Life In Pediatric Patients Research Articles

Quality of Life in Pediatric Patients with Minimal Change Disease

This cross-sectional comparative study was conducted in Tanta University Hospital (TUH), Pediatric nephrology inpatient unit or outpatient clinic between October 2016 and October 2017. In this questionnaire-based study, 100 children with primary idiopathic NS and an equal number of age-matched controls with other chronic diseases were included.

Bleeding after tonsillectomy

Oropharyngeal hemorrhage after tonsillectomy is one of the most common postprocedural ENT emergencies that require immediate attention. Management choices depend on the severity of bleeding, underlying platelet or coagulation disorders, and the patient’s maturity and age. Treatment range from simple observation to an emergent need to both secure the airway and stop the hemorrhage. The workup of the hemorrhagic patient will be discussed from the preoperative to postoperative time period. Surgical techniques in response to various acuity will be discussed to fully elucidate options to control the hemorrhage.

Health-Related Quality of Life in Pediatric Patients With Demyelinating Diseases: Relevance of Disability, Relapsing Presentation, and Fatigue.

Decreased health-related quality of life (HRQOL) in pediatric patients with multiple sclerosis is established, but little research has examined HRQOL in the broader pediatric demyelinating disease population, and predictors of reduced HRQOL are largely unexplored. We sought to (1) compare generic HRQOL and fatigue of pediatric patients with relapsing (i.e., multiple sclerosis and neuromyelitis optica) versus monophasic demyelinating diseases (i.e., acute disseminated encephalomyelitis, optic neuritis, transverse myelitis, clinically isolated syndrome) and (2) examine the extent to which disability, relapsing disease, and fatigue predict HRQOL. Child and/or parent-proxy reports of generic and fatigue-related HRQOL were collected for 64 pediatric patients with demyelinating diseases. HRQOL of the sample was compared with published healthy child norms. Independent samples t-tests compared HRQOL and fatigue for children with monophasic versus relapsing diseases. Regression analyses examined disability, disease presentation, and fatigue as potential predictors of HRQOL. Compared with healthy child norms, generic HRQOL was significantly lower for the demyelinating disorder group, for both child and parent reports across multiple domains. As hypothesized, the relapsing disease group reported lower overall HRQOL and more fatigue than the monophasic group. Disability and relapsing disease predicted lower HRQOL for both parents and children, whereas fatigue was only predictive per the child perspective. Children with demyelinating diseases evidence significantly lower HRQOL than healthy peers, supporting need for intervention. Those with relapsing disease appear particularly at risk; targeting disability and fatigue may be fruitful areas for intervention.

Health-related quality of life in paediatric patients with congenital heart defects: association with the type of heart defect and the surgical technique.

Patients who had received corrective surgery of several congenital heart defects (surgical VSD closure, Fallot, TGA after atrial or arterial switch or Fontan-type circulation for univentricular AV-connection) were interviewed in the office of their home peadiatric cardiologist. HRQoL in children along 7 dimensions was assessed using a standardised questionnaire (PEDQoL); information on the medical case history of each respondent was also collected. HRQoL was assessed in the questionnaire by asking about the frequency (never, rarely, often, always) of specific negative experiences; more frequent experiences indicate a lower quality of life. Frequency expressions were transformed into numerical values (25, 50, 75, 100%), and mean values for HRQoL were calculated for each patient and for each domain. Differences in HRQoL among patients with different types of interventions were analysed using the Mann-Whitney Test or the Kruskal-Wallis Test as appropriate; p values<0.05 were considered to indicate significant differences, while p values<0.1 were considered to indicate notable trends. Patients: 169 patients (60% male, 40% female) were part of the study. The mean age was 11.6years; 50 patients had surgical VSD closure, 52 surgeries for Tetralogy of Fallot (22 transannular patch, 18 no transannular patch, 12 inaccurate description), 40 had complete transposition of the great arteries (28 atrial switch, 12 arterial switch), 22 had a Fontan-type procedure for univentricular AV-connection. HRQoL differed little among patients with different CHDs for the items "relation to friends," "interactions in the affected families", and "own body image". For other items, notable differences emerged: patients with univentricular hearts rated their physical capacity worse and showed a tendency towards negative ratings in other domains. On the other hand, patients after Fallot or TGA correction tended to rate their HRQoL in several domains as relatively high. Focusing on the mode of surgery for Fallot repair, respectively, TGA correction the only significant difference was found for "physical capacity" in TGA (atrial vs. arterial repair). Mustard patients tended to rate most items worse. Physical capacity was rated worst by patients with a Fontan circulation. Repeated surgery led to lower ratings for all domains except "physical capacity" and "body image". Different surgical techniques for CHD do not affect children's and adolescents' self-reported HRQoL to the extent that one would expect. This observation is in line with observations in groups of children with different chronic diseases. Specialised psychosocial support is necessary in order to maintain this positive self-evaluation and to ensure patients are able to lead autonomous personal and professional lives.

