Polysaccharide storage myopathy in an Australian Quarter Horse.
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Biomedical subjects
Publications and source records attributed to S Colgan.
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The human major histocompatibility complex (MHC) on chromosome 6 encodes three classical class-I genes: human leukocyte antigens (HLA) A, B, and C. These polymorphic genes encode a 43- to 45-kDa cell surface glycoprotein that, in association with the 12-kDa beta2-microglobulin molecule, functions in the presentation of nine amino acid peptides to the T-cell receptor of CD8-bearing T lymphocytes and killer inhibitory receptors on natural killer cells. In addition to these ubiquitously expressed, polymorphic proteins, the human genome also encodes several nonclassical MHC class-I-like, or class Ib, genes that, in general, encode nonpolymorphic molecules involved in various specific immunological functions. Many of these genes, including CD1, the neonatal Fc receptor for IgG, HLA-G, HLA-E, the MHC class-I chain-related gene A, and Hfe, are prominently displayed on epithelial cells, suggesting an important role in epithelial cell biology.
The TJ is a highly dynamic rate-limiting barrier for passive transepithelial solute flow. It is not only physiologically regulated but is modulated in various disease states as well. Such modulations occur as a result of epithelial cell interactions with immune cells or immune cell products and thus epithelial barrier function appears to be regulated in disease states.
A secreted product of activated neutrophils, NDS (neutrophil-derived secretagogue), elicits a short circuit current (Isc) in epithelial monolayers derived from the human intestinal cell line T84 (J. Clin. Invest. 1991. 87:1474-1477). Here, we identify and characterize the source of this Isc and examine associated signaling pathways. 125I efflux studies suggested that NDS activates an anion conductive channel. Bidirectional 22Na 36Cl flux studies showed that electrogenic Cl- secretion fully accounts for the NDS-induced Isc response. NDS behaved in many respects as a cAMP-mediated secretagogue: NDS did not further increase maximal cAMP-induced Cl- secretion; NDS potentiated Ca(2+)-mediated Cl secretion; and NDS elicited measurable 125I but not 86Rb effluxes. However, NDS did not elicit a detectable rise in intracellular cAMP. Such data suggest that NDS may elicit Cl- secretion by effecting distal events in the cAMP-mediated pathway. Data derived from cell volume assays of isolated guinea pig intestinal crypt cells indicated that NDS also directly elicits Cl- secretion from natural intestinal epithelia. Additionally, since NDS activity is released from PMN by stimuli normally present in the colonic lumen, since NDS is active when applied apically to this model intestinal epithelium, and since the NDS-elicited Isc response is indicative of electrogenic chloride secretion, we speculate NDS may contribute to the secretory diarrhea encountered in many patients with inflammatory intestinal disease.
The hematologic, biochemical, and light and scanning electron microscopic features of eperythrozoonosis in four llamas are described. One female and three male yearling llamas were presented for evaluation of chronic weight loss. Three of four llamas had historical evidence of chronic inflammatory conditions. On examination, multiple clinical problems were apparent, including poorly to non-regenerative anemia, inflammatory disease, and hypoproteinemia. Coccoid- and ring-shaped basophilic organisms were present on the erythrocytes of all the llamas. On scanning electron microscopy, individual, pairs, and clusters of coccoid-shaped organisms were present on the erythrocytes. The organisms measured 0.4 to 0.6 micron in diameter and caused no marked deformation of the erythrocyte membrane. A rare organism could be found that produced a slight indentation into the erythrocyte membrane. The light and scanning electron microscopic morphologic features suggested that the organism was an Eperythrozoon. Serial evaluation of serum iron concentrations of the llamas showed a decrease serum iron in all animals, with a concurrent decrease in the total iron binding capacity and percent transferrin saturation in two of the llamas. Common abnormalities seen on serum electrophoresis included a decrease in albumin and beta serum fraction in all llamas and a decrease in the gamma globulin fraction of two individuals.
Seventy patients presenting to the gastroenterologist with upper abdominal pain were examined by a psychiatrist to establish the presence of psychiatric disorder, illness behaviour and to record in detail their symptom pattern. The 37 patients who had no organic cause for their abdominal complaints were subdivided into those with and without psychiatric disorder. The former (21 patients) demonstrated more illness behaviour, they complained of more abdominal symptoms and their pain was both more severe and more persistent than in the patients with organic disease and those with non-organic illness who did not have psychiatric disorder. The latter group reported no symptoms of 'psychoneurosis' and should probably be regarded as a separate group if the aetiology of functional abdominal pain is to be clarified. Those with non-organic abdominal complaints who had psychiatric illness could be distinguished by the presence of three symptoms, namely depression, anxiety and fatigue. Detection and treatment of their psychiatric disorder might lead to a decrease in their symptomatic complaints and illness behaviour.
Left ventricular function and oesophageal function (including oesophageal manometry and pH monitoring) were investigated and a psychiatric assessment carried out in 63 patients with angina pectoris and normal coronary angiograms. Twenty two (35%) patients had regional abnormalities of left ventricular wall motion (group A). Thirty six (57%) patients had an oesophageal abnormality (group B); 19 patients had gastro-oesophageal reflux and abnormal oesophageal motility, five had gastro-oesophageal reflux alone, and 12 had abnormal oesophageal motility alone. Only four had regional abnormalities of the left ventricular wall and abnormal oesophageal function. In nine (14%) patients left ventricular and oesophageal function were normal (group C). Psychiatric morbidity was significantly less common in group A than in groups B and C and was similar in group B and group C. A definite abnormality of left ventricular function, oesophageal function, or psychiatric morbidity is present in a high proportion of patients with angina pectoris and normal coronary angiograms and in some instances this may lead to specific treatment. If quantitative assessment of left ventricular function is normal, oesophageal investigations should be performed. Endoscopy of the upper gastrointestinal tract may demonstrate oesophageal disease, but, if findings are normal, oesophageal manometry and ambulatory oesophageal pH monitoring (including during treadmill exercise testing) should be carried out.