Association of spinocerebellar disorders with cystic fibrosis or chronic childhood cholestasis and very low serum vitamin E.

We have investigated whether jejunal hyperacidity leads to bile acid precipitation and thus limits lipid solubilisation in patients with pancreatic steatorrhoea. Jejunal contents from 12 adults with steatorrhoea due to cystic fibrosis were aspirated for three hours after a liquid test meal, and pooled according to their pH. Thirty eight per cent of the total aspirate was collected at pH less than 5 in cystic fibrosis, compared with 18% in healthy controls (p less than 0.05). Forty six per cent of the bile acids were precipitated at pH less than 5, compared with 15% at pH greater than 6 (p less than 0.01), leading to reduced aqueous phase bile acid concentration at low pH (4.7 mmol/l at pH less than 5 vs 12.5 mmol/l at pH greater than 6, p less than 0.01). Aqueous phase lipid concentrations were reduced at low pH (5.6 mmol/l at pH less than 5 vs 10.2 mmol/l at pH greater than 6, p less than 0.01). Lipolysis and total fatty acid concentrations were greatly reduced and did not vary with pH. We therefore conclude that jejunal hyperacidity leads to bile acid precipitation in pancreatic steatorrhoea due to cystic fibrosis, and imposes a further limitation on lipid solubilisation over that of lipase deficiency.

https://gut.bmj.com/content/gutjnl/25/5/500.full.pdf

Effect of intrajejunal acidity on aqueous phase bile acid and lipid concentrations in pancreatic steatorrhoea due to cystic fibrosis.

https://aasldpubs.onlinelibrary.wiley.com/doi/pdf/10.1002/hep.1840140505

Gallstones in cystic fibrosis: a critical reappraisal.

Patients with cystic fibrosis have an increased proportion of glycine conjugated bile acids with diminished tauroconjugates which could contribute to fat malabsorption. Twenty-two CF children with documented steatorrhea were supplemented with taurine capsules (30 mg/kg/day) and placebo during separate 6-month treatment periods. Alteration of the glycine/taurine conjugation pattern was verified in two patients who showed a predominance of tauroconjugates as a result of taurine supplementation. On taurine, steatorrhea was reduced (p less than 0.05) by 17.6 +/- 9.7% in 19 patients who completed the study as was the excretion of long-chain saturated fatty acids. There was no change in linoleic acid (C 18:2) excretion. In the 10 patients with a more severe degree of steatorrhea the decrease in fat loss approached 20% and a close relationship was found (r = 0.84, p less than 0.01) between the extent of the fatty acid loss on placebo and the decrease of this loss on taurine. A linear relationship was found between the percentage decrease of individual fatty acids and their log solubility in water. No change was found in the daily excretion of bile acids, neutral sterols, and nitrogen. Fasting plasma fatty acids, cholesterol, and triglycerides were also unchanged. Monitoring of growth over the two 6-month periods revealed a marginal (p less than 0.1) increase of weight velocity expressed as a percentage expected for age (83.4 +/- 11.3----117.1 +/- 16.5). The increase in height velocity in response to taurine showed a more modest trend (95.3 +/- 7.8----110.7 +/- 10.6).(ABSTRACT TRUNCATED AT 250 WORDS)

/pdf/effect-of-taurine-supplements-on-fat-absorption-in-cystic-3p9vgs780r.pdf

Effect of Taurine Supplements on Fat Absorption in Cystic Fibrosis

Compound solubility serves as a surrogate indicator of oral biopharmaceutical performance. Between infancy and adulthood, marked compositional changes in gastrointestinal (GI) fluids occur. This study serves to assess how developmental changes in GI fluid composition affects compound solubility. Solubility assessments were conducted in vitro using biorelevant media reflective of age-specific pediatric cohorts (i.e., neonates and infants). Previously published adult media (i.e., FaSSGF, FeSSGF, FaSSIF.v2, and FeSSIF.v2) were employed as references for pediatric media development. Investigations assessing age-specific changes in GI fluid parameters (i.e., pepsin, bile acids, pH, osmolality, etc.) were collected from the literature and served to define the composition of neonatal and infant media. Solubility assessments at 37°C were conducted for seven BCS Class II compounds within the developed pediatric and reference adult media. For six of the seven compounds investigated, solubility fell outside an 80–125% range from adult values in at least one of the developed pediatric media. This result indicates a potential for age-related alterations in oral drug performance, especially for compounds whose absorption is delimited by solubility (i.e., BCS Class II). Developmental changes in GI fluid composition can result in relevant discrepancies in luminal compound solubility between children and adults.

/pdf/assessment-of-age-related-changes-in-pediatric-v3hgiq8c5c.pdf

Assessment of Age-Related Changes in Pediatric Gastrointestinal Solubility

The quantitative and qualitative distribution of bile salts in the duodenal juice of 13 patients with cystic fibrosis (CF) was studied after a test meal. The effects of triolein (TO), bovine serum albumin (BSA), and ricinoleic acid (RA) on the absorption of taurocholate (TCA) in the distal ileum of the rat in vivo was also studied. The mean (and ranges) of total bile salt concentrations, glycine: taurine conjugate ratios, and percentage of dihydroxy bile salts in the patients with CF and pancreatic insufficiency were 3.5 (1.3--6.6) mmol/l, 8.6 (greater than 10-3.1), and 37 (10--60) compared with control values of 7.4 (3.0--16.0) mmol/l, 3.0 (1.3--4.5), and 61 (52--70) respectively. The differences between the control and CF values were statistically significant (P less than 0.01--P less than 0.001). Three of the 13 CF patients had total bile salt concentrations less than 2 mmol/l, 8 had much higher glycine: taurine ratios, and 8 had a reduced percentage of dihydroxy bile salts. In 2 patients with normal pancreatic enzyme activities, duodenal bile salts were both quantitatively and qualitatively normal. TO (10 and 30 mmol/l), BSA (3%), and RA (5 mmol/l) had no inhibitory effect on the ileal absorption of TCA. These results show pronounced abnormalities of duodenal juice bile salts in CF with pancreatic insufficiency consistent with a broken enterohepatic circulation (EHC); such abnormalities may contribute to defective lipid absorption in CF. The data in the experimental animal do not support the suggestion that unhydrolysed dietary substrates play a role in the pathophysiology of the broken EHC.

https://adc.bmj.com/content/archdischild/54/1/19.full.pdf

Intestinal bile salts in cystic fibrosis: studies in the patient and experimental animal.

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Intestinal bile salts in cystic fibrosis: studies in the patient and experimental animal.