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 of patients with genetic skeletal dysplasias

While medical knowledge on genetic skeletal dysplasias has expanded, very little is known about the patients' health-related quality of life (HRQOL). To test the validity and sensitivity of the fifteen and sixteen dimensional measures of HRQOL in patients with chondrodysplasias, we examined 121 adults aged 16–54 and 19 adolescents aged 12–15 with achondroplasia, cartilage-hair hypoplasia and diastrophic dysplasia. The age and sex standardized overall HRQOL score of the adult patients (0.885 on a scale from 0 to 1) was significantly lower than that of normal controls (0.928) (P < 0.001). The difference in the HRQOL between the patients and the controls consisted mainly of the differences in dimensions of mobility, usual activities, sexual activity and discomfort, for which the patients reported a significantly lower status (more problems) than the controls (P < 0.001). Adolescent patients demonstrated HRQOL profiles similar to those of adults. However, age-specific problems occurred on the dimensions of school and hobbies, friends and physical appearance (P < 0.05). In a detailed examination of different patient groups, some clinically unexpected findings were observed, suggesting that denial is at least in part involved. Conclusion Subjective assessment of HRQOL of patients with chondrodysplasias is valuable for provision of optimal care. In treatment and management special attention should be paid to those dimensions on which the patients' perceived status is currently suboptimal, especially during adolescence which probably is the most critical period in the lives of these individuals. Received: 7 April 1997 / Accepted in revised form: 31 July 1997     

Pediatric health-related quality of life after intestinal transplantation

As outcomes after ITx improve, greater emphasis is needed on HRQOL. The primary aims of this study were to (i) assess the feasibility of measuring HRQOL in pediatric ITx recipients, (ii) measure HRQOL using validated instruments, and (iii) compare HRQOL in ITx recipients to healthy normal (NL) children. The CHQ and Pediatric Quality of Life (PedsQL4.0) instruments were administered to both patients and parents at outpatient visits. All 24 eligible patients were enrolled. The median age at study enrollment was 6.0 yr (range: 2-18 yr), and the median time from transplant to study enrollment was 2.8 yr (range: 0.5-11.8 yr). The majority of subjects were male (58%), Latino (58%), and liver-inclusive (92%) recipients. For CHQ and PedsQL4.0, parental responses were significantly lower in multiple categories including physical health and social functioning compared to healthy norms. Patient responses were not different from NL using CHQ but using PedsQL4.0 were significantly lower in the school functioning subcategory and psychosocial health summary score. HRQOL as reported by children and families after ITx is significantly lower in multiple categories compared to NL.     

Health related quality of life in survivors of pneumococcal meningitis

Aim: To estimate the overall long-term health related quality of life implications of an episode of pneumococcal meningitis in childhood.
Method: Cases were identified through two regional UK surveillance studies and traced via their general practitioners (GPs) or local hospital paediatrician. Siblings were used as controls where available. Health related quality of life was assessed using the health utilities index (HUI). Mean utility scores were compared between cases and controls and univariate linear regression was used to identify factors that influenced the overall utility scores.
Results: HUI data were available for 71 cases and 66 controls. The mean overall utility score for cases 0.774 (95% CI 0.711– 0.837) was significantly lower than for controls 0.866 (95% CI 0.824–0.907) (p-value = 0.0185). Hearing was the most significantly affected health attribute (p-value < 0.006). In cases, males had lower quality of life scores than females (p-value = 0.018), however this was not seen in controls.
Conclusion: An episode of pneumococcal meningitis results in a long-term decrement in overall health related quality of life and is significantly related to hearing loss.     

Health-related quality of life in overweight and nonoverweight black and white adolescents

OBJECTIVES: To assess the impact of obesity on quality of life (QOL) in black and white adolescents. STUDY DESIGN: One hundred ten overweight (body mass index [BMI], 41.7 +/- 8.9 kg/m2) and 34 nonoverweight adolescents (BMI, 20.6 +/- 2.9 kg/m2) and their parents completed measures of QOL. RESULTS: Overweight was associated with poorer adolescent-reported QOL and parent reports of their children's QOL. Examining groups by weight status and race, overweight whites reported the greatest impairment on Social/Interpersonal, Self-Esteem, and Physical Appearance QOL (all P < .01), whereas parents of overweight blacks reported the poorest General Health Perceptions scores regarding their children. Interactions between BMI z-score and race were detected for Social/Interpersonal, Self-esteem, Daily Living, Self-Efficacy, Self-regard, and Physical Appearance QOL (all P < .05): Higher BMI in whites was associated with greater impairments in QOL than in blacks. Parents reported similar relations for their children. CONCLUSIONS: According to adolescent and parent reports, overweight is associated with poorer QOL in adolescence, regardless of race; however, compared with overweight white adolescents, blacks report less impairment in QOL. Future research is required to determine whether differences in QOL are predictive of treatment success.     

