炎症性肠病的肠外表现及其临床意义

目的分析并探讨炎症性肠病(IBD)患者肠外表现的特点和临床意义。方法收集、整理并记录251例IBD患者的临床资料,对比溃疡性结肠炎(UC)和克罗恩病(CD)患者出现肠外表现的情况。结果251例患者中,共61例(24.3%)有肠外表现。174例UC者中有肠外表现38例(21.8%),77例CD患者中有肠外表现23例(29.9%)(P>0.05)。UC患者左半结肠型肠外发生率与直肠型比较有显著性差异(P<0.05);CD患者回结肠型和回盲部小肠型分别与单纯结肠型比较,均无显著性差异(P>0.05)。两组患者肠外表现发生率依次为骨关节病变(8.0%、11.7%)、口腔溃疡(4.6%、9.1%)、肝胆(4.0%、6.5%)、皮肤(2.9%、2.6%)、眼部(2.2%、1.3%)、代谢性骨病(0.6%、0),但组间均无显著性差异(P>0.05)。结论UC和CD患者都是以骨关节病变、口腔溃疡、肝胆表现居多,但单纯依据肠外表现情况来区分UC和CD意义可能不大,肠外表现和IBD的炎症累及部位之间的关系还需进一步研究确定。     

Complex extra-intestinal complications of ulcerative colitis in a patient with alpha1-antitrypsin deficiency

Ulcerative colitis (UC) can manifest with a variety of extra-intestinal disorders frequently affecting the skin, joints, and liver. An aetiologic role of alpha1-antitrypsin deficiency in chronic inflammatory bowel disease has recently been suggested. We report a patient with UC and alpha1-antitrypsin deficiency who presented with disseminated cutaneous leucocytoclastic vasculitis clinically appearing with target-like purpuric patches and haemorrhagic oedemas. In addition, he displayed acute haemorrhage of the eyes and the respiratory tract consistent with a systemic vasculitic process. Moreover, he had autoimmune haemolytic anaemia. Systemic vasculitides, such as Wegener's granulomatosis, Churg-Strauss syndrome, and microscopic polyangiitis, could widely be excluded. Systemically administered glucocorticosteroids and azathioprine led to dramatic improvement of extra-intestinal symptoms. On the basis of alpha1-antitrypsin deficiency and UC, the present patient likely developed severe systemic vasculitis with multi-organ involvement. UC should at times be viewed within the context of a more generalized immune imbalance affecting multiple organs, and not as an isolated pathological entity. Testing for alpha1-antitrypsin deficiency in UC patients may detect individuals at higher risk of severe extra-intestinal involvement.     

Refractory and Active Ulcerative Colitis Complicated by Venous Thrombosis：a Case Report

ULCERATIVE colitis is a chronic inflammatorydisease characterized by diffuse mucosalinflammation involving the rectum and extendingproximally to involve all or part of the colon. Astep-up approach for medical therapy is routinely applied,from 5-aminosalicylic acid （ASA） compounds, corticosteroids,immunomodulators, and biologics.1 Thrombosis, mainlyvenous, is a rare and well-recognized extra-intestinalmanifestation of inflammatory bowel disease.2 In thisarticle, we report the use of thrombolytic and antithrombotictherapy for the venous thrombosis in a 55-year-old malepatient with refractory and active ulcerative colitis withexcellent outcome.     

Serological diagnosis of celiac disease

Celiac disease CD is an inflammatory disease of the small intestine brought about by exposure to gluten in genetically predisposed individuals. Celiac disease most often presents with non specific, or extra-intestinal, manifestations and, consequently, the disease remains under diagnosed. Untreated CD is associated with high morbidity and, therefore, early diagnosis is essential. The availability of non-invasive and relatively cheap serological tests has made it possible to screen large numbers of patients and resulted in increased, and earlier, diagnosis of patients with CD. However, these tests have varying degrees of sensitivities and specificities and the results generated can lead to a lot of confusion with regards to the diagnosis, or exclusion, of CD. In the present review, we discuss in detail these tests and suggest how they can be used in screening patients for CD with the hope that such information will help clinicians to select the right tests and interpret the generated results more effectively, and thus lead to improved identification and treatment of patients with CD.     

