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PubMed1983

Architecture of human pancreas: implications for early changes in pancreatic disease.

An increased proportion of ductulelike structures, sometimes dilated, occurs in association with pancreatic adenocarcinoma, chronic pancreatitis, cystic fibrosis, and pancreatic ectasia associated with conditions such as uremia. This frequently is interpreted as ductular proliferation, implying origin in ductal elements. Recent animal studies have provided an altered view of pancreatic architecture that is consistent with acinar dedifferentiation leading to the ductulelike structures, or tubular complexes. The architecture of pancreas from organ donors was studied by light and scanning electron microscopy and by wax reconstruction of serial sections. It is concluded that the zymogen granule-containing cells of normal human exocrine pancreas are arranged as branching tubules that vary in diameter and curve acutely. The tubules frequently end blindly to form acinar structures; less frequently they anastomose. This arrangement is consistent with the interpretation that tubular complexes associated with pancreatic disease result from dedifferentiative changes in acinar cells. Tubular complexes may reflect a defect or defects common to pancreatic disease rather than ductular proliferation specific to each one.

Adolescent↗
PubMed1991

Assessment of pancreatic function in screened infants with cystic fibrosis.

Previously we have reported that 37% of infants with cystic fibrosis diagnosed by neonatal screening with the dried blood spot immunoreactive trypsin assay have pancreatic sufficiency. However, 34 of the 78 infants had pancreatic function tests an average 2.3 years after diagnosis, thus it was possible that the percentage with neonatal pancreatic sufficiency was underestimated, due to the loss of pancreatic function with time in some infants. To assess this hypothesis we have assessed pancreatic function at the time of diagnosis in a further 20 infants since the completion of the previous study. Results of fecal fat determinations and/or pancreatic stimulation tests indicate that 10 (50%) of these infants have pancreatic sufficiency. Combining these results with those of the previous study, 31 of 64 patients (48%) have pancreatic sufficiency at this early age. We have also monitored the progression of pancreatic disease in the 39 patients with pancreatic sufficiency recognized to date. Eleven have developed pancreatic insufficiency and require enzyme replacement therapy. Five others have shown further improvement of colipase secretion with age. We conclude that the dried blood immunoreactive trypsin screening program for cystic fibrosis does recognize patients with pancreatic sufficiency, and at diagnosis nearly half the patients are in this category. To date, 28% of patients with pancreatic sufficiency have demonstrated a variable decline in pancreatic function with age.

Cystic Fibrosis↗
PubMed1988

Bile acid sequestration by the solid phase of stools in cystic fibrosis patients. Role of pancreatic enzymes.

The distribution of bile acids in the stool of seven cystic fibrosis (CF) patients with severe or mild steatorrhea was examined and compared with that of three controls. Results indicated significantly lower endogenous bile acid concentrations in the stool water phase, obtained by centrifugation, in the CF patients (12.0 +/- 3.5%), compared with the controls (25.5 +/- 8.1%). In vitro incorporation of labeled cholic acid (CA) and deoxycholic acid (DCA) demonstrated a stronger binding of both to the particulate matter of stools in the CF group. Using equilibrium dialysis, the calculated concentrations of unbound CA and DCA in the CF group measured 0.78 and 0.3 mumol/g homogenate, respectively, and in the control patients 1.76 and 1.39 mumol/g homogenate, respectively. Partial release of bile acids from CF stool pellets was achieved by the addition of trypsin and elastase, as well as by alkalinization. It is suggested that in patients with CF, stool bile acids are bound to the undigested protein fraction, which makes them unavailable for colonic resorption.

Adolescent↗
PubMed1986

Assay of human pancreatic lipase in biological fluids using a non-competitive enzyme immunoassay.

