[Purpose] We compared problematic behaviors of children according to the severity of their mental retardation (MR) of intellect as categorized by the Ryouiku Techou in this study, to investigate the influence of MR of intellect on children's…
We assessed a microswitch-based program to improve self-determination to access to preferred stimuli and to foster locomotor behavior by two girls with Rett syndrome and multiple disabilities. To enhance the first behavior (access to preferred…
Ataxia-telangiectasia is known for cerebellar degeneration, but clinical descriptions of abnormal tone, posture, and movements suggest involvement of the network between cerebellum and basal ganglia. We quantitatively assessed the nature of…
The purpose of this study was to validate the Behavior Problem Inventory-01 (BPI-01; Rojahn J Autism Dev Disord 31:577-588, 2001) in a population of 179 individuals with Cornelia de Lange Syndrome (CdLS) and to extend the literature on the behavioral…
Fatigue and depression have been shown to be significant problems in children with multiple sclerosis. The rate at which these conditions occur in children with other acquired demyelinating syndromes is unknown. In this cross-sectional study, the…
Ataxia-telangiectasia is a cerebellar neurodegenerative disorder presenting with ataxia, chorea, myoclonus, and bradykinesia. Literature on treatment of movement disorders is scarce. We treated 17 children (aged 11.2 +/- 3.9 years) for 8 weeks with…
Purpose: This study examined the reliability and validity of the Test of Infant Motor Performance Screening Items (TIMPSI) in infants with type I spinal muscular atrophy (SMA). Methods: After training, 12 evaluators scored 4 videos of infants with…
Children with the 22q11.2 deletion syndrome (22q11DS) are at an increased risk of psychiatric problems from pre-adolescence; little is known, however, about behavioral problems at a preschool age and the relationship between speech and behavior in…
Patients with 22q11DS are at risk of behavioral problems and cognitive impairment. Recent studies suggest a possible intellectual decline in 22q11DS children. To date it is unknown if cognitive development is related to the behavioral problems in…
Costello syndrome is a rare genetic condition caused by heterozygous alterations in HRAS and characterized by multi-system abnormalities. Individuals with Costello syndrome usually present with severe feeding difficulties in infancy, short stature,…
Objective: To validate a useful version of the Motor Function Measure (MFM) in children with neuromuscular diseases aged .90), and discriminant validity was good. Conclusions: The MFM-20 can be used as an outcome measure for assessment of motor…
The mucopolysaccharide disorders (MPS) are a group of recessively inherited metabolic disorders resulting in progressive physical and cognitive decline. MEDLINE, PsycINFO and Embase databases were searched, alongside manual screening, to identify…
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:…
PURPOSE: Rett syndrome is a severe neurodevelopmental disorder mainly affecting females and usually linked to mutations in the methyl-CpG-binding protein 2 gene, with an estimated prevalence of 1 in 10,000 live female births. Clinical features which…
Research indicates that approximately 40% to 70% of children with disabilities are identified and treated for feeding difficulties such as reflux. The available literature on children with trisomy 18 does not describe feeding needs or treatment. The…
Objective: Sleep disturbance and daytime restlessness are present in 50% to 80% of children with severe psychomotor impairment due to neurologic or other complex diseases. Although these issues severely impair the quality of life of the children and…
OBJECTIVE: A growing multicultural society presents healthcare providers with a difficult task of providing appropriate care for individuals who have different life experiences, beliefs, value systems, religions, languages, and notions of healthcare.…
Managing end-of-life care (EoL) is always emotionally taxing. Honest discussions about prognosis and EoL decision-making are often delayed due to questions about the legal competence of adolescents and young adults (AYA) to make decisions for…
IMPORTANCE: While data exist regarding the frequency and timing of the do-not-resuscitate (DNR) order in children, little is known about clinician attitudes and behaviors regarding this order. OBJECTIVE: To identify clinician attitudes regarding the…
IMPORTANCE Advance care planning (ACP) prepares patients and their families for future health care decisions; however, the needs of adolescent oncology patients for participation in ACP have not been well studied. OBJECTIVE To examine the efficacy of…
Trisomy 13 and trisomy 18 (T13-18) are associated with high rates of perinatal death and with severe disability among survivors. Prenatal diagnosis (PND) may lead many women to terminate their pregnancy but some women choose to continue their…
The increasing longevity of patients with congenital and developmental disorders of the nervous system reflects the palliative and social success of pediatrics in the past 2 decades. This success has resulted in an increasing number of adult patients…
Percutaneous transluminal pulmonary valvuloplasty may be indicated in not only isolated pulmonary valve stenosis, but also complex congenital heart diseases. Because palliative surgery for increasing pulmonary blood flow entails a risk of scar…
BACKGROUND: Hospice is an important provider of end of life care. Adult minorities are less likely to enroll on hospice; little is known regarding the prevalence of pediatric hospice use or the characteristics of its users. Our primary objective was…
BACKGROUND: Although sleep and physical activity often are impaired among adult cancer patients, there is limited data among pediatric oncology populations. We conducted a prospective study to investigate the relationship between physical activity…
BACKGROUND: Parents' perceptions of the quality of information communicated by their child's oncologist about the child's cancer are not well understood. PROCEDURE: We conducted a cross-sectional survey of 194 parents of children with cancer…
BACKGROUND: Pediatric patients experience significant symptoms during cancer treatment. Symptom management is frequently inadequate. We studied perceptions of pediatric oncology care providers regarding early integration of palliative care (PC) for…
BACKGROUND: Cancer-related fatigue is one of the most pervasive and debilitating side-effects of cancer treatment and adolescents consistently rate cancer-related fatigue as one of the most distressing aspects of treatment. Because fatigue is also…
BACKGROUND: Current options for location of end-of-life (EOL) care for children with cancer include home, hospital, and freestanding pediatric hospice (FSPH). However, access to these options varies greatly depending on geographical location. We…
Pediatric patients between the ages of 12 months and 17 years with a confirmed malignancy who were scheduled to receive aprepitant as part of triple therapy antiemetic prophylaxis for a cycle of moderately- or highly emetogenic chemotherapy were…
Seizures are a frequent complication associated with several neurogenetic disorders. Antiepileptic medications remain the mainstay of treatment in these patients. We summarized the available data associated with various antiepileptic therapies used…
Pediatric pharmacotherapy is often challenging due to the paucity of available clinical data on the safety and efficacy of drugs that are commonly used in children. This quandary is even more prevalent in children with rare diseases. Although…
UNLABELLED: Pain in children with cognitive impairment and cerebral palsy is a particularly relevant issue due to its high prevalence and impact on quality of life. We review available evidence about prevalence of pain, causes and specific treatment,…
Continuous sedation until death (CSD), the act of reducing or removing the consciousness of an incurably ill patient until death, often provokes medical-ethical discussions in the opinion sections of medical and nursing journals. A content analysis…
Pain is a complex construct that contributes to profound physical and psychological dysfunction, particularly in individuals coping with chronic pain. The current paper builds upon previous research, describes a balanced conceptual model that…
Ethically charged situations are common in pediatric critical care. Most situations can be managed with minimal controversy within the medical team or between the team and patients/families. Familiarity with institutional resources, such as hospital…
PURPOSE: Suffering is a complex multifaceted phenomenon, which has received limited attention in relation to children with terminal illness. As part of a wider study we interviewed parents of children with terminal illness to elicit their…
In the GRADE approach, the strength of a recommendation reflects the extent to which we can be confident that the composite desirable effects of a management strategy outweigh the composite undesirable effects. This article addresses GRADE's approach…
