Osteogenesis Imperfecta (OI) is characterized by bone fragility. At least seven discrete types have been described ranging from mild disease to a lethal form. In a large number of cases, mutations in one of the two genes encoding type I collagen have…
Informed consent constitutes one of the important considerations included in the myriad ethical dilemmas in the pediatric intensive care unit. Traditionally, the law has viewed children as incompetent to make medical decisions, and society has…
The purpose of this study was to investigate how adolescents and adults with cystic fibrosis (CF) view preventive counseling and their transition to adult-centered care within a children's hospital. Thirty-two patients >/=16 years old diagnosed with…
OBJECTIVES: To examine young people's and parents' accounts of communication about cancer in childhood. DESIGN: Semistructured interviews analysed using the constant comparative method. SETTING: Paediatric oncology unit. PARTICIPANTS: 13 families,…
Post-traumatic growth is an emerging area of research concerned with the positive psychological changes that can follow the experience of traumatic events. The aim of this study is to explore themes of post-traumatic growth within personal experience…
Despite the finding that each child and family experienced cancer in their own unique way, they all shared the need to maintain a sense of spirit. Just as the suffering united the families, so too did their sense of spirit. Talk of the spirit is not…
This manual begins with an introduction to the basic "how-to's" of chronic, nonmalignant pain treatment and some of the theories, as well as approaches to pain management with a focus on the cognitive model. The core of the book delineates the…
Siblings of children with chronic illness or disability have been reported to have a 1.6 to 2.0 risk for behavioral and mental health problems. Our objective was to examine the effects of an intervention for siblings (age 7-15 years) of children with…
eizures are often initially treated at home. The standard treatment is rectal diazepam. Sublingual (SL) lorazepam is easy to administer but has not been well evaluated for efficacy in epilepsy. It has been reported to be effective for serial seizures…
BACKGROUND: Respiratory depression is the opioid adverse effect feared most by physicians. This may hinder adequate dosing in cancer pain. The study was conducted to examine the respiratory function of patients with advanced cancer receiving…
OBJECTIVE: Infantile Pompe's disease is a lethal cardiac and muscular disorder. Current developments toward enzyme replacement therapy are promising. The aim of our study is to delineate the natural course of the disease to verify endpoints of…
PURPOSE: The purpose of this study was to examine changes in item-specific functional activities and caregiver support in children and youths with acquired brain injury (ABI) in an inpatient setting using the Pediatric Evaluation of Disability…
BACKGROUND: Sucrose is an effective analgesic for procedural pain in preterm infants. It has been hypothesized that its analgesic effects are mediated by the release of endogenous opioid neurotransmitters such as beta-endorphin. OBJECTIVE: To…
In this paper, we provide a brief update of diagnostic considerations and biochemical phenotype in L-amino acid decarboxylase deficiency. We review clinical features and outcome data in 11 affected patients, including 7 previously unreported cases.…
BACKGROUND: Wilson's disease associated with severe liver disease is effectively cured by orthotopic liver transplantation (OLT). However, there are also anecdotal reports of improved or resolved neurologic symptoms after OLT in patients with stable…
OBJECTIVE: To determine types, sources, and predictors of conflicts among patients with prolonged stay in the ICU. DESIGN AND SETTING: We prospectively identified conflicts by interviewing treating physicians and nurses at two stages during the…
OBJECTIVE: To determine the frequency, types, sources, and predictors of conflict surrounding the care of pediatric intensive care unit (PICU) patients with prolonged stay. SETTING: A tertiary care, university-affiliated PICU in Boston. PARTICIPANTS:…
Type I glutaric aciduria (GA1) results from mitochondrial matrix flavoprotein glutaryl-CoA dehydrogenase deficiency and is a cause of acute striatal necrosis in infancy. We present detailed clinical, neuroradiologic, molecular, biochemical, and…
Between October 2001 and May 2002 the Chairperson and Vice-Chairperson of each Multicentre Research Ethics Committee (MREC) in England, Wales and Scotland took part in a semi-structured interview to ascertain the attitudes of MRECs to palliative care…
The treatment of severe pain with opioids has thus far been limited by their unwanted central side effects. Recent research promises new approaches, including opioid analgesics acting outside the central nervous system, targeting of opioid…
The MTT assay was used to measure the effects of pamidronate, clodronate and mevastatin on the cell viability of Ewing's sarcoma cell lines 6647, CADO-ES-1, ES-2, ES-3, RD-ES, SK-ES-1, STA-ET-2.1 and VH-64. Treatment of these cells with pamidronate…
Children's Hospital Boston began a major pain assessment and management initiative 3 years ago: Pain assessment and management are considered one of the institution's primary standards of care. The initiative included State of the Science meetings…
Patients with cancer sometimes are admitted to the emergency room due to severe pain. Despite the fact that morphine's hydrophilicity can delay its peak effects after intravenous administration up to 30 minutes, it is still the most commonly used…
The clinical presentation of mitochondrial disorders in childhood is highly variable causing difficulties in diagnosis and management. We assessed records of 75 children (48 male, 27 female) with a biochemically and/or molecularly established…
BACKGROUND: Commentators have suggested that patients may understand quantitative information about treatment benefits better when they are presented as numbers needed to treat (NNT) rather than as absolute or relative risk reductions. OBJECTIVE: To…
Non-invasive ventilation (NIV) is increasingly being used in hospitals to treat respiratory failure. The use of NIV with palliative intent in a district general hospital is described and ten illustrative cases where NIV was used in an attempt to…
Children with cognitive and adaptive disabilities are at increased risk for developing feeding difficulties and secondary nutritional deficiencies. Problems such as poor oral-motor coordination, swallowing dysfunction, gastroesophageal reflux, and…
