A 61-year-old man developed a pyrescia accompanied by a massive intravascular hemolysis after abdominal surgery (Whipple's operation) of a pancreatic adenocarcinoma. Abdominal ultrasound and the abdominal CT-scan showed marked aerobilia and multiple liver abscesses. Laboratory tests demonstrated the presence of the Thomsen-Friedenreich cryptantigen (TCA) on the membranes of the patient's erythrocytes. The enzymatic cleavage of N-acetyl-neuraminic acid usually covering the TCA may lead to a life threatening intravascular hemolysis. Since Clostridial bacteriae typically synthesize neuraminidase, the presumptive diagnosis of Clostridial sepsis complicated by massive hemolysis was made. Immediate antibiotic therapy including penicillin G and metronidazole stopped hemolysis within a few hours and the patient servived. On the following day, microbiological examination identified Clostridium perfringens in the patient's blood cultures. Clostrial sepsis should be suspected in patients with underlying infections and/or malignant diseases, particularly of the gastrointestinal or genitourinary tract, who present with septic shock and acute intravascular hemolysis. Whereas microbiological specification of the organism is time consuming, the relatively simple agglutination test with anti-TCA peanut lectin can provide a rapid presumptive diagnosis. The immediate onset of an appropriate antimicrobial therapy is of central importance and might be life-saving.

In its acute phase the diagnosis of TSS is often uncertain. The initial symptoms are nonspecific and numerous conditions need to be considered in the differential diagnosis. The diagnosis can be confirmed, if at all, only in the convalescent phase by the skin desquamation or a rise in anti-TSST-1 antibody titre. A search for a focus of infection is essential for differentiation from a non-menstrual TSS, even if there is as association with menstruation.

The central venous-arterial differences in neutrophil production of reactive oxygen species support the concept of compartmentalization of activated neutrophils from the systemic to the pulmonary compartment.

Objective-To assess the efficacy of early accelerated dose tissue plasminogen activator on in-hospital patency of the infarct related artery in patients with inferior myocardial infarction with and without right ventricular involvement. Design-Single centre prospective assessment before discharge of infarct related vessel patency after early thrombolysis. Setting-Tertiary cardiac referral centre at a university hospital. Patients and methods-90 consecutive unselected patients with acute myocardial infarction, of whom 35 (39%) had electrocardiographic evidence ofright ventricular involvement (ST segment elevation greater than 0.1 mV in right precordial lead V4R), were studied. All patients received accelerated dose tissue plasminogen activator 100 mg within six hours from the onset of symptoms and had control angiography before discharge. Main outcome measures-Infarct related coronary artery patency using the Thrombolysis in Myocardial Infarction (TIMI) grading system before discharge. Incidence of prolonged systemic hypotension, sinus bradycardia, complete atrioventricular block, and ventricular tachyarrhythmia during early hospitalisation.Results-Despite aspirin and bolus heparinisation before thrombolysis and high dose heparinisation thereafter for at least 48 hours the infarct related artery was more likely to be occluded (TIMI 0 or 1 flow) in patients with right ventricular involvement than in those without (69 v 29%, P < 0.001), as shown by control angiography performed a mean of 12-8 days after thrombolysis. These findings may be explained, at least in part, by predominant involvement of the proximal right coronary artery (66 v 31%, P < 0.05) and a low cardiac output syndrome, being indirectly reflected by a high incidence of prolonged hypotension (26 v 7%, P = 0.02), bradycardia (34 v 14%, P = 0.03), and complete atrioventricular block (37 v 5%, P = 0-0001). Conclusion-Primary angioplasty should be considered as the treatment of choice in patients with acute inferior infarction with right ventricular involvement because of the high failure rate of thrombolysis.

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Analysis of ETCO2 allows monitoring of the efficacy of thrombolysis and may reflect recurrent embolism. Thus, on the basis of this small study, analysis of ETCO2 appears to be useful for noninvasive monitoring in mechanically ventilated patients with massive pulmonary embolism.

The congenital long QT syndrome (LQTS) is characterized by a prolonged QT interval on the surface electrocardiogram and an increased risk of recurrent syncope and sudden cardiac death. Mutations in seven genes have been identified as the molecular basis of LQTS. beta-blockers are the treatment of choice to reduce cardiac symptoms. However, long-term follow-up of genotyped families with LQTS has been rarely reported. We have clinically followed a four-generation family with LQTS being treated with beta-blocker therapy over a period of 23 years. Seven family members were carriers of two amino acid alterations in cis (V254M-V417M) in the cardiac potassium channel gene KCNQ1. Voltage-clamp recordings of mutant KCNQ1 protein in Xenopus oocytes showed that only the V254M mutation reduced the IKs current and that the effect of the V417M variant was negligible. The family exhibited the complete clinical spectrum of the disease, from asymptomatic patients to victims of sudden death before beta-blocker therapy. There was no significant reduction in QTc (556 +/- 40 ms(1/2) before therapy, 494 +/- 20 ms(1/2) during 17 years of treatment; n = 5 individuals). Of nine family members, one female died suddenly before treatment, three females of the second generation were asymptomatic, and four individuals of the third and fourth generation were symptomatic. All mutation carriers were treated with beta-blockers and remained asymptomatic for a follow-up up to 23 years. Long-term follow-up of a LQT1 family with a common mutation (V254M) being on beta-blocker therapy was effective and safe. This study underscores the importance of long-term follow-up in families with specific LQT mutations to provide valuable information for clinicians for an appropriate antiarrhythmic treatment.