Respiratory complications of mucopolysaccharide storage disorders
Male; Child; Humans; Adult; Adolescent; Female; Child Preschool; Infant; Retrospective Studies; Sleep Apnea Syndromes/et [Etiology]; Mucopolysaccharidoses/co [Complications]; Respiratory Tract Diseases/et [Etiology]; Airway Obstruction/et [Etiology]; Anesthesia General/ae [Adverse Effects]; Cardiovascular Diseases/et [Etiology]; Intubation Intratracheal/ae [Adverse Effects]; Lung Diseases/et [Etiology]; Lung Diseases/pp [Physiopathology]; Mucopolysaccharidoses/mo [Mortality]; Mucopolysaccharidoses/pp [Physiopathology]; Respiratory Tract Diseases/pp [Physiopathology]; Respiratory Tract Diseases/ra [Radiography]; Spinal Diseases/et [Etiology]; Spinal Diseases/ra [Radiography]; breathing difficulties; MLII; MLIII; MPSI; MPSII; MPSVI; surgical interventions; pharmacologic interventions; tonsillectomy; adenoidectomy; tracheostomy; diuretics; theophylline; digoxin; beta adrenergic blockers; calcium antagonists; respiratory problems
Twenty-one patients with the diagnosis of mucopolysaccharidosis or mucolipidosis and a history of respiratory complaints or thorough respiratory evaluation were studied retrospectively. Anatomic factors affecting respiratory status included: (i) upper airway narrowing by hypertrophied tongue, tonsils, adenoids, and mucous membranes; (ii) lower airway narrowing by glycosaminoglycan deposition within the tracheobronchial mucosa; (iii) decreased thoracic dimensions due to scoliosis and thoracic hyperkyphosis; and (iv) decreased abdominal dimensions due to lumbar hyperlordosis, gibbus formation and hepatosplenomegaly. Cardiac and neurologic involvement, while present, did not play primary roles in the development of respiratory disease. The functional consequences of these findings included increased risk of developing: (i) respiratory tract infections; (ii) airway compromise during or after anesthesia or sedation; (iii) dyspnea on exertion; (iv) obstructive lung disease; (v) obstructive sleep apnea; and (vi) cor pulmonale. A management approach is presented which can reduce the morbidity and mortality experienced by these patients.
Semenza G L; Pyeritz R E
Medicine
1988
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1097/00005792-198807000-00002" target="_blank" rel="noreferrer noopener">10.1097/00005792-198807000-00002</a>
Effect of hand splints on stereotypic hand behavior of three girls with Rett syndrome
Child; Humans; Adolescent; Female; Syndrome; Splints; Stereotyped Behavior; Autistic Disorder; Hand; Intellectual Disability; Neuromuscular Diseases/rehabilitation; tone and motor problems; Rett syndrome; physical intervention; Thumb abduction splints; stereotypic hand behavior; finger-feeding skills; hand splints
The purpose of this multiple baseline study was to examine the effect of bilateral hand splints on the persistent stereotypic hand movements of three adolescent girls with Rett syndrome. Among the most characteristic features of Rett syndrome are stereotypic hand-writing and hand-biting behavior and loss of previously acquired functional hand skills. The hand splints used in this study consisted of cuffs encircling the palm that positioned the subjects' thumbs in abduction. Duration percentages of subjects' stereotypic hand behavior and functional hand use were calculated from five-minute videotaped segments recorded during a finger-feeding condition and a free-time condition. All three subjects demonstrated a decrease in the amount of time spent in stereotypic hand behavior after application of hand splints, and one subject showed an increase in finger-feeding skills while wearing hand splints. Limitations of the study are discussed, and suggestions for clinical application and future research are offered.
Naganuma G M; Billingsley F F
Physical Therapy
1988
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<a href="http://doi.org/10.1093/ptj/68.5.664" target="_blank" rel="noreferrer noopener">10.1093/ptj/68.5.664</a>
Use of patient-controlled analgesia for management of acute pain
Humans; Acute Disease; Pain/drug therapy; Analgesics/administration & dosage/adverse effects; Infusion Pumps; Infusions; Injections; Intravenous; Self Administration/adverse effects/instrumentation
Patient-controlled analgesia (PCA) provides improved titration of analgesic drugs, thereby minimizing individual pharmacokinetic and pharmacodynamic differences. Patient-controlled analgesia decreases patient anxiety resulting from delays in receiving pain-relieving medication and from the slow onset of analgesic action when these drugs are administered either intramuscularly or in the extradural space. With PCA therapy, patients are reportedly able to maintain a near optimal state of analgesia with minimal sedation and few side effects. The potential for overdose can be minimized if small bolus doses are used with a mandatory lockout interval between successive doses. Finally, studies of the cost-effectiveness of PCA therapy are important if this therapeutic approach is to achieve more widespread acceptance.
