Seven patients with familial amyloid neuropathy (AF amyloidosis) were studied to clarify the pathophysiology of the diarrhea associated with this disorder. Fecal weight and fat determinations, 14C-glycocholate breath tests, and a test of B12 absorption were performed before and after treatment with co-trimoxazole. Gastric emptying was assessed with conventional roentgen contrast medium and radio-opaque markers. Gastric emptying was delayed, fecal weight and fat excretion increased, and the 14C-glycholate breath test abnormal in all but one patient. In most cases co-trimoxazole reduced diarrhea, steatorrhea, and 14C-glycine deconjugation; vitamin B12 absorption returned to normal in one patient after co-trimoxazole treatment. In a jejunal mucosal biopsy specimen, amyloid was absent in the villi, but small deposits were detected along small vessels and nerves in the lamina propria. These findings suggest altered gastrointestinal motility due probably to an autonomic neuropathy which in turn leads to enteral bacterial overgrowth and subsequently to diarrhea and steatorrhea. This diarrhea can be temporarily alleviated by co-trimoxazole treatment.
Xenin, a recently discovered peptide produced by specific endocrine cells of the duodenal mucosa, has shown exocrine, endocrine and motility effects in the gastroenteropancreatic system in animal experiments. The aim of the present investigation was to study the role of xenin in the regulation of duodenojejunal motility of humans. Twenty-nine healthy volunteers from the hospital staff gave informed consent to participate in this investigation. In 20 volunteers, we determined plasma concentrations of immunoreactive xenin at 15 min intervals over a mean time period of 8 h fasting and recorded the interdigestive motor activity of the duodenojejunum. In a double-blind randomized crossover study on other nine subjects, synthetic xenin in a dose of 4 pmol kg-1 min-1 or placebo was infused for 10 min intravenously in the interdigestive period and postprandially after a liquid meal. Duodenojejunal motility was recorded simultaneously. Predefined interdigestive xenin plasma peaks were found to be significantly associated with the phases III of the migrating motor complex. In the interdigestive period, xenin induced a premature phase III activity in each volunteer; this was followed by a second phase III in five out of nine subjects. In the postprandial state, xenin significantly increased contraction frequency and the percentage of aborally propagated contractions. These findings suggest a role of the peptide hormone xenin in modulating interdigestive and postprandial duodenojejunal motility in humans.
Impairment of social function was measured by questionnaire in 270 patients with Crohn's disease. In about half of them, the disease had had its onset below the age of 20 years. In 10%, schooling or job training was delayed or even had to be abandoned. Ten percent of the patients were forced to change their occupation because of the disease. 23.5% of the patients stated that their illness had prevented them from having whole-day jobs. Ten percent had temporary or permanent pensions; 3.8% were unemployed. 43.7% of patients stated that the relation to their family and (or) partner was impaired by the disease; in 36.4% the disease had interfered with leisure time activities. The results show that Crohn's disease has social effects which in the individual case can be very serious; they correlate with the duration and severity of the disease. Nonetheless, the majority of patients succeeds in mastering their chosen training and occupation and leading an active life.
Peripheral polyneuropathy associated with recurrent diarrhoea and orthostatic hypotension was observed in two unrelated German kinships and two sporadic cases. Congo red staining and polarization microscopy of biopsy specimens revealed amyloid deposits. Immunohistochemical investigation using the indirect immunoperoxidase staining with antisera to several purified amyloid fibril proteins showed a positive reaction with an antiserum to the prealbumin-related AF-amyloid in the families and one of the sporadic cases and with an antiserum to the immunoglobulin light chain amyloid (A lambda) in the other sporadic case. Therefore, the amyloid of the families and one of the sporadic cases was identified as the prealbumin-related AF amyloid, while the amyloid of the other sporadic case was of immunoglobulin origin. It is concluded that immunohistochemistry with antisera to the different amyloid proteins is useful in the differential diagnosis of amyloid neuropathy.
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