Liver disease and bileduct stenosis in cystic fibrosis.
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Adult↗
Biomedical subjects
K J Gaskin
Publications and source records attributed to K J Gaskin.
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↗
Body composition in cystic fibrosis.
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Body Composition↗
The role of body protein studies in clinical trials.
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Adolescent↗
Malnutrition in cystic fibrosis: psychosocial functioning of patients and their families.
The relationship between nutritional status and psychosocial functioning was examined in 35 children with cystic fibrosis, aged 7-16 years. Twelve malnourished children and their families were compared with 23 well nourished children and their families. Established measures of adjustment and coping in the children, parents and families were used. Few statistically significant differences between the two groups emerged, and all comparisons of psychosocial functioning were not significant. The results of the study suggest that there is no relationship between the nutritional status of the child with cystic fibrosis and the current psychosocial adjustment and coping of child, parents and family.
Adaptation, Psychological↗
Nutritional status, growth and development in children undergoing intensive treatment for cystic fibrosis.
Dietary intakes were measured over a period of 5 days in 36 malnourished and 36 well-nourished patients with cystic fibrosis. Both energy and protein intakes were significantly less in the malnourished patients for the two age groups studied: 4-9.99 years (p less than 0.01 for both parameters), and 10-16 years (p less than 0.05 and p less than 0.01, respectively). In both age groups and both patient groups, average protein intakes were well in excess of the recommended daily intake, but energy intake in the malnourished patients was below the recommended daily intake. Nutritional supplementation of 10 malnourished patients with a polymeric formula, infused overnight via a gastrostomy tube, resulted in a seven-fold increase in weight gain (p less than 0.001) and a doubling of linear growth velocity (p less than 0.01) over a period of 18 months, compared to the 18 months prior to gastrostomy feeding. Measurements of total body nitrogen in eight of these patients demonstrated a 38% increase in body nitrogen content over 12 months, indicating a replenishment of the protein deficit.
Adolescent↗
Body protein of patients undergoing haemodialysis.
The total body protein status of 18 patients undergoing regular haemodialysis was assessed by measuring total body nitrogen (TBN) using in vivo neutron activation analysis (NAA). Eighteen healthy controls, who were selected according to their height, age and sex match with the patients were also measured. The male and female patients were both found to have lower mean values for total body protein (P less than 0.01, P less than 0.025 respectively) although they had similar weights compared with their matched controls. Seven patients were measured on further occasions and only two patients showed a change in their body protein. One female showed an increase of 11 per cent in body protein (with an increase of 25 per cent in body weight) after intensive nutritional repletion therapy. In vivo NAA provides a direct means of measuring body protein and is a reliable method to monitor changes with treatment regimes.
Adult↗
Determination of total body water by Fourier transform infrared analysis.
A new technique for determining body water using deuterium isotope dilution and Fourier transform infrared (FTIR) analysis is described. The advantages of the FTIR over conventional dispersion and filter infrared instruments include greater flexibility through computer controlled operations and availability of 'on-line' analytical software. The technique was further improved by the development of a simple procedure for determining D2O concentration in untreated serum samples. A validation study of six normal adults showed that the fat-free-mass determined from the deuterium-space (total body water) correlated well with the results obtained by total body nitrogen (r = 0.997), total body potassium (r = 0.996) and anthropometric (r = 0.995) measurements.
Adult↗
Outcome of surgery for biliary atresia.
