The objective of this study was to investigate the presence of nailfold capillary abnormalities and extrahepatic signs of connective tissue disease in patients with primary biliary cirrhosis (PBC), as compared to patients with other chronic liver diseases. We evaluated 22 patients with PBC and 15 patients with other chronic liver diseases as a control group. Nailfold capillaroscopy was performed by two observers blinded to clinical findings using a Wild M3 stereomicroscope with an Intralux 5000 Volpi cold light lamp. We detected nailfold capillary abnormalities in 20 out of 22 (91%) PBC patients. Twelve of these 20 patients (54%) showed capillary alterations characteristic of systemic sclerosis. In the control group only two out of 15 patients (13%) presented alterations and in both cases they were a non-specific type. The presence of nailfold capillary abnormalities was significantly greater in PBC patients than in the control group (P < 0.001). Eleven out of the 22 PBC patients (50%) had extrahepatic signs of connective tissue disease and most of them were related to systemic sclerosis; patients with other chronic liver diseases did not present rheumatic manifestations (P < 0.001). In PBC patients there was a significant association between systemic sclerosis capillary pattern and rheumatic manifestations (P < 0.03). The high prevalence of nailfold capillary abnormalities characteristic of systemic sclerosis in patients with PBC and the correlation with sclerodermal manifestations suggests that this capillaroscopic finding could be a useful indicator to investigate rheumatic manifestations in these patients.
Background: The regulated ability of sphincters to relax allows adequate control of digestive transit. Relaxation of the lower esophageal sphincter (LES) is essential for esophageal emptying and, similarly, pyloric relaxation permits gastric emptying. When the relaxatory response of these sphincters is impaired, luminal transit is altered, as occurs in achalasia and hypertrophic pyloric stenosis. Nitric oxide (NO) has been identified as the main inhibitory neurotransmitter in both sphincteric regions. Moreover, the absence of NO synthase in the LES and the pylorus has been implicated in the pathogenesis of infantile hypertrophic pyloric stenosis (IHPS) and achalasia, respectively. Case Report: We present the case of a 12-year-old boy diagnosed with these two different conditions attributed to NO absence: IHPS and achalasia. Conclusion: To our knowledge this is the first time that such an association has been reported. Whether IHPS and achalasia have been associated in this patient by chance or because they share common pathophysiological mechanisms remains speculative, but is a tantalizing dilemma.
Gastric relaxation invariably precedes nausea and emesis and is of the same magnitude whether induced by apomorphine (central stimulus) or intragastric lipid infusion (peripheral stimulus). Nevertheless, in some instances and depending on the nature of the stimulus, fundic relaxation is a physiological event unassociated with nausea.
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