Psychosocial Health Summary Score Research Articles

Investigation of the Relationship between Physical Activity Level and Quality of Life in Children with Specific Learning Disabilities using Regression Analysis

It has been reported that the academic skills of children with specific learning disabilities (SLDs) and their participation in physical activity may be affected. This study aimed to examine the relationship between physical activity level and quality of life in children with SLDs. The study was conducted with children diagnosed with SLDs at the Afyonkarahisar Special Education and Rehabilitation Center. Once the sociodemographic information of the participants was recorded, their physical activity levels were determined using the Physical Activity Questionnaire for Older Children (PAQ-C, ages 8-14 years), and their quality of life levels were assessed using the Pediatric Quality of Life Inventory (PedsQL). A total of 51 individuals, 21 girls and 30 boys, were included in the study. The mean age of the participants was 10.58±1.51 years. Physical activity score had a weak positive correlation with the physical health summary score (rspearman=0.342, p=0.014); weak positive correlations with the emotional functionality score (rspearman=0.308, p=0.028) and the school functionality score (rspearman=0.337, p=0.016); and moderate positive correlations with the school functionality score (rspearman=0.427, p=0.002), the psychosocial health summary score (rspearman=0.440, p=0.001), and the total PedsQL score (rspearman=0.462, p=0.042). In addition, the physical activity score was determined to statistically significantly predict the total PedsQL score (F=13.297; p=0.001). In conclusion, this study revealed that the physical activity levels of children with SLDs significantly affected their quality of life.

Prospective Evaluation of Health-Related Quality-of-Life in Children with Craniosynostosis.

To investigate health-related quality of life (HRQL) in children aged 2 to 7 years, who have undergone surgery for craniosynostosis. Cross-sectional survey. A tertiary pediatric academic medical center. Children with craniosynostosis who underwent surgical correction, and who were 2-7 years old at the time of the study. Children from families that did not speak English were excluded. Caregivers were asked to fill out the Pediatric Quality of Life Inventory (PedsQL) Core Parent Report and the PedsQL Cognitive Functioning Scale. PedsQL: Psychosocial Health Summary Score, Physical Health Summary Score, Total Core Score, Cognitive Functioning Scale Score. Scores range from 0 to 100, with higher scores reflecting greater QoLSubject factors: comorbidities, syndromic status, type of craniosynostosis, type of surgery. The study included 53 subjects, of whom 13.2% had a syndrome. Core and cognitive scores did not depend on presence of a syndrome or suture involved. Subjects who underwent posterior cranial distraction achieved higher Total Core Scores than subjects who underwent open vault remodeling. Among subjects with sagittal craniosynostosis, there was a tendency for higher scores among children who underwent minimally-invasive surgery compared to those who underwent open vault remodeling. This study demonstrates similar HRQL among children with and without a syndrome, higher HRQL among children undergoing posterior cranial distraction than those undergoing open vault remodeling, and trends towards higher HRQL in children with sagittal craniosynostosis who underwent minimally-invasive surgery compared to those who underwent open vault remodeling.

Evaluation of quality of life in children with precocious puberty.

This study aimed to investigate the quality of life of patients with central precocious puberty (CPP) who required treatment and premature thelarche (PT) followed up without treatment and to compare the groups with and without treatment among themselves and with healthy children. This study is designed as a case-control study. A total of 193 children including 59 children with CPP, 53 children with PT, 81 healthy children and their parents were included in the study. A questionnaire was applied to evaluate the sociodemographic characteristics that would affect the quality of life. The 'Pediatric Quality of Life Inventory (PedsQL)' was used to assess the quality of life. The PedsQL total scale score was 78.10 ± 17.13, 79.35 ± 11.54 and 79.52 ± 14.65, the psychosocial health summary score was 78.86 ± 16.83, 79.40 ± 12.54 and 79.94 ± 14.94 and physical health summary score was 75.81 ± 20.69, 79.41 ± 15.04 and 78.25 ± 17.52 in CPP, PT and control groups, respectively; however, there was no statistical difference (p > .05). In the scale administered to the parents, scores were similar in the three groups. No difference was found between CPP, PT and control groups in terms of sociodemographic data in the study (p > .05). Unlike previous studies, in this study the effects of sociodemographic characteristics and whether treatment was initiated or not on quality of life were investigated. Although the scale scores of the CPP group were lower than the PT and control group, there was no statistically significant difference, indicating that quality of life was not negatively affected in the CPP group receiving treatment.

