EMG myokymia as a cause of ptosis in hypothyroidism

Ptosis is known to be associated with thyroid disorders. We describe two biochemically corrected hypothyroid patients presenting with isolated bilateral ptosis. EMG of the orbicularis oculi showed continuous grouped motor unit potentials. In the absence of obvious aetiology, it is hypothesised that focal demyelination of terminal branches to the orbicularis oculi may play a role in the generation of the discharges.     

Distribution of α-integrin subunits in fetal polycystic kidney diseases

An alteration in cell/matrix interactions is one of the suggested mechanisms leading to cyst formation in polycystic kidney diseases. Most of these interactions are mediated by β1-integrins, a subfamily of integrin receptors, formed by the association of the β1-chain with different α-subunits. To date, no study on α-integrin subunit distribution during the early stages of cyst development has been reported. Using immunofluorescence, we analyzed the distribution of α-integrin subunits (α1, α2, α3, α5, and α6) and basement membrane proteins in kidneys of fetuses with autosomal dominant (ADPKD) or autosomal recessive polycystic kidney disease (ARPKD). The distribution was compared with that observed in normal fetal and post-natal kidneys, and in fetal cystic dysplasia and Meckel syndrome. Marked increase in α1-integrin staining was observed in normal and cystic collecting duct cells of both polycystic diseases (PKD), compared with normal and cystic controls. The distribution of integrin subunits α2, α3, and α6 was irregular in cyst epithelial cells of PKD and cystic controls. The increased expression of the α1-subunit specifically observed in PKD collecting duct cells may be an early consequence of the genetic defect in ARPKD. In ADPKD it parallels the reported expression of polycystin, the protein product of PKD1. The irregular expression of α2, α3, and α6 integrin subunits observed in all types of cysts suggests that cell/matrix interactions are altered early and may participate in the development of cysts, perhaps by contributing to the deregulation of cell survival in cystic diseases. Received May 28, 1996; received in revised form October 2, 1996; accepted October 25, 1996     

砀山县2001年肾综合征出血热疫源地主要宿主调查分析

目的:探讨砀山县肾综合征出血热(EHF)的宿主动物,明确主要传染源种类,为预防和控制EHF的发生与流行,给制订防治对策提供科学依据。方法:根据地理景观特点选择近几年疫情较重的黄楼、关帝庙为调查点,用夹夜法进行鼠密度、种类调查,对捕获的鼠检测EHF抗原、抗体,同时对健康人群用间接免疫荧光法(IFA)检测EHF抗体。结果:室内外平均鼠密度为4.14%(63/1520),室内以小家鼠(占60.0%)、室外黑线姬鼠(占70.8%)为优势鼠种;鼠肺EHF带毒率为1.59%(1/63)、鼠血EHF抗体阳性率为7.94%(5/63),健康人群EHF抗体阳性率为3.7%(2/54)。结论:鼠类有1目2科6种鼠,另有食虫目鼠句鼠青科的臭鼠句鼠青;黑线姬鼠为砀山县EHF的主要宿主动物,健康人群血清学调查证实有隐性感染存在。     

Do house officers learn from their mistakes?*

Mistakes are inevitable in medicine. To learn how medical mistakes relate to subsequent changes in practice, we surveyed 254 internal medicine house officers. One hundred and fourteen house officers (45%) completed an anonymous questionnaire describing their most significant mistake and their response to it. Mistakes included errors in diagnosis (33%), prescribing (29%), evaluation (21%), and communication (5%) and procedural complications (11%). Patients had serious adverse outcomes in 90% of the cases, including death in 31% of cases. Only 54% of house officers discussed the mistake with their attending physicians, and only 24% told the patients or families. House officers who accepted responsibility for the mistake and discussed it were more likely to report constructive changes in practice. Residents were less likely to make constructive changes if they attributed the mistake to job overload. They were more likely to report defensive changes if they felt the institution was judgmental. Decreasing the work load and closer supervision may help prevent mistakes. To promote learning, faculty should encourage house officers to accept responsibility and to discuss their mistakes.     

Liver transplantation for chronic hepatitis B with lamivudine-resistant YMDD mutant using add-on adefovir dipivoxil plus lamivudine.

