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At least 1,171 records · Page 65Linked to original sources
PubMed1986

Diagnosis of cystic fibrosis in premature infants.

Meconium ileus and pancreatic changes, as described in cystic fibrosis, were found, at autopsy, in a series of six infants who received prolonged neonatal intensive care for prematurity. Cystic fibrosis had not been suspected clinically. These pathological findings are so frequent in sick premature infants, amounting to 12% of all neonatal autopsies conducted over a period of 2 yr in our unit, that we question their specificity for cystic fibrosis and suggest they may be a manifestation of disordered physiology in the severely ill neonate.

Autopsy↗
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↗
PubMed2003

Cystic fibrosis.

On a daily basis, pathologists examine the fundamental basis of human diseases using morphologic, immunologic, and molecular techniques. Cystic fibrosis (CF), as a clinically heterogeneous disease, exemplifies the complex challenges of genetic diseases for the pathologist who attempts to explain the mechanisms of disease and provide rationale for clinical management. This review includes an overview of CF and a discussion of pathophysiologic features and practical components of clinical and anatomic pathology, and concludes with a review of molecular diagnostics.

Bone and Bones↗
PubMed1977

Differential serum amylase determination by use of an inhibitor, and design of a routine procedure.

We describe a new method for measuring pancreatic and salivary-type amylases in serum that requires no electrophoresis or chromatography. An inhibitor protein (from wheat) with a 100-fold greater specificity for human salivary than for human pancreatic amylase was used to analyze mixtures of the two enzymes. The concentration of pancreatic and salivary amyalase was determined in 141 normal sera (72 men and 69 women). Statistically significant differences were found for serum pancreatic amylase between mean and women, higher values being shown in women. No sex-related difference was found for the salivary component of serum amylase. With this method, the increase in serum amylase activity in pancreatitis was shown to be attributable to the pancreatic component. In mumps, the increase is attributable to the salivary component. In pancreatic insufficiency, serum pancreatic amylase activities were significantly lower than in the controls. Our method is simple and rapid; our results agree well with those of other authors who used chromatographic or electrophoretic methods.

Adolescent↗
PubMed1998

Lung function in South African children with cystic fibrosis.

OBJECTIVE: To determine the pattern of lung function in stable cystic fibrosis (CF) patients and to investigate the relationship of abnormal lung function to demographic variables, CF genotype and pulmonary colonisation with Pseudomonas aeruginosa (PA). DESIGN: A descriptive study done at the CF clinic at Red Cross War Memorial Children's Hospital in Cape Town. METHODS: Data were recorded and pulmonary function testing (PFT) was performed in 42 CF patients. RESULTS: 29 patients (69%) had mild disease, while 11 (26%) and 2 (5%) had moderate and severe disease respectively. Twenty-four patients (57%) demonstrated lower airway obstruction (LAO). Patients with moderate or severe disease were significantly older than those with mild disease (13.3 (3.7) years (mean (SD)) compared with 11.1 (3.0) years (t = 2.1; P = 0.04). PA colonisation status differed significantly with the pattern of lung function (chi 2 = 6.6; P = 0.04) and severity of lung disease (chi 2 = 12.6; P = 0.002). Nine (35%) of the 26 patients tested before and after bronchodilator therapy showed a positive response. CONCLUSION: The majority of patients had mildly impaired or normal lung function, with LAO predominating. A minority of patients were bronchodilator-responsive. PA colonisation may be associated with the development of abnormal lung function and more severe pulmonary disease.

Adolescent↗
PubMed1993

Serum immunoglobulin G directed against porcine trypsin in the serum of cystic fibrosis children receiving porcine pancreatic enzyme supplements.

Cystic fibrosis (CF) patients require pancreatic enzyme replacement therapy to correct pancreatic insufficiency. These enzymes are derived from porcine pancreas and are known to be antigenic. To determine the possible clinical consequences, a specific ELISA was developed to detect IgG antibody directed against porcine trypsin (PTAb) in the sera of CF patients. The assay was used to evaluate the occurrence of PTAb in a cross sectional study of 103 CF patients in relation to the introduction of porcine enzyme therapy, clinical status and genotype. Antibodies against porcine trypsin were detected in the sera of 63% of patients unrelated to the age of commencement or the duration of enzyme therapy. No differences were observed in the clinical status of CF patients who had developed PTAb (n = 65) and those who had no detectable PTAb (n = 38) as determined from: the current prescribed dose of porcine pancreatic enzyme capsules; Z scores for height and weight; and respiratory function tests. It is suggested that the PTAb commonly found in the sera of CF patients are of doubtful clinical significance but the prospect of PTAb contributing to immune complex disease should be examined further.

Adolescent↗