The chameleon rash: a review of the polyphenotypic dermatoses of dermatomyositis.

Dermatomyositis (DM) is an autoimmune connective tissue disease that is included in the idiopathic inflammatory myopathies. Cutaneous manifestations are a prominent part of the condition: some skin signs in DM are common to most patients, while other signs are encountered infrequently. A number of features are pathognomic for DM. The demonstration of myositis-specific antibodies (MSAs) in DM has extended the ability to define phenotypic subgroups. It appears that the presence of certain MSAs confers susceptibility to specific clinical features, an association which reveals a serotype-phenotype relationship. In this review article we have provided a detailed summary of common and under-recognized cutaneous manifestations of DM.

Congenital heart block: clinical features and therapeutic approaches.

Isolated congenital heart block is strongly associated with anti-Ro antibodies. It occurs in 2% of anti-Ro antibody positive pregnancies with a recurrence rate of 17-19%. Mortality is high in the first year of life (12-41%) and is predominantly due to dilated cardiomyopathy. A prolonged QTc occurs in 15-22% of cases and minor structural defects such as atrial septal defects and patent arterial ducts are well recognized. The 'mechanical' PR interval can now be measured in utero allowing for the detection of first-degree heart block. Both first and second-degree heart block detected in utero respond to therapy with fluorinated steroids. Complete congenital heart block is not reversible. Progression from a normal PR interval to complete heart block can occur within a week. IVIG is under investigation for the prevention of recurrence of congenital heart block, while dexamethasone should not be used for this purpose due to unacceptable toxicity. Data on the use of fluorinated steroids for established complete heart block is conflicting, although their use in cases where there is evidence of hydrops, poor ventricular function or both is not controversial.

Atherosclerosis and autoimmunity.

Novel risk factors for the progression of atherosclerosis such as C-reactive protein (CRP) and adhesion molecules have stimulated much recent interest in the role of inflammation in atherosclerotic disease. There is also evidence emerging that autoimmunity may have a role in the pathogenesis of atherosclerosis. In this article we explore the evidence for the role of autoimmunity in human atherosclerosis, both in the general population and in the context of the antiphospholipid syndrome. In particular we will focus on several autoantigens, review the evidence for their role in the process of atherosclerosis and the nature of the immune responses.

Absence of conduction defects in the electrocardiograms [correction of echocardiograms] of mothers with children with congenital complete heart block.

OBJECTIVE: To investigate the association between complete congenital heart block (CCHB) in the fetus and adult disease. METHODS: We prospectively studied 111 consecutive patients with systemic lupus erythematosus (SLE) or a positive extractable nuclear antigen (Ro, La, or RNP) attending a connective tissue disease (CTD) clinic and 19 patients who had had children with CCHB. Electrocardiographs were recorded on all patients and subsequently analyzed blindly. RESULTS: There was a significantly shorter PR interval in the mothers of children with CCHB compared to patients attending the CTD clinic (p < 0.02). There was no significant difference in PR interval between Ro positive and Ro negative patients in the CTD clinic. There were no significant differences in QRS or QTc duration between mothers with CCHB children and Ro positive or Ro negative patients attending the CTD clinic. The shorter PR interval in the mothers with CCHB children may be explained by their significantly younger age compared to patients in the CTD clinic (p < 0.02, 95% CI) and the lower incidence of CTD. CONCLUSION: We were unable to confirm the association between anti-Ro antibodies and cardiac conduction defects in adults. This supports theories that CCHB is due to vulnerability peculiar to the fetal heart during a particular stage of development between 16 and 30 weeks of gestation.

The road to success with a mentor.

Mentoring is a valuable career development tool used to build nursing leadership skills. Our present nursing leaders must consider it their responsibility to mentor the novice leader of the future, just as they may have been mentored. During the mentoring process, the mentor will use the roles of teacher, counselor, intervenor, and sponsor to develop the protégé. The mentor will facilitate the development of independence, self-confidence, job satisfaction, upward mobility, decision-making skills, and problem-solving skills in the protégé. During this process the mentor and protégé will move through three developmental phases. These phases include the first phase of recognition and development, the second phase of emerging protégé independence, and the final phase of letting go. If the "fit" is right, the protégé will experience the many positive outcomes. If the "fit" is not quite right, then the movement through the phases will be incomplete and the protégé may not develop independence. The protégé and the mentor may also experience a number of other negative outcomes, such as feelings of being over pressured or let down.

