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Lysosomal acid lipase deficiency (LAL-D) is an ultra-rare lysosomal storage disease that may present from infancy to late adulthood depending on residual enzyme activity. While the severe form manifests as a rapidly progressive disease with near universal mortality within the first 6 months of life, milder forms frequently go undiagnosed for prolonged periods and typically present with progressive fatty liver disease, enlarged spleen, atherogenic dyslipidemia and premature atherosclerosis. The adult variant of LAL-D is typically diagnosed late or even overlooked due to the unspecific nature of the presenting symptoms, which are similar to common changes observed in the context of the metabolic syndrome. This review is aimed at delineating clinically useful scenarios in which pediatric or adult medicine clinicians should be aware of LAL-D as a differential diagnosis for selected patients. This is particularly relevant as a potentially life-saving enzyme replacement therapy has become available and the diagnosis can easily be ruled out or confirmed using a dried blood spot test.

a b s t r a c t 8 Associations between personality and motivation have been investigated using various operationalizations for 23 both constructs, but no study so far has linked the HEXACO personality space to achievement goals. The aim of 24 the present study was thus to explore relations between the HEXACO personality domains and achievement 25 goals focusing particularly on Q4 honesty-humility. Participants were 173 high-school students with a mean age 26 of 14.49 (SD = 1.04) years. Data were collected using questionnaires assessing the HEXACO personality invento-27 ry and students' mastery goals, performance-approach goals, and performance-avoidance goals for school in gen-28 eral. With few exceptions, findings were consistent with associations reported for the Big Five dimensions and 29 achievement goals. Moreover, honesty-humility showed a distinct pattern of systematic relations with achieve-30 ment goals, correlating positively with mastery goals and negatively with both performance-approach goals and 31 performance-avoidance goals. Finally, relative weights analyses revealed that honesty-humility accounted for 32 substantial proportions of explained variance in all achievement goals. 33

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