Surgical complications and outcome of paediatric liver transplantation: the Singapore experience

The purpose of this study was to analyse the early and late results of paediatric liver transplantation (LT), with particular reference to complications that required surgical intervention. The charts of all children who underwent LT between 1990 and 2002 were reviewed retrospectively. Results were analysed with a minimum follow up of 9 months. Thirty-five children have undergone 38 LTs; 22 received grafts from their parents, 16 received cadaveric organs and three children had retransplantation. The ages of the children ranged from 12 to 168 months. Biliary atresia was the most frequent indication for transplant (n=27). Twenty-seven children had complications that required surgical or radiological interventional procedures. Vascular complications included hepatic artery thrombosis (n=2), hepatic vein (HV) thrombosis (n=1), and the majority being portal vein thrombosis (n=6). Bile leaks were observed in eight children. Other complications included intestinal perforation (n=2), intra-abdominal abscesses (n=1), wound dehiscence (n=2), post-operative bleed (n=2), intestinal obstruction (n=2), ventral hernia (n=1), and multiple abdominal wound sinuses (n=1). Three children underwent retransplantation, two for hepatic artery thrombosis with multiple episodes of cholangitis and intrahepatic biliomas and the third was done for hepatic vein thrombosis. Patient and graft survival at 1 year is 81.5 and 74.2%, respectively. Paediatric LT is associated with significant morbidity, the main complications being vascular and biliary. The article was presented at the 8th Congress of the Asian Society of Transplanatation (8th CAST) in September 2003 held in Kuala Lumpur, Malaysia.     

Outcome of paediatric intensive care survivors

The development of paediatric intensive care has contributed to the improved survival of critically ill children. Physical and psychological sequelae and consequences for quality of life (QoL) in survivors might be significant, as has been determined in adult intensive care unit (ICU) survivors. Awareness of sequelae due to the original illness and its treatment may result in changes in treatment and support during and after the acute phase. To determine the current knowledge on physical and psychological sequelae and the quality of life in survivors of paediatric intensive care, we undertook a computerised comprehensive search of online databases for studies reporting sequelae in survivors of paediatric intensive care. Studies reporting sequelae in paediatric survivors of cardiothoracic surgery and trauma were excluded, as were studies reporting only mortality. All other studies reporting aspects of physical and psychological sequelae were analysed. Twenty-seven studies consisting of 3,444 survivors met the selection criteria. Distinct physical and psychological sequelae in patients have been determined and seemed to interfere with quality of life. Psychological sequelae in parents seem to be common. Small numbers, methodological limitations and quantitative and qualitative heterogeneity hamper the interpretation of data. We conclude that paediatric intensive care survivors and their parents have physical and psychological sequelae affecting quality of life. Further well-designed prospective studies evaluating sequelae of the original illness and its treatment are warranted.     

Health‐related quality of life among siblings of kidney transplant recipients

Studies are increasingly recognizing health‐related quality of life (HRQOL) as a key pediatric outcome in both clinical and research settings and an essential health outcome measure to assess the effectiveness of medical treatment. However, it has not yet been studied among the healthy siblings of kidney transplant recipients. The aim of this study, therefore, is to examine HRQOL among this population. We asked the following three groups to complete a validated measure of HRQOL among children (KIDSCREEN‐52 ) : siblings of children who had received kidney transplants (n = 50), kidney transplant recipients (n = 43), and a healthy control group (n = 84). We found that siblings of kidney transplant patients exhibited lower scores for financial resources and autonomy than kidney transplant recipients. They also served lower on physical well‐being, financial resources, autonomy, and parent relations/home life than the control group. However, they scored higher on social acceptance than kidney transplant recipients. Our study underscores the importance of assessing HRQOL in families including a child diagnosed with a chronic illness. Siblings require social and psychological support to promote coping and adaptation.     

Health-related quality of life in men with corrected hypospadias: An explorative study

ObjectiveThe aim of the study was to evaluate health-related quality of life (HRQoL) of men with a corrected hypospadias in comparison to circumcised controls. Furthermore, determinants of HRQoL were examined.Patients and methodsIn a cross-sectional study, HRQoL of 45 men with corrected hypospadias (mean age: 26.2 years +/− 5.1) was compared with a control group of 46 circumcised men (mean age: 25.5 years +/− 4.9). Participants answered three questionnaires: The Medical Outcomes Study Short Form-36 item questionnaire (SF-36), the International Index of Erectile Function (IIEF), and the Penile Perception Score (PPS).ResultsPhysical and mental dimensions of HRQoL were not impaired among men with corrected hypospadias if compared to circumcised men. Neither socio-demographic and medical variables nor erectile function (EF) predicted HRQoL. However, a negative genital self-perception (PPS) and a lower orgasmic function (OF) were risk factors for an impaired mental HRQoL.ConclusionsOur data suggest that the HRQoL of adult hypospadias patients is comparable to that of circumcised men. However, patients should be supported in developing a positive genital self-perception, because poor genital self-perception correlated with an impaired mental HRQoL. Since studies with non-operated men suggest that some adapt well to their penile condition, further studies should also include non-operated hypospadias.     

