A case of neurofibromatosis type 1 with an aldosterone-producing adenoma of the adrenal.

Neurofibromatosis type 1 is a phacomatosis. Neurofibromas are the most common tumours associated with the disease, and along with other tumours, make neurofibromatosis type 1 the most common tumour predisposing syndrome in humans. Hypertension may be coincidental, but at least two specific neurofibromatosis related causes must be considered, namely neurofibromatous involvement of the renal artery and pheochromocytoma. We have described the first known case of a patient with neurofibromatosis type 1 who developed hypertension due to an aldosterone-producing adenoma of the adrenal. The question of whether this association was coincidental or due to the tumour predisposition of neurofibromatosis type 1 was debated.

[Evaluation of regression of left ventricular hypertrophy after antihypertensive therapy. Comparative echo-Doppler study of ace inhibitors and calcium antagonists]. / Valutazione della regressione della ipertrofia ventricolare sinistra dopo trattamento antipertensivo. Studio comparativo eco-Doppler tra Ace-inibitore e calcio-antagonista.

This study evaluates 33 patients suffering from mild-moderate arterial hypertension (systolic blood pressure over 160 mmHg; diastolic blood pressure over 95 mmHg) and with concomitant indications of concentric left ventricular hypertrophy at echocardiography. Twenty patients completed the trial according to the criteria established in the protocol and were found suitable for evaluation of the results at the end of the study. Ten patients were treated with 40 mg of nifedipine retard tablets, administered twice (1 x 20 mg tablet twice daily) and 10 patients were treated with a single administration of 20 mg quinapril (1 x 20 mg tablet per day). Total follow-up was performed for one year (variability 12-14 months) with evaluation of the pressure response at 1-3-6-12 months and clinical evaluation and echocardiographic control for comparison with the baseline at the end of the study. The left ventricular mass values measured at the end of the study presented a statistically significant reduction (p < 0.001) with respect to the baseline in both groups. In the patients treated with quinapril the percentage regression of left ventricular hypertrophy was 19.39%, and slightly higher (19.5%) in the patients treated with nifedipine. Finally, although the series is perhaps too limited to draw conclusions, the finding that the indicators of diastolic filling of the left ventricle improved, and, in particular, were correlated with an increase in the speed of the first fast filling phase, and thus that there was a partial improvement in left ventricular compliance, is important.

[Tricuspid endocarditis caused by Staphylococcus aureus in AIDS. Discussion of a case]. / Endocardite tricuspidale da Stafilococco aureus in AIDS. Discussione di un caso.

Right-sided staphylococcal endocarditis has been described in drug-addict, but not in association with an Acquired Immunodeficiency Syndrome. A case of a 26 years old drug-addict woman with AIDS related complex is reported. Antibiotic therapy, produced a favourable evolution on the illness. This case support the usefulness of two-dimensional echocardiography for detecting one of the possible complications associated with AIDS.

[Diagnosis of total block in the bundle of His with rhythm of idioventricular substitution]. / Diagnosi di blocco totale intra-hissiano con ritmo di sostituzione idioventricolare.

His bundle (HB) recording does not allow the recognition of third degree intrahisian block in patients with complete atrio-ventricular block (AVB) associated with idioventricular rhythm, due to the absence of pacemaker activation in the distal HB region. We have observed fixed retrograde distal HB activation in the standard HB recording of a patient with complete AVB and ventricular rhythm at a rate of 28/min. Retrograde distal HB activation (h'r) did not disappear during apical right ventricular pacing, in association with the complete absence of retrograde nodal conduction: concealed retroconduction into the proximal HB did not allow the recording of anterograde hisian deflection when the interval between h'r deflection and the subsequent sinus atriogram was shorter than 200 msec. Distal HB bipolar pacing using low energy stimulus resulted in 1:1 ventricular response and normal QRS duration in the absence of nodal retroconduction, thus proving the localization of bidirectional block within the HB. The unmasking of retrograde V-h' conduction during idioventricular rhythm was likely related to phase 4 retrograde delay in the branch ipsilateral to the site of the emergency ventricular focus and to the subsequent trans-septal activation of the other side of His-Purkinje system. Referring to arrhythmic problems after DDD pace-marker implantation the localization of complete AV blocks and retrograde conduction patterns are discussed.