Impact of the implantation of the vagus nerve stimulator on the quality of life and the reduction of epileptic seizures in Spanish pediatric population

Introduction: Epilepsies in the pediatric age become refractory to pharmacological treatment in a third of cases. Implantation of the vagus nerve stimulator (VNS) is a palliative option for patients who are not candidates for resective surgery. Objective: To describe the impact of the implantation of VNS on the reduction of the crisis and quality of life in pediatric patients with drug-refractory epilepsies. Methods: A descriptive, retrospective study was carried out after reviewing the medical records of 48 patients who were implanted with VNS between 2008 and 2016. The quality of life was assessed through the scale of Quality of life of the child with epilepsy (CAVE) validated for the Spanish population through phone survey. Results: We studied 18 girls (38%) and 30 children (62%). The median age at onset of the crisis was 1.5 years (range 1 – 12 years). The mean age of implantation of VNS was 9.8 years (range: 3 months to 21.9 years). The mean follow-up age was 2.75 years (range: 9 months to 7.5 years). The overall response rate (reduction of more than 50% of seizures) is maintained between 48–52% in the different periods evaluated post-implantation. 57% of families rated the effect of VNS on the patient's quality of life as good or very good. Conclusions: The overall response rate remains above 45% in the first two post-implantation years. More than 50% of families rated the effect of VNS on quality of life as good or very good.

Health-related Quality of Life in Pediatric Patients With Chronic Hepatitis B Living in the United States and Canada.

The aim of the study was to determine whether selected sociodemographic and hepatitis B virus (HBV)-specific clinical factors are associated with health-related quality of life (HRQoL) among pediatric patients chronically infected with HBV. Children with chronic HBV enrolled in the Hepatitis B Research Network completed the Child Health Questionnaire at study entry. Caregivers of children 5 to <10 years completed the parent-reported form (CHQ-Parent Report Form); youth 10 to <18 years completed the child-reported CHQ-Child Report Form. We examined univariable associations of the Child Health Questionnaire scores with selected independent variables: sex, adoption status, maternal education, alanine aminotransferase (U/L), aspartate aminotransferase-to-platelet ratio index, and HBV-specific symptom count. A total of 244 participants (83 young children 5-<10 years, 161 youth 10-<18 years) were included, all HBV treatment-naïve. Among young children, increased alanine aminotransferase level was negatively associated with CHQ-Parent Report Form psychosocial summary t score (r = -0.28, P = 0.01). No other subscale comparisons for young children were statistically significant. Among youth, adoption was associated with better physical functioning and general health (P < 0.01). Higher maternal education was associated with better role/functioning-physical and -emotional scores (P < 0.05). Maternal education and adoption status were linked with adoption associated with higher maternal education. Increased symptom count in youth was associated with worse HRQoL in subscales measuring bodily pain, behavior, mental health, and self-esteem (P < 0.01). Although overall HRQoL is preserved in children with chronic HBV, some sociodemographic and HBV-related clinical factors were associated with impaired HRQoL in our pediatric patients at baseline. Measurement of HRQoL can focus resources on education and psychosocial support in children and families most in need.

Quality of Life in Pediatric Patients with Allergic Rhinitis treated at the Medical Clinic of Integrated Education - Unisul.

Introduction Allergic rhinitis is a common disease among children and adolescents, reaching up to 40% of the population. During childhood, it is usually underdiagnosed because it has nonspecific symptoms. It has a negative impact on quality of life and may predispose to comorbidities. The diagnosis is clinical and treatment aims prevention. Objective The objective of this study is to evaluate the quality of life in pediatric patients with allergic rhinitis. Methods This is an observational study with cross-sectional design. The population consisted of pediatric patients with allergic rhinitis treated at the Medical Clinic of Integrated Education (MCIE) – Universidade do Sul de Santa Catarina - Unisul, Tubarão, SC, Brazil. We collected data from March to June 2016 through the application of the Sociodemographic and Health Questionnaire, rhinitis module of the International Study of Asthma and Allergies in Childhood Questionnaire and the Rhinoconjunctivitis Quality of Life Questionnaire Modified. Results Out of the 69 respondents, 52.2% were boys with a mean age of 10.13 years old. The predominant education level of parents/guardians was incomplete second grade and average income level was two minimum wages. 81.2% said they had previous treatment for AR, 30.4% had asthma and 7.2% eczema. Incidence of patients smoking was absent and family (parents/guardians) smoking was 17.4%. March to July were the months of highest symptom occurrence, slightly disturbing daily activities. The mean value of severity was 51.9, nasal symptoms were the most uncomfortable, and nasal itchiness was the most cited. Conclusion Our results highlight that allergic symptoms negatively impact the life of people with allergic rhinitis, with a predominance of nasal symptoms, especially nasal itchy, representing a poor quality of life of the interviewed.

