Health-related quality of life in children with celiac disease

We have investigated the health-related quality of life of children with celiac disease (n = 133) using two generic and one disease-specific questionnaires. In general, the children reported an adequate quality of life, similar to that of the reference sample (n = 1183).     

Medical problems in adolescents with myelomeningocele (MMC): an inventory of the Swedish MMC population born during 1986-1989

AIM: To describe the prevalence of myelomeningocele (MMC) and the medical needs of adolescents, 15-18 years, with MMC in Sweden, at a time when they are on the threshold of adulthood, leaving paediatrics. METHODS: In a retrospective study, we identified all adolescents with MMC, born during 1986-1989 and living in Sweden on July 1, 2004. An inventory was agreed upon with questions concerning their medical problems and need for medical care. RESULTS: There were 175 persons 15-18 years of age, born with MMC or lipoMMC (prevalence 3.8 per 10,000). Hydrocephalus was seen in 86%, 31% had been operated because of tethered cord syndrome, and 6% for Chiari malformation symptoms. The majority had motor impairments. Clean intermittent catheterisation for bladder emptying was used by 85%, and 59% used enemas on a regular basis because of the neurogenic bowel dysfunction. Renal dysfunction was seen in 1.7% of the adolescents. CONCLUSION: Lifelong follow-up by many specialists, among others neurologists and neurosurgeons, urotherapists and urologists, orthopaedic surgeons and orthotists, is necessary for individuals with MMC. The complex medical situation, often in combination with cognitive difficulties, makes it necessary to coordinate medical services for this increasing group of adults with multiple impairments.     

Health-related quality of life in children with inflammatory brain disease

Objective

To quantify the impact of inflammatory brain diseases in the pediatric population on health-related quality of life, including the subdomains of physical, emotional, school and social functioning.

Methods

This was a multicenter, observational cohort study of children (<?18?years of age) diagnosed with inflammatory brain disease (IBrainD). Patients were included if they had completed at least one Health Related Quality of Life Questionnaire (HRQoL). HRQoL was measured using the Pediatric Quality of Life Inventory Version 4.0 (PedsQL) Generic Core Scales, which provided a total score out of 100. Analyses of trends were performed using linear regression models adjusted for repeated measures over time.

Results

In this study, 145 patients were included of which 80 (55%) were females. Cognitive dysfunction was the most common presenting symptoms (63%), and small vessel childhood primary angiitis of the CNS was the most common diagnosis (33%). The mean child’s self-reported PedsQL total score at diagnosis was 68.4, and the mean parent’s proxy-reported PedsQL score was 63.4 at diagnosis. Child’s self-reported PedsQL scores reflected poor HRQoL in 52.9% of patients at diagnosis. Seizures or cognitive dysfunction at presentation was associated with statistically significant deficits in HRQoL.

Conclusion

Pediatric IBrainD is associated with significantly diminished health-related quality of life. Future research should elucidate why these deficits occur and interventions should focus on improving HRQoL in the most affected subdomains, in particular for children presenting with seizures and cognitive dysfunction.

     

Health-related quality of life in children with cerebral palsy and their families

Objective

To determine the health-related quality of life in children with cerebral palsy and their families.

Methods

One hundred children (3–10 years of age) receiving regular rehabilitation therapy for cerebral palsy for last 1 year at a Child Development Centrer were enrolled and the Lifestyle assessment questionnaire — cerebral palsy was administered to the parents.

Results

9% had good, 24% had mildly-affected, 37% had moderately-affected and 30% had severely-affected healthrelated quality of life. The physical independence, mobility and social integration dimensions were much more severely affected than the clinical burden, economic burden and schooling dimensions.

Conclusion

Health-related quality of child is affected in most children with cerebral palsy.     

Health-related quality of life in children born with congenital diaphragmatic hernia

Purpose

The aim of this study was to examine health-related quality of life (HRQoL) in children born with congenital diaphragmatic hernia (CDH).

Methods

Between 1993 and 2003, a total of 102 children born with CDH were treated at Astrid Lindgren Children’s hospital in Stockholm. In 2012, long-term survivors (n?=?77) were asked to participate in the present study, which resulted in a 46% (n?=?35) response rate. The KIDSCREEN-52 questionnaire was used for measuring HRQoL and a detailed review of medical records was performed.