肠内肠外营养在重度颅脑损伤病人中的应用

目的 探讨重度颅脑损伤后早期不同营养途径的合理性及临床意义.方法 将GCS≤8分的重度颅脑损伤患者随机分为试验组(肠内、肠外序贯营养支持组)、对照组(单纯肠内营养组,EN),每组各24例.结果 两组病人营养治疗后血清白蛋白(ALB)、血红蛋白(HB)、总淋巴细胞计数(TLC)均明显升高,体重略下降.两组病人伤后21天时各项营养指标无明显差异(P＞0.05).结论 重度颅脑损伤后早期肠外、肠内序贯营养支持等同于传统匀浆膳加用乳清蛋白.     

Sensorineural deafness--a new complication of ulcerative colitis?

A case of sudden bilateral sensorineural deafness associated with active ulcerative colitis is reported. Evidence for an autoimmune basis for this condition is reviewed and the potential benefit of systemic corticosteroids emphasized. The condition may represent a recently recognized extra-intestinal complication of ulcerative colitis.     

Intestinal permeability and orocaecal transit time in elderly patients with Parkinson's disease.

The aetiology of weight loss in patients with Parkinson''s disease is likely to be multifactorial. We studied 15 patients with Parkinson''s disease and 15 age- and sex-matched controls and looked for evidence of malabsorption due to small bowel bacterial overgrowth or alteration of intestinal permeability. There was a marked increase in orocaecal transit time in the patients with Parkinson''s disease, although lactulose hydrogen breath testing did not show evidence of small bowel bacterial contamination. Intestinal permeability measured by the differential sugar absorption test was also deranged. There was reduced absorption of mannitol in patients with Parkinson''s disease while lactulose absorption was similar in both groups, suggesting decreased non-mediated uptake across the enterocyte brush border membrane in patients with Parkinson''s disease.     

Symptomatic cardiomyopathy as a presentation in Whipple's disease.

A patient presenting with congestive cardiac failure and anaemia underwent investigation which led to the diagnosis of Whipple''s disease, associated with dilated cardiomyopathy. Conventional antibiotic therapy for Whipple''s disease resulted in resolution of the traditional features of Whipple''s disease and a marked improvement in the patient''s heart failure.     

Study of familial Parkinson's disease in Russia, Uzbekistan, and Zambia

Objective: The aims of this study were (A) to determine inheritance patterns of familial Parkinson''s disease in three different geographical areas (Russia, Uzbekistan, and Zambia); (B) compare clinical characteristics of familial with sporadic Parkinson''s disease; and (C) assess whether there were ethnic differences in clinical manifestations of the disease. Methods: Fifty two index cases of familial Parkinson''s disease in Moscow, 55 in Tashkent, and 27 in Lusaka were selected on the basis of the typical clinical features of Parkinson''s disease with a familial history. The sex ratio, transmission patterns, and segregation ratio were determined by pedigree analysis. Results: Familial Parkinson''s disease was found in all three countries (30 families in Russia, 12 in Uzbekistan, and seven in Zambia), and appeared more common in Russia. Both autosomal dominant and autosomal recessive patterns of inheritance were seen, but autosomal dominance was more common in all countries. Conclusions: In all three countries men have a higher risk of developing Parkinson''s disease than women and there are ethnic differences in clinical manifestations of the disease. The onset of both familial and sporadic Parkinson''s disease in Zambian patients occurs at a younger age and is associated with slow progression and a benign course, and generally responds well to levodopa treatment.     

