Multicentre study of piroxicam versus naproxen in juvenile chronic arthritis, with special reference to problem areas in clinical trials of nonsteroidal anti-inflammatory drugs in childhood.

Clinical trials of nonsteroidal anti-inflammatory drugs (NSAIDs) are necessary in juvenile chronic arthritis (JCA) but pose certain problems highlighted and discussed in this study, including recruitment, the assessment of efficacy, and the heterogeneity of the disease. In a multicentre 8-week double-blind cross-over study using the double-dummy technique, piroxicam was compared with naproxen in 47 children with seronegative JCA aged 5-16 years. No significant difference between the two treatments was found in either the clinical variables measured or the parent/patient and physician preference at the end of the study. Side-effect profiles of the two drugs were similar, mainly gastrointestinal disturbances. Piroxicam may be a useful alternative NSAID in JCA, particularly in view of its once-daily dosage.

Long-term evaluation of naproxen suspension in juvenile chronic arthritis.

Twenty-six children suffering from juvenile chronic arthritis were entered into a 6-month open evaluation of naproxen suspension. Sixteen patients completed 6-months' treatment, 6 were lost to follow-up and 4 dropped out, 2 because of side-effects (rash, and burning on swallowing the formulation), 1 for lack of efficacy and 1 because of no further need of treatment. Pain severity scores were significantly reduced from admission levels at all monthly follow-up visits and significant overall improvement since the last visit was noted up to third month of treatment, as assessed by doctor and parent/patient. Transient indigestion was reported by 2 patients. No clinically significant trends were noted in any of the laboratory investigations carried out. The results add to the accumulation of data on the use of naproxen in children and underline its long-term efficacy and tolerance.

Primary amoebic meningoencephalitis.

The third case in the UK of primary amoebic meningoencephalitis is reported; it affected an 11-year-old girl. Six days before admission the girl had swum in a pool fed by hot spring water in which the causative agent Naegleria fowleri was found. Early treatment with amphotericin B would seem to offer the only hope of recovery in this almost uniformly fatal infection.

Renal tubular acidosis and nerve deafness.

Two brothers are described with renal tubular acidosis and nerve deafness: the elder also had rickets and hypokalaemia. The parents were unaffected. Studies of urinary acidification and bicarbonate excretion were consistent with a distal tubular abnormality. This report strengthens the view previously proposed in similar cases that nerve deafness and renal tubular acidosis constitute a genetic entity. Examination for nerve deafness is indicated in any child with renal tubular acidosis.

High liver glycogen in hereditary fructose intolerance.

A case of hereditary fructose intolerance is reported in a girl aged 2 years at the time of her death. She had apparently progressed normally until the age of 14 months. At 19 months she was admitted to hospital with failure to thrive, hepatomegaly, and superficial infections. Investigations revealed hypoglycaemia, persistent acidosis, aminoaciduria, and a high liver glycogen level which suggested that she had glycogen storage disease. There was also some evidence of malabsorption. At necropsy the liver enzyme estimations showed that fructose 1-phosphate aldolase activity was absent and that fructose 1,6-diphosphate aldolase activity was reduced. Hereditary fructose intolerance and glycogen storage disease have been confused in the past on clinical grounds, but a high liver glycogen level has not previously been reported in hereditary fructose intolerance.