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Chronic recurrent multifocal osteomyelitis (CRMO), first described in 1972, usually affects the long bones but it has affected the clavicle in about half of the 120 reported cases, and may be the paediatric equivalent of an adult syndrome given the name SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis). Affected children may also have pustulosis palmoplantaris. The clavicular disease has been variously called chronic sclerosing osteomyelitis, condensing osteomyelitis, sclerosis and hyperostosis, and pustolotic arthro-osteitis. Radiologically there is hyperostosis, osteosclerosis, and osteolysis. In Würzburg, Germany six of 11 children with CRMO had clavicular disease (European Journal of Pediatrics 1998;157:28–33). Plain radiography and radioisotope bone scan were the most helpful investigations; computed tomography and magnetic resonance imaging were misinterpreted as showing malignancy. Non-steroidal antiinflammatory drugs were symptomatically effective and the long term prognosis was good. The cause is unknown.
A study in Denmark (Acta Psychiatrica Scandinavica1997;96:402–4) illustrates well the near impossibility of drawing conclusions from non-randomised groups. There were 50 children of schizophrenic mothers, 25 of whom were brought up apart from their mothers and 25 with their mothers. Eighteen of the reared apart children and 13 of those who stayed with their mothers had developed psychiatric problems by their late 30s. Schizophrenia had developed in six of the reared apart and three of the mother reared group. The problem is that the mothers whose children were separated from them, as might be expected, had more severe schizophrenia so the study tells us little about the relative contributions of genes and environment. Nor does it tell us anything about the quality of childhood experienced by the two groups.
Orthopaedic surgeons in Doncaster have claimed good results from non-operative treatment of late diagnosed congenital dislocation of the hip (Journal of Bone and Joint Surgery1998;80-B:78–82). They treated 29 children (31 hips) aged 9 months to 3 years using skin traction, closed reduction, and six months in a spica cast. Twelve hips needed secondary extra-articular surgery. The end result was described as excellent in 25 hips, good in four, and fair in two. Their conclusion that good technique and attention to detail may be more important than the method used could apply to other areas of medical treatment.
Investigators in Vienna have described a girl with Wiskott-Aldrich syndrome and have shown how X linked diseases may become manifest in some girls (New England Journal of Medicine1998;338:291–5). Female carriers of the Wiskott-Aldrich gene usually show random inactivation of their somatic cell X chromosomes but preferential selection of the normal X chromosome in blood cells. This girl was shown to have a disease-causing mutation of the paternally derived Wiskott-Aldrich gene and preferential inactivation of the maternally derived normal gene in blood cells and cells of the buccal mucosa. Non-random X chromosome inactivation may itself be genetically determined via the X inactivation specific transcript (XIST) gene expressed in the inactive X chromosome (see editorial Ibid: 325-8).
Lucina has always assumed that the readers ofArchives will usually be familiar with articles of paediatric interest appearing in theBMJ but one finding of the 23 year follow up of the British national child development study cohort (British Medical Journal1998;316:339–42) seems particularly important. There were 124 people with epilepsy in the study and all nine known deaths (up to the age of 28) happened after the age of 16. Obviously some people with epilepsy do die in childhood but this study suggests that epilepsy becomes more dangerous at around the time the patients are leaving paediatric care. The implications for the care of people with epilepsy as they reach late adolescence and early adult life clearly deserve considerable thought.
A 2 g dose of ampicillin given intravenously during labour to women with vaginal group B streptococcal carriage prevents colonisation of the newborn only if given at least two hours before delivery (Obstetrics and Gynecology1998;91:112–14) In Granada, Spain the neonatal colonisation rate was 46% when the antibiotic was given less than an hour before delivery, 29% when given one to two hours before, 2.9% when given two to four hours before, and 1.2% when given more than four hours before delivery. When no antibiotic was given 47% of the babies were colonised.
Measles still kills. In the islands of Micronesia (population around 300 000) there were 1300 cases of measles and 16 measles related deaths in just under three years (December 1991 to October 1944) (Pediatric Infectious Disease Journal1998;17:33–9) although measles had not been a problem in the previous 20 years. Virus studies suggested both transmission between islands and importation from outside. Mass vaccination shortened the outbreaks. High levels of cover with two-dose measles vaccination is important in relatively isolated island populations.
Researchers in the USA hypothesised that the neurotropic properties of human herpesvirus 6 (HHV-6) might lead to increased recurrence risk after febrile convulsions brought on by HHV-6 infection (Pediatric Infectious Disease Journal1998;17:43–8). In fact, they found that recurrent seizures occurred twice as commonly after non-HHV-6 febrile convulsions (40%) as after HHV-6 induced febrile convulsions (20%). The only explanation they offer is that HHV-6 infection causes a high fever and may therefore precipitate febrile convulsions in less susceptible individuals.
The malassezia species of yeasts may be a cause of infection in small preterm babies. M furfurhas been associated with parenteral feeding using lipid emulsions. Now an outbreak of infection with M pachydermatishas been described from an intensive care unit in New Hampshire, USA (New England Journal of Medicine1998;338:706–11). This organism is known to cause otitis externa in dogs, and 12 of 39 dogs owned by workers on the unit were colonised. DNA analysis showed that one member of staff and three dogs carried an organism identical to that isolated from all 15 patients. Hand washing practices in the nursery had been inadequate and the outbreak stopped after re-education of staff about hand washing and discharge of the colonised babies. (M pachydermatiswas first described as causing dermatitis in a rhinoceros. Lucina has limited information about contacts between neonatal staff and rhinoceroses.)
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