally thick with irregular pebbled cortical-white matter border on MRI), hydrocephalus, scattered small periventricular heterotopia and subependymal hemorrhages and calcifications, z-shaped brainstem, and in addition an occipital encephalocele, vermian agenesis, and an elongated and thick tectum (tectocerebellar dysraphia).

Conclusions

The novel association of cobblestone malformation with tectocerebellar dysraphia as part of WWS is characteristic of the homozygous c.743C > del frameshift mutation in the DAG1 gene.  相似文献   

11.

Background

Dystonia is characterised by involuntary movements and postures. Deep Brain Stimulation (DBS) is effective in reducing dystonic symptoms in primary dystonia in childhood and to lesser extent in secondary dystonia. How families and children decide to choose DBS surgery has never been explored.

Aims

To explore parental decision-making for DBS in paediatric secondary dystonia.

Methods

Data was gathered using semi-structured interviews with eight parents of children with secondary dystonia who had undergone DBS. Interviews were analysed using Interpretative Phenomenological Analysis.

Results

For all parents the decision was viewed as significant, with life altering consequences for the child. These results suggested that parents were motivated by a hope for a better life and parental duty. This was weighed against consideration of risks, what the child had to lose, and uncertainty of DBS outcome. Decisions were also influenced by the perspectives of their child and professionals.

Conclusions

The decision to undergo DBS was an ongoing process for parents, who ultimately were struggling in the face of uncertainty whilst trying to do their best as parents for their children. These findings have important clinical implications given the growing referrals for consideration of DBS childhood dystonia, and highlights the importance of further quantitative research to fully establish the efficacy of DBS in secondary dystonia to enhance informed decision-making.  相似文献   

12.

Objective

Acute treatment of childhood arterial ischaemic stroke and prevention strategies for recurrent stroke episodes depend strongly on each child's individual risk profile. The aim of this study is to characterize risk factors for childhood stroke, their occurrence in isolation or combination, and to identify possible common risk factor patterns.

Methods

This population-based study was conducted via ESPED, a surveillance unit for rare paediatric diseases in Germany. Children aged >28days and <18 years with an acute arterial ischaemic stroke occurring between January 2015 and December 2016 were included.

Results

Among 99 reported children with arterial ischaemic stroke, 56 children were male. Male predominance was significant in adolescents from 12 years old onward. Arterial ischaemic stroke was more common in very young children <2 years of age and in adolescence. No risk factor was identified in 27 children. Hypercoagulable states (29%), cardiac disorders (24%), and arteriopathies (21%) were the most common risk factors. Some risk factor categories were more likely to be identified in isolation (i.e. cardiac disorders, prothrombotic abnormalities and chronic head and neck disorders) than others. The number of risk factors (n = 0–4) per patient and risk factor categories did not differ by age.

Conclusion

Although we could not identify common patterns of risk factor combinations, several risk factors occurred more likely in isolation than others. Further research should focus on the impact of isolated presumed childhood stroke risk factors like certain prothrombotic abnormalities, migraine or a patent foramen ovale. With regard to different age groups, stroke mechanisms in male adolescents require particular attention.  相似文献   

13.

Background

Ataxia telangiectasia (AT) is a neurodegenerative cerebellar disorder, caused by mutations in the ATM gene, involved in DNA repair. Radiosensitivity, progressive ataxia, immune deficiency and malignancies, are well known symptoms, but urological manifestations are scarcely described.

Objective

To characterize urologic manifestations in a large cohort of AT patients.

Methods

Retrospective cross-sectional chart study comprising 52 AT patients followed at a National AT Center.

Results

25% of the cohort (13 patients/8 males) had urologic symptoms, which presented at 11 ± 4.3 years. The most common symptom was secondary enuresis affecting 15% of the patients (8 children/4 males). Incontinence appeared at 8 ± 6.2 years of age, and resolved spontaneously within 15 ± 8.3 months in 6 patients. It preceded loss of ambulatory capacity by 1–2 years in 7 patients. Lumbosacral MRI were normal (4 children) and urine cultures (all) were negative. Urodynamic evaluation that was performed in only one patient revealed overactive bladder. Additional manifestations were macroscopic hematuria due to bladder telangiectasia in a 12-year-old, and renal cell carcinoma in a 22-year-old. Other manifestations unrelated to AT were neprolithiasis, vesico-ureteral reflux and scrotal pain, each in 1 patient.

Discussion

Transient secondary enuresis is a frequent finding in AT patients, heralding loss of ambulatory capacity, tough it's pathophysiological mechanism is largely no understood.  相似文献   

14.

