Myelodysplastic syndrome associated with relapsing polychondritis: Unusual transformation from refractory anemia to chronic myelomonocytic leukemia

Summary The authors report an unusual case of myelodysplastic syndrome (MDS) associated with relapsing polychondritis (RP), which developed at almost the same time as MDS. The initial diagnosis was MDS, refractory anemia (RA) subtype, according to the FAB classification [3]. Symptoms of RP were apparently controlled by oral administration of prednisolone (PSL), although MDS was not. Within 1 month after the diagnosis, monocytosis and thrombocytopenia without excess of blasts became prominent and transformation from RA to chronic myelomonocytic leukemia (CMML) was recognized. Combination chemotherapy including daunorubicin (DNR) and cytosine arabinoside (ara-c) did not subdue the progressive monocytosis and thrombocytopenia. Finally, the patient died of pulmonary hemorrhage 3 months after the onset of the disease. The prognosis of MDS may be poorly influenced by association with RP.     

Esophageal function worsens with long duration of diabetes

BACKGROUND: The aim of this study was to assess the relationship between the duration of diabetes and esophageal dysfunction. METHODS: We examined 66 patients with type 2 diabetes. Duration of diabetes was determined by asking patients and from their medical records. The patients were divided into three groups according to the duration of their diabetes: group A, 1-4 years, n=26; group B, 5-9 years, n=20; and group C, 10+ years, n=20. Ambulatory esophageal 24-h pH and motility were monitored, and gastroesophageal reflux and esophageal motility disorders were estimated in detail. RESULTS: When the duration of diabetes was long, the percentage of time with pH<4 tended to increase. The amplitude of esophageal peristaltic waves and the frequency of effective peristalsis were reduced when the duration of diabetes was long. A significant correlation was observed between the duration of diabetes and the frequency of effective peristalsis. The number of esophageal peristaltic waves per minute and the percentage of multipeaked peristaltic waves increased significantly in group B, and decreased when the duration of diabetes became longer. CONCLUSIONS: Gastroesophageal reflux and esophageal motility disorders worsened with long duration of diabetes. These esophageal dysfunctions should be considered in patients with long-standing diabetes.     

亚太地区炎症性肠病处理共识意见(一)

虽然目前亚太地区尚无炎症性肠病（IBD）的大规模流行病学资料，但一系列研究显示其发病率和患病率呈上升趋势。与西方国家相比仍呈滞后现象。溃疡性结肠炎（UC）的发病率仍较克罗恩病（CD）高。除地域差异外。在一些多民族国家中，IBD尚可见种族差异。亚太地区IBD的遗传背景有异于西方国家。如据报道该地区CD患者未检出NOD2／CARDI5变异。一般而言，该地区IBD患者的临床过程似不如西方国家严重。 亚太地区IBD的诊断存在一些特殊问题。如缺乏IBD诊断金标准。存在多种小肠结肠炎，与IBD临床表现相似，使鉴别诊断特别困难。迄今为止，亚太地区IBD的诊断标准多采用西方国家的诊断标准。诊断必须逐步排除非IBD的小肠结肠炎。确诊应有典型的组织学表现。某些患者需借助随访和诊断性治疗才能确诊。进一步研究IBD发病机制将有助于开发更好的诊断标记物。 亚太地区IBD的治疗亦存在特殊问题。由于诊断困难。IBD患者常未能及时接受适当的药物治疗，但该地区仍广泛采用药物治疗方案。结合西方指南和本地经验可制定类似的处理原则。以利诱导缓解和维持缓解。提倡逐级使用基于病变范围、活动性和严重度的阶梯式治疗方案。对不同病例采用综合性、个体化的方法。随着对IBD发病机制和亚太地区IBD独特性的深入理解。合理、实用的药物治疗指南和应用生物制剂治疗将改善该地区IBD的治疗前景。     

Significance of the time course of ST segment elevation just after reperfusion therapy for acute myocardial infarction

