Longitudinal Hand Function in Rett Syndrome
tone and motor problems; Rett syndrome; trajectory; characteristics
Loss of hand function is a core feature of Rett syndrome. This article describes longitudinal hand function at 3 time points for 72 subjects participating in the Australian Rett Syndrome Database. Approximately 40% of subjects with some grasping abilities lost skill over the 3- to 4-year period between video assessments. In these subjects, a decrease in hand function was seen less frequently in girls 13 to 19 years old than in those younger than 8 years, in subjects with some mobility compared with those who were wheelchair bound, and in those who had previously been able to finger feed. Relationships with the magnitude of change reflected these findings. Change in hand function did not vary with clinical severity. The results for all subjects were similar to results obtained when analysis was restricted to those with a pathogenic mutation. Variability in the longitudinal course of hand function in Rett syndrome was observed.
Downs J; Bebbington A; Kaufmann W E; Leonard H
Journal of Child Neurology
2011
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/10.1177/0883073810381920" target="_blank" rel="noreferrer noopener">10.1177/0883073810381920</a>
Quantifying physical decline in juvenile neuronal ceroid lipofuscinosis (Batten disease)
Cross-Sectional Studies; Disease Progression; Young Adult; Child; Humans; Adult; Prospective Studies; Adolescent; Child Preschool; Regression Analysis; Reproducibility of Results; Analysis of Variance; Neuropsychological Tests; Mutation; Disabled Persons; Genotype; Homozygote; Membrane Glycoproteins; Molecular Chaperones; Neuronal Ceroid-Lipofuscinoses; tone and motor problems; NCL3; tool development; scale development; UBDRS
OBJECTIVE: To use the Unified Batten Disease Rating Scale (UBDRS) to measure the rate of decline in physical and functional capability domains in patients with juvenile neuronal ceroid lipofuscinosis (JNCL) or Batten disease, a neurodegenerative lysosomal storage disorder. We have evaluated the UBDRS in subjects with JNCL since 2002; during that time, the scale has been refined to improve reliability and validity. Now that therapies are being proposed to prevent, slow, or reverse the course of JNCL, the UBDRS will play an important role in quantitatively assessing clinical outcomes in research trials. METHODS: We administered the UBDRS to 82 subjects with JNCL genetically confirmed by CLN3 mutational analysis. Forty-four subjects were seen for more than one annual visit. From these data, the rate of physical impairment over time was quantified using multivariate linear regression and repeated-measures analysis. RESULTS: The UBDRS Physical Impairment subscale shows worsening over time that proceeds at a quantifiable linear rate in the years following initial onset of clinical symptoms. This deterioration correlates with functional capability and is not influenced by CLN3 genotype. CONCLUSION: The UBDRS is a reliable and valid instrument that measures clinical progression in JNCL. Our data support the use of the UBDRS to quantify the rate of progression of physical impairment in subjects with JNCL in clinical trials.
Kwon J M; Adams H; Rothberg P G; Augustine E F; Marshall F J; Deblieck E A; Vierhile A; Beck C A; Newhouse N J; Cialone J; Levy E; Ramirez-Montealegre D; Dure L S; Rose K R; Mink J W
Neurology
2011
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<a href="http://doi.org/10.1212/WNL.0b013e318237f649" target="_blank" rel="noreferrer noopener">10.1212/WNL.0b013e318237f649</a>
Needs based palliative care commissioning for children, young people, and their families
ACT
2011
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Journal Article
Respiratory events and obstructive sleep apnea in children with achondroplasia: investigation and treatment outcomes
Cross-Sectional Studies; Age Factors; Male; Treatment Outcome; Comorbidity; Child; Humans; Adolescent; Cohort Studies; Female; Child Preschool; Infant; Polysomnography; Body Mass Index; Achondroplasia/di [Diagnosis]; Achondroplasia/th [Therapy]; Sleep Apnea Central/di [Diagnosis]; Sleep Apnea Central/th [Therapy]; Sleep Apnea Obstructive/di [Diagnosis]; Sleep Apnea Obstructive/th [Therapy]; Achondroplasia/ep [Epidemiology]; Airway Obstruction/di [Diagnosis]; Airway Obstruction/ep [Epidemiology]; Airway Obstruction/th [Therapy]; Sleep Apnea Central/ep [Epidemiology]; Sleep Apnea Obstructive/ep [Epidemiology]; breathing difficulties; achondroplasia; trajectory; characteristics; obstructive sleep apnea
PURPOSE: We report aspects of sleep-disordered breathing in a cohort of achondroplastic children who attended our hospital. METHODS: A retrospective chart review was conducted for a 15-year period to further evaluate the diagnosis and treatment of sleep-disordered breathing in children with achondroplasia. RESULTS: A review of the medical records was undertaken for 46 children (63%, mean age 3.9 years) with achondroplasia that had overnight polysomnography. Among them, 25 (54.3%) had obstructive sleep apnea (OSA). For 19 out of 46 patients (follow-up rate, 41.3%) with a mean follow-up of 31.3 months (range, 3 month to 11 years), 13 had undergone adenotonsillectomy, while nine were treated with continuous positive airway pressure. CONCLUSIONS: Prospective evaluation of our clinic population confirms a high incidence of SDB in achondroplastic children. OSA has been linked to raise intracranial pressure as well as neurocognitive deficits in children and we hypothesize that associations between neurological and respiratory abnormalities in this disorder are a consequence of the early onset of associated respiratory, rather than the neurological complications.
