Cystic fibrosis continued

A worldwide survey of cystic fibrosis centers, including four in the United States [13], indicated that the use of aerosolized antibiotics was being considered on an increasing basis. In the survey, suppressive therapy was more likely to be used in older age groups (> 19 years), with half the centers surveyed indicating that they were prescribed in at least 50% of their patients. Although this survey is relatively recent, it is likely this trend has continued to increase and has extended to younger patients, and additional evidence has likely been reported. 

The mechanism by which cholera toxin causes secretory diarrhea is through continuous stimulation of the CFTR-regulated CL channel (Figure 12-15). In cystic fibrosis, CFTR defects cause the abolition of intestinal chloride secretion without affecting the absorptive capacity. In homozygous CF patients, the disease is eventually lethal (discussed earlier). In cholera infections, however, CFTR is overactivated with fluid and electrolyte losses that lead to... 

The side effects inherent to some of these drugs used as lifelong treatment must be taken into account. Also, reduced gastric acidity eliminates a natural physical protective barrier and may cause, with continuous use, bacterial overgrowth [91] and gastroenteral infections [92]. This would be especially problematic for cystic fibrosis patients, who, because of the nature of their disease, are more susceptible to intestinal infections. 

Within the Aviptadil and bacteriophage programs, mondoBIOTECH is determined to obtain - as soon as possible - marketing approvals for the indications of pulmonary arterial hypertension (Aviptadil) and cystic fibrosis (bacteriophages). The company will also continue to seek additional opportunities for the TheraNostics concept. 

Vinks, A.A.T.M.M. Touw, D.J. Heijerman, H.G.M. Danhof, M. de Leede, G.P.J. Bakker, W. Pharmacokinetics of ceftazidime in adult cystic fibrosis patients during continuous infusion and ambulatory treatment at home. Ther.Drug Monit., 1994,16, 341-348 [pharmacokinetics serum urine sputum LOD 500 ng/mL 8-chlorotheophylline (IS)]... 

The administration of a diet restricted in sodium chloride induces a normal response of the kidney and reduced electrolytes in urine, but the sweat glands continue to excrete excessive amounts of electrolytes. As a result, patients with cystic fibrosis are oversensitive to heat in that they lose large amounts of sodium chloride in the sweat. The loss of sodium leads to a reduction of the extracellular fluid with vascular collapse. The situation is readily reversed by the intravenous administration of saline [139]. 

Stored ester reserves. It should be noted that there are reports indicating that continued dietary supplementation of alcoholic cirrhosis (Mobarhan et al.y 1981) or cystic fibrosis (Fulton et al, 1982) patients with vitamin A in high doses does not always cause a sustained rise in plasma levels, though it may correct abnormalities in dark adaptation. These findings indicate that in these subjects with diseased livers, an impaired RBP synthetic rate is a major contributing factor to low circulating levels of retinol. Furthermore, alcohol per se has been shown in baboons and rats to increase the rate at which retinol is catabolized by hepatic tissue (Sato and Lieber, 1981) and some data from rats suggest that alcohol may potentiate the sensitivity of tissues to vitamin A, even in the presence of normal blood levels (Leo and Lieber, 1982). 

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