Fathers' experiences in the neonatal intensive care unit: a search for control.
Humans; Infant, Newborn; Critical Illness; Male; Professional-Family Relations; Communication; Qualitative Research; Interviews as Topic; Religion; Stress, Psychological; Infant, Premature; Father-Child Relations; *Intensive Care Units, Neonatal; *Fathers/px [Psychology]; Intensive Care Units, Neonatal/og [Organization & Administration]; *Internal-External Control
OBJECTIVE: This qualitative study aimed at understanding the experiences of fathers of very ill neonates in the NICU., METHODS: Sixteen fathers of very ill and/or very preterm infants who had been in the NICU for >30 days were interviewed by a male physician. Fathers were asked about their level of comfort with or concerns about staff communication regarding their infant, about accessing information, and about more general perceptions of their experience in the neonatal intensive care unit. Interviews were audiotaped and transcribed for analysis. Coding used content analysis with construction of themes by 3 researchers., RESULTS: The overarching theme for fathers was a sense of lack of control. Their world view, as a "backdrop" theme, provided context for all of the themes. Four other interrelated subthemes were identified, including information; communication, particularly with the health care team; fathers' various roles; and external activities. Fathers reported that relationships with friends/family/health care team, receiving information consistently, and receiving short written materials on common conditions were ways of giving them support. The fathers said that speaking to a male physician was a positive and useful experience., CONCLUSIONS: Fathers experience a sense of lack of control when they have an extremely ill infant in the NICU. Specific activities help fathers regain a sense of control and help them fulfill their various roles of protectors, fathers, partners, and breadwinners. Understanding these experiences helps the health care team offer targeted supports for fathers in the NICU.
Arockiasamy, Vincent; Holsti, Liisa; Albersheim, Susan
Pediatrics
2008
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/10.1542/peds.2007-1005" target="_blank" rel="noreferrer noopener">10.1542/peds.2007-1005</a>
Autonomic Dysregulation in Young Girls With Rett Syndrome During Nighttime in-Home Recordings
Pediatrics; patterns; autonomic dysregulation; cardiorespiratory dysregulation; disorder; disturbances; dysfunction; hyperventilation; mecp2 mutations; mice; nervous-system; Respiratory System; breathing difficulties; Rett syndrome; trajectory; characteristics; irregular breathing; increased breathing frequency
This study was designed to specifically characterize the autonomic phenotype of cardiorespiratory dysregulation during the nighttime in young girls with MECP2 mutation-confirmed Rett Syndrome (RS), studied in their home environment. Computerized breath-to-breath and beat-to-beat characterization of at-home continuously recorded respiratory inductance plethysmography of chest/abdomen and ECG (VivoMetrics, Inc.) was obtained during overnight recordings in 47 girls with MECP2 mutation-confirmed RS and 47 age-, gender-, and ethnicity-matched screened controls (ages 2-7 years). We determined that although the breathing and heart rate appear more regular during the night compared to the day, young girls with RS demonstrate apparent nocturnal irregularities. Comparing daytime versus nighttime, breathing was more irregular, with an increased breathing frequency (and irregularity), mean amplitude of respiratory inductance plethysmography sum (AMP)/T-I, and heart rate and decreased AMP in girls with RS. Comparing girls with RS versus controls during nighttime recording, breathing was more irregular, with an increased breathing frequency (and irregularity), mean AMP/T-I, and heart rate. An increased uncoupling between measures of breathing and heart rate control indicates malregulation in the autonomic nervous system, and is apparent during the day as well as the night. This uncoupling may represent a mechanism that renders the girls with RS more vulnerable to sudden death. Pediatr Pulmonol. 2008;43:1045-1060. (C) 2008 Wiley-Liss, Inc.
Weese-Mayer D E; Lieske S P; Boothby C M; Kenny A S; Bennett H L; Ramirez J M
Pediatric Pulmonology
2008
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/10.1002/ppul.20866" target="_blank" rel="noreferrer noopener">10.1002/ppul.20866</a>
Movement disorders in Rett syndrome: An analysis of 60 patients with detected MECP2 mutation and correlation with mutation type
Neurosciences & Neurology; dystonia; Rett syndrome; females; rigidity; stereotypies; chorea; Parkinson; tremor; tone and motor problems; trajectory; characteristics; movement disorders
Rett syndrome (RS) is one of the best human models to study movement disorders. Patients evolve from a hyperkinetic to a hypokinetic state, and a large series of abnormal movements may be observed along their lives Such as stereotypies, tremor, chorea, myoclonus. ataxia, dystonia, and rigidity. The aim of this work was to analyze movement disorders in RS patients with a detected MECP2 mutation, as well as their correlation with genotype, in a clinically and genetically well-characterized sample of patients, and thus Contribute to redefine the clinical profile of this disease. In this study, we included 60 patients with detected MECP2 mutations. These were categorized and grouped for analysis, according to (1) type of change (missense or truncating. including nonsense and frameshift but also large deletions) and (2) location of the mutation. Differences were found concerning the frequency of independent gait. dystonia, type of tremor. and global score severity when comparing the group of patient S with missense and truncating, Mutations. We also found differences in the presence. distribution, severity, or type of movement disorders in the two groups of patients according to the median duration of the disease (less than 60 months: 60 months or more). We conclude that movement disorders seem to reflect the severity and rate of progression of Rett disorder, patients with truncating mutations presenting a higher rate and more severe dystonia and rigid-akinetic syndrome. when comparing groups with similar time of disease evolution. (C) 2008 Movement Disorder Society.
Temudo T; Ramos E; Dias K; Barbot C; Vieira J P; Moreira A; Calado E; Carrilho I; Oliveira G; Levy A; Fonseca M; Cabral A; Cabral P; Monteiro J P; Borges L; Gomes R; Santos M; Sequeiros J; Maciel P
Movement Disorders
2008
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<a href="http://doi.org/10.1002/mds.22115" target="_blank" rel="noreferrer noopener">10.1002/mds.22115</a>
Non-endoscopic percutaneous gastrostomy placement in children with recessive dystrophic epidermolysis bullosa
Pediatrics; Surgery; anesthesia; epidermolysis bullosa; management; dilation; esophageal strictures; "Push"; chile; gastrostomy; iind international-symposium; non-endoscopic percutaneous gastrostomy; nutrition; santiago; feeding difficulties; surgical intervention; non-endoscopic percutaneous gastrostomy; gastrostomy tube placement
Recessive dystrophic epidermolysis bullosa (RDEB) is associated with high nutritional demands, esophageal strictures and dysphagia. About one quarter of the patients require gastrostomy tube placement to maintain adequate nutritional status. To minimize trauma to the skin and pharyngoesophageal mucosa caused by commonly used gastrostomy tube insertion techniques, we used a non-endoscopic, percutaneous, image-guided approach. This approach combines the use of ultrasound for mapping of the liver and spleen, water-soluble contrast enema to visualize the colon, and gastric insufflation to define the stomach. The gastrostomy tube is replaced by a low-profile button gastrostomy tube 10-12 weeks postoperatively. The five female patients reported in this series ranged in age from 6 to 9 years. They all tolerated the procedure well and no perioperative complications were encountered. All were able to tolerate feedings on postoperative day 1 and all underwent successful replacement of gastrostomy tubes by low-profile button tubes. Our experience suggests that a non-endoscopic, image-guided approach to gastrostomy tube placement offers a safe, effective, and minimally traumatic alternative to more commonly used approaches. It minimizes the risk of procedure-related morbidity and leads to overall improvement in the quality of life. As such, we strongly recommend that it be included in the treatment armamentarium for patients with epidermolysis bullosa and nutritional failure.
