成人自身免疫性肠病三例及文献复习

目的明确成人自身免疫性肠病（AIE）临床诊断标准，探讨相关治疗方法。方法对我院过去3年中3例持续性严重腹泻且对禁食无效患者的病史进行回顾性分析。结果3名患者符合AIE描述的典型腹部症状并伴有严重低白蛋白血症，并排除其他自身免疫性疾病。病变部位限于十二指肠至回肠，病理检查示肠黏膜重姨慢性活动性炎症，绒毛结构消失。1例尝试性使用大剂量皮质激素后症状迅速改善，另2例在激素减量后出现腹泻症状反复，予甲氨蝶呤后好转。结论据此认为3名患者罹患罕见的成人自身免疫性肠病，建议有上述病症且禁食无效的患者应行肠黏膜活检，检测上皮细胞自身抗体及进行胶囊胃镜检查。     

Wilson's disease with superimposed autoimmune features: report of two cases and review

We describe two females, 15 and 23 years old, respectively, who presented with classical features of Wilson's disease (WD) and several features of autoimmune hepatitis (AIH). The first patient was initially diagnosed as AIH and treated with prednisolone which caused clinical improvement, with an increase of serum albumin from 22 to 30 g/L, and a decrease of aspartate aminotransferase from 103 to 47 U/L. Subsequent diagnosis of WD and introduction of penicillamine gave excellent improvement and complete normalization of liver function tests. The second patient, at first also diagnosed as having AIH, was treated with steroids and azathioprine with initial improvement, but subsequent deterioration. The diagnosis of WD was made 2 years after initial diagnosis of AIH, as the patient reached end-stage liver disease and required a transplant. Therefore, d-penicillamine treatment was not attempted. We conclude that, in patients with AIH, a thorough screening for WD is necessary, particularly when the response to steroid therapy is poor. Conversely, in patients suffering from WD with superimposed features of AIH, a combination of steroids and penicillamine may be of benefit.     

Autoimmune pancreatitis:report of two cases and literature review

BACKGROUND:Autoimmune pancreatitis(AIP)is a unique chronic inflammation of the pancreas in which autoimmune mechanisms are involved in the pathogenesis.It is characterized by clinical,histopathological,radiographic,serologic and therapeutic features.Since it was first described in 1995,increasing numbers of AIP patients have been diagnosed.METHODS:In this study,the data from 2 patients with AIP who had elevation of serum immunoglobulin G,positive autoantibody,swelling of the pancreas and narrowing of the ma...     

Paraquat poisoning: report of two cases and literature review

Cases of poisoning with pesticides, especially suicidal ones, continue to be an important therapeutic problem. The heribicide paraquat (1.1' dimethyl-4.4' bipyridylium dichloride) is the second cause of pesticide poisoning in our country, which is associated with a high mortality rate. We report two cases of suicidal ingestion of paraquat who developed multiorgan failure with a lethal outcome. We also present a brief review of the literature, mainly focused on the different therapeutic options.     

Bladder leiomyoma: report of two cases and literature review

Mesenchymal tumors of the urinary bladder are rare and the majority of them are malignant. We report two cases of leiomyoma of the urinary bladder presenting with symptoms of bladder outlet obstruction. The first patient was managed with staged transurethral resections, and the second with holmium laser transurethral inoculation. The outcome was excellent in both cases. A review of the literature is also outlined.     

Retrocaval ureter: report of two cases and literature review

Retrocaval ureter is a rare congenital anomaly in which the ureter passes behind, and is compressed by, the inferior vena cava. Its etiology is assumed to be abnormal embryologic development of the inferior vena cava as a result of atrophy failure of the right subcardinal vein in the lumbar portion. We report two cases of retrocaval ureter and review the relevant literature. One patient was a 7-year-old boy who presented with right flank pain. The other was a 40-year-old male who was found to have right hydronephrosis accidentally on abdominal sonography during a health examination. Both underwent retrograde pyelography which showed the typical S-shape of a retrocaval ureter. Abdominal computerized tomography and magnetic resonance imaging confirmed the diagnosis of retrocaval ureter. Ureteroureterostomies were performed. One patient showed focal squamous metaplasia of the ureter and the other had chronic inflammation and fibrosis. Follow-up studies showed improvement in hydronephrosis and renal function in both patients. We conclude that retrocaval ureter is a rare disorder and surgical correction is usually effective.     

Primary cardiac echinococcosis: report of two cases with review of the literature

Two case reports of Saudi patients with primary cardiac hydatid cysts are presented. In the first case, a multilocular cyst was located in the wall of the left ventricle, whereas in the second case a cyst was located in the pericardial sac and another cyst in the left ventricular wall. The diagnosis was based on a history of animal contact, full clinical examination, serological tests and the use of plain radiography, including conventional tomogram, two-dimensional echocardiography, computed tomography and thallium perfusion isotope scan. This study has indicated that non-invasive radiological methods are sufficient to diagnose cardiac echinococcosis and could provide the same information as, or even more than if invasive techniques were used. Further screening of the two patients showed no involvement of other organs by hydatid cysts.     

