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At least 55 records · Page 3Linked to original sources
PubMed1975

Electrical polarization of rectal mucosa and excretion of tetrahydroaldosterone in patients with cystic fibrosis of pancreas and in normal subjects.

The electrical potential difference (PD) across the rectal wall was measured in 26 patients with cystic fibrosis of pancreas (CFP) and in 18 healthy subjects. The PDs obtained in normal children were identical to those previously obtained in normal adults. A significantly greater dispersion of the values was observed in CFP. When the patients were divided into groups according to metachromasia in fibroblast cultures, the mean PD was increased only in the ametachromatic group. True enough, this observation suggests a difference between various forms of CFP, distinguished by metachromasia, and thus is a further indication of the heterogeneity of the disease. The greater abnormalities in metachromasia negative patients may, however, be due solely to the fact that these patients are more severely affected by the disease. The urinary excretion of tetrahydroaldosterone in patients was within the ranges obtained in controls, which excludes the possibility of secondary hyperaldosteronism as the source of increased PD. No evidence was provided in favour of a basic defect in the intestinal transport of Na+ or Cl minus, but K+ concentrations in faecal fluids of patients were significantly lower than in controls. The equilibrium concentration of K+ could be accounted for by simple passive diffusion, suggesting that the epithelium behaved inertly with respect to this ion in CFP.

Adolescent↗
PubMed1976

Meconium ileus equivalent in adults with cystic fibrosis of pancreas: a report of six cases.

Eleven episodes of "meconium ileus equivalent" have been seen in six adults with cystic fibrosis of the pancreas. Three patients were initially treated surgically; one died and the other two developed serious postoperative chest infections. Six episodes were successfully treated medically with acetylcysteine orally and by enema, nasogastric suction, and intravenous fluids. Operation should be avoided if possible, and maintenance treatment with acetylcysteine may be necessary to prevent relapse.

Acetylcysteine↗
PubMedDate not supplied

Cystic fibrosis of the pancreas: report of a Japanese autopsy case.

Cystic fibrosis of the pancreas is considered to be rare in the Japanese population. Forty-five cases from the Japanese literature (1951-1983) are reviewed and one autopsy case added. The patient was a 17-year-old Japanese male with recurrent episodes of pulmonary infection. His sweat test was positive (sodium 332 mg/l, chloride 316 mg/l). No particular hereditary trait was identified. Autopsy findings showed cystic dilatation of pancreatic ducts and ductules with mucous hypersecretion. Acini of the pancreas were replaced by fibrosis, while islands of Langerhans remained. These findings were compatible with cystic fibrosis of the pancreas (mucoviscidosis). The lungs showed chronic hypertrophic bronchitis and acute obliterating bronchiolitis. Cystic dilatation and hypersecretion were seen in the esophageal glands, Brenner's glands, and enteric glands. Goblet-cell hyperplasia was seen in the enteric glands. Central fatty metamorphosis and focal bile duct proliferation were seen in the liver. No obvious intralobular cholestasis was present. The epithelium of the gall bladder was highly columnar.

Adolescent↗
PubMed1984

Endocrine pancreas in cystic fibrosis: an immunohistochemical study.

The pancreases of 17 patients who had cystic fibrosis with and without diabetes mellitus were evaluated at autopsy by routine staining and immunohistochemical methods for insulin, glucagon, somatostatin, and pancreatic polypeptide. Qualitative assessment of the number of islets of Langerhans and the degrees of exocrine pancreatic atrophy, fibrosis, and fat replacement was made for each pancreas. Quantitative assessment of islet composition was performed in 15 of the 17 based on the immunochemical reactivity of each cell type. Nondiabetic patients with cystic fibrosis in the latter part of the first decade of life have classic fibrocystic changes of the pancreas, with some persisting exocrine tissue, islets that appear normal, and prominent nesidioblastosis. The latter process may protect these patients from glucose intolerance. Young adult diabetic patients with cystic fibrosis have total loss of exocrine pancreas with fat replacement, lack of nesidioblastosis, a qualitative decrease in the number of islets, fibrosis of and amyloid deposits in islets, decreased numbers of insulin-containing cells in each islet, and atrophy of islet cells, probably resulting from progressive ischemia. Although the potential exists for an increasing incidence of diabetes mellitus in patients with cystic fibrosis as their life spans increase, individual variation occurs in this disease.

Adult↗
PubMed1999

Cystic fibrosis and the pancreas: recent scientific advances.

Cystic fibrosis (CF) is inherited as an autosomal recessive trait due to a mutated gene carried on the long arm of chromosome 7. The protein coded by the CF gene (CFTR) is an apical chloride channel that regulates active chloride transport across epithelial cell membranes. The role of CFTR in pancreatic exocrine secretion is based on the concept that CFTR is localized to the apical membrane of the proximal duct epithelial cells. The primary ductal cell chloride channel abnormality results in dehydrated protein-rich secretions that obstruct the proximal ducts, leading to secondary acinar cell destruction, fibrosis, and exocrine pancreatic insufficiency in 85% of the CF population. A strong correlation between genotype and pancreatic phenotype is recognized. Advances in molecular biology such as cloning of lipase genes and gene therapy are challenging the field of pancreatic enzyme supplementation therapy.

Cystic Fibrosis↗
PubMed1984

Structural and developmental abnormalities of the exocrine pancreas in cystic fibrosis.

Normal infants from 32 to 54 weeks post-conceptional age show a clear pathway of development in the exocrine pancreas, involving differentiation of acinar tissue, reflected by increased acinar to connective tissue volumes. In contrast, an abnormal pattern of development has been found in cystic fibrosis. Early signs of a deficiency in exocrine tissue at 32-38 weeks post-conceptional age suggest that there is a lack of normal maturation of pancreatic exocrine tissue that occurs in utero, with a degenerative process supervening after birth. The volumes of the acinar and duct lumen is markedly increased, up to 10 fold normal volume in cystic fibrosis subjects. However, the lumenal changes are not a function of age. Accumulation of secretory material in the duct is a characteristic feature in cystic fibrosis and may cause dilation of the duct and acinar lumen and degradation of acini. Alternatively, the greater volume and lack of direct relationship to exocrine acinar volume may reflect a persistence of the fetal pattern of pancreatic development in cystic fibrosis. The diagnosis of cystic fibrosis within the first few months of life is difficult when based on conventional or subjective histologic criteria. By quantitative microscopy of the pancreas, an objective approach is available that clearly distinguishes cystic fibrosis from control subjects. In this retrospective survey, 93% of cystic fibrosis infants were discriminated from normals; only 2 of 30 cases (70%) were not clearly differentiated from controls. As a genetic disorder, the manifestation of cystic fibrosis would be expected to result in pancreatic dysfunction in utero. Other predisposing factors, however, may be involved in the pancreatic lesions such as obstruction of the pancreatic duct, failure in the synthesis or secretion of pancreatic enzymes or abnormal mucus production in the intestine. Further ultrastructural and functional investigations will be important to understand the underlying defect in cystic fibrosis.

Cystic Fibrosis↗