Expanding the spectrum of Gorham Stout disease exploring a single center pediatric case series.

Gorham-Stout Disease (GSD), also named vanishing bone disease, is an ultrarare condition characterized by progressive osteolysis with intraosseous lymphatic vessel proliferation and bone cortical loss. So far, about 300 cases have been reported. It may occur at any age but more commonly affects children and young adults. The aim of this study is to retrospectively review our internal patient series and to hypothesize a diagnostic-therapeutic protocol for earlier diagnosis and treatment. Clinical datasets from our center were examined to identify all GSD patients for collection and analysis. We identified 9 pediatric cases and performed a retrospective case-series review to examine and document both diagnosis and treatment. We found that delay in diagnosis after first symptoms played a critical role in determining morbidity and that multidisciplinary care is key for proper diagnosis and treatment. Our study provides additional insight to improve the critical challenge of early diagnosis and highlights a multidisciplinary treatment approach for the most appropriate management of patients with rare GSD disease. Although GSD is an ultrarare disease, physicians should keep in mind the main clinical features since neglected cases may result in potentially fatal complications.

Glanular Venous Malformation in Pediatric Age: An Uncommon Vascular Disorder.

Glanular venous malformations are uncommon in pediatric patients. The diagnosis can be easily achieved by observation, even if color Doppler ultrasound is useful for a better characterization. Abdomino-pelvic MRI is necessary to assess the extension of complex lesions and check for associated anomalies. Several therapeutic options are reported in literature. We report 3 paediatric cases successfully treated by surgery with no complications and functional sequelae. Cosmetic results were satisfactory, with minimal surgical scarring. In our opinion, surgery for small glanular venous malformations is indicated within puberty to prevent traumatic bleeding and psychological impact.

Electrochemotherapy of unresectable cutaneous tumours with reduced dosages of intravenous bleomycin: analysis of 57 patients from the International Network for Sharing Practices of Electrochemotherapy registry.

BACKGROUND: Electrochemotherapy (ECT) is currently used to treat unresectable superficial tumours of different histotypes through the combination of cytotoxic chemotherapy and local application of electric pulses. In 2006, a collaborative project defined the ESOPE (European Standard Operating Procedures of Electrochemotherapy) guidelines to standardize the procedure. The International Network for Sharing Practices of Electrochemotherapy (InspECT) aims to refine the ESOPE and improve clinical practice. Limiting patient exposure to systemic chemotherapy would be advisable to ameliorate ECT safety profile. OBJECTIVE: The aim of this study was to evaluate the efficacy and toxicity of ECT with reduced chemotherapy dosages. METHODS: In a retrospective analysis of a prospectively maintained database (InspECT registry), we evaluated the outcome of patients who received ECT with reduced dosages of bleomycin (7500, 10 000 or 13 500 IU/m2 , instead of the standard dose of 15 000 IU/m2 ). Tumour response in melanoma patients was compared with melanoma patients of the InspECT registry who received the standard dose of bleomycin. RESULTS: We identified 57 patients with 147 tumours (melanoma, 38.6%; squamous cell carcinoma, 22.8%; basal cell carcinoma, 17.5%; breast cancer 7%; Kaposi sarcoma 7%; other histotypes, 7.1%). Per-tumour complete response (CR) rate at 60 days was 70.1% (partial, 16.3%); per-patient CR was 57.9% (partial, 21.1%). Local pain was the most frequently reported side-effect (n = 22 patients [39%]), mostly mild; two patients experienced flu-like symptoms, one patient nausea. We observed the same CR rate (55%) in patients with melanoma treated by reduced or conventional bleomycin dosages (P = 1.00). CONCLUSIONS: Electrochemotherapy performed with reduced bleomycin dosages could be as effective as with currently recommended dose. Patients with impaired renal function or candidate to multiple ECT cycles could benefit from a reduced dose protocol. Our findings need prospective confirmation before being adopted in clinical practice.

Electrochemotherapy in the treatment of metastatic malignant melanoma: a prospective cohort study by InspECT.