Stehr W; Farrell M K; Lucky A W; Johnson N D; Racadio J M; Azizkhan R G
Pediatric Surgery International
2008
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<a href="http://doi.org/10.1007/s00383-007-2100-x" target="_blank" rel="noreferrer noopener">10.1007/s00383-007-2100-x</a>
Self-injurious behavior and tuberous sclerosis complex: Frequency and possible associations in a population of 257 patients
Epilepsy; Self-injurious behavior; Neurosciences & Neurology; epilepsy; Tuberous sclerosis complex; autism; Psychiatry; gene; aggression; identification; adults; Behavioral problems; Behavioral Sciences; disabilities; Frontal; frontal-lobe seizures; lobe; Tuber location; behavioral problems; tuberous sclerosis; trajectory; characteristics
Self-injurious behavior (SIB) has been observed in people with tuberous sclerosis complex (TSC), although the frequency of SIB in TSC is largely unknown. SIB is associated with intellectual and developmental disabilities, but there is no single cause of SIB. We retrospectively examined the frequency of SIB in a population of 257 patients with TSC and determined possible associations with SIB. We found a 10% frequency of SIB in our TSC population. When compared with patients without psychiatric symptoms, we identified a significantly higher rate of electroencephalographic interictal spikes in the left frontal lobe and a significantly lower number of tubers in the left occipital, parietal, and posterior temporal lobes. We also found that frequency of TSC2 Mutation, history of infantile spasms, history of seizures, mental retardation, and autism are significantly associated with SIB. (C) 2008 Elsevier Inc. All rights reserved.
Staley B A; Montenegro M A; Major P; Muzykewicz D A; Halpern E F; Kopp C M C; Newberry P; Thiele E A
Epilepsy and Behavior
2008
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<a href="http://doi.org/10.1016/j.yebeh.2008.07.010" target="_blank" rel="noreferrer noopener">10.1016/j.yebeh.2008.07.010</a>
Behavioural phenotype of Cornelia de Lange syndrome: case-control study
Male; Case-Control Studies; Young Adult; Surveys and Questionnaires; Child; Humans; Adult; Adolescent; Female; Child Preschool; Phenotype; Autistic Disorder/psychology; Communication Disorders/psychology; Compulsive Behavior/psychology; De Lange Syndrome/psychology; behavioral problems; De Lange syndrome; trajectory; characteristics; global behavior disorder
BACKGROUND: Cornelia de Lange syndrome is associated with abnormalities on chromosomes 5, 10 and X. AIMS: To delineate the behavioural phenotype of Cornelia de Lange syndrome with specific reference to autistic-spectrum disorder. METHOD: A total of 54 individuals with Cornelia de Lange syndrome (mean age 13.88 years; s.d.=8.58) and 46 comparable individuals with intellectual disability (mean age 13.74 years; s.d.=7.99) were assessed on measures of autistic-spectrum disorder, and adaptive, compulsive and disordered behaviour. RESULTS: There was no difference between the groups in global behaviour disorder. Severe autism was significantly more prevalent in the syndrome group (32.1%) than the comparison group (7.1%). In addition, the syndrome group also evidenced significantly higher levels of compulsive behaviour. CONCLUSIONS: These data suggest that autistic-spectrum disorder is part of the behavioural phenotype of Cornelia de Lange syndrome and that compulsive behaviours are evident. Future research should investigate this behavioural phenotype using contemporary diagnostic algorithms for autism with detailed examination of the phenomenology of compulsive behaviours.
Oliver C; Arron K; Sloneem J; Hall S
The British Journal of Psychiatry
2008
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<a href="http://doi.org/10.1192/bjp.bp.107.044370" target="_blank" rel="noreferrer noopener">10.1192/bjp.bp.107.044370</a>
Olfactory stimulation using black pepper oil facilitates oral feeding in pediatric patients receiving long-term enteral nutrition
feeding difficulties; Costello syndrome; lissencephaly type I; physical intervention; black pepper oil; increased oral intake; enteral nutrition
Patients with severe neurological disorders often require enteral nutrition (EN). Since long-term EN can cause multiple complications, reinstating the oral intake of food is beneficial. Olfactory stimulation using black pepper oil (BPO), a strong appetite stimulant, was reported to facilitate swallowing in order people. Therefore, the effects of olfactory stimulation with BPO were investigated in pediatric patients receiving long-term EN due to neurological disorders. The effects of scenting with BPO for 1 min immediately before every meal were evaluated in ten patients: 4 boys and 6 girls, aged 19-97 months (51 +/- 26 months). The neurological disorders included periventricular leukomalacia (3 patients), hypoxic ischemic encephalopathy (3). Costello syndrome (1), Russell-Silver syndrome (1), Miller-Dieker syndrome (1), and cerebral palsy of unknown etiology (1). In eight of these patients, BPO intervention was continued for 3 months. Five of these eight patients showed increases in the amount of oral intake with desirable effects including facilitated swallowing movement, although complete elimination of the need for EN was not achieved. In the other three patients: BPO intervention was not effective; severe cerebral tissue loss, profound malformation or intractable seizures seemed to reduce the efficacy of BPO. In two cases, BPO intervention was discontinued due to cough or because the odor of BPO was unbearable to the family. In conclusion, olfactory stimulation with BPO facilitated oral intake in a subset of patients on long-term EN. BPO stimulation may be useful or facilitating oral intake when used in combination with conventional methods. © 2008 Tohoku University Medical Press.
Munakata M; Kobayashi K; Niisato-Nezu J; Tanaka S; Kakisaka Y; Ebihara T; Ebihara S; Haginoya K; Tsuchiya S T; Onuma A
Tohoku Journal of Experimental Medicine
2008
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<a href="http://doi.org/10.1620/tjem.214.327" target="_blank" rel="noreferrer noopener">10.1620/tjem.214.327</a>
The use of pulse oximetry as a screening assessment for paediatric neurogenic dysphagia
children; Pediatrics; Rehabilitation; dysphagia; Neurosciences & Neurology; child; rett syndrome; intellectual disability; Neurological impairment; acute stroke; deglutition disorder; desaturation; feeding problems; oxygen-saturation; patients; pulse oximetry; reliably detect aspiration; respiratory patterns; severe cerebral-palsy; swallow; feeding difficulties; trajectory; characteristics
Purpose: Early screening and intervention for dysphagia is crucial to offset potential outcomes such as compromised nutrition or reduced respiratory function. Current paediatric dysphagia screening tests are subjective with poor sensitivity and specificity. The present study examined whether an objective method, pulse oximetry (measuring oxygen saturation (SpO(2)) levels), could differentiate between children with and without dysphagia, in relation to (1) Average pre-feeding baseline SpO(2) levels; (2) Average feeding SpO(2) levels; (3) Average post-feeding SpO(2) levels; and (4) The number of events of oxygen desaturation pre-, during and after feeding. Methods: Nine participants with chronic neurological disability (CND) (7 F, 2M) (9; 7-15; 11 years) and nine control participants matched for age (9; 5-16; 0 years) and sex were assessed using a clinical bedside evaluation (CBE) and pulse oximetry. Results: A statistically significant difference was found in SpO(2) levels between the two groups (p<0.001) during oral feeding only (sensitivity, 88.9%; specificity, 88.9%). Only three children with dysphagia experienced 'events' of SpO(2) desaturation during feeding. Conclusion: Pulse oximetry may provide a useful adjunct to the CBE for dysphagia screening, with average SpO(2) levels during feeding predicting those with and without dysphagia with moderate levels of sensitivity and specificity. The finding of individual variation in desaturation ` events', however, warrants the provision of further data on large homogenous populations to provide definitive criterion for pathological SpO(2) levels associated with dysphagia during oral feeding.
