Summary Serum dopamine-fl-hydroxylase (DBH) activity has been reported to be raised in some patients with neuoblastoma but this has been challenged. We have studied serum DBH levels on 26 children with neuroblastoma and 58 age-matched controls. Only in 2 patients were the levels higher than in the controls, and then only transiently. In both, the rise in DBH levels could be accounted for by the transfusion of adult blood. Serum DBH levels in children with neuroblastoma were unrelated to the response of this neoplasm to treatment or to urinary catecholamine output and thus are unlikely to have any value in diagnosis or as a marker of tumour activity.The main pathways by which catecholamines are synthesized and metabolised are shown in Figure 1. The enzyme DBH catalyses the last step in the biosynthesis of noradrenaline and is found in chromaffin tissue and in the synaptic vesicles of sympathetic tissue. In vitro studies demonstrated the coupled proportional release of noradrenaline and DBH from sympathetic nerves by a process of exocytosis . The main source of serum DBH in the rat is the adrenergic neuron whence it is discharged during sympathetic activity Weinshilboum, 1978). There is very little information available on the source, half-life, and fate of human serum DBH (Weinshilboum, 1978).Neuroblastomas are composed of primitive cells derived from the neural crest. They form and discharge noradrenaline and its precursors DOPA and dopamine. These substances are metabolised both within the tumour and elsewhere, and the metabolites, together with some free catecholamines are excreted in excess in the urine. Tumours producing predominantly noradrenaline and its metabolites have a more favourable prognosis (Gitlow et al., 1973;Laug et al., 1978). However, the measurement of these metabolites in urine is timeconsuming and the common methods lack specifity. Since in vitro noradrenaline production is associated with DBH release, elevated serum DBH levels may be expected to occur in children with neuroblastoma, and, if present, carry diagnostic and prognostic value. The spectrophotometric assay of serum DBH utilizes optimum conditions for measurement of enzyme activity. It is specific, relatively quick and requires only small volumes of serum, making it suitable for routine use in the hospital laboratory (Weinshilboum, 1978). Elevated serum DBH levels have been reported in children with neuroblastoma Rockson et al., 1976), but a recent study has challenged these observations (Brewster & Berry, 1979).We have studied serum DBH levels of neuroblastoma patients and age-matched controls and have analysed the results in relation to age, clinical status, and urinary catecholamine output.
Patients and methods
PatientsFourteen boys and 12 girls with neuroblastoma, aged from one month to 8 years were studied. Serum DBH and urinary catecholamine excretion were measured, in some patients serially. Clinical staging was performed using the method of Evans et al. (1971) and the clinical status of each patient was recorded.The contro...