The significance of the time course of ST segment elevation just after reperfusion therapy for acute myocardial infarction was investigated in 25 consecutive patients with acute myocardial infarction and ST elevation. The most elevated ST lead from the standard electrocardiogram on admission was continuously monitored as the ST trend for 72 hr including during the reperfusion procedure. The culprit artery was totally occluded and reperfused without flow delay. Left ventriculograms were obtained after reperfusion and 3-4 weeks later. The most elevated ST level before reperfusion was measured as the maximal level and the ST level 30 min after reperfusion as the reperfused level. The patients were divided into 2 groups, group ST > or = 50% (n = 12) with a decrease of less than 50% of the maximal ST level and group ST < 50% (n = 13) with a decrease of 50% or over 50%. Regional left ventricular wall motion of infarct site, end-diastolic left ventricular volume and ejection fraction were compared between the acute and chronic phase left ventriculograms in each group. In group ST > or = 50%, no significant change was detected in both regional and global ejection fraction, whereas end-diastolic left ventricular volume enlarged significantly in the chronic phase (acute 80 +/- 25 ml/m2 to chronic 95 +/- 17 ml/m2, p < 0.01). In group ST < 50%, regional and global ejection fraction both improved significantly (SD/chord: acute -2.2 +/- 1.5 to chronic -1.4 +/- 0.4, p < 0.001; ejection fraction: acute 50 +/- 14% to chronic 57 +/- 13%, p < 0.01) but end-diastolic left ventricular volume remained the same in the chronic phase. The peak creatine kinase level and the frequency of ST re-elevation at reperfusion were significantly higher in group ST > or = 50% than in group ST < 50% (creatine kinase: group ST > or = 50% 5,496 +/- 2,219 IU/l, group ST < 50% 1,913 +/- 1,180 IU/l, p < 0.0001; ST re-elevation: group ST > or = 50% 83%, group ST < 50% 38%, p < 0.05), although the time from onset of myocardial infarction to reperfusion, the maximal ST level, the degree of collateral development, and the frequency of pre-infarction angina were not different between the 2 groups. Rapid resolution of ST elevation after reperfusion is a useful marker of the improvement of left ventricular function in acute myocardial infarction.     

Mass spectrometric identification of citrullination sites and immunohistochemical detection of citrullinated glial fibrillary acidic protein in Alzheimer's disease brains

Peptidylarginine deiminases (PADs) are posttranslational modification enzymes that convert protein arginine to citrulline residues in a calcium ion‐dependent manner. Previously, we reported the abnormal accumulation of citrullinated proteins and the increase in the amount of PAD2 in hippocampi from Alzheimer's disease (AD) patients. Moreover, glial fibrillary acidic protein (GFAP), an astrocyte‐specific marker protein, and vimentin were identified as citrullinated proteins by using two‐dimensional gel electrophoresis and MALDI‐TOF mass spectrometry. To clarify the substrate specificity of PADs against GFAP, we prepared recombinant human (rh)PAD1, rhPAD2, rhPAD3, rhPAD4, and rhGFAP. After incubation of rhGFAP with rhPAD1, rhPAD2, rhPAD3, and rhPAD4, citrullinated (cit‐)rhGFAP was detected by Western blotting. The citrullination of rhGFAP by rhPAD2 was unique, specific, and time dependent; additionally, rhPAD1 slightly citrullinated rhGFAP. We then generated eight anti‐cit‐rhGFAP monoclonal antibodies, CTGF‐125, ?128, ?129, ?1212, ?1213, ?1221, ?122R, and ?1224R, which reacted specifically with cit‐rhGFAP. Two of those eight monoclonal antibodies, CTGF‐122R and ?1224R, reacted with both cit‐rhGFAP and rhGFAP in Western blots. By using the CTGF‐1221 antibody and a tandem mass spectrometer, we identified the two independent citrullination sites (R270Cit and R416Cit) of cit‐rhGFAP. Immunohistochemical analysis with CTGF‐1221 antibody revealed cit‐GFAP staining in the hippocampus of AD brain, and the cit‐GFAP‐positive cells appeared to be astrocyte‐like cells. These collective results strongly suggest that PAD2 is responsible for the citrullination of GFAP in the progression of AD and that the monoclonal antibody CTGF‐1221, reacting with cit‐GFAP at R270Cit and R416Cit, is useful for immunohistochemical investigation of AD brains. © 2015 Wiley Periodicals, Inc.