Evaluation of Psychiatric Symptomatology, Quality of Life, and Caregiver Burden in Mothers and Children with Primary Immunodeficiency

Background: The present study aimed to evaluate the quality of life, depression, and anxiety scores of children with primary immunodeficiency (PID) and depression, anxiety scores, and the caregiving burden of their mothers. Methods: A total of 149 children aged 2-18 years and their mothers were included in the present study, along with 125 healthy children and their mothers as a control group. The Pediatric Quality of Life Inventory (PedsQL), Child Depression Inventory (CDI), and Screening for Child Anxiety-Related Emotional Disorders (SCARED) questionnaire were used based on the views of children and their mothers. The Beck Depression Inventory (BDI), Beck Anxiety Inventory (BAI), Temperament Evaluation of Memphis, Pisa, Paris, San Diego Autoquestionnaire (TEMPS-A), and Zarit Caregiver Burden Scale (ZCB) were used for the mothers. Results: According to children and their mothers, the scores of the PedsQL were lower than that of the control group (P < 0.05). In addition, according to the views of children and mothers, we found that PID children had higher depression and anxiety scores than healthy children (P < 0.05). The depression and anxiety levels of mothers in the patient group were also significantly higher than those in the control group (P = 0.05 and P = 0.001). Conclusion: Statistically, we found significantly lower psychosocial health summary scores and total scale score levels from the subclass of PedsQL in the patient group than in the control group. According to the views of both children and mothers, we observed that PID children had higher depression and anxiety scores than healthy children. It was also found that the BDI and BAI values in case of mothers in the patient group were significantly higher than those in the control group.

Longitudinal health-related quality of life analysis in childhood cancer survivors after proton beam therapy.

Whilst proton beam therapy (PBT) for children with cancer is expected to reduce their comorbidities, to date only a limited number of studies have been published. To analyze the long-term comorbidity and health-related quality of life (HRQoL) of childhood cancer survivors (CCSs) after PBT, we conducted a questionnaire-based study. Questionnaires were sent to CCSs who underwent PBT at the University of Tsukuba Hospital during the period from 1984 to 2020. Scores from 41 CCSs who did not undergo PBT (noPBT-CCSs) and from the general population were used for comparison. In total, 110 individuals who underwent PBT participated in the study. Among them, 40 individuals were longitudinally analyzed. The range of change in the scores was significantly greater in the CCSs whose initial scores were low. Although the comorbidity levels were more severe, HRQoL tended to be better in the PBT-CCSs than in the noPBT-CCSs with central nervous system (CNS) or solid tumors, respectively. When compared with the general population, the psychosocial health summary scores and its components were not different in the noPBT-CNS-CCSs. On the other hand, the psychosocial health summary scores and/or at least one of the scores of emotional, social, and school functioning were significantly higher in the other CCSs groups. The HRQoL scores of CCSs with low initial scores can be greatly changed over time. Appropriate psychosocial support for this population is warranted. PBT may avoid reduction in HRQoL in terms of the psychosocial functioning of CCSs with CNS tumors.

