Clinical analysis of 78 cases of paroxysmal nocturnal hemoglobinuria diagnosed in the past ten years

Objective To learn more about the clinical and laboratory features of patients with paroxy smal nocturnal hemoglobinuria (PNH) diagnosed in the past ten years. Methods Clinical and laboratory data for 78 cases of PNH diagnosed from January 1990 to November 1999 in our hospital were analyzed retrospectively. Results In comparison with PNH cases reported in the 1980s, the newly diagnosed PNH cas es showed the following features: (1) older age of disease onset (from 27 to 34 years); more female cases (from 18. 5% to 38. 5%); more cases without hemoglobin uria (from 24. 2% to 38. 5%). (2) No positive family hereditary history. (3) Bone marrow dysplasia, abnormal karyotype and negative sister chromatid differen tiation were found in 19. 2%, 12. 2% and 8. 9% of the PNH patients, respectively . 12. 3% of the patients had bone marrow hypoplasia, and most of them had no he moglobinuria. Ham’s tests were negative in about 34. 2% of the cases. CD55 and CD59 on peripheral blood cells were deficient in 100. 0% of the cases, suggesti ng that CD55 and CD59 tests can improve the diagnosis of PNH. (4) Adrenocortic al hormone was effective in 83. 8% of the patients, 54. 2% of whom relapsed with in one year. Eight refractory and relapsed patients were treated with low dose chemotherapy (MP therapy: Melphalan 2-6 mg·d; Prednisone 0. 5 mg·kg(-1) ·d(-1) ). Five (62. 5%) of them showed positive responses. Bone marrow failure and other side effects were not serious in this group of patients Conclusions PNH, an acquired blood disease seen more often among adult males, can be diagno sed more sensitively by hemocyte member CD55 and CD59 tests and treated more eff ectively with adrenocortical hormone or low dose chemotherapy.     

Diffuse panbronchiolitis with histopathological confirmation among Chinese

Background Diffuse panbronchiolitis (DPB) was originally and is still primarily reported in Japan, rarely in other countries. As macrolide therapy is effective for this disease with once dismal prognosis, familiarity with its clinical features is urgently needed, especially for clinicians outside Japan. The objectives of this study were to investigate the clinical features of DPB in a Chinese population and propose diagnostic procedures that will lead to increased awareness of this treatable disease among clinicians, ultimately allowing for more rapid diagnosis.Methods After a literature review, the clinical features of DPB were histopathologically confirmed in a series of 9 cases either by open lung biopsy or video-assisted thoracic surgical biopsy, resulting in the largest series of confirmed DPB cases in a non-Japanese population. Here, the cases are retrospectively described and diagnostic procedures are discussed.Results Persistent cough, sputum, and exertion dyspnea occurred in 89% of patients, a history of or current chronic sinusitis in 78%, centrilobular micronodules appearing on chest CT scans in 100%, coarse crackles in 78%, FEV1/FVC＜70% in 44%, PaO2＜80 mmHg in 56%, and titer of cold hemagglutinin≥1∶64 in 11%. According to its clinical diagnostic criteria, diagnosis was definitive in 44%, suggested in 33%, and excluded in 23% at the time of diagnosis. However, DPB was clinically considered before confirmation in only 22% of patients, with the remaining 78% of cases missed or mistaken for other diseases. Of the 9 cases, 8 received transbronchial biopsies before confirmation of the diagnosis, but all showed non-specific inflammation.Conclusions Although its clinical features may vary with disease course and ethnic populations, most cases of DPB can be diagnosed or suggested according to clinical diagnostic criteria. However, underdiagnosis as a result of unfamiliarity with its clinical features and diagnostic criteria prevails. If difficulty in diagnosis arises, the diagnosis should be based on clinicopathological features and the exclusion of other diseases. Few cases can be confirmed by transbronchial biopsies; in these cases, either an open-lung biopsy or a video-assisted thoracic surgical lung biopsy should be recommended.     

Blastomycosis in China: a case report and literature review

Blastomycosis is a fungal disease that is endemic in parts of North America. It is very rare in China and also commonly misdiagnosed, often as cancer or other infectious diseases. The clinical profile of a case of disseminated blastomycosis with pulmonary changes and skin ulcers was described. He had been misdiagnosed with tuberculosis, after adequate therapy with a lipid formulation of amphotericin B, followed by itraconazole, the lung and skin lesions improved. Then the five cases reported in China and literatures were reviewed. The aim of this report was to improve the knowledge regarding blastomycosis for physicians in China to avoid delaying adequate therapy.

