Browse Items · Pediatric Palliative Care Library

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…

Moral Distress of Clinicians in Canadian Pediatric and Neonatal ICUs

OBJECTIVE: To quantify moral distress in neonatal ICU and PICU clinicians and to identify associated factors. DESIGN: A national cross-sectional survey of clinicians working in an neonatal ICU or PICU. Moral distress was assessed with the Moral…

Neonatal Palliative Care: Assessing the Nurses Educational Needs for Terminally Ill Patients

BACKGROUND: For terminally sick neonates and their families, it's crucial to provide holistic nursing care that incorporates both curative and palliative care as much as feasible. It is well known that the biggest obstacle to delivering palliative…

Neonatologists and neonatal nurses have positive attitudes towards perinatal end-of-life decisions, a nationwide survey

AIM: Perinatal death is often preceded by an end-of-life decision (ELD). Disparate hospital policies, complex legal frameworks and ethically difficult cases make attitudes important. This study investigated attitudes of neonatologists and nurses…

Newborn genetic screening for spinal muscular atrophy in the UK: The views of the general population

BACKGROUND: Spinal muscular atrophy (SMA) is an inherited neuromuscular disorder and a leading genetic cause of infant death worldwide. However, there is no routine screening program for SMA in the UK. Lack of treatments and the inability of…

Paediatric Palliative Care: A Survey Of Paediatricians And Family Practitioners

BACKGROUND: Paediatric palliative care focuses on the enhancement of the quality of life for a child and family through a combination of active and compassionate therapies intended to comfort and support the child and family who are living with a…

Pain in Nonverbal Children with Medical Complexity: A Two-Year Retrospective Study

Pain therapy, pediatric palliative care and end-of-life care: training, experience, and reactions of pediatric residents in Italy

UNLABELLED: Pediatric palliative care represents the ideal response to life-limiting and life-threatening diseases and requires a specific and multidisciplinary training. This study aims at evaluating in Italy the training programs offered in pain…

Parental assessment of comfort in newborns affected by life-limiting conditions treated by a standardized neonatal comfort care program

OBJECTIVE: To assess the perception of parents concerning the state of comfort maintained in their infants born with life-limiting conditions and treated by a standardized neonatal comfort care program. STUDY DESIGN: Participants were parents (n=35…

Parents' experiences of pediatric palliative care and the impact on long-term parental grief.

CONTEXT: Approximately 25% of children diagnosed with cancer eventually die. Losing a child puts parents at increased risk for developing psychological problems. OBJECTIVES: To explore parents' perceptions of the interaction with health care…

Pediatrician Ambiguity In Understanding Palliative Sedation At The End Of Life

CONTEXT: Palliative sedation is a means of relieving intractable symptoms at the end of life, however, guidelines about its use lack consistency. In addition, ethical concerns persist around the practice. There are reports of palliative sedation in…

Practical Recommendations For Diagnosis And Management Of Respiratory Muscle Weakness In Late-onset Pompe Disease

Pompe disease is an autosomal-recessive lysosomal storage disorder characterized by progressive myopathy with proximal muscle weakness, respiratory muscle dysfunction, and cardiomyopathy (in infants only). In patients with juvenile or adult disease…

Provision Of Services In Perinatal Palliative Care: A Multicenter Survey In The United States.

BACKGROUND:
Congenital anomalies account for 20% of neonatal and infant deaths in the United States. Perinatal palliative care is a recent addition to palliative care and is meant to meet the needs of families who choose to continue a pregnancy…

Psychological distress, health, and socio-economic factors in caregivers of terminally ill patients: a nationwide population-based cohort study

PURPOSE: At some point in life, most people become caregivers to a terminally ill relative. Previous studies have shown that many caregivers experience psychological distress and declining physical health, but these studies have predominantly been…

Psychosocial adjustment in siblings of young people with Duchenne muscular dystrophy

Duchenne muscular dystrophy (DMD) is a progressive, impairing, life-limiting disorder of childhood. Little is known about how siblings adapt to this. The aim of this study is to document psychosocial adjustment in siblings of patients with DMD.…