Maternal adverse childhood experiences and health-related quality of life in preschool children: a cross-sectional study

BackgroundThe intergenerational association between maternal adverse childhood experiences (ACEs) and their children’s health-related quality of life (HRQOL) is underexplored. This study aimed to examine such association in Chinese preschool children and to test the moderation role of children’s sex.MethodsA cross-sectional study was conducted among 4243 mother–child dyads who attended randomly selected preschools. Mothers self-reported their experience of 12 forms of ACEs, including emotional abuse, physical abuse, emotional neglect, physical neglect, intimate partner violence, substance abuse in the household, incarcerated household member, mental illness in household, parental death, parental separation or divorce, bullying, and community violence. Children’s HRQOL was evaluated through mother report of the Pediatric Quality of Life Inventory version 4.0. Linear regression models were established to estimate the associations between maternal ACEs and their children’s HRQOL sub-scores and total scores. Stratified analysis and test for interaction were further conducted to evaluate whether the associations were moderated by children’s sex.ResultsOf the included mothers, 85.8% (n = 3641) had reported exposure to at least one ACE, and 22.3% (n = 948) were exposed to three or more ACEs. Compared to children of mothers without any ACE exposure, those of mothers with 1, 2, or ≥ 3 ACEs all had significantly lower scores of physical, social, and school functioning, as well as lower psychosocial health summary score and total scale score in both crude and adjusted models. However, only children of mothers with two or more ACEs had significantly poorer emotional functioning when compared to their counterparts whose mothers had no ACE exposure. A significant dose-response pattern was also observed between the number of maternal ACEs and children’s HRQOL sub-scores and total scores. Stratified analysis revealed sex-specific pattern between maternal ACEs and their children’s HRQOL. Nonetheless, children’s sex was not a significant moderator.ConclusionsOur study showed that preschool children of mothers who had any experience of ACEs were at risk of poorer HRQOL. Our findings indicated that screening maternal ACEs in young children and promoting targeted interventions might be a feasible way to mitigate or stop the potential negative intergenerational health and wellbeing implications of ACEs.

Evaluation of Psychiatric Symptomatology, Quality of Life, and Caregiver Burden in Mothers and Children with Primary Immunodeficiency.

The present study aimed to evaluate the quality of life, depression, and anxiety scores of children with primary immunodeficiency (PID) and depression, anxiety scores, and the caregiving burden of their mothers. A total of 149 children aged 2-18 years and their mothers were included in the present study, along with 125 healthy children and their mothers as a control group. The Pediatric Quality of Life Inventory (PedsQL), Child Depression Inventory (CDI), and Screening for Child Anxiety-Related Emotional Disorders (SCARED) questionnaire were used based on the views of children and their mothers. The Beck Depression Inventory (BDI), Beck Anxiety Inventory (BAI), Temperament Evaluation of Memphis, Pisa, Paris, San Diego Autoquestionnaire (TEMPS-A), and Zarit Caregiver Burden Scale (ZCB) were used for the mothers. According to children and their mothers, the scores of the PedsQL were lower than that of the control group (P < 0.05). In addition, according to the views of children and mothers, we found that PID children had higher depression and anxiety scores than healthy children (P < 0.05). The depression and anxiety levels of mothers in the patient group were also significantly higher than those in the control group (P = 0.05 and P = 0.001). Statistically, we found significantly lower psychosocial health summary scores and total scale score levels from the subclass of PedsQL in the patient group than in the control group. According to the views of both children and mothers, we observed that PID children had higher depression and anxiety scores than healthy children. It was also found that the BDI and BAI values in case of mothers in the patient group were significantly higher than those in the control group.

