PedsQL™ Multidimensional Fatigue Scale in sickle cell disease: feasibility, reliability, and validity.

BACKGROUND: Sickle cell disease (SCD) is an inherited blood disorder characterized by a chronic hemolytic anemia that can contribute to fatigue and global cognitive impairment in patients. The study objective was to report on the feasibility, reliability, and validity of the PedsQL™ Multidimensional Fatigue Scale in SCD for pediatric patient self-report ages 5-18 years and parent proxy-report for ages 2-18 years. PROCEDURE: This was a cross-sectional multi-site study whereby 240 pediatric patients with SCD and 303 parents completed the 18-item PedsQL™ Multidimensional Fatigue Scale. Participants also completed the PedsQL™ 4.0 Generic Core Scales. RESULTS: The PedsQL™ Multidimensional Fatigue Scale evidenced excellent feasibility, excellent reliability for the Total Scale Scores (patient self-report α = 0.90; parent proxy-report α = 0.95), and acceptable reliability for the three individual scales (patient self-report α = 0.77-0.84; parent proxy-report α = 0.90-0.97). Intercorrelations of the PedsQL™ Multidimensional Fatigue Scale with the PedsQL™ Generic Core Scales were predominantly in the large (≥0.50) range, supporting construct validity. PedsQL™ Multidimensional Fatigue Scale Scores were significantly worse with large effects sizes (≥0.80) for patients with SCD than for a comparison sample of healthy children, supporting known-groups discriminant validity. Confirmatory factor analysis demonstrated an acceptable to excellent model fit in SCD. CONCLUSIONS: The PedsQL™ Multidimensional Fatigue Scale demonstrated acceptable to excellent measurement properties in SCD. The results demonstrate the relative severity of fatigue symptoms in pediatric patients with SCD, indicating the potential clinical utility of multidimensional assessment of fatigue in patients with SCD in clinical research and practice.

Diagnostic challenges between takotsubo cardiomyopathy and acute myocardial infarction-where is the emergency?: a literature review.

BACKGROUND: Takotsubo cardiomyopathy (TC) is an emergency cardiovascular disease, with clinical and paraclinical manifestations similar to acute myocardial infarction (AMI), but it is characterized by reversible systolic dysfunction of the left ventricle (LV) in the absence (most of the time) of obstructive coronary artery disease (CAD). MANAGEMENT OF PATIENTS WITH TC: TC seems to be more frequent in post-menopausal women and it is triggered by emotional or physical stress. The diagnosis of TC is based on the Mayo Clinic criteria. Initially, patients with TC should be treated as those with AMI and carefully monitored in intensive care unit. Urgent clinical and paraclinical distinction between TC and AMI is mandatory in all patients, because of the different therapeutical management between the two diseases. Chest pain and dyspnea are the most common symptoms in TC. Paraclinical diagnosis is based on cardiac biomarkers, electrocardiogram (ST-segment elevation/T wave inversion in precordial leads without reciprocal ST-segment depression in inferior leads and absence of Q waves), echocardiography (LV systolic dysfunction, regional wall motion abnormalities extended in more than one coronary territory), cardiac magnetic resonance and in most of the cases the positive diagnosis is established by performing CA to exclude obstructive CAD. The prognosis of patients with TC is considered benign in most cases, with a complete LV function recovery, but severe complications may occur, such as cardiogenic shock, LV free wall rupture, life-threatening arrhythmia, and cardiac arrest. Postoperative TC may develop after any type of surgical intervention due to acute stress and it should be differentiated from postoperative AMI. The management of patients with TC is medical and it is based on supportive care and the treatment of heart failure, while patients with AMI require myocardial revascularization. CONCLUSIONS: TC leads to transient LV dysfunction that mimics AMI from which it should be differentiated for a good therapeutic approach. Patients with TC should be carefully monitored during hospitalization because they have a high recovery potential if optimally treated.

PedsQL™ sickle cell disease module: feasibility, reliability, and validity.

BACKGROUND: Sickle cell disease (SCD) is an inherited chronic disease that is characterized by complications such as recurrent painful vaso-occlusive events that require frequent hospitalizations and contribute to early mortality. The objective of the study was to report on the initial measurement properties of the new PedsQL™ SCD Module for pediatric patient self-report ages 5-18 years and parent proxy-report for ages 2-18 years. PROCEDURE: The 43-item PedsQL™ SCD Module was completed in a multisite study by 243 pediatric patients with SCD and 313 parents. Participants also completed the PedsQL™ 4.0 Generic Core Scales and PedsQL™ Multidimensional Fatigue Scale. RESULTS: The PedsQL™ SCD Module Scales evidenced excellent feasibility, excellent reliability for the Total Scale Scores (patient self-report α = 0.95; parent proxy-report α = 0.97), and good reliability for the nine individual scales (patient self-report α = 0.69-0.90; parent proxy-report α = 0.83-0.97). Intercorrelations with the PedsQL™ Generic Core Scales and PedsQL™ Multidimensional Fatigue Scales were medium (0.30) to large (0.50) range, supporting construct validity. PedsQL™ SCD Module Scale Scores were generally worse for patients with severe versus mild disease. Confirmatory factor analysis demonstrated an acceptable to excellent model fit. CONCLUSIONS: The PedsQL™ SCD Module demonstrated acceptable measurement properties. The PedsQL™ SCD Module may be utilized in the evaluation of SCD-specific health-related quality of life in clinical research and practice. In conjunction with the PedsQL™ Generic Core Scales and the PedsQL™ Multidimensional Fatigue Scale, the PedsQL™ SCD Module will facilitate the understanding of the health and well-being of children with SCD.