Subcutaneous sarcoidosis: report of two cases and review of the literature

Sarcoidosis is a multisystemic disease with cutaneous lesions present in about one fourth of patients. Cutaneous lesions may be specific or nonspecific based on the presence or the absence of sarcoidal granulomas. Subcutaneos sarcoidosis is the less frequent of the specific cutaneous lesions of sarcoidosis. We report here 2 new cases and review 83 cases reported in literature of subcutaneous sarcoidosis. Subcutaneous sarcoidosis present usually with asymptomatic firm nodules covered by normal-appearing skin, mostly on the forearms and legs. Diagnosis may require a high index of suspicion. In the vast majority of patients, subcutaneous nodules were the manifestation that allowed the diagnosis of systemic sarcoidosis. There is a strong association between subcutaneous sarcoidosis and bilateral hilar lymphadenopathy (72.7%). About 15% of patients have in order of frequency uveitis, parotitis, arthritis, mucositis, dactylitis, neurological and renal involvement, hepatosplenomegaly.     

Gallstone ileus： Report of two cases and review of the literature

Gallstone ileus is a rare disease and accounts for 1%-4% of all cases of mechanical intestinal obstruction. It usually occurs in the elderly with a female predominance and may result in a high mortality rate. Its diagnosis is difficult and early diagnosis could reduce the mortality. Surgery remains the mainstay of treatment. We report two cases of gallstone ileus. The first was a 78-year old woman who had a 2-d history of vomiting and epigastralgia. Plain abdominal film suggested small bowel obstruction clinically attributed to adhesions. Later on, gallstone ileus was diagnosed by abdominal computed tomography (CT) based on the presence of pneumobilia, bowel obstruction, and an ectopic stone within the jejunum. She underwent emergent laparotomy with a one-stage procedure of enterolithotomy, cholecystectomy and fistula repair. The second case was a 76-year old man with a 1-wk history of epigastralgia. Plain abdominal film showed two round calcified stones in the right upper quadrant. Fistulography confirmed the presence of a cholecystoduodenal fistula and gallstone ileus was also diagnosed by abdominal CT. We attempted to remove the stones endoscopically, but failed leading to an emergent laparotomy and the same one-stage procedure as for the first case. The postoperative courses of the two cases were uneventful. Inspired by these 2 cases we reviewed the literature on the cause, diagnosis and treatment of gallstone ileus.     

Osteonecrosis in patients with acquired immunodeficiency syndrome (AIDS): report of two cases and review of the literature

An increase in the incidence of abnormalities on bone and mineral metabolism (osteopenia/osteoporosis) and the development of osteonecrosis has been observed in patients with acquired immunodeficiency syndrome (AIDS). Two cases of osteonecrosis in patients with AIDS are reported. Both patients were receiving highly active antiretroviral therapy (HAART) and presented with one or more known risk factors for osteonecrosis. We review the literature and discuss the pathogenesis, diagnosis, prevention and treatment of this entity in patients with AIDS.     

异基因骨髓移植治疗自身免疫病2例报告并文献复习

目的：通过2例自身免疫病在异基因骨髓移植后达治愈及长期缓解的病例分析和文献复习，探讨异基因骨髓移植对自身免疫病的疗效和安全性。方法：病例分析和文献复习。结果：2例骨髓移植前分别确诊系统性红斑狼疮和强直性脊柱炎的患者接受异基因骨髓移植后体内异基因骨髓均长期稳定植入，自身免疫病达完全缓解，已经分别随诊126个月和44个月。结论：自身免疫病可以由异基因骨髓移植治愈或达长期缓解。     

Adult Kawasaki disease: report of two cases and literature review

OBJECTIVES: To describe 2 cases of adult Kawasaki Disease (KD) and to review the medical literature to better define the epidemiological, clinical, laboratory, histopathological, cardiovascular, and therapeutic aspects of adult KD compared with pediatric KD. METHODS: Report of 2 cases, and review of the literature using a Medline search from 1967 to June 2003. RESULTS: Including our 2 cases, there are 57 reports of adult KD, 74% among patients aged 18 to 30 years. Nine cases of KD associated with human immunodeficiency virus (HIV) infection were described, suggesting that an immunocompromised state may predispose to this syndrome. The incidence of specific diagnostic criteria was roughly similar in adults and in children. However, cheilitis, meningitis, and thrombocytosis were observed in a larger percentage of children, while arthralgia, adenopathy, and liver function abnormality were more common in adults. Although adult KD often was diagnosed after the acute phase, when a significant beneficial effect from gammaglobulin infusion could not be expected, this treatment did appear to shorten the course of the disease. Coronary aneurysms were less frequent in adults than in children. Prognosis was more favorable in adults, with less cardiovascular complications and no deaths. CONCLUSIONS: Adult KD is a rare condition, which may go unrecognized. Other known disease processes with similar clinical presentations such as hypersensitivity drug reaction and toxic shock syndrome must be ruled out. For adult KD, exclusion criteria such as absence of hypotension, visceral impairment, staphylococcal infection, and any drug able to induce a drug hypersensitivity reaction are suggestive of the diagnosis, in the presence of the inclusion criteria, rash, conjunctival effusion, oropharynx changes, extremity changes, or adenopathy.     

