A postsynaptic dysfunction of the neuromuscular junction has been reported in patients with alpha-dystroglycanopathy associated with mutations in guanosine diphosphate (GDP)-mannose pyrophosphorylase B gene (GMPPB), some of whom benefit from symptomatic treatment. In this study, we determine the frequency of myasthenic and fatigue symptoms and neuromuscular junction transmission defects in a fukutinrelated protein (FKRP)-predominant alpha-dystroglycanopathy cohort. Thirty-one patients with alpha-dystroglycanopathies due to mutations in FKRP (n = 25), GMPPB (n = 4), POMGNT1 (n = 1), and POMT2 (n = 1) completed a six-question modified questionnaire for myasthenic symptoms and the PROMIS Short Form v1.0-Fatigue 8a survey, and they underwent 3 Hz repetitive nerve stimulation of spinal accessory nerve-trapezius and radial nerve-anconeus pairs. Results showed that fatigue with activity was common; 63% of the cohort reported fatigue with chewing. A defective postsynaptic neuromuscular junction transmission was not identified in any of the patients carrying FKRP mutations but only in one mildly affected patient with GMPPB mutations (c.79 G > C, p.D27H and c.402 + 1G > A, splice site variant). We conclude that symptoms of fatigue with activity did not predict abnormal neuromuscular junction transmission on electrodiagnostic studies in this cohort and that, unlike GMPPB subgroup, a defective neuromuscular junction transmission does not appear to be present in patients with FKRP-associated muscular dystrophies.

The effect of different concentrations of fetal bovine serum (FBS) on the growth and survival of three insect cell lines—Aedes aegypti, Antheraea eucalypti, and Bombyx mori—was studied in a series of 10 experiments. Cell viability was low and no growth occurred in any of the cell lines when FBS was omitted from the medium. Maximum growth of A. aegypti cells was obtained with 10% FBS. There was no further increase in this growth when FBS was increased to 20 and 30%, and neither did the increased concentrations have any appreciable adverse effect on the growth or survival of these cells. Maximum growth of A. eucalypti and B. mori cells was obtained in 5% FBS; the growth of these cells was significantly less in 20 and 30% FBS. Viability of A. eucalypti cells was quite low in 20 and 30% FBS. There was, however, no adverse effect on the viability of B. mori cells at these high concentrations.

Summary: Glycosylated haemoglobin (HbAl), as an assessment of overall control, was estimated in 858 insulin-treated diabetics attending for changeover to U100 insulin in North East Wales. Details of age, sex, duration of diabetes, insulin regimen and monitoring method were recorded. Multivariate analysis revealed lower HbAl in patients receiving twice-daily (mean 11.01) as compared with once-daily insulin (1 1.42, P= 0.01 1), but no difference was found between urine and blood glucose testers when other variables were taken into consideration. HbA1 was lower in men (mean 10.94) compared with women (mean 11.39, P=0.004) and there was a positive correlation of HbA1 in men with age (P=0.046) and in women with duration of disease (P = 0.005).

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