Plasminogen deficiency

Summary. Plasminogen deficiency has emerged as a well‐recognized disorder in which reduced levels of plasminogen lead to the development of pseudo membranes on mucosal surfaces, with subsequent end‐organ damage of the affected tissue. Ligneous conjunctivitis is the most recognizable, well‐documented, and common presentation of the clinical syndromes associated with plasminogen deficiency, although numerous other organs have been reported to be affected. Interestingly, while plasminogen deficiency was initially believed to be related to development of venous thromboembolic disease, more recent data suggest that decreased plasminogen levels may not, in and of themselves, increase the risk of thrombosis. Two types of plasminogen deficiency have been described in the literature. Type I represents a quantitative deficiency and type II a qualitative deficiency. It appears that hypoplasminogenaemia (type I deficiency) is the type most associated with pseudomembrane disease. A variety of genetic mutations has been identified recently and is reported to lead to these disorders. These defects have been identified in diverse populations, with no specific ethnic predilection. However, this disorder may have increased prevalence in areas and communities where consanguinity is more common. Despite the fact that the characteristic lesions are now better recognized and plasminogen levels are accurately and easily measured, adequate treatment of the clinical manifestations of this disorder is lacking. For ligneous conjunctivitis, a plasminogen concentrate formulated into an ophthalmologic preparation has been found to be an effective local therapy. Unfortunately, no plasminogen concentrate is currently available commercially for either systemic or local therapy.     

Autoimmune haemolytic anaemia associated with ciprofloxacin

Summary Ciprofloxacin has become the treatment of choice for salmonella infections due to its clinical efficacy and the development of resistance to other antibiotics. It shortens the course of clinical disease and the period of excretion in enteric salmonella infections, has excellent in-vitro activity against all the bacterial pathogens that commonly cause infective diarrhoea (Wilcox & Spencer 1992) and side effects are uncommon. We report here the first case of autoimmune haemolytic anaemia (AIHA) associated with its use.     

An overview of the history, clinical practice concerns, comparative studies and strategies to optimize therapy of bypassing agents

Summary.  Despite significant advances in the treatment of haemophilia, including availability of recombinant coagulation factor replacement products and the use of prophylactic infusion regimens, the segment of haemophilic patients who develop inhibitory antibodies remain at higher risk for morbidity and mortality associated with recurrent or uncontrolled bleeding events. Bypassing agents represent the mainstay of treatment and prevention of bleeding. The most commonly used of the currently available therapeutic agents are a plasma-based therapy, factor eight inhibitor bypassing activity, vapour heated, and a recombinant therapy, NovoSeven (recombinant activated factor VIIa). A substantial body of literature exists to document efficacy and adverse event profiles for these two products. There is, however, a paucity of data arising from adequately powered prospective trials to determine optimal treatment and dosing in various clinical situations. Certain clinical circumstances, patient profiles, individual responses, or provider predilection may lead to preferential use of one of these products over the other; however, the continued presence of both agents in the therapeutic armamentarium remains critically important to this fragile population. The historical use, clinical practice concerns, published comparative studies and methods for optimization of these two bypassing agents are reviewed.     

Major Adverse Cardiac Events at Long-Term Follow-Up in Patients Treated With Single versus Multiple Stents during Single-Vessel Percutaneous Coronary Intervention

Background: Although insertion of multiple stents into a single coronary vessel during single-vessel percutaneous coronary intervention (PCI) is common, there are no data on long-term occurrence of major adverse cardiac events (MACE) in patients treated with multiple stents versus a single stent.
Methods: The incidence of MACE (death, myocardial infarction, or target vessel revascularization) during long-term follow-up was investigated in 634 patients who underwent single-vessel PCI. Of the 634 patients, 319 (50%) had a single stent, and 315 (50%) had multiple stents inserted. Stepwise Cox regression analyses were performed to identify significant independent prognostic factors for MACE.
Results: At 47-month follow-up, MACE occurred in 61 of 319 patients (19%) who had a single stent versus in 57 of 315 patients (18%) who had multiple stents (P not significant). Significant independent predictors of MACE were use of vein grafts (hazard ratio = 1.94; 95% CI, 1.24–3.03; P = 0.0038) and use of drug-eluting stents (hazard ratio = 0.49; 95% CI, 0.34–0.72; P = 0.0002).
Conclusions: At long-term follow-up of single-vessel PCI, the incidence of MACE was similar in patients with multiple or single stents inserted even after controlling for the length of stents.     

Variation in the latencies and amplitudes of N400 and NA as a function of semantic priming

The purpose of this study was to determine whether the latencies of two event-related potential (ERP) components, the NA and N400, were sensitive to semantic priming. Subjects performed a semantic judgment task, which was designed in such a way that the N400 could be examined without overlap from the P3. Priming effects on the latencies of both NA and N400 were most apparent at frontocentral sites. The amplitude of NA was not significantly affected by priming. The amplitude of N400 was smaller for primed than for unprimed words, but the effect was significant only at centroposterior sites. Current source density (CSD) analyses performed on the ERP data suggested the activation of multiple generators in the N400 time region. The ERP and CSD data were consistent with the existence of two types of N400, a frontal N400 that varies in latency as a function of semantic priming, and a posterior N400 that varies in amplitude.     

Atypical Aortic Dissection Diagnosed by Transesophageal Echocardiography

This case report describes a patient in whom a thin dissection of the proximal aorta was diagnosed by transesophageal echocardiography. Dissection was not firmly diagnosed in our patient with computed tomography, angiography, or magnetic resonance imaging. A definitive diagnosis was made prospectively using intraoperative transesophageal echocardiography by demonstrating the presence of a thin immobile dissection flap located very close to the anterior aortic wall. Color Doppler examination showed absence of flow signals in the small false lumen, suggesting that it may be clotted. At surgery the presence of dissection involving the ascending aorta, as well as a clotted false lumen were confirmed. This finding demonstrates the utility of transeophageal echocardiography in the diagnosis of aortic dissection and the ability of this test to differentiate a thin dissection from intimal thickening. (ECHOCARDIOGRAPHY, Volume 11, May 1994)