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BACKGROUND: Fathers' interactions with children who have intellectual disabilities (ID) or developmental delays (DD) have increased over the past few decades and may be expected to continue to increase as maternal and paternal roles, along with other…
Participant recruitment is one of the most significant challenges in research on intellectual disability (ID). One potential solution is to develop a participant contact registry, which allows the researcher to contact participants directly rather…
Background: Children with severe psychomotor impairment (SPMI) often experience sleep disturbances that severely distress both the child and his or her parents. Validated questionnaires for the assessment of parents' distress related to their child's…
Introduction: Obstructive Sleep Apnea (OSA) is a common medical problem in adults that is becoming increasingly recognized in children. It occurs in the pediatric age group, from newborns to teens. More recently, many specialists have estimated OSA…
We compared PECS and VOCA intervention strategies to promote communication opportunities for three girls with Rett syndrome and severe to profound developmental disabilities. The first aim of the study was to assess the effectiveness of both…
Cornelia de Lange syndrome (CdLS) is a cohesinopathy causing delayed growth and limb deficits. Individuals with CdLS have mild to profound intellectual disability and autistic features. This study characterizes the behavioral phenotype of children…
BACKGROUND: Chromosome 22q11.2 deletion syndrome (22q11.2DS), fragile X syndrome (FXS), and Turner syndrome (TS) are complex and variable developmental syndromes caused by different genetic abnormalities; yet, they share similar cognitive impairments…
Rett syndrome (RTT) is a neurodevelopmental disorder primarily affecting females. It is characterized by apparently normative development of motor and communicative abilities followed by deterioration in these domains. Stereotypic hand movements are…
Studies of individuals with Cornelia de Lange syndrome (CdLS) have described changes in mood and behavior with age, although no empirical or longitudinal studies have been conducted. Caregivers of individuals with CdLS (N = 67), cri du chat syndrome…
The aim of this prospective longitudinal multi centric study was to evaluate the correlation between the Hammersmith Functional Motor Scale and the 20 item version of the Motor Function Measure in non ambulant SMA children and adults at baseline and…
Sleep disturbances are prevalent in mucopolysaccharidosis Type III (MPS III), yet there is a lack of objective, ecologically valid evidence detailing sleep quantity, quality or circadian system. Eight children with MPS III and eight age-matched…
Background: Research reporting prevalence rates of self-injurious and aggressive behaviour in people with tuberous sclerosis complex (TSC) is limited. No studies have compared rates of these behaviours in TSC with those in other syndrome groups…
PubMed Central Canada (PMC Canada) provides free access to a stable and permanent online digital archive of full-text, peer-reviewed health and life sciences research publications. It builds on PubMed Central (PMC), the U.S. National Institutes of…
Cataplexy is a brief episode of bilateral loss of muscle tone with intact consciousness, triggered by a variety of strong emotions such as anger, laugh, humor or surprise and it is considered to represent the physiologic atonia of rapid eye movement…
BACKGROUND: Children with 22q11.2 deletion syndrome (22q11DS) are at risk for social-behavioural and neurocognitive sequelae throughout development. The current study examined the impact of family environmental characteristics on social-behavioural…
In this study, we present preliminary data on cognitive, behavioral and communication domains of individuals with Cornelia de Lange Syndrome (CdLS), collected through a specific protocol combining direct and indirect tools. Seventeen subjects with…
PURPOSE: The purpose was to examine the characteristics of children who use hospice care. DESIGN AND METHODS: Using the Andersen Model of Health Services Use, California Medicaid administrative databases were analyzed to describe the characteristics…
Palliative care services in England lack data on the number of children with 'life limiting conditions' (LLCs). Recent research determined that the prevalence of LLCs in children in England was double previous estimates. We build on this by analysing…
Differences in perspective between physicians caring for children with trisomy 18 may be confusing and stressful for parents. The hypothesis of this study was that neonatologists and pediatric pulmonologists differ in their opinions regarding…