Associations of excessive screen time and early screen exposure with health-related quality of life and behavioral problems among children attending preschools

BackgroundBoth excessive screen time and early screen exposure have been linked to children’s health outcomes, but few studies considered these two exposures simultaneously. The aim of this study was to explore the independent and interactive associations of excessive screen time and early screen exposure with health-related quality of life (HRQOL) and behavioral problems among Chinese children attending preschools.MethodsA cross-sectional study of 4985 children aged between 3 and 6 years was conducted in Chengdu, China. Each parent has finished an online questionnaire regarding their children’s screen use, HRQOL, and behavioral problems. Children with screen time over 1 h/day were considered as having excessive screen time. Early screen exposure was defined if the children had started using screen-based media before the age of 2 years. HRQOL was assessed by the Pediatric Quality of Life Inventory version 4.0 (PedsQL 4.0), while behavioral problems were confirmed with the 48-item Conners’ Parent Rating Scale (CPRS-48).ResultsOf the 4985 children (2593 boys and 2392 girls) included, the mean age was 4.6 (SD: 1.0) years. After adjustment for confounders and early screen exposure, excessive screen time was significantly associated with worse HRQOL scores in all dimensions and summary scales, as well as each type of behavioral problems (all p values < 0.05). We also found that compared to children with later initiation of screen exposure, those with screen use before the age of 2 years had significantly lower emotional functioning score (β: − 2.13, 95%CI: − 3.17, − 1.09) and psychosocial health summary score (β: − 0.82, 95%CI: − 1.54, − 0.10) of HRQOL, as well as higher risks of conduct problems, learning problems, psychosomatic problems, impulsive-hyperactive, and hyperactivity index, which were independent of excessive screen use. Furthermore, there were significant interactive effects of excessive screen time and early screen exposure on emotional functioning domain of HRQOL scores and conduct problems.ConclusionExcessive screen time and early screen exposure are two independent and interactive factors to children’s HRQOL and behavioral problems. Our findings support current guidelines to limit screen exposure in children. Appropriate screen use may represent an important intervention target to improve children’s HRQOL and reduce their behavioral problems.

Minimal important difference in childhood interstitial lung diseases

BackgroundMonitoring disease progression in childhood interstitial lung diseases (chILD) is essential. No information for the minimal important difference (MID), which is defined as the smallest change in a parameter that...

Association between maternal intimate partner violence and health-related quality of life in their preschool children: The mediating role of maternal parenting styles.

Although intimate partner violence (IPV) against women is a public health issue around the world, there is a lack of evidence regarding the impact of maternal IPV on preschool children's health-related quality of life (HRQOL). Therefore, the aim of this study was to investigate the association between maternal IPV and HRQOL among Chinese preschool children, as well as the mediating role of maternal parenting styles. A cross-sectional study was conducted with 4,243 mother-child dyads who attended preschools. Mothers self-reported their parenting styles and experience of IPV. Children's HRQOL was collected through mother-proxy report with the Pediatric Quality of Life Inventory version 4.0 (PedsQL 4.0). Multivariate linear regression analysis was conducted to evaluate the association between maternal IPV and children's HRQOL. Mediation models were further applied to explore the possible mediating role of maternal parenting styles. Of the included mothers, 7.4% had experience of IPV. Compared to children of mothers without any IPV exposure, those of mothers with experience of IPV had significantly lower scores in all HRQOL dimensions and summary scales. After adjustment for covariates, maternal IPV was significantly associated with children's lower physical health summary score, psychosocial health summary score, and total scale score. Mediation analysis showed that both rejection and overprotection mediated such associations, but not for the emotional warmth. Our findings indicated the need to screen maternal IPV supplemented with targeted interventions focusing on parenting styles, in order to mitigate the negative impact of maternal IPV on children's HRQOL.

Quality of life and symptom burden in children with neurodegenerative diseases: using PedsQL and SProND, a new symptom-based scale