Morgan A T; Omahoney R; Francis H
Developmental Neurorehabilitation
2008
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<a href="http://doi.org/10.1080/17518420701439910" target="_blank" rel="noreferrer noopener">10.1080/17518420701439910</a>
Sleep problems in individuals with 11q terminal deletion disorder (Jacobsen syndrome)
children; Medical; behavior; Ethics; Genetics & Heredity; sleep; disturbance; sleep problems; disabilities; Research & Experimental Medicine; 11q terminal deletion disorder; Biotechnology & Applied Microbiology; Jacobsen syndrome; questionnaire; sleep disturbance/disorders; trajectory; characteristics
Characteristics of sleep and sleep problems were investigated in 43 individuals with 11q terminal deletion disorder (Jacobsen syndrome). Data were collected using a sleep questionnaire. Ten individuals (23%) had a sleep problem. Settling problems, frequent night waking and early waking occurred in 2 (4%), 7 (16%) and 2 (6%) individuals, respectively. Twenty-two individuals (54%) had a history of sleep problems. Twenty-five individuals (60%) showed restless sleep and 23 individuals (54%) slept in an unusual position. Apart from frequent coughs, no significant relationships were found between the presence of a sleep problem and other variables, such as age, level of ID, breathing problems, heart defects, constipation, daytime activity and behavioral diagnosis, restless sleep and sleeping in an unusual positions.
Maas A; Grossfeld P D; Didden R; Korzilius H; Braam W J; Smits M G; Curfs L M G
Genetic Counseling
2008
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<a href="http://doi.org/" target="_blank" rel="noreferrer noopener"></a>
Behavior problems in children with tuberous sclerosis complex and parental stress
cancer; families; adolescents; Neurosciences & Neurology; seizure; attention; epilepsy; disorders; autism; Psychiatry; hyperactivity; autism spectrum disorder; diagnostic-criteria; population; tuberous sclerosis complex; Behavioral Sciences; mental; behavioral problems; frequency; infantile spasms; methylphenidate; mixed seizure disorder; parenting stress; retardation; tsc1; tuberous sclerosis; trajectory; characteristics
Behavioral problems are common in children with tuberous sclerosis complex (TSC) and can be challenging to manage at home. Standardized measures were used to assess behavior in 99 pediatric patients with TSC and to evaluate parenting stress in their parents. About 40% of the pediatric patients presented clinically significant behavioral problems, most frequently involving symptoms of autism spectrum disorder, inattention, and hyperactivity. Higher seizure frequency, mixed seizure disorder, and low intellectual functioning placed the patient at significant risk for behavior problems. Almost 50% of participating parents reported experiencing clinically significant parenting stress, which was associated with specific characteristics of the child, including the presence of current seizures, a history of psychiatric diagnosis, low intelligence, and behavioral problems. Clinicians should be aware that behavioral problems are prominent in children with TSC. Referrals for behavioral intervention and monitoring of parental stress should be included in the medical management of children with TSC. (C) 2008 Elsevier Inc. All rights reserved.
Kopp C M C; Muzykewicz D A; Staley B A; Thiele E A; Pulsifer M B
Epilepsy and Behavior
2008
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<a href="http://doi.org/10.1016/j.yebeh.2008.05.010" target="_blank" rel="noreferrer noopener">10.1016/j.yebeh.2008.05.010</a>
Health and sleep problems in Cornelia de Lange Syndrome: a case control study
Male; Severity of Illness Index; Case-Control Studies; Comorbidity; Surveys and Questionnaires; Humans; Adolescent; Prevalence; Female; Health Status; De Lange Syndrome/epidemiology/psychology; Ear Diseases/epidemiology/psychology; Eye Diseases/epidemiology/psychology; Gastrointestinal Diseases/epidemiology/psychology; Hernia/epidemiology/psychology; Intellectual Disability/epidemiology/psychology; Self-Injurious Behavior/epidemiology/psychology; Skin Diseases/epidemiology/psychology; Sleep Wake Disorders/epidemiology; Stomatognathic Diseases/epidemiology/psychology; behavioral problems; sleep disturbance; De Lange syndrome; trajectory; characteristics; self-injury
BACKGROUND: Self-injury, sleep problems and health problems are commonly reported in Cornelia de Lange Syndrome (CdLS) but there are no comparisons with appropriately matched participants. The relationship between these areas and comparison to a control group is warranted. METHOD: 54 individuals with CdLS were compared with 46 participants with intellectual disability (ID) of mixed aetiology who were similar in terms of degree of ID, mobility, age and gender using informant-based measures of health problems, sleep and self-injury. RESULTS: Participants with CdLS experienced significantly more current and lifetime health problems with eye problems and gastrointestinal disorders prominent. Although 55% of those with CdLS experienced sleep problems this prevalence was not different from the comparison group. Sleep disorder was not associated with self-injury in the CdLS group, skin problems were associated with severity of self-injury but not presence. DISCUSSION: People with CdLS experience a range of significant health problems and have more health concerns than others with the same degree of ID. Although this has been well documented in the past, the current high prevalence of health concerns indicates the need for regular health assessment and appropriate intervention.
Hall S; Arron K; Sloneem J; Oliver C
Journal of Intellectual Disability Research
2008
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<a href="http://doi.org/10.1111/j.1365-2788.2008.01047.x" target="_blank" rel="noreferrer noopener">10.1111/j.1365-2788.2008.01047.x</a>
Gastrointestinal complications of epidermolysis bullosa in children
constipation; epidermolysis bullosa; acquisita; alpha-6-beta-4; balloon-dilatation; colitis; colon interposition; crohns-disease; Dermatology; esophageal strictures; expression; gastro-oesophageal reflux; gastrointestinal; manifestations; oesophageal strictures; pyloric atresia; vii collagen; feeding difficulties; trajectory; characteristics; GI problems; gastrointestinal problems
Background Epidermolysis bullosa (EB) is a group of inherited disorders characterized by skin and mucous membrane fragility. Gastrointestinal (GI) complications have been described in many types of EB and are responsible for significant morbidity. Objectives To delineate the nature and frequency of GI complications in a large cohort of paediatric patients with EB and to postulate why some complications occur more commonly in some specific subtypes. Methods The case notes of 223 children with EB seen at a national referral centre were examined retrospectively for the presence of GI symptoms, investigations and interventions. Results GI complications were present in 130/223 (58%) of all patients. In EB simplex, constipation and gastro-oesophageal reflux (GOR) were frequently observed. In junctional EB, failure to thrive and protein-losing enteropathy (PLE) were the prominent GI manifestations. Constipation was common in patients with dystrophic EB (DEB) requiring laxatives and in some cases fibre supplementation. GOR affected three-quarters of those with recessive DEB, two-thirds also having significant oesophageal strictures. Over half of patients with recessive DEB required gastrostomy insertion. Diarrhoea affected a small but significant proportion of children with recessive DEB with macroscopic and/or microscopic changes of colitis in the majority. Conclusion GI problems in EB are very common with subtype specificity for some of these complications. The occurrence of diarrhoea, PLE and colitis in the context of EB has not been highlighted previously, and may arise secondarily to antigenic exposure in the gut lumen as a result of mucosal fragility.
Freeman E B; Koglmeier J; Martinez A E; Mellerio J E; Haynes L; Sebire N J; Lindley K J; Shah N
British Journal of Dermatology
2008
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<a href="http://doi.org/10.1111/j.1365-2133.2008.08507.x" target="_blank" rel="noreferrer noopener">10.1111/j.1365-2133.2008.08507.x</a>
Esophageal motility dysfunction in children with Rett syndrome, gastroesophageal reflux, and dysphagia
feeding difficulties; Rett syndrome; trajectory; characteristics; gastroesophageal reflux; GERD; dysphagia; abnormal esophageal peristalsis
Fortunato JE; Darbari A; Cuffari C; Bibat G; Koch K; Desbiens J; Naidu S
Journal of Applied Research
2008
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<a href="http://doi.org/" target="_blank" rel="noreferrer noopener"></a>
The test of infant motor performance: reliability in spinal muscular atrophy type I
tone and motor problems; SMA1; tool development; scale development; TIMP
PURPOSE: The aim of this article was to determine reliability of the Test of Infant Motor Performance (TIMP) in infants with spinal muscular atrophy, type I (SMA-I). METHODS: Interrater reliability training was undertaken by 17 physical therapist evaluators using 6 infants with hypotonia and weakness (5 videotaped, 1 live). Eight trained evaluators then conducted a test-retest reliability study at their own center, performing 2 tests approximately 1 month apart on each of 11 infants with genetically confirmed SMA-I (5 boys, 6 girls; age range 37-501 days; 3 on pulmonary support). RESULTS: The interrater reliability training session had an overall weighted Kappa of 0.61 (95% confidence interval 0.59-0.63). For the test-retest reliability study, the intraclass correlation coefficient for the TIMP Total Score was 0.85 (95% confidence interval: 0.54-0.96). The test scores were not significantly different between the 2 sessions (Bradley-Blackwood test was nonsignificant). CONCLUSION: TIMP scores can be reliably obtained in infants with SMA-I.
