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PubMed1991

[Extrapulmonary manifestations of Pneumocystis carinii infection in AIDS].

Between 1986 and 1990 we found in 7 out of 100 continuously performed AIDS-autopsies at Auguste-Viktoria-Hospital (AVH) an extrapulmonary manifestation of Pneumocystis carinii (Pc). 4 of these cases showed only a singular infiltration of pulmohilar lymphnodi, while the remaining 3 cases presented various other organ involvements: spleen, liver, kidney, adrenal gland, prostate gland, pancreas, myocardium, thyroidea and eyes. All these AIDS-patients had a chronic or relapsing Pc-pneumonia with focal interstitial fibrosis, emphysema, and cavernous-cystic lesions. 6 patients developed a spontaneous pneumothorax due to ruptured subpleural bullae or cystic changes. Apparently the rare dissemination of Pc develops in the context of ruptured tissue and vessels in the pneumothorax lung of AIDS-patients during the final stage of the immunodeficiency associated with chronic lung changes.

Acquired Immunodeficiency Syndrome↗
PubMed1986

Effects of malnutrition and chronic reserpine treatment on pancreatic exocrine function.

The chronically reserpine-treated rat, an experimental model for cystic fibrosis, exhibits generalized exocrinopathy, impaired pancreatic secretion, and decreased pancreatic amylase. Although chronic reserpine treatment induces malnutrition by decreasing food consumption and growth, the effects of this malnutrition per se on the exocrine pancreas have not been considered. In this study, the effects of chronic reserpine treatment and malnutrition on the exocrine pancreas were determined using pair-fed controls. Male, Sprague-Dawley rats were treated daily subcutaneously for 5 to 7 days with: no injection (control), 1.0 ml/kg vehicle or sham (control-sham, pair fed-sham), or 0.5 mg/kg reserpine (chronically reserpine-treated). Both chronic reserpine-treatment and pair-feeding significantly decreased food consumption (40%), body weight (51 and 59%), total pancreatic amylase (49 and 56%) and specific amylase activity (62 and 61%), pancreatic protein (65 and 75%), and pancreatic weights (62 and 65%) compared to controls. These decreases, however, were comparable between the chronically reserpine-treated and pair fed-sham rats. In contrast, the secretory response to the biologically active cholecystokinin analog cholecystokinin octapeptide was significantly attenuated in isolated pancreatic acini prepared from reserpine-treated rats compared to that from either control or pair-fed sham rats. Malnutrition decreased pancreatic amylase activity and protein comparably to reserpine treatment, but only partially attenuated the secretory response to cholecystokinin octapeptide. Based on the results of this study, pair-fed controls should be used to distinguish between the effects of reserpine alone and the induced malnutrition on pancreatic exocrine function in studies of this experimental model of cystic fibrosis.

Amylases↗
PubMed1990

Current recommendations for breast-feeding in cystic fibrosis centers.

In recent years, breast-feeding has been strongly recommended, and the decision to breast-feed is made by a large percentage of women before pregnancy. We performed a survey to determine the current recommendations given to mothers in cystic fibrosis (CF) centers. For mothers of infants with CF, 77% of centers recommend breast-feeding alone or with pancreatic enzyme supplements and/or hydrolyzed formula. For mothers with CF, the following responses were given: 11% recommend breast-feeding, 8% do not recommend it, 42% make the recommendation according to the health status of the mother, and 32% make the recommendation according to the personal wishes of the mother. The duration of breast-feeding for mothers of infants with CF was reported to be from 3 to 6 months by 43% of centers, whereas for mothers with CF, 41% of centers reported the duration to be less than 3 months. Several centers reported that their experiences were not always positive, thus indicating a need to establish criteria to predict and ensure a successful outcome.

Breast Feeding↗
PubMed1995

Decreased beta-adrenergic stimulation of glycoprotein secretion in CF mice submandibular glands: reversal by the methylxanthine, IBMX.

beta-adrenergic stimulation of glycoprotein secretion was shown to be decreased in submandibular glands of Cystic Fibrosis (CF) mice. The defective response was partially restored by the methylxanthine, IBMX or cpt-cyclic AMP. Cholinergic stimulation of pancreatic amylase secretion was not affected in CF mice, demonstrating that this is not a generalised depression of protein secretion. The data are the first to show that the CF mouse mimics the protein secretion defect in CF human submandibular cells and that the mechanism of correction of the CF defect is via elevation of cyclic AMP. The results are therefore invaluable towards devising a rational pharmaceutical therapy for CF patients.

