Dataset Information

Health-Related Quality of Life in Children With Congenital Hyperinsulinism.

ABSTRACT: Background: Quality of life (QoL) has not been studied in patients with congenital hyperinsulinism (CHI). Objectives: To examine whether the health-related quality of life (HRQoL) is worsened in patients with persistent or transient CHI. Methods: We studied HRQoL of 65 children with CHI aged 3-17 years (60% males) recruited from the nationwide CHI registry. The median ages were 9.6 (range 3.5-16.3) and 7.4 (3.1-17.9) years in persistent (P-CHI, n = 33) and transient (T-CHI, n = 32) CHI groups, respectively. HRQoL was examined by generic KINDL-R questionnaire and the scores were compared to the age- and gender-specific reference values. Results: In self-reports of subjects aged 11-17 years and in parent reports of children aged 3-17 years, P-CHI or T-CHI children did not have statistically lower scores in any of the six dimensions (physical well-being, emotional well-being, self-esteem, family, friends, and school) or in total scores compared to the reference values. Conclusions: CHI is not associated with low HRQoL in childhood or adolescence.

SUBMITTER: Mannisto JME

PROVIDER: S-EPMC6779698 | biostudies-literature | 2019

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

Health-Related Quality of Life in Children With Congenital Hyperinsulinism.

Männistö Jonna M E JME Jääskeläinen Jarmo J Huopio Hanna H

Frontiers in endocrinology 20191001

Background: Quality of life (QoL) has not been studied in patients with congenital hyperinsulinism (CHI). Objectives: To examine whether the health-related quality of life (HRQoL) is worsened in patients with persistent or transient CHI. Methods: We studied HRQoL of 65 children with CHI aged 3-17 years (60% males) recruited from the nationwide CHI registry. The median ages were 9.6 (range 3.5-16.3) and 7.4 (3.1-17.9) years in persistent (P-CHI, n = 33) and transient ( ...[more]

PMID: 31632349

Similar Datasets

Project description:BackgroundCongenital heart disease (CHD) is the most common congenital anomaly at birth, affecting approximately 1% of live births. In recent decades great medical and surgical advances have significantly increased life expectancy, shifting healthcare professionals' and researchers' interests in patients' Quality of Life (QoL). The main aims of our study were to evaluate generic and condition-specific QoL in a group of Italian children and adolescents with CHD and their parents and examine the level of agreement and directional disagreement between child/adolescent and parents reports on generic and condition-specific QoL.MethodsA cross-sectional study was designed with CHD children and adolescents and their parents referred to the Cardiology Department of "Bambino Gesù" Children's Hospital. The PedsQL scale was used, including generic (PedsQL 4.0) and cardiac-specific modules (PedsQL 3.0) were administered to patients and caregivers. A Kruskal-Wallis test was used to compare generic and cardiac module scores between patients with different ages, CHD diagnoses, and between patients who underwent surgery interventions and/or are currently taking cardiac medications.Results498 families were enrolled in this study. On average, patients reported a good level of generic and condition-specific QoL, as well as their mothers and fathers. Children aged between 5-7 years old reported lower generic and cardiac-specific total QoL levels than children aged 8-12 years and adolescents (13-18 years). With regard to the agreement, patient-parent agreement on condition-specific QoL ranged from 25 to 75% while on generic QoL, it ranged from 19 to 76%. The highest percentage of disagreement between parents and children was found in patients aged 5-7 years old, both for condition-specific and generic QoL rates.ConclusionsOur study contributed to the growing body of knowledge on QoL in CHD, emphasizing the need for these families to receive support from multidisciplinary standardized care, including psychological consultations and support.

Project description:ObjectiveThere are limited data on health-related quality of life (HRQOL) for children and adolescents with uncorrected congenital heart disease (CHD) from low-income and middle-income countries where late presentation is common. We sought to compare HRQOL of children and adolescents with uncorrected CHD to that of controls using the Pediatric Quality of Life Inventory (PedsQL 4.0).MethodsThe study design is a cross-sectional analytical survey. The study setting was (1) Hospital-based survey of patients with CHD and their parents. (2) Community survey of controls and their parents. Subjects included (1) Children/adolescents with CHD between the ages of 2 years and 18 years and their parents enrolled in a previous study (n=308). (2) Unmatched community controls (719 children/adolescents, aged 2-18 years) and their parents. Participants were given PedsQL 4.0 to fill out details. Parents assisted children 5-7 years of age in filling the questionnaires. Children younger than 5 years had only parent-reported HRQOL and those above 5 years had both self-reported and parent-reported HRQOL.ResultsThe median (IQR) total generic HRQOL from self-reports for CHD subjects and controls were 71.7 (62.0, 84.8) and 91.3 (82.6, 95.7), respectively. The corresponding figures for parent-reports were 78.3 (63.0, 90.5) and 92.4 (87.0, 95.7) respectively. The adjusted median difference was -20.6 (99% CI -24.9 to -16.3, p<0.001) for self-reported and -14.1 (99% CI -16.7 to -11.6, p<0.001) for parent-reported total HRQOL between patients with CHD and controls. Cardiac-specific HRQOL by self-reports was 75.0 (53.6, 92.9) for heart problems, 95.0 (73.8, 100.0) for treatment barriers, 83.3 (66.7, 100.0) for physical appearance, 87.5 (62.5, 100.0) for treatment-related anxiety, 91.7 (68.8, 100.0) for cognitive problems and 83.3 (66.7, 100.0) for communication. The values for parent-reports were 71.4 (53.6, 85.7), 100.0 (75.0, 100.0), 100.0 (75.0, 100.0), 81.3 (50.0, 100.0), 100.0 (81.2, 100.0) and 83.3 (50.0, 100.0), respectively.ConclusionsChildren and adolescents with uncorrected CHD reported significant reductions in overall quality of life compared with controls.

Dataset Information

Health-Related Quality of Life in Children With Congenital Hyperinsulinism.

Publications

Health-Related Quality of Life in Children With Congenital Hyperinsulinism.

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets