Multiple hormonal and metabolic deficiency syndrome in chronic heart failure: rationale, design, and demographic characteristics of the T.O.S.CA. Registry.

Recent evidence supports the concept that progression of chronic heart failure (CHF) depends upon an imbalance of catabolic forces over the anabolic drive. In this regard, multiple hormonal deficiency syndrome (MHDS) significantly has impacts upon CHF progression, and is associated with a worse clinical status and increased mortality. The T.O.S.CA. (Trattamento Ormonale nello Scompenso CArdiaco; Hormone Therapy in Heart Failure) Registry (clinicaltrial.gov = NCT02335801) tests the hypothesis that anabolic deficiencies reduce survival in a large population of mild-to-moderate CHF patients. The T.O.S.CA. Registry is a prospective multicenter observational study coordinated by "Federico II" University of Naples, and involves 19 centers situated throughout Italy. Thyroid hormones, insulin-like growth factor-1, total testosterone, dehydroepiandrosterone , and insulin are measured at baseline and every year for a patient-average follow-up of 3 years. Subjects with CHF are divided into two groups: patients with one or no anabolic deficiency, and patients with two or more anabolic deficiencies at baseline. The primary endpoint is the composite of all-cause mortality and cardiovascular hospitalization. Secondary endpoints include the composite of all-cause mortality and hospitalization, the composite of cardiovascular mortality and cardiovascular hospitalization, and change of VO2 peak. Patient enrollment started in April 2013, and was completed in July 2017. Demographics and main clinical characteristics of enrolled patients are provided in this article. Detailed cross-sectional results will be available in late 2018. The T.O.S.CA. Registry represents the most robust prospective observational trial on MHDS in the field of CHF. The study findings will advance our knowledge with regard to the intimate mechanisms of CHF progression and hopefully pave the way for future randomized clinical trials of single or multiple hormonal replacement therapies in CHF.

Mixofibrosarcoma de bajo grado de la pared torácica / A low-grade myxofibrosarcoma of the chest wall

El mixofibrosarcoma es una neoplasia maligna infrecuente, que puede originarse en los tejidos blandos, se lo ha estadificado como de alto o bajo grado y la localización más frecuente son los miembros inferiores. El reconocimiento clínico del mismo es dificultoso, en primer lugar porque la clínica de otros sarcomas de partes blandas es similar y además semejan lipomas o aún quistes. La extirpación quirúrgica amplia es el tratamiento de elección, por la propensión de este sarcoma a la recidiva local. Los catalogados como de alto grado en un 30 % de los casos, pueden originar metástasis a distancia, especialmente a hueso, pulmón y ganglios linfáticos. La histopatología asegura el diagnóstico en la mayoría de los casos. Comunicamos el caso de un mixofibrosarcoma de bajo grado, en un hombre de 43 años, que a los tres años de control evolutivo, luego de la resección quirúrgica, no presenta recaída local ni distante.

Myxofibrosarcoma is an uncommon soft tissue sarcoma that is grading as low or high malignancy. The principal sites of involvement are the lower limbs. Clinical recognition is difficult since it resembles a cyst, a lipoma, other soft tissue sarcoma or even benign conditions as panniculitis. The wide surgical excision is the main therapeutic approach because local recurrence is frequent. The high grade variant is associated in 30 % of the cases with distant metastasis, especially to the lung, bone and lymph nodes. The histopathological features allow an accurate diagnosis in most cases. A 43 year-old man with a myxofibrosarcoma of the chest wall is reported with a follow-up of two years without recurrence.

Metalloproteinases and advanced glycation end products: coupled navigation in atherosclerotic plaque pathophysiology?

Matrix metalloproteinases (MMPs), their inhibitors (TIMPs) and inflammatory cytokines, such as interleukin-1 (IL-1), are considered markers of evolution and/or instability of atherosclerotic plaques. Accumulation of Advanced Glycation Endproducts (AGE) is a well known phenomenon in diabetes and has also been considered in the pathogenesis of atherosclerosis. Aim of the present study was to analyse the levels of pentosidine, a fluorescent AGE, and to evaluate the expression of MMP-2, TIMP-3, and IL-1 in an ex vivo model of human advanced atherosclerotic plaques. We intended to test the possible correlation between pentosidine and markers of ECM remodelling and inflammation in the atherosclerotic process, and to investigate if classic risk factors, such as diabetes and hypertension, influenced these biochemical parameters. We found that diabetic plaques showed higher level of pentosidine, as expected, but much lower, or even undetectable, expression levels of MMP-2 and TIMP-3; IL-1 expression was not different between diabetic and non diabetic plaques. Hypertension did not influence any of these parameters. Although the statistical correlations between the expression of the considered genes and pentosidine did not reach significance, slight negative trends were noted between TIMP-3 and IL-1 expression vs. pentosidine content. We suggest that in mature diabetic plaques AGE accumulation can exert stabilizing effects on matrix proteins, while scanty cell presence leads to poor capacity of reactive responses, such as remodelling and inflammation.