1988
White PF
Jama
1988
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1001/jama.259.2.243" target="_blank" rel="noreferrer">10.1001/jama.259.2.243</a>
Therapeutic modification of membrane lipid abnormalities in juvenile neuronal ceroid-lipofuscinosis (Batten disease).
Humans; Follow-Up Studies; Longitudinal Studies; adolescent; Corn Oil/therapeutic use; Dietary Fats; Fish Oils/therapeutic use; Lipid Metabolism; Lipoproteins/blood; Neuronal Ceroid-Lipofuscinoses/diet therapy/drug therapy/therapy
Five patients with juvenile neuronal ceroid-lipofuscinosis, who were shown to have absent or reduced serum prebetalipoprotein and a deficiency of polyunsaturated fatty acids in erythrocyte membrane lipids, were treated for a period of one year with supplements of fish oil extract (rich in omega-3 fatty acids) and encouraged to increase dietary intake of corn oil lipid (rich in omega-6 fatty acids) in an attempt to promote incorporation of these fatty acids into membranes. After one year there was a significant increase in omega-3 fatty acids (P less than 0.05) and in the total polyunsaturated fatty acids (P less than 0.02) in erythrocyte membranes but no change in the incorporation of omega-6 fatty acids. The patients were assessed clinically, psychometrically and neurophysiologically before and after the supplementation. No significant changes were noted after one year of treatment.
1988
Bennett MJ; Hosking GP; Gayton R; Thompson G; Galloway JH; Cartwright IJ
American Journal Of Medical Genetics
1988
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Journal Article
<a href="http://doi.org/10.1002/ajmg.1320310630" target="_blank" rel="noreferrer">10.1002/ajmg.1320310630</a>
Beta-endorphin disregulation in autistic and self-injurious behavior: a neurodevelopmental hypothesis
Humans; Male; Adult; Non-U.S. Gov't; Research Support; Comparative Study; Clinical Trials; Autistic Disorder/drug therapy/physiopathology; beta-Endorphin/blood/physiology; Developmental Disabilities/drug therapy/physiopathology; Homeostasis; Naloxone/therapeutic use; Naltrexone/diagnostic use
Peptides derived from pro-opiomelanocortin (POMC) influence neurodevelopmental processes. Earlier studies indicated that MSH/ACTH compounds improved behavioral efficiency in retarded individuals. Recent studies have shown that opiate blockers reduce treatment-resistant self-injurious behavior (SIB), an autistic-like, developmental disorder. Although the exact mechanisms are unknown, prenatal POMC disregulation, addiction to endogenous opiates and elevated pain threshold have been proposed to account for this behavior. In study one, four SIB patients were given 0, 25, 50 or 100 mg of naltrexone on separate weeks in a double blind, Latin square design. A specific dose dependent reduction in SIB was observed in three patients. In study two, plasma b-endorphin was measured in 40 patients with SIB, a related behavior, stereotypy (ST) or controls. SIB and ST patients had higher levels of endorphin than controls. These data added new support for the role of b-endorphin in a treatment-resistant patient group.
1988
Sandman CA
Synapse (new York, N.Y.)
