Conjugated bile acid replacement therapy for short-bowel syndrome.

BACKGROUND & AIMS: Although fat malabsorption in the short-bowel syndrome is caused in part by decreased bile acid secretion, bile acid replacement therapy is not used because of the belief that ingested bile acids would worsen diarrhea, outweighing the benefits of improved fat absorption. This study compared the effect of a natural conjugated bile acid mixture from ox bile with that of cholylsarcosine, a synthetic conjugated bile acid, on fat absorption and diarrhea in a patient with the short-bowel syndrome. Cholylsarcosine is resistant to bacterial metabolism and has no cathartic activity. METHODS: Metabolic balance studies and a clinical trial were performed in an emaciated patient with the short-bowel syndrome and ileostomy in whom parenteral nutrition could not be used. RESULTS: In balance studies, conjugated bile acid replacement therapy with either preparation caused fat absorption to increase by approximately 40 g/day. Calcium absorption also increased. Neither bile acid product caused a clinically significant increase in ileostomy water output. During a 4-month outpatient trial, while the patient ingested 2 g/meal natural bile acids, her weight increased from 80 to 98 lb, without side effects. CONCLUSIONS: Conjugated bile acid replacement therapy should be part of the armamentarium for the treatment of selected patients with the short-bowel syndrome.

Diagnostic value of fasting plasma peptide concentrations in patients with chronic diarrhea.

To evaluate the utility of screening for multiple gastrointestinal peptides in the evaluation of patients with chronic diarrhea, we studied 193 patients referred for evaluation of chronic diarrhea and eight patients with known peptide-secreting tumors as a reference group. Fasting plasma samples were assayed for motilin, neurotensin, pancreatic polypeptide, somatostatin, substance P, vasoactive intestinal polypeptide, gastrin-releasing peptide, and calcitonin during a protocol evaluation for causes of chronic diarrhea. Although none of the referred patients were found to have tumors, abnormal levels of one or more peptides were found in 86 of 193 patients (45%). Abnormal plasma peptide levels were sometimes as high in these patients as in patients with known peptide-secreting tumors and would have led to mistaken diagnoses of tumors much more often than they would have led to correct diagnoses. The positive predictive value of elevation of any assayed peptide was < 2% at realistic prevalence rates for peptide-secreting tumors; the negative predictive value of a series of normal results was > 99%, but much of this was due to the rarity of these tumors. Patients with chronic diarrhea should not be screened routinely with a panel of plasma peptide assays in an effort to detect tumors; instead, peptide levels should be ordered selectively. Elevated fasting concentrations of the plasma peptides measured in this study are most likely epiphenomena due to diarrhea and should not be the sole basis for invasive diagnostic or surgical management of these patients.

The self-limited nature of chronic idiopathic diarrhea.

BACKGROUND: Little is known about the clinical presentation and natural history of previously healthy patients in whom chronic idiopathic diarrhea develops. METHODS: We reviewed the case records of 152 patients with chronic diarrhea who had no history of gastrointestinal surgery and who were evaluated in detail as part of a chronic-diarrhea protocol from 1985 to 1990. Patients were considered to have chronic idiopathic diarrhea if they had persistently loose stools for more than four weeks, no systemic illness, and no identifiable cause of diarrhea. RESULTS: Seventeen patients (10 men and 7 women) ranging in age from 33 to 72 years met the criteria for chronic idiopathic diarrhea. Each patient had a history of a relatively abrupt onset of symptoms, often soon after returning home from a trip, starting two to seven months before evaluation. Their diarrhea did not occur during a local outbreak of diarrhea, and other family members did not become ill. Stool frequency ranged from 5 to 25 movements per day, stool weights ranged from 417 to 1480 g per day, and fecal electrolyte and osmolality values were consistent with a diagnosis of secretory diarrhea. The results of biopsies of the small intestine and colon were normal, as were small-bowel roentgenograms. Extensive studies for infectious causes of diarrhea were negative, and no patient responded to antibiotic therapy. In every patient the diarrhea stopped without specific therapy after 7 to 31 months (mean, 15) and did not recur during a follow-up period averaging 38 months. CONCLUSIONS: Sporadic idiopathic chronic diarrhea is a recognizable syndrome that can last many months, but is self-limited.

Treatment of severe steatorrhea with ox bile in an ileectomy patient with residual colon.

Patients with ileectomy can present with severe steatorrhea due to bile acid depletion. While exogenous bile acid would seem to be ideal therapy for this condition, it is not often used because it is thought that the bile acid would be malabsorbed and would enter the colon, producing a secretory diarrhea. This report describes a patient who had an ileectomy and partial right colon resection for Crohn's disease and then developed severe steatorrhea due to bile acid malabsorption. Although steatorrhea was reduced from 134 to 9 g/24 hr with chronic ingestion of ox bile extract, stool weight did not increase with ox bile (stool weight 669 g/24 hr before therapy and 507 g/24 hr after therapy). In this patient, oral bile acid supplementation reduced fat excretion markedly, but did not aggravate diarrhea, even though the colon was still present. This result may have been due to impaired solubility of bile acid in fecal fluid due to an acid pH or to binding of bile acid with particles of solid stool. Ingestion of ox bile extract does not necessarily increase stool weight in patients with defective fat absorption due to bile acid malabsorption and can be tried with caution in an effort to diminish steatorrhea.

