There have been no previous attempts to estimate the incidence of juvenile dermatomyositis (JDM) in the UK and Ireland. Consultant paediatricians from these countries were asked to report all cases of JDM which were diagnosed in 1992 and 1993 to the British Paediatric Surveillance Unit. One-hundred-and-twenty-one reports were received, of which 48 were confirmed cases of JDM and three were confirmed cases of polymyositis. The estimated incidence of JDM was 1.9 per million children aged under 16 yr (95% confidence interval 1.4-2.6). The median age at onset was 6.8 yr and the median delay in diagnosis was 4 months. Girls were affected much more often than boys (ratio 5.0:1). In the cases with an onset in 1992 there appeared to be a cluster of cases with an onset in April and May.

Reflex sympathetic dystrophy (RSD), an unusual diagnosis in general paediatrics, is well recognised by paediatric rheumatologists. This study reports the presentation and the clinical course of 46 patients (35 female, age range 8-15.2) with RSD. The patients saw professionals from an average of 2.3 specialties (range 1-5). Twenty five (54%) had a history of trauma. Median time to diagnosis was 12 weeks (range 1-130). Many children had multiple investigations and treatments. Once diagnosis was made, treatment followed with physiotherapy and analgesics. Median time to recovery was seven weeks (range 1-140), with 27.5% relapsing. Nine children required assessment by the child and adolescent psychiatry team. This disease, though rare, has significant morbidity and it is therefore important to raise clinicians' awareness of RSD in childhood. Children with the condition may then be recognised and referred for appropriate management earlier, and spared unnecessary investigations and treatments which may exacerbate the condition. (Arch Dis Child 2000;82:231-233) Keywords: reflex sympathetic dystrophy; complex regional pain syndrome Reflex sympathetic dystrophy, a disorder characterised by severe and continuous pain in an extremity associated with vasomotor instability, was first described by Mitchell in 1864 during the American Civil War.1 It is a well recognised diagnosis in adults but, although it occurs in children, is rarely mentioned in paediatric textbooks. Before 1978 only eight cases had been reported. [2][3][4][5][6] It is still probably underrecognised and underdiagnosed in children.Since it was first described there has been much disagreement about the condition. It has been given a number of diVerent titles, such as algodystrophy, Sudek's atrophy, causalgia, sympathetic maintained pain syndrome, and hand-shoulder syndrome.7 More recently, a revised taxonomic system for these disorders has been developed, and what we shall refer to in this paper as reflex sympathetic dystrophy (RSD) is now known as chronic regional pain syndrome type 1 (CRPS 1). 8Since it was first described in paediatric patients, it has been realised that there are differences in presentation and treatment responses between adults and children. Generally, there is less often an antecedent history of trauma or surgery in children who develop RSD, and the outlook for recovery is better than in adults.9 Children rarely appear to develop trophic changes. 10 11 Psychological factors are thought to have a major role in the cause of this syndrome. 12The diverse symptomatology of RSD leads it to cross a wide spectrum of clinical disciplines. The resulting varied modes and routes of presentation may lead to a delay in diagnosis. Failure to recognise the condition may lead to unnecessary investigations and treatment, which may even exacerbate the condition.We report the clinical course and treatment response of 46 children with RSD, presenting to a large children's hospital in the north west of England. This study aimed at determining the ...

Children with meningococcal disease have a wide range of initial plasma cortisol levels, with lower levels found in those who die. Many factors may affect cortisol levels, but adrenal insufficiency is probably uncommon.

Cardio-pulmonary manifestations of systemic lupus erythematosus (SLE) are well recognized in adults. We report the occurrence of clinically significant cardio-pulmonary disease in a cohort of predominantly Caucasian children with SLE. All children with SLE attending the Royal Liverpool Children's NHS Trust between 1995 and 2003 were reviewed. Of 29 children with SLE, 27 (93%) were Caucasian. Nine (31%) had cardio-respiratory complications: cardiac only (n = 1); respiratory only (n = 4); both cardiac and respiratory manifestations (n = 4). Median (range) duration of follow-up of affected children: four years (six months to 11 years). Six out of eight (75%) presented with respiratory complications before SLE was diagnosed. Three children had pericardial effusions, one requiring pericardiocentesis for tamponade. One had cardiac conduction defects and another significant pulmonary hypertension. Respiratory complications comprised: interstitial lung disease (n = 4), with two showing evidence of pulmonary fibrosis; pleural effusions (n = 2), pulmonary haemorrhage (n = 1) and lupus pneumonitis (n = 1). Disease course was complicated by CMV infection in one child. Lung biopsy was performed in five cases. Seven were treated with cyclophosphamide with significant improvement in symptoms/lung function. Of this predominantly Caucasian paediatric cohort with SLE, 31% had significant cardio-pulmonary involvement. All children with SLE should have regular monitoring of their cardio-respiratory status.

Aims-To determine long term neurodevelopmental outcome following the spectrum of meningococcal infection. Methods-Between 1988 and 1990, 152 cases of meningococcal disease were recruited; 139 survived. Between 1998 and 1999, 115 survivors (83%) were evaluated, together with 115 sex and age matched controls. Standard measures of neurological function, coordination, cognition, behaviour, and hearing were used to assess neurodevelopmental status. Results-One case has spastic quadriplegia. Gross neurological examination was normal in all other cases and all controls. Five cases and no controls have significant hearing loss. Cases performed at a lower level than controls on measures of coordination, cognition, and behaviour. Four cases and no controls had major impairments. The adjusted odds ratios for moderate and minor impairments were 3.6 (95% CI 1.3 to 10.3) and 1.6 (95% CI 0.8 to 3.4) respectively. Conclusion-The majority of survivors from this cohort do not have gross neurological deficits. However, when objective measures of motor function, cognitive ability, and behaviour were applied significant detriments were found in meningococcal survivors. (Arch Dis Child 2001;85:6-11)

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