1988
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Journal Article
<a href="http://doi.org/10.1002/syn.890020304" target="_blank" rel="noreferrer">10.1002/syn.890020304</a>
Withholding nutrition from seriously ill newborn infants: a parent's perspective
Humans; infant; Male; Adult; Parents; Withholding Treatment; Euthanasia; Age Factors; Patient Selection; Morals; Enteral Nutrition; Death and Euthanasia; Newborn; Euthanasia; Brain Diseases; Diseases; Jurisprudence; Passive
1988
Miraie ED
The Journal Of Pediatrics
1988
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Journal Article
<a href="http://doi.org/10.1016/s0022-3476(88)80262-2" target="_blank" rel="noreferrer">10.1016/s0022-3476(88)80262-2</a>
Risk of secondary bacterial infection in infants hospitalized with respiratory syncytial viral infection
Child; Humans; Prospective Studies; Risk Factors; Preschool; infant; Anti-Bacterial Agents/therapeutic use; Bacterial Infections/etiology; Pneumonia/etiology; Respiratory Syncytial Viruses; Respiratory Tract Infections/complications/drug therapy; Respirovirus Infections/complications/drug therapy
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 effect antibiotic treatment might have on the course of illness. In a 9-year prospective study of 1706 children hospitalized with acute respiratory illnesses, 565 children had documented RSV infections. A subsequent bacterial infection rarely developed in those with RSV lower respiratory tract disease. The rate of subsequent bacterial infection was 1.2% in the total group of children infected with RSV, and 0.6% in the 352 children who received no antibiotics. A significantly greater proportion (4.5%) of subsequent bacterial infections occurred in infants who received parenteral antibiotics (p = 0.01), and especially in a subgroup who received parenteral antibiotics for 5 or more days (11%, p less than 0.001). We conclude that the risk of secondary bacterial infection appears to be low for most infants with RSV infection. In a few infants given parenteral broad-spectrum antibiotics the risk may be greater, but whether this is related to the antibiotic therapy or to other risk factors is not clear.
1988
Hall CB; Powell KR; Schnabel KC; Gala CL; Pincus PH
The Journal Of Pediatrics
1988
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Journal Article
<a href="http://doi.org/10.1016/s0022-3476(88)80263-4" target="_blank" rel="noreferrer">10.1016/s0022-3476(88)80263-4</a>
Structural equation modelling in practice: a review and recommended two-step approach.
1988
Anderson J; Gerbing D
Psychological Bulletin
1988
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Journal Article
<a href="http://doi.org/10.1037//0033-2909.103.3.411" target="_blank" rel="noreferrer">10.1037//0033-2909.103.3.411</a>
Sublingual absorption of selected opioid analgesics
Humans; Adult; Analgesics; Time Factors; Analysis of Variance; Non-U.S. Gov't; P.H.S.; Research Support; U.S. Gov't; Comparative Study; Administration; Biological Availability; Buprenorphine/pharmacokinetics; Fentanyl/pharmacokinetics; Heroin/pharmacokinetics; Hydromorphone/pharmacokinetics; Levorphanol/pharmacokinetics; Methadone/pharmacokinetics; Morphine/blood/pharmacokinetics; Mouth/metabolism; Naloxone/pharmacokinetics; Opioid/administration & dosage/pharmacokinetics; Oxycodone/pharmacokinetics; Sublingual
Ongoing interest in the improvement of pain management with opioid analgesics had led to the investigation of sublingual opioid absorption. The present report determined the percent absorption of selected opioid analgesics from the oral cavity of normal subjects under conditions of controlled pH and swallowing when a 1.0 ml aliquot of the test drug was placed under the tongue for a 10-minute period. Compared with morphine sulfate at pH 6.5 (18% absorption), buprenorphine (55%), fentanyl (51%), and methadone (34%) were absorbed to a significantly greater extent (p less than 0.05), whereas levorphanol, hydromorphone, oxycodone, heroin, and the opioid antagonist naloxone were not. Overall, lipophilic drugs were better absorbed than were hydrophilic drugs. Plasma morphine concentration-time profiles indicate that the apparent sublingual bioavailability of morphine is only 9.0% +/- 11.9% (SD) of that after intramuscular administration. In the same subjects the estimated sublingual absorption was 22.4% +/- 9.2% (SD), indicating that the sublingual absorption method may overestimate apparent bioavailability. When the oral cavity was buffered to pH 8.5, methadone absorption was increased to 75%. Thus, an alkaline pH microenvironment that favors the unionized fraction of opioids increased sublingual drug absorption. Although absorption was found to be independent of drug concentration, it was contact time dependent for methadone and fentanyl but not for buprenorphine. These results indicate that although the sublingual absorption and apparent sublingual bioavailability of morphine are poor, the sublingual absorption of methadone, fentanyl, and buprenorphine under controlled conditions is relatively high.