Hepatic sinusoidal ectasia in association with Hodgkin's disease

A patient developed abnormal liver function tests in association with hepatic sinusoidal ectasia concurrently with relapse of Hodgkin''s disease. Tests returned to normal following successful chemotherapy for the Hodgkin''s disease. Sinusoidal ectasia and the related condition, peliosis hepatis, should be recognized as rare non-infiltrative manifestations of Hodgkin''s disease.     

Herbal Medicine in the Treatment of Alzheimer’s Disease

Associated with the aging of our world population is a sharp increase in the incidence of Alzheimer’s disease,which not only poses a significant health issue but also presents a serious social problem.Although pharmacological treatments were developed based on existing hypotheses,the disease pathogenesis remains to be fully elucidated.Given the complexity of Alzheimer’s disease,Chinese herbal medicine appears to have therapeutic potential for Alzheimer’s disease through multi-target and multi-pathway approach at cellular and molecular levels and holistic adjustment of the body at organ system levels.Recently,a significant breakthrough has been made in the research of Chinese medicine for Alzheimer’s disease.In this article,we review the experimental research progress in understanding how Chinese medicine could be used for the treatment of Alzheimer’s disease.     

克罗恩病的诊疗现状及临床、病理特点

目的:评价克罗恩病的诊疗现状,总结其临床、病理特点及治疗转归.方法:回顾性分析220例炎症性肠病住院患者中48例克罗恩病的病历资料,详细记录临床表现、实验室检查、X线和内镜、病理及治疗情况.结果:1987年至1995年共诊断克罗恩病4例,1995年后诊断44例.克罗恩病高峰发病年龄为17～40岁,占75.0%(36/48);30岁以下发病者占43.8%(21/48).临床主要表现为腹痛、腹泻和便血;伴肠道外表现16例(33.3%),伴有肛周疾病3例(6.3%),肠瘘2例(4.2%).内镜诊断符合率85.7%(36/42),表现为节段性病变,溃疡形成、肠管狭窄和卵石征.X线诊断符合率为84.2%(32/38),主要表现为龛影、肠管狭窄及卵石征.病理非干酪性上皮样肉芽肿总检出率为43.2%(19/44).90%(27/30)经药物治疗症状改善,有13例行手术治疗.结论:近10年来克罗恩病诊断例数明显增加.内镜+病理+X线造影检查是诊断克罗恩病的主要手段.小肠镜、胶囊内镜及多次病理检查、随访有助于提高诊断率.     

Medical disorders in Alzheimer's disease and vascular dementia.

The clinical and postmortem findings of 29 patients with Alzheimer''s disease were evaluated and compared to the findings of 19 patients with vascular dementia. The patients with Alzheimer''s disease had received treatment for an average of 2.0 internal medical disorders, the patients with vascular dementia for 2.1 disorders. The average number of medical diseases found at postmortem was 3.7 in the group with Alzheimer''s dementia and 4.1 in vascular dementia. Apart from a marginally increased rate of peripheral occlusive artery disease in vascular dementia, the proportions of the most common disorders of the cardiovascular, genitourinary, gastrointestinal and endocrinological disorders and the most common causes of death did not differ significantly between these groups. Therefore patients with Alzheimer''s disease should receive the same internal medical attention as patients with vascular dementia.     

Parkinson's disease.

Parkinson''s disease is a common disabling disease of old age. The diagnosis of idiopathic Parkinson''s disease is based on clinical signs and has poor sensitivity, with about 25% of patients confidently diagnosed as having the disease actually having other conditions such as multi-system atrophy and other parkinsonism-plus syndromes. Benign essential tremor and arteriosclerotic pseudo-parkinsonism can easily be confused with Parkinson''s disease. The cause of Parkinson''s disease remains unknown. Speculative research highlights the role of oxidative stress and free radical mediated damage to dopaminergic cells. Parkinson''s disease is the one neurodegenerative disorder in which drugs have been demonstrated to be of value. There is now a wide variety of drugs and formulations available, including anticholinergics, amantidine, L-dopa, dopamine agonists including apomorphine, selegiline and soon to be available catechol-O-methyltransferase inhibitors. Disabling side-effects of treatment, fluctuations, dyskinesias and psychiatric problems require strategic use of the drugs available. There is an increasing potential for neurosurgical intervention.     