Biomarkers of Disease Severity Predict Neurocognitive Functioning in Pediatric SCD

Introduction: Research clearly documents the increased risk of silent and overt strokes in pediatric patients with sickle cell disease (SCD), as well as the associated neurocognitive deficits in domains such as working memory (WM) and attention regulation. However, less research has examined the effects of biomarkers of SCD severity, such as cerebral blood flow velocity (CBFV), hemoglobin, and blood pressure, on neurocognitive functioning. This investigation aimed to explore the utility of using biomarkers to predict caregiver-reported neurocognitive and behavioral functioning in pediatric SCD.Methods: Fifty-nine children with SCD ages 7-16 (M = 10.44, SD = 2.87, 42% male) enrolled in a larger study of the feasibility and efficacy of a computerized cognitive training program. As part of this study, a primary caregiver completed the Behavior Rating Inventory of Executive Function (BRIEF) and Conners-3, both caregiver-reported measures of behavioral, attentional, and emotional functioning in children and adolescents. Scores on the BRIEF and Conners-3 are both represented with T scores, where higher scores reflect greater problems. Complete blood count (CBC) and vital signs were extracted from patients' medical charts if results had been documented within 45 days of date caregivers completed the BRIEF and Conners-3. Transcranial Doppler (TCD) ultrasonography results were extracted if a TCD had been performed within 12 months of the testing date.Results: Mean hemoglobin was 9.05 (SD = 1.33), mean hematocrit was 25.84 (SD = 3.71), mean systolic blood pressure was 107.43 (SD = 8.89), and mean diastolic blood pressure was 63.69 (SD = 9.49). TCD results revealed a mean right middle cerebral artery (MCA) CBFV of 206.00 (SD = 21.30), left MCA CBFV of 127.84 (SD = 22.40), right distal cerebral artery (DiCA) CBFV of 86.44 (SD = 23.00), and left DiCA CBFV of100.45 (SD = 27.28). Bivariate Pearson correlations and linear regression analyses were conducted to examine the relationships between biomarkers (e.g., CBFV, blood pressure, CBC results) and caregiver-reported neurocognitive and behavioral functioning. Modest correlations were observed between CBC results (e.g., hemoglobin, hematocrit) and caregiver-rated cognitive and behavioral outcomes; though, these relationships were no longer significant after controlling for age and gender in subsequent regression analyses. However, higher left DiCA velocities significantly predicted more problems on the BRIEF Working Memory and Initiate subdomains (R2 = .569, b = 0.442, p < .001; R2 = .746, b = .466, p < .001, respectively). Additionally, higher left DiCA velocities significantly predicted more caregiver-rated problems on the BRIEF Organization of Materials and Plan/Organize scales (R2= .447, b = 0.191, p = .049; R2 = .377, b = 0.372, p = .00), respectively. TCD and blood pressure results also significantly predicted scores on the caregiver-reported Connors-3 Executive Functioning subscale, with higher left DiCA (R2 = .432, b = 0.446, p = .001) and systolic BP (R2 = .196, b = 0.859,p =.003) predicting more problems with executive functioning.Conclusion: Certain biomarkers of SCD severity appear to have a detectable and direct influence on neurocognitive and behavioral functioning in pediatric SCD. TCD results, specifically left DiCA CBFV, and systolic blood pressure significantly predicted scores on the BRIEF and Conners-3, two caregiver-reported measures of neurocognitive and behavioral functioning. More specifically, higher CBFV and systolic blood pressure were predictive of more caregiver-rated problems with working memory, executive functioning, and organizational skills. These cognitive and behavioral domains are of particular importance in children, who depend on such skills to learn and succeed academically. Progressive disease-related neurocognitive deficits may contribute to long-term difficulties in academic achievement, social functioning, and vocational attainment. Biomarkers of SCD severity, such as CBFV and blood pressure, may be useful indicators of worsening neurocognitive status and signal the need for preventative care to protect neurological integrity and preserve quality of life in pediatric patients with SCD. DisclosuresNo relevant conflicts of interest to declare.

