Browse Items (382 total)

Problem behaviors of individuals who had one of three chromosome deletion disorders (5p- cri-du-chat, 15q- Prader-Willi, or 17p- Smith-Magenis) were investigated. The Aberrant Behavior Checklist was used. Results were contrasted with those of two…

Sanfilippo syndrome is a rare degenerative disorder which has severe intellectual and behavioural sequelae, commonly including sleep problems. A parental questionnaire was used to gather information on the sleep patterns of 80 children with…

Melatonin was tested as a sleeping pill in five patients with neuronal ceroid lipofuscinoses. The single-blind, placebo-controlled study consisted of motor activity recordings, sleep logs, and administration of placebo or melatonin (2.5 or 5 mg).…

In epidermolysis bullosa (EB), chronic constipation, painful defecation, and fecal impaction frequently contribute to malnutrition and growth failure. Standard treatments for constipation, such as increased intake of conventional dietary fiber and…

Neuromuscular disorders are common causes of weakness and hypotonia in the infantile period and in childhood. Accurate diagnosis of specific neuromuscular disorders depends first on identification of which aspect of the peripheral neuromuscular…

Using data from Americans' Changing Lives: Wave 1, 1986, this study examined the long-term effects on the personal functioning of older women and men following the death of an adult child or a spouse. Guided by Weiss's (1993) theoretical framework,…

We describe a male patient with late onset globoid cell leukodystrophy (GLD) (Krabbe's disease) still alive at 24 years of age, with a well preserved intellectual and communicative capacity, in contrast to visual failure and severe central pyramidal…

Mucopolysaccharidosis type III (Sanfilippo syndrome) is an autosomal recessive disorder characterised by progressive nervous system involvement with mental retardation, behavioural problems and seizures. Three patients, of 20 months to 12 years of…

Cerebral palsy refers to a neurologic disorder of motor skills that is static in nature and is the result of injury to the brain before its development is complete. Many neurodegenerative or metabolic disorders have a slow rate of progression and can…

Part of the nurse manager's role is to understand the complexities of running a specialist unit and to be aware of the increased focus on quality assurance programmes. This article describes how one model can be applied to involve all members of a…

While our understanding of adolescent bereavement has greatly expanded in recent years, one area yet to be clarified is the relationship between grief following a significant loss and spirituality. This article strengthens our understanding of this…

Sedation with analgesia is frequently required to perform painful or invasive procedures in children. The best medication combination for pediatric sedation with analgesia is yet to be identified. Sixty-four of 243 total sedation with analgesia…

This paper reports a study of the nature and prevalence of behaviour problems in 258 children with mucopolysaccharide disorders. Questionnaire data obtained through the post was supplemented by home visits to 42 families in the sample and by regular…

A postal questionnaire was used to study 49 individuals with Cornelia de Lange syndrome (including both the classical and the mild forms) to ascertain behavioural phenotype. Ages ranged from early childhood to adulthood (mean age, 10.2 years; SD,…

Forty-seven mothers and 33 fathers, representing 48 families, participated in a propective longitudinal study of the effects on family members of a child's dying. The purpose of this article is to describe parents' health during the terminal illness…

This presentation will cover the history, recent developments in, and implications of the ability of both the immune and neuroendocrine systems to produce POMC. The discovery of POMC in immune cells was one of the events that heralded a molecular…

We examined the strategies that people with osteoarthritis (OA) use to cope with illness-related problems in four areas: household activities, leisure activities, pain management, and social relationships. We also examined the relationship between…

STUDY OBJECTIVES: No large study has addressed whether parents want to be present when invasive procedures are performed on their children in the emergency department. We conducted a survey to address this question. METHODS: The study used a…

For nearly a century, bereavement theorists have assumed that recovery from loss requires a period of grief work in which the ultimate goal is the severing of the attachment bond to the deceased. Reviews appearing in the 1980s noted a surprising…

The purpose of this quasi-experimental (pre and posttest) study was to test a model pain management program (PMP) to implement the American Pain Society (APS) quality assurance standards for the management of acute and chronic cancer pain using a…

Adolescent health problems are often undetected in physicians' offices. The Dartmouth Primary Care Cooperative Information Project has developed a validated and reliable approach to identify adolescent health problems and initiate education in a…

beta-Endorphin (beta-EP) and cholecystokinin 8 (CCK-8) concentrations in peripheral blood mononuclear cells were measured in 12 drug-free autistic (AU) children, in 10 drug-free children with pervasive developmental disorders (PDD) and in 11 healthy…

In this review a case is presented for the use of mathematical modelling in the study of pain. The philosophy of mathematical modelling is outlined and a recommendation is made for the use of modern nonlinear techniques and computational neuroscience…

Ketamine has been used parenterally for pain unresponsive to opioids, including neuropathic pain, and has also been used as an alternative analgesic agent after surgery. Although oral administration of ketamine has been used for some time as a single…

One hundred fifty-nine young adults in the 17-18 year old age group suffering from chronic disease or handicap participated in this study which examined their attitudes towards enforced treatment and euthanasia in terminal patients. The comparison…
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