Afsharpaiman S; Sillence D O; Sheikhvatan M; Ault J E; Waters K
Sleep and Breathing
2011
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<a href="http://doi.org/10.1007/s11325-010-0432-6" target="_blank" rel="noreferrer noopener">10.1007/s11325-010-0432-6</a>
Development and implementation of a bereavement follow-up intervention for grieving fathers: an action research.
Humans; Male; Health Services Research; Finland; Program Development; *Bereavement; *Fathers/px [Psychology]; *Social Support; Evidence-Based Nursing
AIMS AND OBJECTIVES: The aim is to present the development and implementation of a bereavement follow-up intervention for grieving fathers. The development and implementation process and components of the intervention are presented., BACKGROUND: There is a lack of research into fathers' grief and bereavement support for them after the death of a child. Promotion of evidence-based nursing requires transparent development and implementation of nursing interventions and models to be able to repeat, evaluate their effectiveness and redevelop them., DESIGN: Phases of action research were used when developing and implementing the intervention., METHODS: Results of the baseline study of father's grief and social support, the study of current bereavement support and the literature review were combined using triangulation., RESULTS: In this action research, the development of a bereavement follow-up intervention for grieving fathers began with the planning phase that included a baseline study about fathers' grief and social support, a study of current bereavement support systems in Finnish university hospitals and a systematic review of literature on the topic area and collaboration with a panel of experts. The developed bereavement follow-up intervention included three complementary components: support package, peer supporters' contact and health care personnel's contact. Implementation of the intervention included the development of a programme to be used in nursing practice, intervention training for programme implementers and intervention implementation., CONCLUSIONS: Developing and implementing an intervention is a complex, demanding and long-term process. The planning required theoretical knowledge as well as understanding the experiences of fathers, nursing practice and collaboration with those who implemented the intervention., RELEVANCE TO CLINICAL PRACTICE: New information about the fathers' grief and bereavement follow-up support is described. The model developed is evidence-based and can be applied in nursing care where grieving fathers and families are met. Copyright © 2010 Blackwell Publishing Ltd.
Aho, Anna L; Astedt-Kurki, Paivi; Tarkka, Marja-Terttu; Kaunonen, Marja
Journal of Clinical Nursing
2011
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<a href="http://doi.org/10.1111/j.1365-2702.2010.03523.x" target="_blank" rel="noreferrer noopener">10.1111/j.1365-2702.2010.03523.x</a>
Deep brain stimulation in children: experience and technical pearls
Adolescent; Adult; Age; Factors Brain/pa [Pathology]; Brain/ra [Radiography]; Child; Preschool; Deep Brain Stimulation/ae [Adverse Effects]; Deep Brain Stimulation/mt [Methods]; Dystonia/pp [Physiopathology]; Dystonia/th [Therapy]; Female; Follow-Up Studies; Humans; Magnetic Resonance Imaging; Male; Movement; Retrospective Studies; Tomography; X-Ray Computed Treatment Outcome; tone and motor problems; Glutaric acidemia type I; Lesch-Nyhan syndrome; deep brain stimulation; secondary dystonia
OBJECT: Deep brain stimulation (DBS) is an established technique for the treatment of several movement disorders in adults. However, the technical approach, complications, and results of DBS in children have not been well documented. METHODS: A database of DBS implantations performed at a single institution, prospectively established in 1998, was reviewed for patients who received DBS prior to the age of 18. Diagnoses, surgical technique, and complications were noted. Outcomes were assessed using standard rating scales of neurological function. RESULTS: Of 815 patients undergoing DBS implantation over a 12-year period, 31 were children (mean age at surgery 13.2 years old, range 4-17 years old). Diagnoses included the following: DYT1 primary dystonia (autosomal dominant, Tor1ADELTAGAG mutation, 10 cases), non-DYT1 primary dystonia (3 cases), secondary dystonia (11 cases), neurodegeneration with brain iron accumulation (NBIA, 3 cases), levodopa-responsive parkinsonism (2 cases), Lesch-Nyhan disease (1 case), and glutaric aciduria Type 1 (1 case). Six children ages 15-17 years old underwent awake microelectrode-guided surgery. For 25 children operated under general anesthesia, the surgical technique evolved from microelectrode-guided surgery to image-guided surgeries using real-time intraoperative MR imaging or CT for lead location confirmation. Complications included 5 hardware infections, all in children younger than 10 years old. At 1 year after implantation, patients with DYT1 dystonia had a mean improvement in the Burke-Fahn-Marsden Dystonia Rating Scale movement subscore of 75%, while those with secondary dystonia had only small improvements. Outcomes in the 3 children with NBIA were disappointing. CONCLUSIONS: Results of DBS in children with primary and secondary dystonias were similar to those in adults, with excellent results for DYT1 dystonia in children without fixed orthopedic deformity and much more modest results in secondary dystonia. In contrast to reported experience in adults with NBIA, these results in children with NBIA were poor. Infection risk was highest in the youngest patients.
