Browse Items · Pediatric Palliative Care Library

Correlates of maladaptive behavior in individuals with 5p- (cri du chat) syndrome

This study examined the range, distinctiveness, and correlates of maladaptive behavior in 146 subjects with 5p- (cri du chat) syndrome using the Aberrant Behavior Checklist as a standardized measure. Hyperactivity was the most significant and…

Self-injurious behaviour in young children with Lesch-Nyhan syndrome

The early development of self-injurious behaviour in three young boys (aged 17, 25, and 30 months at start of study) with Lesch-Nyhan syndrome was examined by means of parental interviews and by direct observations completed at 3 to 4 monthly…

Tags: 2001, behavioral problems, characteristics, Child Preschool, Developmental Medicine and Child Neurology, Environment, Extinction Psychological, Hall S, Humans, Lesch-Nyhan syndrome, Lesch-Nyhan Syndrome/diagnosis/prevention & control, Male, Murphy G, Oliver C, Self-Injurious Behavior/prevention & control, self-injury, Severity Of Illness Index, Sign Language, Trajectory

Sleep disorder and epilepsy in children with tuberous sclerosis: a questionnaire-based study

Sleep disorders were investigated in 40 children with tuberous sclerosis (TS) and compared with those found in non-disabled children and those reported in a mixed group with learning disabilities. Significantly higher levels of sleep disturbance were…

Assessment of impaired coordination between respiration and deglutition in children and young adults with ataxia telangiectasia

AimThis cross-sectional investigation aimed to assess the value of non-invasive measures of temporal respiratory-swallow coupling in individuals with ataxic swallowing. MethodTwenty participants (11 males, 9 females; range 9-21y) with ataxia…

Sleep dysfunction in Rett syndrome: a trial of exogenous melatonin treatment

Nine girls with Rett syndrome (mean age, 10.1 years) were monitored 24 hours a day over a period of 10 weeks using wrist actigraphy. Baseline sleep-wake patterns were assessed for 1 week. Subsequently, patients underwent a 4-week melatonin treatment…

Tags: 1998, Budden S S, Developmental Medicine and Child Neurology, McArthur A J, melatonin, pharmacologic intervention, Rett syndrome, sleep disturbance/disorders

Air swallowing in Rett syndrome

The possible causes of excessive swallowing of air leading to bloating, which is common in Rett syndrome (RS), were investigated during feeding and at rest. Seven individuals with RS aged between 4 and 33 years (three with air bloat) underwent…

Behaviour in Cornelia de Lange syndrome: a systematic review

AIM Careful study and accurate description of behaviour are important to understand developmental challenges for individuals with Cornelia de Lange syndrome (CdLS). Here we present a systematic review of current understanding of behaviour in CdLS.…

Tags: 2017, behavioral problems, characteristics, De Lange syndrome, Developmental Medicine and Child Neurology, Hennekam R C, Huisman S A, Mulder P A, Oliver C, Piening S, Trajectory, Van Balkom I D C

Use of melatonin to treat sleep disorders in tuberous sclerosis

The results of a therapeutic trial of the use of melatonin in patients with tuberous sclerosis complex who also have severe sleep problems are reported. We used a randomized double-blind placebo-controlled crossover design. Seven patients with…

Tags: 1999, Clarke A, Developmental Medicine and Child Neurology, Hancock E, Hunt A B A, melatonin, O'Callaghan F J K, Osborne J P, pharmacologic intervention, sleep disturbance/disorders, Tuberous Sclerosis

Systematic review of melatonin treatment in children with neurodevelopmental disabilities and sleep impairment

Sleep disturbances in children with neurodevelopmental disabilities are common and frequently difficult to treat with conventional pharmacological and behavioural methods. Melatonin is a pineal hormone known to be important in the regulation of the…

Tags: 2004, Antioxidants/therapeutic use, Appleton R E, Brain/physiopathology, Child, Developmental Disabilities/drug therapy/epidemiology/physiopathology, Developmental Medicine and Child Neurology, Humans, melatonin, Melatonin/therapeutic use, pharmacologic intervention, Phillips L, Rett syndrome, sleep disturbance/disorders, Sleep Wake Disorders/epidemiology, Wakefulness/physiology

