The prevalence of peripheral nerve dysfunction was assessed in 24 randomly selected adult patients with cystic fibrosis. Median, peroneal and sural nerves were studied. In 15 patients (62%), one or more electrical abnormality were detected. In the median nerve, conduction velocity was slowed in 29% of sensory studies and 12.5% of motor studies; compound muscle action potential (CMAP) and sensory nerve action potential (SNAP) amplitudes were normal. The CAMP was low in 17% of peroneal studies with motor conduction velocity slow in 8%. The sural SNAP was low in 17% and sensory nerve conduction velocity slow in 25%. Using a multiple regression analysis only age at diagnosis was identified as a significant predictor of peripheral nerve dysfunction (multiple R = 0.55; p < 0.01). This study demonstrates that mild peripheral nerve dysfunction is common in patients with cystic fibrosis. With the improving long term survival of these patients we predict that the prevalence and severity of this complication will increase.

Action Potentials↗

PubMedDate not supplied

Diabetes mellitus in an adult cystic fibrosis population.

Medical records of 132 patients attending an adult cystic fibrosis (CF) clinic were analysed to define the prevalence and clinical significance of diabetes mellitus (DM) in CF. Eighty four (63.6%) had normal blood glucose levels, 30 (22.8%) had hyperglycaemia only during intercurrent illness and 18 (13.6%) had DM. No significant differences were noted between the diabetic and non-diabetic groups for age, gender, height, weight, body mass index (BMI), forced expiratory volume in one second (FEV1) and forced vital capacity (FVC) and pancreatic supplementation. Patients with hyperglycaemia during intercurrent illness had significantly lower BMI, FEV1% and FVC% than those with normal blood glucose levels. Of the diabetics four were managed on diet, three received oral hypoglycaemic agents and eleven were insulin requiring. The prevalence of DM in CF is considerable, severity of CF does not correlate with development of overt DM, and CF patients should be screened for DM by an oral glucose tolerance test on reaching adulthood.

Adult↗