     

Clinical spectrum of diffuse crescentic glomerulonephritis in Chinese patients

Objective To investigate retrospectively the incidence, distribution of primary disease and clinicopathologic characteristics of diffuse crescentic glomerulonephritis (DCGN) in Chinese patients.Methods One hundred and seventy-two consecutive patients diagnosed as having DCGN out of 9828 cases of non-transplanting renal biopsies over sixteen years, were studied. DCGN is categorized into three types according to immunopathologic characteristics. The incidence of this disease, its primary diseases, clinical characteristics and serum antineutrophil cytoplasmic antibodies (ANCAs) were analyzed.Results The distribution of patients among the three classifications was 8.7% type Ⅰ, 68.6% type Ⅱ and 22.7% type Ⅲ. Clinically, the majority of patients (69.8%) presented rapidly progressive glomerulonephritis (RPGN), but 30.2% manifested a chronic nephritic syndrome or chronic renal failure. In terms of related conditions, 93% were anemic, 61.6% had hypertension, 50.6% oliguria, 45.3% nephrotic syndrome, 43% uremic syndrome and 39.5% displayed gross hematuria. Those patients who were positive in serum for ANCAs had predominantly type Ⅲ DCGN. Two cases with anti-GBM-antibody crescentic glomerulonephritis and three with lupus nephritis were also positive for ANCAs in serum.Conclusion DCGN is not rare in Chinese patients. A majority of patients in our study presented with RPGN, but 30.2% manifested a chronic renal failure. Lupus patients with DCGN that were positive for ANCAs had more severe vasculitic lesions.     

Embolic events in 93 elderly Chinese patients with atrial fibrillation

Objectives To evaluate the prevalence of embolic events and relevant factors in elderly Chinese patients with atrial fibrillation(AF), and to provide evidence on ways to prevent embolic events.Methods Autopsy data from ninety-three continous elderly Chinese patients with AF were analysed. The incidence of embolic events and its relationship to underlying disease, pathologic changes in the heart, and other clinical characteristics were examined.Results Embolism were observed in 27 of 93 cases, with an incidence of 29.03%. The incidence of embolic events was higher in elderly patients with rheumatic heart disease than those with coronary artery disease, hypertensive myocardiopathy and heart diseases. Patients with chronic AF,with a course of AF≥3 years, and those with heart failure or diabetes had a higher incidence of embolic events than those without these complications. There was significant difference in incidence between paroxysmal and chronic AF. Patients with left atrial or ventricular enlargement, mural thrombosis in cardiac chambers, valvular calcification and valvular vegetation also had a higher incidence of embolic events. Oral dipyridamole (75-150 mg/d) or aspirin (50-150 mg/d) showed no definite effects in preventing embolism in some patients.Conclusions There was a high incidence of embolic events in elderly Chinese patients with AF. Anticoagulation therapy should be provided to the elderly patients with AF, especially to the patients with risk factors for embolism.     

Clinical features and management of Crohn’s disease in Chinese patients

Background An increasing incidence of Crohn‘s disease has been found in China in recent years.Our study has been focused on evaluating the diversity of the clinical manifestations of Crohn‘ s disease in order to improve early diagnostic accuracy and therapeutic efficacy.Methods Thirty patients with active Crohn‘s disease were enrolled and their clinical data, including diagnostic and therapeutic results, were analyzed. Endoscopy combined with histological examination of biopsy specimens provided characteristic features of the disease. Transabdominal bowel sonography (TABS) was used for detecting intestinal complications. Nutritional supportive therapy was given to 20 subjects with active cases of the disease.Results Most patients were young adults with a higher proportion of females to males ( ratio: 1.14:1 ). The disease affects any segment or a combination of segments along with the alimentary tract(from the mouth to the anus). In this study, the colon and small bowel were the major sites involved.Recurrent episodes of abdominal pain in the right lower quadrant and watery diarrhea were the most common symptoms. Granulomas were identifiable in nearly one-third (30. 8%) of all biopsy specimens. In moderate cases of the disease, remission was achieved more quickly through the use of oral prednisone therapy than with SASP or 5-ASA. Beneficial effects on the host‘ s nutritional status were observed. Immunosuppressives were used on an individual basis and showed variable therapeutic effects. Sixteen patients had surgery due to intestinal obstruction or failure to respond to drug therapies. Rapid improvement after surgery was reported.Conclusion Endoscopy (with biopsy) and TABS were both crucial procedures for diagnosis. SASP (or 5-ASA) and prednisone were effective as inductive therapies. Azathioprine has demonstrable benefits after induction therapy with prednisone. Surgery, as an alternative treatment, provided another effective choice in selected patients.     

