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Sialorrhoea, dystonia reduce HRQoL in kids with leukoencephalopathy

12 Jul 2018
Chan witnessed the effectiveness of Dejian Mind-Body Intervention (DMBI) from a clinical perspective. Photo source: Shutterstock

Health-related quality of life (HRQoL) is lower in patients with more severe genetically determined leukoencephalopathy, according to a recent study.

Using the Paediatric Quality of Life Inventory (PedsQL), researchers assessed HRQoL in 59 patients diagnosed with genetically determined leukoencephalopathies, of whom 38 were male (mean age 8 years, 10 months) and 21 were female (mean age 9 years, 3 months). T-tests were used to determine differences in HRQoL.

Mean PedsQL scores were significantly lower in patients with at least one severe clinical involvement (52.35±18.40 vs 84.03±9.845; p<0.001). This was also true for the physical (40.73±27.79 vs 87.38±19.99; p<0.001), emotional (64.45±24.14 vs 89.39±7.736; p<0.001) and social (54.24±23.72 vs 80.83±18.55; p=0.01) functioning scores.

In terms of specific clinical features, patients with sialorrhoea had significantly lower mean PedsQL scores (48.18±20.12 vs 61.63±18.90), gastrotomy (41.59±21.54 vs 59.49±19.33) and dystonia (47.5±19.4 vs 67.33±14.80; p<0.05 for all) relative to those without the nonmotor symptom.

Wheelchair use also led to significantly lower PedsQL scores (47.66±18.83 vs 63.00±19.49; p<0.05). All four severe clinical presentations likewise resulted in significantly lower physical functioning scores, while only sialorrhoea was associated with significantly poorer emotional functioning.

“By assessing HRQoL, we have identified areas of concern that can be targeted and prioritized when developing care strategies. Interventions should address these specific concerns regarding physical and social functioning,” said researchers, referring to sialorrhoea, gastrotomy, dystonia and wheelchair use.

“Clinicians should pay particular attention to these features when deciding on supportive treatment strategies,” they added.

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Most Read Articles
6 days ago
Electroretinogram-assessed localized retinal dysfunction in type 1 diabetes mellitus patients appears to occur even in the absence of clinical signs of diabetic retinopathy (DR), and this incidence is associated with ageing, according to a study.
Roshini Claire Anthony, 18 hours ago

Patients with mild hypertension who are at low risk for cardiovascular disease (CVD) do not appear to derive mortality or CVD benefit from antihypertensive treatments, raising questions on the need for treatment in this population, according to a recent study from England.

Pearl Toh, 3 days ago
A personalized computerized neurofeedback intervention for training attention and memory shows potential in cognitive training for healthy elderly men, who improved in cognitive performance after the training, although no significant improvements were seen in the overall study population.
6 days ago
Greater home blood pressure (BP) variability, particularly in home systolic (S)BP, correlates with higher carotid, aortic and peripheral but not coronary atherosclerosis burdens independent of the mean home BP, suggests a recent study.