Psychological effects of liver disease and transplantation

Psychological adjustment in children with liver disease was investigated. Three groups of children 6–15 years old participated: ten had undergone a liver transplant (Gp1), 15 had ongoing chronic liver disease (Gp2) and 15 were healthy controls (Gp3). Children who had had a transplant appeared well adjusted and thought of themselves as healthy rather than ill, although areas of vulnerability were present, for example increased anxiety. No differences emerged in terms of coping with common or illness-related problems or understanding of the causes of illness and use of medication. Gps 1 and 2 showed higher levels of understanding of the functions of the liver but less understanding of illness prevention when compared to their healthy peers. Gp2 experienced less control over their health when compared to the other two groups. Gp1 rated themselves as more “healthy” than Gp2 but less so than Gp3. Conclusion Children with chronic liver disease are able to communicate how they deal with the stresses of the condition. Though well adjusted in many ways, those who have had a transplant still show areas of psychological vulnerability which need to be addressed in clinical practice. Received: 6 January 1997 / Accepted in revised form: 5 January 1998     

Bocavirus infection following paediatric liver transplantation

HBoV is an emergent virus, which is frequently detected as a co‐infective agent. However, it can cause disease on its own. It is associated with respiratory and diarrhoeal illness in children and adults, whether immunocompetent or immunocompromised. We report HBoV infection in a child post‐liver transplantation, who presented with persistent fever and mild tachypnea, 3 weeks after a successful transplant. She recovered spontaneously with no graft dysfunction.     

Health-related quality of life in paediatric patients with inflammatory bowel disease related to disease activity

Aim: Impaired health‐related quality of life (HRQoL) and an increased risk of psychosocial problems may encounter children and adolescents with inflammatory bowel disease (IBD). Generic HRQoL questionnaires, 15D designed for subjects over 16 years of age, 16D for adolescents aged 12–15 and 17D for younger children, allow comparison to healthy peers and have not been used in children with IBD before. Further, in paediatric IBD patients, HRQoL has not been related to disease activity. We evaluated the applicability of 15D, 16D and 17D questionnaires in the paediatric IBD population and examined how HRQoL is influenced by changes in clinical activity of IBD. Methods: The study subjects recruited at their scheduled, routine appointment in the outpatient clinic of the children's hospital completed the HRQoL questionnaire at baseline and again after 3–5 months. Disease activity was estimated by a three‐level scale. The HRQoL of the study population was compared with that of the age‐standardised general population. Results: Fifty‐five children, aged 7–19 years, were recruited. The HRQoL scores strongly correlated with the activity of the disease (P < 0.001). The two oldest age groups with IBD had lower HRQoL scores than age‐standardised peers (P= 0.001/0.04). There was no gender difference in HRQoL scores. Conclusions: IBD has a considerable impact on the HRQoL of children and adolescents. The generic HRQoL instruments used appeared to be promising tools for examining HRQoL in paediatric IBD patients in different age groups, but larger studies to establish their usefulness in the follow‐up of young patients are still warranted.     

Physical activity and health related quality of life in children following kidney transplantation

Abstract:  Participation in PA is often diminished in children with CKD. Limited research exists on exercise tolerance/capacity but no studies to date have investigated lifestyle PA and its determinants in these children. The aim of this study was to investigate level of PA and potential physiological and psychological associations in a group of pediatric KTx recipients compared with CS. Twenty KTx and 33 CS participated. PA was measured by PAQ. HRQOL (PedsQL 4.0) and CY-PSPP were also measured. BMI and WC was recorded in all subjects; GFR, BP and immunosuppressants in KTx. Body measurements indicated the two groups were similar: 25% KTx and 24% CS had BMI >85th percentile. KTx were less physically active than CS in total exercise minutes (p = 0.005). CS reported higher HRQOL than KTx (p = 0.001). Higher perceptions of HRQOL were significantly correlated with higher number of steps/day in both groups (p = 0.034). KTx showed significantly lower perceptions of sports competence (p = 0.007) and physical conditioning (p = 0.001) than CS. Higher PAQ activity scores were significantly correlated with higher perceptions of body attractiveness (p = 0.019), Sport (p = 0.003) and Conditioning (p = 0.001). These results suggest that PA may play a role in overall well-being and HRQOL in KTx.     

The association between urinary continence and quality of life in paediatric patients with spina bifida and tethered cord

OBJECTIVE:

To determine the association between urinary continence and quality of life (QoL) in a paediatric spina bifida population.