BACKGROUND: (ECT) is an effective local treatment for cutaneous metastasis. Treatment involves the administration of chemotherapeutic drugs followed by delivery of electrical pulses to the tumour. OBJECTIVES: To investigate the effectiveness of ECT in cutaneous metastases of melanoma and to identify factors that affect (beneficially or adversely) the outcome. METHODS: Thirteen cancer centres in the International Network for Sharing Practices on Electrochemotherapy consecutively and prospectively uploaded data to a common database. ECT consisted of intratumoral or intravenous injection of bleomycin, followed by application of electric pulses under local or general anaesthesia. RESULTS: In total, 151 patients with metastatic melanoma were identified from the database, 114 of whom had follow-up data of 60 days or more. Eighty-four of these patients (74%) experienced an overall response (OR = complete response + partial response). Overall, 394 lesions were treated, of which 306 (78%) showed OR, with 229 showing complete response (58%). In multivariate analysis, factors positively associated with overall response were coverage of deep margins, absence of visceral metastases, presence of lymphoedema and treatment of nonirradiated areas. Factors significantly associated with complete response to ECT treatment were coverage of deep margins, previous irradiation of the treated area and tumour size (< 3 cm). One-year overall survival in this cohort of patients was 67% (95% confidence interval 57-77%), while melanoma-specific survival was 74% (95% confidence interval 64-84%). No serious adverse events were reported, and the treatment was in general very well tolerated. CONCLUSIONS: ECT is a highly effective local treatment for melanoma metastases in the skin, with no severe adverse effects noted in this study. In the presence of certain clinical factors, ECT may be considered for local tumour control as an alternative to established local treatments, or as an adjunct to systemic treatments.

Treatment efficacy with electrochemotherapy: A multi-institutional prospective observational study on 376 patients with superficial tumors.

BACKGROUND: Cutaneous metastases represent a therapeutic challenge. An increasing body of experience suggests that electrochemotherapy (ECT) provides effective tumor control, although its evidence basis should be strengthened. METHODS: This prospective, multicenter, observational study enrolled patients with superficial metastases, who underwent ECT at 10 centers between 2008 and 2013. Outcomes included adherence to European Standard Operating Procedures of ECT (ESOPE), tumor response, local progression-free survival (LPFS), toxicity and patient-reported outcomes (PROs, EORTC QLQ-C30 plus an 8-item questionnaire). RESULTS: We enrolled 376 eligible patients. Tumor histotype distribution was as follows: melanoma, 56%; squamous cell carcinoma, 11%; Kaposi sarcoma, 11%; breast carcinoma, 8%; basal cell carcinoma, 6%; soft tissue sarcomas, 3%; others, 5%. We registered 1304 target tumors (median size 1 cm). Treatment adhered to ESOPE in 88% of patients as to the route of drug administration, and in 70% as to electrode application. The procedure was mainly performed under sedation (64.6%) and by using intravenous chemotherapy (93.4%). Tumor response rate at 60 days was 88% (complete, 50%). Small tumor size predicted complete response achievement (OR 2.24, p = 0.003), higher LPFS (HR 0.68, p = 0.004) and improved PROs (Global Health Status, p < 0.001; wound bleeding, p < 0.001; healing, p = 0.002; and aesthetics, p < 0.001). Skin toxicity (grade ≥3, 7.8%) was lower in patients with tumors <2 cm (p≤0.001). One-year LPFS was 73.7% (95%CI 68.4-78.3). CONCLUSIONS: ECT represents a valuable skin-directed therapy across a range of malignancies. The most frequently applied treatment modality is intravenous chemotherapy under sedation. Small tumor size predicts durable tumor control, fewer side-effects and better PROs.

Efficacy of electrochemotherapy in ulcerated basal cell carcinoma.

Basal cell carcinoma is the most common cutaneous malignant tumor, accounting for up to 80% of non melanoma skin cancers. Surgery, radiotherapy and chemotherapy have been for long time the main options for its treatment. Electrochemotherapy (ECT) is a novel local treatment successfully used in primary skin tumors. We report a case of a man affected by ulcerated basal cell carcinoma treated with ECT. In our case ECT was successful in the management of extensive basal cell carcinoma in clinical conditions whereas other approaches, would have been dangerous and inappropriate. To our knowledge, ECT must be considered as an alternative of traditional techniques when they are contraindicated in relation to the appearance of the lesions or the patient medical history.

["The memory effect" in healthy heart and transplanted heart. "Cor se ipse alit, se ipse movet, se ipse reget"]. / "L'effetto memoria" nel cuore sano e nel cuore trapiantato. "Cor se ipse alit, se ipse movet, se ipse reget".

