Tadashi Matsumura

Cronkhite–Canada syndrome showing elevated levels of antinuclear and anticentromere antibody

A 56-year-old female initially visited an otorhinolaryngologist because of an impaired sense of taste in September, 2010 and was referred to our facility in October, 2010. She was diagnosed with Basedow’s disease for which she underwent subtotal thyroidectomy in 1984 and arthritis involving multiple joints, primarily affecting her hands. In addition, the anticentromere antibody (ACA) level was markedly high. On physical examination, alopecia as well as hyperpigmentation of the dorsum of the hands and back was observed. Dystrophic changes of the fingernails and a bilateral thumb abduction deformity were observed. Antinuclear antibodies were elevated. Gastrointestinal endoscopy and colonoscopy revealed the mucosa carpeted with strawberry-like polypoid lesions. Histopathological examination of the biopsied specimen of the stomach revealed a corkscrew-like appearance. Thus, the patient was diagnosed with Cronkhite–Canada syndrome (CCS). She admitted to our hospital in November, 2010. Oral prednisolone was administered with success. In July, 2012, her antimitochondrial M2 antibody level was elevated. To the best of our knowledge, the present case is the first patient with CCS, a history of Basedow’s disease, and elevated levels of ACA and antimitochondrial M2 antibody. We consider the present case suggests CCS could be caused by immunological abnormality.

development And Evaluation Of A Quantitative Lateral Flow Immunoassay For The Detection Of Ferritin As Point-of-care Testing

Introduction: The liver is central to the metabolism of both iron and cholesterol. Cholesterol is synthesised and further metabolised to bile acids in the liver and the liver plays an important role in regulation of iron metabolism. It is also the organ in which excess iron is stored. Clinically, links have been noted between lipid and iron metabolism, with approximately one - third of patients with non - alcoholic fatty liver disease exhibiting altered iron parameters. On a molecular level, we have previously reported that wild - type mice fed iron - deficient, normal or iron - loaded diets exhibited increased hepatic cholesterol and increased hepatic gene expression of enzymes in the cholesterol biosynthesis pathway with increasing hepatic iron burden. In the genetic disorder, haemochromatosis, the liver can become overloaded with iron; however, clinical studies have suggested that lipid metabolism may not be perturbed in haemochromatosis. n nMethods and Materials: We investigated hepatic cholesterol metabolis m in three mouse models of hereditary haemochromatosis: Hfe - / - , Tfr2 Y245X single mutant and Hfe - / - x Tfr2 Y245X double mutant animals as well as wild - type controls. Mice were fed normal mouse chow and sacrificed at 10 weeks of age. Hepatic gene expression, total cholesterol and non – haem iron were measured. Liver non - haem iron was similar in Hfe - / - and Tfr2 Y245X mice (16.6±0.8 and 17±1 μmol Fe /g liver, respectively) and significantly higher in the double mutant animals (22.4±0.7 μmol Fe /g liver ; P<0.004) than either of the single mutant mice. n nResults: Only one group of genes increased significantly with increasing hepatic iron: those involved in cholesterol transport. Gene expression of apolipoproteins A4, C1, C2, C3 and E increased significantly with increasing hepatic iron as did expression of VLDL receptor. In contrast to our findings in wild - type mice, gene expression of cholesterol biosynthetic enzymes did not increase significantly with liver iron burden and there were no differences in hepatic cholesterol between the groups of mutant mice. We also measured expression of genes involved in cholesterol regulation, which similarly, did not increase with increasing hepatic iron. Approximately 50% of cholesterol synthesised in the liver is directed to bile acid synthesis; however, gene expression of bile acid pathway enzymes did not change with respect to hepatic iron burden. Conclusion: These results suggest that iron - associated cholesterol regulation may be ameliorated by the genetic changes which occur in haemochromatosis.Poster presented at Fifth Congress of the International BioIron Society that took place in University College London (London, United Kingdom) during 14-18th April 2013.

A Case of Systemic Lupus Erythematosus Presenting with an Acute Abdomen: Successful Treatment with Steroid

Abdominal pain continues to pose diagnostic challenges for emergency clinicians. A 56-year-old Japanese woman was referred to our hospital due to severe abdominal pain which presented as occasional epigastric pain five months before and intermittent abdominal pain. She had a past history of ileus twice, for both of which laparotomy was performed without an alimentary tract resection. The wall thickening with marked three-wall structure from terminal ileum to sigmoid colon was seen and bladder wall was irregularly thick and enhanced irregularly. Among the differential diagnosis of the acute abdomen, autoimmune diseases were suspected, especially lupus erythematosus and Henoch-Schönlein purpura. On the second day of admission, abdominal pain worsened. The results of examinations of antinuclear antibody, anti-double-stranded DNA antibody, ANCA, and the complements were not obtained at that time; however, we started 1-g steroid pulse treatment for three days with success. With the results obtained later, the patient was given a diagnosis of probable systemic lupus erythematosus (SLE). The present case shows that SLE can present with acute abdomen and should be included in the wide range of the differential diagnosis of acute abdomen.