Air E L; Ostrem J L; Sanger T D; Starr P A
Journal of Neurosurgery - Pediatrics
2011
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<a href="http://doi.org/10.3171/2011.8.PEDS11153" target="_blank" rel="noreferrer noopener">10.3171/2011.8.PEDS11153</a>
Endoscopic balloon dilatation of esophageal strictures in children
Children; Pediatrics; experience; management; Dysphagia; Otorhinolaryngology; atresia; Balloon dilatation; conservative treatment; dilation; epidermolysis bullosa; Esophageal strictures; ingestion; repair; feeding difficulties; surgical intervention; endoscopic balloon dilatation
Aim of the study: To assess the efficacy and safety of endoscopic balloon dilatation of esophageal strictures in children. Material and methods: Design: retrospective case series; population: 49 patients under 18 years of age referred to our center for esophageal strictures; treatment: endoscopic balloon dilatation: outcome parameters: residual dysphagia, weight gain, iatrogenic esophageal perforation, assessment of the esophageal lumen by endoscopy or esophagogram. Results: The three main etiologies were esophageal atresia (49%; n = 24), corrosive injury (25%; n = 12), and epidermolysis bullosa (14%; n = 7), followed by a heterogeneous group of rarer causes (12%; n = 6). The number of dilatations ranged from 1 to 8 sessions per patient (median +/- SEM: 2 +/- 0.3). The length of the follow-up period ranged from 20 to 109 months (median +/- SEM: 40 +/- 4 months). Treatment was successful in 86% of cases (n = 42). Twelve percent of patients (n = 6) had a residual stenosis requiring surgery, and a further one still experienced swallowing difficulties requiring enteral nutrition via gastrostomy in spite of the absence of significant residual stricture. Results were less satisfactory in cases of corrosive injury than with other etiologies. Three esophageal perforations were observed (6% of patients; 2% of procedures). All were medically treated. Conclusions: Endoscopic balloon dilatation is a simple, safe and efficacious treatment of esophageal strictures in children. (C) 2011 Elsevier Ireland Ltd. All rights reserved.
Alshammari J; Quesnel S; Pierrot S; Couloigner V
International Journal of Pediatric Otorhinolaryngology
2011
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<a href="http://doi.org/10.1016/j.ijporl.2011.07.031" target="_blank" rel="noreferrer noopener">10.1016/j.ijporl.2011.07.031</a>
Palliative care services in families of males with Duchenne muscular dystrophy
INTRODUCTION: Palliative care services that address physical pain and emotional, psychosocial, and spiritual needs may benefit individuals with Duchenne muscular dystrophy (DMD). METHODS: The objective of this study was to describe the palliative care services that families of males with DMD report they receive. A questionnaire was administered to families of males with DMD born prior to January 1, 1982. Thirty-four families responded. RESULTS: Most families (85%) had never heard the term palliative care. Only attendant care and skilled nursing services showed much usage, with 44% and 50% indicating receipt of these services, respectively. Receipt of other services was reported less frequently: pastoral care (27%); respite care (18%); pain management (12%); and hospice care (6%). Only 8 respondents (25%) reported having any type of directive document in place. CONCLUSION: The data suggest a need for improved awareness of palliative care and related services among families of young men with DMD.
Arias R; Andrews J; Pandya S; Pettit K; Trout C; Apkon S; Karwoski J; Cunniff C; Matthews D; Miller T; Davis MF; Meaney FJ
Muscle & Nerve
2011
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Journal Article
<a href="http://doi.org/10.1002/mus.22005" target="_blank" rel="noreferrer">10.1002/mus.22005</a>
Swedish parents' experiences of parenthood and the need for support to siblings when a baby is stillborn
adolescent; Child; Female; Humans; infant; Male; Adult; Siblings; Sweden; Stillbirth; Preschool; sibling bereavement; parenting
BACKGROUND: It has been argued that having a stillborn baby in the family affects older siblings more than parents realize. The aim of this study was to describe parenthood and the needs of siblings after stillbirth from the parents' perspective. METHODS: Six focus groups were held with 27 parents who had experienced a stillbirth and who had had children before the loss. The discussion concerned parents' support to the siblings, and the sibling's meeting, farewell, and memories of their little sister or brother. Data were analyzed using qualitative content analysis. RESULTS: The overall theme of the findings was parenthood in a balance between grief and everyday life. In the analysis, three categories emerged that described the construction of the theme: support in an acute situation, sharing the experiences within the family, and adjusting to the situation. CONCLUSIONS: The siblings' situation is characterized by having a parent who tries to maintain a balance between grief and everyday life. Parents are present and engaged in joint activities around the stillbirth together with the siblings of the stillborn baby. Although parents are aware of the sibling's situation, they feel that they are left somewhat alone in their parenthood after stillbirth and therefore need support and guidance from others.
Avelin P; Erlandsson K; Hildingsson I; Rådestad I
Birth (berkeley, Calif.)