[Cardiac echinococcosis. Report of a case]. / Echinococcosi cardiaca. Descrizione di un caso clinico.

Cardiac hydatid disease represents 0.02-2% of the possible locations of the parasite. The case of an asymptomatic 60-year-old male patient, hospitalised for mnesic and cognitive disease, and with history of hepatic and pulmonary echinococcosis is reported. The patient showed a cardiac cyst localised in the mediobasal portion of the ventricular septum, with partial obstruction of the left ventricular outflow tract and with alteration of the left ventricular compliance. This case is of particular interest either for the rarity of cardiac localization or disagreement between the severity of anatomic involvement and absence of correlated symptoms. Therefore all patients affected by hydatid disease could be routinely investigated by 2D echo because this imaging technique is non invasive, feasible and has a high sensitivity for detecting intracardiac echinococcal cysts.

[Diagnosis of nonobstructive hypertrophic cardiomyopathy in a patient with Down's syndrome]. / Rilievo di cardiomiopatia ipertrofica non ostruttiva in un paziente con sindrome di Down.

This report describes the case of a patient with nonobstructed hypertrophic cardiomyopathy and Down's syndrome (47,XY, +21) in a 31 year old patient. Diagnosis of hypertrophic cardiomyopathy was made subsequently to ECG and two dimensional echocardiography findings. Personal and familial anamnesis and physical examination were negative. Standard ECG revealed signs of left ventricular enlargement and secondary alterations of repolarization (negative and giant T waves in DI-DII-V3-V4-V5-V6). Two dimensional and Doppler echocardiography disclosed left ventricular walls hypertrophy, particularly of the ventricular septum, of apex, lateral and inferior walls with normal systolic and diastolic function's index. Any intracavitary gradients were sound and Holter ECG monitoring repeated three times, revealed normal cardiac findings. In the literature this association was never described. We can't claim familial genesis of hypertrophic cardiomyopathy because parents and brothers didn't present any cardiopathy. It's important to mark that anatomic alterations functional features didn't correspond in our case, as demonstrated by clinical and echo-Doppler findings.

[High-dose chemotherapy, G-CSF and the use of peripheral blood progenitor cells in patients with poor-prognosis neuroblastoma]. / Chemioterapia ad alte dosi, G-CSF ed uso di cellule progenitrici da sangue periferico in pazienti affetti da neuroblastoma a cattiva prognosi.

In the past decade there has been an increasing use of high dose of chemo-radiotherapy in the treatment of poor prognosis solid tumors of childhood. The autologous bone marrow transplantation is the most used technique for circumventing the infectious and haemorrhagic complications occurring in the prolonged period of myelotoxicity. The faster recovery assured by the peripheral blood progenitor cells (PBPC) makes this procedure an attractive alternative. The advent of new apheretic modalities and the use of combinations of active antineoplastic drugs with various growth factors, such as G-CSF, GM-CSF and IL-3, has allowed to collect and concentrate the mononuclear fraction of peripheral blood leukocytes. The optimal timing for the collection is a crucial point and the utilization of flow cytometry for the determinations of circulating CD34+ cells in the peripheral blood is so far the best indicator for successful apheresis. The authors describe their experience in 16 children affected by poor prognosis neuroblastoma who had undergone high dose chemotherapy followed by G-CSF administration and PBPC collection. The details of apheretic techniques and the characteristics of conditioning regimen and haematologic recovery after PBPC reinfusion are also presented.

[The echocardiographic findings of cardiac rhabdomyomas in a female patient with tuberous sclerosis and Down's syndrome]. / Rilievo ecocardiografico di rabdomiomi cardiaci in una paziente con sclerosi tuberosa e sindrome di Down.