Introduction

Shapiro syndrome (SS) is characterized by spontaneous recurrent episodes of hypothermia, hyperhidrosis and corpus callosum (CC) agenesis. Less than 60 cases have been reported to date and the pathogenic mechanism as well as the prognosis of this syndrome are still debated. We describe the clinical features and long-term follow-up of a pediatric cohort of SS patients.

Methods

We collected 13 (10 novel) pediatric cases of SS and report their long-term follow-up and neurological outcome.

Results

All patients experienced recurring hypothermia, with body temperature below 35 °C during the episodes, often accompanied by hyperidrosis. CC agenesis was an inconstant structural feature in the present series (2/13 patients). Seven patients received antiepileptic drugs (AEDs) or other drug therapy for a mean period of 12 months. At long-term follow-up (mean = 61 months, range: 60–96), all individuals were free from episodes of paroxysmal hypothermia independently from previous AED use or other drug therapy.

Conclusion

Paroxysmal hypothermia, the core symptom of SS, behaved as a age-dependent feature in our cohort, supporting a good long-term prognosis for SS. A prompt diagnosis of SS is crucial to avoid unnecessary diagnostic investigations.  相似文献   

15.

Background

Dysphagia is a well-known dysfunction in patients with Duchenne muscular dystrophy (DMD), but long-term follow-up of this symptom has not been reported. The aims of this study were to investigate swallowing status, prospectively study the prognosis of swallowing function in adult patients with DMD over the course of 6 years, and to identify clinical factors related to prognosis.

Methods

Participants were 81 patients with DMD. Swallowing status was assessed using the Neuromuscular Disease Swallowing Status Scale (NdSSS) every 2 years for 6 years.

Results

NdSSS was related to age, functional ability assessed using the Swinyard staging and the status of ventilator dependence. Median NdSSS changed from 6 to 4, 4 and 3 over the 4 time points. Number of patients with tube feeding increased by 5–10% every 2 years. Initial NdSSS was a predictor of future swallowing status.

Conclusion

Swallowing status declined over time in patients with adult DMD, with initial NdSSS as a predictor.  相似文献   

16.

Objective

Despite the importance of child assent, there is little consensus on what information should be disclosed and what information is most important to children for decision-making. This study was designed to compare children's/adolescents' priorities for research information with the information parents believe is most important to their children.

Methods

Child-parent dyads completed separate and independent surveys regarding information (risks, benefits, etc) that they perceived to be most important to the child to make decisions about participating in a hypothetical randomized controlled trial. Parents responded in the context of what information they believed their child (not themselves) would think important.

Results

Fifty-five parent-child dyads completed surveys. Although all information was deemed important, children/adolescents put greater emphasis on privacy and less on knowing the purpose of the study and the benefits compared with what their parents believed was important to their child. Adolescents (13–17 years old) placed greater importance on knowing the procedures, direct benefits, and the voluntary nature of participation compared with younger children (8–12 years old). Parents of older girls in particular placed greater emphasis on their daughter's need to know the purpose of the study, the procedures, benefits, and voluntary nature, compared with parents of boys.

Conclusions

Results show that the information priorities of children/adolescents considering participation in a randomized controlled trial differ from that which their parents think is important to them. Pediatric researchers can use this knowledge to ensure that parents do not conflate their own expectations/priorities with those of their child and that children receive the information they need.  相似文献   

17.

Background

In non-walking children with severe spasticity, daily care can be difficult and many patients suffer from pain. Selective dorsal rhizotomy (SDR) reduces spasticity in the legs, and therefore has the potential to improve daily care and comfort.

Aim

To examine effects of SDR on daily care and comfort in non-walking children with severe spasticity due to different underlying neurological conditions.

Methods

Medical history, changes in daily care and comfort and satisfaction with outcome were assessed retrospectively in non-walking children who underwent SDR in our center, with a mean follow-up of 1y 7m (range 11m–4y 3m). All eligible patients (n = 24, years 2009–2014) were included.

Results

Mean age at SDR was 12y 4m (SD 4y 3m, range 2y 8m–19y 3m). Associated orthopaedic problems were frequent. Seven patients underwent scoliosis correction in the same session. Most improvements were reported in dressing (n = 16), washing (n = 12) and comfort (n = 10). Median score for satisfaction was 7 on a scale of 10 (range 1–9). SDR resulted in reduction of spasticity in leg muscles. In nine patients dystonia was recorded post-operatively, mainly in children with congenital malformations and syndromes.