Scurvy and vitamin C deficiency in Crohn's disease.

A case of scurvy presenting in a patient with Crohn''s disease is reported. A normal response to replacement therapy is seen. Vitamin C (ascorbic acid) deficiency was found in 7 out of 10 patients with clinically quiescent Crohn''s disease, 4 of whom had an adequate oral intake of vitamin C. There was no significant difference in oral intake between patients with Crohn''s disease and matched controls but there was a significant difference (P less than 0.001) in leucocyte ascorbic acid levels. It is recommended that patients with Crohn''s disease be screened for vitamin C deficiency and receive prophylactic vitamin C supplements daily.     

帕金森病的异常姿势研究进展

帕金森病患者常出现姿势异常,这些异常姿势限制了患者的日常生活能力。本文将对帕金森病常见的异常姿势及其治疗研究进展进行综述。     

GRAVES' DISEASE WITH ORGANIC MOOD SYNDROME (A Case Report)

A case of Graves'' disease with organic mood syndrome in a 3G year old man is reported. Patient had thyrotoxicosis and developed features of mania while in the hospital which necessitated antipsychotic drug therapy.KEY WORDS: Graves'' disease, ManiaFrank psychotic decompensation occurring in the background of Graves'' disease is an explosive clinical situation as manifestations range from severe manic excitement to total apathy. Although rare, the gravity of such situation warrants energetic intervention on both fronts. One such instance of organic mood syndrome with Graves'' disease is being presented, highlighting the problems encountered in the management.     

Occult small bowel adenocarcinoma complicating Crohn's disease: a report of three cases.

Three patients with Crohn''s disease are described who were treated by ileal resection for intestinal obstruction. Histological examination of the resected specimen in each case established the diagnosis of adenocarcinoma of the small intestine complicating Crohn''s disease. This diagnosis should be considered in patients with longstanding macroscopic Crohn''s disease who present with severe or recurrent symptoms. The diagnosis may not be apparent on routine radiological examination or even macroscopically at laparotomy.     

Perthes' disease and multiple epiphyseal dysplasia.

Five atypical cases were observed amongst ninety children with Perthes'' disease, ten of whom had bilateral hip joint involvement. All five were boys, four being under 4 years of age. Four had bilateral hip joint disease, four presented with hip pain, three showing some degree of retardation of bone growth. In one case the hip disorder was familial, and in four there were bony abnormalities elsewhere. Despite the absence of the classic signs of multiple epiphyseal dysplasia, a mild form of this condition is a possible alternative diagnosis for these children. Racial and familial differences are known in the prevalence of Perthes'' disease which itself may represent a dysplasia. The pathogenesis of Perthes'' disease is still uncertain, although some abnormality of the blood supply to the proximal femoral epiphysis is postulated. That such a vascular defect may be engrafted on to multiple epiphyseal dysplasia is possible, with subsequent joint degeneration which may come to resemble Perthes'' disease either clinically or radiologically. A plea is made for the closer study of young children presenting with what may seem to be atypical Perthes'' disease.     

张伯礼教授治疗克罗恩病验案一例

克罗恩病是一种以慢性肠道炎症为主要表现的全身性疾病,具有难治愈,易复发的特点,目前尚缺乏根治的方法。中医具有辨证论治特色,且不良反应较小,治疗克罗恩病具有控制病情进展,改善症状的优势。张伯礼教授临证具有较丰富的临床经验,现将张教授治疗克罗恩病验案一则进行整理报告,希冀体悟张教授临床辨证思维及遣方用药经验。