An analysis was made of the symptom diaries of 65 patients with primary sclerosing cholangitis (PSC) who recorded their symptoms of itching, pain and fever during a 3 year period. Symptoms occurred in 84% of the patients, with considerable individual variations in symptom load. Symptoms were usually intermittent. "Itching only" and "pain only" were the most frequent episode features. Most episodes lasted only 1-2 days. Fever seemed to be the most embarrassing symptom. Apart from close correlations between pruritus and serum ALP levels, there were no significant correlations between the symptom load and serum biochemistry or eleven different histological features. Even daily symptoms over a period of several months could disappear spontaneously. We conclude that most symptom episodes in PSC are mono-symptomatic and brief. Biochemical or histological data do not predict the appearance of symptoms.

Alanine Transaminase↗

PubMedDate not supplied

Ultrasound examination in diffuse liver disease. Clinical significance of enlarged lymph nodes in the hepato-duodenal ligament.

Seventy-three patients with pathologic liver function tests were examined using ultrasound one day prior to liver biopsy. The ultrasound findings were compared with the histologic findings. In 23 patients enlarged lymph nodes were found in the hepatoduodenal ligament and 21 of these had active immune-mediated liver disease. Of the remaining 2 patients one had ulcerative colitis (and fatty liver) and one chronic cholecystitis (and haemosiderosis). Of the 33 patients with biopsy-proven active immune-mediated liver disease 21 had pathologic lymph nodes in the hepato-duodenal ligament at ultrasound. It was not possible to identify the ligament in 8 patients and in the remaining 4 no pathologic lymph nodes could be found. Twenty-one of these patients had normal liver echoes on ultrasound, 5 exhibited increased echogenicity and 5 had heterogeneous echogenicity. In a further 2 patients both increased echogenicity and heterogeneous parenchyma were found. Ultrasound examination of the liver parenchyma alone would thus lead to 21 of the 33 patients being classified as normal and a further 5 being classified as having fatty changes of the liver. Only 7 would be regarded as having significant liver pathology. However, if demonstration at ultrasound of pathologic lymph nodes in the hepato-duodenal ligament is regarded as being consistent with significant hepatic pathology a further 15 patients could be added to these 7 patients, giving a total of 22 out of 33 patients (67%) identified as having significant liver pathology using ultrasound alone. The reliability of ultrasound in the diagnosis of immune-mediated liver disease can thus be improved considerably by actively searching for lymph nodes in the hepato-duodenal ligament.

Adult↗

PubMedDate not supplied

Tissue concentrations of gastrointestinal regulatory peptides in the duodenal mucosa in systemic sclerosis.

OBJECTIVE: Esophageal hypomotility and abnormalities of intestinal function are important manifestations in systemic sclerosis (SSc), but their pathogenesis is not well understood. Since there is evidence that plasma concentrations of certain gastrointestinal regulatory peptides are increased in SSc, we were interested in examining the peptide concentrations and localization in biopsy specimens from the intestinal mucosa in SSc patients. We studied 12 patients with gastrointestinal disease. METHODS: Levels of corticotrophin-releasing hormone (CRH), motilin, neuropeptide Y (NPY) and peptide YY (PYY) were determined by radioimmunoassay and high-performance liquid chromatography (HPLC), and the occurrence of motilin, PYY, somatostatin, and NPY were studied with immunohistochemistry. RESULTS: Except for the concentrations of CRH, which were increased 2-fold, the tissue concentration of motilin, NPY and PYY were decreased by approximately 50% among patients with esophageal and intestinal dysfunction (group B) compared to patients with impaired esophageal motility alone (group A). In addition, HPLC-characterization of motilin, NPY, and PYY showed a different pattern of fragments among patients in groups A and B. In all patients duodenal motilin, PYY, and somatostatin were localized in the endocrine cells. The distribution and frequency of the cells did not differ among the patients. NPY was localized to neuronal elements; there was no overt difference among the patients with respect to the frequency of NPY-containing nerves. CONCLUSION: This study shows that patients with widespread gastrointestinal disease have lower tissue concentrations of regulatory peptides compared to patients with less widespread disease.

Adult↗