Finkel R S; Hynan L S; Glanzman A M; Owens H; Nelson L; Cone S R; Campbell S K; Iannaccone S T; AmSmart Group
Pediatric Physical Therapy
2008
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<a href="http://doi.org/10.1097/PEP.0b013e318181ae96" target="_blank" rel="noreferrer noopener">10.1097/PEP.0b013e318181ae96</a>
Gross motor profile in rett syndrome as determined by video analysis
tone and motor problems; Rett syndrome; trajectory; characteristics
Movement impairment is a fundamental but variable component of the Rett syndrome phenotype. This study used video supplemented by parent report data to describe the gross motor profile in females with Rett syndrome (n=99) and to investigate the impact of age, genotype, scoliosis and hand stereotypies. Factor analysis enabled the calculation of general and complex gross motor skills scores. Most subjects were able to sit, slightly less than half were able to walk and a minority were able to transfer without assistance. General gross motor skills declined with age and were poorer in those who had surgically treated scoliosis but not conservatively managed scoliosis. Complex gross motor skills did not decline with age and were better in those without scoliosis. Those with a p.R133C, p.R294X, or a p.R255X mutation appear to have better motor skills overall than those with a p.R270X or large deletion mutation. Motor scores were not related to the frequency of hand stereotypies. This information is useful for the clinician and family when planning support strategies and interventions.
Downs J A; Bebbington A; Jacoby P; Msall M E; McIlroy O; Fyfe S; Bahi-Buisson N; Kaufmann W E; Leonard H
Neuropediatrics
2008
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<a href="http://doi.org/10.1055/s-0028-1104575" target="_blank" rel="noreferrer noopener">10.1055/s-0028-1104575</a>
Melatonin treatment in individuals with intellectual disability and chronic insomnia: a randomized placebo-controlled study
Saliva; Middle Aged; Treatment Outcome; Humans; Adolescent; Child Preschool; Double-Blind Method; Time Factors; Central Nervous System Depressants/adverse effects/therapeutic use; Melatonin/adverse effects/therapeutic use; Mental Retardation/epidemiology/psychology; Sleep Initiation and Maintenance Disorders/drug therapy/epidemiology/psychology; Q3 Literature Search; chronic disease; child; female; male; adult; comorbidity; aged; sleep disturbance/disorders; chromosome 18q deletion; MPS III; pharmacologic intervention; melatonin
BACKGROUND: While several small-number or open-label studies suggest that melatonin improves sleep in individuals with intellectual disabilities (ID) with chronic sleep disturbance, a larger randomized control trial is necessary to validate these promising results. METHODS: The effectiveness of melatonin for the treatment of chronic sleep disturbance was assessed in a randomized double-blind placebo-controlled trial with 51 individuals with ID. All of these individuals presented with chronic ideopatic sleep disturbance for more than 1 year. The study consisted of a 1-week baseline, followed by 4 weeks of treatment. Parents or other caregivers recorded lights off time, sleep onset time, night waking, wake up time and epileptic seizures. Endogenous melatonin cycle was measured in saliva before and after treatment. RESULTS: Compared with placebo, melatonin significantly advanced mean sleep onset time by 34 min, decreased mean sleep latency by 29 min, increased mean total sleep time by 48 min, reduced the mean number of times the person awoke during the night by 0.4, decreased the mean duration of these night waking periods by 17 min and advanced endogenous melatonin onset at night by an average of 2.01 h. Lights off time, sleep offset time and the number of nights per week with night waking did not change. Only few minor or temporary adverse reactions and no changes in seizure frequency were reported. CONCLUSIONS: Melatonin treatment improves some aspects of chronic sleep disturbance in individuals with ID.
Braam W; Didden R; Smits M; Curfs L
Journal of Intellectual Disability Research
2008
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/10.1111/j.1365-2788.2007.01016.x" target="_blank" rel="noreferrer noopener">10.1111/j.1365-2788.2007.01016.x</a>
Interventions for noisy breathing in patients near to death
Death; Humans; Terminal Care/mt [Methods]; Cholinergic Antagonists/tu [Therapeutic Use]; Respiratory Sounds/de [Drug Effects]; Scopolamine/tu [Therapeutic Use]; 0 (Cholinergic Antagonists); 0 (Muscarinic Antagonists); DL48G20X8X (Scopolamine); Muscarinic Antagonists/tu [Therapeutic Use]
BACKGROUND: Noisy breathing (death rattle) occurs in 23 to 92% of people who are dying. The cause of death rattle remains unproven but is presumed to be due to an accumulation of secretions in the airways. It is therefore managed physically (repositioning and clearing the upper airways of fluid with a mechanical sucker) or pharmacologically (with anticholinergic drugs). OBJECTIVES: To describe and assess the evidence for the effectiveness of interventions used to treat death rattle in patients close to death. SEARCH STRATEGY: Randomised controlled trials (RCTs), before and after studies and interrupted time series (ITS) studies in adults and children with death rattle were sought by MEDLINE (1966 to 2007), EMBASE (1980 to 2007), CINAHL (1980 to 2007), the Cochrane Pain, Palliative and Supportive Care Trials Register and the Cochrane Central Register of Controlled Trials. In addition, the reference lists of all relevant trials and reports were checked and investigators who are known to be researching this area were contacted for unpublished data or knowledge of the grey literature. SELECTION CRITERIA: RCTs, controlled before and after studies and ITS reporting the outcome of pharmacological and non-pharmacological interventions for treating death rattle. DATA COLLECTION AND ANALYSIS: Data was extracted by two independent review authors and trials were quality scored. There was insufficient data to carry out an analysis. MAIN RESULTS: Thirty studies were identified, of which only one study met the inclusion criteria. This small study was a randomised placebo-controlled trial of the use of hyoscine hydrobromide in patients with death rattle. Hyoscine hydrobromide tended to reduce death rattle compared to placebo but this was not significant. A larger randomised study, comparing atropine, hyoscine butylbromide and scopolamine, is in progress. AUTHORS' CONCLUSIONS: There is currently no evidence to show that any intervention, be it pharmacological or non-pharmacological, is superior to placebo in the treatment of death rattle. We acknowledge that in the face of heightened emotions when death is imminent, it is difficult for staff not to intervene. It is therefore likely that the current therapeutic options will continue to be used. However, patients need to be closely monitored for lack of therapeutic benefit and adverse effects while relatives need time, explanation and reassurance to relieve their fears and concerns. There is a need for more well-designed multi-centre studies with objective outcome measures and the ability to recruit sufficient numbers. [References: 37]
Wee B; Hillier R
Cochrane Database of Systematic Reviews
2008
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<a href="http://doi.org/10.1002/14651858.CD005177.pub2" target="_blank" rel="noreferrer noopener">10.1002/14651858.CD005177.pub2</a>
Care for patients in the last months of life: the Belgian Sentinel Network Monitoring End-of-Life Care study
Child; Delivery of Health Care; Female; Humans; Male; Palliative Care; Terminal Care; Adult; Aged; Middle Aged; Belgium; adolescent; Preschool; 80 and over; decision making; infant; Sentinel Surveillance
BACKGROUND: Population-based studies monitoring end-of-life care are lacking. This study describes involvement of caregivers, access to specialist palliative care, treatment goals (cure, life-prolonging, or palliation), and content of end-of-life care (physical, psychosocial, or spiritual) in a representative sample of dying persons in Belgium. METHODS: We performed a mortality follow-back study in 2005 (Sentinel Network Monitoring End-of-Life Care [SENTI-MELC] study). Data were collected via the nationwide Sentinel Network of General Practitioners, an epidemiological surveillance system representative of all Belgian general practitioners. Each week, all 205 participating practices reported all deaths of patients in their practice and registered the care provided in the final 3 months of life. Sudden, unexpected deaths were excluded. RESULTS: We studied 892 deaths. General practitioners, nurses or geriatric caregivers, and informal caregivers were often involved in end-of-life care in 76%, 78%, and 75% of cases, respectively. Specialist multidisciplinary palliative care services were provided in 41% of cases. Two to 3 months before death, a palliative treatment goal was in place for 37% of patients, increasing to 81% in the last week of life (P < .001). Two to 3 months before death, physical, psychosocial, and spiritual care was provided to a (very) large extent to 84%, 36%, and 10% of patients, respectively. These numbers increased to 90%, 54%, and 25%, respectively, in the last week of life (P < .001). CONCLUSIONS: In Belgium, most dying patients have both formal and informal caregivers. Provision of specialist palliative care is far less frequent. A transition from cure to palliation often occurs late in the dying process and sometimes not at all. Psychosocial and spiritual care is delivered considerably less frequently than physical care.