Tags: 2010, Adaptation Psychological, Adolescent, Child, December 2017 List, European Journal of Paediatric Neurology, Female, Garralda ME, Humans, Kinali M, Male, Mental Health, Muntoni F, Muscular Dystrophy Duchenne/px [psychology], Psychiatric Status Rating Scales, Psychology, Read J, Siblings/px [psychology], Surveys And Questionnaires

Quality of life in childhood advanced cancer: from conceptualization to assessment with the Advance QoL tool

BACKGROUND: Advanced childhood cancer, a condition with no available cancer-focused treatment options, greatly impacts Quality of Life (QoL). We need appropriate assessment strategies to select adapted treatment targets, improve care and optimize…

Research barriers in children and young people with life-limiting conditions: a survey

Studies indicate research ethics committee (REC) approval and clinician gatekeeping are two key barriers in recruiting children and young people (CYP) with life-limiting conditions (LLCs) and life-threatening illnesses (LTIs) and their families to…

Research participation experiences of parents of children with cancer who were asked about their child's prognosis

BACKGROUND: In questionnaire-based research, human subject protection committees must assess the emotional impact of the study on participants. Without clear data about the risks and benefits of participating in such studies, however, review board…

Sixty-four patients with Brachmann-de Lange syndrome: a survey

We surveyed 64 individuals with the diagnosis of Brachmann-de Lange syndrome (BDLS) to determine the natural course and cause of the disorder. The 64 individuals were ascertained through membership in a national organization, the Cornelia de Lange…

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…

Sleep disordered breathing in a cohort of children with achondroplasia: correlation between clinical and instrumental findings

BACKGROUND: The aim of the present study was to show the results of an overnight polysomnography in a cohort of 9 children (7 females and 2 males) with achondroplasia, aged between 1 and 12 years (5.56+/-4.7 years). All of the children carried the…

Sleep disturbance in Mowat-Wilson syndrome

Mowat-Wilson syndrome (MWS) is a multiple congenital anomaly syndrome caused by a heterozygous mutation or deletion of the ZEB2 gene. It is characterized by a distinctive facial appearance in association with intellectual disability (ID) and variable…

Tags: 0 (Homeodomain Proteins), 0 (Repressor Proteins), 0 (ZEB2 protein human), 2016, Adolescent, Adult, American Journal of Medical Genetics Part A, Australia, Behavior Rating Scale, characteristics, Child, Child Preschool, Einfeld S, Evans E, Facies, Female, Gene Expression, Hirschsprung Disease/co [Complications], Hirschsprung Disease/di [Diagnosis], Hirschsprung Disease/ge [Genetics], Hirschsprung Disease/pp [Physiopathology], Homeodomain Proteins/ge [Genetics], Humans, Intellectual Disability/co [Complications], Intellectual Disability/di [Diagnosis], Intellectual Disability/ge [Genetics], Intellectual Disability/pp [Physiopathology], Male, Microcephaly/co [Complications], Microcephaly/di [Diagnosis], Microcephaly/ge [Genetics], Microcephaly/pp [Physiopathology], Middle Aged, Mowat D, Mowat-Wilson syndrome, Mutation, Repressor Proteins/ge [Genetics], Severity Of Illness Index, sleep disturbance/disorders, Sleep Initiation and Maintenance Disorders/co [Complications], Sleep Initiation and Maintenance Disorders/di [Diagnosis], Sleep Initiation and Maintenance Disorders/ge [Genetics], Sleep Initiation and Maintenance Disorders/pp [Physiopathology], Surveys And Questionnaires, Trajectory, Wilson M

Sleep disturbances in aspartylglucosaminuria (AGU): a questionnaire study.

