Punctal and canalicular stenosis associated with systemic fluorouracil therapy

Ocular side effects of systemic fluorouracil include excessive lacrimation, due to punctal and canalicular stenosis and fibrosis. Obstruction of the tear ducts after systemic therapy with fluorouracil is more frequent than is assumed. Five patients with breast cancer and punctal or canalicular stenosis are presented. Although complaints of epiphora usually resolve two weeks after cessation of systemic therapy, local antibiotics and steroids may be indicated. In patients with persisting complaints, and patients treated with fluorouracil for a prolonged period of time, prophylactic intubation of the tear ducts with silastic tubes has been advocated. (Conjunctivo)dacryocystorhinostomy may be unavoidable.     

Effects and systemic uptake of the new mitomycin C analogue KW-2149 in beagle dogs after intravesical administration

The present study was designed to evaluate the local effects of the new mitomycin C analogue KW-2149 after intravesical instillation, together with its penetration into the systemic circulation in healthy beagle dogs. Two reference dogs were treated with two instillations of mitomycin C (30 mg in 30 ml phosphate buffer). Four dogs were given two, three, four and six instillations, respectively, of KW-2149 (60 mg in 30 ml phosphate buffer). KW-2149 concentrations measured in the systemic circulation were very low and were frequently found to be below the limit of determination. The number of instillations had no influence on the KW-2149 concentrations measured in the systemic circulation. Blood analysis showed no systemic toxicity. The histopathological findings in the bladder were comparable in both groups. The number of instillations had no influence on the severity of the lesions found in the bladder wall. On the basis of its in vitro activity KW-2149 can be regarded as a promising agent for intravesical treatment of superficial bladder cancer.     

Methotrexate therapy in systemic-onset juvenile rheumatoid arthritis in Saudi Arabia: A retrospective analysis

Eighteen patients (nine girls, nine boys) with systemic onset juvenile rheumatoid arthritis (SO-JRA) treated with methotrexate (MTX) for a mean period of 18 months (range 6–41 months) were analysed to evaluate the safety and efficacy of MTX in this disease subtype. The MTX dose ranged from 2.5 to 15 mg/week with a mean cumulative dose of 684.9 mg/patient at the last follow-up visit. Systemic features were severe in 10 patients before MTX was started. None of these patients showed systemic features at the last follow-up visit. Sixteen patients (89%) showed improvement in both the active joint count (from a mean of 12.0 to 1.3 joints/patient) and function class (from a mean of 3.0 to 1.3) while receiving MTX. Eleven patients (61%) showed a significant decrease in the erythrocyte sedimentation rate (>50% of the initial value), an improvement in anaemia (haemoglobin >2 g) and reduced thrombocytosis (platelets 2×10⁵). Of the patients receiving corticosteroids, three patients (20%) were able to discontinue prednisone and the dose was reduced to less than 50% of the initial dose in seven patients (47%). At these doses of MTX, no gastrointestinal, hepatic or haematological toxicity was encountered and none of the patients withdrew because of toxicity or lack of efficacy. This report suggests that MTX is an effective and safe treatment in controlling systemic and articular features in this subtype of JRA.     

Mycobacterium intracellulare infection of the shoulder and spine in a patient with steroid-treated systemic lupus erythematosus

Atypical mycobacterial infections of bone are rare. A patient with systemic lupus erythematosus treated with steroids developed an M. intracellulare infection of the shoulder and spine. These infections are insidious and diagnosis is difficult. Marked involvement of one joint, large effusion, or aspirated small synovial fragments suggest an atypical tuberculous joint infection.     

Use of the Bio-Rad silver stain to identify gel purified RNA components of small nuclear ribonucleoprotein antigens

The ultrasensitive silver staining procedure developed for the detection of proteins in polyacrylamide gels has been used to identify the RNA component of small nuclear ribonucleoprotein antigens recognised by antinuclear antibodies. The technique described is a simple, safe and inexpensive procedure with a sensitivity comparable with that obtained by biosynthetic labelling of RNA with 32P.     

可诱导共刺激分子在系统性红斑狼疮病人外周血T细胞亚群的表达及与临床的关系

目的　检测可诱导共刺激分子(inducibleco stimulator,ICOS)及相关免疫分子在SLE病人外周血T细胞亚群的表达,探索其与SLE疾病活动程度、病程及血清抗dsDNA抗体和免疫球蛋白含量间的关系,为进一步研究ICOS在SLE免疫病理中的作用奠定基础。方法　采用流式细胞术检测第二军医大学附属长海医院住院和门诊SLE患者 (n=51)以及健康体检者 (n=30)外周血T细胞亚群表面ICOS及其他免疫分子CD45RO、CD45RA、HLA DR的表达水平,观察ICOS在不同细胞亚群表达水平与SLE活动程度、病程、血清抗dsDNA抗体和血清免疫球蛋白含量间的关系。结果　与正常人相比,活动期及稳定期SLE患者外周血CD4+T细胞、CD8+T细胞表达ICOS的水平显著升高,但活动期患者与稳定期患者之间无统计学差异(P≥0 05);同一SLE患者在疾病活动期,外周血CD4+、CD8+、CD45RO+、CD4+CD45RO+和CD8+CD45RO+细胞表面ICOS的表达水平明显高于该患者经过治疗疾病缓解阶段(P<0 05);初发SLE患者CD45RO+细胞表达ICOS水平明显高于复发患者 (P<0 05);血清抗dsDNA抗体( +)及免疫球蛋白(IgG、IgM和IgA中任何一种 )异常升高的患者外周血CD45RO+及CD4+CD45RO+细胞表达ICOS的水平分别明显高于血清抗dsDNA抗体 ( -)和免疫球蛋白含量正常的患者(P<0 05)。结论　SLE患者外周血某些T     

