Alpha-1-antitrypsin deficiency in a large sibship.

We studied alpha 1-antitrypsin deficiency in a large family of 10 siblings: 3 subjects had PiZZ phenotype, but only 1 had emphysema; 2 subjects had no respiratory complaint. The patient with emphysema was a heavy smoker. According to the literature, this case suggests that, in PiZZ phenotype, emphysema appears earlier and is more severe if the patients smoke.

Evolution of pulmonary haemodynamics in COLD patients under long-term oxygen therapy.

The North-American Trial (NOTT) and the British MRC study clearly indicated that long-term oxygen therapy (LTO) increases the survival rate of patients with severe chronic obstructive long disease (COLD), but O2 must be given for more than 15 h/24 h. At present OT is largely prescribed in COLD patients whose (stable) PaO2 less than or equal to 55 mmHg. The pulmonary haemodynamic effects of OT are still a matter of debate.

Prognostic value of pulmonary artery pressure in chronic obstructive pulmonary disease.

In 175 patients with chronic obstructive lung disease (157 chronic bronchitic and 18 emphysematous patients) exhibiting moderate to severe airway obstruction (mean FEV1/vital capacity = 40.2 +/- 11.1%), cumulative survival rates calculated by the actuarial method were compared in subgroups according to the initial level of mean pulmonary artery pressure, pulmonary volumes, and arterial blood gases.

[The prognostic significance of pulmonary arterial hypertension in chronic obstructive airway disease (author's transl)].

In 175 cases of chronic obstructive airway disease (most often chronic bronchitis) with severe ventilatory deficit in the majority of cases, the prognostic value of mean pulmonary arterial pressure (PAP) and other respiratory function parameters (FEV1, blood gases) was studied by calculation of survival rates using the actuarial method. The patients were cathetherised between 1968 and 1972 and the maximum period of follow-up is 10 years. The results demonstrated the definite prognostic value of PAP : 4 and 7 year survival rates were markedly different according to whether initial PAP was above or below 20 mmHg.

Course of pulmonary hemodynamics in patients with chronic obstructive pulmonary disease.

Eighty-five patients with chronic obstructive pulmonary disease, mainly chronic bronchitis (71 patients), who had arterial hypoxemia and moderate to severe obstruction of the airways underwent at least two right cardiac catheterizations in a clinical steady state, with a delay of three years or more between the first and the last catheterization. The average delay was 60 +/- 19 months (range, 36 to 119 months). Patients were regularly examined (quarterly clinical and functional checkups).

Hormonal stimulation of intestinal brush border enzymes release.

The rat small bowel was perfused in vivo and ex vivo in the absence of biliary and pancreatic secretion. Intraluminal release of sucrase, alkaline phosphatase, aminopeptidases and enterokinase was significantly increased after administration of pentagastrin, caerulein and glucagon at doses ranging between 1 pg and 10 microgram. This suggests that there is a direct hormonal stimulation of the intestinal mucosa.

Prostaglandins E1 and E2 stimulate release of intestinal brush border enzymes.

Rat small bowel was perfused in vivo and ex vivo in the absence of biliary and pancreatic secretion. Intraluminal release of sucrase, alkaline phosphatase, aminopeptidase and enterokinase was significantly increased after administration of PG E1 and E2 1 and 5 microgram/kg. This suggests a direct stimulation of the intestinal mucosa, which might be mediated through cyclic AMP; dibutyryl cAMP significantly stimulates intraluminal release of proteins, sucrase and enterokinase.

Ex vivo vascular perfusion of the isolated rat small bowel. Importance of the intestinal brush border enzyme-release in basal conditions.

The vascular perfusion of the whole isolated intestine of the rat with homologous non-diluted blood is compared with classical in vivo experiments. During a 2 1/2-hour perfusion procedure no significant difference was noted between the two experimental schedules as far as haemodynamic, histological, mechanical and/or electrical activities of the gut are concerned. However, striking changes in the intestinal brush border enzyme output within the lumen appear between ex vivo and in vivo experiments.