scholarly journals Child and Family Centered Care for Pediatric Patients Diagnosed with Pulmonary Arterial Hypertension

2011 ◽  
Vol 10 (2) ◽  
pp. 94-96
Author(s):  
Aimee K. Doran
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Pediatric Patients ◽  
Family Centered Care ◽  
Centered Care ◽  
Pulmonary Arterial ◽  
Family Centered

Caring in Pediatric Emergency Nursing

2012 ◽  
Vol 26 (3) ◽  
pp. 216-232 ◽  
Author(s):  
Gordon Lee Gillespie ◽  
Melanie Hounchell ◽  
Jeanne Pettinichi ◽  
Jennifer Mattei ◽  
Lindsay Rose
Keyword(s):  
Pediatric Patients ◽  
Internal Consistency Reliability ◽  
Pediatric Emergency ◽  
Family Centered Care ◽  
Caring Behaviors ◽  
Human Needs ◽  
Nurse Caring ◽  
Centered Care ◽  
Carative Factors ◽  
Family Centered

An environment committed to providing family-centered care to children must be aware of the nurse caring behaviors important to parents of children. This descriptive study assessed the psychometrics of a revised version of the Caring Behaviors Assessment (CBA) and examined nurse caring behaviors identified as important to the parents of pediatric patients in a pediatric emergency department. Jean Watson’s theory of human caring provided the study’s theoretical underpinnings. The instrument psychometrics was determined through an index of content validity (CVI) and internal consistency reliability. The instrument was determined to be valid (CVI = 3.75) and reliable (Cronbach’s alpha = .971). The revised instrument was completed by a stratified, systematic random sample of 300 parents of pediatric emergency patients. Participants rated the importance of each item for making the child feel cared for by nurses. Individual survey item means were computed. Items with the highest means represented the most important nurse caring behaviors. Leading nurse caring behaviors centered on carative factors of “human needs assistance” and “sensitivity to self and others.” Nearly all nurse caring behaviors were important to the parents of pediatric patients, although some behaviors were not priority. It is important for nurses to provide family-centered care in a way that demonstrates nurse caring.

scholarly journals Oral treprostinil in transition or as add-on therapy in pediatric pulmonary arterial hypertension

2019 ◽  
Vol 9 (3) ◽  
pp. 204589401985647 ◽  
Author(s):  
D. Dunbar Ivy ◽  
Jeffrey A. Feinstein ◽  
Delphine Yung ◽  
Mary P. Mullen ◽  
Edward C. Kirkpatrick ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Pediatric Patients ◽  
Cardiopulmonary Exercise Testing ◽  
Functional Class ◽  
World Health ◽  
Walk Distance ◽  
Pulmonary Arterial ◽  
Health Organization

Treprostinil, a prostacyclin analogue, is approved for the treatment of pulmonary arterial hypertension (PAH) in adults. Transition from parenteral to oral treprostinil has been successfully accomplished in adults with PAH but not in children. In this multicenter study, pediatric patients treated with parenteral (Cohort 1) or inhaled (Cohort 2) treprostinil were transitioned to oral treprostinil. Prostacyclin-naïve individuals on background oral PAH therapy received oral treprostinil as add-on therapy (Cohort 3). Successful transition was oral treprostinil dose maintenance through week 24. Patients were monitored for adverse events (AEs), 6-min walk distance (6MWD), PAH symptoms, World Health Organization (WHO) Functional Class (FC), cardiac magnetic resonance imaging (cMRI), cardiopulmonary exercise testing (CPET), and quality of life through 24 weeks. A total of 32 patients were enrolled in the study; 23 (72%) were girls (mean age = 12.2 years). All patients were on background oral PAH therapy. Overall, patients (96.9%) maintained transition to oral treprostinil; one patient (Cohort 1) transitioned to oral treprostinil, then back to parenteral after experiencing syncope and WHO FC change from II to III. Cohorts 1, 2, and 3 received a final mean oral treprostinil dose of 5.6, 3.3, and 4.5 mg t.i.d., respectively. All cohorts had variable changes in 6MWD, cMRI, and CPET. Overall, 12 serious AEs were reported. All patients had drug-related AEs including headache (81%), diarrhea (69%), nausea (66%), vomiting (66%), and flushing (56%). Pediatric patients maintained transition to oral treprostinil with preservation of exercise capacity and WHO FC. Prostanoid-related AEs were most common and similar to those reported in adults.

scholarly journals Where the congenital heart disease meets the pulmonary arterial hypertension, FLNA matters: a case report and literature review

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Xiaoxian Deng ◽  
Shanshan Li ◽  
Qiu Qiu ◽  
Bowen Jin ◽  
Menghuan Yan ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Congenital Heart ◽  
Pediatric Patients ◽  
De Novo ◽  
Wide Spectrum ◽  
Heart Defects ◽  
Pulmonary Arterial