1988
Weinberg DS; Inturrisi CE; Reidenberg B; Moulin DE; Nip TJ; Wallenstein S; Houde RW; Foley KM
Clinical Pharmacology And Therapeutics
1988
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Journal Article
<a href="http://doi.org/10.1038/clpt.1988.159" target="_blank" rel="noreferrer">10.1038/clpt.1988.159</a>
Elderly psychiatric patient status and caregiver perceptions as predictors of caregiver burden
Humans; Family; Parent-Child Relations; Interpersonal Relations; Aged; Activities of Daily Living; Stress; Non-U.S. Gov't; Research Support; Psychological Tests; Psychological/psychology; Home Nursing/psychology; Mental Disorders/psychology; Mental Status Schedule
1988
Pearson J; Verma S; Nellett C
The Gerontologist
1988
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Journal Article
<a href="http://doi.org/10.1093/geront/28.1.79" target="_blank" rel="noreferrer">10.1093/geront/28.1.79</a>
Children's perceptions of physicians and medical care: two measures
Child; Humans; Questionnaires; Physicians; Clinical Competence; Child Health Services; Consumer Satisfaction; adolescent; Social Perception
1988
Rifkin L; Wolf MH; Lewis CC; Pantell RH
Journal of Pediatric Psychology
1988
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Journal Article
<a href="http://doi.org/10.1093/jpepsy/13.2.247" target="_blank" rel="noreferrer">10.1093/jpepsy/13.2.247</a>
Development of a brief coping checklist for use with pediatric populations.
Child; Humans; Longitudinal Studies; Psychometrics; Reference Values; adolescent; Adaptation; Psychological; Chronic Disease/psychology
1988
Spirito A; Stark LJ; Williams C
Journal of Pediatric Psychology
1988
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Journal Article
<a href="http://doi.org/10.1093/jpepsy/13.4.555" target="_blank" rel="noreferrer">10.1093/jpepsy/13.4.555</a>
Pediatric risk of mortality (PRISM) score
Child; Humans; infant; Intensive Care Units; mortality; Severity of Illness Index; Risk Factors; Pediatric; Preschool; Infant Mortality; Multicenter Studies as Topic; ROC Curve
The Pediatric Risk of Mortality (PRISM) score was developed from the Physiologic Stability Index (PSI) to reduce the number of physiologic variables required for pediatric ICU (PICU) mortality risk assessment and to obtain an objective weighting of the remaining variables. Univariate and multivariate statistical techniques were applied to admission day PSI data (1,415 patients, 116 deaths) from four PICUs. The resulting PRISM score consists of 14 routinely measured, physiologic variables, and 23 variable ranges. The performance of a logistic function estimating PICU mortality risk from the PRISM score, age, and operative status was tested in a different sample from six PICUs (1,227 patients, 105 deaths), each PICU separately, and in diagnostic groups using chi-square goodness-of-fit tests and receiver operating characteristic (ROC) analysis. In all groups, the number and distribution of survivors and nonsurvivors in adjacent mortality risk intervals were accurately predicted: total validation group (chi 2(5) = 0.80; p greater than .95), each PICU separately (chi 2(5) range 0.83 to 7.38; all p greater than .10), operative patients (chi 2(5) = 2.03; p greater than .75), nonoperative patients (chi 2(5) = 2.80, p greater than .50), cardiovascular disease patients (chi 2(5) = 4.72; p greater than .25), respiratory disease patients (chi 2(5) = 5.82; p greater than .25), and neurologic disease patients (chi 2(5) = 7.15; p greater than .10). ROC analysis also demonstrated excellent predictor performance (area index = 0.92 +/- 0.02).