Effects of position of chest drainage tube on volume drained and pressure.

BACKGROUND: The nursing practice of avoiding dependent loops in the tubing of chest drainage systems because such loops may impede drainage and alter the intrapleural pressure is not research based. OBJECTIVES: To determine if the volume of fluid drained and pressure vary when the chest drainage tubing is straight, coiled, has a dependent loop, or has a dependent loop that is periodically lifted and drained. METHODS: A repeated-measures design was used. For each tubing position, 500 mL of fluid was infused into the pleural space of 8 adult pigs during 45 minutes. The volume of fluid drained and the pressure at 2 locations within the drainage tubing were measured for 1 hour. RESULTS: After 60 minutes, significantly less fluid (least significant difference test, P = .03) was drained with the dependent-loop tubing position (65 mL) than with the other 3 positions. However, the amount of fluid drained was not significantly different among the lift and drain (250 mL), coiled (301 mL), or straight (337 mL) tubing positions. Throughout the entire study, pressure at the connection between the chest tube and the drainage tube was significantly higher (least significant difference test, P = .003) for the dependent loop with and without periodic lifting and draining. CONCLUSIONS: Straight and coiled tube positions are optimal for draining fluid from the pleural space. If a dependent loop cannot be avoided, lifting and draining it every 15 minutes will maintain adequate drainage.

Rapid detection of anticardiolipin antibodies.

A rapid screening method for the detection of antiphospholipid antibodies is described. Dense, red dyed polystyrene beads coated with cardiolipin were incubated with test sera for a short period of time, then added to a microtube containing anti-human IgG in a gel provided within a pre-cast card (DiaMed ID Microtyping System). The card was centrifuged at 150g for 5 min and then examined for movement of the beads through the gel. Beads without bound antibody travelled through the gel and formed a pellet on the bottom of the tube. Anti-human IgG within the gel matrix impeded cardiolipin-coated beads when antiphospholipid antibodies bound to the beads. Positivity was indicated by the formation of a layer of beads on the top of the gel matrix. Prospective analysis of 103 samples for the presence of antiphospholipid antibodies by flow cytometry and the gel-card technique showed good correlation between the two methods. All samples found to be positive by flow cytometry (23 of 103) were identified as positive by the gel-card technique. Two samples were identified as positive by the gel-card method but negative by flow cytometry. The technique is simple to perform and should prove useful as a rapid screening method for the detection of antiphospholipid antibodies.

Determining outcomes of a lower extremity vascular reconstruction program.

Demonstrating positive outcomes after surgical intervention is an important aspect for the patient and payor in today's health care setting. A lower extremity arterial reconstruction program was established and has shown a decrease in length of stay. Additional long-term outcome measures related to graft patency surveillance, readmission rate and related cause, and functional health status are being implemented.

Platelet activation by a novel solid-phase agonist: effects of VWF immobilized on polystyrene beads.

The interaction between platelets stirred in suspension and VWF immobilized on polystyrene beads was studied. Platelets aggregated and released ATP in response to stirring with VWF beads. Closer examination of the interaction using transmission electron microscopy revealed that the platelets did not simply aggregate with one another but initially adhered to the beads and spread. Platelets in suspension then bound to the bead-adherent platelets forming layers of platelets associated with each bead. The VWF bead-induced platelet activation was completely inhibited by addition of monoclonal antibody (mAb) to GPIb or GPIIb/IIIa. In addition, the activation response was inhibited in the presence of aspirin, indomethacin or the thromboxane receptor antagonist BM13.177, demonstrating a dependence on an intact cyclo-oxygenase pathway. Platelet function studies were carried out on 30 patients with a history of mild bleeding using conventional optical aggregation and VWF bead-induced platelet activation. 12 patients were abnormal by conventional optical aggregometry, whereas 27 patients showed depressed ATP release in response to VWF beads. The results suggest that easily-bruised patients may have a platelet function defect rather than a vascular-based abnormality and that VWF bead-induced platelet activation is a more sensitive test for detecting platelet dysfunction.

