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PubMed1963

THE PATHOLOGY OF LIVER CIRRHOSIS IN PATIENTS WITH CYSTIC FIBROSIS OF THE PANCREAS.

The peculiarities of the type of liver cirrhosis that occurs in patients with cystic fibrosis of the pancreas depend on a number of factors. Two such factors, which have received little attention in the past, became apparent during a study of the livers of patients dying of this disease at the Henry Ford Hospital, Detroit. Firstly, the onset of the disease in fetal life may disturb the development of the bile duct system whose normal development is essential for normal structural relationships to be maintained in the liver. Focal lesions of intrahepatic biliary atresia will then complicate the histologic picture of "multi-lobular biliary cirrhosis". Secondly, scars formed in an infantile liver will considerably distort the subsequent growth of the liver, resulting in bizarre nodularity. Despite massive deformation large portions of the liver will still be composed of primary parenchyma that will enable normal liver functions as revealed by laboratory tests.

Bile↗
PubMed1981

[Pneumothorax as complication of cystic fibrosis of the pancreas].

During recent years, 14 episodes of pneumothorax in 4 children and adolescents with cystic fibrosis of the pancreas (CF) have been treated in the University Department of Pediatrics in Zurich. Treatment with pleural drainage, associated with instillations of sclerosing agents was usually tedious and did not prevent relapses. One patient died after the second episode, another was successfully treated by pleurectomy. The incidence of pneumothorax in adolescent CF patients increases rapidly mostly due to the longer life expectancy of CF patients. Both lungs are equally affected. In more than 50% one or more relapses occur. The prognosis is usually bad. No specific etiologic factors can be incriminated, pneumothorax occurring mostly during periods of physical inactivity. The pathogenesis seems, in the majority of cases, to be the bursting of a subpleural emphysematous bulla or bleb. Such bullae can mostly be recognised radiologically many years prior to the first pneumothorax episode, usually in severely affected lungs in children over 10 years of age. From the reviewed literature as well as from our own experience it seems that pleurectomy is the only efficient treatment, being well tolerated, curing the actual episode of pneumothorax, and preventing relapses.

Adolescent↗
PubMed1975

Excretion of trypsin-like activity, electrolytes and protein in mixed and parotid saliva of patients with cystic fibrosis of the pancreas.

The esterolytic activity of mixed and parotid saliva in cystic fibrosis (CF) patients and normal subjects was determined using BAEE (alpha-Benzoyl-1-arginine-ethylester) as the substrate. Using soybean-trypsin-inhibitor the trypsin-like activity (TLA) was measured and plotted as a function of parotid flow rate. In addition calcium, protein and pH were determined. Trypsin-like activity in mixed and parotid saliva showed large individual variations in CF and normal children. In parotid saliva we could not find any significant difference, whereas a reduction of TLA in mixed saliva of CF patients was observed. The fact that our normal values fell within the range of heterozygotes reported by Rao et al. (19), makes their hypothesis of a close relationship between reduced TLA and the genetic defect very doubtful. Protein, calcium and pH increased with augmented salivation and no difference between CF patients and normal age matched children could be found except for the pH at a flow rate above 0.75 ml/min per m2 body surface where significantly lower pH values resulted. The relevance of reduced TLA to the pathogenesis of cystic fibrosis is discussed.

Calcium↗
PubMed1986

[Nutritional management of neonates and infants with cystic fibrosis of the pancreas detected at birth].

From a systematic neonatal screening for cystic fibrosis in the Basse-Normandie area and to prevent disorders of the intestinal transit related to malabsorption, neonates then infants were given a semi-elemental hypercaloric diet, with supplements in nitrogen, MCT, minerals, vitamins and low in LCT. Diets were adjusted every month during a consultation using clinical and biological parameters. Results in the first 14 children showed that clinically as well as biologically, these children may remain within the normal range, avoiding the previously reported growth retardation and mineral or vitamin deficiencies. This procedure should allow an improvement in the quality of life and prognosis of such children, by maintaining adequate nutritional status.

Avitaminosis↗
PubMed1997

Cystic fibrosis of the pancreas: involvement of MUC6 mucin in obstruction of pancreatic ducts.

BACKGROUND: Cystic fibrosis (CF) is characterized by pancreatic destruction following the gradual obstruction of small pancreatic ducts, from the mid-trimester of gestation onwards. To date, the material causing the obstruction has not been identified. The MUC6 mucin cDNA was isolated from human stomach and has been shown to be expressed in a number of other tissues in the gastrointestinal tract, including the gall bladder and parts of the ileum and colon. MATERIALS AND METHODS: We have examined the expression of MUC6 mucin in the human pancreas, both during development and postnatally, by mRNA in situ hybridization and immunocytochemistry. RESULTS: In this report we establish that MUC6 transcripts are abundant in pancreatic epithelial cells and show a very similar pattern of expression in the epithelium lining small ducts and centroacinar cells to that shown by the cystic fibrosis transmembrane conductance regulator gene (CFTR). In addition, material obstructing the pancreatic ducts of CF pancreas was shown to contain MUC6 mucin. CONCLUSION: We have identified MUC6 mucin as a significant constituent of the material obstructing the small pancreatic ducts in CF.

Adolescent↗