PROGNOSTIC FEATURES OF TERATOMAS WITH MALIGNANT TRANSFORMATION: A CLINICOPATHOLOGICAL STUDY OF 21 CASES

Purpose

Teratomas with malignant transformation comprise up to 6% of metastatic teratomas. The prognosis of patients with these tumors can vary considerably. We delineate factors that may be related to prognosis in a cohort of men with teratoma with malignant transformation.

Materials and Methods

We analyzed pathological features, treatment, response, recurrence, time to recurrence, subsequent followup and survival for 21 patients (median age 28 years) diagnosed with teratoma with malignant transformation during a 7-year period at our institution.

Results

Malignant nongerm cell elements were present in the primary tumor in 11 cases (52%). Of 18 patients with testicular primaries 17 (94%) presented with metastatic disease. Despite aggressive treatment with surgery and chemotherapy 17 of 21 cases (81%) recurred (median time 6 months). Overall, 5 patients (24%) died of disease (median survival 23 months), 5 (24%) are alive with metastases (median followup 41 months) and 11 (52%) have no evidence of disease (median followup 50 months). Progression/recurrence was substantially greater for 2 of 2 cases with a mediastinal origin, 3 of 4 with rhabdomyosarcomatous differentiation and 5 of 6 with neural differentiation compared with the remainder of the cohort (p <0.05).

Conclusions

Teratomas with malignant transformation are usually metastatic at presentation, have a high recurrence rate and are more aggressive than teratomas without malignant transformation. Prognosis is especially poor for mediastinal teratomas with malignant transformation and for those with neural or rhabdomyosarcomatous differentiation. Complete surgical resection of residual or recurrent disease appears to offer the best chance for prolonged survival.     

Satisfaction guaranteed? What clients on methadone and buprenorphine think about their treatment

Introduction and Aims . A consumer satisfaction survey was conducted among clients receiving methadone or buprenorphine treatment for opioid dependence. The survey aimed to assess client perceptions across a number of treatment domains, including the clinic environment, service provision, clinical relationships, medication and treatment outcomes. Design and Methods . Participants were 432 clients receiving treatment at nine public clinics in New South Wales, Australia. An interviewer‐administered questionnaire was utilised, designed by the researchers. Participation was voluntary and anonymous. All participants received $10 remuneration. Results . Seventy‐eight per cent of participants were on methadone treatment. Overall satisfaction with treatment was high (mean: 3.8; very satisfied = 5). Participants were mainly satisfied with service provided by the clinic, although had concerns over the inflexibility associated with the clinic atmosphere, frequency of clinic attendance, dosing hours and lack of takeaway doses. While relationships with prescribers and case managers were rated positively, 16% and 21% of participants wanted to see their prescriber and case manager more often, respectively; 53% reported that they did not have input into their care plan. Regarding the helpfulness of case managers in assisting clients with problems experienced in identified domains of case management (e.g. drug use, physical and mental health, psychosocial supports), the mean rating was 5.2 (excellent = 10). Discussion and Conclusions . While participants reported being mainly satisfied with their treatment, results must be viewed within the context of what a consumer reasonably expects to receive from a service. The concept of ‘expectation’ and ‘relative experience’ is crucial in measuring consumer satisfaction among pharmacotherapy consumers.     

Effect of the Implantable Atrial Defibrillator on the Natural History of Atrial Fibrillation

INTRODUCTION: The purpose of our study was to evaluate the effect of repeated cardioversion with an implantable atrial defibrillator on the clinical outcome of patients with atrial fibrillation. METHODS AND RESULTS: The effects of the implantable atrial defibrillator on the total duration of atrial fibrillation, number of atrial fibrillation recurrences, and left atrial size were evaluated prospectively in 16 patients with atrial fibrillation (13 men and 3 women; mean age 58 +/- 11 years). Seven patients had no cardiovascular disease, 5 patients had hypertension, 3 patients had coronary heart disease, and 1 patient had congenital heart disease. Eight patients had paroxysmal atrial fibrillation for a mean duration of 80 +/- 61 months, and eight patients had persistent atrial fibrillation for a mean duration of 68 +/- 119 months. Except for one patient who received digoxin throughout the study, all patients received the same Class I or III antiarrhythmic agent throughout the study. The implantable atrial defibrillator successfully converted 50 (93%) of 54 spontaneous episodes of atrial fibrillation in 12 patients. During the initial 3 months of clinical follow-up, the atrial defibrillator documented 261 +/- 270 hours of atrial fibrillation compared with 126 +/- 172 hours (P = 0.01) during the subsequent 3 months. The left atrial size decreased from 4.4 +/- 0.7 cm at the time of atrial defibrillator implantation to 4.1 +/- 0.6 cm (P = 0.02) 6 months later. The number of atrial fibrillation recurrences did not change. These findings were observed in the absence of changes in drug therapy. No complications were observed. CONCLUSION: Restoration and maintenance of sinus rhythm in patients with atrial fibrillation by repeated cardioversion with an implantable atrial defibrillator was associated with a reduction in the total arrhythmia duration and a reduction in left atrial size. These results suggest that maintenance of sinus rhythm with the atrial defibrillator may reverse the remodeling process associated with atrial fibrillation.     