2008
Van den Block L; Deschepper R; Bossuyt N; Drieskens K; Bauwens S; Van Casteren V; Deliens L
Archives Of Internal Medicine
2008
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Journal Article
<a href="http://doi.org/10.1001/archinte.168.16.1747" target="_blank" rel="noreferrer">10.1001/archinte.168.16.1747</a>
Missed opportunities for interval empathy in lung cancer communication
2008
Morse DS; Edwardsen EA; Gordon HS
Archives Of Internal Medicine
2008
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Journal Article
<a href="http://doi.org/10.1001/archinte.168.17.1853" target="_blank" rel="noreferrer">10.1001/archinte.168.17.1853</a>
Progressive cerebral vascular degeneration with mitochondrial encephalopathy
Child; Female; Humans; Cerebrovascular Circulation; Q3 Literature Search; Acyl-CoA Dehydrogenase/deficiency/genetics; Brain/blood supply/physiopathology; Leu/genetics; Magnetic Resonance Angiography; MELAS Syndrome/ge [Genetics]; Point Mutation; RNA; Transfer
MELAS (mitochondrial encephalopathy with lactic acidosis and stroke-like episodes) is a maternally inherited disorder characterized by recurrent cerebral infarctions that do not conform to discreet vascular territories. Here we report on a patient who presented at 7 years of age with loss of consciousness and severe metabolic acidosis following vomiting and dehydration. She developed progressive sensorineural hearing loss, myopathy, ptosis, short stature, and mild developmental delays after normal early development. Biochemical testing identified metabolites characteristic of medium-chain acyl-CoA dehydrogenase (MCAD) deficiency (hexanoylglycine and suberylglycine), but also severe lactic acidemia (10-25 mM) and, in urine, excess of lactic acid, intermediates of the citric cycle, and marked ketonuria, suggesting mitochondrial dysfunction. She progressed rapidly to develop temporary cortical blindness. Brain imaging indicated generalized atrophy, more marked on the left side, in addition to white matter alterations consistent with a mitochondrial disorder. Magnetic resonance angiography indicated occlusion of the left cerebral artery with development of collateral circulation (Moyamoya syndrome). This process worsened over time to involve the other side of the brain. A muscle biopsy indicated the presence of numerous ragged red fibers. Molecular testing confirmed compound heterozygosity for the common mutation in the MCAD gene (985A>G) and a second pathogenic mutation (233T>C). MtDNA testing indicated that the muscle was almost homoplasmic for the 3243A>T mutation in tRNALeu, with a lower mutant load (about 50% heteroplasmy) in blood and skin fibroblasts. These results indicate that mitochondrial disorders may be associated with severe vascular disease resulting in Moyamoya syndrome. The contribution of the concomitant MCAD deficiency to the development of the phenotype in this case is unclear.
2008
Longo N; Schrijver I; Vogel H; Pique LM; Cowan TM; Pasquali M; Steinberg GK; Hedlund GL; Ernst SL; Gallagher RC; Enns GM
American Journal Of Medical Genetics.Part A
2008
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Journal Article
<a href="http://doi.org/10.1002/ajmg.a.31841" target="_blank" rel="noreferrer">10.1002/ajmg.a.31841</a>
The nervous system and gastrointestinal function
Humans; Brain/physiology; Central Nervous System/physiology; Gastrointestinal Motility/physiology; Gastrointestinal Tract/anatomy & histology/physiology; Intestinal Mucosa/physiology; Pain/physiopathology; Parasympathetic Nervous System/physiology; Sympathetic Nervous System/physiology
The enteric nervous system is an integrative brain with collection of neurons in the gastrointestinal tract which is capable of functioning independently of the central nervous system (CNS). The enteric nervous system modulates motility, secretions, microcirculation, immune and inflammatory responses of the gastrointestinal tract. Dysphagia, feeding intolerance, gastroesophageal reflux, abdominal pain, and constipation are few of the medical problems frequently encountered in children with developmental disabilities. Alteration in bowel motility have been described in most of these disorders and can results from a primary defect in the enteric neurons or central modulation. The development and physiology of the enteric nervous system is discussed along with the basic mechanisms involved in controlling various functions of the gastrointestinal tract. The intestinal motility, neurogastric reflexes, and brain perception of visceral hyperalgesia are also discussed. This will help better understand the pathophysiology of these disorders in children with developmental disabilities.
2008
Altaf MA; Sood MR
Developmental Disabilities Research Reviews
2008
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Journal Article
<a href="http://doi.org/10.1002/ddrr.15" target="_blank" rel="noreferrer">10.1002/ddrr.15</a>
The natural history of Unverricht-Lundborg disease: a report of eight genetically proven cases
Child; Female; Humans; Male; Adult; Disease Progression; Age Factors; Severity of Illness Index; Magnetic Resonance Imaging; adolescent; Q3 Literature Search; Diagnosis; Differential; Chromosomes; Human; Electroencephalography; Atrophy/complications/pathology; Auditory; Brain Stem/physiology; Brain/pathology/physiopathology; Cerebellar Ataxia/complications/diagnosis; Cerebellum/pathology/physiopathology; Dementia/complications/diagnosis; Dystonia/complications/diagnosis; Electromyography; Evoked Potentials; Evoked Potentials/physiology; Myoclonus/complications/diagnosis; Neuropsychological Tests; Pair 21/genetics; Seizures/complications/diagnosis; Unverricht-Lundborg Syndrome/diagnosis/genetics/physiopathology
We report eight cases of genetically proven ULD, with the aim of reassessing the clinical characteristics and natural history of ULD in genetically characterized patients. The eight patients had their first symptoms at mean age of 10.6 years (range: 6-14 years). The main clinical features were action myoclonus, cerebellar ataxia, seizures, and mild intellectual dysfunction. We report three new clinical features of ULD; ocular motor apraxia, dystonia, and rapidly progressive dementia. All patients needed a combination of at least four antimyoclonic drugs, but despite this, all patients were severely disabled by their action myoclonus. After a mean duration of disease of 29.9 years (range: 21-37 years), four patients were walking with aids while another four were wheelchair bound. The clinical phenotypes associated with ULD are more diverse than previously recognized and even though the long term functional outcome and survival have improved, the overall efficacy of antimyoclonic drugs remains unsatisfactory.