Abstract Background Pediatric patients with genetic disorders have a higher incidence of pulmonary arterial hypertension (PAH) regardless of their heart defects. Filamin A (FLNA) mutation is recently recognized to be associated with pediatric pulmonary disorders, however, the clinical courses of PAH related to the mutation were reported in limited cases. Here, we presented a case and pooled data for better understanding of the correlation between FLNA mutation and pediatric PAH. Case presentation The patient was a 8-month-old female with repeated episodes of pneumonia. Physical examination revealed cleft lip, cleft palate and developmental retardation. Imaging examination showed a small atrial septal defect (ASD), central pulmonary artery enlargement, left upper lobe of lung atelectasis, and pulmonary infiltration. Genetic test showed she carried a de novo pathogenic variant of FLNA gene (c.5417-1G > A, p.-). Oral medications didn’t slow the progression of PAH in the patient, and she died two years later. Conclusions FLNA mutation causes rare but progressive PAH in addition to a wide spectrum of congenital heart disease and other comorbidities in pediatric patients. We highly recommend genetic testing for pediatric patients when suspected with PAH. Given the high mortality in this group, lung transplantation may offer a better outcome.

scholarly journals Brain Natriuretic Peptide Levels in Managing Pediatric Patients With Pulmonary Arterial Hypertension

CHEST Journal ◽  
2009 ◽  
Vol 135 (3) ◽  
pp. 745-751 ◽  
Author(s):  
Anna Bernus ◽  
Brandie D. Wagner ◽  
Frank Accurso ◽  
Aimee Doran ◽  
Heidi Kaess ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Brain Natriuretic Peptide ◽  
Natriuretic Peptide ◽  
Pediatric Patients ◽  
Pulmonary Arterial

scholarly journals Association between MDR1 gene polymorphism and clinical course of pediatric pulmonary arterial hypertension

2018 ◽  
Vol 26 (3) ◽  
pp. 305-312
Author(s):  
Iolanda Muntean ◽  
Carmen Şuteu ◽  
Rodica Togănel ◽  
Claudia Bănescu
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Progressive Disease ◽  
Pediatric Patients ◽  
Clinical Course ◽  
Heart Defects ◽  
Mdr1 Gene ◽  
Pulmonary Arterial ◽  
Mdr1 Polymorphism ◽  
Clinical Worsening

Abstract Pulmonary arterial hypertension (PAH) is a progressive disease with a complex pathogenesis. The polymorphism of the gene of multidrug resistance-1 (MDR1) has been associated with many diseases including PAH. Objective. In this study we aimed to investigate the relevance of the MDR1 polymorphism to pediatric PAH clinical course. Methods. A total of 40 pediatric patients with PAH (secondary to congenital heart defects or idiopathic) and 40 control subjects were enrolled. Patients with PAH were divided into 2 groups, according to their evolution: 28 patients who remained clinically stable at 12-months (non-worsening group) and 12 patients who presented clinical worsening at 12-months (worsening group). Genomic DNA was genotyped for MDR1 gene polymorphisms as follows: C1236T, G2677T and C3435T. Results. There were no significant differences between PAH children groups (clinical worsening and non-worsening) nor between PAH children and controls in terms of frequency distribution of the three studied genotypes or alleles. Conclusions. The MDR1 polymorphism could not be correlated with the clinical evolution of pediatric PAH patients in our study.

scholarly journals Evaluation of pediatric patients with severe pulmonary arterial hypertension

2019 ◽  
Vol 24 (2) ◽  
pp. 146-154 ◽  
Author(s):  
Serdar Epçaçan ◽  
Mehmet Gökhan Ramoğlu ◽  
Emrah Şişli ◽  
Çayan Çakır ◽  
Zerrin Karakuş Epçaçan ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Pediatric Patients ◽  
Pulmonary Arterial ◽  
Severe Pulmonary Arterial Hypertension

scholarly journals Palliative balloon atrial septostomy in two pediatric patients with severe pulmonary arterial hypertension requiring extracorporeal membrane oxygenation support

2020 ◽  
Vol 10 (3) ◽  
pp. 004947552095973
Author(s):  
Samantha L. Brackett ◽  
Nina Deutsch ◽  
Chinwe Unegbu
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Extracorporeal Membrane Oxygenation ◽  
Arterial Hypertension ◽  
Pediatric Patients ◽  
Case Series ◽  
Balloon Atrial Septostomy ◽  
Membrane Oxygenation ◽  
Atrial Septostomy ◽  
Pulmonary Arterial ◽  
Severe Pulmonary Arterial Hypertension

Pulmonary arterial hypertension is a pernicious disease with a diverse etiology in the pediatric population. Despite the increased availability of drug therapies, pulmonary arterial hypertension continues to cause significant morbidity and mortality. In pediatric patients with severe pulmonary arterial hypertension who have failed medical therapy, a few studies have demonstrated the role of balloon atrial septostomy as a bridge to lung transplantation or a means of improving symptomatology. However, no data exists on the utilization of balloon atrial septostomy as a palliative intervention to wean from extracorporeal membrane oxygenation (ECMO) when all other therapies are exhausted. Here we describe a case series of two pediatric patients with severe pulmonary arterial hypertension, requiring ECMO support, who were successfully weaned from ECMO following balloon atrial septostomy.

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