PubMed · 2647990
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Z Török. 1989. Diving: occupation or physiological experiment?. https://doi.org/10.1177/014107688908200205
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CONTEXT: Human neurodevelopmental consequences of exposure to methyl-mercury (MeHg) from eating fish remain a question of public health concern. OBJECTIVE: To study the association between MeHg exposure and the developmental outcomes of children in the Republic of Seychelles at 66 months of age. DESIGN: A prospective longitudinal cohort study. PARTICIPANTS: A total of 711 of 779 cohort mother-child pairs initially enrolled in the Seychelles Child Development Study in 1989. SETTING: The Republic of Seychelles, an archipelago in the Indian Ocean where 85% of the population consumes ocean fish daily. MAIN OUTCOME MEASURES: Prenatal and postnatal MeHg exposure and 6 age-appropriate neurodevelopmental tests: the McCarthy Scales of Children's Abilities, the Preschool Language Scale, the Woodcock-Johnson Applied Problems and Letter and Word Recognition Tests of Achievement, the Bender Gestalt test, and the Child Behavior Checklist. RESULTS: The mean maternal hair total mercury level was 6.8 ppm and the mean child hair total mercury level at age 66 months was 6.5 ppm. No adverse outcomes at 66 months were associated with either prenatal or postnatal MeHg exposure. CONCLUSION: In the population studied, consumption of a diet high in ocean fish appears to pose no threat to developmental outcomes through 66 months of age.
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BACKGROUND: Globoid-cell leukodystrophy is caused by a deficiency of galactocerebrosidase, which results in progressive central nervous system deterioration. We investigated whether allogeneic hematopoietic stem-cell transplantation can provide a source of leukocyte galactocerebrosidase and thereby prevent the decline of central nervous system function in patients with the disease. METHODS: Five children with globoid-cell leukodystrophy (one with the infantile type and four with late-onset disease) were treated with allogeneic hematopoietic stem-cell transplantation. Measurement of leukocyte galactocerebrosidase levels, neurologic examinations, neuropsychological tests, magnetic resonance imaging of the central nervous system, cerebrospinal fluid protein assays, and neurophysiologic measurements were performed before and after transplantation, with follow-up ranging from one to nine years. RESULTS: Engraftment of donor-derived hematopoietic cells occurred in all patients and was followed by restoration of normal leukocyte galactocerebrosidase levels. In the four patients with late-onset disease, the central nervous system deterioration was reversed, and in the patient with the infantile form of the disease, signs and symptoms have not appeared. Magnetic resonance imaging showed a decrease in signal intensity in the three patients with late-onset disease who were assessed both before and after transplantation. Abnormalities in cerebrospinal fluid total protein levels were corrected in three patients with late-onset disease and substantially reduced in the patient with the infantile form. CONCLUSIONS: Central nervous system manifestations of globoid-cell leukodystrophy can be reversed by allogeneic hematopoietic stem-cell transplantation.