Results

The study participants did not differ from the non-participants in terms of prenatal diagnosis, gender, side of lesion, method of surgical repair, time to intubation, need for ECMO support, or way of discharge from the hospital. Children born with CDH considered themselves to have a good HRQoL, as good as healthy Swedish children. There were only a few significant HRQoL differences within the group of children with CDH, although several median scores in ECMO-treated patients were somewhat lower. Correlations between child and parent scores on HRQoL were low.

Conclusions

Health-related quality of life in children born with CDH is good overall, however, a correlation between the severity of the malformation and HRQoL cannot be excluded.

     

Health-related quality of life in Egyptian children and adolescents with hemophilia A

Quality of life (QoL) in hemophilia is an important area in hemophilia outcome assessment. The Haemo-QoL instrument is a set of questionnaires to measure QoL in those children. The objectives of this study was to assess health-related quality of life (HRQoL) in Egyptian hemophilic children and adolescents using an Arabic version of the Haemo-QoL questionnaire. Sixty patients with severe hemophilia A were recruited from 2 hemophilia treating centers in Egypt. Assessment of quality of life was done using the Haemo-QoL questionnaire. The scores of HRQoL were found to be for all dimensions widely above 50. It was highly significant in the 3 dimensions (physical health-family-treatment) in different age groups, but it was impaired in the dimension of "physical health" for 2 groups, and in the dimension of "family" for the oldest group, whereas the youngest group had highly impaired scores concerning the "treatment." The HRQoL in this study was not affected by the presence of factor VIII (FVIII) inhibitors. The QoL in hemophilic patients in Egypt needs strenuous efforts from hemophilia care-integrated teams of pediatric hematologists and psychiatrists in order to properly assess and improve QoL.     

Health-related quality of life among children with cancer in Hyderabad,India

Objective  

Health-related quality of life (HRQL) experienced by children with cancer is more important than ever before as survival rates are increasing. The aim of this study was to assess the HRQL of children with cancer in a developing country, using physician proxy assessments.     

Health-related quality of life and neurodevelopmental outcomes among children with intestinal failure

Treatment results of pediatric intestinal failure have improved markedly during the last decades. With improved survival the attention is turning to other essential outcomes including quality of life and neurodevelopment. So far, relatively few studies with limited number of patients and variable methodology have addressed these issues. Based on these studies using generic health related quality of life tools, children with intestinal failure demonstrate decreased physical health, while PN-dependence is also associated with compromised emotional functioning. Impairments of social functioning are frequently observed among older children and parents. Few recent studies on neurodevelopment imply significant impairments in motor and mental skills among children with intestinal failure despite small sample sizes and limited follow-up times. Development of a disease-specific survey designed for the pediatric intestinal failure population could better reveal the health issues with greatest impact on quality of life. Robust studies with appropriate methodology on neurodevelopment in pediatric intestinal failure with extended follow-up times are urgently needed. Quality of life and neurodevelopment requires greater attention from medical professionals managing children with intestinal failure.     

儿童恶性肿瘤幸存者健康相关生活质量研究进展

随着医疗科学技术的发展,儿童恶性肿瘤的幸存者越来越多,幸存者的健康相关生活质量(HRQoL)逐渐成为关注的焦点。儿童恶性肿瘤幸存者除了要承受疾病带来的生理、心理、社会的压力,还将要面对由治疗带来的生长发育障碍、性腺功能障碍及癌症远期效应,如脏器功能损害和二次肿瘤。大量研究显示,恶性肿瘤HRQoL显著低于健康人群,但也有少量的研究认为癌症经历对幸存者的生活质量可能有积极的影响。目前HRQoL的评价主要通过生活质量量表完成,后者可以从躯体健康、心理健康、社会健康和精神健康等方面进行综合评价。期待更多的观察性、横断面、尤其是长期随访研究来进一步描述儿童恶性肿瘤幸存者的HRQoL,这样可以为更多的干预研究提供基础,更为临床工作者的临床决策提供循证依据。     

