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Ataxia with gluten sensitivity (gluten ataxia) is increasingly recognised in adults but the age range of 68 patients in North Trent and London (
) was 14–81 years. Patients presented with ataxia of gait, upper and lower limb ataxia, eye signs (nystagmus or abnormal eye movements), and dysarthria, and had cerebellar atrophy and white matter intensities on MRI. Almost half had a sensorimotor axonal neuropathy on neurophysiological testing and almost a quarter had coeliac disease. Testing for antigliadin antibodies is suggested for patients with sporadic (non-familial) ataxia but data on children seem sparse. There is some evidence that a gluten free diet may be beneficial.
An environment containing tobacco smoke is not good for children. More evidence of this has come from a study in Puerto Rico (
). This study included 512 children aged 2–12 years 243 of whom were exposed to tobacco smoke in the home because of parental smoking. Mean plasma vitamin C concentrations were significantly lower in the exposed group (48.9 v 52.1 μmol/l) even though dietary intake of vitamin C was similar in the two groups and urinary cotinine measurements suggested only mild exposure to tobacco smoke. The practical importance of this reduction in vitamin C concentrations is unknown but these authors maintain that it could be important in vulnerable children and even suggest that children exposed to tobacco smoke in the home should be encouraged to eat more vitamin C-rich foods or to take a vitamin C supplement.
Researchers at the Great Ormond Street Children’s Hospital (
) obtained data from social service and police files up to 1999 about 224 boys who had been referred to the sexual abuse clinic between 1980 and 1992. Twenty-six victims (12%) had themselves become sexual abusers. The risk of this progression was increased if the child had suffered from physical neglect, lack of adult supervision, or sexual abuse by a female, or had witnessed severe violence in the family, usually against his mother by her male partner. Cruelty to animals was committed more often by victims who became abusers. No factors were identified that reduced the risk of becoming an abuser. It is hoped that by concentrating intervention on boys at high risk of becoming abusers prevention might be possible.
It can come as no surprise to anybody that even within the poorest societies there are those who have more than others and those who are more capable than others. In rural southern Tanzania, where poverty is universal, it was possible to divide families into five socio-economic groupings from least poor to poorest according to household possessions, education, and income sources (
; see also commentary, ibid: 540–1). Childhood illness was the norm (52% of under 5 year olds had been ill in the last 2 weeks) but not dependent on sex or socioeconomic status. But the least poor families were more likely to be aware of the signs of severe illness and to present their children to appropriate health care providers. As a consequence their children were more likely to receive antibiotics or antimalarials when they needed them. Programmes aimed at reducing inequities among children must reach out to those parents or carers least likely to act appropriately when their children are ill; a conclusion that may seem self-evident to many.
Adjuvant chemotherapy improves the otherwise poor outlook of children with Ewing’s sarcoma. Now a US multicentre trial (
; see also editorial, ibid: 747–9) has shown that, by alternating ifosfamide and etoposide with standard chemotherapy (doxorubicin, vincristine, cyclophosphamide, and dactinomycin), survival can be improved further. Among 398 patients without metastatic disease 5 year overall survival increased from 61% (standard chemotherapy) to 72% (alternating chemotherapy) and 5 year event-free survival from 54% to 69%. The alternating regimen did not benefit patients with metastases.
Are habits and tics related or are they different phenomena? Can a habit cough, for instance, be an isolated tic comparable to the vocal tics of Tourette’s syndrome? A 15 year old boy (
) had a 2 year history of repetitive and socially disruptive cough unresponsive to intensive treatments for asthma and for rhinitis, diagnoses made on apparently tenuous grounds. His “seal barking” cough was stereotyped (he would lean forward and cover his mouth with his hand) and did not occur in sleep. It stopped within hours of beginning treatment with pimozide 2 mg daily and a relapse one month later responded to the same treatment. He has now been free of his cough for 3 years. The authors of this report warn about the potential toxicity of pimozide including prolongation of the QT interval.
A study in five general practices in Somerset (
) has shown no benefit from adding individualised homeopathic medication to conventional treatment for children with mild or moderate asthma. Ninety-six children all had consultations with a homeopath who issued an individualised homeopathic prescription. They were then randomised centrally to receive either the prescribed homeopathic preparation or placebo. After 12 months there were no significant group differences in quality of life measured by age-appropriate Childhood Asthma Questionnaire or in other measures of asthma control.
In eosinophilic gastroenteropathy intestinal obstruction is caused by bowel wall thickening due to eosinophilic infiltration of bowel muscles. Now clinicians in London (
) have described three girls aged 1 month, 11 years, and almost 16 years who presented with severe functional intestinal obstruction and had eosinophilic infiltration of colonic lamina propria and myenteric plexus without bowel wall thickening (eosinophilic myenteric ganglionitis). Myenteric ganglion neurones expressed interleukin 5, a potent attractant of eosinophils, and there was no lymphocytic infiltration. The children did not respond to dietary exclusion but all three responded rapidly to steroid treatment. The youngest has received maintenance treatment with sulphasalazine and the two older children with azathioprine.
In newborn babies with hypoplastic left heart syndrome or multiple left heart obstructive lesions initial stenting of the ductus arteriosus and bilateral pulmonary artery banding may buy time to think about what to do next. In Germany (
) 20 neonates had percutaneous duct stenting and open pulmonary banding. Two died during these procedures; two more while waiting for a heart transplant, and one after a palliative one stage procedure. Two babies had a heart transplant and nine survived the palliative one stage procedure in which the aortic arch was reconstructed and a bidirectional cavopulmonary connection was established. This operation is planned for another patient. Two had biventricular repair and one is waiting for biventricular repair.