Neonatal intensive care unit nurses knowledge and attitude toward neonatal palliative care: review of the literature
attitude; Infant Newborn; Intensive Care Units; Intensive Care Units Neonatal; knowledge; Npc; nurse; Palliative Care
AIM: To review studies regarding neonatal nurses' knowledge and attitude toward neonatal palliative care (NPC). METHOD: The researchers searched internet sources such as Google Scholar for NPC, Nurses, Knowledge, Attitude, and Educational Intervention. RESULTS: Subheadings identified in the literature review were Nurses Knowledge toward NPC in NICU, Nurses Attitude toward NPC in NICU, correlation between Knowledge and Attitude toward NPC in NICU, The Effect of Educational program on Nurses Knowledge and Attitude toward NPC in NICU, and the Predictors of Knowledge and Attitude toward NPC among Nurses in NICU and Barriers to NPC provision and improvement. CONCLUSION: There are few studies from different nations regarding NPC found nurses have an inadequate knowledge of NPC, which also reflects their attitude. Objective: To review studies regarding neonatal nurses' information and behavior toward end-of-life care. Method: The researcher searched internet sources such as Google Scholar, PubMed, Medline, and ResearchGate for end of life, Nurses, Information, Behavior, And Educational Program. Results: Subheadings identified in the literature review were nurses information toward end of life care in neonate care unit, nurses attitude toward end of life in neonate care unit, correlation between knowledge and attitude toward end of life in neonate care unit, the effect of educational program on nurses information and behavior toward end of life in neonate care and the predictors of information and behavior toward end of life among nurses in neonate and barriers to end of life. Conclusion: There are few studies from different nations regarding end of life found nurses have an inadequate information of end of life, which also reflects their behaviors. eng
Abuhammad S; Elayyan M; Ababneh H
Future Science Open Access
2023
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/10.2144/fsoa-2022-0062" target="_blank" rel="noreferrer noopener">10.2144/fsoa-2022-0062</a>
Otorhinolaryngological, Audiovestibular and swallowing manifestations of patients with Niemann-Pick disease Type C
adolescent; hearing; dysphagia; priority journal; school child; preschool child; prospective study; human; article; child; female; male; controlled study; clinical article; auditory response; Balance disorders; body equilibrium; endoscopy; Flexible endoscopy; hearing impairment; Hearing problems; Niemann Pick disease/di [Diagnosis]; Niemann-Pick disease; otorhinolaryngology; pure tone audiometry; stabilography; swallowing; Swallowing disorders; vestibular system; feeding difficulties; tone and motor problems; NPC; penetration; aspiration scale; trajectory; characteristics; postural imbalance; dysphagia
Objectives: The aim of this study is to evaluate audiovestibular and swallowing impairment of patients with NPC. Methods: Audiovestibular and swallowing evaluation were performed on patients with Niemann-Pick disease type C (NPC) at Hacettepe University between 20013 and 2015 prospectively. Pure-tone audiometry (PTA), Auditory Brain stem response (ABR), Flexible endoscopic evaluation of swallowing (FEES) test and posturography were done. Hearing, swallowing and balance states were measured. Results: There were 16 patients (5 male and 11 female, with a median age of 6.5 years old). The most common ABR abnormalities observed were absent waves I and III (%70 absent I waves, %43.75 absent III waves). Twelve of sixteen patients (%75) had an ABR abnormality in at least one ear, of these, four patients had normal hearing and three of them had periferal hearing loss. 12 (75%) patients had complaint of postural imbalance. 11(69%) of patients had peripheral and one (6%) patient had central impairment. Nine of sixteen patients (56.25%) show some degree of dysphagia (either penetration or aspiration). Two patients (12.5%) showed aspiration both liquid and viscous nutrition. Three patients (18.75%) showed aspiration primarily in liquids and two of them had penetration with viscous nutrition. Three patients (18.75%) had penetration with no aspiration neither liquid nor viscous nutrition (PEN-ASP score was 3, 3, 5, respectively). Conclusion: There is no curative treatment for this devastating and fatal disorder and hearing impairment, balance and swallowing disorders can be seen especially late onset form of disease.
