Comparison of Problematic Behavior According to the Ryouiku Techou Standard
Rehabilitation; Problematic behavior; Ryouiku Techou; The Japanese version of the Aberrant Behavior Checklist; behavioral problems; Mowat-Wilson syndrome; Pena Shokeir syndrome; tuberous sclerosis; West syndrome; trajectory; characteristics; stereotyped behaviors; lethargy
[Purpose] We compared problematic behaviors of children according to the severity of their mental retardation (MR) of intellect as categorized by the Ryouiku Techou in this study, to investigate the influence of MR of intellect on children's problematic behaviors. [Subjects] The subjects were 86 mentally retarded children undergoing physical therapy at hospitals and other facilities. [Methods] The examiners were 13 physical therapists and 8 occupational therapists who worked at the hospital and knew the children well. The examiners individually assessed the subjects using the Japanese version of the Aberrant Behavior Checklist. The subjects were divided into two groups (A and non-A) according to the Ryouiku Techou standard. [Results] No significant differences were observed between the groups except in the items of stereotypy and lethargy. [Conclusion] Problematic behaviors other than stereotypy and lethargy were not influenced by the Ryouiku Techou standard.
Uesugi M; Inoue Y; Gotou M; Nanba Y; Otani Y; Takemasa S
Journal of Physical Therapy Science
2013
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1589/jpts.25.877" target="_blank" rel="noreferrer noopener">10.1589/jpts.25.877</a>
Promoting adaptive behaviors by two girls with Rett syndrome through a microswitch-based program
Assistive technology; Indices of happiness; Locomotor behavior; Microswitches; Rett syndrome; adaptive behavior; adolescent; article; case report; child behavior; female; hand washing; happiness; human; locomotion; priority journal; school child; stereotypy; tone and motor problems; Rett syndrome; physical intervention; Wobble microswitch; optic sensors; stereotyped movements
We assessed a microswitch-based program to improve self-determination to access to preferred stimuli and to foster locomotor behavior by two girls with Rett syndrome and multiple disabilities. To enhance the first behavior (access to preferred stimuli) a wobble microswitch (sensitive touch sensor) was used while for the second behavior (step responses) optic sensors were applied. A second aim of the study was to monitor indices of happiness as consequence of the use of assistive technology. Finally, a third objective of the study was the reduction of hand washing and body rocking related stereotypies. The study was carried out according to a multiple probe design across behaviors for both participants, where the two behaviors were first learned independently, then combined together. Results showed an increasing of performance and of indices of happiness and a decreasing of stereotyped behaviors for both participants during intervention phases. Practical, psychological and clinical implications of the findings are discussed. © 2013 Elsevier Ltd. All rights reserved.
Stasolla F; Caffo A O
Research in Autism Spectrum Disorders
2013
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<a href="http://doi.org/10.1016/j.rasd.2013.07.010" target="_blank" rel="noreferrer noopener">10.1016/j.rasd.2013.07.010</a>
Disorders of Upper Limb Movements in Ataxia-Telangiectasia
Cross-Sectional Studies; Age Factors; Male; Severity of Illness Index; Young Adult; Child; Humans; Adult; Adolescent; Female; Child Preschool; Periodicity; Ataxia Telangiectasia/pp [Physiopathology]; Motor Activity; Movement; Upper Extremity; Accelerometry; Biomechanical Phenomena; Motor Activity/ph [Physiology]; Movement Disorders/pp [Physiopathology]; Movement/ph [Physiology]; Posture/ph [Physiology]; Tremor/pp [Physiopathology]; Upper Extremity/pp [Physiopathology]; tone and motor problems; ataxia telangiectasia; trajectory; characteristics; abnormal involuntary movements; kinetic tremor; postural tremor; resting tremor
Ataxia-telangiectasia is known for cerebellar degeneration, but clinical descriptions of abnormal tone, posture, and movements suggest involvement of the network between cerebellum and basal ganglia. We quantitatively assessed the nature of upper-limb movement disorders in ataxia-telangiectasia. We used a three-axis accelerometer to assess the natural history and severity of abnormal upper-limb movements in 80 ataxia-telangiectasia and 19 healthy subjects. Recordings were made during goal-directed movements of upper limb (kinetic task), while arms were outstretched (postural task), and at rest. Almost all ataxia-telangiectasia subjects (79/80) had abnormal involuntary movements, such as rhythmic oscillations (tremor), slow drifts (dystonia or athetosis), and isolated rapid movements (dystonic jerks or myoclonus). All patients with involuntary movements had both kinetic and postural tremor, while 48 (61%) also had resting tremor. The tremor was present in transient episodes lasting several seconds during two-minute recording sessions of all three conditions. Percent time during which episodic tremor was present was greater for postural and kinetic tasks compared to rest. Resting tremor had higher frequency but smaller amplitude than postural and kinetic tremor. Rapid non-rhythmic movements were minimal during rest, but were triggered during sustained arm postures and goal directed arm movements suggesting they are best considered a form of dystonic jerks or action myoclonus. Advancing age did not correlate with the severity of involuntary limb movements. Abnormal upper-limb movements in ataxia-telangiectasia feature classic cerebellar impairment, but also suggest involvement of the network between the cerebellum and basal ganglia.
Shaikh A G; Zee D S; Mandir A S; Lederman H M; Crawford T O
PLoS ONE
2013
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<a href="http://doi.org/10.1371/journal.pone.0067042" target="_blank" rel="noreferrer noopener">10.1371/journal.pone.0067042</a>
Behavior Problems in Individuals with Cornelia de Lange Syndrome: Population-Specific Validation of the Behavior Problem Inventory-01
reliability; Rehabilitation; Intellectual disabilities; behavior; validity; prevalence; phenomenology; delange-syndrome; intellectual disabilities; Aggressive/destructive behavior; behaviors; Cornelia de Lange Syndrome; repetitive; scale; Self-injurious; self-injurious-behavior; short form; Stereotyped behavior; behavioral problems; De Lange syndrome; trajectory; characteristics
The purpose of this study was to validate the Behavior Problem Inventory-01 (BPI-01; Rojahn J Autism Dev Disord 31:577-588, 2001) in a population of 179 individuals with Cornelia de Lange Syndrome (CdLS) and to extend the literature on the behavioral phenotype of individuals with CdLS. We also set out to determine the relationship between the frequency of self-injurious behavior (SIB), stereotyped behavior, and aggressive/destructive behavior topographies with the level of intellectual disability (ID). The BPI-01 among the individuals with CdLS showed a sufficient factor structure and internal consistency of each of the three assessed construct (SIB, stereotyped behavior, and aggressive/destructive behaviors). Similar to the findings in other populations with intellectual disabilities, those with severe/profound intellectual impairment exhibited significantly more frequent SIB and stereotypic behavior than those with moderate or mild intellectual impairment or average intellectual abilities while no statistically a differences were noted for aggressive behavior across levels of intellectual impairment. There is a need for uniformity of assessment tools across different study to improve our ability to compare results across different prevalence studies.
Rojahn J; Barnard-Brak L; Richman D; Dotson W; Medeiros K; Wei T L; Abby L
Journal of Developmental and Physical Disabilities
2013
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<a href="http://doi.org/10.1007/s10882-012-9329-6" target="_blank" rel="noreferrer noopener">10.1007/s10882-012-9329-6</a>
Fatigue and depression in children with demyelinating disorders
adolescent; Canada; epidemiology; child behavior; disease duration; priority journal; school child; health survey; self report; cohort analysis; cross-sectional study; psychological aspect; human; article; child; female; male; controlled study; clinical article; comorbidity; childhood disease; mood; sleep; therapy; acute disseminated encephalomyelitis; demyelinating disease; depression; fatigue; multiple sclerosis; acute disseminated encephalomyelitis; demyelinating disorders; multiple sclerosis; acquired demyelinating syndromes; trajectory; characteristics; alertness
Fatigue and depression have been shown to be significant problems in children with multiple sclerosis. The rate at which these conditions occur in children with other acquired demyelinating syndromes is unknown. In this cross-sectional study, the authors evaluated 49 children with demyelinating disorders (multiple sclerosis and acute disseminated encephalomyelitis) and 92 healthy controls for depression and/or fatigue using the Behavior Assessment System for Children, Second Edition behavior and mood rating scale and Varni PedsQL Multidimensional Fatigue Scale. The parents of acquired demyelinating syndrome patients were more likely to report elevated depressive symptoms (30.8% vs 10.8%, P =.008). Elevated parent and self-reported total fatigue (25% vs 0%, P <.001, 26.7% vs 8.6%, P =.024) was seen in the patient cohort. The authors conclude that fatigue and depression are far more common in children with acquired demyelinating syndromes than in controls. Clinical attention to and implementation of effective therapies oriented toward these conditions in children with acquired demyelinating syndromes is needed. © The Author(s) 2012.
