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The prevalence of alpha-thalassaemias is up to 5% in Hong Kong and up to 40% in some parts of South East Asia. Haemoglobin H consists of four betaglobin chains. Instead of the usual complement of four normal alphaglobin genes (two on each chromosome 16), people with haemoglobin H disease have only one. At the other three alphaglobin gene sites, they have either three deletions or two deletions and one mutation. Now, work in Hong Kong (New England Journal of Medicine2000;343:544–50) has shown that those with a mutation have more severe disease but iron overload is equally a problem in both types.
Eight of 13 American and four of five French boys with cerebral X-linked adrenoleukodystrophy survived bone marrow transplantation at ages 5 to 11 years and were followed for five to ten years (Lancet2000;356:713–18). Verbal intelligence remained normal in 11 and non-verbal abilities remained stable or improved in seven. Eight showed continuing demyelination on magnetic resonance imaging (MRI) but later stabilised, one showed no change, one improved, and two showed complete reversal of MRI abnormalities.
Is laparoscopic surgery safe in children with ventriculoperitoneal shunts? A small study in St Louis, Missouri (Journal of Pediatric Surgery 2000;35:1104–5) suggests that it is. Three of ten children had postoperative complications but none was related to the shunt. Six of the laparoscopies were …
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