A sandwich enzyme immunoassay has been developed for human pancreatic lipase using polystyrene balls coated with specific IgG as the first antibody and peroxidase-labelled IgG as the second antibody. The detection limit was 0.5 microgram/l. Good parallelism was observed with the curves obtained from standard lipase and lipase present in serum, pancreatic juice and duodenal contents, demonstrating that the assay may be used to measure the level of the protein in different biological fluids. Mean values of lipase in human sera were 12.3 +/- 6.8 micrograms/l in adults and 4.5 +/- 2.7 in newborns. In all cases a good correlation was found in serum between the catalytic activity and the enzyme immunoassay. Lipase is detectable in amniotic fluids at the 18th week of pregnancy but at a very low level (0.95 +/- 0.32 microgram/l). In pancreatic juices, lipase concentration was 14.6% of the total protein content. A study on cystic fibrosis patients showed a poor correlation between blood pancreatic lipase concentration and fat malabsorption underlying the difficulty in assessing pancreatic function by the measurement of serum pancreatic enzymes. The use of the lipase assay in duodenal contents would permit better assessment of pancreatic function in patients presenting a severe or borderline defect in fat digestion and absorption.

Amniotic Fluid↗
PubMed1984

The role of the laboratory in the diagnosis of cystic fibrosis.

Laboratory investigations used for detecting cystic fibrosis have been critically reviewed. The sweat test is still the only investigation that can be used for detecting the disease at any age. Trypsin in blood or serum might have the same discriminatory power during the first three months of life and could be used for neonatal screening. There is no immediate prospect for prenatal diagnosis or heterozygote detection. The change of the pattern of plasma proteins in cystic fibrosis is the same as in other chronic disorders with varying clinical intensity. Albumin and alpha-1-antitrypsin may be used to monitor treatment. The plasma concentration of alpha-2-macroglobulin is considerably decreased in many younger patients.

Adolescent↗
PubMed1984

Magnetic resonance imaging in cystic fibrosis.

A clinical dilemma in patients with cystic fibrosis is the determination of the nature of linear areas of decreased aeration in the lungs. It is difficult using chest roentgenograms or even computed tomography to differentiate atelectasis, mucoid impacted bronchi, or peribronchial inflammatory disease from normal pulmonary vascularity. Magnetic resonance imaging is a noninvasive sensitive means that provides the distinction. Pulmonary vessels are easily identified, because with the spin-echo sequence that we use, the rapidly flowing blood within the vessels has no signal intensity. In contradistinction, mucoid-impacted bronchi appear as high-intensity linear branching structures. Peribronchial inflammatory disease appears as curvilinear areas of high intensity, representing inflammatory edema, around central lucencies representing bronchi.

Adolescent↗
PubMed1998

Purinoceptor activation of chloride transport in cystic fibrosis and CFTR-transfected pancreatic cell lines.

The regulation of chloride efflux from cystic fibrosis pancreatic adenocarcinoma cells (CFPAC-1) and wild-type CFTR-transfected CFPAC-1 cells (TPAC) was compared. Forskolin (10 microM) stimulated chloride efflux from the corrected TPAC cells but not from CFPAC-1 cells. Chloride efflux from both cell types was activated by thapsigargin (0.5 microM). The nucleotides ATP and UTP and the non-hydrolyzable ATP analogue, adenosine 5'-O-(3-thio) triphosphate (ATPgammaS), stimulated chloride efflux from both cell types. None of the other P2 purinoceptor agonists investigated elicited a response. The order of potency was ATP > or = UTP > or = ATPgammaS. Adenosine (10-100 microM) activated choride efflux from the TPAC but not the CFPAC cell line with no increase in intracellular cyclic AMP. Small but statistically significant inhibitions of the adenosine-(50 microM)-stimulated increase in chloride efflux were elicited by the A1 receptor antagonist 8-cyclopentyl-1,3-dipropylxanthine (CPX, 100 nM) and the A2 receptor antagonist 3,7-dimethyl-1-propylargylxanthine (DMPX, 10 microM). The A2A receptor antagonist 8-(3-chlorostyryl)caffeine (CSC, 100 nM) had no significant effect. These results provide evidence for the regulation of chloride efflux by P2Y2 purinoceptors in genetically-corrected and CF pancreatic cell lines. Studies with adenosine receptor antagonists indicate some possible involvement of A1 and A2 (but not A2A) receptors in the adenosine stimulation of chloride efflux, but the relatively small effects of the inhibitors coupled with lack of increase in cyclic AMP and a response only in the CFTR-transfected cells also suggests a possible direct effect of adenosine on CFTR.