Myasthenia gravis in pediatric and elderly patients

Objective To determine whether the clinical and pathologic characteristics and prognoses of myasthenia gravis (MG) patients below 15 years differ from those patients over 50 years after thymectomy. Methods We reviewed the registry material of 30 pediatric and 32 elderly MG patients after thymectomy, including their age, sex, clinical classification, pathological types, and prognoses. The Chi-square test or Wilcoxon’s rank-sum test was used to determine the statistical differences between the children and elderly groups.Results No significant difference was seen in sex distribution between the two groups (Chi-square test, P=0.625), but there were differences in clinical classification: more type Ⅰ was observed in the pediatric group than in the elderly group, but more type Ⅱor Ⅲ was seen in the elderly group (Wilcoxon’s rank-sum test, P<0.001). As to pathological types, the pediatric group was also significantly different from the elderly group (Chi-square test, P<0.01). All of the patients (100%) in the pediatric group had thymus hyperplasia, but in the elderly group more than half (56.26%) were found to have thymoma (benign or malignant). The prognoses after thymectomy were better in the pediatric group than in the elderly group (Wilcoxon’s rank-sum test, P<0.001). Conclusions Because the prognoses are generally better than those of the elderly patients, we should be careful when operating on pediatric patients of ocular type. The elderly patients tend to receive more aggressive treatment because of more severe generalized types often associated with thymoma and poor prognoses. Both pediatric and elderly patients are seldom associated with other autoimmune disease.     

Glutamic acid decarboxylase 65 autoantibody levels discriminate two subtypes of latent autoimmune diabetes in adults

Objective To compare the clinical characteristics between type 2 diabetes mellitus (T2DM) and latent autoimmune diabetes in adults (LADA) with different titers of glutamic acid decarboxylase autoantibody (GADA) and to define the two distinct subtypes of LADA.Methods Sera of 750 patients with an initial diagnosis of T2DM from central south of China were screened for GADA using a radioligand assay. The distribution and frequency of GADA levels were described. Two hundred and ninety-five patients were divided into the T2DM group (n=233) and the LADA group (n=62) to compare the age of onset, body mass index, HbA1c, C-peptide, hypertension, dyslipidemia and chronic diabetic complications. Furthermore, LADA patients with different GADA titers were subdivided to analyze the same indexes as the above. Results The prevalence of LADA (defined as GADA≥0.05, namely GADA positive) was 9.7% in the 750 initially diagnosed type 2 diabetic patients. Compared with T2DM, LADA patients were younger at their ages of onset, had lower C-peptide and body mass index, and also had less cases with hypertension and with dyslipidemia. However, only patients with high titer of GADA had poorer beta cell functions and less diabetic complications compared to T2DM and low GADA titer of LADA patients. Patients with low GADA titer were similar to T2DM patients, except that they were prone to develop ketosis more frequently.Conclusions Two clinically distinct subtypes of LADA can be identified by GADA levels in patients initially-diagnosed as type 2 diabetes. Patients with high titer of GADA (GADA≥0.5) subsequently develope more insulin dependency, which are classified as LADA-type 1; while those with lower GADA titer (0.05≤GADA<0.5) and having clinical and metabolic phenotypes of type 2 diabetes are classified as LADA-type 2.     