1988-11
Pollack MM; Ruttimann UE; Getson PR
Critical Care Medicine
1988
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Journal Article
<a href="http://doi.org/10.1097/00003246-198811000-00006" target="_blank" rel="noreferrer">10.1097/00003246-198811000-00006</a>
Uncertainty in illness
Humans; Sick Role; Cognition; Professional-Patient Relations; Adaptation; Psychological; Models; social support; Educational Status; Disease/psychology; Mental Processes
1988
Mishel MH
Image--the Journal Of Nursing Scholarship
1988
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Journal Article
<a href="http://doi.org/10.1111/j.1547-5069.1988.tb00082.x" target="_blank" rel="noreferrer">10.1111/j.1547-5069.1988.tb00082.x</a>
Juvenile neuronal ceroid lipofuscinosis (JNCL): quantitative description of its clinical variability
Child; Female; Humans; Male; Adult; Follow-Up Studies; Longitudinal Studies; adolescent; retrospective studies; Neuronal Ceroid-Lipofuscinoses/diagnosis
The clinical courses of 17 JNCL patients were analyzed retrospectively with the use of a simple, disease-specific scoring system. The mean observation period was 14 years (range 8-18 years). Scores of 0 (maximal dysfunction) to 3 (normal function) were assigned to each patient's vision, intellect, language, motor function, and epilepsy for each year of observation. The lapse of medians and ranges of all patients' scores were established from age 3 to 20 years. This scoring system allowed quantitative description of an individual course in context of the wide natural variability of the disease. Patients with seizures starting before the age of 10 years tended to have intractable epilepsy, to receive multiple antiepileptic drug therapies, and to have poor courses including problems not related to epilepsy. One patient had a course clearly outside the usual variability of JNCL and is thought to represent a genetic variant.
1988
Kohlschutter A; Laabs R; Albani M
Acta Paediatrica
1988
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Journal Article
<a href="http://doi.org/10.1111/j.1651-2227.1988.tb10770.x" target="_blank" rel="noreferrer">10.1111/j.1651-2227.1988.tb10770.x</a>
Dad's double binds. Rethinking fathers' bereavement from a men's studies perspective
Fathers; bereavement
1988
Cook JA
Journal Of Contemporary Ethnography
1988
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Journal Article
<a href="http://doi.org/10.1177/089124188017003003" target="_blank" rel="noreferrer">10.1177/089124188017003003</a>
Hospice volunteer turnover: a measure of quality assurance in the utilization of volunteers
1988
Brichacek GB
American Journal Of Hospice Care
1988
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Journal Article
<a href="http://doi.org/10.1177/104990918800500603" target="_blank" rel="noreferrer">10.1177/104990918800500603</a>
The family environment in bereaved families and its relationship to surviving sibling behavior
Family
1988
Davies B
Child Health Care
1988
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Journal Article
<a href="http://doi.org/10.1207/s15326888chc1701_4" target="_blank" rel="noreferrer">10.1207/s15326888chc1701_4</a>
"Safe passage": social work roles and functions in hospice care
Female; Humans; Male; Terminal Care; Aged; Professional-Family Relations; Interprofessional Relations; Hospices; Professional-Patient Relations; Social Work
What are social work's unique roles and functions in behalf of patients and their families in hospice care? The question is answered in the first phase of a Joint Research Project of social work faculty, hospice social workers and graduate social work students. The special roles, illustrative functions and potential outcomes of social work in hospice are identified. Case examples are utilized to illustrate social work's response to patients, families, staff and the service network. The authors hypothesize that social workers in hospice and other health care settings are instrumental in charting and facilitating within a nurturing environment a "safe passage" for the person-in-transition.
1988
Rusnack B; Schaefer SM; Moxley D
Social Work In Health Care
1988
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Journal Article
<a href="http://doi.org/10.1300/j010v13n03_02" target="_blank" rel="noreferrer">10.1300/j010v13n03_02</a>
TOWARD A MODEL OF PARENTAL GRIEF
Bereavement Leave Policy Paper
1988
Klass D; Marwit SJ
Omega
1988
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Journal Article
<a href="http://doi.org/10.2190/bvur-67kr-f52f-vw35" target="_blank" rel="noreferrer">10.2190/bvur-67kr-f52f-vw35</a>
Family Therapy in Cases of Interminable Grief for the Loss of a Child.
SSHRC CURA
1988
Rosen E
Omega
1988
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Journal Article
<a href="http://doi.org/10.2190/l4fu-m3f3-n8yw-5m9j" target="_blank" rel="noreferrer">10.2190/l4fu-m3f3-n8yw-5m9j</a>
Shared life space and sibling bereavement responses.
bereavement; IM; sibling bereavement; N
1988-12
Davies B
Cancer Nursing
1988
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Journal Article
The effects of time on the adolescent sibling bereavement process.
sibling bereavement; N
1988-08
Hogan NS
Paediatric Nursing
1988
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Journal Article
Unending work and care: managing chronic illness at home
Family Relations; Psychology; Chronic disease; home care services
Corbin JM; Strauss A
1988
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Book/Book Section