2011
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Journal Article
<a href="http://doi.org/10.1111/j.1523-536X.2010.00457.x" target="_blank" rel="noreferrer">10.1111/j.1523-536X.2010.00457.x</a>
Palliative Medicine and Decision Science: The Critical Need for a Shared Agenda To Foster Informed Patient Choice in Serious Illness
DNAR Outcomes
Bakitas M; Kryworuchko J; Matlock DD; Volandes AE
Journal Of Palliative Medicine
2011
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Journal Article
<a href="http://doi.org/10.1089/jpm.2011.0032" target="_blank" rel="noreferrer">10.1089/jpm.2011.0032</a>
A Strategy for Conversion From Subcutaneous to Oral Ketamine in Cancer Pain Patients: Effect of a 1:1 Ratio
cancer pain; dose ratio; oral ketamine; Subcutaneous ketamine
Context No consensus exists about the most appropriate dose ratio for conversion from parenteral to oral ketamine. Objectives To confirm that a 1:1 dose ratio is suitable for converting subcutaneous (s.c.) to oral ketamine in cancer patients. Methods Patients with opioid poorly responsive cancer pain, who responded to 0.4, 0.6, or 0.8 mg s.c. ketamine bolus, were treated with 0.1, 0.15, or 0.2 mg/kg/h ketamine infusion, respectively. Switching to the oral route, by applying a 1:1 dose ratio, was carried out in patients who experienced adequate pain relief and continued to need ketamine as a coanalgesic. Pain, somnolence, feelings of insobriety, confusion, and cardiovascular parameters were assessed throughout the process. Results Twenty-nine patients were enrolled in the study. Ketamine infusion decreased pain intensity from severe to no pain or slight pain in 23 of 29 and six of 29 patients, respectively. The median of s.c. ketamine doses was 0.2 mg/kg/h (range 0.1–0.5). After oral switching, 27 of 29 patients remained as successfully controlled as when receiving s.c. ketamine. The other two patients needed a slight dose ratio readjustment, to 1:1.3 and 1:1.5, to maintain pain control. The median of oral ketamine doses was 300 mg/day (interquartile range 240–382.5). Seven of 29 patients receiving s.c. ketamine developed moderate and transitory side effects, such as feelings of insobriety and somnolence. No side effects were present while receiving oral ketamine. No significant changes were observed in cardiovascular parameters. Conclusion A 1:1 dose ratio for conversion from s.c. to oral ketamine is safe and effective in cancer pain patients.
Benítez-Rosario MA; Salinas-Martín A; González-Guillermo Toribio; Feria M
Journal Of Pain And Symptom Management
2011
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Journal Article
<a href="http://doi.org/10.1016/j.jpainsymman.2010.08.017" target="_blank" rel="noreferrer">10.1016/j.jpainsymman.2010.08.017</a>
Frequently Asked Questions
Bereavement Leave Policy Paper
Bereaved Families of Toronto
2011
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Journal Article
Characteristics of hospitalizations for patients who use a structured clinical care program for children with medical complexity
Cohen 2006 BMC HSR Refs
Berry JG; Agrawal R; Kuo DZ; Cohen E; Risko W; Hall M; Casey P; Gordon J; Srivastava R
Journal Of Pediatrics
2011
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Journal Article
<a href="http://doi.org/10.1016/j.jpeds.2011.02.002" target="_blank" rel="noreferrer">10.1016/j.jpeds.2011.02.002</a>
Esophageal coin removal by emergency physicians: a continuous quality improvement project incorporating rapid sequence intubation
Child; Female; Humans; Male; Time Factors; Preschool; infant; retrospective studies; Emergency Medicine/methods; Quality improvement; Esophagus/injuries; Foreign Bodies/surgery; Intubation/methods; Numismatics
OBJECTIVE: The objective of this study was to describe our experience removing esophageal coins from children in a tertiary care pediatric emergency department over a 4-year period. METHODS: We retrospectively reviewed a continuous quality improvement data set spanning October 1, 2004, through September 30, 2008. RESULTS: In 96 of 101 cases (95%), emergency physicians successfully retrieved the coin. The median age of the children was 19 months (interquartile range [IQR] 13-43 months; range 4 months-12.8 years). The median time to removal of coin from initiation of intubation was 8 minutes (IQR 4-14 minutes; range 1-60 minutes). Coins were extracted using forceps only in 56 cases, whereas forceps and a Foley catheter were used in the remainder. Succinylcholine and etomidate were used in almost all cases for rapid sequence intubation prior to coin removal. Complications were identified in 46 cases: minor bleeding (13), lip laceration (7), multiple attempts (5), hypoxia (3), accidental extubation (3), dental injuries (3), bradycardia (2), coin advanced (1), right main-stem bronchus intubation (1), and other (8). CONCLUSIONS: Emergency physicians successfully removed esophageal coins following rapid sequence intubation in most cases. Our approach may be considered for the management of pediatric esophageal coins, particularly in an academic pediatric emergency department.
Bhargava R; Brown L
Canadian Journal Of Emergency Medical Care
2011
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Journal Article
<a href="http://doi.org/10.2310/8000.2011.100298" target="_blank" rel="noreferrer">10.2310/8000.2011.100298</a>
Trailblazers in Adolescent and Young Adult Oncology
Bleyer A; Albritton KH; Barr R; Lewis IJ; Sender LS
Journal Of Adolescent And Young Adult Oncology
2011
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Journal Article
<a href="http://doi.org/10.1089/jayao.2011.1500" target="_blank" rel="noreferrer">10.1089/jayao.2011.1500</a>
Levodopa is not a useful treatment for Lesch-Nyhan disease
behavior problems; tone and motor problems; Lesch-Nyhan syndrome; pharmacologic intervention; levodopa; levodopa-dopa; carbidopa
Lesch-Nyhan disease (LND) is characterized by dystonia, cognitive abnormalities, and self-injurious behavior. No effective therapies are available. LND is associated with a presynaptic dopaminergic deficit, but the reported effects of dopamine replacement therapy are conflicting. The current prospective open-label study assesses the effects of levodopa on both neurological and behavioral features of LND. All 6 study participants discontinued levodopa early, due to lack of effect and sometimes worsening of motor function. The results provide important clues for pathophysiological mechanisms and suggestions for future treatment options.Copyright © 2011 Movement Disorder Society.