1-Methyl-3-isobutylxanthine↗
PubMed2001

Autoantibodies to pancreatic hsp60 precede the development of glucose intolerance in patients with cystic fibrosis.

Persons expressing the genetic disease cystic fibrosis (CF) suffer from a high risk of developing impaired glucose tolerance and diabetes. The development of diabetes in CF has been attributed, in the past, to the destruction of pancreatic islets and their resident beta-cells secondary to the destruction of the surrounding tissue by mechanical clogging of the pancreatic exocrine ducts. However, the discovery that autoimmunity to the 60-kDa heat shock protein (hsp60) may cause type I diabetes in NOD mice raises the possibility that hsp60 autoimmunity may be involved in CF diabetes too; could the hyperimmunization to bacterial hsp60 characteristic of CF spread to self-hsp60 and hence to autoimmune diabetes? We now report that rising levels of IgG autoantibodies to hsp60 do indeed precede the appearance of glucose intolerance and diabetes in CF patients. We produced a recombinant human pancreatic hsp60 protein and investigated the IgG antibody response to hsp60 in prediabetic and non-diabetic patients with CF. To detect hsp60 autoantibodies in the presence of high levels of antibodies to bacterial hsp60, we absorbed test sera with the 60-kDa GroEL of Pseudomonas aeruginosa and used an immunostaining technique. Using this technique, 32 prediabetic CF patients were evaluated over a five-year period, three years, on the average, before the onset of glucose intolerance. We found that a significant increase in hsp60 autoantibody preceded impaired glucose tolerance (P=0.042, n=17), diabetes (P=0.011, n=15) and glucose intolerance (P=0.005, n=32). As has been observed in NOD mice and in type I diabetic patients, the hsp60 autoantibodies decline at the outbreak of glucose intolerance in the CF patients. The association of CF diabetes with the rise and fall of hsp60 autoimmunity suggests that the pathogenesis of the diabetes may not be merely mechanical, but arise in the wake of bacterial hyperimmunisation.

Adolescent↗
PubMed1985

Serum immunoreactive trypsin and pancreatic lipase in cystic fibrosis.

Serum immunoreactive trypsin (IRT) and pancreatic lipase have been measured in 59 patients with cystic fibrosis (age 1 month-27 years). Follow-up values were obtained from 49 patients. Their serum enzyme levels were compared to those of 120 healthy children of all age groups. Faecal fat excretion was determined in selected patients (n = 23) to elucidate the relationship between serum enzyme levels and pancreatic exocrine function. In cystic fibrosis IRT and lipase showed a very similar age-correlated pattern: in infancy levels were markedly elevated. During the following years the concentrations of both enzymes decreased rapidly and were found to be far below the normal range after the 10th year of life. Elevated enzyme levels in infancy as well as low levels in all age groups coincided with steatorrhea. Older patients (11-27 years) without severe pancreatic insufficiency however, had IRT and lipase levels in or above the normal range. In healthy children there was no age dependency of IRT levels, whereas in the first 12 months of life lipase levels were significantly lower than in later childhood.

Adolescent↗
PubMed2001

Regulation of gap junctional communication in CFTR-expressing pancreatic epithelial cells.

Gap junction channels provide a pathway for coordinating multicellular activity. To evaluate the contribution of cell-to-cell communication in the function of epithelial cells, we studied the strength of gap junctional coupling in pancreatic acinar and duct cells exposed to agents known to elevate the intracellular concentration of Ca(2+) or cAMP. In acinar cells, we observed that maximal concentrations of acetylcholine evoked a biphasic increase in cytosolic Ca(2+) mobilization. The second sustained phase, which depends on Ca(2+) influx into the cell, was associated with the rapid closure of gap junction channels. In duct cells, stimulation of CFTR-dependent Cl(-) currents with cAMP analogs markedly increased gap junctional conductance in pairs of cells. Interestingly, cAMP had no effect on intercellular communication between cells harboring the DeltaF508 mutation of CFTR. An abnormal pattern of gap junctional coupling may contribute to the altered functions of tissues affected in cystic fibrosis.