The heart's memory, interpreted as information residue between a cardiac cycle and the subsequent one, is a very definite phenomenon (short- term memory) both in healthy subjects and in heart transplanted recipients. This "memory effect", we have observed by the autocorrelation and by the spectrum analysis of the values expressing the heartbeat acceleration variability (tachogram 24 h). The "memory effect" cannot be absolutely put down in heart transplanted recipients to interference or to the activity of the central and/or peripheral nervous system, because the heart transplanted is a denervated heart by definition. Moreover, an immediate regulating effect of hormonic factors in the short-term heartbeat regulation is not completely plausible. As already known, the heart transplanted responds to the emergency situation autonomously and autochthonously in proportion to the signals reaching it directly from the circle (vascular resistance, blood pressure, etc.). This implies the existence of functional memory that is likely to be inborn in the conduction system of the heart. Looking at the results of our research we can conclude: "cor se ipse alit, se ipse movet, se ipse reget" (it autonomously models itself to all the different modifications in the circle, it feeds itself as the organ propelling blood circulation and, if necessary, it carries on its kinetic activity autonomously).

Pretreatment with verapamil in patients with persistent or chronic atrial fibrillation who underwent electrical cardioversion.

OBJECTIVES: To evaluate, in a prospective and randomized fashion, the efficacy of a pretreatment with verapamil (V) in reducing recurrences of atrial fibrillation (AF) after electrical cardioversion (C). BACKGROUND: The increased vulnerability for AF recurrence is probably due to AF-induced changes in the electrophysiologic properties of the atria. This electrical remodeling seems to be due to intracellular calcium overload. METHODS: One hundred seven patients with persistent or chronic AF underwent external and/or internal C. All patients received oral propafenone (P) (900 mg/day) three days before and during the entire period of follow-up (three months). In the first group, patients received only the P. In the second group, in adjunct to P, oral V (240 mg/day) was initiated three days before C and continued during the follow-up. Finally, in the third group, oral V was administered three days before and continued only for three days after electrical C. RESULTS: During the three months of follow-up, 23 patients (23.7%) had AF recurrence. Mantel-Haenszel cumulative chi-square reached a significant level only when comparing AF free survival curves of group I versus group II and group III (chi-square = 5.2 and 4, respectively; p < 0.05). Significantly, 15 (65.2%) AF relapses occurred during the first week after cardioversion with a higher incidence in group I (10/33 patients, 30.3%) than group II (2/34 patients, 5.9%; p = 0.01) and group III (3/30 patients, 10%; p = 0.04). CONCLUSIONS: Six days of oral V administration centered on the C day, combined with P, significantly reduce the incidence of early recurrences of AF compared with P alone.

[The predictive value of junctional beats during the radiofrequency transcatheter ablation of the slow pathway of the nodal reentry circuit]. / Valore predittivo dei battiti giunzionali durante l'ablazione transcatetere mediante radiofrequenza della via lenta del circuito di rientro nodale.

BACKGROUND: Junctional beats (JB) are often recorded during slow pathway (SP) radiofrequency (RF) ablation in patients with atrioventricular nodal reentrant tachycardia (AVNRT). Neither the correlation between JBs and SP potentials nor the role of mechanically-evoked JBs has been clarified yet. METHODS: Two hundred-eleven consecutive patients, with common AVNRT, underwent RF transcatheter ablation guided by Jackman SP potential searching. If we were unable to record an SP potential or if 4 RF pulses delivered on ideal ablation sites were ineffective, the ablation was carried out on anatomical landmarks. Light pressure was applied with the ablation catheter to each ablation site before RF delivery in order to evaluate the inducibility of JBs. RESULTS: Transcatheter ablation was performed successfully in 209/211 (99%) patients. In 17 (8.1%) patients, no SP potential was recorded. JBs were observed more often delivering RF in the mid-septal region, whereas SP potentials were more often recorded at the base of the Koch triangle. The success rate (successful pulses/overall pulses) was higher in the mid-septal (58.6% in M1, 77.8% in M2) than in the postero-septal region (4% in PSC, 16.8% in P1). JBs showed a higher specificity (73.2 vs 5.3%), positive (55.5 vs 24.6%) and negative predictive value (97.3 vs 63.8%) than SP potential in identifying the successful ablation site. Mechanical JBs were evoked in 23 patients on 29 ablation sites, and 18/29 (62.1%) of them were successful ablation sites. CONCLUSIONS: The recording of JBs during or before RF ablation is a useful parameter to guide SP ablation in patients with AVNRT. Although the underlying mechanism has not been clarified yet, their preferential occurrence in the mid-septal region suggests that they might be due to thermal stimulation of compact atrioventricular node.

[Ablation of paroxysmal tachycardia in Wolff-Parkinson-White syndrome]. / Ablazione del circuito delle tachicardie parossistiche nella sindrome di Wolff-Parkinson-White.