Antiphospholipid antibody-dependent C5b-9 formation.

The relationship between the presence of antiphospholipid antibodies (APA) and the production of the terminal membrane attack complex (MAC) of complement (C5b-9) was studied. Serum samples from known high positive APA patients induced platelet activation and destruction which was inhibited by heat-inactivation of the sera. The response was restored if the heat-inactivated APA-positive sera were supplemented with normal sera. Adsorption of the APA-positive sera with phospholipid (PL)-coated polystyrene beads inhibited platelet destruction. Addition of monoclonal antibody (mAb) to C5b-9 (aE11) also inhibited platelet destruction, suggesting that the APA-dependent platelet destruction might be complement-mediated. Purified APA, in the presence of normal serum, induced C5b-9 formation and binding to PL-coated beads in a dose-dependent manner as detected by flow cytometry. Prospective analysis of 200 serum samples for C5b-9 production showed that all sera testing negative for the presence of APA also tested negative for C5b-9 production. All sera with high levels of IgG binding to PL (GPL) showed evidence of C5b-9 production. Sera with low or moderate GPL values showed varying levels of C5b-9 production. These data suggest that complement may play a key role in APA-dependent platelet activation, in vivo.

vWf inhibitor detection by competitive ELISA.

An inhibitor to von Willebrand factor (vWf) was detected in the plasma from two patients with histories of mild bleeding and one patient with a severe deficiency in the Factor VIII complex using a competitive enzyme-linked immunosorbent assay (ELISA) procedure. IgG antibodies from the patients' plasmas were shown to bind to vWf immobilised on polystyrene beads by flow cytometry. The inhibitor also potentiated a recently described platelet function assay based on stirring vWf immobilised on polystyrene beads with platelet rich plasma (PRP). Upon addition of mAb IV.3, potentiation of vWf bead-induced platelet activation was lost indicating that the enhancement of platelet activation was Fc receptor-dependent. Since the ELISA described can be used to quantitate vWf and to detect inhibitors to vWf in plasma samples, the method should prove useful in differentiating acquired vWd from congenital vWd.

Positioning of chest tubes: effects on pressure and drainage.

BACKGROUND: Maintaining a chest drainage tube in a position that is free of dependent loops, as is commonly recommended, can be very difficult. Is there a beneficial effect on the patient's outcome when the drainage tubing is free of dependent loops? OBJECTIVE: The purpose of this study was to determine, under controlled laboratory conditions, (1) what are the differences in drainage with tubing in straight, coiled, or dependent-loop (with and without periodic lifting) positions and (2) what are the differences in pressure with each of the four tubing conditions? METHODS: In laboratory simulations, pressure and drainage were observed in a chest tube drainage system that was connected to a glass bottle simulating the lung. Pressure and drainage were measured for 1 hour with the drainage tubing placed in straight, coiled, and dependent-loop positions. For the periodic lifting condition, the dependent loop was lifted and drained every 15 minutes. RESULTS: We found no differences in pressure or drainage between straight and coiled positions of the drainage tubing. However, with the dependent-loop position, pressure at the "lung" side increased from about -18 cm H2O to as high as +8 cm H2O. Drainage dropped to zero without tube lifting. When the tube was lifted and drained every 15 minutes, there was no difference in drainage with the tubing in the straight or coiled positions. CONCLUSION: Findings support recommendations to maintain tubing free of dependent loops by placing tubing in straight or coiled positions. Frequently lifting and draining a dependent loop will provide the same total drainage amount as maintaining the tubing in a straight or coiled position, but pressures may be altered sufficiently within the tube to exceed recommended levels.

