Multi-disciplinary clinical protocol for the diagnosis of bulbar amyotrophic lateral sclerosis

Introduction and objectives

The objective of this study was to examine the role of different specialists in the diagnosis of amyotrophic lateral sclerosis (ALS), to understand changes in verbal expression and phonation, respiratory dynamics and swallowing that occurred rapidly over a short period of time.

Diagnostic accuracy and utility of fine-needle aspiration cytology in therapeutic management of parotid gland tumours

Introduction and objectives

The utility of fine-needle aspiration cytology (FNAC) in parotid tumours remains widely debated. This study aims to evaluate the accuracy of FNAC in diagnosing parotid tumours.

特发性慢性荨麻疹患者血清特异性IgE检测

[目的 ]观察IgE过敏原体外检测试剂盒在特发性慢性荨麻疹患者血清特异性IgE水平检测中的应用 .[方法 ]对 60例特发性慢性荨麻疹患者的血清进行特异性IgE检测 .[结果 ]吸入性过敏原阳性率为 67% ,最常见的吸入性过敏原为点青霉、链孢霉、黑根霉、榆树、柳树及杨树 .食入性过敏原阳性率为 62 % ,最常见的食入性过敏原为鱼、牛奶、蟹、虾、猪肉、牛肉及羊肉 .吸入组 44% (8/ 18)和食入组 17% (10 / 60 )对 2种以上过敏原过敏 .[结论 ]IgE过敏原体外检测试剂盒对特发性慢性荨麻疹等过敏性疾病的病因诊断有参考意义     

Blalock–Taussig Shunt for a Neonatal Case of Ebstein’s Anomaly with Unobstructed Right Ventricular Outflow Tract

A neonate with Ebsteins anomaly presented with severe cyanosis because of massive right-to-left shunt through an atrial septal defect and reduced blood flow through an unobstructed right ventricular outflow tract. This atypical patient underwent a right modified Blalock–Taussig shunt, which resolved the respiratory .     

Titel

Objective: Studies aiming to define key cytokines in inflammatory bowel disease have been restricted to gene expression or protein quantitation but lack functional information on cytokine interactions. Some of the major cytokines that govern the extent and duration of the inflammatory process in ulcerative colitis (UC), appear to be interleukin 1 (IL-1), its natural inhibitor IL-1 receptor antagonist (IL-1ra) and transforming growth factor 1 (TGF-1). Indeed, as a predictor of inflammation, the mucosal status of IL-1, depicted as a ratio of IL-1ra/IL-1, has often been used.Methods: Using an IL-1 bioassay and specific anti-cytokine antibodies we have identified the functional role of these cytokines and their interactions in mucosal biopsy samples taken from patients with UC.Results: Compared with control specimens, the secreted and tissue levels of IL-1 were consistently raised in UC samples. Levels of IL-1, rather than IL-1ra or the ratio of IL-1ra/IL-1, most closely mirrored the severity of inflammation. Using specific antibodies we showed that IL-1ra and TGF-1 appear to modulate the degree of inflammation at different stages of the inflammatory process. Only in severely inflamed tissue, when IL-1 levels were high did IL-1ra inhibit IL-1-induced activity. In contrast, the levels of TGF-1, and its effect in controlling inflammation, was most marked in mild but not severe UC.Conclusions: The functional roles of these cytokines in the inflammatory process can now be more carefully elucidated using a bioassay and specific neutralising antibodies.Received 11 March 2003; returned for revision 20 May 2003; accepted by N. Boughton-Smith 12 September 2003     

Low levels of antibodies against <Emphasis Type="Italic">E. coli</Emphasis> and mycobacterial 65kDa heat shock proteins in patients with inflammatory bowel disease

Objective and design: The aim of the present study was to support and extend our initial observation, where we found low levels of antibodies against mycobacterial 65kD heat shock proteins in patients with inflammatory bowel disease (IBD). For this purpose we tested a new group of 124 patients with IBD, and beside measuring antibodies to Mycobacterium bovis 65kD heat shock protein (Hsp65) and human 60kD heat shock protein (Hsp60) as described previously, we also determined IgG antibody levels to Hsp65 from E. coli, called GroEL.Patients and control subjects: seventy-four patients with Crohns disease (CD) (30 males, 44 females, 33 (27–45) years old, median (interquartile range)) and 50 patients with ulcerative colitis (UC) (22 males, 28 females, 38 (30–50) years old) were involved in the study. 110 healthy subjects (34 males, 76 females, 47 (37–53) years old) served as controls. Study subjects were consecutive patients referred to an IBD center for complex treatment of the disease.Methods and statistical analysis: The amounts of IgG-type antibodies reacting with proteins of the chaperonin 60 family were assessed by ELISA. Since the antibody levels to heat-shock proteins as variables were not normally distributed, non-parametric Mann-Whitney test and Dunn post hoc test were used for group comparisons.Results: Median levels of anti-GroEL (7,5 (3,5–18,3)) and anti-Hsp65 (4,8 (2,1–7,85)) were significantly (GroEL p = 0,008; and Hsp65 p < 0,001) lower in the IBD patients than in the healthy subjects (GroEL: 10,0 (5,4–31,0); Hsp65: 7,04 (4,66–12,77)). However this difference was found to be restricted to the CD patients (GroEL: 7,5 (3,7–14,2); p < 0,05; Hsp65: 4,35 (1,90–6,94); p < 0,001). We did not find difference in the concentration of anti-human Hsp60 IgG levels between patients (Hsp60: 45,5 (24,9–69,0)) and healthy controls (38,4 (21,6–69,4). Regarding the serum concentrations of each antibody tested there was no significant difference between the active and inactive stage of disease.Conclusion: Our present findings support conclusion of our previous work, antibody levels not only for Mycobacterium bovis hsp65 but for E. coli GroEl were found to be decreased as well. In contrast no changes in the concentrations of human anti-hsp60 antibodies were observed. These findings indicate that production of antibodies to 65 kDa bacterial heat shock proteins is selectively impaired in IBD.Received 9 March 2004; returned for revision 9 April 2004; accepted by A. Falus 18 May 2004     