Gastrointestinal symptoms predictors of health-related quality of life in pediatric patients with functional gastrointestinal disorders.

To investigate the patient-reported multidimensional gastrointestinal symptoms predictors of generic health-related quality of life (HRQOL) in pediatric patients with functional gastrointestinal disorders (FGIDs). The Pediatric Quality of Life Inventory™ (PedsQL™) Gastrointestinal Symptoms Scales and PedsQL™ 4.0 Generic Core Scales were completed in a 9-site study by 259 pediatric patients with functional constipation, functional abdominal pain (FAP), or irritable bowel syndrome (IBS). Gastrointestinal Symptoms Scales measuring stomach pain, stomach discomfort when eating, food and drink limits, trouble swallowing, heartburn and reflux, nausea and vomiting, gas and bloating, constipation, blood in poop, and diarrhea were identified as clinically important symptom differentiators from healthy controls based on prior findings, and subsequently tested for bivariate and multivariate linear associations with overall HRQOL. Gastrointestinal symptoms were differentially associated with decreased HRQOL in bivariate analyses for the three FGIDs. In predictive models utilizing hierarchical multiple regression analyses controlling for age, gender, and race/ethnicity, gastrointestinal symptoms differentially accounted for an additional 47, 40, and 60% of the variance in patient-reported HRQOL for functional constipation, FAP, and IBS, respectively, reflecting large effect sizes. Significant individual gastrointestinal symptoms predictors were identified after controlling for the other gastrointestinal symptoms in the FGID-specific predictive models. Gastrointestinal symptoms represent potentially modifiable predictors of generic HRQOL in pediatric patients with FGIDs. Identifying the condition-specific gastrointestinal symptoms that are the most important predictors from the patient perspective facilitates a patient-centered approach to targeted interventions designed to ameliorate impaired overall HRQOL.

Comparison of Patient and Parent-Reported Quality of Life in Pediatric Patients After Radiation Therapy

Comparison of Patient and Parent-Reported Quality of Life in Pediatric Patients After Radiation Therapy

Poster 478 A Review of the Health-Related Quality of Life in Pediatric Patients with Cerebral Palsy and Associated Spasticity

Poster 478 A Review of the Health-Related Quality of Life in Pediatric Patients with Cerebral Palsy and Associated Spasticity

The Discrepancy between Performance-Based Measures and Questionnaires when Assessing Clinical Outcomes and Quality of Life in Pediatric Patients with Neurological Disorders

ABSTRACTIn clinical outcome assessment, the relation between performance-based measures and questionnaire ratings of the same domain is weak, but correlations between questionnaires proposed for the evaluation of different domains are strong. The present study aims to illustrate these phenomena in a group of patients with neurofibromatosis type 1 (NF1) and to propose an explanatory hypothesis. A single neuropsychologist interviewed the parents about the child's situation and current difficulties and then assessed this parental view as overall positive or overall negative. The same assessor then administered the Wechsler Intelligence Scales and neuropsychological tests to 78 children and adolescents with NF1 (5–18 years). Parents then completed the Child Behavioral Checklist (CBCL), the Conners’ Parent Rating Scale, the Behavior Rating Inventory of Executive Function (BRIEF), as well as questionnaires assessing quality of life, impact of the medical disorder, and their own difficulties. All questionnaires were inter-correlated (r = 0.29 – 0.84) and associated with the overall positive or negative parental view of the child's progress (effect size = 0.41–1.46). Conversely, questionnaires were weakly or not significantly related to intelligence, cognitive measures, or clinical severity. In conclusion, the parental view of the child's progress was related to the answers to questionnaires assessing quality of life or strengths and difficulties of patients with a neurological disorder. This factor should be assessed independently and taken into account when assessing clinical outcome.

The influence of itch: Quality of life in pediatric patients with chronic pruritus

The influence of itch: Quality of life in pediatric patients with chronic pruritus

Can early diagnosis and partial meniscectomy improve quality of life in patients with lateral discoid meniscus?