Surgical or catheter ablation of accessory pathways by means of high energy shock has been substantial associated with morbidity and mortality. On the contrary radiofrequency current, an alternative energy source for ablation, has a low incidence of complications and a very high success rate. Aim of this study was to relate about the results using catheter ablation of accessory pathways by radiofrequency current in our Electrophysiologic laboratory. Radiofrequency current (20-40 W) was delivered between a large-tip catheter electrode positioned against the mitral or tricuspid anulus and a standard adhesive electrosurgical dispersive pad applied to the left chest wall. When possible radiofrequency delivery was guided by catheter recordings of accessory pathway activation. Ablation was attempted in 181 patients with 191 accessory pathways. We obtained successful ablation of 187/191 accessory pathways and accessory pathway conduction was eliminated in 177/181 patients. During a mean follow-up of 14 +/- 10 months preexcitation returned in 7 patients (3.9%). All underwent a second successful ablation. In 1 patient, during the procedure, we observed a transient ischemic attack, with no complications. In accordance to other reports we conclude that radiofrequency ablation is highly effective in ablating accessory pathways, with low morbidity and no mortality.

[The psychodynamic basis of diver training]. / Le basi psicodinamiche dell'addestramento del subacqueo.

The techniques employed in teaching diving and especially diving safety, were studied. Since it was felt that most accidents are not merely fortuitous but also derive from unconscious urges towards risk-taking, an attempt was made to understand the psychodynamics of the question. The study also investigated the effectiveness of the teaching method as a means not only of transmitting intrinsically useful skills and knowledge, but also as a psychotherapeutic technique aiding the diver to control any self-destructive aggressive urges.

[Psychodynamic aspects of sports diving]. / Aspetti psicodinamici dello sport subacqueo.

Individual and group discussions with divers and instructors were used to discover the psychodynamic bases of the diver's psychology. The clearly narcisistic compensation mechanisms, based essentially on so-called "cross-identification" appear to explain the apparently contradictory personality traits revealed by both psychodiagnostic techniques and phenomenological analysis. The recognition of cross identification as the basis of the narcisism-masochism collusion appears to be a useful addition to the field of accident prevention.

Relation between voice-onset time and vowel duration.

As part of an investigation of the temporal implementation rules of English, measurements were made of voice-onset time for initial English stops and the duration of the following voiced vowel in monosyllabic words for New York City speakers. It was found that the VOT of a word-initial consonant was longer before a voiceless final cluster than before a single nasal, and longer before tense vowels than lax vowels. The vowels were also longer in environments where VOT was longer, but VOT did not maintain a constant ratio with the vowel duration, even for a single place of articulation. VOT was changed by a smaller proportion than the following voiced vowel in both cases. VOT changes associated with the vowel were consistent across place of articulation of the stop. In the final experiment, when vowel tensity and final consonant effects were combined, it was found that the proportion of vowel duration change that carried over to the preceding VOT is different for the two phonetic changes. These results imply that temporal implementation rules simultaneously influence several acoustic intervals including both VOT and the "inherent" interval corresponding to a segment, either by independent control of the relevant articulatory variables or by some unknown common mechanism.

[Atmospheric pollution and chronic respiratory diseases in the blast-furnace areas of iron-works]. / Inquinamento atmosferico ed affezioni respiratorie croniche nella zona altoforni di uno stabilimento siderurgico.

An epidemiologic research together with a study on the environmental pollution were carried out in order to evaluate the risk of chronic respiratory diseases of blast furnace workers. The environment study was performed mainly using personal samplers given to workers with different jobs. Observations on 222 work shifts have shown that the total dust concentration to which cast workmen, maintenance men and blast furnace service men were exposed, marginally exceed the TLV values. Furthermore the level of respirable dusts for blast furnace service men was found slightly excessive. The average SO2 concentration was largely below the TLV values. However this gas could be found in excess for very short periods during the work. The epidemiologic study, conducted on a cohort of blast furnace area workers against a control group cohort, indicated a moderate prevalence of pneumoconiosis and chronic bronchitis amongst blast furnaces workers. The clinic and radiological pictures do not seem to go beyond the initial stages.

[Environmental lead pollution. An epidemiologic study in road gasoline station workers (author's transl)]. / Sull'inquinamento atmosferico da piombo. Indagine epidemiologica tra gli addetti ai distributori di benzina

Erythrocyte ALA-D and urinary coproporphyrins were investigated in 29 road gasoline station workers in order to detect a possible abnormal lead absorption. The ALA-D test represents the most sensitive laboratory device to detect both abnormal lead absorption and possible initial lesions due to lead poisoning. Using the above mentioned laboratory tests evidence of abnormal lead absorption was found in five of the examined subjects. Some remarks on prevention of possible lead intoxication are done.