Forty-seven infants (26 male, 21 female) with biliary atresia under- went hepatic portoenterostomy during the 16-year period 1971-87. Twenty-six patients (55%) are alive 1-17 years after surgery, with 21 (45%) being jaundice-free. For children who became jaundice-free, the mean age at surgery was 78 days (range: 34-125 days), compared with 97 days (range: 48-224 days) for those who did not. Of 39 patients operated on at less than 120 days of age, 24 (60%) are alive. All four patients operated on after 125 days of life died. Of 31 patients operated on more than 5 years ago, 12 (39%) have survived, the oldest being 17 years. Ten (32%) have normal serum bilirubin concentrations, have non-active cirrhosis on liver biopsy, have had normal growth and development, and lead normal lives. The oldest two patients suffered variceal haemorrhage in their teenage years. In our recent experience, 11 of 16 patients (69%) have had complete clearing of jaundice, lead normal lives and do not currently require assessment for liver transplantation. It is believed that early referral of children with biliary atresia to experienced surgical units for portoenterostomy will lead to long-term survival, without the need for liver transplantation in a majority of cases. Liver transplantation should be offered in infancy only after failed portoenterostomy, except for patients presenting after 120 days in whom transplantation may be considered primary therapy.
Age Factors↗
Liver disease and common-bile-duct stenosis in cystic fibrosis.
To determine the incidence of common-bile-duct lesions and their relation to liver disease in cystic fibrosis, we performed hepatobiliary scanning in 50 of 61 patients with cystic fibrosis who had hepatomegaly, abnormal liver function, or both and in 31 of 92 patients with cystic fibrosis who did not have hepatomegaly or abnormal liver function. Ninety-six percent of the patients with liver disease had evidence of biliary tract obstruction, which was defined cholangiographically as a stricture of the distal common bile duct in the majority of cases. All the patients without liver disease had normal intrahepatic and common-duct excretion of tracer. Abdominal pain was significantly more common in patients with common-duct obstruction (P less than 0.001), and enlarged gallbladders occurred only in such patients. Since fasting levels of serum bile acids were elevated in nearly half these patients, irrespective of the severity of their liver disease, serum bile acids may be markers of the severity of the common-duct lesion. We conclude that strictures of the distal common bile duct are common in patients with cystic fibrosis and liver disease. This association requires further study, since surgical relief of common-duct obstruction may prevent or ameliorate the hepatic complications of cystic fibrosis.
Adolescent↗
The impact of nutrition in cystic fibrosis: a review.
A high energy intake, compensating for malabsorption, and the energy cost of lung disease, lung infections, and the underlying metabolic abnormality, is required to ensure normal growth in patients with cystic fibrosis. This goal can be readily achieved by adherence to a high quantity, normally balanced diet (with 40% of the energy as triglycerides of long-chain fatty acids). In contrast, this goal cannot be reached in the majority of patients adhering to low-fat and thus low-energy-containing diets, which almost inevitably lead to malnutrition with wasting and stunting. The prevention of malnutrition may well have considerably enhanced the prognosis of patients at one clinic. Further work is needed to define the interrelationship of nutrition and lung disease, and to define the appropriate nutrient requirements induced by the lung disease per se, recurrent infections, and the underlying disease process.
Cystic Fibrosis↗
Common bile duct obstruction causing right upper abdominal pain in cystic fibrosis.
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Abdomen↗
Age-related alterations of immunoreactive pancreatic cationic trypsinogen in sera from cystic fibrosis patients with and without pancreatic insufficiency.
Serum immunoreactive cationic trypsinogen levels were determined in 99 control subjects and 381 cystic fibrosis (CF) patients. To evaluate the status of the exocrine pancreas all CF patients had previously undergone fecal fat balance studies and/or pancreatic stimulation tests. Three hundred fourteen CF patients had fat malabsorption and/or had inadequate pancreatic enzyme secretion (pancreatic insufficiency) requiring oral pancreatic enzyme supplements with meals. Sixty-seven CF patients did not have fat malabsorption and/or had adequate enzyme secretion (pancreatic sufficiency) and were not receiving pancreatic enzyme supplements with meals. Mean serum trypsinogen in 99 control subjects was 31.4 +/- 14.8 micrograms/liter (+/- 2 SD) and levels did not vary with age or sex. In CF infants (less than 2 yr) with pancreatic insufficiency, mean serum trypsinogen was significantly above the non-CF values (p less than 0.001). Ninety-one percent of the CF infants had elevated levels. Serum trypsinogen values in the pancreatic insufficient group declined steeply up to 5 years, reaching subnormal values by age 6. An equation was developed which described these age-related changes very accurately. Only six CF patients with pancreatic insufficiency had serum trypsinogen levels above the 95% confidence limits of this equation. In contrast, there was no age related decline in serum trypsinogen among the CF group with pancreatic sufficiency. Under 7 yr, serum trypsinogen failed to distinguish the two groups. In those over 7 yr of age, however, serum trypsinogen was significantly higher than the CF group with pancreatic insufficiency (p less than 0.001), and 93% had values within or above the control range.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
Chronic undernutrition/growth retardation in cystic fibrosis.