Parrish J B;Weinstock-Guttman B;Smerbeck A;Benedict R H B;Yeh E A
Journal of Child Neurology
2013
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<a href="http://doi.org/10.1177/0883073812450750" target="_blank" rel="noreferrer noopener">10.1177/0883073812450750</a>
Movement disorder in ataxia-telangiectasia: treatment with amantadine sulfate
Adolescent; Amantadine/tu [Therapeutic Use]; Antiparkinson Agents/Antiparkinson [Therapeutic Use]; Ataxia Telangiectasia/co [Complications]; Child; Preschool; Disability; Evaluation; Humans; Movement Disorders/dt [Drug Therapy]; Movement Disorders/et [Etiology]; Neurologic Examination; Prospective Studies; Severity of Illness Index; 0 (Antiparkinson Agents); BF4C9Z1J53 (Amantadine); tone and motor problems; ataxia telangiectasia; pharmacologic intervention; amantadine sulfate
Ataxia-telangiectasia is a cerebellar neurodegenerative disorder presenting with ataxia, chorea, myoclonus, and bradykinesia. Literature on treatment of movement disorders is scarce. We treated 17 children (aged 11.2 +/- 3.9 years) for 8 weeks with the dopaminergic and anti-N-methyl-d-aspartate (NMDA) agent amantadine sulfate 6.3 +/- 0.87 mg/kg/d. Ataxia was assessed by using the International Cooperative Ataxia Scale, parkinsonism by the Unified Parkinson Disease Rating Scale, and chorea/myoclonus by the Abnormal Involuntary Movement Scale. Responders were considered those patients who had at least 20% improvement in the summation of the 3 scales. Overall, 76.5% of patients were responders, with a mean 29.3% improvement. Ataxia, involuntary movements, and parkinsonism improved significantly (25.3%, 32.5%, and 29.5%, respectively); (P < .001, t test). Side effects were mild and transient, and they did not lead to drug discontinuation. Amantadine is a well-tolerated and effective treatment for motor symptoms in ataxia telangiectasia. Assessment of long-term effects and a double-blind study should follow.
Nissenkorn A; Hassin-Baer S; Lerman S F; Levi Y B; Tzadok M; Ben-Zeev B
Journal of Child Neurology
2013
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1177/0883073812441999" target="_blank" rel="noreferrer noopener">10.1177/0883073812441999</a>
Reliability and Validity of the TIMPSI for Infants With Spinal Muscular Atrophy Type I
infant; children; Pediatrics; Rehabilitation; motor; female; male; assessments; childhood/physiopathology; humans; spinal muscular atrophies of; child development; childhood/diagnosis; functional motor scale; observer variation; performance; physical therapy; physical therapy specialty/standards; reproducibility of results; skills/physiology; specialty/instrumentation; video recording; tone and motor problems; SMA1; tool development; scale development; TIMP
Purpose: This study examined the reliability and validity of the Test of Infant Motor Performance Screening Items (TIMPSI) in infants with type I spinal muscular atrophy (SMA). Methods: After training, 12 evaluators scored 4 videos of infants with type I SMA to assess interrater reliability. Intrarater and test-retest reliability was further assessed for 9 evaluators during a SMA type I clinical trial, with 9 evaluators testing a total of 38 infants twice. Relatedness of the TIMPSI score to ability to reach and ventilatory support was also examined. Results: Excellent interrater video score reliability was noted (intraclass correlation coefficient, 0.97-0.98). Intrarater reliability was excellent (intraclass correlation coefficient, 0.91-0.98) and test-retest reliability ranged from r = 0.82 to r = 0.95. The TIMPSI score was related to the ability to reach (P <= .05). Conclusion: The TIMPSI can reliably be used to assess motor function in infants with type I SMA. In addition, the TIMPSI scores are related to the ability to reach, an important functional skill in children with type I SMA. (Pediatr Phys Ther 2013;25:140-148)
Krosschell K J; Mazulski J A; Scott C; King W; Hartman J T; Case L E; Viazzo-Trussell D; Wood J; Roman C A; Hecker E; Meffert M; Leveille M; Kienitz K; Swoboda K J; Project Cure Spinal Muscular Atrophy Investigators
Pediatric Physical Therapy
2013
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<a href="http://doi.org/10.1097/PEP.0b013e31828a205f" target="_blank" rel="noreferrer noopener">10.1097/PEP.0b013e31828a205f</a>
Behavior in preschool children with the 22q11.2 deletion syndrome
behavioral problems; 22q11.2 deletion syndrome; trajectory; characteristics
Children with the 22q11.2 deletion syndrome (22q11DS) are at an increased risk of psychiatric problems from pre-adolescence; little is known, however, about behavioral problems at a preschool age and the relationship between speech and behavior in this group. Parents of 90 children (aged 1.425.99 years) with 22q11DS filled out the Child Behavior Checklist, documenting behaviors including speech problems. Their profiles were compared with those of a comparison group consisting of 33 children with nonsyndromic orofacial clefts without 22q11DS, since both children with 22q11DS and children with clefts are expected to have speech problems. In the 22q11DS group, data on intelligence was acquired by means of formal tests. Parents of children with 22q11DS reported significantly higher mean scores on withdrawn behavior, affective problems and pervasive developmental problems compared to children with nonsyndromic clefts. Approximately 30% of children with 22q11DS had a score above the 97th percentile on at least one of the behavior subscales, indicating psychopathology. In children with 22q11DS, the reported behavioral problems were not associated with speech problems. Behavioral problems were found in 30% of young children with 22q11DS and were unlikely to be caused by speech problems. Within the 22q11DS group, behavioral problems were not related to the degree of cognitive impairment. This shows that many children with 22q11DS, known to be at an increased risk of psychiatric problems from pre-adolescence, already show behavioral problems before the age of 6 years. (c) 2012 Wiley Periodicals, Inc.
Klaassen P; Duijff S; de Veye H S; Vorstman J; Beemer F; Sinnema G
American Journal of Medical Genetics Part A
2013
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<a href="http://doi.org/10.1002/ajmg.a.35685" target="_blank" rel="noreferrer noopener">10.1002/ajmg.a.35685</a>
Cognitive and behavioral trajectories in 22q11DS from childhood into adolescence: A prospective 6-year follow-up study
behavioral problems; 22q11.2 deletion syndrome; trajectory; characteristics; internalizing behaviors
Patients with 22q11DS are at risk of behavioral problems and cognitive impairment. Recent studies suggest a possible intellectual decline in 22q11DS children. To date it is unknown if cognitive development is related to the behavioral problems in 22q11DS. We studied 53 children with 22q11DS who underwent cognitive and behavioral assessments at 9.5 years (T1) and 15.3 years (T2). In about one third, IQ data obtained at 7.5 years (T0) were also available. Results showed that internalizing behaviors intensified while externalizing behaviors decreased. Simultaneously, in about a third a significant decline in IQ was found, which, surprisingly, was unrelated to the behavioral changes. It can be concluded that children with 22q11DS follow a unique developmental trajectory. Cognitive deterioration is severe in some but does not appear to predict behavioral problems in early adolescence. (c) 2013 Elsevier Ltd. All rights reserved.
