Browse Items (45 total)

AIMS: In France, most of children die in the hospital. This national survey aimed to achieve better understanding of end-of life care in specialized medical pediatrics departments for children facing the end-of-life, identify the available resources,…

Background Psychological tests can be useful to record adaptive and maladaptive behaviours of children with intellectual disability. The objective of this study was to describe the adaptive and maladaptive behaviour of children and adolescents with…

To study the effect of dopaminergic drugs on the parkinsonism in juvenile neuronal ceroid lipofuscinosis, the authors conducted an open study of 21 patients. According to the motor Unified PD Rating Scale (UPDRS) score, treatment was initiated with…

Although medicolegal challenges to the use of neurologic criteria to declare death in the USA have been well-described, the management of court cases in the United Kingdom about objections to the use of neurologic criteria to declare death has not…

Pediatric palliative medicine/care (PPC) is an approach to care that focuses on improving the quality of life of children facing a life limiting condition (LLC). LLCs are classified by the ACT (Association for children with life-threatening or…

PURPOSE: As the morbidity and mortality associated with communicable diseases continue to decrease in the developing world, the medical burden of childhood cancer continues to expand. Although international aid and relief groups such as the World…

Background: Neonatal seizures are associated with death and neurological morbidity; however, little is known about how neonates with seizures die. Method(s): This was a prospective, observational cohort study of neonates with seizures treated at…

Fifty-two untreated patients with late-onset Pompe disease completed questionnaires about their clinical condition and level of handicap at baseline and at 1-year (n = 41) and 2-year follow-ups (n = 40). During this period, declines in functional…

Background: Communication around serious illness is a core competency for all residencies. One-fifth of neurology residencies have no curriculum. Published curricula use didactics or role-play to assess confidence performing this skill without…

Palliative care services are beneficial for pediatric neurology patients with chronic, life-limiting illnesses. However, timely referral to palliative care may be impeded due to an inability to identify appropriate patients. The aim of this pilot…

Objectives: To identify barriers to delivering patient and family centred end of life care to children with neurodegenerative disease, of which seizures and myoclonic epilepsy is a significant symptom. To highlight good practice in delivery of end of…

Objectives: We sought to document the prevalence and nature of sleep-disordered breathing (SDB) in individuals with Friedreich ataxia (FRDA) as well as establish the relationship, if any, between SDB and clinical parameters of FRDA. Methods:…

Measuring the severity of neurologic dysfunction in patients with inherited ataxias, including Friedreich ataxia (FA), is difficult because of the variable rate of progression, the variable age at onset and the variety of neural systems that may be…

OBJECTIVE: To describe the disease course and natural history of Type A Niemann-Pick disease (NPD). METHODS: Ten patients with NPD-A (six male, four female; age range at entry: 3 to 6 months) were serially evaluated including clinical neurologic,…

BACKGROUND: Despite advances in neuroimaging and molecular genetics of holoprosencephaly (HPE), the clinical spectrum of HPE has remained inadequately described. OBJECTIVE: To better characterize the clinical features of HPE and identify specific…

Background: The Centre for Pediatric Palliative Care at the Medical Center of the University of Munich is one of the largest in Germany. Care is provided yearly to 90-100 children with advanced lifelimiting diseases living at home (at a distance of…

In this review, we report the developments across pediatric subspecialties that have been published in the Italian Journal of Pediatrics in 2021. We highlight advances in allergy and immunology, critical care, endocrinology, gastroenterology,…

Objective: To describe the characteristics of children and adolescentes with chronic diseases of outpatient clinics at a tertiary university hospital. Methods: A cross-sectional study was performed with 16,237 patients with chronic diseases…

OBJECTIVE: The Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society develop practice parameters as strategies for patient management based on analysis of evidence. For this…

Objectives: Dystonia and seizures can be common, unpredictable and distressing neurological symptoms in paediatric palliative care. Emergency seizure management is increasingly under joint neurology and palliative care, often incorporated into…

Fetal neurology is a rapidly evolving and expanding field. Discussions about diagnosis, prognosis, treatment options, and goals of care often begin in the antenatal period. However, there are inherent challenges to fetal counseling of neurological…

We documented seizures in 33 of 68 (48.5%) children with congenital hydrocephalus not associated with myelomeningocele. Mental retardation (MR) and CNS malformations correlated with seizure occurrence; age at shunt insertion and number of shunt…

BACKGROUND: Sleep-disordered breathing (SDB) and respiratory failure (RF) are complications of acid maltase deficiency (AMD), a rare hereditary myopathy. OBJECTIVE: To define the relationship between lung and respiratory muscle function, to establish…

Background: Hand stereotypies are considered a hallmark of Rett syndrome (RTT) and are usually described as symmetric movements at the midline. However, related pathologies may show the same type of involuntary movement. Furthermore, patients with…

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