2008
Chew NK; Mir P; Edwards MJ; Cordivari C; Martino D; Schneider SA; Kim HT; Quinn NP; Bhatia KP
Movement Disorders: Official Journal Of The Movement Disorder Society
2008
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Journal Article
<a href="http://doi.org/10.1002/mds.21812" target="_blank" rel="noreferrer">10.1002/mds.21812</a>
Do as I say or die: compliance in adolescents with cancer
Adolescent Transitions; cancer; Adolescents; Compliance
Adolescence is a time of great physical change and maturing brain function. This leads to adolescents establishing independence and coming to terms with the implications of their own actions. Not surprisingly, this phase is characterized by experimentation with both constructive and destructive behavior. Studies in many areas of chronic illness have shown that adolscents frequently neglect their care and revolt against the rules established during their childhood. It is therefore to be expected that teenagers diagnosed with a life threatening illness, such as cancer, may on occasion not fully comply with their therapy. They way forward includes improving communication and fully involving these young persons in their treatment planning, thereby moving from compliance to concordance. Additional improvements should be sought in medication, early recognition and support of familial or social problems, and using a specific adolescent multidisciplinary team. Research should not be limited to clinical trials.
2008
Windebank KP; Spinetta J
Pediatric Blood & Cancer
2008
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Journal Article
<a href="http://doi.org/10.1002/pbc.21460" target="_blank" rel="noreferrer">10.1002/pbc.21460</a>
Integration of a palliative and terminal care center into a comprehensive pediatric oncology department
location of death
BACKGROUND: The sharp division between curative cancer therapy and palliative care results in the late introduction of palliative care and a high incidence of suffering in children with cancer. We established a Palliative Care Unit (PCU) that is fully integrated with the Pediatric Hematology Oncology Department (PHOD). We wished to explore the impact of such integrative model on patterns of hospitalizations and exposure to palliative care of pediatric oncology patients. PROCEDURES: Retrospective search of medical records of patients admitted to the PHOD since PCU establishment in 1999, and of children who died from progressive disease between 1990 and 2005 was performed. Differences in clinical and prognostic variables between PCU and non-PCU patients, and differences in location of death before and after PCU establishment were evaluated. RESULTS: The majority (59%) of patients, who were hospitalized after the PCU establishment, were hospitalized in the PCU, including 49% of the good prognosis patients and 91% of the poor prognosis patients. Poor prognosis patients were hospitalized in the PCU earlier and with higher frequency compared to children with curable disease. After PCU opening there was a significant decline in the percentage of patients who died in the general pediatric ward, hematology-oncology ward, and at home from 40%, 26% and 28% to 4%, 8%, and 16%, respectively. CONCLUSIONS: Our integrative model results in exposure of the majority of children with cancer to palliative care. For poor prognosis patients, palliative care is introduced early enough to allow gradual transition from symptom control after diagnosis to end of life care. Pediatr Blood Cancer (c) 2008 Wiley-Liss, Inc.
2008
Hana G; Bella B; Dorit G; Shai I; Gideon R; Amos T
Pediatric Blood & Cancer
2008
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Journal Article
<a href="http://doi.org/10.1002/pbc.21476" target="_blank" rel="noreferrer">10.1002/pbc.21476</a>
Integration of a palliative and terminal care center into a comprehensive pediatric oncology department
2008
Golan H; Bielorai B; Grebler D; Israeli Shai; Rechavi G; Toren A
Pediatric Blood & Cancer
2008
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Journal Article
<a href="http://doi.org/10.1002/pbc.21476" target="_blank" rel="noreferrer">10.1002/pbc.21476</a>
Methadone analgesia for children with advanced cancer
Methadone
BACKGROUND: Methadone is frequently used in the treatment of adults with advanced cancer. A criticism of relevant research is the use of single or fixed doses, which does not reflect use in clinical practice. Literature about use of methadone in the treatment of pediatric patients is limited to case reports. The objective of this study is to describe methadone use as primary opioid analgesic for advanced pediatric cancer over a 6.5-year period. PROCEDURE: All 17 patients who received methadone as their primary opioid analgesic through the Northern Alberta Children's Cancer Program from January 2000 to June 2007 were included. Children who received combination opioid therapy were excluded. RESULTS: Rotation to methadone was usually by a complete switch from primary opioid. Conversion ratios of morphine equivalent daily dose (MEDD)/methadone daily dose (TMDD) ranged widely from 1:2 in one patient with sudden pain crisis just prior to death, to 60:1 in a patient who had been treated with opioids for months. Methadone was used for a total of 925 patient-days. There were no significant adverse events in any patient, and all but one patient remained on methadone until the time of their death. Clinically, the effectiveness of analgesia clearly improved at time of conversion in 16 patients. CONCLUSION: With close monitoring, methadone therapy can be done safely in pediatric oncology patient populations in both inpatient and outpatient settings. Our experience suggests improvement in analgesia with the use of methadone, with 16 patients remaining on methadone until they died. Pediatr Blood Cancer (c) 2008 Wiley-Liss, Inc.
2008
Davies D; Devlaming D; Haines C
Pediatric Blood & Cancer
2008
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Journal Article
<a href="http://doi.org/10.1002/pbc.21584" target="_blank" rel="noreferrer">10.1002/pbc.21584</a>
IMPaCCT: standards of paediatric palliative care.
The needs of children and families living with life-limiting and life-threatening illness are similar across all European countries. Meeting these needs requires a comprehensive and integrative approach, with the input of a skilled multidisciplinary paediatric team. It is essential that the core standards for paediatric palliative care recommended in this document of the European Association for Palliative Care (EAPC) now be implemented across Europe.
2008
Craig F; Abu-Saad HH; Benini F; Kuttner L; Wood C; Feraris PC; Zernikow B
Schmerz (berlin, Germany)
2008
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Journal Article
<a href="http://doi.org/10.1007/s00482-008-0690-4" target="_blank" rel="noreferrer">10.1007/s00482-008-0690-4</a>
A medical home center: specializing in the care of children with special health care needs of high intensity
Cohen 2006 BMC HSR Refs
2008
Kelly A; Golnik A; Cady R
Maternal And Child Health Journal
2008
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Journal Article
<a href="http://doi.org/10.1007/s10995-007-0271-7" target="_blank" rel="noreferrer">10.1007/s10995-007-0271-7</a>
Comfort of general internists and general pediatricians in providing care for young adults with chronic illnesses of childhood
Cross-Sectional Studies; Female; Humans; Male; Young Adult; Adult; Attitude of Health Personnel; Aged; Middle Aged; adolescent; Adolescent Transitions; Pediatrics/methods/trends; Chronic Disease/psychology/therapy; Family Practice/methods/trends; Physician's Role/psychology
BACKGROUND: As an increasing number of patients with chronic conditions of childhood survive to adulthood, experts recommend that young adults with chronic conditions transfer from child-focused to adult-focused primary care. Little, however, is known about how comfortable physicians are caring for this population. OBJECTIVES: To assess the comfort of general internists and general pediatricians in treating young adult patients with chronic illnesses originating in childhood as well as the factors associated with comfort. PARTICIPANTS: In a random sample, 1288 of 2434 eligible US general internists and pediatricians completed a mailed survey (response rate = 53%). METHODS: We measured respondents' comfort level in providing primary care for a patient with sickle cell disease (SCD) or cystic fibrosis (CF). We also measured levels of disease familiarity, training and subspecialty support, as well as individual physician characteristics. RESULTS: Fifteen percent of general internists reported being comfortable as the primary care provider for adults with CF and 32% reported being comfortable providing primary care for adults with SCD, compared with 38% of pediatricians for CF (p 0.05). Less than half of general internists felt that their specialty should take primary care responsibility for adult patients with CF and SCD. CONCLUSIONS: A majority of general internists and pediatricians are not comfortable providing primary care for young adults with chronic illnesses of childhood origin, such as CF and SCD. Efforts to increase treatment comfort among providers may help with the transition to adult-focused care for the growing numbers of young adults with complex chronic conditions.
