Double disadvantage: a case control study on health-related quality of life in children with sickle cell disease

Background  

Low health-related quality of life (HRQoL) of children with sickle cell disease (SCD) may be associated with consequences of the disease, or with the low socio-economic status (SES) of this patient population. The aim of this study was to investigate the HRQoL of children with SCD, controlling for SES by comparing them to healthy siblings (matched for age and gender), and to a Dutch norm population.     

Measuring thyroid gland volume: should we change the correction factor?

OBJECTIVE: In the assessment of thyroid volume with sonography (formula of an ellipsoid), a correction factor is used. Whereas previously 0.524 was used, the World Health Organization has recently changed (after the first review) this correction factor to 0.479. We compare volume measurement of the thyroid using different correction factors to automated volume measurement using MDCT, and we define an optimal correction factor in thyroid volume assessment. CONCLUSION: Acceptable correction factors are situated in the range of 0.494-0.554. We propose a correction factor of 0.529 when using the ellipsoid formula.     

Neurocognitive deficits in children with sickle cell disease: a comprehensive profile

Background

Sickle cell disease (SCD) can lead to profound cerebral damage, associated with neurocognitive deficits. The aim of the current study was to evaluate a broad range of neurocognitive functions in children with SCD compared to a SES‐matched control group, in order to gain more insight into the specific deficits of these patients.

Methods

Forty‐one children with homozygous SCD (HbSS or HbS‐β0‐thalassemia) and 38 controls were assessed on a comprehensive set of well‐defined and validated measures of neurocognitive functioning. Besides general intelligence, we evaluated executive functioning extensively (including response inhibition, sustained attention, planning, visuo‐spatial working memory, and verbal working memory) as well as visuo‐motor functioning.

Results

SCD was clearly associated with lower IQ scores. More than one in three children with SCD had a Full‐scale IQ below 75. Furthermore, children with SCD showed deficits in visuo‐motor functioning. Some evidence was found for executive dysfunction: Children with SCD displayed poor visuo‐spatial working memory, as well as subtle deficits in sustained attention and planning. No significant differences were found between children with SCD and controls in terms of response inhibition and verbal working memory.

Conclusions

Children with SCD are at increased risk of lower intelligence, visuo‐motor impairments, and executive dysfunction. These neurocognitive deficits may underlie high rates of scholastic impairments in these children. The present findings further illuminate the importance of regular neurocognitive evaluations and future neurocognitive rehabilitation programs for children with SCD. Pediatr Blood Cancer 2011;56:783–788. © 2010 Wiley‐Liss, Inc.     

An indentation apparatus for evaluating discomfort and pain thresholds in conjunction with mechanical properties of foot tissue in vivo

The mechanical properties of human foot tissue in vivo as well as discomfort and pain thresholds are important for various applications. In this study, an apparatus for measuring the discomfort and pain thresholds and the mechanical properties of human tissues is presented. The apparatus employs a stepper motor that controls the indentation speed, as well as a load cell and potentiometer that determine the corresponding reaction force and tissue deformation (displacement), respectively. A LabVIEW program (LabVIEW 8, National Instruments Corporation; Austin, Texas) was developed to control the indentation via a data acquisition card. The apparatus can accommodate indentor displacements up to 35 mm and can impart forces up to 150 N at a controlled indentation speed in the range of 0 to 10 mm/s. Tests showed that the displacement measurement error is <0.17 mm in the nominal range (0.5% in the full scale) and the measurement error of force is <1.6 N in the nominal range (1.1% in the full scale). Experimental results indicate that the apparatus is reliable and flexible for measuring the mechanical properties of foot tissue in vivo in conjunction with pain and discomfort thresholds.