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At least 91 records · Page 5Linked to original sources
PubMed1992

Decline of exocrine pancreatic function in cystic fibrosis patients with pancreatic sufficiency.

Patients with cystic fibrosis and pancreatic sufficiency were investigated for evidence of progressive pancreatic disease. From a cohort of 630 patients, 20 pancreatic-sufficient patients became pancreatic insufficient after an average duration of 5.6 y (range 0.6-20.6 y) from diagnosis. Among 54 patients documented to be pancreatic sufficient by direct pancreatic stimulation test, 47 remained pancreatic sufficient and seven developed pancreatic insufficiency. The patients who ultimately developed pancreatic insufficiency were younger and had greatly reduced outputs of enzyme, fluid, and electrolytes. Those who remained pancreatic sufficient showed enzyme secretion close to or within the non-cystic fibrosis control range. Twenty of these patients underwent a second pancreatic stimulation test after an average interval of 4 y (range 1.3-6.2 y). No significant alteration in enzyme, fluid, or electrolyte output was seen in the patients who remained pancreatic sufficient, but there was further reduction in enzyme and fluid output in the patients who developed pancreatic failure. In conclusion, the majority of pancreatic-sufficient patients with pancreatic enzyme secretion within the control range showed no deterioration of function over an extended time period. However, a small number of pancreatic-sufficient patients with reduced enzyme and fluid secretion are at risk of pancreatic failure.

Adolescent↗
PubMed1982

Serum pancreatic lipase as a screening test for cystic fibrosis.

Pancreatic lipase catalyses the hydrolysis of emulsified triglycerides to form a transparent solution of monoglycerides and fatty acids. Levels of serum pancreatic lipase were measured in neonates known to have cystic fibrosis and compared with levels in control infants. During the first weeks of life infants with cystic fibrosis had raised serum pancreatic lipase values in parallel with raised serum trypsin values. A simple and specific turbidimetric dried blood spot assay for serum pancreatic lipase was used as a screening test fo cystic fibrosis in the neonate.

Cystic Fibrosis↗
PubMed2005

[Cystic fibrosis presenting as acute recurrent pancreatitis].

Cystic fibrosis is a multiorgan autosomal recessive disease resulting from mutations in a gene located on the long arm of chromosome 7. The disease is usually diagnosed in the first few years of life when it typically presents with severe pulmonary manifestations and pancreatic insufficiency; however, a small percentage of patients with less dramatic symptoms is not diagnosed until adolescence or even adulthood. The genotype of each patient seems to influence the various forms of clinical presentation. Although uncommon, acute recurrent pancreatitis can be one of the forms of delayed presentation of cystic fibrosis. We report the case of an 17-year-old man who was diagnosed with cystic fibrosis after presenting 2 episodes of acute pancreatitis without associated pancreatic insufficiency. Subsequent study also revealed nasal polyps.

Acute Disease↗
PubMed2000

Evaluation of fecal pancreatic elastase-1 as a measure of pancreatic exocrine function in children with cystic fibrosis.

Pancreatic elastase-1 (EL-1) is a specific human protease synthesised by the acinar cells. It is stable, unaffected by exogenous pancreatic enzyme treatment, and correlates well with stimulated pancreatic function tests. We report our experience of EL-1 measurements in 142 patients from a large cystic fibrosis (CF) clinic. The median patient age was 7.7 years (range, 0.1-20.8 years), 93 were homozygous and 38 heterozygous for DeltaF508, and 11 had other or unidentified mutations. There were 85 non-CF control subjects. Seven were pancreatic sufficient (PS). The median (quartile 1-quartile 3) fecal EL-1 of the 135 pancreatic insufficient (PI) patients was 10 microg/g stool (2.5-33); of the 7 PS patients, 698 microg/g stool (400.5-824.5), and of the non-CF controls, 615 microg/g stool (420-773). Using the Mann-Whitney U test, there was a statistically significant difference for fecal EL-1 activity between the PS and PI patients (P = 0.0001) and the PI and control group (P < 0.0001), but not between the control and PS groups (P = 0.63). Median (quartile 1-quartile 3) fecal EL-1 in the pancreatic insufficient DeltaF508 homozygotes was 10 microg/g stool (2-33), and in the heterozygotes 12 microg/g stool (4-39) (not significant, P = 0.62). We now use fecal EL-1 as evidence of PI in screened CF infants (reliable over the age of 2 weeks); in older CF patients at diagnosis; for confirming the need for pancreatic enzymes in patients referred to the clinic already taking enzymes; for annual monitoring of PS patients to detect the onset of PI; and as supporting evidence when excluding the diagnosis of CF in patients attending the pediatric gastroenterology clinic. The low values in the first 2 weeks in some normal and premature infants, and the persisting normal values in PS infants, make the fecal EL-1 test unsuitable for neonatal CF screening.

