Osteogenic Sarcoma Arising in Polyostotic Fibrous Dysplasia A Case Report

A 66 year old man with a longstanding history of polyostotic fibrous dysplasia was found to have an osteogenic sarcoma arising from the upper end of the left femur. As he was surgically unfit, he was treated with a course of external radiotherapy, with good palliation.     

ALCOHOL MISUSE IN PATIENTS ATTENDING A GENITOURINARY CLINIC

Two hundred male and 200 female attenders at a genitourinaryclinic were studied to determine the prevalence of alcohol misuse.The CAGE Questionnaire was used to estimate the presence ofprobable alcohol misuse, and the General Health Questionnaire(GHQ) was used to assess psychiatric morbidity. Over 18% ofmen and over 12% of women were found to be alcohol misusersas measured by the CAGE Questionnaire. Their characteristicsare reported, and the implications of the findings considered.     

Ventricular Tachycardia with Alternating Cycle Lengths: Self-Entrainment of Parasystolic Rhythm?

A patient had ventricular tachycardia with cycle lengths consistently varying between 400 and 490 ms. Arrhythmias with a similar electrocardiographic pattern had been thought to result from the regular activity of an automatic pacemaker with either 3:2 exit block of the Wenckebach type or alternation in exit conduction times. However, the findings in our patient could also be explained by postulating the existence of modulated ventricular parasystole having 3:2 exit block or with alternating (short-long) exit conduction times. In either case, the action potentials elicited by the parasystole must have reached (with variable delays) the ventricular tissue beyond the zone of protection to produce electrotonic depolarizations which, in turn, were reflected back to the ectopic focus itself. This resulted in runs of 2:3 or 2:2 "self-entrainment" of the parasystolic pacemaker. Similar phenomena have been produced in biologic and mathematical models of modulated parasystole.     

DISSEMINATED AMERICAN CUTANEOUS LEISHMANIASIS

Background. While studying cutaneous leishmaniasis in the central part of western Venezuela, we found four cases of disseminated American cutaneous leishmaniasis, three from the Lara State and one from Portuguese State. Methods. A clinical history was taken for each of these patients, followed by microscopic examination of the Giemsa-stained smears from their cutaneous lesions and by a Montenegro skin test. Serum from a skin lesion were grown in Novy-MacNeal-Nicolle medium (nnn). Hamsters were inoculated with suspension of tissues taken from the patient's lesions. Biopsies were taken for histopathologic examination. Isolates from cultures on nnn medium and from hamsters were subcultured in Schneider's medium for parasite identification, using molecular techniques. Treatment with injections of N-methyl glucamine antimonate, 25 mg/kg/day was precribed for each patient for 20 consecutive days and, after a week of rest, a second course of injections was administered. Results. Patients had disseminated papular, ulcerous, nodular, and ulceronodular lesions on the skin. Smears of the skin lesions from all of the patients showed abundant amastigotes within histiocytes or free in the tissues. The skin test was negative in two patients. On histopathologic examination of skin lesions, mainly numerous vacuolated histiocytes filled with amastigotes were observed. Isolates from all the patients were identified as Leishmania venezuelensis. One of the patients healed after treatment with N-methyl glucamine antimonate. The others were resistant to this therapy. Conclusions. Diffuse cutaneous leishmaniasis can be caused also by Leishmania venezuelensis. Patients with nodular lesions who presented a negative Montenegro skin test were more resistant to treatment with specific pentavalent antimonials.     

NEW FINDINGS IN APPARENT MINERALOCORTICOID EXCESS

We report two female siblings (ages 4 and 9 years) and one 8-year-old male with the syndrome of apparent mineralocorticoid excess (AME) presenting with low renin hypertension and hypoaldosteronism. The deficiency of 11β-hydroxysteroid dehydrogenase results in a defect of the peripheral metabolism of Cortisol (F) to cortisone (E). As a result, the serum Cortisol half-life (T½) is prolonged, ACTH is suppressed, and serum F is normal. The specific diagnosis of the disorder was made by the decreased ratio of the urinary metabolites of E (tetrahydrocortisone, THE) and F (tetrahydrocortisol, THF). Continuous i.v. hydrocortisone administration caused an increase in blood pressure and decrease in serum potassium demonstrating the abnormal mineralocorticoid activity of Cortisol in these patients. Addition of spironolactone resulted in a decrease in blood pressure, rise in serum potassium and a gradual increase in plasma renin activity. These studies suggest that an abnormality in Cortisol action or metabolism results in Cortisol behaving as a potent mineralocorticoid and causing the syndrome of AME.     

Ablation of Ventricular Tachycardia Associated with Tetralogy of Fallot: Demonstration of Bidirectional Block

Ablation of VT in Tetralogy of Fallot. Introduction : Ventricular tachycardia is commonly seen in patients following surgical repair for tetralogy of Fallot. The technique of ablation for this arrhythmia is not well defined.
Methods and Results : In two patients with ventricular tachycardia following surgical repair of tetralogy of Fallot, the traditional indicators for a site for ventricular tachycardia ablation did not yield cure. Based on careful mapping, the circuit was found to involve the isthmus between the outflow tract patch and the tricuspid annulus; linear radiofrequency lesions across this isthmus resulted in cure of ventricular tachycardia. Not only was the tachycardia no longer inducible, but bidirectional block at the line of ablation confirmed interruption of the reentrant circuit.
Conclusion : A linear radiofrequency lesion was effective in eliminating ventricular tachycardia in both patients. The demonstration of bidirectional block confirms a cure independent of inducibility of ventricular tachycardia.