Lamivudine treatment in patients with chronic hepatitis B virus (HBV) infection may improve clinical state and suppress viral replication before liver transplantation. Emergence of lamivudine-resistant YMDD mutant is common. We report the results of liver transplantation in 16 patients with pretransplantation YMDD mutants after receiving lamivudine treatment for a median of 738 days (range, 400-1799 days). Adefovir dipivoxil (10 mg daily) was added on to lamivudine for a median of 20 days (range, 8-271 days) before (n = 11) or at (n = 5) liver transplantation, and the combination was continued indefinitely thereafter. Eight patients received additional intravenous hepatitis B immune globulin (HBIG) for a median of 24 months. Fifteen patients with known pre-adefovir HBV DNA levels had a median titer of 14,200 x 10(3) copies/mL (2 x 10(3) to 4,690,000 x 10(3) copies/mL), and 14 had HBV DNA >10(5) copies/mL. All but 1 patient remained positive for HBV DNA (by quantitative polymerase chain reaction [qPCR]) at the time of liver transplantation, and the titer was greater than10(5) copies/mL in 8 patients. The median follow-up after liver transplantation was 21.1 (range, 4.4-68.9) months. One patient (6%) died of an unrelated cause 12.2 months after transplantation, and 15 patients (94%) were alive with the original graft. All patients cleared HBV DNA and had no detectable HBV DNA by qPCR at the latest follow-up. Fourteen patients had cleared hepatitis B surface antigen (HBsAg), but 2 patients who received only adefovir dipivoxil and lamivudine without HBIG remained HBsAg positive after 7.7 and 9.5 months. Serum HBV DNA, however, was negative, and there was no biochemical or histological evidence of recurrence. Adefovir dipivoxil was well tolerated with no significant renal toxicity. In conclusion, a combination of add-on adefovir dipivoxil plus lamivudine therapy provides effective prophylaxis in patients with pretransplantation YMDD mutant that may be actively replicating. The cost effectiveness of additional passive immunoprophylaxis remains to be defined.     

RelB regulation of chemokine expression modulates local inflammation.

The resolution of acute inflammation is incompletely understood but presumably requires the elimination of both inflammatory cells and production of inflammatory cytokines. In the case of recruited bone-marrow-derived inflammatory cells such as granulocytes and macrophages, their short life span helps eliminate these cells and the cytokines they produce. By contrast, resident permanent cells such as fibroblasts require other mechanisms to stop the production of chemokines generated in response to inflammatory triggers such as lipopolysaccharide. Here we demonstrate that RelB is an important regulator of chemokine expression in fibroblasts, thereby playing a key role in the resolution of acute inflammation. Activation of normal fibroblasts by lipopolysaccharide induced a transient production of chemokines, closely followed by induction of RelB expression. However, stimulated RelB-/- fibroblasts exhibited dramatic persistent induction of seven chemokines (RANTES, MIP-1 alpha, MIP-1 beta, MIP-2, IP-10, JE/MCP-1, and KC/CINC). The persistent overexpression of chemokines correlated with increased NF- kappa B binding as well as with increased p50, p65/RelA, and I kappa B alpha expression. Transfection of RelB cDNA into RelB-deficient fibroblasts reversed the lipopolysaccharide-induced chemokine overexpression. In vivo, activated RelB-/- fibroblasts dramatically increased recruitment of granulocytes into tissues. In view of the apparent role of RelB in the resolution of acute inflammation in tissues and previous work showing a requirement for RelB in the initiation of immune responses through the differentiation of antigen-presenting cells, RelB may be an important factor regulating the transition from innate to adaptive immunity.     

Personality dimensions of sexually transmitted disease repeaters assessed with the Millon Clinical Multiaxial Inventory

OBJECTIVE: To assess the personality characteristics of patients with repeated sexually transmitted diseases (STD). METHOD: A case-control study comparing 101 STD repeaters (subjects with a lifetime history of three or more STDs) with 182 controls who had no history of STD. All subjects attended the City Department for Skin and Venereal Diseases in Belgrade (Yugoslavia) from June 1997 to April 1998. Personality characteristics was assessed by the Millon Clinical Multiaxial Inventory (MCMI). RESULTS: The analysis of MCMI test showed that STD repeaters had higher scores on narcissistic, antisocial and paranoid scales. The difference between STD repeaters and the controls was significant on antisocial, psychotic thinking and psychotic delusion scales, although scores on clinical syndromes were low for both cases and controls. Discriminant analysis showed that antisocial personality was predictive for STD repeaters. CONCLUSIONS: This study support the hypothesis that STD repeaters are different from controls in terms of their psychological characteristics. The behaviour of STD repeaters is ego-syntonic, which makes the treatment of their personality difficult and emphasizes the importance of work on primary and secondary prevention of STD.     

嗜麦芽窄食单胞菌临床株的多重耐药外排泵的研究

目的　研究嗜麦芽窄食单胞菌临床株外排泵SmeDEF的表达与耐药的关系及其表达调控。方法　琼脂稀释法检测嗜麦芽窄食单胞菌对抗生素敏感性并检测泵抑制剂的作用 ,提取临床菌的RNA进行smeD的RT PCR扩增。提取DNA进行smeT片段的PCR扩增 ,扩增产物进行序列分析。结果　随机选取的 6株嗜麦芽窄食单胞菌均有扩增产物。SmeT的N端氨基酸序列相当保守 ,smeD smeT间区测序发现耐药且泵抑制阳性株基因序列与敏感株明显不同。推测与耐药有关的突变出现在smeT的 82～ 16 5区间。结论　嗜麦芽窄食单胞菌临床株SmeDEF外排泵的表达强弱与其耐药性有关。smeDEF基因的表达可能与调控基因间区的变化有关。