Senirli R T; Kuscu O; Akyol U; Topcu M; Yigit O; Aksoy S; Demir N
International Journal of Pediatric Otorhinolaryngology
2016
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/10.1016/j.ijporl.2015.11.009" target="_blank" rel="noreferrer noopener">10.1016/j.ijporl.2015.11.009</a>
Gait, Balance, and Coordination Impairments in Niemann Pick Disease, Type C1
adolescent; retrospective study; priority journal; scoring system; school child; outcome assessment; preschool child; intervention study; human; article; child; female; male; adult; clinical article; young adult; comparative study; body equilibrium; stabilography; performance; neurologic examination; balance impairment; coordination disorder; gait disorder; Niemann Pick disease; 2 hydroxypropyl beta cyclodextrin; 94035-02-6 (2 hydroxypropyl beta cyclodextrin); case control study; clinical evaluation; disease severity assessment; human development; molecular stability; motion analysis system; NIH NPC Neurologic Severity Scale; research; spatiotemporal analysis; upper limb; vts 270; tone and motor problems; NPC; trajectory; characteristics
This is the first study to objectively measure gait, balance, and upper limb coordination in a group of patients with NPC1 and compare the results to age and gender matched controls. This is also the first study to report effect sizes in these measures. Spatiotemporal gait analysis, static and dynamic posturography, and upper limb reaching motion analysis were performed. The findings showed that the NPC1 subjects had statistically significant deficits on 12 out of the 16 parameters investigated compared to controls, and large effect sizes for all but 1 parameter. When ranking the variables in terms of the effect sizes, the top 5 included at least 1 parameter from each of the 3 motor domains investigated. These results can provide insight to clinical researchers on the selection of outcome measures for longitudinal and interventional studies.
Sansare A; Zampieri C; Alter K; Stanley C; Farhat N; Keener L A; Porter F
Journal of Child Neurology
2018
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/10.1177/0883073817741054" target="_blank" rel="noreferrer noopener">10.1177/0883073817741054</a>
Niemann-Pick C disease in Spain: clinical spectrum and development of a disability scale
Adolescent; Adult; Age of Onset; Carrier Proteins/ge [Genetics]; Cerebellar Ataxia/di [Diagnosis]; Cerebellar Ataxia/ep [Epidemiology]; Cerebellar Diseases/di [Diagnosis]; Cerebellar Diseases/ep [Epidemiology]; Child; Preschool; Comorbidity; Disability; Evaluation; Dysarthria/di [Diagnosis]; Dysarthria/ep [Epidemiology]; Female; Genetic Predisposition to Disease/ge [Genetics]; Infant; feeding difficulties; sleep disturbance; tone and motor problems; NPC; tool development; scale development
OBJECTIVES: To describe the clinical evolution of Niemann-Pick C disease to identify possible factors involved in the diagnosis and severity of the disease. METHODS: A clinical database and a severity scale was created to evaluate 45 patients diagnosed with Niemann-Pick type C in the last 28 years in Spain. RESULTS: Complete clinical data were obtained from 30 patients, all were confirmed to have mutations in the NPC1 gene. Regarding clinical form, 3 were perinatal, 7 severe infantile, 6 late infantile, 11 juvenile and 3 adult. Biochemical phenotype was classic in 26. Splenomegaly was present in 28 patients (93%) with a wide range of age at detection. The first symptom of neurological disease was clumsiness, followed in 2-4 years by cerebellar signs. Ophthalmoplegia appeared 2-4 years later and became complete 1-2 years after onset. Dysarthria appeared by the time of complete ophthalmoplegia. Diagnosis was made before the onset of neurological signs in patients with the severe infantile form, at the time of onset of cerebellar signs in the late infantile form and complete ophthalmoplegia in late onset forms. CONCLUSIONS: In our series, splenomegaly is present in 96% of patients, even in late onset forms during the first years of life. Clumsiness in children with otherwise normal motor development precedes the onset of ataxia by 2-4 years in Niemann Pick type C. A disability scale could be useful for monitoring evolution, establishing possible phenotypic correlations and evaluating future therapies.
Iturriaga C; Pineda M; Fernandez-Valero E M; Vanier M T; Coll M J
Journal of the Neurological Sciences
2006
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/10.1016/j.jns.2006.05.054" target="_blank" rel="noreferrer noopener">10.1016/j.jns.2006.05.054</a>
Successful treatment of cataplexy in patients with early-infantile Niemann-Pick disease type C: use of tricyclic antidepressants
tone and motor problems; NPC; pharmacologic intervention; imipramine; cataplexy
Cataplexy is a brief episode of bilateral loss of muscle tone with intact consciousness, triggered by a variety of strong emotions such as anger, laugh, humor or surprise and it is considered to represent the physiologic atonia of rapid eye movement sleep. On the other hand, Niemann-Pick type C is a neurodegenerative lysosomal storage disease, characterized by the accumulation of cholesterol and glycosphingolipids. Cataplexy is a relatively specific and common neurologic sign seen in almost 50% of all patients with Niemann-Pick type C. The aim of this report is to demonstrate the successful treatment of cataplexy with the use of a tricyclic antidepressant imipiramine, in two patients between the ages 6-9, with mild to moderate mental retardation, molecularly diagnosed as Niemann-Pick type C 1 and currently under miglustat treatment and to discuss the possible mechanisms of drug action in the light of cataplexy and Niemann-Pick type C pathophysiology.Copyright © 2014 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.
Cak H T; Haliloglu G; Duzgun G; Yuce A; Topcu M
European Journal of Paediatric Neurology
2014
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/10.1016/j.ejpn.2014.07.009" target="_blank" rel="noreferrer noopener">10.1016/j.ejpn.2014.07.009</a>