Sleep disturbances are common in many progressive metabolic encephalopathies. The possible presence of disturbed sleep-wake behaviour in the lysosomal storage disorder aspartylglucosaminuria, has not been previously studied, however. The sleep-wake…

Sleep in individuals with Cri du Chat syndrome: a comparative study

BACKGROUND: Sleep problems are common in individuals with intellectual disability. Little is known about sleep in children and adults with Cri du Chat syndrome (CDC).;METHOD: Sleep was investigated in 30 individuals with CDC using a sleep…

Social and behavioral problems of children with agenesis of the corpus callosum

Archival data from a survey of parent observations was used to determine the prevalence of social and behavioral problems in children with agenesis of the corpus callosum (ACC). Parent observations were surveyed using the Child Behavior Checklist…

Survey of paediatricians caring for children with life-limiting conditions found that they were involved in advance care planning

AIM: Advance care planning (ACP) is a strategy to align future care and treatment with preferences of patients and families. This study assesses the experiences of ACP among paediatricians caring for children with life-limiting conditions. METHODS:…

Tags: 2020, Acta Paediatrica, Advance Care Planning, Child, Communication, Fahner JC, July 2021 List, Kars MC, paediatric palliative care, Parents, Pediatricians, Rietjens JAC, Shared Decision-making, Surveys And Questionnaires, van Delden JJM, van der Heide A

The effect of multidrug-resistant organisms on social participation and quality of life in children with life-limiting conditions

BACKGROUND: Children with life-limiting conditions have a high risk of colonisation with a multidrug-resistant organism (MDRO). To avoid the spread of hospital-aquired infections to other patients, children with a MDRO are moved to an isolated room…

The needs of professionals in the palliative care of children and adolescents

UNLABELLED: The main objectives of this qualitative study were to describe the perceptions and needs of pediatric health care professionals (HCPs) taking care of children with palliative care needs and to develop a concept for the first Center of…

Tags: 2013, Abbruzzese R, Adolescent, Adolescent Health Services/ma [Manpower], Bergstraesser E, Child, Child Care/ma [Manpower], European Journal of Pediatrics, Health Services Needs And Demand, Hornung R, Hosek M, Humans, Inglin S, January 2018 List, Marfurt-Russenberger K, Palliative Care/ma [manpower], Qualitative Research, Surveys And Questionnaires, Switzerland

The prevalence and phenomenology of repetitive behavior in genetic syndromes

We investigated the prevalence and phenomenology of repetitive behavior in genetic syndromes to detail profiles of behavior. The Repetitive Behaviour Questionnaire (RBQ) provides fine-grained identification of repetitive behaviors. The RBQ was…

The reactions to research participation questionnaires for children and for parents (RRPQ-C and RRPQ-P)

Systematic assessment of the effect of clinical research studies on child and parent participants has been limited. Such assessment could provide an empirical basis for the ethical conduct of research, assisting investigators and institutional review…

The Relationship Between The Nursing Environment And Delivering Culturally Sensitive Perinatal Hospice Care

BACKGROUND: Wide variations exist among perinatal hospices, and barriers to perinatal palliative care exist at the healthcare level. Research in the area of culturally sensitive perinatal palliative care has been scarce, a gap which this study…

To explore the neonatal nurses' beliefs and attitudes towards caring for dying neonates in Taiwan

(1) To explore attitudes and beliefs of neonatal nurses toward nursing care for dying neonates; (2) to estimate the influence of neonatal nurses' personal and professional characteristics on their attitudes towards end-of life care for dying infants.…

When Parents Face The Death Of Their Child: A Nationwide Cross-sectional Survey Of Parental Perspectives On Their Child’s End- Of Life Care.

Background

Parents facing the death of their child have a strong need for compassionate professional support. Care services should be based on empirical evidence, be sensitive to the needs of the families concerned, take into account the…

Why is corneal donation so rare in children's hospices? A survey of multidisciplinary team members attitudes, knowledge, practice, and experience

BACKGROUND: Corneal donation is a rare event among pediatric patients dying in children's hospices in Italy. Previous research suggests that lack of knowledge and negative attitude of Health Care Professionals (HCPs) are the most relevant factors for…

Browse Items (74 total)