近端指间关节周围胶原沉积症

近端指间关节周围胶原沉积症（pachydermodactyly）是一种少见的、主要发生于青少年男性的、以近端指间（PIP）关节周围非炎症性膨大为特征的胶原沉积症。组织病理学特征为真皮层大量胶原纤维沉积。本病在全球范围内累计报道不多，尚不为风湿病专科医师所熟悉，临床上易误诊为其它风湿性疾病。为了提高临床医生对本病的认识，避免临床误诊误治，现将近端指间关节周围胶原沉积症的临床特点、病因病理、影像学表现、诊断、鉴别诊断、治疗及预后作一综述。     

近端指间关节周围胶原沉积症1例并文献复习

目的探讨近端指间关节周围胶原沉积症(pachydermodactyly,PDD)的临床表现、诊断和鉴别诊断。方法对1例PPD患者的临床资料、关节累及情况、实验室检查、X线表现及病理学检查进行总结分析,并对2005～2011年国内相关内容文献进行复习。结果该患者双手近端指间关节肿胀,无疼痛;查体可见双手第2、3、4近端指间关节侧面呈对称性梭形膨大,关节无压痛,活动自如;实验室及免疫学检查均为阴性。X线片示软组织肿胀,无骨质和关节异常改变;皮肤组织病理学检查显示表皮增生伴角化过度,真皮层增厚伴胶原纤维增多和成纤维细胞轻度增生,无炎性细胞浸润,诊断为PDD。结论 PDD虽是一种少见的风湿科良性疾病,但根据其临床表现、放射学、血清学检查、组织病理学检查即可作出诊断。临床上应引起重视,避免误诊、误治。     

近端指间关节周围胶原沉积症6例临床分析

目的探讨近端指间关节周围胶原沉积症(pachydermodactyly)的发病机制、临床表现、诊断和鉴别诊断。方法总结6例患者的一般临床资料、实验室检查、关节累及情况、X线表现及病理检查。结果6例患者平均发病年龄为(14±3)岁,男性5例,女性1例。临床表现为慢性、无症状性近端指间关节(PIP)周围膨大、肿胀。6例平均累及关节数(5±3)个关节,基本呈对称性分布,主要累及双侧的PIP2,3,4关节,仅有1例为单关节受累。实验室检查均正常。X线仅示软组织肿胀,而无关节和骨质的破坏。组织病理学特征为真皮层大量胶原纤维沉积。结论本病尚不为风湿病专科医师所熟悉,应与各种可以累及PIP的关节炎进行鉴别。本研究试将pachydermodactyly的中文病名定名为“近端指间关节周围胶原沉积症”。     

近端指间关节周围胶原沉积症二例

例1,男性,19岁。因双手指间关节对称性肿胀1年半就诊。其于1年半前渐发生双手近端指间关节(PIP)肿胀,无疼痛、活动受限、晨僵、口干、眼干。体检:双手第2、3、4PIP肿胀,为侧面肿胀,未影响到伸侧及掌侧面,无压痛,无活动受限。实验室检查:血常规、尿常规(-)。类风湿因子(RF)(-),血沉3mm/h,C反应蛋白(CRP)<10mg/L,抗核抗体(-)。血清免疫球蛋白:IgG8.51g/L,IgA1.91g/L,IgM0.93g/L,补体C30.93g/L,C40.32g/L。双手X线检查显示:双手诸骨未见异常。诊断为PIP周围胶原沉积症,未予治疗,随访1年无变化。例2,男性,13岁。因双手指间关节对称…     

近端指间关节周围胶原沉积症13例临床特征分析

目的探讨近端指间关节周围胶原沉积症的免疫学改变及临床特点。方法总结1998年3月至2006年1月中国医科大学附属第一医院风湿免疫科收治的13例近端指间关节周围胶原沉积症患者的临床表现、一般实验室检查、免疫学检查和X线检查结果并进行临床分析。结果13例患者中男11例,女2例,发病年龄12~19岁,平均(16±3)岁。临床特点为慢性无痛性近端指间关节周围膨大、肿胀,多呈对称性分布。免疫学检测结果无异常所见。X线检查除软组织肿胀影外,关节及骨质无改变。结论本病属于风湿免疫性疾病,主要应与累及近端指间关节的幼年型类风湿关节炎相鉴别。     