METHODS:

After appropriate ethics approval, a prospective study was initiated using multiple validated QoL instruments that were distributed to patients as they presented for their annual appointment at the Northern Alberta Spina Bifida Clinic (Edmonton, Alberta). General demographic information was collected and validated questionnaires were used. The survey package included two instruments to assess overall QoL: Global Pediatric QoL (PedsQL 4.0) and Health Specific QoL-Spina Bifida (HRQoL-SB). Two instruments were also included to quantify urinary symptoms and assess urinary specific QoL: the Urinary Incontinence Severity Index – Pediatric (ISI-P) and Urinary Specific QoL (PinQ).

RESULTS:

A total of 71 patients were enrolled in the study. The general QoL (PedsQL 4.0) and health-specific QoL (HRQoL-SB) scores for the population indicated an overall QoL of 66% (n=69) and 83% (n=67), respectively. Approximately 46% (33 of 71) reported >1 episode of urinary incontinence per week. Urinary continence was associated with a significantly higher urinary-specific QoL (PinQ; P<0.001), general QoL (PedsQL 4.0; P<0.05) and health-specific QoL (HRQoL-SB; P<0.05). Furthermore, urinary incontinence and its effect on QoL was not influenced by the presence of a shunt, level of the lesion or manner of dysraphism.

CONCLUSION:

These data suggest that QoL in patients with spina bifida is related to urinary continence. This effect appears to be independent of the type and level of the spinal dysraphism and the presence or absence of a shunt.     

Health-related quality of life in sickle cell disease

Advances in drug therapy, hematopoietic stem cell transplantation, and technology have improved the morbidity and survival for those with sickle cell disease. The effect of this modern therapy on the health-related quality of life (HRQL) of those with sickle cell disease is not known. HRQL provides an assessment of how an illness, its complications, and its treatment are experienced by a patient. This review will examine prior work in HRQL in sickle cell disease and the rationale for utilizing HRQL as an outcome to measure impact of treatment. In addition, issues to consider when reporting HRQL will be presented.     

Health-related quality of life in pediatric liver transplant recipients compared with other chronic disease groups

Limbers CA, Neighbors K, Martz K, Bucuvalas JC, Webb T, Varni JW, Alonso EM, on behalf of the Studies of Pediatric Liver Transplantation (SPLIT) Functional Outcomes Group (FOG). Health‐related quality of life in pediatric liver transplant recipients compared with other chronic disease groups.
Pediatr Transplantation 2011: 15: 245–253. © 2010 John Wiley & Sons A/S. Abstract: This cross‐sectional, multicenter cohort study compares the level of HRQOL of pediatric LT recipients to children with other chronic health conditions. LT sample included 873 children who survived at least 12 months following LT. Six chronic disease samples were compiled from numerous studies, including over 800 patients with JRA, type 1 diabetes, cancer in remission, cardiac disease, end‐stage renal disease, and inflammatory bowel disease. Generic HRQOL was measured from both the parental and patient perspective using the PedsQL? 4.0 Generic Core Scales. Pediatric LT patients reported better physical health than children with JRA. According to parents, pediatric LT recipients had better HRQOL than children on renal dialysis on all domains except school functioning. Across all domains but emotional functioning, pediatric LT recipients reported significantly lower HRQOL than children with type 1 diabetes. Overall, pediatric LT patients reported HRQOL comparable to that of children who had undergone renal transplantation and patients with cancer in remission. Pediatric LT patients manifested impaired HRQOL similar to that of children with chronic diseases and these data suggest that they face ongoing challenges that warrant monitoring and indicate a need for interventions to improve their HRQOL.     

儿童肝移植术后的随访与治疗

目的评估复旦大学附属儿科医院肝移植患儿术后疗效。方法回顾性分析2002年10月至2005年5月期间8例小儿肝移植术后的临床效果和生活质量。本组男4例,女4例,肝移植年龄4—67个月,体重6—19kg。6例亲体活体肝移植,1例劈裂式肝移植,1例减体积肝移植。结果围手术期死亡1例,手术存活率87、5％;手术成功患儿中1例于术后8个月死于消化道出血,1年生存率为75．O％,其他6例存活至今,最长生存期43个月。术后并发症：急性排斥反应2例;远期感染：巨细胞病毒感染2例,Epstein—Barr病毒感染1例,乙型肝炎病毒感染1例;低蛋白血症3例。远期外科并发症：门静脉血栓1例,下腔静脉和肝静脉狭窄1例。他克莫司（FK506）的副作用：高血压3例,肾脏损害1例,肝脏损害1例,腹泻1例。所有存活患儿无心理异常;1例术前体格发育落后者,术后发生了生长追赶。结论肝移植改善了小儿终末期肝病的预后。儿童肝移植后并发症多见。周密的术后随访与管理有助于发现各种内外科并发症和保证术后生活质量。