564例抗磷脂抗体检测的临床意义

目的总结抗心磷脂抗体(ACA)、狼疮抗凝物(LA)、活化蛋白C抵抗性(APCR)检测与脑梗死、习惯性流产、系统性红斑狼疮(SLE)、深静脉血栓等血栓相关性疾病之间的关系及其临床意义。方法采用APIT-LA法筛选LA;酶联免疫吸附试验(ELISA)法检测ACA—IgG、IgM、IgA;活化蛋白C抑制比值法检测APCR。结果在检测的564例患者中,ACA—IgG、IgM、IgA的阳性率分别为30％、24％和11％,LA的阳性率为6．5％,APCR阳性率为7．9％。其中脑梗死64例,ACA—IgG、IgM、IgA的阳性率为14％,APCR的阳性率为7．8％,未发现LA阳性的病例。265例习惯性流产的患者中,ACA—IgG、IgM、IgA阳性率分别为19％、18％和3％,LA阳性率2．7％,APCR阳性率5．8％。171例的SLE患者中,ACA—IgG、IgM、IgA的阳性率分别为35％、25％和18％,LA阳性率8．8％,APCR阳性率为6％。其他原因不明的血栓性疾病患者64例,ACA—IgG、IgG、IgA的阳性率分别为78％、55％、22％,LA阳性率为30％,APCR的阳性率为23％。结论ACA、LA、APCR的检测对SLE、脑梗死、习惯性流产,尤其是原因不明的血栓性疾病患者的诊断和发病机制研究有重要价值。     

Renal artery thrombosis in a pediatric case of systemic lupus erythematosus without antiphospholipid antibodies

A 13-year-old Japanese boy with an 11-month history of systemic lupus erythematosus (SLE) without antiphospholipid antibodies (APAs) suddenly developed severe hypertension, associated with fever and generalized seizures, and mild abdominal pain. Emergency abdominal computed tomography (CT) confirmed left renal artery thrombosis, and a renal scintiscan revealed reduced blood flow to the left kidney. Promptly instituted intravenous anticoagulant therapy was not effective for controlling the infarction-reduced renal arterial blood supply. Moreover, he developed stupor due to central nervous system (CNS) lupus a week after the occurrence of the hypertensive episode. Finally, a percutaneous transluminal angioplasty successfully relieved the occlusion of the left renal artery at its origin. The CNS lupus was also successfully treated with intravenous methylprednisolone pulse therapy combined with intrathecal methotrexate and dexamethasone. Although it is well known that SLE patients with APAs have a high incidence of thrombotic complications, to date, renal artery thrombosis has rarely been reported in young patients. This APA-negative SLE patient unusually manifested renal thrombosis associated with CNS lupus.     

Infantile systemic lupus erythematosus presenting with pulmonary hemorrhage

Systemic lupus erythematosus in infants born to healthy mothers is a rare entity. We describe a male infant who presented at 1 month of age with pulmonary hemorrhage and glomerulonephritis due to systemic lupus erythematosus, confirmed serologically and histologically. He was managed with a combination of prednisone and intermittent cyclophosphamide, but also received mycophenolate mofetil, with a complete serological and clinical remission at 30-month follow-up. This case underscores the importance of a broad approach to the evaluation of pulmonary hemorrhage and glomerulonephritis in the very young and the need for aggressive immunosuppressive therapy to achieve sustained serological and clinical remission.     

Immunophenotypic and genetic characterization of a CD8 positive mantle cell lymphoma in a patient with concomitant Mycosis fungoides

Mantle cell lymphoma (MCL) is immunophenotypically characterized by cell surface co-expression of CD19, CD20, CD5, IgM and FMC7. However, the concomitant presence of other antigens distinctive of a particular leukocyte subset, e.g. T-lymphocytes, is an exceptional finding in MCL. Here, the first case of a blastic MCL in leukaemic phase with aberrant expression of the T-cell associated antigen CD8 occurring in a patient with concomitant Mycosis fungoides is described. Comprehensive immunophenotypic analysis showed that the MCL cells expressed the typical B-lymphocytic markers, were CD5 and CD8 positive, but did not express other T-cell proteins, such as CD2, CD3, CD4, CD7, TCRalphabeta and TCRgammadelta. The MCL cells expressed both CD8alpha and CD8beta chains indicating cell surface presence of CD8alphabeta heterodimers. Intriguingly, expression of the cytotoxic enzymes perforin and granzyme A was detected by RT-PCR. Cytogenetic and molecular genetic analysis of the lymphoma cells confirmed cyclin D1 overexpression secondary to the t(11;14)(q13;32) chromosomal translocation. Furthermore, trisomy 11, trisomy 14 and extra copies of t(11;14) translocated chromosomes were detected in sub clones of the analyzed MCL cells. Clinically, an aggressive course of disease including cerebral lymphoma involvement was noted in the reported patient. Hence, systematic studies addressing the incidence, biology and clinical behavior of this form of MCL seem to be justified in future.