Bloem B R; Schretlen D J; Jinnah H A; Visser J E
Movement Disorders
2011
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<a href="http://doi.org/10.1002/mds.23478" target="_blank" rel="noreferrer noopener">10.1002/mds.23478</a>
Association between opioid prescribing patterns and opioid overdose-related deaths
Female; Humans; Male; Young Adult; Cohort Studies; Adult; Analgesics; Aged; Middle Aged; Risk; Acute Disease; Case-Control Studies; adolescent; Physician's Practice Patterns/statistics & numerical data; Chronic disease; United States/epidemiology; Drug Prescriptions/statistics & numerical data; Opioid/administration & dosage/poisoning; Overdose/epidemiology; Pain/drug therapy; Veterans/statistics & numerical data
CONTEXT: The rate of prescription opioid-related overdose death increased substantially in the United States over the past decade. Patterns of opioid prescribing may be related to risk of overdose mortality. OBJECTIVE: To examine the association of maximum prescribed daily opioid dose and dosing schedule ("as needed," regularly scheduled, or both) with risk of opioid overdose death among patients with cancer, chronic pain, acute pain, and substance use disorders. DESIGN: Case-cohort study. SETTING: Veterans Health Administration (VHA), 2004 through 2008. PARTICIPANTS: All unintentional prescription opioid overdose decedents (n = 750) and a random sample of patients (n = 154,684) among those individuals who used medical services in 2004 or 2005 and received opioid therapy for pain. Main Outcome Measure Associations of opioid regimens (dose and schedule) with death by unintentional prescription opioid overdose in subgroups defined by clinical diagnoses, adjusting for age group, sex, race, ethnicity, and comorbid conditions. RESULTS: The frequency of fatal overdose over the study period among individuals treated with opioids was estimated to be 0.04%.The risk of overdose death was directly related to the maximum prescribed daily dose of opioid medication. The adjusted hazard ratios (HRs) associated with a maximum prescribed dose of 100 mg/d or more, compared with the dose category 1 mg/d to less than 20 mg/d, were as follows: among those with substance use disorders, adjusted HR = 4.54 (95% confidence interval [CI], 2.46-8.37; absolute risk difference approximation [ARDA] = 0.14%); among those with chronic pain, adjusted HR = 7.18 (95% CI, 4.85-10.65; ARDA = 0.25%); among those with acute pain, adjusted HR = 6.64 (95% CI, 3.31-13.31; ARDA = 0.23%); and among those with cancer, adjusted HR = 11.99 (95% CI, 4.42-32.56; ARDA = 0.45%). Receiving both as-needed and regularly scheduled doses was not associated with overdose risk after adjustment. CONCLUSION: Among patients receiving opioid prescriptions for pain, higher opioid doses were associated with increased risk of opioid overdose death.
Bohnert AS; Valenstein M; Bair MJ; Ganoczy D; McCarthy JF; Ilgen MA; Blow FC
Jama
2011
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Journal Article
<a href="http://doi.org/10.1001/jama.2011.370" target="_blank" rel="noreferrer">10.1001/jama.2011.370</a>
Alternatives to randomisation in the evaluation of public health interventions: design challenges and solutions
Bonell CP; Hargreaves J; Cousens S; Ross D; Hayes R; Petticrew M; Kirkwood BR
Journal Of Epidemiology And Community Health
2011
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Journal Article
<a href="http://doi.org/10.1136/jech.2008.082602" target="_blank" rel="noreferrer">10.1136/jech.2008.082602</a>
Approaches to the difficult patient/parent encounter
Child; Female; Humans; Male; Physician-Patient Relations; Pediatrics; Parents; Conflict (Psychology); adolescent; Patients/classification
Most pediatricians have experienced uneasy interactions involving patients and/or their parents. The majority of literature on this topic reflects encounters in adult medicine, without providing much information for pediatricians who also face this challenge. Unique to the pediatric approach is the added quotient of the parent/family dynamic. Patients or their parents may have personality disorders or subclinical mental health issues, physicians may be overworked or have a lack of experience, and the health care system may be overburdened, fragmented, and inundated with poor communication. Recognizing the physical or emotional responses triggered by challenging patients/families may allow the provider to effectively partner with, instead of confront, the patient or the family. In this article we review existing literature on this subject and describe possible strategies for the pediatrician to use during a difficult encounter.
Breuner CC; Moreno MA
Pediatrics
2011
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Journal Article
<a href="http://doi.org/10.1542/peds.2010-0072" target="_blank" rel="noreferrer">10.1542/peds.2010-0072</a>
Parental assessment of pain coping in individuals with intellectual and developmental disabilities
Child; Female; Humans; Male; Young Adult; Pain Measurement; Adult; Questionnaires; adolescent; Preschool; Adaptation; Psychological; Parents/psychology; social support; Developmental Disabilities/complications/psychology; Intellectual Disability/complications/psychology; Pain/complications/psychology
Pain coping is thought to be the most significant behavioural contribution to the adjustment to pain. Little is known about how those with intellectual and developmental disabilities (IDD) cope with pain. We describe parental reported coping styles and how coping relates to individual factors. Seventy-seven caregivers of children and adults with IDD reported on coping styles using the Pediatric Pain Coping Inventory (PPCI), pain behaviour using the Non-Communicating Children's Pain Checklist-Revised (NCCPC-R), illness-related interactions using the Illness Behaviour Encouragement Scale (IBES) and past pain experience using the Structured Pain Questionnaire. Scores were compared across mental ages and interactions between pain coping and the other factors were explored. A Multivariate Analysis of Variance (MANOVA) by mental age group ('/=12 years') revealed that those in the '5-11 years' mental age group used more coping styles than those in the '/=12 years' group used more cognitively demanding coping styles than the other two groups (F(10,130)=2.68, p=.005). Seeking Social Support (r=.39, p=.001) and Catastrophizing/Helplessness (r=.33, p<.01) coping styles were significantly related to a greater display of pain behaviour. Those with younger mental ages, who Seek Social Support or Catastrophize, also displayed more pain behaviour, which may be an attempt to seek external resources when pain is beyond their ability to deal with independently.