TEACHING SELF-FEEDING SKILLS TO PATIENTS WITH RETT-SYNDROME

Few studies have examined the extent to which functional hand-use may be taught to girls with Rett syndrome after those skills have been lost. In this investigation, five females with classic Rett syndrome taught to feed themselves using a method of…

Tags: 1993, Anderson C, Developmental Medicine and Child Neurology, feeding difficulties, Fisher W, girls, Neurosciences & Neurology, Pediatrics, Piazza C C, psychological intervention, Rett syndrome, splints, tone and motor problems

Modes and patterns of self-mutilation in persons with Lesch-Nyhan disease

Lesch-Nyhan disease (LND) is a rare X-linked recessive genetic disorder associated with cognitive impairment, choreoathetosis, hyperuricemia, and the hallmark symptom of severe and involuntary self-mutilation. This study examines data gathered from a…

Gross motor abilities in children with Hurler syndrome

Hurler syndrome is the most severe form of mucopolysaccharidosis type I. There is a paucity of literature reporting the gross motor abilities of children with untreated Hurler syndrome. The purpose of this case series is to describe the gross motor…

Tags: 2006, characteristics, Developmental Medicine and Child Neurology, Dusing S C, Escolar M L, Female, Humans, Infant, Male, Mercer V, Motor Activity, MPSI, Mucopolysaccharidosis I, Rosenberg A, Thorpe D, tone and motor problems, Trajectory

Melatonin treatment for sleep disorders in children with neurodevelopmental disorders: an observational study

The study aim was to quantify melatonin-associated improvement in sleep by means of a parent-completed sleep diary during routine outpatient activity. An investigation into sleep disturbance was made at neurology outpatient appointments. Those…

Behavioral aspects of Lesch-Nyhan disease and its variants

Self-injury is a defining feature of lesch-nyhan disease (LND) but does not occur in the less severely affected Lesch-Nyhan variants (LNV). The aim of this study was to quantify behavioral and emotional abnormalities in LND and LNV. Thirty-nine…

End of life care in PICU across the UK

Objective: The care provided in the time surrounding the death of a child shapes long-term memories and has potential to impact on the grieving process. There are no specific guidelines for PICU staff in relation to what good care looks like at this…

Tags: 2020, Developmental Medicine and Child Neurology, End-of-life Care, Huey R, Jennings J, July 2020 List, Maxwell V, Pediatric Intensive Care Unit, Picu, Richardson J, UK, United Kingdom

Challenges primary care providers face when caring for children with medical complexity

Background and Objective(s): Children with medical complexity (CMC) have multiple chronic health problems resulting in functional limitations and high resource utilization. Providing primary care for CMC can be time consuming and costly. This study…

Tags: 2019, Children, Children With Medical Complexity, CMC, Developmental Medicine and Child Neurology, Goodwin E, July 2020 List, Kennedy M, Medical Complexity, PCP, primary care providers, Savolt T, Smith T

The Chameleon Project: A children's end of life care quality improvement project

Objective: NHS England's Marginal Rate Emergency Threshold (MRET) and Readmission Fund funded the Chameleon Project 2018 (Twitter account: @chameleonproje1), to improve children's end of life care. This funded a lead disability paediatrician with…

Gastrostomy tube insertion in children with developmental or acquired disorders: a register-based study

Aim: To describe trends in gastrostomy tube insertion in children with developmental or acquired disorders in Sweden and assess their demographic characteristics. Method(s): Children aged 0 to 18 years with gastrostomy tube insertions recorded…

Tags: 2020, acquired disorders, Backman E, Children, Developmental disorders, Developmental Medicine and Child Neurology, gastronomy tube, gastronomy tube insertion, September 2020 List, Sjogreen L, Sweden

Advance Care Plan Discussion among Parents of Children with Cerebral Palsy

Aim: To evaluate parental perception of advance care plan (ACP) discussions in families of Malaysian children with bilateral cerebral palsy (CP) classified in Gross Motor Function Classification System levels IV or V for (1) acceptance of the ACP…

Tags: 2022, Adnan A, advance care plan, Cerebral Palsy, Child, Comorbidity, Developmental Medicine and Child Neurology, Fong CY, Ganesan V, Khalid F, Li L, Lim WK, Ng Voon SI, Ong L C, School, September 2022 List, Teh C M, Terminal Care

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