Anti-neutrophil cytoplasmic antibody for proteinase 3 in a child with polyarteritis nodosa

We report a case of a 15 year old boy with polyarteritis nodosa associated with antineutrophil cytoplasmic antibody (ANCA) against proteinase 3 (PR3). After months of steroid and immunosuppressant therapy, the symptoms subsided and the polyaneurysms almost disappeared. The levels of anti-PR3 antibody and of cytokines also decreased. The results indicate that ANCA is a good indicator of this disease activity, and may play some pathogenic role in the disease.     

EFFECT OF FUSARIC ACID (A DOPAMINE β-HYDROXYLASE INHIBITOR) ON PHAEOCHROMOCYTOMA

Fusaric acid, an inhibitor of dopamine beta-hydroxylase, which converts dopamine to noradrenaline, lowered the blood pressure and induced a subjective improvement in patients with phaeochromocytoma. These effects may be due either to an impairment of catecholamine biosynthesis or to a direct action on the blood vessels. The use of this drug in the treatment of patients with inoperable malignant phaeochromocytoma or neuroblastoma may improve symptoms and prolong survival.     

The morphology and hardness of the nail in two cases of congenital onychoheterotopia

Two cases of congenital onychoheterotopia (ectopic nail) are described. In the first case electron microscopy showed spherical keratohyalin granules, and cytoplasmic desmosomes were seen in the keratinocytes of the nail matrix. The ectopic nail differed from the normal nail in the widening of the angle between the nail matrix and proximal nail fold, and in its radial growth to the skin surface. In the second case, clippings of the ectopic nail were taken and their hardness proved to be very similar to that of normal nails.