BULLOUS DISEASES IN MALAYSIA: EPIDEMIOLOGY AND NATURAL HISTORY

The characteristics of three primary bullous diseases, pemphigus, pemphigoid, and dermatitis herpetiformis, seen in this country, probably reflecting this region, are discussed and compared to those reported in the literature in the West. One hundred and forty-eight patients with bullous diseases were seen over a period of 15 years. The criteria for confirmation of clinical diagnosis were the findings of the direct immunofluorescent test. Pemphigus vulgaris was the commonest bullous disease. The incidence of bullous pemphigoid was highest in the Indians of Malaysian origin, and they are also more likely to develop pemphigus vulgaris than any other ethnic group. Linear IgA type formed the entire group of dermatitis herpetiformis. The granular type was not seen at all. The patients were treated with prednisolone alone or together with methotrexate or azathioprine. Dapsone alone controlled the dermatitis herpetiformis. The known association between pemphigus and other diseases with immunologic disturbances was not found in this study. The natural history of the bullous disease as seen in the follow-up patients is described. Deaths in pemphigus and pemphigoid were more due to either complications of steroid therapy or unassociated diseases than the primary disease itself. Introduction of immunofluorescence as a diagnostic tool in pemphigus and extension of this facility to other bullous diseases has led to detailed characterization of these diseases as seen in the West; however, publications in English dealing with epidemiologic aspects of bullous diseases in this region are rare.(ABSTRACT TRUNCATED AT 250 WORDS)     

Platelet adhesion to multimerin 1 in vitro: influences of platelet membrane receptors, von Willebrand factor and shear

Summary.  Background : Multimerin 1 (MMRN1) is a large, homopolymeric adhesive protein, stored in platelets and endothelium, that when released, binds to activated platelets, endothelial cells and the extracellular matrix. Objectives : The goals of our study were to determine if (i) MMRN1 supports adhesion of resting and/or activated platelets under conditions of blood flow, and (ii) if MMRN1 enhances platelet adhesion to types I and III collagen. Patients / methods : Platelet adhesion was evaluated using protein-coated microcapillaries, with or without added adhesive proteins and receptor antibodies. Platelets from healthy controls, Glanzmann thrombasthenia (GT) and severe von Willebrand factor (VWF)-deficient donors were tested. Results : MMRN1 supported the adhesion of activated, but not resting, washed platelets over a wide range of shear rates. At low shear (150 s⁻¹), this adhesion was supported by integrins αvβ3 and glycoprotein (GP) Ibα but it did not require integrins αIIbβ3 or VWF. At high shear (1500 s⁻¹), adhesion to MMRN1 was supported by β3 integrin-independent mechanisms, involving GPIbα and VWF, that did not require platelet activation when VWF was perfused over MMRN1 prior to platelets. MMRN1 bound to types I and III collagen, independent of VWF, however, its enhancing effects on platelet adhesion to collagen at high shear were VWF dependent. Conclusions : MMRN1 supports platelet adhesion by VWF-dependent and -independent mechanisms that vary by flow rate. Additionally, MMRN1 binds to, and enhances, platelet adhesion to collagen. These findings suggest that MMRN1 could function as an adhesive ligand that promotes platelet adhesion at sites of vascular injury.     

Differential Effect of Adenosine on Anterograde and Retrograde Fast Pathway Conduction in Patients with Atrioventricular Nodal Reentrant Tachycardia

Adenosine and Retrograde Fast Pathway Conduction . Introduction : Several studies have shown that the fast pathway is more responsive to adenosine than the slow pathway in patients with AV nodal reentrant tachycardia. Little information is available regarding the effect of adenosine on anterograde and retrograde fast pathway conduction.
Methods and Results : The effects of adenosine on anterograde and retrograde fast pathway conduction were evaluated in 116 patients (mean age 47 ± 16 years) with typical AV nodal reentrant tachycardia. Each patient received 12 mg of adenosine during ventricular pacing at a cycle length 20 msec longer than the fast pathway VA block cycle length and during sinus rhythm or atrial pacing at 20 msec longer than the fast pathway AV block cycle length. Anterograde block occurred in 98% of patients compared with retrograde fast pathway block in 62% of patients ( P < 0.001). Unresponsiveness of the retrograde fast pathway to adenosine was associated with a shorter AV block cycle length (374 ± 78 vs 333 ± 74 msec, P < 0.01), a shorter VA block cycle length (383 ± 121 vs 307 ± 49 msec, P < 0.001), and a shorter VA interval during tachycardia (53 ± 23 vs 41 ± 17 msec, P < 0.01).
Conclusion : Although anterograde fast pathway conduction is almost always blocked by 12 mg of adenosine, retrograde fast pathway conduction is not blocked by adenosine in 38% of patients with typical AV nodal reentrant tachycardia. This indicates that the anterograde and retrograde fast pathways may be anatomically and/or functionally distinct. Unresponsiveness of VA conduction to adenosine is not a reliable indicator of an accessory pathway.