2Pediatric Department of Tanta Faculty of Medicine, Tanta University, Egypt
Subjects: 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. Their clinical and demographic details were recorded and QOL was assessed using PedsQL™ 4.0 Generic Core Scales.
Conclusions: The present study shows that the overall QOL in children with MCD was better than in children with other chronic illnesses.
Keywords: Quality of Life; Pediatric; Minimal change disease;
The aim of this work was to evaluate the HRQOL in children with MCD and delineate the HRQOL domains in various clinical subtypes of INS. The primary objective was to assess the QOL in children between 2 and 18 years of age with the commonest idiopathic NS (MCD) using Pediatric Quality of Life Inventory (PedsQL™ 4.0 Generic Core scales) in relation to controls. The secondary objective was to evaluate the physical, emotional, and social domains affected in various clinical phenotypes of pediatric MCD.
Subtype of steroid sensitive nephrotic syndromes which were:
a. Frequent relapsing nephrotic syndrome: which was defined as relapse ≥ 4 times per year or ≥ 2 times per 6 months. Relapse was defined as recurrence of proteinuria [urine albumin dipstick ≥ 2+ on 3 consecutive days, most often in association with recurrence of edema.
b. Infrequent relapsing nephrotic syndrome: which was defined as relapse < 4 times per year or < 2 times per 6 months.
c. Steroid dependent nephrotic syndrome :which was defined as relapse every time with the withdrawal of steroids from every day to every other day therapy [5].
The tool evaluates the QOL in five domains: physical functioning (Eight Items), psychosocial functioning including emotional functioning (five items), social functioning (five items), and school functioning (5 items). The PedsQL scores range from 0 to 100 points. Recall time was one month and a 5-point response scale was used from 0 (never a problem) to 4 (almost always a problem). The scores of each item were then reversed and linearly transformed into a 0–100 scale (0 = 100, 1 = 75, 2 = 50, 3 = 25, and 4 = 0), with higher PedsQL scores indicating a better QOL. Parameters studied were QOL total score, QOL in social, emotional, physical, and school domains [4].
The questionnaires were filled through an interview with the child and the Caregiver, the questions were asked in lay language to the child if he was older than 6 years and to the caregiver if the child was ≤ 6 years [4].
One-way analyses of variance or Kruskal - Wallis tests were used to analyze statistical differences between three or more groups for parametric and nonparametric data, respectively. P < 0.05 was considered statistically significant. Data were evaluated using Statistical Package for the Social Science (SPSS) software version 16.0 (SPSS Inc., Chicago, IL, USA). Predictive risk factors for poor total and school performance scores were determined by univariate analysis using Student’s t-test [6].
|
MCD Group (N=100) |
Control group(N=100) |
P value |
||
Range |
Median |
Range |
Median |
||
Age at enrollement (years) |
2.2-15 |
7 |
3.5-13 |
8.5 |
0.1
|
Disease Duration |
0.5-132 |
33 |
1-132 |
36 |
0.22 |
Relapse number |
1-16 |
4 |
- |
- |
- |
Family income(Egyptian pound/month) |
400-1.250 |
800 |
285-3750 |
1000 |
0.8 |
Serum Albumin(g/dl) |
1.3-4.6 |
2.5 |
- |
- |
- |
Serum cholesterol(mg/dl) |
151-450 |
323.3 |
- |
- |
- |
|
No |
% |
No |
% |
|
Age (years) |
24 |
24 |
6 |
6 |
0.1 |
Sex: |
60 |
60 |
52 |
52 |
0.4 |
Clinical types: |
32 |
32 |
- |
- |
- |
Clinical picture: |
22 |
22 |
- |
- |
|
Prior medications: |
82 |
82 30 |
- |
- |
- |
Complications: |
8 |
8 |
- |
|
|
|
Cases |
Controls |
P value |
||
No (%) |
Range (Median) |
No (%) |
Range |
||
Physical scores: Range (Median) |
100(100%) |
58.6-75(71.9) |
100(100%) |
58.6-68.8(63) |
0.004* |
Emotional scores: Range (Median) |
100(100%) |
55-65(65) |
100(100%) |
55-65(60) |
0.03* |
Social scores Range (Median) |
100(100%) |
63.8-75(70) |
100(100%) |
60-70(65) |
0.01* |
School scores Range (Median) |
80(80%) |
56.3-65(63) |
98(89%) |
53-65(65) |
0.75 |
Total score |
80(80%) |
59-68.8(65) |
89(98%) |
58-65.8 (62.2) |
0.01* |
|
Steroid dependent and |
Steroid resistant |
Infrequently relapsing and first episode (N=32) |
P value |
Physical scores: Range (Median) |
56.3-75(68.8) |
65.6-75(71.9) |
65.6-75(71.9) |
0.72 |
Emotional scores Range (Median) |
51.3-70(63) |
60-71.3(60) |
56.3-75(63) |
0.3 |
Social scores Range (Median) |
65-75(70) |
63.8-75(73) |
60-75(70) |
0.66 |
School scores Range (Median) |
58.8-65(60) |
60-65(65) |
55-65(60) |
0.53 |
A better understanding of HRQOL in such pediatric patients could help to improve the prognosis of the disease. The PedsQL™ 4.0 Generic Core Scale is a brief, standardized generic assessment instrument that evaluate children, adolescents and parents perceptions of HRQOL in this growing age in a systematic way with chronic diseases. The flexibility, reliability, and validity of PedsQL core and modular design made it a simple famous tool in a variety of research publications and clinical applications for chronic health hazards in children and adolescents.
