Epidermolysis bullosa acquisita (EBA) is an acquired subepidermal bullous disease characterized by IgG autoantibodies directed against type VII collagen, the major component of anchoring fibrils. The classical phenotype of EBA is a non-inflammatory, mechanobullous disease resembling the dystrophic forms of inherited epidermolysis bullosa. Mucous membrane involvement is frequent but usually mild. We report a 1-year-old girl suffering from IgA-EBA, who presented with an initial eruption of disseminated urticarial lesions and tense blisters of the skin but subsequently developed severe oral and ocular lesions reminiscent of cicatricial pemphigoid. Direct immunofluorescence of the skin and buccal mucosa revealed linear IgA and C3 at the basement membrane zone (BMZ). IgA anti-BMZ autoantibodies stained the dermal side of salt-split skin by indirect immunofluorescence and recognized a dermal protein of 290 kDa co-migrating with type VII collagen by immunoblotting. Direct and indirect immunoelectron microscopy revealed IgA deposits overlying the anchoring fibrils. The ocular involvement led to total blindness in spite of intense treatment. This case of childhood IgA-EBA is particularly striking because of the cicatricial pemphigoid phenotype with severe ocular involvement which resulted in blindness. It reinforces the necessity to use modern immunological methods to classify autoimmune bullous diseases in order to allow early and appropriate treatment.
In order to compare the Maximal Aerobic Speed (MAS) evaluated with different methods, eleven male physical education students (22.2 +/- 3.0 years) were submitted to a maximal treadmill protocol and to the Université de Montréal Track Test (UMTT). Four methods were used to calculate MAS. After treadmill measurement of VO2max, MAS was calculated (MAS_calc) by the following formula: MAS_calc = (VO2max - 0.083)/C, where VO2max is the maximal oxygen uptake (ml.kg-1.s-1) and C the energy cost of running (ml.kg-1.m-1). The extrapolated MAS (MAS_ex) was obtained from the measured VO2max and by extrapolation of the VO2 versus speed relationship. The MAS for treadmill measurement (MAS_tr) and for UMTT (MAS_UMTT) were the velocities at the last completed stages. The average MAS_calc (4.71 +/- 0.48 m.s-1), MAS_ex (4.62 +/- 0.48 m.s-1), MAS-tr (4.75 +/- 0.57 m.s-1) and MAS_UMTT (4.64 +/- 0.35 m.s-1) were not significantly different and were significantly correlated, between 0.85 (MAS_ex vs MAS_UMTT) and 0.99 (MAS_calc vs MAS_tr), with p < 0.001 in both cases. MAS measurements were significantly correlated to measured VO2max but independent of C.
Over the past few years, many studies, including one on our previous work, have examined the chronic effects of fumes from stainless steel (SS) welding on the health of welders. These chronic effects have been related to concentrations of chromium and nickel in SS welding fumes. The present study examined the acute respiratory effects of welding fumes in the workplace by measuring the across-shift changes in a population of 144 SS and mild steel (MS) welders and 223 controls. Manual Metal Arc, Metal Inert Gas, and Tungsten Inert Gas welding processes were studied. Pulmonary function tests were performed at the start (ante, or A) and at the end (post, or P) of the work shift. The study of sensitization to harmful respiratory effects of welding was based on the study of the (P-A)/A ratio (%) of the spirometric variations during the shift. The means of these ratios in the control subjects were used to account for the circadian effect. In SS welders we observed a significant decrease in forced vital capacity (FVC) during the shift. Significant across-shift decrements in forced expiratory volume in 1 second (FEV1) and FVC were related to the SS welding exposure compared with MS welding. Moreover, the across-shift decreases in FEV1, FVC, and peak expiratory flow (PEF) were significantly related to the Manual Metal Arc welding process, compared with Metal Inert Gas techniques (respectively, PEF = -2.7% of baseline values [SD, 11.9] vs 2.0% of baseline values [SD, 7.7] P = 0.04; FVC = -1.5% of baseline values [SD, 4.8] vs 0.2% of baseline values [SD, 4.5] P = 0.05). We also demonstrated the influence of duration of SS welding exposure on the course of lung function during the work shift. After 20 years of SS welding activity, SS welders had more significant across-shift decreases than MS welders with a similar MS exposure duration (respectively, FEV1 = -2.7% of baseline values [SD, 5.9] vs 0.7% of baseline values [SD, 4.2] P = 0.008; PEF = -3.8% of baseline values [SD, 9.6] vs 2.3% of baseline values [SD, 6.5] P = 0.04). We concluded that welding-related lung function responses are seen in SS compared with MS welders and in those with a longer lifetime welding history.
Aim: To compare plasma lactate concentration recovery kinetics when measured and corrected for variations in plasma volume between children and adults. Methods: Nine boys (11.3 AE 1.1 y) and 8 men (21.9 AE 1.9 y) performed a maximal and a supramaximal exercise until exhaustion. Plasma lactate concentrations, haemoglobin and haematocrit were measured at rest, immediately on completion of exercise and after the 2nd, 5th, 12th and 30th minute of recovery. The plasma lactate concentrations and the rate of recovery were corrected for variations in plasma volume. Results: The maximal decreases in plasma volume were significantly higher in adults than in children for maximal exercise (À18.7 AE 2.6% vs À14.5 AE 3.2%; p < 0.05), but similar for the supramaximal exercise (À16.9 AE 3.4% vs À15.2 AE 3.4%). During recovery, measured and corrected plasma lactate concentrations were significantly higher in adults. The rate of plasma lactate recovery was higher in adults for maximal exercise only. The same results were obtained when the rates of plasma lactate decrease were calculated from corrected plasma lactate concentrations. Conclusion:The correction of the plasma lactate concentrations for variations in plasma volume did not influence the comparison of the concentrations obtained in adults and children, or their rate of recovery.
The aims of the present study were: first, to assess the interindividual variations of a spontaneously chosen crank rate (SCCR) in relation to the power developed during an incremental upper body exercise on an arm ergometer set at a constant power regime, and second, to compare heart rate (HR) responses, expired minute ventilation (V[E]) and oxygen consumption (VO2) when the pedal rates were chosen spontaneously (T[SCCR]) or set at +/- 10% of the freely chosen rates (T[+10%] and T[-10%], respectively). The mean pedal rate values were linearly related (P < 0.01) with the power developed during arm cranking (r = 0.96), although large variations of pedalling rate strategies were observed between subjects. Maximal power (MP) and time to exhaustion values were significantly higher (P < 0.05) during T(SCCR) than during T(+10%) and T(-10%). Peak VO2 values were significantly higher (P < 0.05) in T(+10%) than in T(SCCR) and T(-10%). The increase in HR, V(E), and VO2 mean values, in relation to the increase in the power developed, was significantly higher (P < 0.05) when the pedal rate was set at plus 10% of the SCCR (T[+/-10%]) than in the two other conditions. The findings of the present study suggest that the use of an electromagnetically braked ergometer, which automatically adjusts the resistance component to maintain a constant work rate, should be used in order to achieve the highest MP values during an incremental upper body exercise. A 10% increase of the SCCR should be used in order to provide the highest peak VO2 value.
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