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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
Stephen Padilla, 3 days ago
Diabetic patients in a decompensated state and who develop type 2 myocardial infarction (MI) have an increased risk for mortality and major adverse cardiac events (MACE), according to a new study. In addition, these patients are potentially at risk for undiagnosed coronary artery disease.
5 days ago
Chocolate consumption is not associated with risk of coronary heart disease (CHD), stroke or both combined in postmenopausal women free of pre-existing major chronic disease, a study suggests.
Pearl Toh, 6 days ago
More intensive lowering of LDL-C levels was associated with a progressively greater survival benefit than less intensive approach, when the baseline LDL-C levels were ≥100 mg/dL, reveals a meta-analysis of 34 randomized trials.
5 days ago
Switching from thiazide diuretic to ipragliflozin, a sodium-glucose co-transporter 2 (SGLT2) inhibitor, leads to improvements in metabolic parameters and body mass composition without affecting blood pressure in type 2 diabetes (T2D) patients, a recent study has found.