Interpretation

SDR is a single event intervention that can improve daily care and comfort in non-walking children with severe spasticity, and can safely be combined with scoliosis correction. Despite the improvements, satisfaction is variable. Careful attention is necessary for risk factors for dystonia, which may be unmasked after SDR.  相似文献   

18.

Background

Chemotherapy-induced Peripheral Neuropathy (CIPN) of large-fibers affects up to 20% of survivors of pediatric acute lymphoblastic leukemia (ALL). We aimed to describe small-fiber toxicity and pain sensitization in this group.

Methods

In a cross-sectional, bicentric study we assessed 46 survivors of pediatric ALL (Mean age: 5.7 ± 3.5 years at diagnosis, median 2.5 years after therapy; males: 28). Inclusion criteria: ≥6 years of age, ≥3 months after last administration of Vincristine, and cumulative dose of Vincristine 12 mg/m2. We used a reduced version of the Pediatric-modified Total Neuropathy Score (Ped-mTNS) as bedside test and Quantitative Sensory Testing (QST) for assessment of small- and large-fiber neuropathy as well as pain sensitization. We employed Nerve Conduction Studies (NCS) as the most accurate tool for detecting large-fiber neuropathy.

Results

Fifteen survivors (33%) had abnormal rPed-mTNS values (≥4 points) and 5 survivors (11%) reported pain. In QST, the survivor group showed significant (p < 0.001) inferior large-fiber function and pain sensitization when compared to healthy matched peers. We identified deficits of vibration in 33 (72%) and tactile hypoesthesia in 29 (63%), hyperalgesia to blunt pressure in 19 (41%), increased mechanical pain sensitivity in 12 (26%) and allodynia in 16 (35%) of 46 survivors. Only 7 survivors (15%) had pathologic NCS.

Conclusion

QST is a sensitive tool that revealed signs of large-fiber neuropathy in two thirds, small-fiber neuropathy and pain sensitization in one third of survivors. Prospective studies using QST in pediatric oncology may help to elucidate the pathophysiology of small-fiber neuropathy and pain sensitization as well as their relevance for quality of survival.  相似文献   

19.

Objective

To assess differences in health care access, utilization, and experiences among Latino children in California according to parental citizenship status and language use.

Methods

Data are from the 2011 and 2012 California Health Interview Survey public use child files. A total of 2841 interviews of parents of Latino children younger than the age of 12 years were conducted. Analyses were conducted to determine the associations between access (usual of source of care, delay in receiving needed care, health insurance), utilization (physician visits in past year, emergency department visits), and experiences (doctor listens, doctor explains instructions clearly, communication via telephone or e-mail) according to parental citizenship status and household language use after adjusting for confounders.

Results

In multivariate analyses, there were no significant differences in access to care according to parental citizenship status. Children with 2 noncitizen parents had fewer doctor visits and were less likely to go to the emergency department in the past year than those with 2 citizen parents. Among children with 1 or 2 noncitizen parents, their parents reported worse experiences in care than those with 2 citizen parents. Similar results were observed for language use. Parents of children in bilingual and Spanish-only households were less likely to report that their children's doctors explained things clearly, and parents in Spanish-only households were less likely to communicate via telephone or e-mail than those in English-only households.

Conclusions

Health policy should focus on provider-parent communication to ensure health care equity for Latino children whose parents are not citizens or do not speak English.  相似文献   

20.

Background

The main source of knowledge on adverse drug events (ADE) are physicians' reports in controlled clinical trials. In contrast, little is known about the parents' perception of ADE of anticonvulsants their children receive.

Methods

After approval by the local ethics committee, we performed a survey in a neuropediatric outpatient clinic of a university hospital. Based on a structured questionnaire, we interviewed parents of children with current anticonvulsant treatment regarding (i) their fears about potential ADE, (ii) experienced ADE according to parents, and (iii) implications of ADE on the child's life.

Results

Parents of 150 patients took part in the interview. (i) 95 (63.3%) parents expressed fears concerning ADE, mostly liver injury/liver failure (33 [22%]). (ii) 129 (86%) parents reported experienced ADE, mostly sedation (65 [43.3%]) and abnormal behavior (54 [36%]). (iii) Parents reported substantial implications of ADE on the child's daily life for 84 (56%) children, and 63 (42%) parents expressed a negative impact on the child's development.

Conclusion

We recognized a great discrepancy between those ADE that were feared and those that were experienced. Parents feared life-threatening ADE and experienced less severe ADE that nevertheless have a negative impact on the child's daily life.  相似文献   

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