Plasma adiponectin response to acute exercise in healthy subjects

Adipose tissue secretes adiponectin, an adipocytokine that is involved in the regulation of insulin sensitivity. Following acute exercise, insulin sensitivity has been shown to increase. Increased adiponectin following exercise may be related to the change in insulin sensitivity. The purpose of the present study was to characterize the effect of a single cycle exercise session on adiponectin and to compare the exercise effects between healthy male and female subjects. Plasma concentrations of adiponectin, tissue necrosis factor-alpha (TNF-), insulin, glucose, and leptin were assessed before and immediately after a 60-minute stationary cycle ergometry session at 65% of O_2max. Male and female subjects were matched for cardiorespiratory fitness and body composition and dietary intake was controlled for the three days prior to the exercise trial. At rest, adiponectin concentration was not associated with percentage body fat, body mass index (BMI), fitness, or resting plasma variables (P>0.05). Following exercise, neither male nor female subjects exhibited changes in adiponectin or leptin concentrations (P>0.05). TNF- exhibited a time main effect increase with exercise (P<0.05), but there were no gender differences. These results suggest that plasma adiponectin concentrations do not change with exercise in healthy male or female subjects. Results are given as mean (SE).     

Familial frontotemporal dementia: a report of three cases of severe cerebral atrophy with rare inclusions that are negative for tau and synuclein,but positive for ubiquitin

We describe the brain autopsy findings from three of five siblings who suffered dementia with clinical diagnoses including Alzheimers, Parkinsons, and Picks disease. Five other living siblings appear unaffected. All of the autopsied brains showed severe atrophy (brain weights 613, 641, and 750 g) of the frontal and temporal lobes, and to a slightly lesser extent of the parietal lobes, while the occipital lobes were relatively preserved. The substantia nigra showed marked neuronal loss with gliosis. No ballooned neurons, neurofibrillary tangles, neuritic plaques, Pick bodies, or Lewy bodies were found in these brains. Immunohistochemistry for tau protein failed to reveal neuronal or glial inclusions, and normal tau protein was found in a separate Western blot study [Adamec et al. (2001) Neurosci Lett 315:21–24]. Rare neurons with ubiquitinated cytoplasmic inclusions were scattered in the neocortex and hippocampus. The overall pathological features were consistent with a severe form of frontotemporal dementia (FTD) with involvement of the substantia nigra. Whether the rare ubiquitinated inclusions are sufficient to classify these cases as FTD with motor neuron disease type inclusions but without motor neuron disease, or FTD dementia lacking distinctive histological features remains unclear. The features of lobar circumscribed atrophy without Pick bodies and without ballooned neurons, however, are consistent with Pick disease group C in the Constantinidis classification [Constantinidis et al. (1974) Eur Neurol 11:208–217].     

卡培他滨应用于鼻咽癌同期放化疗的I期临床试验

背景与目的:卡培他滨(capecitabine)是一种口服的氟尿嘧啶类药物。在肿瘤组织中,由特异高表达的胸腺嘧啶磷酸化酶(thymidinephosphorylase,TP酶)转变为5-氟尿嘧啶(5-fluorouracil,5-FU),因此在肿瘤细胞内5-FU浓度较高。本试验探讨卡培他滨在鼻咽癌同期放化疗的最大耐受剂量(maximumtolerateddose,MTD)。方法:对36例病理诊断为鼻咽癌的放疗患者同时给予不同剂量单药卡培他滨化疗,观察不良反应并随访治疗效果。卡培他滨采用间断服用方法,放疗后开始口服,每天2次,服药14天,然后休息7天,再开始第二疗程化疗,一共2疗程。中位剂量为1600mg·(m2·d)-1,剂量为700～2500mg·(m2·d)-1。放疗剂量66～74Gy(6～7.4周)。结果:2例Ⅲ级剂量限制性毒性(dose-limitingtoxicity,DLT)发生在2300mg·(m2·d)-1剂量组,1例为Ⅲ级腹泻,1例为Ⅲ级手足综合症(hand-footsyndrome,HFS)。结论:鼻咽癌患者对卡培他滨单药化疗联合同期放疗耐受良好,最大耐受剂量为2300mg·(m2·d)-1。我们推荐卡培他滨Ⅱ期临床试验的剂量为1700mg·(m2·d)-1(间断服用方法)。