Advances in antithrombin therapy for ST-elevation myocardial infarction

Thrombin is a pivotal molecule in acute myocardial infarction ( Ml) because of its extensive procoagulant and prothrombotic actions. Antithrombin therapy is an important component of the pharmacotherapy for acute Ml. The standard agent used in clinical practice, unfractionated heparin (UFH), is associated with the disadvantages of variable anticoagulant effect, inability to inhibit clot-bound thrombin, neutralization by platelet factor 4, and the propensity to cause thrombocytopenic complications. Novel thrombin inhibitors have been developed to overcome these disadvantages. Although possessing the property of inhibiting both fluid-phase and clot-bound thrombin, the direct thrombin inhibitor hirudin has been shown to give marginal benefits over UFH as adjunct to fibrinolysis in ST-elevation Ml. Bivalirudin, another direct thrombin inhibitor, is able to reduce reinfarction in patients treated with streptokinase and is a new anticoagulant treatment option in this setting. The pharmacokinetic characteristi     

Current clinical management strategies for benign prostatic hyperplasia

This article summarizes the major new findings on clinical management for benign prostatic hyperplasia （BPH） that were presented at the annual meeting of the American Urological Association （AUA） in May 2008. The management of symptomatic BPH has been changed significantly over the last decade in response to the availability of new treatment options. Prior to the 1980s, open prostatectomy was the only widely accepted intervention for BPH. Since then, the advent of new medical therapies for BPH and the introduction of a range of minimally invasive therapies have provided for men with lower urinary tract symptoms （LUTS） secondary to BPH.1 In this year＇s AUA meeting, several new findings were reported in the filed of BPH which involved epidemiology, clinical progression, drug therapy and new technologies in surgical therapy of BPH.     

弥漫性泛细支气管炎给我们的启示

弥漫性泛细支气管炎是发现于日本的新呼吸系统疾患,与种族相关抗原关系密切,是一种遗传相关性疾病.十四、十五元环大环内酯类抗生素对其有特殊疗效.该病及大环内酯类药物疗法的发现过程,提示我们应重视疾病的人种差别,不要轻易放过医疗过程中遇到的偶然现象.     

弥漫性泛细支气管炎(DPB)的临床特征分析

 目的  总结弥漫性泛细支气管炎(diffuse panbronchiolitis,DPB)的临床特征,提高诊疗水平。方法  收集31例就诊于中国医科大学呼吸疾病研究所的DPB确诊病例,总结和分析其临床表现、影像学特征及其对红霉素治疗的反应。结果  31名患者均咳嗽、咳痰和/或呼吸困难,闻及湿罗音。平均用力呼气容积占预计值的百分比(FEV1%pred)为54.1%±11.2%,平均动脉血氧分压为(63±12.5) mmHg。31名患者均有慢性鼻窦炎。胸部CT均显示弥漫性小叶中心性分布小结节的24例(77.4%)伴树芽征;19例(61.3%)有周围性支气管扩张,其中12例(38.7%)伴中央性支气管扩张。13例(41.9%)患者有铜绿假单胞菌感染,其中3例有周围性支气管扩张,另外10例同时有中央性和周围性支气管扩张。经过3个月肠溶红霉素治疗后,平均FEV1%pred为67.5%±14.7% (t=2.451,P=0.036),平均动脉血氧分压为(71.0±8.1) mmHg (t=2.153,P=0.029),铜绿假单胞菌培养2例(χ2=4.65,P=0.031)。结论  慢性鼻窦炎和肺CT表现为弥漫性小叶中心性小结节伴树芽征和周围性支气管扩张是DPB的突出临床表现;红霉素可有效治疗该病。     

Diffuse panbronchiolitis--a case report

Diffuse panbronchiolitis (DPB) is a chronic progressive obstructive disease of the airways. Of unknown aetiology, it is responsive to macrolide therapy. Although not uncommon among the Japanese, it is rare in other parts of the world. We report a case of DPB in a Chinese patient in Singapore.     

弥漫性泛细支气管炎误诊4例临床分析

[目的]探讨弥漫性泛细支气管炎的临床特征诊断及治疗方法。[方法]分析4例弥漫性泛细支气管炎患者临床表现、实验室检查、影像学、肺功能特点以及治疗效果。[结果]弥漫性小叶中心性结节3例,两肺弥漫性小叶中心性颗粒样结节状阴影合并广泛支气管扩张1例。4例患者鼻窦CT均提示副鼻窦炎,2例合并鼻息肉。肺功能检查3例患者呈阻塞性通气功能障碍,1例呈混合性通气功能障碍;4例FEV1/FVC(一秒钟用力呼气容积/用力肺活量)均70%。4例经过大环内酯类药物3～6个月后均取得较好的疗效。[结论]弥漫性泛细支气管炎可根据病史、临床表现、影像学及肺组织活检进行诊断,及时使用红霉素等大环内酯类药物治疗则可以阻止病变进展,改善预后。     