Austrian syndrome: report of two new cases and literature review

In April 2004, Rammeloo et al. reported a child-aortic-valve endocarditis following pneumococcal meningitis. Classically, the association of pneumococcal pneumonia along with meningitis and infectious endocarditis (IE) is known as Austrian syndrome. We assessed the incidence, clinical manifestations and follow-up of patients diagnosed with Austrian syndrome in Lugo (Northwest Spain) between 1987 and 2002. A computerized MEDLINE search was also performed for literature review. During the study period 165 Lugo patients met criteria for definite IE. Two of the 3 patients with definite streptococcus pneumoniae IE also met definitions for Austrian syndrome. The incidence of this syndrome in Lugo patients with definite IE was 1.2%. In the literature review 48 cases, including our two patients, were found. Complete clinical information was only available on 16 patients. Alcoholism constituted the most common predisposing factor. Aortic valve involvement and high grade of valve regurgitation leading to cardiac failure and the need of cardiac surgery during the admission were common in these patients. This review confirms that Austrian syndrome is a rare but severe disease. Early recognition of these patients is required to avoid ominous complications.     

Interferon beta 1a-induced severe autoimmune hepatitis in patients with multiple sclerosis: report of two cases and review of the literature

Type I interferons are potent cytokines that possess antiviral, immunomodulating and antiproliferative actions. The development of autoimmune hepatitis is a well recognized complication of treatment with alpha IFN in patients with chronic viral hepatitis. Yet, the occurrence in patients under treatment with beta IFN for other indications is controversial and its occurrence often underestimated. We report two cases of severe acute autoimmune hepatitis in two patients undergoing therapy with IFN beta 1a for multiple sclerosis who recovered under early immunosuppressive therapy.     

成人气管性支气管2例报告并文献复习

目的 提高对成人气管性支气管疾病的认识和诊断水平.方法 对2例成人气管性支气管的临床资料进行回顾性分析,分析其临床特征及电子支气管镜检查在诊断和治疗方面的作用.结果 该2例患者因肺部占位性病变行电子支气管镜检查,发现伴有气管性支气管,予电子支气管镜下活检占位组织后,气管性支气管未予处理.结论 气管性支气管是少见的气道先天发育异常,目前国内成人报告病例极少,随着介入肺脏病学及医学影像学的发展,其检出率日益增多,若无临床症状可不予特殊处理.     

支气管肺蠊缨滴虫感染二例并文献复习

目的 提高对支气管肺蠊缨滴虫病的认识与诊断水平.方法 分析武警部队上海总队医院呼吸内科确诊的2例支气管肺蠊缨滴虫病的临床资料,结合1993-2006年文献报道的13例共15例进行文献复习.结果 (1)2例患者中,1例表现为重症哮喘,反复夜间发作咳嗽、胸闷、气喘,肺部听诊可闻及哮鸣音,支气管镜检查提示右上叶支气管狭窄、黏膜充血水肿;1例表现为支气管扩张伴感染迁延不愈,患者反复咳嗽、咳痰伴低热,胸部X线片、CT示双肺多个叶段支气管扩张伴感染,支气管镜检查提示支气管黏膜充血水肿,管腔狭窄,支气管管腔内可见白色坏死样物.2例均经支气管镜检查采集标本检验后明确诊断.(2)文献报道的13例均经病原学检查并由寄生虫病专家鉴定,诊断依据确凿.支气管肺蠊缨滴虫病最常见的症状是发热(64.3%)和咳嗽、咳痰(71.4%),半数患者外周血嗜酸性粒细胞数增多,X线胸片及胸部CT影像以肺炎样改变多见(占摄片数83.3%),慢性患者可表现为支气管哮喘、支气管扩张、肺脓肿等.痰液和经支气管镜检查采集标本,直接涂片检查是明确病原的方法.结论 支气管肺蠊缨滴虫病为新发感染性疾病,感染人体的蠊缨滴虫的宿主、传播途径、易感人群等尚不清楚,治疗亦需进一步研究.     

Cerebral thrombophlebitis revealing hyperthyroidism: two cases report and literature review

INTRODUCTION: Two cases of hyperthyroidism revealed by a cerebral venous thrombosis are reported. EXEGESIS: Clinical symptomatology consisted of intra-cranial hypertension and convulsive crisis associated to thyrotoxicosis. Evolution was favorable with etiologic and symptomatic therapy. CONCLUSION: The cerebral venous thrombosis risk on hyperthyroidism is discussed.