Duijff S N; Klaassen P W J; de Veye H; Beemer F A; Sinnema G; Vorstman J A S
Research in Developmental Disabilities
2013
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<a href="http://doi.org/10.1016/j.ridd.2013.06.001" target="_blank" rel="noreferrer noopener">10.1016/j.ridd.2013.06.001</a>
Orthopedic manifestations and implications for individuals with Costello syndrome
tone and motor problems; Costello syndrome; trajectory; characteristics; orthopedic manifestations
Costello syndrome is a rare genetic condition caused by heterozygous alterations in HRAS and characterized by multi-system abnormalities. Individuals with Costello syndrome usually present with severe feeding difficulties in infancy, short stature, coarse facial features, increased tumor risks, cardiac and neurological complications, intellectual disability and orthopedic complications. This study further defines the orthopedic manifestations affecting individuals with Costello syndrome. We studied 43 participants and performed medical records review, clinical examinations and orthopedic inquiry forms. In 23 participants, hip and or spinal imaging assessments were completed. Serial radiographs were analyzed when available. A total of 25 orthopedic manifestations were identified. Ten manifestations were seen in the majority of the participants: hypotonia (87%), ligamentous laxity (85%), scoliosis (63%), kyphosis (58%), characteristic hand deformities (85%), ulnar deviation of the wrist (63%), elbow (55%) and shoulder contractures (65%), tight Achilles tendon (73%), and pes planus (53%). Other characteristics of special note were hip dysplasia (45%), foot deformities requiring surgical intervention (38%) and osteopenia/osteoporosis (47%). We also studied the development of the hips and spine. Uni- or bilateral hip dysplasia was congenital in some, while it developed throughout childhood in others. Spinal involvement included scoliosis, kyphosis, lordosis, and curvature reversal (thoracic lordosis and lumbar kyphosis). Based on these findings, we recommend routine referral to an orthopedic surgeon as well as instituting screening protocols for hips and spine for individuals with Costello syndrome.Copyright © 2013 Wiley Periodicals, Inc.
Detweiler S; Thacker M M; Hopkins E; Conway L; Gripp K W
American Journal of Medical Genetics Part A
2013
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<a href="http://doi.org/10.1002/ajmg.a.36047" target="_blank" rel="noreferrer noopener">10.1002/ajmg.a.36047</a>
Motor Function Measure: Validation of a Short Form for Young Children With Neuromuscular Diseases
reliability; Rehabilitation; scale; spinal muscular-atrophy; ambulation; Disability evaluation; dystrophy; Neuromuscular diseases; outcome measure; sma-iii patients; Sport Sciences; SMA1; tool development; scale development
Objective: To validate a useful version of the Motor Function Measure (MFM) in children with neuromuscular diseases aged <7 years old. Design: Two prospective cohort studies that documented the MFM completion of children aged between 2 and 7 years old. Setting: French-speaking rehabilitation departments from France, Belgium, and Switzerland. Participants: Healthy children (n=194) and children with a neuromuscular disease (n=88). Interventions: Patients were rated by the MFM either once or twice by trained medical professionals, with a delay between the 2 MFMs ranging between 8 and 30 days. Main Outcome Measure: Intra- and interrater reliability of the MFM. Results: The subtests making up the MFM-32, a scale monitoring severity and progression of motor function in patients with a neuromuscular disease in 3 functional domains, were carried out in healthy children aged 2 to 7 years. Twenty items of the MFM-32 were successfully completed by these children and were used to constitute the MFM-20. Principal component analysis of the MFM-20 confirmed the 3 functional domains. Inter- and intrarater reliability of the 3 subscores and total score were high (intraclass correlation coefficient >.90), and discriminant validity was good. Conclusions: The MFM-20 can be used as an outcome measure for assessment of motor function in young children with neuromuscular disease. (C) 2013 by the American Congress of Rehabilitation Medicine
de Lattre C; Payan C; Vuillerot C; Rippert P; de Castro D; Berard C; Poirot I;MFM Study Group
Archives of Physical Medicine and Rehabilitation
2013
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<a href="http://doi.org/10.1016/j.apmr.2013.04.001" target="_blank" rel="noreferrer noopener">10.1016/j.apmr.2013.04.001</a>
Behavioural phenotypes of the mucopolysaccharide disorders: a systematic literature review of cognitive, motor, social, linguistic and behavioural presentation in the MPS disorders
children; Medicine; Endocrinology & Metabolism; Genetics & Heredity; Research & Experimental; syndrome; mental-retardation; annotation; clinical variability; hunters; mild; sanfilippo b-disease; behavior; tone and motor problems; MPSI; MPSII; MPSIIIA; MPSIIIB; trajectory; characteristics; sleep disturbance; challenging behavior
The mucopolysaccharide disorders (MPS) are a group of recessively inherited metabolic disorders resulting in progressive physical and cognitive decline. MEDLINE, PsycINFO and Embase databases were searched, alongside manual screening, to identify relevant literature. Papers were included in the review if they were published in a peer reviewed journal and conducted empirical research into cognitive, motor, social or linguistic development or behaviour in one or more MPS disorders. Twenty-five papers were reviewed. Two papers used methodology of a sufficiently high standard to demonstrate a behavioural phenotype; both found sleep disturbance to be part of the phenotype of MPS III. Fearfulness and sleep disturbance were frequently observed in people with MPS I and II. Cognitive and motor impairment and decline, and challenging behaviour were highly prevalent in the severe form of MPS II. Cognitive decline and severe behavioural problems relating to aggression, hyperactivity, orality, unusual affect and temper tantrums were seen in MPS III. Sleep disturbance is part of the behavioural phenotype of MPS III, and challenging behaviour is highly prevalent in MPS II and MPS III, therefore the efficacy of behavioural interventions for these populations should be investigated. Further research into the behaviour and adaptive skills of children with MPS III and MPS IV is required.
Cross E M; Hare D J
Journal of Inherited Metabolic Disease
2013
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<a href="http://doi.org/10.1007/s10545-012-9572-0" target="_blank" rel="noreferrer noopener">10.1007/s10545-012-9572-0</a>
Increased prevalence of sleep-disordered breathing in Friedreich ataxia
reliability; Neurosciences & Neurology; disease; scale; adults; apnea syndrome; daytime sleepiness; breathing difficulties; Friedreich's ataxia; trajectory; characteristics; obstructive sleep apnea
Objectives: We sought to document the prevalence and nature of sleep-disordered breathing (SDB) in individuals with Friedreich ataxia (FRDA) as well as establish the relationship, if any, between SDB and clinical parameters of FRDA. Methods: Eighty-two individuals with FRDA were administered the Epworth Sleepiness Scale on an annual basis for up to 3 years. Individuals were referred for a sleep study if they had an Epworth Sleepiness Scale score >8 or had clinical symptoms suggestive of SDB. Results: From this cohort, 21 individuals underwent a sleep study and 17 were diagnosed with obstructive sleep apnea syndrome, giving a minimum prevalence of 21%, which is greater than that found in the general population (3%-7%). Moreover, the presence of obstructive sleep apnea was significantly correlated with the duration of disease and clinical severity of FRDA. Conclusion: It is recommended that individuals with FRDA undergo regular screening for obstructive sleep apnea to identify the need for a sleep study and subsequent treatment if SDB is diagnosed.
Corben L A; Ho M; Copland J; Tai G; Delatycki M B
Neurology
2013
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<a href="http://doi.org/10.1212/WNL.0b013e318297ef18" target="_blank" rel="noreferrer noopener">10.1212/WNL.0b013e318297ef18</a>
Polysomnographic findings in Rett syndrome: a case-control study
Case-Control Studies; Child; Humans; Female; Reference Values; Sleep; Polysomnography; Signal Processing Computer-Assisted; Sleep REM; Rett Syndrome; Cerebral Cortex; Wakefulness; breathing difficulties; sleep disturbance; tone and motor problems; Rett syndrome; trajectory; characteristics; periodic limb movement; obstructive apnea
PURPOSE: Rett syndrome is a severe neurodevelopmental disorder mainly affecting females and usually linked to mutations in the methyl-CpG-binding protein 2 gene, with an estimated prevalence of 1 in 10,000 live female births. Clinical features which usually become more apparent over time include breathing dysfunction, seizures, spasticity, peripheral vasomotor disturbance, scoliosis, growth retardation, and hypotrophic feet, with a great variety of presentations. The clear immaturity in brainstem mechanisms is expressed by the presence of early sleep disorders such as nocturnal awakenings, bruxism, and difficulty falling asleep, and no conclusive findings were derived from the few polysomnographic studies about the sleep macrostructural aspects. The aim of this study is to analyze the sleep macrostructural parameters, the nocturnal respiratory characteristic, and the presence of periodic limb movements in a sample of children affected by Rett syndrome. MATERIALS: Thirteen Rett subjects underwent a polysomnographic study, and the findings were compared with those obtained by a group of 40 healthy children. RESULTS: The Rett group shows a great impairment in sleep macrostructural and respiratory parameters, with a higher percentage of pathological periodic limb movements than the controls. CONCLUSIONS: This study may be considered a report about the ventilatory impairment during sleep in Rett syndrome and the first approach to the macrostructural aspects of sleep supported by the PSG data that could be considered mandatory for a better comprehension of this very complex syndrome.
