Crohn's disease and the Melkersson-Rosenthal syndrome.

A 30-year-old woman with a 10-year history of recurrent bloody diarrhea and documented colitis of the descending colon, consistent with Crohn's disease, presented with an exacerbation of her gastrointestinal disease and an 18-month history of recurrent facial and genital swelling. Her course evolved to include severe ear pain, dysphagia and colonic dysmotility. She was diagnosed with Melkersson-Rosenthal syndrome and treated with multiple agents. The neurological aspects of her presentation are highlighted, and the Melkersson-Rosenthal syndrome is reviewed.

Comparison of salivary and serum enzyme immunoassays for the diagnosis of Helicobacter pylori infection.

Infection with Helicobacter pylori has been established as an important risk factor for the development of peptic ulcer disease, gastritis and gastric cancer. The diagnosis of H pylori infection can be established by invasive or noninvasive techniques. Two noninvasive enzyme immunoassays (EIAs) for antibody detection - HeliSal and Pylori Stat - were compared with histology. Both assays detect immunoglobulin (Ig) G directed against purified H pylori antigen. The test populations consisted of 104 consecutive patients scheduled for upper gastrointestinal endoscopy. Of these patients, 97 (93%) had symptoms compatible with peptic ulcer disease. Saliva and serum were collected simultaneously at the time of endoscopy. Salivary EIA had a sensitivity of 66%, specificity of 67%, positive predictive value of 67% and negative predictive value of 66% compared with the serum EIA, where the results were 98%, 48%, 64% and 96%, respectively. Although the salivary EIA is an appealing noninvasive test, it was not a sensitive and specific assay. The serum EIA also lacked specificity, but was highly sensitive with a good negative predictive value. Although a negative serum EIA rules out H pylori infection, a positive result must be interpreted in the clinical context and confirmed with a more specific measure.

Reproducibility of ambulatory esophageal pH monitoring in the aperistaltic esophagus.

The reproducibility of ambulatory 24-h esophageal pH monitoring was evaluated in 16 consecutive patients by comparing the difference in two consecutive 24-h periods. The study group included 8 patients with scleroderma esophagus and 8 treated achalasia patients. The amount of reflux was expressed as the percentage of time the pH was < 4.0. Both groups demonstrated excellent intrapatient reproducibility overall: 96% in scleroderma patients and 95% in those patients with achalasia. The least concordance was found in the lengths of the longest reflux events-70% when supine in scleroderma patients and 59% when upright in patients with achalasia. There was no significant difference (p > 0.05) between day 1 and day 2 for either group of patients for any of the elements studied. These results indicate that intrapatient variability of gastroesophageal reflux in patients with scleroderma esophagus and treated patients with achalasia is very low and following therapeutic intervention, a high level of confidence can be placed in subsequent pH monitoring as an indicator of treatment effect.

Somatostatinoma syndrome. Biochemical, morphologic and clinical features.

Diabetes mellitus, steatorrhea, cholelithiasis and a tumor distorting the duodenum prompted a work-up for somatostatinoma in a 52-year-old man. The responses of pancreatic B-cells but not of A-cells to nutrient stimuli were inhibited, and growth-hormone release was suppressed, suggesting somatostatin resistance in some target tissues. Plasma somatostatin-like immunoreactivity ranged from 9000 to 13,000 pg per milliliter (normal: 88+/-8, mean +/- S.E.M.) and was distributed in four molecular forms, including free somatostatin. The primary tumor contained 5 microgram of somatostatin-like immunoreactivity per milligram of wet tissue, distributed in three of the molecular forms noted in plasma. Plasma calcitonin was also elevated (4650 pg per milliliter; normal: less than 120). Immunocytochemical studies showed that cells of the primary tumor contained somatostatin and calcitonin but no other peptide hormones. Only somatostatin was present in the metastases. Somatostatin was localized electron microscopically in all secretory granules, irrespective of size and shape, whereas calcitonin was present only within a single subpopulation of small granules in the same cells.

Liver function and gastric acid secretion in parkinsonian patients under prolonged treatment with L-dopa and a peripheral decarboxylase inhibitor.

Twelve patients with long-standing Parkinson's disease, treated successfully with a combination of l-dopa and an inhibitor of aromatic l-amino acid decarboxylase (Ro 4-4602), in the ratio 4:1, were screened for damage to various organ systems, in particular liver and skeleton. Among other tests, liver biopsies were obtained before and after 6 months of treatment. One patient discontinued therapy becuase of an accentuation of pre-existent liver damage, another because of psychomental manifestations. The remaining ten patients were followed for 8-15 months and longer. The liver biopsies remained practically unaltered. Elevation of alkaline phosphatase was found in 10 out of 12 subjects. In five patients this rise fluctuated around the upper limit of normal. In two patients, who discontinued the treatment, the raised alkaline phosphatase values soon returned to normal. Analysis of isozymes proved this phosphatase to be of liver origin. All other liver function tests remained unchanged, except for an increased retention of bromsulphalein in two patients. In the one patient with the initially damaged liver, all tests became normal soon after discontinuation of therapy. No changes could be found in gastric acid secretion. All other parameters studied remained within normal limits, including urinary excretion of calcium and phosphate.