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At least 1,099 records · Page 61Linked to original sources
PubMed1978

PABA screening test for exocrine pancreatic function in infants and children.

P-Amino-benzoic acid (PABA) is split specifically by pancreatic chymotrypsin from the synthetic tripeptide N-benzoyl-L-tyrosyl-PABA. The urinary excretion of absorbed PABA serves as an index for exocrine pancreatic function. The peptide (0.015 g/kg) was administered orally to 20 controls (aged between 5 months and 16 years), 6 patients with exocrine pancreatic insufficiency caused by cystic fibrosis (CF), and 9 newborn infants. In the controls the mean 6-hour PABA recovery was 58.5% (+/- 11.2 SD). Recovery in patients with CF was lower (P less than 0.001) with no overlap. In newborn infants the mean 6-hour PABA recovery was 23.4 (+/- 17.7 SD); overlapping in 3 instances with the results in CF patients. This simple, noninvasive test thus appears promising and merits further investigation in younger infants, especially newborns.

4-Aminobenzoic Acid↗
PubMed1993

Dual marker one day pancreolauryl test.

There is a pressing need for a simple non-invasive test of exocrine pancreatic function for use in children. The pancreolauryl test has been modified by the addition of a second marker (mannitol) to achieve a single day test without the need for two timed urine collections. Six healthy subjects and nine patients with cystic fibrosis were studied. Fluorescein, fluorescein dilaurate, and mannitol were taken by mouth, alone or in combinations, followed by 10 hour urine collections in two hourly aliquots to study the comparative pharmacokinetics of these markers. Urinary fluorescein was determined spectrophotometrically and urinary mannitol enzymatically. When fluorescein dilaurate and mannitol were taken together and the results expressed as ratios of percentage fluorescein to percentage mannitol recovery (F:M ratio) (mean (SD)) there was clear discrimination between healthy subjects and those with cystic fibrosis regardless of enzyme replacement treatment (57.3 (18.2) v 3.4 (1.4) v 3.2 (1.6) respectively). The differences in F:M ratios reached statistical significance in urinary aliquots collected between two and eight hours after marker ingestion. This single day tubeless test will greatly simplify the investigation of the child with suspected exocrine pancreatic dysfunction.

Administration, Oral↗
PubMed1988

The reserpine-treated rat as an experimental animal model for cystic fibrosis: abnormal Cl transport in pancreatic acinar cells.

Pancreatic acini of control and reserpine-treated rats were incubated with the isotopic tracer 36Cl to compare Cl accumulation in the absence and presence of secretagogues and transport inhibitors. Two phases of Cl accumulation were ascertained in resting control cells: an initial rate (0-5 min) and a steady state level (10-30 min) of accumulation. Both phases were enhanced by acetylcholine (1 microM) and caerulein (10 nM), but not by 10 nM vasointestinal peptide or 10 microM forskolin. Exposure to 1 mM DIDS (4,4'-diisothiocyano-2,2'-stilbene disulfonic acid) inhibited both phases of Cl accumulation, whereas exposure to 1 mM amiloride had a delayed effect on the initial rate and reduced the steady state phase in both resting (unstimulated) or acetylcholine-stimulated cells. Furosemide (1 mM) had no effect on Cl accumulation when added to the cells just before tracer, but reduced it when added 10 min before. Neither the initial phase nor the steady state level of Cl accumulation were enhanced by acetylcholine in acini of reserpine-treated rats and the effect of DIDS on the initial phase was smaller than in control cells. Continued exposure to this inhibitor resulted, furthermore, in a significantly larger steady state Cl content. The inhibitory effects of amiloride and of a 10-min preincubation with furosemide were similar to those observed in control cells. These results suggest that Cl accumulates in rat pancreatic acini by way of DIDS-sensitive mechanisms that are activated by Ca2+-mediated, but not by cAMP-mediated, secretagogues. These mechanisms are altered in acini of reserpine-treated rats.(ABSTRACT TRUNCATED AT 250 WORDS)

4,4'-Diisothiocyanostilbene-2,2'-Disulfonic Acid↗
PubMedDate not supplied

Control of malabsorption in cystic fibrosis.