Burkitt CC; Breau LM; Zabalia M
Research In Developmental Disabilities
2011
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Journal Article
<a href="http://doi.org/10.1016/j.ridd.2011.01.050" target="_blank" rel="noreferrer">10.1016/j.ridd.2011.01.050</a>
Palliative Social Work in Canada
PPC Book Chapter 2011 (Kim Widger)
Cadell S; Bosma H
Oxford Textbook Of Palliative Social Work
2011
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Book/Book Section
Discovering strengths and growth in palliative care
Cadell S; Shermak S; Johnston M
Oxford Textbook Of Palliative Social Work
2011
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Book/Book Section
Children's palliative care in South Africa: An urgent need for an evidence base
Child Health Care; palliative Therapy; Child; Childhood Mortality; Evidence Based Practice; Human; Human Immunodeficiency Virus Infection/ep [epidemiology]; Quality Of Life; Short Survey; South Africa
Campbell LM
Southern African Journal Of HIV Medicine
2011
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<a href="http://doi.org/10.4102/sajhivmed.v12i1.204" target="_blank" rel="noreferrer">10.4102/sajhivmed.v12i1.204</a>
Effect of Hospital-Based Comprehensive Care Clinic on Health Costs for Medicaid-Insured Medically Complex Children
Cohen 2006 BMC HSR Refs
Casey PH; Lyle RE; Bird TM; Robbins JM; Kuo DZ; Brown C; Lal A; Tanios A; Burns K
Archives Of Pediatrics & Adolescent Medicine
2011
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/archpediatrics.2011.5" target="_blank" rel="noreferrer">10.1001/archpediatrics.2011.5</a>
A Good Death: Palliative Care in Canada
CBC
2011
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
Reflection of a career in paediatrics: A calling becomes a fulfililng profession
Chan-Yip AM
Paediatrics & Child Health
2011
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/10.1093/pch/16.3.139" target="_blank" rel="noreferrer">10.1093/pch/16.3.139</a>
Association Between Fatigue and Autistic Symptoms in Children With Cri du Chat Syndrome
alertness; sleep disturbance; cri-du-chat; trajectory; characteristics; fatigue; autistic symptoms
In the current study, the authors examined whether the fatigue level of children diagnosed with cri du chat syndrome was associated with the expression of autistic symptoms. Sixty-nine children with cri du chat syndrome were compared with 47 children with moderate to severe intellectual disabilities who did not differ on intellectual severity. Participants were assessed using the Infant Sleep Questionnaire (J. M. B. Morrell, 1999) for fatigue-level rating and the Childhood Autism Rating Scale (E. Schopler, R. J. Reichler, & B. R. Renner, 1988) for autism-level rating. In support of the authors' hypothesis, results indicated that children who exhibited high levels of fatigue were more likely to express high levels of autistic symptoms. Contrary to the authors' hypothesis, children in the comparison group who exhibited high levels of fatigue conferred the greatest vulnerability to the expression of autistic symptoms.