To evaluate clinical and radiographic mid-term outcomes and improvement in quality of life in pediatric patients affected by LDM and who underwent partial or total/subtotal meniscectomy. 28 patients (12 M, 16 F), mean age 8.3 years old (range: 6-13) affected by LDM with symptoms and/or meniscal tears, mean follow up: 2 years and 4 months (range: 24-52 months). Symptoms included: pain, swelling, articular block, limitation to knee extention, meniscal instability and formation of meniscal cysts. 23 patients treated with partial meniscectomy, 5 by total/subtotal meniscectomy. All of them have been clinically evaluated using the Ikeuchi scale and the POSNA questionnaire; for radiological evaluation we used the Tapper-Hoover grading scale. Ikeuchi: In 23 patients underwent partial meniscectomy: 21 excellent and 2 good; in 5 patients underwent total/subtotal meniscectomy: 1 excellent, 2 good and 2 average. POSNA presurgical mean score: partial meniscectomy: 70 (range 66.8-73.6), total/subtotal meniscectomy: 58.7 (56.9-62.2). POSNA presurgical mean score: partial meniscectomy: 92.4 (range 88.7-98), total/subtotal meniscectomy: 81.2 (range 78.3-85.6). Tapper-Hoover: in 23 patients underwent partial meniscectomy: 17 were classified grade I, 5 grade II, 1 grade III; in 5 patients underwent total/subtotal meniscectomy: 2 were classified grade II and 3 grade III. These ones evidenced further signs of chondromalacia on articular surfaces, in the lateral area of knee joint. Significant improvement in quality of life, evidenced in all patients (p = 0.048 in those underwent total/subtotal meniscectomy, p = 0.011 in those underwent partial meniscectomy), but especially in patients who underwent partial meniscectomy with absence of meniscal tears (p = 0.033), supports how important can be early diagnosis of LDM in childhood. We assert that early diagnosis and conservative treatment (partial meniscectomy) could reduce risks in development of chondromalacia, in those patients affected by LDM, because of its association with better clinical, radiographic and in quality of life mid-term results.

Metronomic therapy can increase quality of life during paediatric palliative cancer care, but careful patient selection is essential.

Children with refractory or high-risk malignancies frequently suffer from poor quality of life during palliative care. This study explored the effect of metronomic drug administration on survival and quality of life in paediatric patients with various refractory or high-risk tumours. We treated 17 patients with a maintenance therapy that consisted of metronomic thalidomide, etoposide and celecoxib. The endpoints of the study were overall and progression-free survival, changes in the Karnofsky-Lansky scores from baseline to the end of the study therapy and radiological responses. The median overall survival after the start of the study therapy was 6.2months (range 2.0-57.7), and the six-, 12- and 24-month survival rates were 59%, 18% and 18%, respectively. The median progression-free survival was 3.2months (range 0.3-17.8). The Karnofsky-Lansky scores increased significantly during the study therapy (p=0.02), with 35% of the patients having a transient improvement in their clinical status. Radiologically, one partial response and two disease stabilisations were encountered. Grade III-V adverse events occurred in 76% of the patients. Metronomic therapy may increase the quality of life during palliative care for childhood cancer, but requires careful patient selection to minimise the risk of serious adverse events.

Cross-cultural adaptation and validation of the Sinus and Nasal Quality of Life Survey (SN-5) into Brazilian Portuguese

IntroductionThe concept of quality of life is subjective and variable definition, which depends on the individual's perception of their state of health. Quality of life questionnaires are instruments designed to measure quality of life, but most are developed in a language other than Portuguese. Questionnaires can identify the most important symptoms, focus on consultation, and assist in defining the goals of treatment. Some of these have been validated for the Portuguese language, but none in children. ObjectiveTo validate the translation with cross-cultural adaptation and validation of the Sinus and Nasal Quality of Life Survey (SN-5) into Portuguese. MethodsProspective study of children aged 2–12 years with sinonasal symptoms of over 30 days. The study comprised two stages: (I) translation and cross-cultural adaptation of the SN-5 into Portuguese (SN-5p); and (II) validation of the SN5-p. Statistical analysis was performed to assess internal consistency, test-retest reliability, and sensitivity, as well as construct and discriminant validity and standardization. ResultsThe SN-5 was translated and adapted into Portuguese (SN-5p) and the author of the original version approved the process. Validation was carried out by administration of the SN-5p to 51 pediatric patients with sinonasal complaints (mean age, 5.8±2.5 years; range, 2–12 years). The questionnaire exhibited adequate construct validity (0.62, p<0.01), internal consistency (Cronbach's alpha=0.73), and discriminant validity (p<0.01), as well as good test–retest reproducibility (Goodman–Kruskal gamma=0.957, p<0.001), good correlation with a visual analog scale (r=0.62, p<0.01), and sensitivity to change. ConclusionThis study reports the successful translation and cross-cultural adaptation of the SN-5 instrument into Brazilian Portuguese. The translated version exhibited adequate psychometric properties for assessment of disease-specific quality of life in pediatric patients with sinonasal complaints.

