medwireNews: Children and adolescents with congenital adrenal hyperplasia (CAH) appear to have impaired psychosocial quality of life (QoL), particularly in the school domain, while physical QoL is preserved, suggest findings of a systematic review and meta-analysis.
“Good disease control and prevention of disease/treatment-related complications could be important for improving QoL of children with CAH ”, suggest Sumudu Nimali Seneviratne (University of Colombo, Sri Lanka) and colleagues in the Journal of Clinical Endocrinology & Metabolism.
The team identified 12 observational studies for the systematic review that assessed QoL in 781 children and adolescents aged 21 years or younger with CAH, compared with their healthy peers. The majority (90%) of children had classic CAH and 63% were female. QoL was assessed using the Pediatric Quality of Life Inventory (PedsQL) in nine studies, while three studies each used a self-designed questionnaire, the World Health Organization QoL Brief Version and the Children QoL Questionnaire-Revised (KINDL-R).
The meta-analysis included data from six studies on 227 children with CAH, with self-reported QoL measured using the PedsQL and parent-reported QoL by the PedsQL or Child Health Questionnaire-Parent Form 50.
The meta-analysis showed that QoL reported by the children with CAH was lower compared with healthy children, but only significantly so for school QoL, with a mean difference of 8.5 points. There was no significant difference for other individual psychosocial domains or for physical QoL.
“[W]e postulate that impaired school QoL in children with CAH could be ascribed to both issues common to those faced by any child with a chronic disease such as school absenteeism due to hospital/clinic visits, as well as issues more specific to CAH such as the stress of having an ‘invisible’ condition, and stigma”, says the team.
For parent-reported QoL, scores were significantly lower in children with CAH versus healthy children for overall psychosocial QoL, with a mean difference of 9.9 points, and for all individual psychosocial domains, specifically school (mean difference 7.4 points), emotional (5.6 points) and social (4.3 points).
Again, there was no significant difference in physical QoL, the researchers highlight. “This resilience in physical QoL among children with CAH could be explored as a positive factor to improve their wellbeing”, Seneviratne et al suggest.
They found a “lack of uniformity” among the range of sociodemographic, disease, complication and treatment-related factors associated with CAH, but possible contributors that did emerge included older age, pubertal advancement and being female, which were associated with a higher risk of impaired psychosocial QoL.
Reduced QoL was associated with undertreatment, as indicated by features such as hyperpigmentation, hirsutism, virilisation, advanced bone age, low BMI and increased serum levels of 17-hydroxyprogesterone, androstenedione and testosterone, as well as over-treatment, as evidenced by hypertension, high BMI and high mineralocorticoid dose. Treatment adherence, lack of regular follow-up and increased hospital admissions were also linked to a worse QoL.
The researchers also comment on the impact of genital surgery in girls, noting that one study found that feminising genitoplasty had no impact on psychosocial QoL, whereas a second indicated that early clitoroplasty (vs no or late clitoroplasty) was a positive psychosocial QoL predictor, whereas vaginoplasty and postoperative urinary incontinence were negative factors.
This suggests that the “need and timing of each component of genital surgery needs careful consideration”, they say.
The researchers conclude that “[t]here is a need for further high-quality research that investigates the relationship between disease-control, provision of psychosocial support and improvement in QoL in children/adolescents with CAH.”
To support this, they say that a “validated questionnaire that assesses QoL in children with CAH in routine clinical practice would prove useful.”
By Lucy Piper
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