When is a shunt no longer necessary? An investigation of 300 patients with hydrocephalus and myelomeningocele: 11-22 year follow up.

Three hundred consecutive patients treated with a shunt for hydrocephalus associated with open spina bifida were followed for a period ranging from 11 to 22 years with a view to answering the question: How long is a shunt necessary? At most, in 51 patients (17%) the shunt was no longer functioning and was not causing any trouble. It is possible that many of these will still develop problems of raised intracranial pressure and their shunt would have to be revised, or reinserted. In fact, this occurred in 14 patients who had a shunt without revision for 10 to 15 years before trouble started and the shunt had to be revised.

Location of the gene for 21-hydroxylase deficiency.

HLA typing of 33 families with one or more children suffering from congenital adrenal hyperplasia confirmed that the gene for 21-hydroxylase deficiency is closely associated with the HLA region. Analysis of two families in which recombination of chromosome 6 had occurred indicated that the gene locus is between the A and D loci of the HLA region. The rare allele Bw47 was observed in 18 parents and was always associated with the carrier state for 21-hydroxylase deficiency.