Claro A; Cornish K; Gruber R
American Journal on Intellectual and Developmental Disabilities
2011
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<a href="http://doi.org/10.1352/1944-7558-116.4.278" target="_blank" rel="noreferrer noopener">10.1352/1944-7558-116.4.278</a>
Integrated complex care model: lessons learned from inter-organizational partnership
Cohen 2006 BMC HSR Refs
Cohen E; Bruce-Barrett C; Kingsnorth S; Keilty K; Cooper A; Daub S
Healthcare Quarterly
2011
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Journal Article
<a href="http://doi.org/10.12927/hcq.0000.22580" target="_blank" rel="noreferrer">10.12927/hcq.0000.22580</a>
Hospital-based comprehensive care programs for children with special health care needs: a systematic review
Cohen 2006 BMC HSR Refs
Cohen E; Jovcevska V; Kuo DZ; Mahant S
Archives Of Pediatrics & Adolescent Medicine
2011
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Journal Article
<a href="http://doi.org/10.1001/archpediatrics.2011.74" target="_blank" rel="noreferrer">10.1001/archpediatrics.2011.74</a>
Children with medical complexity: An emerging population for clinical and research initiatives
Cohen 2006 BMC HSR Refs
Cohen E; Kuo D; Agrawal R; Berry J; Simon T; Bhagat S; Srivastava R
Pediatrics
2011
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Journal Article
<a href="http://doi.org/10.1542/peds.2010-0910" target="_blank" rel="noreferrer">10.1542/peds.2010-0910</a>
Adverse events in hospice and palliative care: a pilot study to determine feasibility of collection and baseline rates
Female; Humans; Male; Young Adult; Adult; Aged; Middle Aged; Pilot Projects; Feasibility Studies; Australia; Urinary Retention; 80 and over; retrospective studies; Palliative Care/standards; Hospice Care/standards; Accidental Falls; Confusion; Hyperglycemia; Hypoglycemia; Hypotension
BACKGROUND: Continuous quality improvement is fundamental in all health care, including hospice and palliative care. Identifying and systematically reducing symptomatic adverse events is limited in hospice and palliative care because these events are mostly attributed to disease progression. OBJECTIVES: The aim of this study was to assess the feasibility of symptomatic adverse events in hospice and palliative care and assessing their incidence. METHODS: A retrospective, consecutive cohort of notes from a specialist palliative care inpatient service was surveyed by a clinical nurse consultant for symptomatic adverse events: falls, confusion, decreased consciousness, hypo- and hyperglycaemia, urinary retention, and hypotension. Demographic and clinical factors were explored for people at higher risk. RESULTS: Data were available on the most recent admissions of 65 people, generating >900 inpatient days. Fifty people (78%) had events precipitating admission, of whom 31 (62%) had at least one further event during admission. Eleven of 15 people who were admitted without an event experienced at least one during their admissions. Only 4 did not have an adverse event. During their stay, there were 0.13 (standard deviation [SD] = 0.19) events per patient per day. No drug-drug or drug-host events were noted. No clinical or demographic factors predicted groups at higher risk. CONCLUSIONS: This pilot highlights the feasibility of collecting, and ubiquity of, symptomatic adverse events, and forms a baseline against which future interventions to decrease the frequency or intensity can be measured. Given the frailty of hospice and palliative patients, any adverse event is likely to accelerate irreversibly their systemic decline.
Currow DC; Agar MR; To TH; Rowett D; Greene A; Abernethy AP
Journal Of Palliative Medicine
2011
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Journal Article
<a href="http://doi.org/10.1089/jpm.2010.0392" target="_blank" rel="noreferrer">10.1089/jpm.2010.0392</a>
Perceptions of discrimination among Mexican American families of seriously ill children
Middle Aged; Male; Young Adult; Child; Humans; Adult; Adolescent; Female; Child Preschool; Infant; Retrospective Studies; Critical Illness; California; Mexican Americans/px [Psychology]; Prejudice
This paper describes Mexican American family members' descriptions of perceived discrimination by pediatric health care providers (HCPs) and the families' reactions to the HCPs' discriminatory conduct. A retrospective, grounded theory design guided the overall study. Content analysis of interviews with 13 participants from 11 families who were recruited from two children's hospitals in Northern California resulted in numerous codes and revealed that participants perceived discrimination when they were treated differently from other, usually white, families. They believed they were treated differently because they were Mexican, because they were poor, because of language barriers, or because of their physical appearance.
Davies B; Larson J; Contro N; Cabrera AP
Journal of Palliative Medicine
2011
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<a href="http://doi.org/10.1089/jpm.2010.0315" target="_blank" rel="noreferrer noopener">10.1089/jpm.2010.0315</a>
Research considerations in pediatric palliative care
Davies B; Widger K; Steele R; Cadell S; Siden H; Straatman L
In Wolff J, Sourkes B, & Hinds P (Eds.) Textbook of Interdisciplinary Pediatric Palliative Care, First Edition. New York: Elsevier, 96-103.
New York: Elsevier
2011
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Palliative care: what approaches are suitable in developing countries?
The enormous burden of life-threatening illnesses, including cancer, human immunodeficiency virus infection, and others, such as sickle cell disease, associated with physical and psychosocial suffering explains the illustrious need for palliative care in developing countries. Despite the demonstrated need, current provision of palliative care in Africa is at best limited, and at worst non-existent. Access to essential pain medicines, particularly oral morphine, for control of pain is extremely limited and far below the global mean. There is a general lack of government policies that recognize palliative care as an essential component of health care and there is inadequate training for both health care professionals and the general public about palliative care. A public health strategy, as recommended by the World Health Organization (WHO), offers the best approach for translating knowledge and skills into evidence-based, cost-effective interventions that can reach everyone in need of palliative care in developing countries.
Ddungu H
British Journal Of Haematology
2011
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Journal Article
<a href="http://doi.org/10.1111/j.1365-2141.2011.08764.x" target="_blank" rel="noreferrer">10.1111/j.1365-2141.2011.08764.x</a>
Prolonged-release melatonin for children with neurodevelopmental disorders
sleep disturbance/disorders; Rett syndrome; pharmacologic intervention; prolonged-release melatonin; PR melatonin; melatonin
Previous studies demonstrated the efficacy and safety of prolonged-release melatonin in children and adolescents with neurodevelopmental and behavioral disorders. The long-term effectiveness and safety of prolonged-release melatonin treatment were assessed in 88 children (42 girls and 46 boys) with neurodevelopmental disorders. These patients participated in a compassionate-use program with the drug Circadin (2 mg; Neurim Pharmaceuticals, Tel Aviv, Israel) in France, and received treatment in the context of regular care by a specialized physician. The study involved a structured questionnaire for the parents, comprising a combination of multiple-choice and numeric questions addressing sleep onset/offset, sleep quality problems, and mood. The dose of melatonin ranged from 4-6 mg, and treatment duration ranged from 6-72 months. Within 3 months, sleep latency with prolonged-release melatonin decreased by 44.0% (P < 0.001), sleep duration increased by 10.1% (P < 0.001), the number of awakenings decreased by 75% (P < 0.001), and sleep quality improved by 75%, compared with baseline (P < 0.001). No serious adverse events or treatment-related comorbidities were reported. Prolonged-release melatonin remains a safe, effective therapy for the long-term treatment of sleep disorders in children with neurodevelopmental disorders. © 2011 Elsevier Inc. All rights reserved.
