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Little is known about familial risk factors for mitochondrial DNA deletion disorders. Now data from 226 families have been collected from centres in seven countries (
). Each family had at least one member with chronic progressive external ophthalmoplegia, Kearns Sayre syndrome, or Pearson’s syndrome. For unaffected mothers age did not affect the risk of having a child with a mitochondrial DNA deletion disorder. None of 251 siblings of index cases was affected but the risk for the children of affected mothers was about one in 24.
Virologists at the National Institute of Virology in Pune, India (
; see also comment, ibid: 821–2) have shown that a little known virus, the Chandipura virus, was responsible for an outbreak of acute encephalitis in Andhra Pradesh in 2003 that killed 183 of 329 affected children. After ruling out Japanese encephalitis, West Nile, dengue, measles, coronavirus, paramyxovirus, enterovirus, and influenza viruses, they identified Chandipura virus by electron microscopy, complement fixation, and neutralisation tests on a virus isolated from six patients. The viral RNA was 97% identical with the Chandipura virus reference strain on sequencing and 28 of 55 cases investigated had evidence of recent infection with the virus. The virus was also identified in postmortem brain tissue from one child. Chandipura virus has previously been isolated from only three human beings, all in India. They were two adults with fever in 1965 and one patient with acute encephalitis in 1980. It may, though, have been responsible for other unexplained outbreaks of acute encephalitis. It is probably transmitted through sandflies and has been detected in sandflies in Africa as well as in India.
Another study into the purported link between measles-mumps-rubella (MMR) vaccination and autism has produced negative results (
). The study included 1294 cases (children with a diagnosis of pervasive developmental disorder) and 4469 age- and sex-matched controls. There was a non-significant 14% reduction in risk of pervasive developmental disorder associated with MMR vaccination. The findings were similar for a diagnosis of autism.
About 10% of children with Kawasaki’s disease have coronary aneurysms early in the course of the disease. After the acute phase the aneurysms change shape; some persist but others regress or become stenotic. New aneurysms are uncommon; an incidence of 2.7% has been reported from Japan (
). Fifteen of 562 children with aneurysms in the acute phase were found to have 17 dilated lesions on repeat angiography after 2–19 years. Fifteen of the 17 lesions were new aneurysms and two were newly expanded old aneurysms. Ten of the new aneurysms developed at the site of previous large aneurysms that had regressed and all were associated with localised stenosis (12 post-stenotic, three pre-stenotic). Of the two expanding aneurysms, one had increased from 4.4 mm to 19.5 mm in 17 years and the other from 10 mm to 15 mm in 1 year. None of the new or expanding aneurysms had been associated with new cardiac events, but eight patients underwent coronary artery bypass grafting, and one had percutaneous balloon angioplasty.
In six American cities (
; see also editorial, ibid: 1134-6) investigators taught the parents of children with asthma how to reduce allergen or irritant levels in the home. Intervention was tailored to each child after skin testing and observation of the home and could include measures to reduce exposure to house dust mites, cockroaches, pets, rodents, mould, and tobacco smoke. A total of 937 children aged 5–11 years were randomised to intervention or control groups. Over the year of intervention and the succeeding year allergen levels in the home were reduced significantly and the children in the intervention group had fewer asthma symptoms. This is possibly the first study to show clear benefit from allergen avoidance in children with asthma.
Mycobacterium avium subspecies paratuberculosis (MAP) is the cause of the chronic enteritis of cattle known as paratuberculosis or Johne’s disease. The organism has been cultured from intestinal tissue, lymph nodes, and breast milk of patients with Crohn’s disease and a 7-year old boy with cervical lymphadenopathy caused by MAP developed Crohn’s disease 5 years later. Now MAP has been cultured from the blood of adult patients with Crohn’s disease (
; see also comment, ibid: 1013–4). In Florida, PCR assay and culture for MAP were performed on buffy coat preparations from 52 people; 28 with Crohn’s disease, nine with ulcerative colitis, and 15 without inflammatory bowel disease. MAP DNA was identified in 13/28, 4/9, and 3/15 and viable MAP was cultured in 14/28, 2/9, and 0/15, respectively. There appears to be an association between MAP and Crohn’s disease but it has not been shown to be causative. The results of an Australian therapeutic trial are awaited.
Parents do not regret talking about death with their dying child. In Sweden (
, see also editorial, ibid: 1251–3) completed questionnaires were returned in 2001 by 449 parents of children who had died of malignant disease between 1992 and 1997. None of 147 parents who had talked with their child about death regretted it but 69 of 258 parents who had not done so stated that they wished they had. Parents were more likely to have talked with their child about death if they felt that the child was aware of dying, if they had religious beliefs, or if the child was older. These three factors were also associated with regret among parents who had not discussed death with the child. The content of the discussions and the children’s responses were beyond the scope of the study.
Dogs may be able to smell out some cancers. In Buckinghamshire (
) six dogs were trained to distinguish between the urine of patients with bladder cancer and control urines. Each dog was then presented with nine sets of urine, each set consisting of one sample from a patient with bladder cancer and six controls. The dogs achieved a 41% success rate whereas chance would have dictated a 14% success. Although she likes dogs, Lucina has difficulty envisioning their use in clinical practice. Perhaps it might be possible to establish what it is the dogs are smelling and develop chemical tests. Could dogs be useful as pointers to the development of new tests in paediatric oncology or metabolic diseases?