drugs may not be desirable because of pre-existing intracardiac
MATERNAL EFFECT IN CENTRAL NEUROFIBROMATOSIS
conduction detects.
J. K. VOHRA Departments of Cardiology Royal Melbourne Hospital, Victoria 3050, Australia
and
G. D. BURROWS I. MCINTYRE B. DAVIES
Psychiatry,
FATTY-ACID COMPOSITION OF SERUM-LECITHINS IN CYSTIC-FIBROSIS PATIENTS WITHOUT STEATORRHŒA
SIR Di Sant’Agnese’s team’ found that the fatty-acid composition of various plasma-lipid fractions from cysticfibrosis (c.F.) patients without pancreatic insufficiency did not differ significantly from normal; only c.F. patients with pancreatic
insufficiency had significantly abnormal fatty-acid composition. We have investigated the fatty-acid composition of serum-lecithins from four groups of C.F. patients, defined by their pancreatic function evaluated by 72-h quantitative stool fat and determination of the coefficient of fat absorption. All patients were on a normal diet. PERCENTAGE COMPOSITION
(MEAN±S.E.M.) OF
SELECTED FATTY
ACIDS IN SERUM-LECITHINS
SIR,—Miller and Hall’ have found a maternal effect in peripheral neurofibromatosis (von Recklinghausen’s disease), a disorder inherited as an autosomal dominant trait and characterised by tumours of the nerve sheath and by macular hyperpigmentation. Reviewing 62 cases of peripheral neurofibromatosis, they observed significantly higher morbidity among cases born to affected mothers than in those born to affected fathers or to sporadic cases. To address the question of a maternal effect in the central form of neurofibromatosis, the hallmark of which is bilateral acoustic neuroma,2we compared the mean age of onset for 25 cases born to affected mothers with the mean for 13 born to affected fathers. Patients with affected mothers had a mean (±S.E.M.) age of onset of 18.3 ±1-4 years while the mean age of onset in patients with affected fathers was 24.1 +2.2 years (P