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The length of the gap between upper and lower oesophageal segments in oesophageal atresia is better discovered before rather than while operating. Surgeons in India have proposed a simple method (Journal of Pediatric Surgery1997;32:1453-4): 12 of 61 patients with tracheo-oesophageal fistula had long gap (more than two vertebrae) oesophageal atresia, and chest radiography showed that nine of these 12 patients—but none of the other 49—had 13 pairs of ribs. The suggestion is that having an extra thoracic vertebra stretches the gap between the oesophageal segments.
Passive smoking may increase the risk of meningococcal disease. A US case-control study (Pediatric Infectious Disease Journal 1997;16:979-83) found that, after statistical adjustment for confounding factors, maternal cigarette smoking was the strongest risk factor for invasive meningococcal disease in children, increasing the risk fourfold. Nearly 40% of cases were thought to be attributable to maternal smoking. There was a weaker association with paternal smoking.
Treatments aimed at subduing the inflammatory response in meningococcal septicaemia have generated much hope but little real joy. Now a protein found in the granules of neutrophils has shown some promise (Lancet 1997; 350:1439-43). Bactericidal/permeability increasing protein (BPI) inhibits the inflammatory response by binding to bacteria and to endotoxin, and is bactericidal to many Gram negative organisms. In a US study of 26 children with severe meningococcal septicaemia treated with an intravenous infusion of a recombinant N-terminal fragment of BPI (rBPI21) only one died. On the basis of clinical severity, laboratory data, and recent experience in the participating centres, it was calculated that between four and eight deaths might have been expected. A randomised trial has been organised.
Although retinopathy of prematurity (ROP) leads to myopia it has not been clear whether myopia is significantly more common in children who were born preterm but did not develop ROP. A study of 527 children aged 2 to 7 years in Singapore (Acta Ophthalmologica Scandinavica 1997;75:548-50) has shown no increase in either myopia or astigmatism in preterm or low birthweight children without ROP.
If a child has neonatal skin blisters and stridor, think of recessive epidermolysis bullosa simplex (EBS) with later onset of muscular dystrophy (British Journal of Dermatology1997;137:898-906). In dominant EBS, and some recessive forms, the defect appears to be in basal keratin genes, but in this rare recessive form mutations in the PLEC1 gene encoding plectin, a protein involved in cytoskeleton–plasma membrane attachment, has been found. Two unrelated children with EBS, a hoarse cry from birth, and stridor caused by supragottic obstruction from interarytenoid scarring, both had PLEC1 mutations.
Researchers in Canada cut short a placebo controlled trial of local anaesthesia for neonatal circumcision before recruiting the projected number of patients because of the evident distress in groups given topical placebo or EMLA cream rather than ring block or dorsal penile nerve block (Journal of the American Medical Association 1997;278:2157-62). Assessing heart rate and duration of crying, these workers concluded that the most effective anaesthetic was ring block. They did not consider EMLA cream to be adequately effective.
Two Japanese hospitals treated Perthes’s disease differently (Journal of Bone and Joint Surgery1997;79-B:979-82). One used a unilateral brace that maintained external rotation, flexion, and abduction, and the other used a bilateral cast that maintained internal rotation and abduction. Disease in the other hip appeared during treatment in six of 94 patients in the first hospital but in none of 105 patients in the second. It is suggested that maintaining abduction in the unaffected hip for about 10 months might prevent that hip becoming affected.
An American–European trial of lamotrigine in Lennox-Gastaut syndrome (New England Journal of Medicine 1997;337:1807-12) showed modest improvements. Over 16 weeks of treatment median weekly seizure frequency fell from 16.4 to 9.9 on the drug and one third of treated patients (and one sixth of placebo patients) had a 50% or more reduction in seizures. The effect on drop attacks and on tonic-clonic seizures was similar. The lamotrigine group had more colds and viral illnesses.
Causes of Horner’s syndrome in childhood include congenital varicella, birth trauma, tumours of the neck and mediastinum, and lesions of the internal carotid or subclavian arteries. Between 1978 and 1995 a neuro-ophthalmologist in San Francisco saw 23 cases presenting in infancy (British Journal of Ophthalmology 1998;82:51-4). No cause was found in 16, four had birth trauma, and one each had abdominal neuroblastoma, ganglioneuroma at the apex of the left lung, and cervical neuroblastoma. It is suggested that infants with isolated Horner’s syndrome should have urinary vanillylmandelic acid (VMA) measured but do not need computed tomography or magnetic resonance imaging of chest and neck. Such imaging should be reserved for those with known postnatal onset of Horner’s syndrome and those with other signs such as increasing heterochromia iridis, a cervical mass, or cranial nerve palsies.
In 1996 the family of an American child with microphthalmos was awarded US$4 million on the grounds that the defect might have been caused by benomyl, an antifungal agent used by farmers. A commentary in the British Journal of Ophthalmology (1998;82:67) reminds us that several clusters of microphthalmia/anophthalmia have occurred in Britain, some in agricultural areas, and defects including microphthalmia/anophthalmia have been produced in the offspring of rats given benomyl in pregnancy. The agent has been banned in Australia and there is considerable interest in knowing the effect of that ban on the incidence of microphthalmia/anophthalmia. In the meantime it appears that the “don’t knows” remain scientifically correct.
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