BackgroundChildren with neurodegenerative conditions (CNDC) often suffer from severe neurodisability and high symptom burden with multisystemic involvement. However, their symptom burden and health-related quality of life (HRQOL) is not systematically documented in the literature, and there is no existing tool for such purposes. We designed our own tool for scoring of symptom burden amongst CNDCs and adopted the PedsQL generic score 4.0 to quantify the impact of overall symptom burden on children’s overall HRQOL.MethodsThe Symptom Profile for children with neurodegnerative condition (SProND) questionnaire was developed, which consisted of 14 questions grouped according to 5 categories, namely epilepsy, neurobehavioural, movement and mobility related, breathing and swallowing, and other daily activities. CNDCs were recruited during visits to the Comprehensive Neurometabolic / Neurodegenerative Program of the Duchess of Kent Children’s Hospital and Hong Kong Children’s Hospital between November 2019 and March 2020. The SProND and PedsQL 4.0 Generic Core Scales were distributed to consenting parents of CNDCs.Results36 CNDCs were recruited and matched with community controls. The response rate of subject and control were 99.5% and 98.7% respectively. The Cronbach alpha was 0.61 for the neurobehavioural domain and > = 0.7 for other domains. The greater number of symptoms each subject experiences, the worse his/ her PedsQL scores. Subjects displaying hypersalivation and swallowing difficulties had average physical health summary scores of less than 30% compared with subjects without these symptoms. On the other hand, average psychosocial health summary scores of subjects with involuntary movements, joint stiffness, hypersalivation, sleep problem and anorexia were approximately 70% compared to subjects without these symptoms.Discussion and conclusionThis is one of the first studies to look at CNDCs as a group. We propose the SProND questionnaire for evaluation of symptom profile amongst CNDCs with satisfactory internal and external validity. It demonstrates how physical symptoms impact both physical and psychosocial HRQOL, and the cumulative effect of individual symptoms on the overall HRQOL. As such, CNDCs should be systematically screened for multi-systemic symptoms as a routine part of their clinical care, and care plans should be individually catered to individual patients’ symptom burden and specific needs.

333.7: Measurement of Quality of Life in Adolescent Transplanted Patients: A Necessary Approach

Introduction: Liver transplantation is the option for pediatric patients with acute or chronic terminal diseases. Multiple innovations have been made to transplant programs worldwide to improve effectiveness, safety and timely services. Until now, only hard indicators (survival/complication rate) have been used to evaluate effectiveness of transplantation. Since these indicators are not enough, it is necessary to include the perspective of the patient and their family to determine the impact that the transplant has on their quality of life. Even though there is no “gold standard” to measure the health related quality of life (HRQoL) in our center, the use of generic HRQoL questionnaires allows an objective evaluation of their quality of life. The objective of the present study is to analyze and describe the results of the PedsQL 4.0 Generic Questionnaire when applied to adolescent patients who received a liver transplant in a public hospital in Argentina, and compare them to healthy adolescents. Methods: PedsQL 4.0 Generic Core Scale was applied to 45 adolescent (older than 17 years old) who had received a liver transplant more than 10 years ago. The scale is a self-reported 5-point Likert scale with 23 items that ranges from 0 (Never) to 4 (Almost always). It is brief and multidimensional, as it includes Physical, Emotional, Social, and School Functioning dimensions. Results were linearly transformed to a 0-100 scale (0=100, 1=75, 2=50, 3=25, 4=0). Total score (as well as Psychosocial Health Summary Score and Physical Health Summary Score) was calculated as mean and SD. T-test were used to compare with data extracted from the literature from previous validation studies for Argentinian healthy adolescents. Results: A total of 45 patients completed the survey. Missing items were only 0.7% of items, so complete data for all patients was included. No difficulties with the format nor the form of administration of the questionnaire were found. 51% of patients were female, with a median follow-up of 15.52 years. PedsQL Total Score was 73.89 (15.01) vs 72.72 (14.21) in healthy adolescents, and showed no statistical difference among them (p 0.84). The Psychosocial Health Summary Score was 69.96 (16.59) vs 71.20 (14.84), while the Physical Health Summary Score was 81.25 (15.55) vs 75.42 (15.93) in healthy patients respectively (p 0.846 and 0.334). Conclusions: In this study, no difference in HRQoL was found among adolescents transplanted patients when compared with published data from healthy population. Even though this is an initial approach to measuring quality of life and some limitations could be found (small sample size, generic module and literature comparison), further studies are needed to validate specific questionnaires for transplanted patients in our center, focusing on the unique aspects of this chronic condition. We consider this measure a necessary endpoint of the transplant in order to complete and improve patient´s care and results.