艾滋病合并马尔尼菲青霉病83例的胸部影像表现分析

目的 探讨获得性免疫缺陷综合征(AIDS)合并马尔尼菲青霉病(青霉病)的胸部影像表现及治疗后转归.方法 对83例艾滋病合并青霉病患者的胸部影像表现及临床资料进行回顾性分析.结果 83例患者胸部X线、CT影像学表现主要为5种类型:(1)以结节病变为主要表现29例;(2)以斑片或大片状渗出为主要改变27例;(3)以肿块病变表现6例;(4)双肺呈弥漫磨玻璃样改变为主5例;(5)双肺呈弥漫粟粒病变5例;(6)其他表现11例.治疗后肺部病灶明显吸收、消失77例,死亡6例.病灶呈双肺弥漫分布的患者病死率为14.6%(6/41),高于其他病灶分布患者(0/42),差异有统计学意义(P＜0.05);CD4+T细胞计数多少与病死率关系不大(P＞0.05).结论 青霉病绝大部分发生于AIDS晚期患者,胸部表现多种多样,以双肺弥漫性病变患者病死率高.     

弥漫性泛细支气管炎4例诊治分析

目的研究弥漫性泛细支气管炎(DPB)的临床特点,以提高临床医师的认识水平,避免误诊。方法对4例临床诊断为DPB患者的临床资料、辅助检查资料和治疗效果进行分析。结果患者均有咳嗽、咯痰、活动后气促的症状,双肺均有断续性湿性啰音,伴有干性啰音,同时伴有副鼻窦炎3例,冷凝集试验>1:64者1例;动脉血气均有低氧血症,肺功能检测提示阻塞性通气功能障碍为主。胸部X线典型表现为双肺弥漫性小颗粒结节影。胸部HRCT均提示双肺野弥漫性小叶中心性结节,两肺中下叶为明显;伴有支气管扩张1例;伴网状、条索状阴影1例。使用阿齐霉素治疗疗效明显。结论 DPB并非少见,减少误诊、及时予以治疗,能显著改善DPB患者预后。阿奇霉素可作为治疗DPB首选药物。     

大环内酯类抗生素治疗恙虫病的效果

目的研究大环内酯类抗生素与氯霉素和强力霉素治疗恙虫病的疗效区别。方法选取南海某群岛上近8年发生的恙虫病病例279例,分别应用了氯霉素、强力霉素、红霉素、罗红霉素、阿齐霉素治疗,回顾性分析退热时间及治愈率。结果与氯霉素和强力霉素相比,大环内酯类抗生素治疗恙虫病退热时间较长,其中红霉素时间最长、罗红霉素次之、阿齐霉素时间最短,而大环内酯类抗生素、氯霉素、强力霉素治疗恙虫病治愈率无统计学意义。结论大环内酯类药物、氯霉素和强力霉素对恙虫病均有较好疗效,而大环内酯类抗生素副作用小,孕产妇、婴幼儿及肝肾功能受损等病人适用大环内酯类抗生素治疗恙虫病,其中阿齐霉素为首选。     

A report of the first three cases of diffuse panbronchiolitis in Malaysia

Three cases of diffuse panbronchiolitis (DPB) occurring in two Malaysian Chinese patients and one Malay patient are reported. They had similar clinical, radiological and physiological features which are characteristic of DPB. The diagnosis in one of the cases was confirmed histologically by transbronchial lung biopsy. These could be the first three cases identified in Malaysia.     

系统性红斑狼疮并弥漫性肺泡出血的临床研究(附2例报道)

目的提高对系统性红斑狼疮(SLE)并弥漫性肺泡出血(DAH)的认识。方法报道2例SLE并DAH的临床资料,并复习相关文献。结果通过分析病例,总结SLE并DAH的临床特点、发病机制、诊断标准、治疗手段和预后。结论DAH为SLE少见而致命的并发症,发热、咯血、呼吸困难、血红蛋白下降等临床表现无特异性。影像学显示双肺弥漫性浸润影。血性支气管肺泡灌洗液内见含铁血黄素巨噬细胞。肺组织学呈弥漫性肺泡出血。早期足量激素和免疫抑制剂治疗可改善其预后。