Carotenuto M; Esposito M; D'Aniello A; Rippa C D; Precenzano F; Pascotto A; Bravaccio C; Elia M
Sleep & Breathing
2013
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<a href="http://doi.org/10.1007/s11325-012-0654-x" target="_blank" rel="noreferrer noopener">10.1007/s11325-012-0654-x</a>
Feeding changes in children with trisomy 18: longitudinal data on primary feeding method and reflux identification and treatment
feeding difficulties; chromosome 18 trisomy; trajectory; characteristics; gastroesophageal reflux; GERD
Research indicates that approximately 40% to 70% of children with disabilities are identified and treated for feeding difficulties such as reflux. The available literature on children with trisomy 18 does not describe feeding needs or treatment. The results described here address primary feeding method along with identification and longitudinal treatment of gastroesophageal reflux in 10 children with trisomy 18. Data indicate videofluoroscopic swallowing study as the primary diagnostic procedure and treatment with medication as largely effective. The majority of the sample also moved to tube feedings to address feeding difficulties. It is hoped that this information is an initial step to further understanding the nutritional needs of this unique population. Implications for practice and a need for further research are recommended. Copyright © 2013 Lippincott Williams & Wilkins.
Bruns D A; Springer S A
Topics in Clinical Nutrition
2013
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<a href="http://doi.org/10.1097/01.TIN.0000437408.58941.d4" target="_blank" rel="noreferrer noopener">10.1097/01.TIN.0000437408.58941.d4</a>
Snake: the development and validation of a questionnaire on sleep disturbances in children with severe psychomotor impairment
Validity; Screening; Neurosciences & Neurology; psychometric properties; behaviors; scale; disabilities; Children with severe psychomotor impairment; Daytime restlessness; instrument; Parent questionnaire; school-aged children; Sleep disturbance/disorders; sleep disturbance; Q3 conditions; tool development; scale development; SNAKE
Objective: Sleep disturbance and daytime restlessness are present in 50% to 80% of children with severe psychomotor impairment due to neurologic or other complex diseases. Although these issues severely impair the quality of life of the children and their families, they are not well addressed or managed by professionals. The lack of validated assessment tools prevents further research and the development of adequate therapies. Our aim is to develop and validate a sleep questionnaire for these children that could be used both for clinical management and systematic research. Methods: The sleep questionnaire for children with severe psychomotor impairment (Schlaffragebogen fur Kinder mit Neurologischen und Anderen Komplexen Erkrankungen, SNAKE) is based on expert opinion and consultation with parents. The psychometric quality of the questionnaire was assessed in a sample of 224 children with severe psychomotor impairment. Results: Confirmative factor analysis showed that SNAKE comprises of five factors (based on ICSD-2). Fit indices, analysis of item characteristics and convergent validity (coherence with measures of sleep [i.e., sleep efficiency]) and correlation with selected subscales of the Sleep Disturbance Scale for Children (SDSC) were good. Re-test analysis (n = 62) depicted high stability and good replication of validity. Conclusions: SNAKE is a reliable and valid tool for the diagnosis of sleep disturbances in children with severe psychomotor impairment. The SNAKE questionnaire is the first tool that addresses the specific relationship between sleep disturbance and severe disability in children. (c) 2013 Elsevier B.V. All rights reserved.
Blankenburg M; Tietze A L; Hechler T; Hirschfeld G; Michel E; Koh M; Zernikow B
Sleep Medicine
2013
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<a href="http://doi.org/10.1016/j.sleep.2012.12.008" target="_blank" rel="noreferrer noopener">10.1016/j.sleep.2012.12.008</a>
Cultural and religious considerations in pediatric palliative care
OBJECTIVE: A growing multicultural society presents healthcare providers with a difficult task of providing appropriate care for individuals who have different life experiences, beliefs, value systems, religions, languages, and notions of healthcare. This is especially vital when end-of-life care is needed during childhood. There is a dearth of literature addressing cultural considerations in the pediatric palliative care field. As members of a specific culture often do not ascribe to the same religious traditions, the purpose of this article was to explore and review how culture and religion informs and shapes pediatric palliative care. SIGNIFICANCE OF RESULTS: The review of the literature provides insight into the influence of religion and how culture informs lifestyle and shapes the experiences of illness, pain, and end-of-life care. Recommendations for providing culturally sensitive end-of-life care are offered through the framework outlined in the Initiative for Pediatric Palliative Care Quality Improvement Project of 2002. Cultural traditions are dynamic, never static, and cannot be generalized to all families. Guidelines to aid in approaches to palliative care are provided, and providers are encouraged to define these important differences for each family under their care.
Wiener L; McConnell DG; Latella L; Ludi E
Palliative & Supportive Care
2013
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1017/S1478951511001027" target="_blank" rel="noreferrer noopener">10.1017/S1478951511001027</a>
When silence is not golden: Engaging adolescents and young adults in discussions around end-of-life care choices
Managing end-of-life care (EoL) is always emotionally taxing. Honest discussions about prognosis and EoL decision-making are often delayed due to questions about the legal competence of adolescents and young adults (AYA) to make decisions for themselves [1], providers feeling unprepared or without adequate skills to guide EoL discussions, or parental concern that discussing plans, including life support options or presenting an EoL planning document may send the message that the medical team wishes to withdraw care or that death is imminent. Data suggest that allowing AYA involvement in EoL planning can help parents and healthcare providers make informed decisions, alleviate distress, avoid decisional regret, and perhaps improve the patient’s quality of life (QoL) by respecting their values, beliefs ,and preferences [2–4]. This commentary addresses recent progress in the field and focuses on how healthcare professionals can approach advance care planning (ACP) with AYA, involve their family members, and engage the entire health care team.
Wiener L; Zadeh S; Wexler LH; Pao M
Pediatric Blood & Cancer
2013
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<a href="http://doi.org/10.1002/pbc.24490" target="_blank" rel="noreferrer noopener">10.1002/pbc.24490</a>
Why Don't Physicians (and Patients) Consistently Follow Clinical Practice Guidelines?: Comment on "Worsening Trends in the Management and Treatment of Back Pain"
Casey DE
Jama Internal Medicine
2013
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Journal Article
<a href="http://doi.org/10.1001/jamainternmed.2013.7672" target="_blank" rel="noreferrer">10.1001/jamainternmed.2013.7672</a>
Tackling taboo topics: how to have effective advanced care planning discussions with adolescents and young adults with cancer
ICU Decision Making
Walter JK; Rosenberg AR; Feudtner C
Jama Pediatrics
2013
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1001/jamapediatrics.2013.1323" target="_blank" rel="noreferrer">10.1001/jamapediatrics.2013.1323</a>
Clinician perspectives regarding the do-not-resuscitate order
Female; Humans; Male; decision making; Critical Illness; Data Collection; Attitude of Health Personnel; Physicians; Boston; Resuscitation Orders; Nurses; Heart Arrest; advance care planning
IMPORTANCE: While data exist regarding the frequency and timing of the do-not-resuscitate (DNR) order in children, little is known about clinician attitudes and behaviors regarding this order. OBJECTIVE: To identify clinician attitudes regarding the meaning, implication, and timing of the DNR order for pediatric patients. DESIGN: Physicians and nurses from practice settings where advance care planning typically takes place were surveyed regarding their attitudes and behaviors about DNR orders. RESULTS: In total, 107 physicians and 159 nurses responded to the survey (N = 266). There was substantial variability in the interpretation of the DNR order. Most clinicians (66.9%) believe that a DNR order indicates limitation of resuscitative measures only on cardiopulmonary arrest. In reality, however, more than 85% believe that care changes beyond response to cardiopulmonary arrest, varying from increased attention to comfort to less clinician attentiveness. In addition, most clinicians reported that resuscitation status discussions take place later in the illness course than is ideal. CONCLUSIONS AND RELEVANCE: Clinicians use the DNR order not only as a guide for therapeutic decisions during a cardiopulmonary arrest but also as a surrogate for broader treatment directives. Most clinicians believe that DNR discussions should take place earlier than they actually do. Interventions aimed at improving clinician knowledge and skills in advance care discussions as well as the development of orders that address overall goals of care may improve care for children with serious illness.