2008
Okumura MJ; Heisler M; Davis MM; Cabana MD; Demonner S; Kerr EA
Journal Of General Internal Medicine
2008
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Journal Article
<a href="http://doi.org/10.1007/s11606-008-0716-8" target="_blank" rel="noreferrer">10.1007/s11606-008-0716-8</a>
Associations between orthopaedic findings, ambulation and health-related quality of life in children with myelomeningocele
PEDI Study
PURPOSE: Modern principles for treatment of patients with myelomeningocele include early closure of the neural tube defect, neurosurgical treatment of hydrocephalus and treatment aimed at minimizing contractures and joint dislocations. The aim is to achieve a better survival rate and a better quality of life (QOL). Better ambulatory function is thought to improve the management of activities of daily living. This study focused on evaluating which factors might affect ambulation, function and health-related QOL in children with myelomeningocele. METHODS: Thirty-eight patients with neurological deficit from myelomeningocele were examined in an unbiased follow-up. This included a physical examination using validated methods for ambulatory function and neuromuscular status, chart reviews and evaluation of radiographs in terms of hip dislocation and spine deformity. The Pediatric Evaluation of Disability Inventory (PEDI) was used to measure mobility, self-care and social function, and the Child Health Questionnaire (CHQ-PF50) was used to measure QOL. RESULTS: Muscle function class, quadriceps strength, spasticity in hip and/or knee joint muscles and hip flexion contracture as well as the ambulatory level all affected functional mobility as well as self-care/PEDI. Patients with hip dislocation, spinal deformity or those who were mentally retarded also had significantly worse functional mobility. Besides being affected by the severity of the neurological lesion, self-care/PEDI was significantly impaired by hip flexion contracture and absence of functional ambulation. General health-related QOL was significantly lower in this patient group than for US norms. Nonambulatory and mentally retarded patients had a significantly lower physical function of their QOL (CHQ). CONCLUSIONS: The severity of the disease, i.e. reduced muscle strength and occurrence of spasticity around hip/knee, affected ambulation, functional mobility and self-care. Acquired deformities (hip dislocation and spine deformity) affected functional ambulation only. Patients with reduced functional mobility and self-care experienced lower physical QOL. Children with myelomeningocele had significantly reduced QOL compared to healthy individuals.
2008-02
Danielsson AJ; Bartonek A; Levey E; McHale K; Sponseller P; Saraste H
Journal Of Children's Orthopaedics
2008
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Journal Article
<a href="http://doi.org/10.1007/s11832-007-0069-6" target="_blank" rel="noreferrer">10.1007/s11832-007-0069-6</a>
Identity-in-context is not yet identity development-in-context
Adolescent Transitions
In this discussion, we summarize the main aspects of the research presented in the papers and discuss what we see as their strong points. To analyze recent developments in identity research, we compared the present issue with the special issue of the Journal of Adolescence in 1996 on 'Identity Development in Context'. In that issue it was concluded that further research is necessary to "detail the ways in which contextual variables may interact both with one another as well as with individual personality variables to determine the course of the identity formation process". In the papers in this issue, the influence of the context has received much more attention than 20 years ago. However, they still have serious limitations with regard to the study of the developmental process. Not more than one study uses a longitudinal design. To stimulate research into developmental processes we conclude with a plea for longitudinal studies over long periods, with enough measurement points to distinguish between different patterns, and for more conceptual attention for what identity development is about.
2008
Bosma H; Kunnen ES
Journal Of Adolescence
2008
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Journal Article
<a href="http://doi.org/10.1016/j.adolescence.2008.03.001" target="_blank" rel="noreferrer">10.1016/j.adolescence.2008.03.001</a>
Implementation of transition programs can prevent another lost generation of patients with congenital heart disease
Child; Humans; Adult; Health Services Needs and Demand; Treatment Outcome; Program Development; Longevity; Morbidity; adolescent; Adolescent Transitions; Heart Defects; Continuity of Patient Care/organization & administration; Aftercare/organization & administration; Congenital/complications/epidemiology/prevention & control; Patient Education as Topic/organization & administration
Congenital heart disease is the most frequently occurring birth defect. To date, more than 90% of the children born with a heart defect reach adulthood. Since many patients are prone to residua and sequelae, lifelong specialized care is required. However, studies indicate that about one-half to three-quarters of the patients are lost to follow-up when they have grown up. This has resulted in a virtual lost generation. Lapse of care is associated with significant morbidity. Therefore, implementation of strategies to prevent patients from failing to continue regular follow-up is critical. It is argued that transition programs that inform patients about the rationale for ongoing follow-up and that teach them how to navigate the medical system can avoid another lost generation.
2008
Moons P; Hilderson D; Van Deyk K
European Journal Of Cardiovascular Nursing
2008
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Journal Article
<a href="http://doi.org/10.1016/j.ejcnurse.2008.10.001" target="_blank" rel="noreferrer">10.1016/j.ejcnurse.2008.10.001</a>
Parental perception of cold extremities and other accompanying symptoms in children with cerebral palsy
Child; Female; Humans; Male; Adult; Data Collection; Parents; Questionnaires; Health Status; adolescent; Preschool; Pain/etiology; Q3 Literature Search; Body Temperature/physiology; Cerebral Palsy/complications/physiopathology; Constipation/etiology; Extremities/blood supply/physiology; Muscle Tonus/physiology; Sleep Disorders/etiology
Cold extremities have been noted in non-walking children with cerebral damage compared with healthy controls. Whether this is a general problem in children with cerebral palsy (CP) and associated with other symptoms is unknown. This study describes accompanying symptoms such as cold extremities, constipation, pain, sleeping disorders and impaired well-being in children with CP as well as treatment the children have undergone. Associations between cold extremities and other symptoms borne by the children were analysed and discussed. From information in postal surveys received from parents of children with CP, 107 children (60 boys and 47 girls) aged 5-13 years, mean 11 years 8 months (SD 2 years 11 months), were described and analysed. Besides neurological impairments, many children had cold extremities and pain, sleeping disorders, constipation, and impaired well-being. Most children had had one or more of these symptoms for over 1 year but the symptoms were largely untreated. Non-walkers generally had more symptoms than walkers. Although pain, constipation, and sleeping disorders may have different underlying causes in children with CP, these symptoms might also be mediated or aggravated by dysfunction in the autonomic nervous system. To improve the child's well-being, early recognition and treatment of accompanying symptoms is important.