Heparin-induced thrombocytopenia: an improved method of detection based on lumi-aggregometry.

Heparin-induced thrombocytopenia (HIT) is a recognized complication of heparin administration. Early detection of this syndrome is essential in the prevention of immune-mediated thromboembolic sequelae. The 14C-serotonin release assay (SRA) has been used in reference laboratories to identify sera from patients on heparin therapy capable of inducing platelet dense granule release. In an attempt to improve existing methodologies, we employed luminographic detection of platelet-dense granule ATP release as an endpoint of HIT antibody-mediated platelet activation. Sera tested included 10 SRA confirmed positive and five SRA confirmed negative samples (to establish the assay), five samples from patients with thrombocytopenia not on heparin therapy and 34 patients suspected of HIT syndrome. All SRA confirmed positive sera (n = 19) were positive by the luminographic procedure. 24/26 SRA confirmed negative sera and five sera from thrombocytopenic patients not on heparin therapy were negative using luminography. Two of four sera yielding equivocal SRA results were found to be positive by the luminographic technique. The data suggest that the use of a lumiaggregometer in the coagulation laboratory to detect HIT antibody-induced platelet activation is a reliable alternative to the SRA. The luminographic procedure is both rapid and sensitive, and does not require the use of biohazardous radio-isotopes.

Binding of cardiolipin to polystyrene beads: evidence for a lamellar phase orientation.

The association of cardiolipin with polystyrene beads was studied using 31P-NMR and electron microscopy. In the presence and absence of fetal calf serum, cardiolipin appeared to bind to the polystyrene beads in lamellar phase as assessed by 31P-NMR imaging. Electron microscopic analysis revealed an even coating of phospholipid about the beads with extensive micelle binding. Cardiolipin-coated beads challenged with ACA-positive sera followed by immunogold indicated antibody bound to micelles associated with the bead. Studies conducted with ACA IgG purified from patient sera indicated that some ACA bound to CL beads in the absence of a source of ACA cofactor (i.e. gelatin-blocked beads), some ACA required beta 2-GPI for binding (i.e. no binding in the presence of beta 2-GPI-depleted plasma), whereas other ACA which showed negligible binding with gelatin-blocked beads, showed enhanced binding in the presence of beta 2-GPI-depleted plasma. The data indicate that: (1) cardiolipin binds to polystyrene beads in lamellar phase, (2) ACA bind to phospholipid micelles bound directly to the polystyrene beads, and (3) ACA differ between individuals displaying varying phospholipid and phospholipid/cofactor substrate specificities.

Refining chest tube management: analysis of the state of practice.

Critical care nurses routinely care for patients who require chest tube management. To obtain the best patient outcome, critical care nurses develop standards of practice from research derived recommendations. Although there are several studies recommending chest tube management practices, there is limited research in some areas of chest tube management. The authors analyze the body of research and recommend clinical practice changes and timely research projects on chest tube management.

Evaluation of hemostatic activity of desamino-D-arginine vasopressin (DDAVP) in uremic rats.

DDAVP, an analog of vasopressin, has been shown to have hemostatic activity. Although it has been used clinically to control bleeding, there have been very few attempts to characterize this agent in experimental animals. We describe a new animal model which is fast, reliable and inexpensive, and which may be used to screen novel analogs of the peptide. Under sodium pentobarbital anesthesia, rats were bilaterally nephrectomized and implanted with indwelling intravenous cannulae. The next day they were re-anesthetized, a standardized cut was made in the tail and the tail was allowed to bleed into warm isotonic saline (25 ml). After 10 min., the tail was removed from the saline and the blood loss was measured either by laser nephelometry or by colorimetric analysis. DDAVP was then injected intravenously and the rat was allowed to rest quietly for 30 min., after which time a second incision was made in the tail and blood loss again measured for 10 min. Unlike bleeding time which was highly variable, blood loss proved to be a reliable index of the hemostatic activity. Thus we were able to demonstrate that DDAVP reduced blood loss in the uremic rats, whereas it was without effect in intact rats.