Adolescent↗
PubMed1992

Pathophysiology of the pancreas in cystic fibrosis.

Pancreatic insufficiency occurs in the majority of cystic fibrosis (CF) patients. Deficient fluid secretion is apparent at all levels of pancreatic function and leads to pancreatic protein hypersecretion which may in turn result in protein precipitation and ductal plugging. An impaired chloride and bicarbonate secretion appears to account for this fluid secretion deficit. A minority of CF patients have sufficient preservation of pancreatic function to prevent steatorrhoea. These patients are diagnosed at a later age, experience milder symptoms and have a far superior overall prognosis than patients with pancreatic insufficiency (PI). Patients who are homozygous for delta F508 have a high frequency of PI (99%), whereas patients with other genotypes are more often pancreatic sufficient (PS). In 538 patients with CF DNA analysis was performed and related with pancreatic function. The most striking observation was that nearly all given genotypes were associated with either PI or PS and not with both. In addition, we were able to classify mutations as "severe" and "mild" with respect to pancreatic function.

Child↗
PubMed2005

Skin prick test reactivity to supplemental enzymes in cystic fibrosis and pancreatic insufficiency.

BACKGROUND: Patients with cystic fibrosis and pancreatic insufficiency take supplemental pancreatic enzymes of porcine or plant origin on a daily basis. OBJECTIVE: The frequency of immediate skin prick hypersensitivity reactions to such enzymes and the correlations to gastrointestinal symptoms and nutritional status were assessed in a single center prospective descriptive study. METHODS: Skin prick tests using five different freshly prepared porcine pancreatic enzyme preparations, one enzyme preparation of fungal origin, porcine meat and cat epithelia were performed in patients with cystic fibrosis and pancreatic insufficiency and in healthy control subjects. RESULTS: Positive skin tests in the 48 patients with cystic fibrosis were no more frequent than in 21 control subjects. No correlations with the frequency of gastrointestinal symptoms, nutritional status and lung function were found. CONCLUSION: Although immediate hypersensitivity reactions to pancreatic enzyme preparations in subjects with cystic fibrosis are not common, individuals with possible allergic reactions to supplemental enzymes should be identified by skin prick tests and in unclear cases by double-blind placebo-controlled provocation tests.

Adolescent↗
PubMed1995

Maldigestion associated with exocrine pancreatic insufficiency: implications of gastrointestinal physiology and properties of enzyme preparations for a cause-related and patient-tailored treatment.

Clinical conditions in which secondary maldigestion associated with exocrine pancreatic insufficiency occur include chronic pancreatitis, cystic fibrosis, pancreatic cancer, partial or total gastrectomy, and pancreatic resection. Maldigestion can cause serious weight loss, nutritional deficiencies, and subjective complaints associated with steatorrhea. The various causes of exocrine pancreatic insufficiency may be associated with cause-related changes in gastrointestinal physiology, such as changes in gastrointestinal intraluminal pH, bile acid metabolism, gastric emptying, and intestinal motility. Therefore, to optimize the efficacy of treatment, the management of exocrine pancreatic insufficiency must be individually tailored to account for both the underlying cause and any associated disturbance in gastrointestinal physiology. In addition, the properties of the pancreatic enzyme preparations and adjuvant drugs need to be taken into consideration. This paper reviews the pathophysiological mechanisms of maldigestion in exocrine pancreatic insufficiency, discusses the efficacy of different therapy regimens, and gives guidelines for a cause-related and patient-tailored treatment with respect to both drug therapy and dietary counselling.

Diet, Fat-Restricted↗