Background: Pancreatic enzyme replacement therapy frequently fails to correct intestinal fat malabsorption completely in cystic fibrosis (CF) patients. The reason for this failure is unknown. Objective: We investigated whether fat malabsorption in CF patients treated with pancreatic enzymes is caused by insufficient lipolysis of triacylglycerols or by defective intestinal uptake of long-chain fatty acids. Design: Lipolysis was determined on the basis of breath 13 CO 2 recovery in 10 CF patients receiving pancreatic enzyme replacement therapy after they ingested 1,3-distearoyl,2[1- 13 Results: Fecal fat excretion ranged from 5.1 to 27.8 g/d (x -± SD: 11.1 ± 7.0 g/d) and fat absorption ranged from 79% to 93% (89 ± 5%). There was no relation between breath 13 CO 2 recovery and dietary fat absorption (r = 0.04) after ingestion of [ 13 C]MTG. In contrast, there was a strong relation between 8-h plasma [ 13 C]LA concentrations and dietary fat absorption (r = 0.88, P < 0.001). Conclusion: Our results suggest that continuing fat malabsorption in CF patients receiving enzyme replacement therapy is not likely due to insufficient lipolytic enzyme activity, but rather to incomplete intraluminal solubilization of long-chain fatty acids, reduced mucosal uptake of long-chain fatty acids, or both. Am J Clin Nutr 1999;69:127-34. KEY WORDSBreath test, mixed triacylglycerol, [13 C]linoleic acid, fat malabsorption, fat balance, lipolysis, stable isotopes, cystic fibrosis, recommended dietary allowance, children, long-chain fatty acids INTRODUCTIONIn humans, triacylglycerols composed of long-chain fatty acids constitute 92-96% of dietary fats (1). Absorption of these fats is by 2 main processes. Lipolysis, by lipolytic enzymes originating predominantly in the pancreas, leads to hydrolysis of triacylglycerols into fatty acids and 2-monoacylglycerols. Second, intestinal uptake involves the formation of mixed micelles composed of bile components and lipolytic products, followed by the disintegration of the mixed micelles in the unstirred water layer and the translocation of the lipolytic products across the intestinal epithelium (1-4).Most cystic fibrosis (CF) patients malabsorb dietary fats because of pancreatic insufficiency, which leads to impaired lipolysis (5, 6). The symptoms of pancreatic insufficiency, such as steatorrhea and poor growth, can be alleviated by oral supplementation with pancreatic enzymes. However, despite recent improvements in the pharmacokinetics of these supplements, many patients continue to experience a certain degree of steatorrhea (7-9), with 10-20% of the dietary fat they consume being malabsorbed. It has not been elucidated whether this malabsorption is due to insufficient pancreatic enzyme replacement therapy. This possibility is likely because decreased pancreatic bicarbonate secretion may negatively affect enzyme activity by sustaining a low pH in the duodenum (10, 11). At a low duodenal pH, the release of enzymes from the microcapsules is inhibited and denaturation of the enzymes is stimul...

Objective To eva luate the therapeutic efficacy of intravesica l pentosanpolysu lph ate (PPS) compared with placebo in patients with interstitial cystitis (rC). Patients and methods Twenty patients who fullfilled the diagnostic criteria for IC participated in a double-blind placebo-controlled study: 10 received intravesical PPS (300 mg in SO mL of 0.9% sodium chloride) applied twice a week for 3 months and the other 10 received a placebo. Symptomatic relief and objective variables (bladder capacity voiding volumes and urinary frequency) were assessed after 3 months and the long-term outcome of those continuin g treatment was determined. Results Of the patients treated with PPS, four gained

Selective decontamination of the digestive tract does not prevent infection in patients undergoing elective liver transplantation and increases the cost of their care. It does, however, affect the type of infection. Infections with Gram-negative bacilli and with Candida species are replaced by infections with Gram-positive cocci.

To determine the therapeutic effect of adjuvant dexamethasone pulse therapy when given in addition to conventional treatment of pemphigus vulgaris.Design: A randomized, placebo-controlled trial.Setting: International European, multicenter outpatient and inpatient study.Patients: Of the 20 enrolled patients, 11 were randomized to the dexamethasone pulse (DP) group and 9 to the placebo pulse (PP) group.Interventions: Oral dexamethasone in 300-mg pulses or PPs 3 days per month. During the intervention, the DP and PP groups received conventional treatment with prednisolone, 80 mg/d, which was tapered across 19 weeks, and azathioprine sodium, 3 mg/kg per day, until the end of the study. Monthly pulses were continued until prednisolone treatment was tapered to 0 mg.Main Outcome Measures: Number of patients in remission, time to and duration of remission, cumulative prednisolone dose, and occurrence of adverse events during 1 year of follow-up.Results: Eight of the 11 DP-treated patients and all 9 PP-treated patients achieved remission. Mean time to remission was 173 days with DP and 176 days with PP. The mean duration of remission within the first year was 151 days for DP and 141 days for PP. Mean cumulative prednisolone dose was 5300 mg for DP and 4882 mg for PP. Weight gain (Ͼ5% of baseline) occurred in 8 DPtreated patients compared with 1 PP-treated patient (PϽ.01).We found no statistically significant difference (PϾ.05) of an adjuvant effect of DP on remission of pemphigus vulgaris. Conclusion:In patients with new pemphigus vulgaris disease activity, there was no benefit of oral DP therapy given in addition to conventional treatment.

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