Browse Items (55 total)

OBJECTIVE: To characterize patterns of care at the end of life for children and young adults with life-threatening complex chronic conditions (LT-CCCs) and to compare them by LT-CCC type. STUDY DESIGN: Cross-sectional survey of bereaved parents (n =…

OBJECTIVE: To compare the rates of shared decision making (SDM) reported by parents of children with medical complexity (CMC) with the rates of SDM reported by parents of noncomplex children with special health care needs (CSHCN). STUDY DESIGN: We…

OBJECTIVE: To characterize patterns of care at the end of life for children and young adults with life-threatening complex chronic conditions (LT-CCCs) and to compare them by LT-CCC type. STUDY DESIGN: Cross-sectional survey of bereaved parents…

OBJECTIVE: To determine efficacy, safety, and optimal dose of a laxative, polyethylene glycol (PEG) 3350, in children with chronic constipation. STUDY DESIGN: Children with chronic constipation (n = 24) were treated with PEG for 8 weeks at an initial…

OBJECTIVE: To examine whether typical pain behavior, as reported by caregivers, could be used prospectively to predict future pain behavior and to derive a subset of core items from the Non-Communicating Children's Pain Checklist. STUDY DESIGN:…

Long-chain 3-hydroxyacyl-coenzyme A (CoA) dehydrogenase is one of three enzyme activities of the mitochondrial trifunctional protein. We report the clinical findings of 13 patients with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. At…

OBJECTIVES: To document the clinical and neurodevelopmental profiles of a cohort of patients with neonatal-onset propionic acidemia and to determine the efficacy of current therapy with respect to outcome. METHOD: The clinical, neurologic, and…

Local anesthetics are extremely useful for providing anesthesia and analgesia for infants and children of all ages. Despite the toxicity issues raised here, the overall safety record of local anesthetic use in pediatrics has been very good, and local…

A 3-year-old boy had recurrent episodes of lethargy, encephalopathy, and hepatomegaly accompanied by hypoglycemia, elevated liver aminotransferase and creatine kinase values, and nonketotic dicarboxylic aciduria; the serum carnitine level was…

The records of 52 children with Niemann-Pick disease type C were reviewed to establish whether the disease process and outcome varied with the initial clinical pattern; 34 children (65%) had cholestatic liver disease and hepatosplenomegaly in…

The purpose of this study was to assess the use of analgesic agents for invasive medical procedures in pediatric and neonatal intensive care units. The directors of 38 pediatric units and 31 neonatal units reported that analgesics were infrequently…

Alpers disease consists of diffuse cerebral degeneration manifested as developmental delay, seizures, vomiting, and progressive neuromuscular deterioration, with liver disease and death. We report the clinical course of the liver disease, histologic…

We evaluated colon manometry as a means of differentiating causes of intractable constipation in children. We studied pressure changes in the transverse, descending, and rectosigmoid colons of 23 children with intractable constipation. All patients…

Because infants hospitalized with respiratory syncytial virus (RSV) infection frequently receive antibiotics, our study was undertaken to determine what the actual risk of secondary bacterial infections in patients with RSV infection is and what…

The parents of a child who dies feel the emotions of shock, mourning, and confusion as they slowly come to accept the finality of the child's death. In contemporary America they frequently feel isolated and abandoned. The individuals and institutions…

OBJECTIVE: To analyze prospectively the impact of age at diagnosis in childhood idiopathic thrombocytopenic purpura (ITP). STUDY DESIGN: International registry from June 1997 to May 2001, with analysis of data from baseline and 6-month-follow-up…

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…

OBJECTIVE: To determine the duration of the risk period with platelet counts 1 month and 25 had 30 events. Among 93 patients with chronic ITP, 73 were at risk >1 month and 44 had 111 events. Events occurred with an average frequency of 0.39 per month…

OBJECTIVE: To validate a sputum induction technique in cystic fibrosis (CF), we examined the relation between airway inflammation and pulmonary function in children with CF by correlating inflammatory indexes in induced sputum with FEV(1). STUDY…

Complex regional pain syndromes (CRPS; type 1, reflex sympathetic dystrophy, and type 2, causalgia) involve persistent pain, allodynia, and vasomotor signs. We conducted a prospective, randomized, single-blind trial of physical therapy (PT) and…

OBJECTIVE: To examine the economic and psychologic costs of care provided by maternal caregivers to children with gastrostomy tube (GT) feedings. STUDY DESIGN: We conducted a 3-site study of primary maternal caregivers of 101 chronically ill…

OBJECTIVES: To describe the clinical presentation and long-term follow-up of a large cohort of patients with medium-chain acyl-CoA dehydrogenase (MCAD) deficiency. STUDY DESIGN: A nationwide, retrospective analysis of clinical presentation and…

OBJECTIVE: To characterize the natural progression of infantile-onset Pompe disease. STUDY DESIGN: Retrospective chart reviews of 168 patients with documented acid alpha-glucosidase deficiency and symptom onset by 12 months of age; Kaplan-Meier…

Gastroesophageal reflux disease (GERD) presents in different ways in children, most commonly with vomiting, or with esophageal symptoms such as regurgitation, heartburn, or dysphagia. Extraesophageal symptoms and signs also frequently occur. Less…

OBJECTIVES: Validated, noninvasive studies were used to compare sensation and motor function of the upper gastrointestinal tract in adolescents with functional dyspepsia (FD) and in control subjects. STUDY DESIGN: Fifteen adolescents with FD and 15…

OBJECTIVE: To describe outcomes and causes of death in children on chronic positive-pressure ventilation via tracheostomy. STUDY DESIGN: We conducted a retrospective observational cohort analysis of 228 children enrolled in an university-affiliated…

OBJECTIVE: To clarify the process of end-of-life decision-making in culturally different neonatal intensive care units (NICUs). STUDY DESIGN: Review of medical files of newborns >22 weeks gestation who died in the delivery room (DR) or the NICU…

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