De Leersnyder H; Zisapel N; Laudon M
Pediatric Neurology
2011
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<a href="http://doi.org/10.1016/j.pediatrneurol.2011.02.001" target="_blank" rel="noreferrer noopener">10.1016/j.pediatrneurol.2011.02.001</a>
Basic Guide to Family Responsibility Leave
Bereavement Leave Policy Paper
Department of Labour Government of South Africa
2011
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Journal Article
Forgoing life support: how the decision is made in European pediatric intensive care units
Decision Making; Intensive Care Units Pediatric; Life Support Care; Withholding Treatment/sn [statistics & Numerical Data]; Child Preschool; Europe; Female; France; Health Care Surveys; Humans; Male; Prospective Studies; Surveys And Questionnaires; Terminal Care/td [trends]
PURPOSE: To determine how decisions to forgo life support are made in European pediatric intensive care units (PICUs). METHODS: A multicenter, prospective study, the Eurydice II study, among 45 PICUs: 20 in France, 21 in Northern/Western (N/W) European countries, and 4 in Eastern/Central (E/C) Europe. Data were collected between November 2009 and April 2010 through a questionnaire. RESULTS: The decision to forgo life-sustaining treatment was made in 166 (40.6%) out of 409 deceased children (median 42.9%, France 38.2%, N/W European countries 60.0%, E/C European countries 0%; P < 0.001). In the E/C group, more patients died after cardiopulmonary resuscitation (CPR) failure than after forgoing life support (P < 0.001). In all PICUs, caregivers discussed the decision during a formal meeting, after which the medical staff made the final decision. The decision was often documented in the medical record (median 100%). The majority of the parents were informed of the final decision and were at the bedside during their child's death (median 100%). Decision to forgo life-sustaining treatment occurred in 40.6% of children, compared with 33% in Eurydice I. A high percentage of parents from France were now informed about the meeting and its conclusion as compared with Eurydice I (median 100%). CONCLUSIONS: The results of this study and comparison with the Eurydice I study (2002) show a trend towards standardization of end-of-life practices across N/W European countries and France in the past decade.
Devictor DJ; Latour JM
Intensive Care Medicine
2011
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<a href="http://doi.org/10.1007/s00134-011-2357-3" target="_blank" rel="noreferrer">10.1007/s00134-011-2357-3</a>
Transition to adult services for children and young people with palliative care needs: a systematic review
Humans; Young Adult; adolescent; Palliative Care/organization & administration; Models; Continuity of Patient Care/organization & administration; Adolescent Health Services/organization & administration; Delivery of Health Care/organization & administration; Organizational; Evidence-Based Medicine/methods; Health Services Research/methods/standards
OBJECTIVE: To evaluate the evidence on the transition process from child to adult services for young people with palliative care needs. DESIGN: Systematic review. SETTING: Child and adult services and interface between healthcare providers. PATIENTS: Young people aged 13-24 years with palliative care conditions in the process of transition. MAIN OUTCOME MEASURES: Young people and their families' experiences of transition, the process of transition between services and its impact on continuity of care and models of good practice. RESULTS: 92 studies included. Papers on transition services were of variable quality when applied to palliative care contexts. Most focussed on common life-threatening and life-limiting conditions. No standardised transition program identified and most guidelines used to develop transition services were not evidence-based. Most studies on transition programs were predominantly condition-specific (eg, cystic fibrosis (CF), cancer) services. CF services offered high-quality transition with the most robust empirical evaluation. There were differing condition-dependent viewpoints on when transition should occur but agreement on major principles guiding transition planning and probable barriers. There was evidence of poor continuity between child and adult providers with most originating from within child settings. CONCLUSIONS: Palliative care was not, in itself, a useful concept for locating transition-related evidence. It is not possible to evaluate the merits of the various transition models for palliative care contexts, or their effects on continuity of care, as there are no long-term outcome data to measure their effectiveness. Use of validated outcome measures would facilitate research and service development.
Doug M; Adi Y; Williams J; Paul M; Kelly D; Petchey R; Carter YH
Archives Of Disease In Childhood
2011
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Journal Article
<a href="http://doi.org/10.1136/adc.2009.163931" target="_blank" rel="noreferrer">10.1136/adc.2009.163931</a>
Intensive care unit palliative medicine: some issues-part II.
Humans; Resuscitation Orders; Medical; DNAR; Sociology; Palliative Care/og [Organization & Administration]; Palliative Care/ec [Economics]; Health Care Rationing/og [Organization & Administration]; Intensive Care Units/og [Organization & Administration]; Health Care Rationing/ec [Economics]; Intensive Care Units/ec [Economics]
Enck RE
The American Journal Of Hospice & Palliative Care
2011
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Journal Article
<a href="http://doi.org/10.1177/1049909111399408" target="_blank" rel="noreferrer">10.1177/1049909111399408</a>
Employment and Social Affairs
Bereavement Leave Policy Paper
Europa
2011
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Journal Article