PedsQL™ 4.0 Generic Core Scale is a previously well used legacy tool that evaluate the body(physical), feelings(emotional), social, and school achivements [8,9].
There were few research studies regarding QOL in children with NS [3,10]. Most of published articles have included adult patients or have included patients from Western countries with relatively high socioeconomic standars [3]. So, their occlusions cannot be generalized to other communities. The present study concluded a better QOL in pediatric patients with MCD when compared with other chronic pediatric diseases. This work focused on the commonly missed aspects of care (physical, social, emotional, and school health) in children with MCD as the most popular variant of INS pediatric age in the context of Egypt as a developing country. More interestingly, we concluded that the assessment of physical, affective, and social performance was better than pediatric patients with other chronic pediatric diseases .This might be attributed to availability of providing our patients different therapeutic modalities of INS free of cost (as we are a governmental University Hospital)including corticosteroid therapy, diuretics, antihypertensives and immunosuppressive chemotherapeutic agents such as cyclosporine A, cyclophosphamide, and mycophenolate mofetil .This led to a good compliance and a good follow-up of included patients. However, an important conclusion was that the school achievement scores in our nephrotic patients were not different from diseased controls in contrast to the other domains in HRQOL.
An analysis of the PedsQL Inventory questionnaire results revealed that all school-going children with INS enrolled in the study, “missed some school days to go to the doctor or hospital” or “missed some school days because of not feeling well.”
Even though many of them “did not experience difficulties in class” and “did not have trouble keeping up with school work,” the authors believe that the reasons for poor school achievement scores might be partly attributable to frequesnt absence from school secondary to the frequent follow up at their hospitals or clinics for evaluation of the disease activity and assessment of adverse effects related to different medications. The recurrent or relapsing sequlae nature of disease in INS in pediatric age caused irregular school attendance and children may found it was difficult for them to adapt with the burden of missed lessons in addition to interrupted teachers interactions. Moreover, the integration of nephrotic school children into the classroom situation might be disturbed secondary to inadequate school environment included lack of health education of teachers who had insufficient knowledge about the nephrotic syndrome and inability to spend adequate compensatory times with these patients [11].
There are few available publications as regard the evaluation of HRQOL in pediatric patients with MCD, especially from developing countries like asian and Arab countries. Rüth et al evaluated QOL, psychological and social adjustment by standardized questionnaire in 45 European pediatric patients with steroid-sensitive NS from the Netherlands. Only the QOL subscale “social function” was impaired while other domains were not affected [3]. In USA, an evaluation of QOL using PedsQL Inventory in 127 American children with prevalent NS as compared to incident NS showed poor scores in social and school performances [2].
In contrast, most Asian studies from India have described the behavioral profile of children with INS, but data regarding QOL in pediatric nephrotic patients was seldom. The emotional and social domains of HRQOL in children with chronic diseases have been assumed to be markedly attributed to ethnic, sociocultural, and socioeconomic status [12]. So the situation different from children in the Western world and could explain the differences in results of our study in comparison to Western countries. This is the first study evaluating HRQOL in acohort of pediatric NS in South India using a validated and flexible measurement model, namely PedsQL™ 4.0 Generic Core Scale and is particularly consistent with the paltriness of information in QOL in this patient population. The recruitment of controls with chronic ailments involving other systems provides a reasonable comparative assessment of QOL that has been performed in earlier studies as well [12].
The study is also limited by its cross-sectional nature as a result of which a long-term follow up of this cohort was not undertaken.
One of other limitations of our study is difficult to collect large number of control patients with the same disease so the control group was composed of a variety of chronic clinical conditions; each condition has its own peculiar characteristics that will affect the quality of life of the patient. Moreover, the distribution of these chronic clinical conditions within the control group is also heterogeneous. The heterogeneity, both of the clinical conditions and the distribution of the specific conditions within the control group, provides a bias.
Further studies on wider scale should be done for more reliable comparison between QOL of patients with INS and QOL of each separate entity of different chronic diseases which are commonly affecting pediatric age like chronic asthma, epilepsy, chronic hemolytic anemia and others.
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