Browse Items · Pediatric Palliative Care Library

Aberrant sleep patterns in children with the Rett syndrome

Previous studies on the characteristics of disturbed sleep/wake patterns in children with the Rett syndrome have yielded inconsistent findings. In the current study, momentary time sampling procedures were used to measure the sleep/wake patterns of…

Acute onset of X-linked adrenoleukodystrophy mimicking encephalitis

We report the case of a 6-year-old boy with X-linked adrenoleukodystrophy (ALD). In view of the acute onset of vomiting, fever, and coma, encephalitis was initially suspected. However, brain magnetic resonance imaging demonstrated a pattern of…

Behavioral Treatment of Sleep Dysfunction in Patients with the Rett Syndrome

Aberrant sleep patterns are commonly experienced by girls with the Rett syndrome. In this investigation, the problematic sleep of three girls with the Rett syndrome was regulated using a bedtime fading procedure with response cost. The treatment…

Tags: 1991, Backlog, Behavior Therapy, Brain & Development, Child, Female, Fisher W, Human, Journal Article, Moser H, Piazza CC, Preschool, Rett Syndrome/co [Complications], Sleep Disorders/et [Etiology], Sleep Disorders/th [Therapy]

Glutaric aciduria types I and II

Glutaric aciduria type I is an autosomal recessive disorder resulting from a deficiency of glutaryl-CoA dehydrogenase. This leads to an accumulation of glutaric and 3-hydroxyglutaric acids and secondary carnitine deficiency. The symptomatology is…

Tags: 2006, Backlog, Brain & Development, Carnitine/deficiency, Deficiency Diseases/diagnosis/genetics, Disease Specific, Genes, Glutarates/urine, Glutaryl-CoA Dehydrogenase/deficiency/genetics, Gordon N, Humans, Journal Article, Recessive

Improvement of hypertonus after treatment for sleep disturbances in three patients with severe brain damage

Treatment for sleep disturbance was given to three patients with severe brain damage (a 14-year-old boy, an 8-year-old girl and a 9-year-old boy), and changes in their muscle tone were estimated using F-wave analysis. In all patients, F-wave analysis…

Recurrent pain attacks in a 3-year-old patient with myoclonus epilepsy associated with ragged-red fibers (MERRF): a single-photon emission computed tomographic (SPECT) and electrophysiological study

We reported a 3-year-old girl with myoclonus epilepsy associated with ragged-red fibers (MERRF) who was afflicted with recurrent pain attacks and allodynia on the right side of the body. Although magnetic resonance imaging showed normal intensity in…

Respiratory function in handicapped children

The aim of this study was to evaluate respiratory function of severely handicapped children. Tidal volumes and respiratory rates were determined in a total of 130 children with different clinical motor abilities. Tidal volume of non-sitters (n = 39)…

The clinical course of childhood and adolescent adrenoleukodystrophy before and after Lorenzo's oil

The clinical course of Japanese patients with childhood and adolescent adrenoleukodystrophy (ALD) who were treated with Lorenzo's oil after the progression of early symptoms was investigated by Kaplan-Meier analyses. Many of the early symptoms,…

Tags: 2001, Adolescent, Adrenoleukodystrophy/drug therapy/pathology/physiopathology, Backlog, Brain & Development, Child, Disease Progression, Erucic Acids/therapeutic use, Humans, Imamura A, Japan, Journal Article, Kondo N, Male, Q3 Scoping Review Results, Shimozawa N, Suzuki Y, Treatment Outcome

Tracheo-arterial fistula in tracheostomy patients with Duchenne muscular dystrophy

A tracheo-arterial fistula is a serious and life threatening potential complication of a tracheostomy. Since 1984, we experienced nine fatal cases of tracheo-arterial fistula among 60 Duchenne muscular dystrophy (DMD) patients who underwent a…

Tags: 2006, Adolescent Adult Brachiocephalic Trunk/injuries/pathology/physiopathology Fatal Outcome Female Humans Incidence Male Muscular Dystrophy, Backlog, Brain & Development, Duchenne/complications Patient Selection Postoperative Complications/etiology/physiopathology/prevention & control Postoperative Hemorrhage/etiology Preoperative Care/standards Research Support, Fujimura H, Journal Article, Matsumura T, Non-U.S. Gov't Respiratory Insufficiency/etiology/surgery Respiratory Tract Fistula/etiology/pathology/physiopathology Tomography, Nozaki S, PedPal Lit, Saito T, Sawabata N, Shinno S, X-Ray Computed/standards Trachea/injuries/radiography/surgery Tracheostomy/adverse effects/mortality

Unilateral closed-lip schizencephaly and epilepsy: a comparison with cases of unilateral polymicrogyria

We compared the electroclinical features and evolution of patients with two different types of abnormal cortical organization: unilateral closed-lip schizencephaly (SCHZ) and unilateral polymicrogyria (PMG). Between February 1990 and June 2002, 51…

Browse Items (10 total)