Acetylcholine↗
PubMed2002

Clinical evidence of pathogenesis in chronic pancreatitis.

Chronic pancreatitis is a continuing inflammatory disease characterized by irreversible morphological change and, typically, by pain and permanent impairment of function. The pathogenesis of pancreatitis, either acute or chronic, is still controversial. There have been no widely accepted concepts to provide a reasonable explanation linking the known etiological factors and the pathophysiological aspects of the disease. Alcohol is undoubtedly the major etiological factor in most countries, and the relative importance of alcohol as a cause of chronic pancreatitis ranges from 40% to 90% in various countries. As fewer than 10% of alcoholics develop chronic pancreatitis, other nutritional or genetic influences are likely to be involved in the pathogenesis of alcoholic pancreatitis. Accessory pancreas incidentally found in patients with chronic alcoholic pancreatitis does not always have the pathological findings seen in the main pancreas. Integrity of the pancreatic duct seems to be another important factor for chronic alcoholic pancreatitis. Gene mutations of the cystic fibrosis transmembrane conductance regulator (CFTR), cationic trypsinogen, and pancreatic secretory trypsin inhibitor have been investigated in idiopathic chronic pancreatitis. Molecular and cell biology research during the past few years has elucidated pathophysiological factors that are involved in the pathogenesis of chronic pancreatitis, but cannot demonstrate a common pathway between etiological factors and the pathogenesis or development of the disease.

Chronic Disease↗
PubMed1983

Is cystic fibrosis a disease confined to epithelial cells?

We have reviewed evidence regarding the basic tissue involvement in cystic fibrosis (CF). CF is a disease almost exclusively confined to epithelial tissues, particularly those of endodermal origin. Exocrine glands containing such epithelium typically show both obstructive and electrolyte secretory defects. To date most basic CF research has centered on study of mesodermally-derived tissues in which little pathology is seen. The focus of CF research should be changed to concentrate on epithelial tissues.

Cystic Fibrosis↗
PubMed1989

ELISA for human pancreatic elastase 1.

A sandwich-type ELISA for human pancreatic elastase 1 was developed and assessed for clinical feasibility. Using highly purified reagents, the test seems to be as sensitive and specific as the well-documented Radio-immunoassay (RIA) for the detection of elevated elastase 1 serum levels and, consequently, would have the same indications described for the RIA. In addition, the ELISA could be valid as a screening test for cystic fibrosis. Because ELISA reagents are relatively stable, the assay is always ready to use and can be performed without special equipment.

Clinical Enzyme Tests↗
PubMed1991

Pancreatic endocrine function in cystic fibrosis.

To characterize pancreatic endocrine secretion and to examine interrelationships among alterations in alpha, beta, and pancreatic polypeptide cell function in patients with cystic fibrosis (CF), we studied 19 patients with exocrine insufficiency (EXO), including 9 receiving insulin therapy (EXO-IT); 10 patients with no exocrine insufficiency (NEXO); and 10 normal control subjects. First-phase C-peptide response to intravenously administered glucose was significantly impaired in CF patients with exocrine insufficiency (EXO-IT = 0.02 +/- 0.01; EXO = 0.11 +/- 0.02; NEXO = 0.25 +/- 0.05; control subjects = 0.30 +/- 0.04 nmol/L). Lowering fasting glucose levels with exogenous insulin administration in EXO-IT did not improve beta cell responsivity to glucose. The C-peptide response to arginine was less impaired (EXO-IT = 0.12 +/- 0.02; EXO = 0.15 +/- 0.02; NEXO = 0.23 +/- 0.06; control subjects = 0.28 +/- 0.04 nmol/L). Alpha cell function, measured as peak glucagon secretion in response to hypoglycemia, was diminished in EXO but not NEXO (EXO-IT = 21 +/- 10; EXO = 62 +/- 19; NEXO = 123 +/- 29; control subjects = 109 +/- 12 ng/L). Despite diminished glucagon response, EXO patients recovered normally from hypoglycemia. Peak pancreatic polypeptide response to hypoglycemia distinguished CF patients with exocrine insufficiency from those without exocrine insufficiency (EXO-IT = 3 +/- 2; EXO = 3 +/- 1; NEXO = 226 +/- 68; control subjects = 273 +/- 100 pmol/L). Thus CF patients with exocrine disease have less alpha, beta, and pancreatic polypeptide cell function than CF patients without exocrine disease. These data suggest either that exocrine disease causes endocrine dysfunction in CF or that a common pathogenic process simultaneously and independently impairs exocrine and endocrine function.

