Preliminary Study of Neurodevelopmental Outcomes and Parenting Stress in Pediatric Mitochondrial Disease
emotion; child behavior; priority journal; intellectual impairment/dm [Disease Management]; prognosis; preschool child; parental stress; human; article; child; female; male; quality of life; clinical article; daily life activity; disease severity; aggression; intelligence quotient; childhood disease/dm [Disease Management]; comorbidity; Leigh disease/dm [Disease Management]; MELAS syndrome/dm [Disease Management]; nervous system development; problem behavior/dm [Disease Management]; anxiety disorder/dm [Disease Management]; attention disturbance/dm [Disease Management]; brain atrophy/dm [Disease Management]; delinquency; depression/dm [Disease Management]; drug resistant epilepsy/dm [Disease Management]; drug resistant epilepsy/dr [Drug Resistance]; intelligence; lactic acidosis/dm [Disease Management]; muscle disease/dm [Disease Management]; neuroimaging; nuclear magnetic resonance imaging; postnatal depression/dm [Disease Management]; psychomotor development; sleep disorder/dm [Disease Management]; social problem; somatization/dm [Disease Management]; thinking impairment/dm [Disease Management]; behavioral problems; Leigh syndrome; mitochondrial disorders; MELAS syndrome; trajectory; characteristics
Background Little is known regarding the neuropsychological profiles of pediatric patients with mitochondrial diseases or their parents, information that is crucial for improving the quality of life (QOL) for both patients and parents. We aimed to delineate neurodevelopment and psychological comorbidity in children with mitochondrial diseases in the preliminary investigation of adequate intervention methods, better prognoses, and improved QOL for both patients and parents. Methods Seventy children diagnosed with mitochondrial diseases were neuropsychologically evaluated. Neurocognitive (development, intelligence) and psychological (behavior, daily living function, maternal depression, parenting stress) functions were analyzed. Clinical variables, including the first symptom, epileptic classification, organ involvement, lactic acidosis, brain magnetic resonance imaging findings, muscle pathology, biochemical enzyme assay results, and syndromic diagnosis of mitochondrial diseases, were also reviewed. Results Prediagnostic assessments indicated that cognitive and psychomotor developments were significantly delayed. Group mean full scale intelligence quotient (IQ) scores indicated mild levels of intellectual disability, borderline levels of verbal IQ impairment, and mild levels of intellectual disability on performance IQ. Many children exhibited clinically significant levels of behavioral problems, whereas mothers of children with mitochondrial diseases exhibited significant increases in parenting stress relative to mothers of healthy children. Furthermore, 65% of mothers exhibited significant levels of depression. Early onset of the first symptoms, diffuse brain atrophy, and drug-resistant epilepsy negatively influenced neurodevelopmental and adaptive functions. Conclusion Better understanding of the functional levels and profiles of neurodevelopment and psychological comorbidity in children with mitochondrial diseases in the prediagnostic period is essential for adequate support and QOL of children with mitochondrial diseases and their parents.
Eom S; Lee Y M
Pediatric Neurology
2017
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<a href="http://doi.org/10.1016/j.pediatrneurol.2017.01.019" target="_blank" rel="noreferrer noopener">10.1016/j.pediatrneurol.2017.01.019</a>
Costs of Care and Location of Death in Community-Based Pediatric Palliative Care
adolescent; adult; article; cardiovascular disease/dm [Disease Management]; child; child death; child health care; child health insurance; childhood cancer/dm [Disease Management]; childhood disease/dm [Disease Management]; cohort analysis; community care; community-based; congenital disorder/dm [Disease Management]; cost; dying; family income; female; groups by age; health care cost; health maintenance organization; home care; human; major clinical study; male; medicaid; neuromuscular disease/dm [Disease Management]; newborn disease/dm [Disease Management]; palliative therapy; pediatric palliative care; pediatric patient; prematurity/dm [Disease Management]; race difference; retrospective study
Background: Children with complex chronic conditions (CCCs) are dying at home with increased frequency, yet the number of studies on the financial feasibility of community-based pediatric palliative care is limited. Objective(s): The objectives of this study were to (1) describe characteristics of patients who died in a community-based palliative care program and (2) evaluate cost differences associated with participant characteristics and location of death. Design(s): A retrospective cohort analysis of administrative and electronic medical record data was employed. Setting/Subjects: Children enrolled in the community-based pediatric palliative care program, CompassionNet, who died between 2008 and 2015 were included (N = 224). Measurements: Demographic data, program expense, and paid claims were extracted from an insurance provider database and clinical data from the electronic medical record. Result(s): Sixty-six (29%) of the children were \textless1 year old at death; 80 (36%) were 1-9 years old, and 78 (35%) were 10-22 years old. Malignancy was the most common primary CCC diagnosis for the 158 children/adolescents (n = 89, 56%), whereas neuromuscular conditions (n = 20, 30%) were most frequent for infants. Death at home occurred 21% of the time for infants, 48% for children of ages 1-9 years, and 46% for children of ages 10-22 years. The mean total cost in the final year of life for pediatric patients was significantly related to location of death, a malignancy diagnosis, and participation in Medicaid. The largest estimated difference was between costs of care associated with death at home ($121,111) versus death in the hospital ($200,050). Conclusion(s): Multidisciplinary community-based pediatric palliative care teams provide the opportunity for a home death to be realized as desired. Significant cost differences associated with location of death may support program replication and sustainability. Copyright © 2019, Mary Ann Liebert, Inc.
Chirico J; Donnelly J P; Gupton A; Cromwell P; Miller M; Dawson C; Korones D N
Journal of Palliative Medicine
2019
<a href="http://doi.org/10.1089/jpm.2018.0276" target="_blank" rel="noreferrer noopener">10.1089/jpm.2018.0276</a>