Adenosine Triphosphate↗
PubMed2001

[Pathogenetic concepts of chronic pancreatitis].

Summary. The understanding of the pathogenesis of chronic pancreatitis is limited. Several theories (i. e. obstruction hypothesis) were suggested in the past but could not be confirmed by experimental data. As a formal description of the course of the disease, the necrosis-fibrosis concept seems to be very attractive. According to this theory, there is no significant difference in the pathogenesis of acute and chronic pancreatitis. A major step was the identification of mutations of the cationic trypsinogen, the secretory trypsin inhibitor (SPINK 1) and the cystic-fibrosis protein (CFTR) in some patients. Investigation of these mutations may significantly contribute to a better understanding of the pathogenesis of chronic pancreatitis.

Adult↗
PubMed1984

Immunoreactive trypsin in cystic fibrosis.

Since the first observation in 1979 that CF infants have elevated blood IRT, studies in various centres have enabled us to more fully understand the importance of this phenomenon. There is increasing evidence to show that mass newborn screening for CF using the IRT assay is practical and is capable of detecting essentially all CF newborns at a cost comparable to existing screening programs for other disorders such as Hypothyroidism. Although the elevated IRT levels seen in infancy in CF soon decrease, IRT levels in older CF patients appear to quite closely reflect the capability of that patient to secrete pancreatic enzymes and can be helpful in separating CF patients whose ability to secrete enzymes is preserved, from those with diminished exocrine pancreatic function. In all CF patients there appears to be an altered relationship between pancreatic exocrine secretion and circulating IRT levels as compared to control patients. This is probably one further manifestation of a secretory obstructive defect which although not uniformly severe, is common to all CF patients.

Adolescent↗
PubMed1991

Evaluation of growth and changes in body composition following neonatal diagnosis of cystic fibrosis.

Early deficits in nutritional status that might require specific treatment and early response to nutritional therapy were studied longitudinally in 25 infants with cystic fibrosis (CF) diagnosed by neonatal screening, using anthropometric and research body composition methodology, and evaluation of pancreatic function. At the time of confirmed diagnosis (mean 5.4 weeks), body mass, length, total body fat (TBF), and total body potassium (TBK) were all significantly reduced. Following diagnosis and commencement of therapy there was a normalization of weight, length, and TBK by 6-12 months of age, indicating catch-up growth. But in some individuals the response was incomplete, and as a group, mean total body fat remained significantly lower than normal at 1 year of age. Seven of 25 (28%) were pancreatic sufficient at diagnosis, and all but one had evidence of declining pancreatic function requiring the institution of pancreatic enzyme therapy during the next 1-9 months. The median age of commencement of enzyme therapy was 10 weeks (range 5 weeks to 11 months). These longitudinal assessments emphasize the dynamic changes occurring in absorptive function, body composition, and nutritional status following neonatal diagnosis of cystic fibrosis and may reflect previously described abnormalities of energy metabolism in this age group. Abnormal body composition is evident in most CF infants following diagnosis by neonatal screening but pancreatic damage may still be evolving. We suggest that early active nutritional therapy and surveillance for changes in pancreatic function are warranted in CF infants diagnosed by neonatal screening.

Body Composition↗
PubMed1984

Unexpected diagnosis of cystic fibrosis at autopsy.

Over an 11-year period, we encountered four cases in which an unexpected diagnosis of cystic fibrosis (CF) was first established on the basis of typical autopsy findings. Our experience suggests that the occurrence of such cases is more common than generally appreciated and emphasizes the often crucial role of the autopsy in medical practice.

Cystic Fibrosis↗