Adult↗
PubMed1996

Resting energy expenditure, pulmonary inflammation, and genotype in the early course of cystic fibrosis.

To further investigate the early course of cystic fibrosis (CF), specifically to examine factors contributing to energy imbalance, we examined resting energy expenditure (REE) (by indirect calorimetry) per unit body weight and metabolically active body cell mass (by total body potassium), in relation to CF genotype (by genomic DNA analysis), CF pancreatic phenotype, and markers of pulmonary inflammation (from bronchoalveolar lavage fluid). Eighteen subjects with presymptomatic CF who were less than 2 years of age (n = 11, delta F508/ delta F508 genotype; n = 15, pancreatic insufficiency phenotype), identified by newborn screening, were compared with age-, sex-, and length-matched control subjects (n = 13). Those with the delta F508/ delta F508 genotype had significantly higher mean REE expressed per unit body weight (125%) and body cell mass (115%; p < 0.03). Those with other genotypes (n = 7) did not, as a group, have significantly different mean REEs, but individuals with known "severe" genotypes had REEs in the high range and those with a pancreatic-sufficient phenotype had significantly lower REE than those with a pancreatic-insufficient phenotype (p < 0.05), and REEs were in the normal range. Examination of bronchoalveolar lavage fluid revealed positive culture results (7/10) but variable colony counts, neutrophil percentages, and concentrations of interleukin-8 and interleukin-1 beta equally in both CF genotype groups. These markers of pulmonary inflammation were not correlated, individually or collectively, with REE or genotype. We conclude that genotypic variations in energy balance are detectable early in CF unrelated to lung inflammation. Subclinical defects in body composition and pulmonary integrity occur early in CF and, in combination with increased cellular metabolic activity, have important clinical implications with respect to early diagnosis and management.

Body Composition↗
PubMed1978

Does that child really have cystic fibrosis?

A diagnosis of cystic fibrosis was incorrectly made after false-positive sweat tests in 14 children. 13 of these children had been tested at hospitals where it seems likely that sweat tests were not done very often. All the children had normal sweat-electrolyte values when the test was repeated at a regional paediatric centre where approximately 250 sweat tests are done each year. In 5 cases, detailed testing of pancreatic function was normal. None of the children had typical chest disease and only 2 had gastrointestinal symptoms. In the absence of the typical clinical features of the disease a diagnosis of cystic fibrosis should be made with extreme caution and only after meticulous testing of both sweat electrolytes and pancreatic function.

Amylases↗
PubMed2002

Fecal elastase-1 cut-off levels in the assessment of exocrine pancreatic function in cystic fibrosis.

BACKGROUND: Fecal elastase-1 (E1) test is a sensitive and specific indirect test. However, there are few data on the best cut-off level in the assessment of exocrine pancreatic function in cystic fibrosis (CF). MATERIAL AND METHODS: In 725 CF patients and 243 healthy subjects (HS) from Greece, Russia, Poland and the United Kingdom, E1 concentrations were measured. The best cut-off levels for the discrimination between CF and HS (for whole group as well as for individual countries) were calculated. RESULTS: The best cut-off level for the differentiation between CF pancreatic insufficiency and normal pancreatic function in HS was found to be 184 microg/g of feces. However, some inter-country differences were stated. E1 concentrations in the UK subgroup were significantly lower than those found in Polish and Russian CF patients. E1 concentrations in Greek patients were significantly higher than in the other countries. However, E1 concentrations in Delta F508 homozygotes were very similar in all studied subgroups. IN CONCLUSION: In clinical practice, instead of a single best cut-off level for the E1 test, we suggest using a range of values (160-200 microg/g). The presence of different best cut-off levels within countries is a practical consequence of the different distribution of pancreatic function.

