HYPERTENSION IN CHILDREN WITH NEUROFIBROMATOSIS

Hypertension in neurofibromatosis is mostly a consequence of a stenosi s of the renal artery or is due to phaeocromocytoma. Riccardi pointed out primary hypertension in patients with several cervical neurofibrom as in the absence of phaeocromocytoma and he noticed that the elevatio n of BP was often already present in children. Nine (15.8%) of 57 neur ofibromatosis patients (age from 1.5 to 23 years) examined, presented BP levels above the 95th percentile on several occasions and three in particular had severe hypertension with compromised target organs. Two of them had a stenosis of the renal artery, in the third an organic o rigin of hypertension was not demonstrated, but there was an asymptoma tic glioma of the hypothalamus. The other six children had a labile or borderline hypertension and two of them had, respectively, a glioma o f the thalamus and of the optical chiasm. Elevation of the catecholami ne metabolites or other causes of hypertension were not found in any o f these patients. These preliminary data show a high incidence of hype rtension in neurofibromatosis, primary or due to organic causes and ov erall they point out a possible correlation between hypertension and c erebral neoplasia.