Health-related quality of life: changes in children undergoing chemotherapy

BACKGROUND: Information regarding changes in the health-related quality of life (HRQL) of children during chemotherapy is scarce. Furthermore, there exists a general lack of consensus as to which measures are best suited to assess changes in HRQL in this population. The purpose of this study is to compare the responsiveness of 3 pediatric HRQL measures: the Pediatric Quality of Life Inventory (PedsQL), the Child's Health Questionnaire (CHQ), and the Health Utilities Index (HUI). METHODS: Consecutive pediatric oncology patients and their parents completed the questionnaires at 1-week intervals for a total of 4 weeks, starting on the third day of the patient's chemotherapy treatment cycle. RESULTS: Twenty-nine patients were enrolled with the majority (62%) having a diagnosis of leukemia with an average age of 9 years. The parent proxy reports from time 1 to 4 showed a mean change in the PedsQL of 17 for the generic core scale and 12 for the cancer specific module. The mean change in CHQ physical functioning scale was 6, while the psychosocial scale was only 2, while the HUI 2 was 3 (x100), and HUI 3 was 4 (x100). There was significantly more change in the PedsQL generic scores when compared with the HUI 2 and 3 and the CHQ psychosocial scale (P<0.01). CONCLUSIONS: When measuring HRQL repeatedly in a heterogeneous population, the PedsQL is the measure most responsive to change.     

Health-related quality of life in intersexuality

Standardized methods for the assessment of health-related quality of life in patients with intersexuality are yet to be developed. This paper makes recommendations as to the kind of measures that should be included.     

Health-related quality of life in intersexuality

Meyer-Bahlburg HFL. Health-related quality of life in intersexuality. Acta Pædiatr 1999; Suppl 428: 114–15. Stockholm. ISSN 0803–5326
Standardized methods for the assessment of health-related quality of life in patients with intersexuality are yet to be developed. This paper makes recommendations as to the kind of measures that should be included. □ Gender role/identity, health-related quality of life, intersexuality, sexuality     

Health-related quality of life in children with disorders of sex development (DSD)

Disorders of sex development (DSD) are rare genetic conditions resulting in atypical development of the sex organs. While some evidence is available on psychosexual outcomes, much less is known about the quality of life in this population, especially in children. Health-related quality of life (HRQOL) is a widely accepted endpoint for assessment and evaluation of interventions and medical care. Within the German DSD Network study, 86 children aged 8–12 years with several subtypes of DSD were recruited from Germany, Austria and Switzerland. Demographic, medical and psychosocial variables were collected through interviews of the attending physicians, the children and the parents. HRQOL was the primary outcome. It was assessed by the KINDL-R Questionnaire [2001]. Psychosexual determinants included gender identity/gender dysphoria, gender role behaviour, the child’s knowledge about the condition and number/timing of genital surgery. A significant reduction of HRQOL was reported in children’s self-report (p?<?0.001), in particular in the area of self-esteem (p?<?0.001), physical well-being (p?<?0.01) and school functioning (p?<?0.05). Girls with congenital adrenal hyperplasia who experienced gender dysphoria reported lower HRQOL scores compared to the study group at large. Atypical gender role behaviour was not associated with HRQOL. Conclusion: Psychosocial support of children with DSD and their families appears to be necessary in at least some cases and must be accessible for all patients.     

Sexual function of young women with myelomeningocele

ObjectivesTo assess the sexual function of young women with spina bifida and myelomeningocele and to determine the factors influencing their sexual function.MethodsA postal cross-sectional study using a self-administered questionnaire was performed in 44 women, mean age 27.66 ± 5.89 years, with spina bifida and myelomeningocele. The questionnaire included the Brief Index of Sexual Functioning for Women and questions about voiding mode, urinary symptoms, socioeconomic status, education level, lifestyle, and partnership. In parallel, data were also collected from the paediatric surgery records of patients who returned the questionnaire.ResultsThe response rate was 56.8% (25/44). All domains of female sexual function (thoughts/desires, arousal, frequency of sexual activity, receptivity/initiation, pleasure/orgasm, relationship satisfaction) were altered. Urinary incontinence was likely to be the main factor responsible for altered sexual function and was associated with lower thoughts/desires, arousal, and receptivity/initiation scores. Wearing pads also constituted a limitation to achieving intimacy.ConclusionsYoung myelomeningocele women report poor sexual functioning. The presence of urinary incontinence is associated with lower thoughts/desire, arousal, and receptivity/initiation.