Age- and sex-related growth patterns of the craniofacial complex in European children aged 3-6 years.

Background: Craniofacial growth changes in young children are not yet completely understood. Up-to-date references for craniofacial measurements are crucial for clinical assessment of orthodontic anomalies, craniofacial abnormalities and subsequent planning of interventions.

Aim: To provide normal reference data and to identify growth patterns for craniofacial dimensions of European boys and girls aged 3-6 years.

Accuracy of a portable multisensor body monitor for predicting resting energy expenditure in older people: a comparison with indirect calorimetry.

Background: Accurate and comfortable methods are needed to determine resting energy expenditure (REE) in older people who are characterized by a lowered metabolic rate. The portable SenseWear® armband (SWA) body monitor, worn on the right upper arm, can easily be used by this age group in an ambulatory manner.

Objective: The purpose of this study was to evaluate the reliability and accuracy of the SWA armband in determining REE in healthy, normal-weight older people.

Prevalence of sarcopenia among older community-dwelling people with normal health and nutritional state.

This study analyzed whether sarcopenia, a risk factor for disability in the aged, also occurs in healthy community-dwelling elders with normal nutritional state. As indicators, body cell mass (BCM) and lean body mass (LBM) were determined in 110 Germans (ages 60-83) using bioimpedance analysis. Nutritional status, muscle function, anthropometry, and physical activity level were investigated.

Validation of the BIOPAC indirect calorimeter for determining resting energy expenditure in healthy free-living older people.

The BIOPAC indirect calorimeter for measuring resting energy expenditure (REE) is less cumbersome than many other calorimeters. We tested the hypothesis that the BIOPAC calorimeter is as well suited for determining REE in older people as traditional calorimeters. In 50 healthy persons (24 men and 26 women; age range, 61-83 years), REE by BIOPAC was validated against Vmax Spectra indirect calorimeter as a criterion method.

Increasing sulfatide synthesis in myelin-forming cells of arylsulfatase A-deficient mice causes demyelination and neurological symptoms reminiscent of human metachromatic leukodystrophy.

Metachromatic leukodystrophy (MLD) is a lysosomal storage disorder caused by the deficiency of arylsulfatase A (ASA). This results in accumulation of sulfated glycosphingolipids, mainly 3-O-sulfogalactosylceramide (sulfatide), in the nervous system and various other organs. In patients, lipid storage causes a progressive loss of myelin leading to various neurological symptoms.

Sulfatide storage in neurons causes hyperexcitability and axonal degeneration in a mouse model of metachromatic leukodystrophy.

Metachromatic leukodystrophy is a lysosomal storage disorder caused by deficiency in the sulfolipid degrading enzyme arylsulfatase A (ASA). In the absence of a functional ASA gene, 3-O-sulfogalactosylceramide (sulfatide; SGalCer) and other sulfolipids accumulate. The storage is associated with progressive demyelination and various finally lethal neurological symptoms.

Enzyme replacement improves nervous system pathology and function in a mouse model for metachromatic leukodystrophy.

A deficiency of arylsulfatase A (ASA) causes the lysosomal storage disease metachromatic leukodystrophy, which is characterized by accumulation of the sphingolipid 3-O-sulfogalactosylceramide (sulfatide). Sphingolipid storage results in progressive demyelination and severe neurologic symptoms. The disease is lethal, and curative therapy is not available.