Effect of fingolimod on health-related quality of life in paediatric patients with multiple sclerosis: results from the phase 3 PARADIGMS Study

BackgroundIn the PARADIGMS Study, fingolimod demonstrated superior efficacy versus interferon (IFN) β-1a and comparable overall incidence of adverse events but slightly higher rate of serious adverse events in patients with...

Reliability and validity of the Japanese version of the Pediatric Quality of Life Inventory Infant Scales

BackgroundPedsQL Infant Scales (PedsQL-I) are used to assess parent-reported health-related quality of life for children younger than 2 years. We determined the feasibility, reliability, and validity of the Japanese version of the PedsQL-I.MethodsA total of 183 participants (parents) with infants aged 1–30 months were recruited from 8 day care centers and one pediatric clinic. Participants completed the PedsQL-I (infants aged 1–18 months), the PedsQL-I and the PedsQL-Toddler version (infants aged 19–30 months), and the Kessler-6 psychological distress scale (all participants). We determined feasibility, internal consistency, test–retest reliability, concurrent validity, convergent and discriminant validity, known-groups validity with regard to acute and chronic illness, and relative and transitional validity with PedsQL-Toddler for the use in infants aged 25–30 months.ResultsAll subscales were internally consistent (Cronbach’s alpha for 1–12 months: 0.88–0.98 and for 13–24 months: 0.85–0.97); test–retest reliability was acceptable (intra-class correlation coefficients > 0.40); and all scales were concurrently valid with the PedsQL-Toddler version (Pearson’s product-moment correlation coefficient for the total score = 0.74). The scales’ convergent and discriminant validity were acceptable (scaling success rate > 80%). Validation for known-groups showed that the Physical Health Summary score was sensitive to acute and chronic disease, the Psychosocial Health Summary score was sensitive to neither acute nor chronic disease, and the total score was sensitive to acute disease. Relative validity showed a ratio of 1.74 for the squared t values for the total score.ConclusionsThe PedsQL-I is suitable for assessing health-related quality of life in infants aged 1–24 months in prospective studies.

Quality of life of adolescents with menstrual problems in Klang Valley, Malaysia: a school population-based cross-sectional study

ObjectiveTo describe the prevalence of menstrual problems (heavy menses bleeding, dysmenorrhoea and oligomenorrhoea) and its impact towards quality of life among adolescents in Klang Valley, Malaysia.DesignCross-sectional study.SettingAdolescent girls at secondary...

Factors Associated With Quality of Life in Children Receiving Pediatric Palliative Care

ContextSince pediatric palliative care (PPC) aims to improve the health-related quality of life (HRQoL) of children with life-limiting conditions (LLC), assessment of their HRQoL and identification of its determinants is crucial. ObjectivesTo examine the clinical and family factors associated with HRQoL of children with LLC MethodsThis was a cross-sectional study of 136 pediatric patients with LLC who were enrolled in the PPC services at Seoul National University Hospital in South Korea. Patients’ HRQoL was measured using the Pediatric Quality of Life 4.0. Clinical and family characteristics were gathered from the medical records and PPC registry database. ResultsMost children with LLC have a poor HRQoL at their enrollment for PPC services with significant variation in their total HRQoL scores according to the diagnostic categories. Patients with nonmalignant conditions showed significantly lower HRQoL scores than patients with malignancy. Lower HRQoL scores were associated with more caregiver depressive symptoms. In a multivariable regression model, total HRQoL scores of patients were significantly associated with diagnostic categories and caregiver's depressive symptoms after controlling for other clinical and family variables. Physical health summary scores were significantly associated with diagnostic categories and caregiver depressive symptoms. Psychosocial health summary scores were significantly associated with diagnostic categories, patient location, and caregiver's depressive symptoms. ConclusionThe HRQoL of children with LLC receiving PPC differed among underlying disease categories. Lower HRQoL was associated with more caregiver depressive symptoms. These findings suggest the needs for optimized intervention in palliative care for children with nonmalignant conditions and family-centered intervention to address caregivers’ psychosocial problems.

