title: Globus pallidus glial pigment and its changes with age and chronic illness in childhood.
abstract: Glial lipopigment appears in the globus pallidus without accumulating in neurons (except for late adolescence) in multiple chronic childhood diseases. In this observational study, we compared the age-related development of glial pigmentation in children with the chronic illness (cystic fibrosis) and children dying acutely. A secondary goal was to search for pallidal neuronal lipopigment in childhood. We recorded pigmentation in the brains of 37 consecutive cystic fibrosis children ranging in age from 0-23 yr and in 17 controls ranging in age from 0-18 yr. We characterized the lipofuscin histochemically and used several regression models to describe the mode of deposition. We observed that in the controls, intraglial pallidal pigment accumulated in 2 forms (relatively large globules and, separately, as clusters of fine granules) at a slow rate during childhood. In cystic fibrosis, both forms of pallidal glial pigment started accumulating at a younger age and were deposited far more rapidly. There was a further increase in the rate of accumulation between 8 and 10 yr of age. We did not encounter pallidal neuronal lipofuscin at any age. These observations are consistent with 2 propositions: 1) that globus pallidus glial cells are unique in their ability to accumulate lipofuscin before it accumulates in nearby neurons; and 2) that they are particularly susceptible to some systemic effect of this chronic illness.
authors.affiliation: Department of Pathology and Laboratory Medicine, Children's Hospital Los Angeles and University of Southern California Keck School of Medicine, 90027, USA.
authors.forename: Floyd H
authors.initials: FH
authors.lastname: Gilles
authors.forename: C Jane
authors.initials: CJ
authors.lastname: Tavaré
mesh_terms: D000293:Adolescent; D000328:Adult; D000375:Aging; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000473:pathology* / Q000503:physiopathology; D005917:Globus Pallidus / Q000166:cytology / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008062:Lipofuscin / Q000378:metabolism*; D009457:Neuroglia / Q000166:cytology / Q000378:metabolism / Q000473:pathology*; D018384:Oxidative Stress / Q000502:physiology; D012044:Regression Analysis
chemical_list: D008062:Lipofuscin