There is now sufficient evidence to prove that the maintenance of a high energy normally balanced diet prevents malnutrition/growth retardation and may well enhance prognosis in patients with CF. Most patients are able to tolerate the normal to high fat content without undue problems with steatorrhoea and with the recent advent of more effective enzyme replacement therapy, this should be even less of a problem. Conversely, there is an equally large bulk of evidence to indicate that the maintenance of a low fat diet, while controlling symptoms from steatorrhoea in some patients, is energy depriving and produces growth failure. In children or teenagers who are presently growth retarded, installation of a high energy intake may improve growth in some, but not in others. Further investigation of the latter patients is required to evaluate their persistent anorexia and advantages and disadvantages of nutritional supplementation by invasive techniques. Certainly growth and wellbeing can be vastly improved. However, such studies may well have to be multicentered to obtain sufficient patients to control for the many variables involved, in order to demonstrate the effects on pulmonary function.
Adolescent↗
Bentiromide test for assessing pancreatic dysfunction using analysis of para-aminobenzoic acid in plasma and urine. Studies in cystic fibrosis and Shwachman's syndrome.
We evaluated the bentiromide test by analyzing para-aminobenzoic acid (PABA) in plasma and urine (a) for the identification of patients with complete pancreatic insufficiency and (b) as an alternative to the secretin-cholecystokinin test. Nine control subjects, 18 patients with cystic fibrosis, and 4 patients with Shwachman's syndrome were studied. Based upon the secretin-cholecystokinin test, pancreatic function was judged to be less than 0.1% of normal in 7 patients with cystic fibrosis and malabsorption and between 0.7% and 90% of control values in 11 patients with cystic fibrosis and 4 patients with Shwachman's syndrome without malabsorption. The bentiromide test was performed in two stages: first with bentiromide alone, then with equimolar free PABA. After ingestion of free PABA, the plasma profile and urinary excretion of PABA were comparable in controls, patients with cystic fibrosis, and patients with Shwachman's syndrome. Thirty minutes after oral bentiromide, plasma PABA values in patients with and without malabsorption were significantly lower than in the control group. From 60 to 180 min after ingestion, plasma PABA levels in patients without malabsorption were no different from controls; whereas levels in patients with malabsorption were significantly lower than in controls and in those without malabsorption, reaching the highest significance at 90 min. Similar results were obtained when the urinary excretion of PABA was considered. Only the 90-min plasma test reliably detected cystic fibrosis patients with steatorrhea, however. Duodenal colipase output was highly correlated with both the 90-min plasma test and the urinary excretion of PABA, with similar results for lipase and trypsin output. Reliable detection of pancreatic dysfunction, nevertheless, was not obtained even with the plasma test, in cystic fibrosis patients with greater than 5%-10% of the mean normal enzyme output. In patients with Shwachman's syndrome, none of whom had malabsorption, the plasma and urinary test failed to detect pancreatic dysfunction even with enzyme output as low as 1% of normal.
4-Aminobenzoic Acid↗
Elevated serum immunoreactive pancreatic cationic trypsinogen in acute malnutrition: evidence of pancreatic damage.
