Cholecystokinin and Cystic Fibrosis

Jansen, J.B.M.J.; Jongerius, C. M.; Lamers, C. B. H. W.

doi:10.1016/s0140-6736(85)91018-9

Cited by 3 publications

(1 citation statement)

References 4 publications

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“…We are currently studying fasting and postprandial plasma CCK concentrations in our CF patients to verify whether low levels of CCK or reduced sensitivity of the target organ could be associated with larger GB volumes and/or with the most atonic GBs, as observed in a subgroup of patients with CF. This is of particular interest in view of the recent (although controversial) suggestion that the basic defect in CF may be related to the deficiency of specific arginine esterase, which cleaves CCK to its active octapeptide, CCK-8 (13).…”

Section: Discussionmentioning

confidence: 99%

Volume and Emptying of the Gallbladder in Patients with Cystic Fibrosis

Santamaria,

Vajro,

Oggero

et al. 1990

J. pediatr. gastroenterol. nutr.

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SummaryAs in cystic fibrosis (CF), gallbladder (GB) hypokinesia might play a role in the high incidence of either cholelithiasis or atrophic and hypoplastic GBs. We studied the contractility of the GB by ultrasonography (US) in 57 CF patients and in 25 age‐ and sex‐matched healthy controls. We evaluated the status of the GB, the GB volume after an overnight fasting and 30 min after the ingestion of a standardized milk meal, the GB contractility (the percentage of reduction, (%R) of the GB volume after a meal), the presence of gallstones, and finally, the span of the liver. In addition, liver function test results were evaluated in the CF group. We concluded that the mean values of GB contractility in CF patients do not differ from those of healthy controls, the most hypokinetic GBs (%R < 40%) being observed, however, only in the CF group. Gallstones occur in CF patients with a greater frequency than seen in the normal population, even in early childhood. The CF patients' GB mean volumes in fasting conditions are significantly greater than in healthy controls (13.5 ± 2 versus 8.5 ± 1.5 cc, p < 0.0001).

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Section: Discussionmentioning

confidence: 99%

Volume and Emptying of the Gallbladder in Patients with Cystic Fibrosis

Santamaria,

Vajro,

Oggero

et al. 1990

J. pediatr. gastroenterol. nutr.

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Clinical Pharmacology of Antibiotics and Other Drugs in Cystic Fibrosis

Prandota

1988

Drugs

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The disposition of many drugs in cystic fibrosis is abnormal. In general, changes in pharmacokinetics include: increased volume of distribution, decreased plasma concentration, and enhanced renal and sometimes non-renal elimination of drugs. Pathophysiology of the disease important for drug disposition includes: (a) hypersecretion of gastric acid and duodenal secretions which are of small volume, viscous and low in bicarbonate; (b) increased intestinal permeability to some sugars and probe substances; (c) hypergammaglobulinaemia and sometimes hypoalbuminaemia; (d) significant elevation of free fatty palmitoleic acid level and decreased low-density and high-density serum lipoproteins; (e) an average increase by 30 to 45% in plasma volume in patients with cystic fibrosis who have moderately severe pulmonary disease, right ventricle hypertrophy and dilatation, which occurs in 15 to 35% of patients with a Shwachman score of 81 to 100; (f) abnormal bile acid metabolism and enterohepatic recirculation; and (g) enlarged kidneys and glomerulomegaly with increased glomerular filtration rate, tubular clearance and urine flow rate in some patients with cystic fibrosis. Delayed absorption from the gastrointestinal tract has been reported in patients with cystic fibrosis for cloxacillin, epicillin, clindamycin, ciprofloxacin and probably for cephalexin, para-aminobenzoic acid and chloramphenicol. A possible increased absorption was reported for cimetidine. Of 7 drugs studied only theophylline had significantly decreased plasma protein binding. An increased volume of distribution and increased renal clearance reported for several drugs is caused mainly by increases in plasma volume and urine flow rate in many of these patients. Possible increased elimination of some drugs in bile (which probably results from bile acid malabsorption) and in bronchial secretions (which are abundant in some cystic fibrosis patients with acute pulmonary infection) may explain enhanced non-renal elimination of these drugs. The metabolism of cimetidine in cystic fibrosis was reported not to be changed significantly compared to control subjects.

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