A cross-sectional study to assess quality of life in 2-18 years children and adolescent living with beta thalassemia major registered at comprehensive thalassemia care centre attached to tertiary care hospital in a metropolitan city

Background: Thalassemia is a serious inherited blood disorder. Children and adolescent living with thalassemia major require lifelong repeated blood transfusions and costly medicines for their survival. In India, β-thalassemia prevalence is 3-4%. Children are basic pillars of society although they are the most vulnerable part of society in view of nutrition, education and social life.Methods: Cross-sectional, observational study was conducted at comprehensive thalassemia care centre attached to tertiary care hospital in a metropolitan city. 76 children and adolescents living with beta thalassemia major aged between 2 to 18 years registered for blood transfusion were included.Results: The mean age of the study subjects was 13.81±6.71 years, 63.2% were males. Majority of study subjects belonged to Hindu religion. 46.3% study subjects were in primary school, 84.21% parents of study subjects gave the history of non-consangious marriage. 92.1% of the study subjects’ parents and their relatives were not aware of history of thalassemia in the family. Genetic counselling was offered to 71.1%. Age of onset of disease was 0-6 months amongst 64.5% of children. 53.9% children had B positive blood group. Total health summary score, emotional health summary score, social health summary score and psychosocial health summary score was better in age group 2-4 years. School health summary score was very low in all age groups.Conclusions: To improve the quality of life of children and adolescent living with beta thalassemia and to reduce catastrophic out pocket expenditure from parents of study subjects, there is need of developing comprehensive thalassemia care center at medical colleges/district hospitals for preventive and promotive health care of children and adolescents.

Health-Related Quality of Life and Associated Factors of Children with Transfusion-Dependent Thalassemia in Dubai, United Arab Emirates

BACKGROUND: Patients with transfusion-dependent thalassemia require lifelong blood transfusions and iron chelation therapy. Iron overload associated with regular blood transfusion leads to a significant defect in the health-related quality of life (HRQoL) of patients with transfusion-dependent thalassemia. Quality of life has become a significant component of care, and it is therefore necessary to focus on the HRQoL of patients with transfusion-dependent thalassemia. &#x0D; &#x0D; OBJECTIVES: To assess the HRQoL of children with transfusion-dependent thalassemia, identify the sociodemographic and clinical characteristics that affect the HRQoL, and identify the risk factors for poor HRQoL in children with transfusion-dependent thalassemia. &#x0D; &#x0D; STUDY DESIGN: A descriptive cross-sectional study was used to address the study&amp;rsquo;s objectives. &#x0D; &#x0D; SETTING: A representative sample was recruited from the Dubai Thalassemia Center in the United Arab Emirates (UAE). &#x0D; &#x0D; PARTICIPANTS: A total of 68 children with transfusion-dependent thalassemia aged 2-18 years. &#x0D; &#x0D; INTERVENTION: PedsQL 4.0 was applied to assess the HRQoL. The total HRQoL score was treated as the dependent variable in the study. Regression analysis was applied to study the effect of sociodemographic and clinical characteristics on HRQoL. &#x0D; &#x0D; RESULTS: This study included 68 children with transfusion-dependent thalassemia. Among them, 55.9% fell into the 13-18-year age group, and 42.6% were male. In addition, 57.4% were non-UAE nationals, and 44.1% of the children had less than 10,000 AED as monthly household income. The median (IQR) physical health summary score was 89.9 (15.6) and the psychosocial health summary score was 90.0 (10.3). Total PedsQL scores were significantly higher for younger children, those with a higher monthly household income, and those without complications. Older children were significantly correlated with lower total, physical, and psychosocial health summary PedsQL scores, whereas lower monthly household income was significantly correlated with lower total and psychosocial health summary PedsQL scores. Controlling for other variables, increasing age of patients was associated with worse total PedsQL scores. &#x0D; &#x0D; CONCLUSIONS: Children with transfusion-dependent thalassemia and their families require long-term support for the prevention of physical and mental problems associated with this disease.

