903

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

Plasmapheresis in severe asthma.

903 drugs may not be desirable because of pre-existing intracardiac MATERNAL EFFECT IN CENTRAL NEUROFIBROMATOSIS conduction detects. J. K. VOHRA D...
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