We used a sensitive probe of pancreatic dysfunction, serum immunoreactive cationic trypsinogen, to study 50 infants and children with varying degrees of malnutrition. Patients were classified into subgroups according to the severity of malnutrition. Mean serum trypsinogen concentration was significantly elevated in 25 patients with "severe" malnutrition (77.4 +/- 42.0 ng/ml, P less than 0.001) and in 23 with "moderate" malnutrition (55.2 +/- 16.1 ng/ml, P less than 0.02) compared with the mean value (32.5 +/- 10.4 ng/ml) for well-nourished controls. The level of circulating trypsinogen tended to rise with increasing severity of malnutrition. There was no relationship between serum trypsinogen and other variables such as age, specific diagnosis, or mode of feeling. Elevated serum trypsinogen levels could not be attributed to renal disease or cystic fibrosis. In patients who showed an improvement in nutritional status, serum trypsinogen tended to revert toward normal. Elevated serum trypsinogen values in acutely malnourished infants and children may result from pancreatic acinar cell damage or regurgitation of enzymes from obstructed pancreatic ducts.
Acute Disease↗
Serial alterations in the forms of immunoreactive pancreatic cationic trypsin in plasma from patients with acute pancreatitis.
We employed a radioimmunoassay for human cationic trypsin to define the time course for alterations in the molecular forms of this enzyme in plasma from patients with pancreatitis. Six patients developed acute pancreatitis as a complication of a known disorder [three, Reye's syndrome; two, hemolytic uremic syndrome (HUS); one, choledochal cyst]. The immunoreactive forms of cationic trypsin were determined by gel filtration of each plasma sample followed by radioimmunoassay of the column fractions. Early in the course of the disease, predominantly free trypsinogen was released into the circulation in five patients. In the three patients with Reye's syndrome, subsequent plasma samples showed, in addition to free trypsinogen, increasing amounts of immunoreactive trypsin complexed to alpha 2-macroglobulin and alpha 1-protease inhibitor. In contrast, subsequent samples from the two patients with HUS contained little or no inhibitor-bound trypsin. The remaining patient had intermediate concentrations of cationic trypsin complexed to these two circulating protease inhibitors. Five patients died and postmortem studies showed a striking correlation between the histological severity of acute pancreatic inflammation and the amount of immunoreactive trypsin complexed to alpha 2-macroglobulin and alpha 1-protease inhibitor. This preliminary study suggests that measurement of alpha 2-macroglobulin or alpha 1-protease inhibitor-bound trypsin may be a useful method of monitoring the progression and severity of disease in patients with acute pancreatitis. Characterization of serial changes in the forms of circulating pancreatic proteases may enhance our understanding of time-dependent pathophysiologic events, possibly leading to improved forms of specific therapy.
Acute Disease↗
Pancreatic function testing in cystic fibrosis.
None of the currently available clinical techniques for measuring pancreatic function in patients with CF can be considered "ideal". The "direct" pancreatic stimulation test provides the most information and remains the only truly accurate test of exocrine function, particularly in those individuals with pancreatic sufficiency. The invasive, complex nature of the pancreatic stimulation tests precludes its use as a routine method of assessing pancreatic function in all patients and limits its value for monitoring function in those individuals who are expected to deteriorate with time. Many of the indirect tests of pancreatic function are of value for identifying CF patients with complete pancreatic insufficiency who may require therapy with pancreatic enzyme supplements and fat soluble vitamins. However, none of the currently available "indirect" tests of exocrine function are sensitive enough to evaluate or monitor CF patients with pancreatic sufficiency. Blood tests, capable of measuring pancreatic enzymes in the circulation, such as immunoreactive trypsinogen, may be of value for monitoring pancreatic function in patients with CF, but further longitudinal studies are necessary to fully evaluate this approach. More sophisticated "indirect" tests of exocrine pancreatic function are vitally needed to facilitate adequate assessment of this important digestive organ in patients with cystic fibrosis.
Cystic Fibrosis↗