Loss of absorptive capacity for sodium chloride as a cause of diarrhea following partial ileal and right colon resection.

Previous studies have emphasized the role of bile acid and fat malabsorption as the cause of the diarrhea that may follow ileal and right colon resection; unabsorbed bile acids and fat are believed to reduce sodium chloride and water absorption in the remaining colon. In this paper we report studies in eight patients with severe postresection diarrhea, in search of a more basic defect in sodium chloride absorption, ie, a loss of sodium chloride absorptive capacity as a direct consequence of resection of sodium chloride absorption sites. First, we determined whether or not diarrhea persisted during a 48-hr fast; in all patients diarrhea and large fecal electrolyte losses continued during a fast. Second, we measured sodium chloride and water absorption rates during total gut perfusion with a balanced electrolyte solution; compared to normal controls, the patients absorbed 23-31% less water, sodium, and chloride. In three patients who could be studied further, the absorptive defect was markedly accentuated when the perfusing solution was such that sodium chloride absorption had to take place against a concentration gradient. These observations indicate that postresection diarrhea patients have a reduced capacity to absorb sodium chloride, particularly when there is a concentration gradient between lumen and plasma. Although all of these patients had malabsorption of radiolabeled taurocholic acid, there was only a modest and statistically insignificant reduction in daily stool weight during treatment with large doses of cholestyramine, suggesting that bile acid malabsorption was not responsible for a major part of their diarrhea.(ABSTRACT TRUNCATED AT 250 WORDS)

Choledochal cyst.

A 16-year-old girl with recurrent pain in the right upper quadrant and abnormal results of liver function tests was found to have a large choledochal cyst filled with stones. Computerized tomography and endoscopic retrograde cholangiopancreatography showed the large cyst with innumerable primary cyst stones and an anomalous pancreaticobiliary duct junction.

Laser photocoagulation for the treatment of acute peptic-ulcer bleeding. A randomized controlled clinical trial.

We tested the hypothesis that therapeutic endoscopy using the Nd:YAG (neodymium:yttrium-aluminum-garnet) laser would benefit patients with acute peptic-ulcer bleeding. Over 43 months, 174 patients with active bleeding (n = 32) or stigmata of recent bleeding (n = 142) due to peptic ulcers were randomly assigned during endoscopy to either standard treatment with laser photocoagulation or therapy without photocoagulation. There were no significant differences in a number of outcomes between the group treated with laser photocoagulation and the control group. Continued bleeding or rebleeding was observed in 22 percent of the laser-treated group and in 20 percent of the control group. Urgent surgery was necessary in 16 percent of the laser-treated patients and in 17 percent of the controls. Laser-treated patients spent a mean of 41 hours in the intensive care unit, and controls spent a mean of 32 hours. The mean hospital stay was 12 days in the laser-treated group and 11 days in the control group. One death occurred in each group. When patients with active bleeding were analyzed separately, there was no significant difference in outcome, even though laser photocoagulation stopped active bleeding in 88 percent of cases. Among patients with visible vessels, rebleeding occurred in 5 of 14 (36 percent) who received laser treatment and 2 of 15 (13 percent) who did not. Laser treatment precipitated bleeding in four patients and duodenal perforation in one. We conclude that Nd:YAG-laser photocoagulation does not benefit patients with acute upper gastrointestinal bleeding from peptic ulcers.

Cranial nerve deficits due to amyloidosis associated with plasma cell dyscrasia.

Cranial nerve dysfunction is exceedingly rare in amyloidosis. Cranial nerve deficits have been described in the very rare form of familial amyloidosis in which there appears to be an autosomal dominant pattern of inheritance. By contrast, they are virtually unreported in the two common forms of amyloidosis, that is, secondary amyloidosis (AA), usually associated with a chronic inflammatory lesion, and primary amyloidosis (AL), resulting from light-chain immunoglobulins of plasma cell dyscrasias. Although neurologic involvement in these forms of amyloidosis has been reported in the peripheral nerves, spinal nerve roots, and autonomic ganglia, the absence of reports of cranial nerve lesions is remarkable. We report the case of a woman with multiple bilateral cranial nerve deficits involving the left trigeminal, left facial, right abducens, and hypoglossal nerves. She had nonfamilial amyloidosis associated with a plasma cell dyscrasia of the IgG kappa type with increased production of light-chain immunoglobulins. The rarity of cranial nerve involvement in this clinical setting is highlighted by three previous studies, each of which reported involvement of only a single nerve (either the facial or olfactory nerve). Since aging has been correlated with congophilic changes in the brain, the explanation for the sparing of cranial nerves can be only speculative.