The impact of septoplasty on health-related quality of life in paediatric patients.

To assess the impact that septoplasty had on health-related quality of life (HRQoL) in paediatric patients and to determine whether there were patient characteristics that predicted better outcomes. Retrospective cohort study. Academic paediatric otolaryngology practice. All paediatric patients who underwent septoplasty during the study period. The current HRQoL was assessed using the Paediatric Quality of Life Inventory (PedsQL). The Glasgow Children's Benefit Inventory (GCBI) was used to evaluate the perceived change in HRQoL following septoplasty. A total of 29 patients (16 boys, mean age 13years) and their caregivers responded (response rate of 72.5%). There was a statistically significant improvement in HRQoL following septoplasty, as demonstrated by the positive mean GCBI subscores and the total GCBI score (35.1, sd=28.4). The total mean PedsQL score for child self-report was 95.2 (sd=6.9) and for parent-proxy report was 91.8 (sd=8.6), which indicated good current HRQoL. The enhancement in HRQoL post-septoplasty was moderately correlated with self-reported degree of nasal obstruction pre-septoplasty (r=0.621 for total GCBI). Also, there were differences in GCBI scores between the groups of children who wanted to have the surgery versus those who did not want to have the surgery. There was a significant positive change in HRQoL following paediatric septoplasty in our study population. Children who reported more severe nasal obstruction and those who wanted to have the surgery were more likely to experience enhancement of HRQoL following their surgery.

Oral Health-related quality of life in pediatric patients with cystic fibrosis.

To compare the oral health-related quality of life (OHRQoL) of 8- to 12-year-old children and 13- to 17-year-old adolescents diagnosed with cystic fibrosis (CF). Participants were recruited from a Midwest CF center. Parents provided demographic information and their assessment of the child's health. Patients completed the Child Oral Health Impact Profile (COHIP). Thirty-nine child-parent pairs participated. Fifty four percent of the patients were male, 87% Caucasian, and 56% adolescent, with 66% of families reporting an annual income of over $100,000. Excellent or very good health, including oral health, was reported by 67% of the patients. Individuals taking 10 or more medications reported better OHRQoL while Caucasians reported better oral health and total scores than other races. Adolescents had poorer total COHIP, social-emotional well-being, and self-image scores. Although most patients reported good or excellent oral health, adolescents reported a poorer OHRQoL than younger patients.

Assessment of health related quality of life in paediatric patients with asthma - methods and disease determinants

The purpose of this article is to provide an overview of the methods for assessing Health Related Quality of Life (HRQOL) in children with a focus on both the instruments available for measuring HRQOL in pediatric patients with asthma and the disease determinants. Adolescents with bronchial asthma often have substantially lower HRQOL. Frequent exacerbations impair children`s normal daily routines such as school activities, play and sleep. The factors affecting children`s HRQOL largely vary from those affecting the HRQOL in adults. Chronic diseases in childhood are a risk factor associated with serious psychosocial consequences - low self-esteem, behavioral problems, and difficulties in learning. Assessment of HRQOL can be successfully integrated in achieving the primary aim of the disease management - to maintain health as a state of complete physical, mental and social well-being.

Health Related Quality of Life of Patients with Sickle Cell Disease

Objective: The aim of the present study was to evaluate the health-related quality of life in pediatric patients with Cell Disease. Methods: An analytical cross-sectional study was performed. Pediatric patients suffering from Cell Disease below the age of 21 were compared with asymptomatic pediatric individuals. Clinical and demographic data were collected along with the Pediatric Quality of Life Inventory (PedsQL 4.0). Results: The sample was composed of 68 children and adolescents in the Sickle cell group and 44 in the Comparison group. Children and adolescent with SCD had lower scores of health related quality of life compared with healthy subjects in the domains physical (68.3 versus 88.8), social (71.1 versus 90.7), and school functioning (60.2 versus 78.7). The domain emotional functioning was not significantly different (62.1 versus 66.5). Conclusion: The results of the present study demonstrated that children and adolescents with SCD had lower scores of quality of life in the domains activities, social and school functioning when compared to healthy pediatric individuals.