2013-10
Sanderson A; Zurakowski D; Wolfe J
Jama Pediatrics
2013
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Journal Article
<a href="http://doi.org/10.1001/jamapediatrics.2013.2204" target="_blank" rel="noreferrer">10.1001/jamapediatrics.2013.2204</a>
Family-centered advance care planning for teens with cancer
ICU Decision Making
IMPORTANCE Advance care planning (ACP) prepares patients and their families for future health care decisions; however, the needs of adolescent oncology patients for participation in ACP have not been well studied. OBJECTIVE To examine the efficacy of family-centered ACP. DESIGN AND SETTING Two-group randomized controlled trial in a pediatric oncology program. PARTICIPANTS Sixty adolescents aged 14 to 21 years with cancer and their surrogates or families were enrolled in the study between January 17, 2011, and March 29, 2012. INTERVENTION Thirty dyads received 3- to 60-minute sessions 1 week apart. Intervention dyads completed (1) the Lyon Family-Centered ACP Survey, (2) the Respecting Choices interview, and (3) Five Wishes. Control subjects received standard care plus information. MAIN OUTCOME MEASURES Statement of treatment preferences and Decisional Conflict Scale score. RESULTS The mean age of the adolescents was 16 years; 36 (60%) were male, 30 (50%) white, 26 (43%) black, and 4 (7%) Asian. Diagnoses were as follows: leukemia (14 patients [47%]), brain tumor (8 [27%]), solid tumor (6 [20%]), and lymphoma (2 [7%]). Significantly increased congruence was observed for intervention dyads compared with controls for 4 of the 6 disease-specific scenarios; for example, for situation 2 ("treatment would extend my life by not more than 2 to 3 months"), intervention dyads demonstrated higher congruence (kappa = 0.660; P < .001) vs control dyads (kappa = -0.0636; P = .70). Intervention adolescents (100%) wanted their families to do what is best at the time, whereas fewer control adolescents (62%) gave families this leeway. Intervention adolescents were significantly better informed about end-of-life decisions (t = 2.93; effect size, 0.961; 95% CI, 0.742-1.180; P = .007). Intervention families were more likely to concur on limiting treatments than controls. An ethnic difference was found in only one situation. CONCLUSIONS Advance care planning enabled families to understand and honor their adolescents' wishes. Intervention dyads were more likely than controls to limit treatments. Underserved African American families were willing to participate.
Lyon ME; Jacobs S; Briggs L; Cheng YI; Wang J
Jama Pediatrics
2013
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Journal Article
<a href="http://doi.org/10.1001/jamapediatrics.2013.943" target="_blank" rel="noreferrer">10.1001/jamapediatrics.2013.943</a>
Our children are not a diagnosis: The experience of parents who continue their pregnancy after a prenatal diagnosis of trisomy 13 or 18
Trisomy 13 and trisomy 18 (T13-18) are associated with high rates of perinatal death and with severe disability among survivors. Prenatal diagnosis (PND) may lead many women to terminate their pregnancy but some women choose to continue their pregnancy. We sent 503 invitations to answer a questionnaire to parents who belong to T13 and 18 internet support groups. Using mixed methods, we asked parents about their prenatal experience, their hopes, the life of their affected child, and their family experience. 332 parents answered questions about 272 children; 128 experienced PND. These parents, despite feeling pressure to terminate (61%) and being told that their baby would likely die before birth (94%), chose to continue the pregnancy. Their reasons included: moral beliefs (68%), child-centered reasons (64%), religious beliefs (48%), parent-centered reasons (28%), and practical reasons (6%). At the time of the diagnosis, most of these parents (80%) hoped to meet their child alive. By the time of birth, 25% chose a plan of full interventions. A choice of interventions at birth was associated with fewer major anomalies (P < 0.05). Parents describe "Special" healthcare providers as those who gave balanced and personalized information, respected their choice, and provided support. Parents make decisions to continue a pregnancy and choose a plan of care for their child according to their beliefs and their child's specific medical condition, respectively. Insights from parents' perspective can better enable healthcare providers to counsel and support families. © 2013 Wiley Periodicals, Inc.
2013-12
Guon J; Wilfond BS; Farlow B; Brazg T; Janvier A
American Journal Of Medical Genetics.Part A
2013
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Journal Article
<a href="http://doi.org/10.1002/ajmg.a.36298" target="_blank" rel="noreferrer">10.1002/ajmg.a.36298</a>
Life at the interface: adults with "pediatric" disorders of the nervous system
Child; Humans; Neoplasms; Adult; Cerebral Palsy; Epilepsy; Cystic Fibrosis; Heart Defects; Child Development Disorders; Congenital; Pervasive
The increasing longevity of patients with congenital and developmental disorders of the nervous system reflects the palliative and social success of pediatrics in the past 2 decades. This success has resulted in an increasing number of adult patients with residua or sequelae of childhood disease and/or its treatment. It is critically important that residencies and subspecialty fellowships train a cadre of physicians to prepare patients and families for the transition of children with special health care needs to adulthood and to attend to their unique medical, psychological, and social concerns. Health services and education research must better define the needs of this growing population and the best ways to educate their physicians and families and empower them to become as independent as their fullest potential allows.
2013-08
Schor NF
Annals Of Neurology
2013
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Journal Article
<a href="http://doi.org/10.1002/ana.23910" target="_blank" rel="noreferrer">10.1002/ana.23910</a>
Percutaneous transluminal pulmonary valvuloplasty in a child with tricuspid atresia, ventricular septal defect, and severe pulmonary valve stenosis: Usefulness of the Femoral Artery Approach
Percutaneous transluminal pulmonary valvuloplasty may be indicated in not only isolated pulmonary valve stenosis, but also complex congenital heart diseases. Because palliative surgery for increasing pulmonary blood flow entails a risk of scar formation and immediate postoperative complications, catheter intervention is preferred, if possible. However, an acute-angled, twisted, or tortuous access route or a small valve orifice occasionally makes it difficult for the catheter to reach or cross the target. We succeeded in performing this intervention for such a complex stenosis effectively and safely in a patient with tricuspid atresia, ventricular septal defect (VSD), and severe pulmonary valve stenosis, thereby evading surgery. In previous reports, the catheter for this cardiac anomaly was accessed via the femoral vein. In the present case, the catheter was advanced through the femoral artery via the aorta, left ventricle, VSD, and right ventricle to the pulmonary valve, using a micro-catheter in a telescopic manner, in combination with a coronary balloon dilatation catheter. This maneuver, which has not been reported previously, made it much easier to perform the procedure as compared to the femoral vein approach, despite the acute turn and the pinhole orifice. Moreover, reported complications of the femoral vein approach, including bradycardia, hypotension, and valve regurgitation, were not observed in this case. We conclude that the femoral artery approach can be a safe and effective alternative in patients for whom a more conventional procedure has been unsuccessful. © 2013 Wiley Periodicals, Inc.