Intestinal malabsorption is severe and of early onset in virtually all people who have cystic fibrosis. The main cause is deficiency of pancreatic enzymes, but bicarbonate deficiency, abnormalities of bile salts, mucosal transport and motility, and anatomical structural changes are other contributory factors. Appropriate pancreatic replacement therapy will achieve normal or near normal absorption in many patients. It is important to identify both malabsorption and evidence of a pancreatic lesion in all patients who are to receive pancreatic enzymes. All who have evidence of fat malabsorption are deemed pancreatic insufficient and candidates for enzyme replacement therapy. Effective treatment should allow a normal diet to be taken, control symptoms, correct malabsorption and achieve a normal nutritional state and growth. The occurrence of fibrosing colonopathy in some patients receiving very high doses of those enzymes that have the copolymer Eudragit L30 D55 in their covering has resulted in guidelines in the UK to avoid dosages greater than the equivalent of 10,000 IU lipase/kg/day for all patients and also to avoid preparations containing this copolymer in children and adolescents. For patients not responding to 10,000 IU lipase/kg/day, review of adherence to treatment, change of enzyme preparation, variation of the time of administration and reduction in gastric acid may improve absorption. The importance of excluding other gastrointestinal disorders as a cause of the patient's symptoms and the need for early investigations, rather than merely increasing the dosage of enzymes, is stressed. With modern enzymes, adequate control of gastrointestinal symptoms and absorption can be achieved at dosages of 10,000 IU lipase/kg/day or only slightly more, and a normal nutritional state and growth rate maintained in most patients with cystic fibrosis.

Child↗
PubMed1992

Pancreatic morphology and function in adult patients with cystic fibrosis.

To determine the relation between pancreatic morphology and pancreatic exocrine and endocrine function, we have studied 8 adult cystic fibrosis patients and 14 normal control subjects by ultrasonography and pancreatic function testing. In the patients with cystic fibrosis the maximum anteroposterior diameter of the pancreatic head was significantly increased over that in control subjects (p less than 0.01), whereas the maximum diameter of the body was significantly decreased (p = 0.05). Increased echogenicity of the pancreatic body was observed in most patients. In the cystic fibrosis patients postprandial insulin secretion was reduced in the 1st h (p less than 0.005 versus control), whereas pancreatic polypeptide secretion was virtually abolished for at least 3 h (p less than 0.01 versus control). All cystic fibrosis patients had evidence of exocrine pancreatic dysfunction as reflected by a diminished urinary para-aminobenzoic acid excretion. Intraduodenal enzyme and bicarbonate output in response to secretin-cholecystokinin was reduced in all of three patients studied. It is concluded that loss of endocrine and exocrine pancreatic function in adult cystic fibrosis patients is accompanied by a small and echo-dense pancreatic body relative to a large pancreatic head.

4-Aminobenzoic Acid↗
PubMed1988

[Appendicular mucoid impaction disclosing mucoviscidosis with cirrhosis in a young adult without pulmonary lesion].

A 22-year-old man presented with pain in the right iliac fossa. Clinical examination suggested appendicitis and showed splenomegaly. Echography and abdominal CT-sca suggested the diagnosis of cystic fibrosis based on the association of signs of cirrhosis and pancreatic atrophy. The sweat test was positive. The hypothesis of a mucoid appendicular impaction with spontaneous regression was retained based on clinical and radiological signs. This atypical presentation of cystic fibrosis underscores the frequency of obstructive intestinal which occasionally reveals the disease in the adult, and on the absence in this case of otherwise frequently associated problems such as significant pulmonary disease and malnutrition.

Adult↗
PubMedDate not supplied

The steatocrit: a simple method for monitoring fat malabsorption in patients with cystic fibrosis.

Steatocrit was determined through microcentrifugation of fecal homogenate from 110 pediatric controls and 107 patients with cystic fibrosis (CF). For 74 CF patients, steatocrit was determined in the same fecal material collected to determine a fat balance. In controls, steatocrit value was 0.7 +/- 1.0%, which was significantly lower than values found in CF patients with a coefficient of fat excretion less than 10% of intake (1.7 +/- 1.2%). Significantly increased values were found in CF patients with a coefficient of fat excretion ranging between 10 and 25% of intake (4.7 +/- 1.7%) and in those whose coefficient of fat excretion was greater than 25% of intake (11.3 +/- 4.3%). In the 74 CF patients, steatocrit was directly correlated to the coefficient of fat excretion (r = 0.93; P less than 0.001). We performed steatocrit several times in the course of the 1st year of life in 33 infants with CF diagnosed by means of CF screening. Values obtained at the time of diagnosis, before starting enzymatic therapy, were relatively high; they showed a progressive decrease when, using steatocrit as a guide, the dose of pancreatic enzymes had been increased. The normalization of steatocrit values was accompanied by a better growth rate in the majority of these infants, confirming the importance of an optimal early correction of pancreatic insufficiency. We propose that this simple semiquantitative test can be usefully performed for the frequent monitoring of fat absorption and for checking the response to enzymatic therapy in patients with CF.

Adolescent↗