Graves病459例5年追踪研究

目的　研究抗甲状腺药物 (ATD)或甲状腺次全切除术、加碘盐 (AIS)和甲状腺自身抗体对Graves病(GD)的影响。方法　每 3个月观察接受ATD或手术治疗的GD病人复发率、甲状腺自身抗体 ,第 1次复发病例的甲状腺激素和再次ATD治疗的缓解需时及维持ATD用量等 ;比较手术治疗和ATD治疗、AIS饮食或无碘盐(NIS)饮食、自身抗体 (TM -Ab和TG -Ab)增高和自身抗体正常病人的甲亢症状缓解需时、复发率、维持ATD用量、甲状腺激素和甲状腺大小等。结果　手术治疗组病人的TM -Ab水平、甲减发生率、第 1次复发后再次接受ATD治疗缓解后TSH水平明显高于ATD治疗组 (P均 <0 0 5 ) ;而 5年复发率、第 1次复发后的FT3 和FT4 及再次接受ATD治疗的缓解需时和维持ATD用量则明显低于ATD治疗组 (P均 <0 0 5 )。AIS饮食的ATD治疗病人的甲亢缓解需时、1年和 5年复发率、维持ATD用量和FT4 及甲状腺重量明显高于NIS饮食者 (P均 <0 0 5 ) ;而缓解期TSH则明显低于NIS饮食者 (P <0 0 5 )。自身抗体升高病人的 1年和 5年复发率、维持ATD用量和甲减发生率明显高于自身抗体正常者 (P <0 0 5 ) ;而TSH则明显低于自身抗体正常者 (P <0 0 5 )。结论　手术治疗甲亢症的缓解率较高 ,复发时病情较轻 ,再次ATD治疗较易 ;AIS饮食可增加GD病人的ATD治     

Pulmonary leukostasis: role of perfusion lung scan in diagnosis and follow up

A patient with hyperleukocytic myelomonocytic leukemia who presented to the emergency room with sudden pleuritic chest pain and dyspnea is reported. Clinical manifestations included dyspnea tachypnea and hyperventilation. Blood gas analysis revealed hypoxemia, hypocarbia, and respiratory alkalosis. Chest X ray was normal, and perfusion lung scan revealed a diffuse vascular occlusive pattern compatible with pulmonary leukostasis. The patient underwent immediate leukapheresis with subsequent mitigation of symptoms. A second perfusion lung scan showed evidence of significant improvement. To our knowledge this is the first published case of hyperleukocytosis presenting with pulmonary leukostasis that was successfully diagnosed and followed by serial perfusion lung scan.     

Haematuria in rheumatoid arthritis: a follow up study.

Twenty one patients with seropositive rheumatoid arthritis and previously documented isolated haematuria were recalled at a median 7.7 years (range 3-12) after initial investigation. Eighteen subjects had previously undergone renal biopsy, which had shown mesangial glomerulonephritis in all but four cases. Renal function was found to have remained normal in all patients with previous mesangial changes despite the presence of persistent haematuria in most cases.     

Collagenous colitis without diarrhoea at diagnosis – a follow up study

Objective: Chronic watery diarrhoea is a classical symptom of collagenous colitis (CC). However, in some cases, the typical histologic findings of CC can be found in patients without this symptom. In this study we have performed a follow up on patients with a confirmed histological diagnosis of CC without the typical symptom watery diarrhoea.

Patients and methods: A structured medical record follow-up was performed on the subgroup of patients without watery diarrhoea but diagnosed with the typical CC histologic appearance in a previous study of microscopic colitis.

Results: At follow up after a median time of 8 years (range: 0.33–12 years), five of these fifteen patients developed bowel symptoms but only two developed characteristic CC symptoms with watery diarrhoea.

Conclusion: The majority of patients without chronic watery diarrhoea at diagnosis remained free from this symptom during follow up and only in a few cases symptoms attributed to CC developed.     

Lymphoproliferative cancer and other malignancy in patients with rheumatoid arthritis treated with azathioprine: a 20 year follow up study.

Two hundred and two patients with rheumatoid arthritis (RA) starting treatment with large doses of azathioprine (median 300 mg/day) between 1964 and 1974 were followed up until March 1984. All but one patient (99.5%) were traced from either hospital or general practice records; and death certificates, where relevant, were obtained. A comparison group of 202 patients with RA not treated with azathioprine was selected from the diagnostic index of another rheumatology unit and followed up in 1985 to assess their status retrospectively at March 1984. Each patient treated with azathioprine was matched for year of birth, year of diagnosis, sex, and serostatus with a control patient from the latter group. Four lymphoproliferative cancers occurred in the azathioprine treated group compared with two in the control group. Further analysis of these findings suggested an increased risk of lymphoma of one case per 1000 patient years of azathioprine treatment. The lymphoma rates were then compared with those expected based on the incidence in the general population. This comparison suggested a fivefold increase in the RA control group and a 10-fold increase in the azathioprine treated group. There was also an excess of patients with non-lymphoproliferative cancers, including one with myeloma in the azathioprine group (29 v 19), the excess being greater in the group with the longest duration of treatment. This significantly increased risk did not, however, persist on matched analysis, was not related to maximum daily dose, and was not site specific. These results from a possibly unique series of patients treated with high dose azathioprine give some reassurance about the magnitude of the previously postulated carcinogenic risk of such treatment in RA.     