2013-09
Torigoe T; Sato S; Kanazawa H
Catheterization And Cardiovascular Interventions
2013
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Journal Article
<a href="http://doi.org/10.1002/ccd.25193" target="_blank" rel="noreferrer">10.1002/ccd.25193</a>
Racial and ethnic differences in hospice enrollment among children with cancer
adolescent; Child; Female; Humans; Male; Neoplasms; hospice care; Adult; Patient Acceptance of Health Care; Hispanic Americans; Preschool; Texas
BACKGROUND: Hospice is an important provider of end of life care. Adult minorities are less likely to enroll on hospice; little is known regarding the prevalence of pediatric hospice use or the characteristics of its users. Our primary objective was to determine whether race/ethnicity was associated with hospice enrollment in children with cancer. We hypothesized that minority (Latino) race/ethnicity is negatively associated with hospice enrollment in children with cancer. PROCEDURE: In this single-center retrospective cohort study, inclusion criteria were patients who died of cancer or stem cell transplant between January 1, 2006 and December 31, 2010. The primary outcome variable was hospice enrollment and primary predictor was race/ethnicity. RESULTS: Of the 202 patients initially identified, 114 met inclusion criteria, of whom 95 were enrolled on hospice. Patient race/ethnicity was significantly associated with hospice enrollment (P = 0.02), the association remained significant (P = 0.024) after controlling for payor status (P = 0.995), patient diagnosis (P = 0.007), or religion (P = 0.921). Latinos enrolled on hospice significantly more often than patients of other races. Despite initial enrollment on hospice however, 34% of Latinos and 50% of non-Latinos had withdrawn from hospice at the time of death (P = 0.10). Race/ethnicity was not significantly associated with dying on hospice. CONCLUSIONS: These results indicate that race/ethnicity and diagnosis are likely to play a role in hospice enrollment during childhood. A striking number of patients of all race/ethnicities left hospice prior to death. More studies describing the impact of culture on end of life decision-making and the hospice experience in childhood are warranted.
2013-10
Thienprayoon R; Lee SC; Leonard D; Winick N
Pediatric Blood & Cancer
2013
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Journal Article
<a href="http://doi.org/10.1002/pbc.24590" target="_blank" rel="noreferrer">10.1002/pbc.24590</a>
Physical activity (PA) and sleep among children and adolescents with cancer
adolescent; Child; Female; Humans; Male; Neoplasms; Fatigue; Motor Activity; Sleep; Actigraphy
BACKGROUND: Although sleep and physical activity often are impaired among adult cancer patients, there is limited data among pediatric oncology populations. We conducted a prospective study to investigate the relationship between physical activity (PA) and sleep among children with cancer. PROCEDURE: Between 11/12/09 and 02/06/12, PA while awake and sleep variables were assessed by actigraphy collected over 7 days in 36 children (age range 8-18 years) with cancer (23 leukemia/lymphoma, 5 brain tumor, 8 solid tumor). Sleep diaries were used to determine sleep time, sleep quality, and morning mood. Fatigue was assessed at study initiation using fatigue instruments. RESULTS: Participants had impaired sleep based upon normative data compiled from multiple studies of more than 1,700 healthy children from 1 to 18 years of age [1], including decreased total sleep time (mean 6.6 hours, standard deviation (SD) 1.3 hours), increased wake after sleep onset (WASO; mean 2 hours, SD 1.4 hours), increased awakenings during sleep (mean 28.3 wake bouts, SD 7.8 bouts), and decreased sleep efficiency (mean 74.2%, SD 13.3%). Fatigue correlated with self-reported sleep quality but not with disturbances in sleep as measured by actigraphy. In longitudinal models that controlled for age, diagnosis group, gender, race, and steroid use, higher average activity, as measured by actigraphy, was associated with improved sleep quantity (P = 0.005) and efficiency (P = 0.001). CONCLUSION: Pediatric oncology patients demonstrate impaired sleep. Greater PA was significantly associated with improved sleep quantity and efficiency in pediatric oncology participants. As a potentially modifiable factor, PA may offer a mechanism to improve sleep in pediatric oncology patients.
2013-11
Orsey AD; Wakefield DB; Cloutier MM
Pediatric Blood & Cancer
2013
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Journal Article
<a href="http://doi.org/10.1002/pbc.24641" target="_blank" rel="noreferrer">10.1002/pbc.24641</a>
Parent perceptions of the quality of information received about a child's cancer
adolescent; Child; Cross-Sectional Studies; Female; Humans; infant; Male; Neoplasms; Adult; Parents; Questionnaires; Professional-Family Relations; Preschool; Health Communication
BACKGROUND: Parents' perceptions of the quality of information communicated by their child's oncologist about the child's cancer are not well understood. PROCEDURE: We conducted a cross-sectional survey of 194 parents of children with cancer (response rate 70%), treated at the Dana-Farber Cancer Institute and Boston Children's Hospital, Boston, Mass, and the children's physicians. Parents were asked to report the quality of information received about the child's cancer in several domains. RESULTS: Most parents reported that information about their child's cancer had been excellent (49%) or good (41%) overall, and ratings were similar for information about diagnosis (P = 0.62) and treatment (P = 0.59). Fewer parents felt they received high quality information about how cancer treatment is working (P < 0.001), likelihood of cure (P < 0.001), what the diagnosis means for the future (P < 0.001), and whether there is a cause for the child's cancer (P < 0.001). In multivariable models, parents were more likely to consider information of high quality when they also rated physician communication style highly. The accuracy of their knowledge on likelihood of cure and the child's future limitations, however, was not associated with quality ratings. CONCLUSIONS: Although parents feel that they have received high quality cancer information overall, parents feel they receive lower quality information about issues relating to the child's future. Yet quality ratings are not associated with their actual knowledge. Parent perceptions of quality represent one, but not the only, facet of communication quality.
2013-11
Kaye E; Mack JW
Pediatric Blood & Cancer
2013
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Journal Article
<a href="http://doi.org/10.1002/pbc.24652" target="_blank" rel="noreferrer">10.1002/pbc.24652</a>
Pediatric oncology providers' perceptions of barriers and facilitators to early integration of pediatric palliative care
Female; Humans; Male; Palliative Care; Neoplasms; Pediatrics; Adult; Aged; Middle Aged; Health Personnel; Focus Groups; Medical Oncology; Practice; Palliative Care; Attitudes; Health Knowledge
BACKGROUND: Pediatric patients experience significant symptoms during cancer treatment. Symptom management is frequently inadequate. We studied perceptions of pediatric oncology care providers regarding early integration of palliative care (PC) for pediatric patients to identify barriers and facilitators that might assist in understanding how care could be improved. PROCEDURES: Pediatric oncology providers were recruited to participate in four focus groups. A proposal for early integration of a pediatric palliative care team (PPCT) was presented and followed by a facilitated discussion. Data were analytically categorized into themes by three independent coders using constant comparative analysis and crystallization techniques. A consensus approach was used to identify final themes. RESULTS: Barriers to the proposed care model of early integration of a PPCT included provider role, conflicting philosophy, patient readiness, and emotional influence and were more prevalent in the physician participants compared to nurse practitioner, nursing, and social work participants. Facilitators included patient eligibility, improved patient care, education, and evidence-based medicine. Though all participants were invested in providing optimal patient care, physician participants believed the current standard of care model is meeting the needs of patients and family, while the nurse practitioner, nursing, and social work participants working on the same healthcare team believed the proposed care model would improve the overall care of children diagnosed with cancer. CONCLUSIONS: Differing perceptions among healthcare providers regarding the care of children with cancer suggest that team functioning could be improved. Avenues for pilot testing early integration of PC could provide useful information for a next study.
2013-11
Dalberg T; Jacob-Files E; Carney PA; Meyrowitz J; Fromme EK; Thomas G
Pediatric Blood & Cancer
2013
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Journal Article
<a href="http://doi.org/10.1002/pbc.24673" target="_blank" rel="noreferrer">10.1002/pbc.24673</a>
Fatigue in adolescents with cancer compared to healthy adolescents
adolescent; Female; Humans; Male; Neoplasms; Fatigue; Affect; Depression
BACKGROUND: Cancer-related fatigue is one of the most pervasive and debilitating side-effects of cancer treatment and adolescents consistently rate cancer-related fatigue as one of the most distressing aspects of treatment. Because fatigue is also high in adolescents without cancer, the current study aims to describe fatigue in adolescents with cancer relative to a control group and to identify associates of such fatigue. Knowing this is important for understanding the extent of the problem in adolescents with cancer relative to healthy adolescents and for understanding who is most at risk for fatigue and related distress. PROCEDURE: Adolescents with cancer and their caregivers (n = 102) and adolescents without a history of chronic health conditions and their caregivers (n = 97) completed the Multidimensional Fatigue Scale and measures of depression, quality of life (QoL), affect, coping, and family functioning. RESULTS: Adolescents with cancer and their caregivers reported significantly more adolescent fatigue across all domains (with the exception of adolescent reports of cognitive fatigue) relative to adolescents without chronic health conditions. Higher fatigue was significantly related to adolescent report of more symptoms of depression, poorer QoL, higher negative affect, less positive affect, and behavioral disengagement coping style. Fatigue was not related to active coping or family functioning. CONCLUSIONS: Adolescents with cancer experience significantly more fatigue than peers without chronic health conditions. Reports of fatigue are closely related to multiple indicators of psychosocial well-being, suggesting that fatigue may be an important cancer-related symptom to assess and manage to improve adolescent QoL.