It was only after James P. Lovette's death, in 2006, that I discovered that the twenty-four-year-old colleague and friend with whom I had spent so many afternoons debating issues in organ transplantation had been the first successful child heart…
BACKGROUND: The influence of socio-economic and religious background on decisions made by parents of children with incurable cancer regarding DNR orders is not fully understood. PROCEDURE: A retrospective analysis of medical charts of patients who…
BACKGROUND: The most common, debilitating morbidity of sickle cell disease (SCD) is vaso-occlusive crisis (VOC) pain. Although guidelines exist for its management, they are generally not well-followed, and research in other pediatric diseases has…
BACKGROUND: Relapsed childhood acute myeloid leukemia (AML) outcomes have not been documented in resource-limited settings. We examined survival after relapse for children with AML (non-APML) and acute promyelocytic leukemia (APML) in Central…
BACKGROUND: Rhabdomyosarcomas (RMS) of the nasolabial fold can be difficult to manage surgically due to functional and cosmetic limitations. Therefore, brachytherapy (BT) has been proposed to improve local control while limiting the volume of…
BACKGROUND: We evaluated the efficacy of gemcitabine and docetaxel chemotherapy (GEM + DOC) in children and adolescents with recurrent or refractory osteosarcoma. METHODS: Data of 28 patients (20 male, 8 female) who received gemcitabine (675 or 900…
BACKGROUND: There is currently no published, validated measures available that comprehensively capture quality of life (QoL) symptoms for children with poor-prognosis malignancies. The pediatric advanced care-quality of life scale (PAC-QoL) has been…
BACKGROUND: The management of children with cancer during the end-of-life (EOL) period is often difficult and requires skilled medical professionals. Patients with tumors of the central nervous system (CNS) with relapse or disease progression might…
A reliable estimation of prognosis in patients receiving palliative care is desirable in order to facilitate clinical decision finding. For patients with advanced hematological malignancies, only few data are available to estimate prognosis of the…
PURPOSE: Systematic knowledge about the prevalence and the treatment effects of cancer pain in patients attending a general oncology outpatient department is limited. The purpose of this study was to investigate the prevalence of pain in a large…
PURPOSE: The aims of this study are to describe the attitudes and referral practices of pediatric oncologists (POs) to specialized palliative care (SPC), and to compare them with those of adult oncologists (AOs). METHODS: Canadian members of the…
PURPOSE: Promoting parent resilience may provide an opportunity to improve family-level survivorship after pediatric cancer; however, measuring resilience is challenging. METHODS: The "Understanding Resilience in Parents of Children with Cancer" was…
We report the second family with AIMP1 deficiency, due to a homozygous truncating AIMP1 (g.107248613 C > T) mutation. This female showed early-onset developmental arrest, intractable epileptic spasms, microcephaly, and a rapid clinical course leading…
BACKGROUND: Children with acute lymphoblastic leukemia (ALL) undergo multiple lumbar punctures (LPs) during their course of treatment for diagnostic and therapeutic purposes. LP is a stressful and painful procedure, affecting the quality of life of…
Specificities of situation of individuals with multiple disabilities and pediatric neurological pathologies call for specialized and multi-field competences that are commonly allowed and disallowed in contemporary clinical contexts. However what must…
Myocarditis in children can result in significant morbidity and mortality, yet limited prognostic data exist. The aim of this study was to test the hypothesis that pediatric patients with arrhythmias during hospitalization for acute myocarditis have…
This multiorganizational literature review was undertaken to provide an evidence base for determining whether or not recommendations for out-of-hospital termination of resuscitation could be made for children who are victims of traumatic…
Since 2005, forgoing live-support (FLS) is allowed by the French law (known as the Leonetti law) for end-of-life patients only. This study aims at describing the variations over time in the use of the following methods to end life: FLS, brain death…
INTRODUCTION AND OBJECTIVES: To identify the knowledge of caregivers of pediatric intensive care units (PICUs) on the French law related to patients' rights and end of life, their views on withholding/withdrawing life-sustaining treatment (WWLST)…
BACKGROUND: The need for educational training of healthcare professionals in palliative care is an important issue. Training and practice of pediatric residents in the field of pediatric palliative care (PPC) has never been assessed, although the…