开放性手术治疗良性前列腺增生症159例术后3年临床随访

目的评价开放性手术治疗老年人良性前列腺增生症（ＢＰＨ）的远期疗效。方法回顾性分析１９９１年１月至１９９３年１２月耻骨上经膀胱前列腺切除术治疗的老年ＢＰＨ患者１５９例，其中１３０例（８１．８％）得到随访，以填写问答表格的方法，统计患者对疗效的评价。结果术前症状总计分１７．４±８．０分，术后出院时为３．７±３．３分，总分下降幅度７１．３％；梗阻症状评分下降幅度（７７．９％）大于刺激症状评分下降幅度（４０．９％）。术前最大尿流率８．０±３．６ｍｌ／ｓ，平均尿流率２．９±１．９ｍｌ／ｓ；术后２０天左右分别为１６．７±８．１及１０．２±４．８ｍｌ／ｓ。患者对手术效果总满意率为８８．２％。结论开放性手术治疗老年人ＢＰＨ其效果肯定，术后严重并发症少，仍是一种治疗老年人ＢＰＨ的重要手段。     

由一个献血员所致83例丙型肝炎病毒感染者10年的自然转归

Objective To find out clinical characteristics and natural history of post transfusion HCV infection. Methods 83 subjects who have received the blood from a same blood donor from January 1998 to July 2002 were investigated by the method combining cross-sectional study with retrospective study. HC V-antibody, HCV RNA, liver function, abdomen B-ultrasound and Fibroscan were detected. Results The HCV-antibody were all positive. The HCV RNA of 56 out of the 83 cases were positive. The chronicity rates of hepatis C were 76.3% (29/38) in male patients and 60.00% (27/45) in female patients respectively, without significant difference (x2 = 2.99, P = 0.11). The average age of the HCV RNA positive patients was (36.54 ± 14.37) years old. The average age of the HCV RNA negative patients was (27.43 ± 12.51) years old. A significant difference (T = -2.41, P = 0.018) existed between. The HCV genotype was typelb. Among the HCV RNA positive patients, 10 cases were with mild asthenia, anorexia and abdominal distention, 9 cases with increased serum ALT, 12 cases.with chronic hepatitis and 1 case was diagnosed with decompensated liver cirrhosis. Conclusion The clinical manifestations of HCV infection are occult and chronic. The chronicity rate is related to gender and the age when infection was caught.     

由一个献血员所致83例丙型肝炎病毒感染者10年的自然转归

目的 了解输血后丙型肝炎病毒感染者的临床特征及其自然病史.方法 采用横断面调查与回顾性调查相结合的方法,对1998年1月-2002年7月输受同一供血者血液的83侈HCV感染患者进行临床资料调查,同时进行抗-HCV,HCV RNA,肝功能生物化学指标,腹部B型超声检查.计量资料用7检验,计数资料用x2检验,等级资料用秩和检验.结果 抗-HCV阳性83例,HCV RNA阳性56例,丙型肝炎慢性化率为67.4%,其中男性76.3%(29/38),女性60.00%(27/45),两者差异无统计学意义(x2=2.99,P=0.11).HCV RNA阳性组的平均年龄为(36.54±14.37)岁,HCV RNA阴性组平均年龄为(27.43±12.51)岁,差异有统计学意义(T=-2.41,P=0.018).供血者感染HCV基因为1b型.HCV RNA阳性患者中有10例表现为轻度乏力、纳差、腹胀,9例ALT轻度升高,12例B型超声检查提示有慢性肝炎改变,1例为肝硬化失代偿期.结论 输血后感染HCV者临床表现隐匿,慢性化与受感染时的年龄有一定关系.

Abstract：

Objective To find out clinical characteristics and natural history of post transfusion HCV infection. Methods 83 subjects who have received the blood from a same blood donor from January 1998 to July 2002 were investigated by the method combining cross-sectional study with retrospective study. HC V-antibody, HCV RNA, liver function, abdomen B-ultrasound and Fibroscan were detected. Results The HCV-antibody were all positive. The HCV RNA of 56 out of the 83 cases were positive. The chronicity rates of hepatis C were 76.3% (29/38) in male patients and 60.00% (27/45) in female patients respectively, without significant difference (x2 = 2.99, P = 0.11). The average age of the HCV RNA positive patients was (36.54 ± 14.37) years old. The average age of the HCV RNA negative patients was (27.43 ± 12.51) years old. A significant difference (T = -2.41, P = 0.018) existed between. The HCV genotype was typelb. Among the HCV RNA positive patients, 10 cases were with mild asthenia, anorexia and abdominal distention, 9 cases with increased serum ALT, 12 cases.with chronic hepatitis and 1 case was diagnosed with decompensated liver cirrhosis. Conclusion The clinical manifestations of HCV infection are occult and chronic. The chronicity rate is related to gender and the age when infection was caught.