Adolescent↗
PubMed1990

Pancreatic function in infants identified as having cystic fibrosis in a neonatal screening program.

The use of the dried-blood immunoreactive-trypsin assay for the detection of cystic fibrosis in newborns has been questioned on the grounds that it may fail to identify patients with enough pancreatic function to have normal fat absorption. To investigate this possibility, we assessed pancreatic function in 78 patients identified in a neonatal screening program as having cystic fibrosis. The diagnosis of cystic fibrosis was confirmed by abnormal results on a sweat chloride test. The results of measurements of fecal fat excretion, pancreatic-stimulation tests, and estimations of the serum level of pancreatic isoamylase indicated that 29 of the 78 children (37 percent) had substantial preservation of pancreatic function. These children (median age, four years) had growth that was close to normal and comparable to growth in children with severe pancreatic insufficiency who received oral enzyme therapy. Pancreatic insufficiency subsequently developed in 6 of the 29 patients, at 3 to 36 months of age. We conclude that the serum immunoreactive-trypsin assay used in neonatal screening programs identifies patients with cystic fibrosis who have sufficient pancreatic function to have normal fat absorption and that a substantial proportion of infants identified as having cystic fibrosis are in this category.

Child, Preschool↗
PubMed2003

Relation between pancreatic lipase activity and gastric emptying rate in children with cystic fibrosis.

OBJECTIVE: To determine whether abnormal gastric emptying is responsible for the inability of pancreatic enzyme replacement therapy (PERT) to normalize fat digestion in patients with cystic fibrosis (CF) who are pancreatic-insufficient. Study design Gastric emptying of a solid meal and pancreatic lipase function were assessed in 10 children with CF and 12 healthy control subjects with noninvasive breath tests using (13)C-octanoic acid and (13)C-mixed triglyceride, respectively. Lipase function was assessed in the subjects with CF with and without PERT. RESULTS: Without PERT, the lipase activity for the patients was less than that for the control subjects (P<.001); however, with PERT, 40% of the patients had a normalized lipase function. There were no differences between the mean gastric emptying rates of the patients with CF and the control subjects (P>.05), but there was a negative correlation between gastric half emptying time and percentage improvement in (13)C-mixed triglyceride results of the patients with CF with pancreatic enzymes compared with placebo (P<.05), with patients with slow gastric emptying having less improvement with PERT. CONCLUSIONS: The success of PERT in improving pancreatic lipase activity is reduced in patients with slow gastric emptying, which could explain the variations in improvement of fat digestion with enzyme supplementation.

Adolescent↗
PubMed1998

Discrepancies between males and females with cystic fibrosis in dietary intake and pancreatic enzyme use.

BACKGROUND: Length of survival of females with cystic fibrosis is worse than it is in males. Results of current research have shown an important correlation among dietary intake, nutritional status, lung function, and survival. The purpose of this study was to explore gender differences in dietary intake and pancreatic enzyme replacement therapy in males and females with cystic fibrosis. METHODS: The study was a cross-sectional measurement of clinical characteristics, energy, and fat intakes in males and females attending the cystic fibrosis outpatients clinics of the John Hunter Hospital, Newcastle, Australia. Twenty-nine subjects, (17 females and 12 males), completed 4-day weighed food records to measure total energy intake and the contribution of macronutrients and to document use of pancreatic enzyme replacement therapy. Energy intake was assessed as the percentage of the recommended energy intake for age and sex. RESULTS: Females with cystic fibrosis had significantly lower energy and fat intakes than males, whereas the females used significantly more pancreatic enzyme replacement therapy. There were no significant differences in clinical characteristics between groups. CONCLUSION: The results support the possibility that gender differences in the energy and fat intakes of older patients may contribute to differential median survival time of males and females with cystic fibrosis.

Adolescent↗