Health Related Quality of Life of Children with Thalassemia Major in Two Selected Hospitals in Sri Lanka

Introduction: Thalassemia is an inherited hemoglobin disorder. There are two types; thalassemia major and minor. Thalassemia major directly affects children’s physical, emotional, social and school functions. Health Related Quality of Life (HRQoL) is an important indicator to assess the health of human. Methods: A cross sectional study was conducted using 60 children (age 2-12 years) with thalassemia major who attended clinics in two selected hospitals in Sri Lanka from July to September 2018. Demographic data and risk factors were collected using a self-administered questionnaire. HRQoL data were collected using the Pediatric Quality of Life Inventory Version 4.0 Generic Core Scale. Data were analyzed using SPSS. Results: Mean values of the total functioning scores of physical, emotional, social and school were 89.42 (±9.20), 87.83 (±10.43), 95.00 (±7.53) and 78.75 (±14.13) respectively. Age of the children was significantly associated with emotional functioning (p=0.046), school functioning (p=0.023), psychosocial health (p=0.021) and the total summary score (p=0.006). Total number of blood transfusions per year of the patient was significantly associated with physical functioning (p=0.002), psychosocial health (p=0.045) and total summary score (p=0.034). Conclusions: Age and the total number of blood transfusions per year significantly affect the HRQoL of children with thalassemia major. Gender, type of iron chelation and age at first blood transfusion did not affect HRQoL of the children.Keywords: HRQoL, Thalassemia major, Haemoglobin disorder

Quality of life in children with tuberous sclerosis complex: A pediatric cohort study.

AimsTo evaluate the quality‐of‐life (QOL) impairment and identify the possible risk factors in patients with tuberous sclerosis complex (TSC) in China.MethodsThe parent proxy‐report PedsQL 4.0 Generic Core Scales were administered to 124 caregivers of children with TSC (aged 2‐18 years). For comparison, the survey was also conducted in a demographically group‐matched sample of healthy controls (HCs) (aged 2‐18 years).ResultsA total of 124 children with TSC and 206 HCs were recruited. The mean parent proxy‐report total scale score, physical health summary score, and psychosocial health summary score for children with TSC were 65.0 (SD 19.7), 77.6 (SD 22.9), and 58.0 (SD 21.3), respectively, compared with the HC values of 83.6 (SD 14.3), 87.2 (SD 16.9), and 82.8 (SD 15.9). There were statistically significant differences between the two groups (P < .0001). TSC2 mutation (P = .033), epilepsy (P = .011), seizure before 2 years old (P = .001), course of epilepsy (more than 2 years) (P = .001), high reported seizure frequency (more than once a month) (HRSF) (P = .007), multiple antiepileptic drugs (≥2) (P = .002), intellectual disability (ID) (mild and moderate ID, P < .0001, and severe and profound ID, P < .0001), and TANDs (P < .0001) (ADHD, P = .004; agoraphobia, P = .007; and social anxiety disorder, P < .0001) were closely related to lower QOL scores.ConclusionThis study is the first large cohort study on QOL in children with TSC in China. The results of the PedsQL 4.0 indicated that the QOL of children with TSC is significantly lower than that of HCs. TSC2 mutation, epilepsy, early onset, long disease course and HRSF, ID, and TANDs are risk factors for poor QOL.