2013-11
Daniel LC; Brumley LD; Schwartz LA
Pediatric Blood & Cancer
2013
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Journal Article
<a href="http://doi.org/10.1002/pbc.24706" target="_blank" rel="noreferrer">10.1002/pbc.24706</a>
Parent and clinician preferences for location of end-of-life care: Home, hospital or freestanding hospice?
adolescent; Child; Female; Humans; Male; Neoplasms; Terminal Care; home care services; hospice care; Parents; Prognosis; Questionnaires; Follow-Up Studies; Attitude to Death; Physicians; Choice Behavior; Residence Characteristics; Hospitals; quality of life; Preschool
BACKGROUND: Current options for location of end-of-life (EOL) care for children with cancer include home, hospital, and freestanding pediatric hospice (FSPH). However, access to these options varies greatly depending on geographical location. We aimed to determine bereaved parent and clinician preferences for location to EOL care and death. PROCEDURE: We administered questionnaires to 75 bereaved parents (response rate 54%) and 48 pediatric oncology clinicians (response rate 91%) at a large teaching hospital. Main outcome measure was parent and clinician ranking for preferred location of EOL care and death if given the options of home, hospital or FSPH. RESULTS: Majority of parents and clinicians ranked home as their first choice for EOL care (70.2% and 87%, respectively) and death (70.8% and 89.1%, respectively). Compared to clinicians, parents gave a higher ranking to hospital (P < 0.01) and lower ranking to FSPH (P < 0.01) as the preferred location for EOL care and death. Congruence between actual and preferred location of EOL care was more likely when a palliative care team was involved (P < 0.01) and less likely for children with haematologic malignancies (P = 0.03). CONCLUSIONS: Parents and clinicians prefer home as the location for EOL care and death for children with cancer. Hospital based palliative care is a preferred alternative if home is not desired. FSPH is a relatively recent phenomena and further research needs to be directed towards understanding its cost benefit in comparison to home and hospital-based EOL care. Pediatr Blood Cancer © 2013 Wiley Periodicals, Inc.
2013-11
Kassam A; Skiadaresis J; Alexander S; Wolfe J
Pediatric Blood & Cancer
2013
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Journal Article
<a href="http://doi.org/10.1002/pbc.24872" target="_blank" rel="noreferrer">10.1002/pbc.24872</a>
Safety and efficacy of aprepitant for chemotherapy-induced nausea and vomiting in pediatric patients: A prospective, observational study
Pediatric patients between the ages of 12 months and 17 years with a confirmed malignancy who were scheduled to receive aprepitant as part of triple therapy antiemetic prophylaxis for a cycle of moderately- or highly emetogenic chemotherapy were eligible for enrollment. Patients were evaluated for the incidence of nausea, episodes of emesis, interference with activities of daily living (ADLs), and appetite through utilization of a patient survey. Eleven patients were enrolled for a total of 20 patient encounters, mean age 9.55 ± 4.85 (range, 12 months-17 years). Aprepitant was well-tolerated and complete response (CR) rate was 38.9%. Pediatr Blood Cancer © 2013 Wiley Periodicals, Inc.
2013-12
Bodge M; Shillingburg A; Paul S; Biondo L
Pediatric Blood & Cancer
2013
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Journal Article
<a href="http://doi.org/10.1002/pbc.24901" target="_blank" rel="noreferrer">10.1002/pbc.24901</a>
Neurogenetic disorders and treatment of associated seizures.
Humans; Treatment Outcome; Epilepsies; Angelman Syndrome/dt [Drug Therapy]; Anticonvulsants/tu [Therapeutic Use]; Epilepsies; Rett Syndrome/dt [Drug Therapy]; Seizures/dt [Drug Therapy]; Tuberous Sclerosis/dt [Drug Therapy]; Angelman Syndrome/co [Complications]; Anticonvulsants/ad [Administration & Dosage]; Anticonvulsants/ae [Adverse Effects]; KT Synthesis; Myoclonic/co [Complications]; Myoclonic/dt [Drug Therapy]; Rett Syndrome/co [Complications]; Seizures/co [Complications]; Tuberous Sclerosis/co [Complications]
Seizures are a frequent complication associated with several neurogenetic disorders. Antiepileptic medications remain the mainstay of treatment in these patients. We summarized the available data associated with various antiepileptic therapies used to treat patients with neurogenetic disorders who experienced recurrent seizures. A MEDLINE search was conducted to identify articles and abstracts describing the use of antiepileptic therapy for the treatment of various neurogenetic syndromes. Of all the neurogenetic syndromes, only autism spectrum disorders, Angelman syndrome, Rett syndrome, Dravet syndrome, and tuberous sclerosis complex were identified as having sufficient published information to evaluate therapy. Some efficacy trends were identified, including frequent successes with valproic acid with clonazepam for epilepsy with Angelman syndrome; valproic acid, stiripentol, and clobazam (triple combination therapy) for epilepsy with Dravet syndrome; and vigabatrin for infantile spasms associated with tuberous sclerosis complex. Due to a paucity of information regarding the mechanisms by which seizures are generated in the various disorders, approach to seizure control is primarily based on clinical experience and a limited amount of study data exploring patient outcomes. Although exposure of the developing brain to antiepileptic medications is of some concern, the control of epileptic activity is an important undertaking in these individuals, as the severity of eventual developmental delay often appears to correlate with the severity of seizures. As such, early aggressive therapy is warranted. 2013 Pharmacotherapy Publications, Inc.
2013
Faulkner MA; Singh Sanjay P
Pharmacotherapy
2013
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Journal Article
<a href="http://doi.org/10.1002/phar.1201" target="_blank" rel="noreferrer">10.1002/phar.1201</a>
Pharmacotherapy challenges of Fontan-associated plastic bronchitis: a rare pediatric disease
Child; Humans; Rare Diseases; Bronchitis; Fontan Procedure
Pediatric pharmacotherapy is often challenging due to the paucity of available clinical data on the safety and efficacy of drugs that are commonly used in children. This quandary is even more prevalent in children with rare diseases. Although extrapolations for dosing and administration are often made from available adult data with similar disease states, this translation becomes even more problematic in rare pediatric diseases. Understanding of rare disease pathophysiology is typically poor, and few, if any, effective therapies have been studied and identified. One condition that illustrates these issues is plastic bronchitis, a rare, most often pediatric disease that is characterized by the production of obstructive bronchial airway casts. This illness primarily occurs in children with congenital heart disease, often after palliative surgery. Plastic bronchitis is a highly clinically relevant and therapeutically challenging problem with a high mortality rate, and, a generally accepted effective pharmacotherapy regimen has yet to be identified. Furthermore, the disease is ill defined, which makes timely identification and treatment of children with plastic bronchitis difficult. The pharmacotherapies currently used to manage this disease are largely anecdotal and vary between the use of macrolide antibiotics, mucolytics, bronchodilators, and inhaled fibrinolytics in a myriad of combinations. The purpose of this review is 2-fold: first, to highlight the dilemma of treating plastic bronchitis, and second, to bring attention to the continuing need for studies of drug therapies used in children so safe and effective drug regimens can be established, particularly for rare diseases.
2013-09
Brooks K; Caruthers RL; Schumacher KR; Stringer KA
Pharmacotherapy
2013
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Journal Article
<a href="http://doi.org/10.1002/phar.1290" target="_blank" rel="noreferrer">10.1002/phar.1290</a>
Pain in cognitively impaired children: a focus for general pediatricians.