2008
Svedberg LE; Englund E; Malker H; Stener-Victorin E
European Journal Of Paediatric Neurology
2008
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Journal Article
<a href="http://doi.org/10.1016/j.ejpn.2007.06.004" target="_blank" rel="noreferrer">10.1016/j.ejpn.2007.06.004</a>
Understanding Canadian Punjabi-speaking South Asian women's experience of breast cancer: A qualitative study
2008
Gurm BK; Stephen J; Mackenzie G; Doll R; Barroetavena MC; Cadell S
International Journal Of Nursing Studies
2008
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Journal Article
<a href="http://doi.org/10.1016/j.ijnurstu.2006.08.023" target="_blank" rel="noreferrer">10.1016/j.ijnurstu.2006.08.023</a>
ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons
Humans; United States; Adult; Adolescent Transitions; Heart Defects; Congenital/therapy; Heart Diseases/congenital/therapy; American Heart Association; Cardiology/methods/standards
2008
Warnes CA; Williams RG; Bashore TM; Child JS; Connolly HM; Dearani JA; del Nido P; Fasules JW; Graham TP; Hijazi ZM; Hunt SA; King ME; Landzberg MJ; Miner PD; Radford MJ; Walsh EP; Webb GD; Smith SC; Jacobs AK; Adams CD; Anderson JL; Antman EM; Buller CE; Creager MA; Ettinger SM; Halperin JL; Hunt SA; Krumholz HM; Kushner FG; Lytle BW; Nishimura RA; Page RL; Riegel B; Tarkington LG; Yancy CW; American College of Cardiology; American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease); American Society of Echocardiography; Heart Rhythm Society; International Society for Adult Congenital Heart Disease; Society for Cardiovascular Angiography; Interventions; Society of Thoracic Surgeons
Journal Of The American College Of Cardiology
2008
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Journal Article
<a href="http://doi.org/10.1016/j.jacc.2008.10.001" target="_blank" rel="noreferrer">10.1016/j.jacc.2008.10.001</a>
Can you give me respect? Experiences of the urban poor on a dedicated AIDS nursing home unit
2008
Hughes A; Davies B; Gudmundsdottir M
Journal Of The Association Of Nurses In Aids Care
2008
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Journal Article
<a href="http://doi.org/10.1016/j.jana.2008.04.008" target="_blank" rel="noreferrer">10.1016/j.jana.2008.04.008</a>
An international/multicentre report on patients with cystic fibrosis (CF) over the age of 40 years
Female; Humans; Male; Survival Rate; Cohort Studies; Adult; Canada; Aged; Middle Aged; Minnesota; Longevity; Italy; Adolescent Transitions; retrospective studies; Age Distribution; Life Expectancy; Sex Distribution; Cystic Fibrosis/complications/epidemiology/physiopathology; London
BACKGROUND: The lifespan of patients with cystic fibrosis (CF) is increasing significantly. The objective of this international pilot study was to study the characteristics of these long-term survivors. METHODS: Four centres with large CF clinics from London (UK), Minneapolis (USA), Toronto (Canada) and Verona (Italy) identified 366 patients who had survived 40 years and longer. RESULTS: At all centres males survived longer than females. There were more pancreatic sufficient patients in Verona (60%) and Toronto (40%) than in London (16%) and Minneapolis (21%). The percentage of DeltaF508 homozygous patients varied between 47% in London and 45% in Minneapolis to only 26% in Toronto and 9% in Verona. Average FEV(1) and BMI values of the surviving population appeared to stabilise after 40 years of age. FEV(1) was on average 12% higher in patients who were pancreatic sufficient (p > 0.0001). There was no difference in survival between the centres. The overall median survival after the age of 40 was 13 years. The estimated annual death rate was approximately 3.4% from the age of 40-60 years. CONCLUSIONS: Significant numbers of patients are now surviving to 40 years or more, and it is hoped that an in-depth study of these patients may identify the factors contributing to longer survival.
2008
Hodson ME; Simmonds NJ; Warwick WJ; Tullis E; Castellani C; Assael B; Dodge JA; Corey M; International Study Of Aging In Cystic Fibrosis
Journal Of Cystic Fibrosis
2008
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.jcf.2008.06.003" target="_blank" rel="noreferrer">10.1016/j.jcf.2008.06.003</a>
Does palliative care improve quality? A survey of bereaved family members
Female; Humans; Male; Terminal Care; Family; Adult; Data Collection; Aged; Middle Aged; Treatment Outcome; New York; adolescent; 80 and over; bereavement; Palliative Care/statistics & numerical data; Patient Satisfaction/statistics & numerical data; Health Care; Quality Assurance; Pain/epidemiology/nursing
Palliative care is the interdisciplinary specialty that aims to relieve suffering and improve the quality of care for patients with serious illness and their families. Although palliative care programs are becoming increasingly prevalent in U.S. hospitals, the impact of hospital palliative care consultation programs on the quality of care received by family members is not well understood. We conducted prospective quantitative telephonic interviews of family members of patients who died at Mount Sinai Medical Center between April and December 2005 using the validated "After-Death Bereaved Family Member Interview," to assess quality of medical care at the end of life. Multivariable techniques were used to compare family satisfaction of palliative care patients vs. usual care patients controlling for age, race (white vs. nonwhite), diagnosis (cancer vs. noncancer), socioeconomic status (Medicaid vs. non-Medicaid), and functional status (number of dependent activities of daily living). One hundred ninety eligible subjects were contacted and successful interviews were completed with 149 (78.4%) family members (54 palliative care and 95 usual care patients). Palliative care showed benefit, with 65% of palliative care patients' family members reporting that their emotional or spiritual needs were met, as compared to 35% of usual care patients' family members (P=0.004). Sixty-seven percent of palliative care patients' family members reported confidence in one or more self-efficacy domains, as compared to 44% of usual care patients' family members (P=0.03). Our study shows that palliative care consultation is associated with improved satisfaction, with attention to family and enhanced self-efficacy. Palliative care offers a unique approach by integrating the needs of the family into the care of the patient.
2008
Gelfman LP; Meier DE; Morrison RS
Journal Of Pain And Symptom Management
2008
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.jpainsymman.2007.09.008" target="_blank" rel="noreferrer">10.1016/j.jpainsymman.2007.09.008</a>
Haloperidol as a palliative anti-emetic in a toddler: an evidence base challenge
Humans; infant; Male; Palliative Care; Antiemetics; Nausea; Fatal Outcome; Evidence-Based Medicine; Precursor Cell Lymphoblastic Leukemia-Lymphoma; Haloperidol
2008
Siden H
Journal Of Pain And Symptom Management
2008
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.jpainsymman.2007.10.001" target="_blank" rel="noreferrer">10.1016/j.jpainsymman.2007.10.001</a>
Opioid use in palliative care of children and young people with cancer
Child; Female; Humans; Male; Great Britain; Adult; Analgesics; Questionnaires; Prospective Studies; Research Design; Longitudinal Studies; Therapeutic Equivalency; Prescriptions; adolescent; Preschool; infant; Administration; Oral; Pain/drug therapy/etiology; Palliative Care/methods; Infusions; Injections; Intravenous; Neoplasms/complications; Subcutaneous; Drug/statistics & numerical data; Opioid/administration & dosage/therapeutic use; Fentanyl/therapeutic use; Heroin/therapeutic use; Morphine/therapeutic use; Rectal
OBJECTIVE: Identify opioids prescribed, preferred routes, and doses among children with incurable cancer. STUDY DESIGN: Prospective survey with monthly questionnaires regarding patients 0 to 19 years old from oncology centers. Data were collected by professionals on each patient for 6 months or until death, and analyzed from patients who died. Impact of tumor was analyzed with Kruskal-Wallis and Mann-Whitney tests. Major opioid dosages are expressed as oral morphine equivalents. RESULTS: Of 185 children recruited, 164 (88 boys, 76 girls) died. Mean palliative care duration was 67 days. One hundred forty-seven (89.6%) received major opioids. Morphine, diamorphine, and fentanyl were prescribed in 75%, 57.9%, and 11.6%, respectively. Seventy-three (44.5%) received >1 major opioid. Median monthly maximum doses prescribed rose from 2.1 mg/kg/24 h (study entry) to 4.4 mg/kg/24 h (death) (P < .001); overall variable (0.09-1500 mg/kg/24 h, median 3.7 mg/kg/24 h). Opioids were given by the oral (117/164, 71.3%), intravenous (68/164, 41.5%), subcutaneous (40, 28%), rectal (20, 12.2%), and transdermal (18, 11%) routes. There was a shift to intravenous use as death approached. Numbers within each tumor group were too small to show significance. Children with solid tumors outside the central nervous system were likely to receive more opioids, be given multiple different opioids, and receive opioids in the last month. CONCLUSIONS: The study shows the United Kingdom practice of opioid use and provides comparator data for practice in children's palliative medicine.
2008
Hewitt M; Goldman A; Collins GS; Childs M; Hain R
The Journal Of Pediatrics
2008
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.jpeds.2007.07.005" target="_blank" rel="noreferrer">10.1016/j.jpeds.2007.07.005</a>