Child; Humans; Pain Measurement; quality of life; Analgesics/tu [Therapeutic Use]; Cerebral Palsy/co [Complications]; Cognition Disorders/co [Complications]; Pain/et [Etiology]; Pain/di [Diagnosis]; Pain/dt [Drug Therapy]
UNLABELLED: Pain in children with cognitive impairment and cerebral palsy is a particularly relevant issue due to its high prevalence and impact on quality of life. We review available evidence about prevalence of pain, causes and specific treatment, recognition and use of specific pain scales, physiology, and consequences of pain in this subset of patients., CONCLUSIONS: Pain is very common and is a critical determinant of quality of life in children with cognitive impairment and cerebral palsy. The diseases and associated complications that frequently expose these patients to pain can be treated and pain prevented. For patients with communication difficulties, appropriate, effective, validated tools are available and should be used to diagnose pain in itself, to >choose analgesic treatment and to determine effectiveness of these therapies. The level of awareness of pediatricians towards this issue seems to be quite low.
2013
Pastore S; Ventura A; Barbi E
European Journal Of Pediatrics
2013
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1007/s00431-012-1720-x" target="_blank" rel="noreferrer">10.1007/s00431-012-1720-x</a>
Continuous sedation until death: moral justifications of physicians and nurses--a content analysis of opinion pieces
Humans; Palliative Care; Terminal Care; Deep Sedation; Physicians; Morals; Terminology as Topic; Ethics; Conscious Sedation; Nursing
Continuous sedation until death (CSD), the act of reducing or removing the consciousness of an incurably ill patient until death, often provokes medical-ethical discussions in the opinion sections of medical and nursing journals. A content analysis of opinion pieces in medical and nursing literature was conducted to examine how clinicians define and describe CSD, and how they justify this practice morally. Most publications were written by physicians and published in palliative or general medicine journals. Terminal Sedation and Palliative Sedation are the most frequently used terms to describe CSD. Seventeen definitions with varying content were identified. CSD was found to be morally justified in 73% of the publications using justifications such as Last Resort, Doctrine of Double Effect, Sanctity of Life, Autonomy, and Proportionality. The debate over CSD in the opinion sections of medical and nursing journals lacks uniform terms and definitions, and is profoundly marked by 'charged language', aiming at realizing agreement in attitude towards CSD. Not all of the moral justifications found are equally straightforward. To enable a more effective debate, the terms, definitions and justifications for CSD need to be further clarified.
2013-08
Rys S; Mortier F; Deliens L; Deschepper R; Battin MP; Bilsen J
Medicine, Health Care And Philosophy
2013
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1007/s11019-012-9444-2" target="_blank" rel="noreferrer">10.1007/s11019-012-9444-2</a>
Psychological resilience, pain catastrophizing, and positive emotions: perspectives on comprehensive modeling of individual pain adaptation
Female; Humans; Male; Pain; Pain Measurement; Emotions; Cognition; Catastrophization; Motivation; Adaptation; Psychological; Models; Resilience
Pain is a complex construct that contributes to profound physical and psychological dysfunction, particularly in individuals coping with chronic pain. The current paper builds upon previous research, describes a balanced conceptual model that integrates aspects of both psychological vulnerability and resilience to pain, and reviews protective and exacerbating psychosocial factors to the process of adaptation to chronic pain, including pain catastrophizing, pain acceptance, and positive psychological resources predictive of enhanced pain coping. The current paper identifies future directions for research that will further enrich the understanding of pain adaptation and espouses an approach that will enhance the ecological validity of psychological pain coping models, including introduction of advanced statistical and conceptual models that integrate behavioral, cognitive, information processing, motivational and affective theories of pain.
2013-03
Sturgeon JA; Zautra AJ
Current Pain And Headache Reports
2013
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1007/s11916-012-0317-4" target="_blank" rel="noreferrer">10.1007/s11916-012-0317-4</a>
Medical ethics in pediatric critical care
Child; Humans; United States; Terminal Care; decision making; Parental Consent; Personal Autonomy; Pediatrics; Withholding Treatment; Professional-Family Relations; Dissent and Disputes; Truth Disclosure; Critical Care; Tissue and Organ Procurement; Ethics; Medical; Patient Rights; Clinical; Ethics Committees; Resource Allocation
Ethically charged situations are common in pediatric critical care. Most situations can be managed with minimal controversy within the medical team or between the team and patients/families. Familiarity with institutional resources, such as hospital ethics committees, and national guidelines, such as publications from the American Academy of Pediatrics, American Medical Association, or Society of Critical Care Medicine, are an essential part of the toolkit of any intensivist. Open discussion with colleagues and within the multidisciplinary team can also ensure that when difficult situations arise, they are addressed in a proactive, evidence-based, and collegial manner.
2013-04
Orioles A; Morrison WE
Critical Care Clinics
2013
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1016/j.ccc.2012.12.002" target="_blank" rel="noreferrer">10.1016/j.ccc.2012.12.002</a>
Suffering indicators in terminally ill children from the parental perspective
Child; Female; Humans; Male; Neoplasms; Terminal Care; Terminally Ill; Adult; Parent-Child Relations; Parents; Interviews as Topic; Attitude to Death; Qualitative Research; Spain; Stress; quality of life; Adaptation; Psychological
PURPOSE: Suffering is a complex multifaceted phenomenon, which has received limited attention in relation to children with terminal illness. As part of a wider study we interviewed parents of children with terminal illness to elicit their perspectives on suffering, in order to provide initial understanding from which to develop observational indicators and further research. METHODS: Qualitative descriptive study with semi-structured interviews made "ad hoc". Selection through deliberate sampling of mothers and fathers of hospitalised children (0-16 years old) with a terminal illness in Granada (Spain). KEY RESULTS: 13 parents were interviewed. They described children's suffering as manifested through sadness, apathy, and anger towards their parents and the professionals. The isolation from their natural environment, the uncertainty towards the future, and the anticipation of pain caused suffering in children. The pain is experienced as an assault that their parents allow to occur. CONCLUSIONS: The analysis of the interview with the parents about their perception of their ill children's suffering at the end of their lives is a valuable source of information to consider supportive interventions for children and parents in health care settings. An outline summary of the assessed aspects of suffering, the indicators and aspects for health professional consideration is proposed.
2013-12
Montoya-Juárez R; García-Caro MP; Schmidt-Rio-Valle J; Campos-Calderón C; Sorroche-Navarro C; Sánchez-García R; Cruz-Quintana F
European Journal Of Oncology Nursing: The Official Journal Of European Oncology Nursing Society
2013
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1016/j.ejon.2013.04.004" target="_blank" rel="noreferrer">10.1016/j.ejon.2013.04.004</a>
GRADE guidelines: 15. Going from evidence to recommendation-determinants of a recommendation's direction and strength
Humans; United States; Canada; Practice Guidelines as Topic; Treatment Outcome; Research Design; Risk Assessment; Evidence-Based Medicine; Treatment Failure; Germany; Pulmonary Disease; Health Care; Quality Assurance; Chronic Obstructive
In the GRADE approach, the strength of a recommendation reflects the extent to which we can be confident that the composite desirable effects of a management strategy outweigh the composite undesirable effects. This article addresses GRADE's approach to determining the direction and strength of a recommendation. The GRADE describes the balance of desirable and undesirable outcomes of interest among alternative management strategies depending on four domains, namely estimates of effect for desirable and undesirable outcomes of interest, confidence in the estimates of effect, estimates of values and preferences, and resource use. Ultimately, guideline panels must use judgment in integrating these factors to make a strong or weak recommendation for or against an intervention.
2013-07
Andrews JC; Schunemann HJ; Oxman A; Pottie K; Meerpohl Joerg J; Coello PA; Rind D; Montori VM; Brito JP; Norris S; Elbarbary M; Post P; Nasser M; Shukla V; Jaeschke R; Brozek J; Djulbegovic B; Guyatt G
Journal Of Clinical Epidemiology
2013
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1016/j.jclinepi.2013.02.003" target="_blank" rel="noreferrer">10.1016/j.jclinepi.2013.02.003</a>