This report described the case of a patient with cardiac rhabdomyomas and tuberous sclerosis (TS), associated with Down's syndrome. Diagnosis of TS was made subsequently to sebaceus adenomas, peri-ungual fibromas and ash leaf macules, mental retardation, epilepsy, renal and cerebral calcification findings. Physical examination, ECG and Holter ECG monitoring revealed normal cardiac findings. Two dimensional echocardiography disclosed the presence of two areas of increased acoustic density; one of which was at the level of postero-medial papillar muscle and the second appeared to be adherent to ventricular septum. Left ventricular size, function and intracavitary blood flow were normal.

[Mental stress and ambulatory monitoring of arterial pressure]. / Stress mentale e monitoraggio ambulatoriale della pressione arteriosa.

The aim of this study was to validate the use of arithmetical mental stress testing as an indicator of an abnormal subject's response to stress in general. For this purpose 82 males (mean age 31 +/- 7 years, mean body surface area--BSA--1.76 m2), free from cardiovascular disease, underwent mental stress testing. In the sample examined we found 13 so-called reactive subjects, (mean age 32 +/- 7 years, mean BSA 1.8 +/- 0.1 m2), who during the test showed increases in mean blood pressure (greater than 20%) greater than mean values of the whole sample (17.8%). In any case blood pressure was higher than 150/100 mmHg. These data were compared with those of 15 subjects (mean age 32 +/- 4 years, mean BSA 1.77 +/- 0.1 m2) randomly selected among those, whose mean pressure increases were not higher than 17.8% (mean increase within sample). The 2 groups underwent blood pressure ambulatory monitoring and submaximal bicycle test. In reactive subjects, values of systolic (PAS) and diastolic pressure (PAD) were significantly higher than in controls (p less than 0.001) during blood pressure ambulatory monitoring, with a greater variability of such values as can be seen from the higher standard deviation (PAS 121 +/- 19 mmHg vs PAS 113 +/- 13 mmHg; PAD 82 +/- 11 mmHg vs PAD 75 +/- 10 mmHg). During ergometric testing, blood pressure trend was similar in the 2 groups.(ABSTRACT TRUNCATED AT 250 WORDS)

[Cardiovascular aspects of Martin-Bell syndrome]. / Aspetti cardiovasculari della sindrome di Martin-Bell.

Cardiovascular anomalies have been studied in 13 subjects (8 males and 5 females, average age 15 +/- 7 years) affected from fragile X syndrome. This group has been examined by standard-ECG, Holter-ECG, echocardiography (M-mode, B-mode, Doppler and color-Doppler). The results have been compared with a control group of 39 subjects (20 males and 19 females, average age 15 +/- 5 years), with non genetic mental retardation. Clinical examination, ECG and Holter did not show any significant pathological alteration compared with the results of the control group. In the study group echocardiography showed the following results: 10 subjects (77%) had mitral valve prolapse of the anterior leaflet (arching); 4 of which (31%) with associated posterior leaflet prolapse; 2 subjects (15%) with posterior aortic leaflet prolapse; 2 subjects (15%) with tricuspid septal leaflet prolapse; 3 subjects (23%) had mild pulmonary artery dilatation; 1 subject (8%) had a mild aortic regurgitation; in 9 subjects (69%), 3 of whom with pulmonary artery dilatation, has been found pulmonary valve regurgitation; 10 subjects (77%) had tricuspid valve regurgitation. In all subjects cardiac dimensions were within the normal range. The most important result, in accordance with literature, is the high prevalence of mitral valve prolapse. The prolapse is asymptomatic and silent. We have never found aortic root dilatation that was described by other Authors. The described anomalies could be ascribed at the dysfunction of the connective tissue. This theory has been confirmed by necropsy studies. Therefore, we suppose that these alterations, particularly the anterior mitral leaflet prolapse, are non casually associated with the fragile X syndrome.

[Prevalence of pulmonary valve insufficiency in healthy children: a Doppler color study]. / Prevalenza dell'insufficienza polmonare in soggetti sani di età pediatrica: studio con color-doppler.

One hundred patients, institutionalized for mental retardation, aged between 3 and 14 years (mean age 12.2 +/- 3) and free from cardiovascular and pulmonary diseases, were studied using Doppler technique (pulsed wave-continuous wave and color-coded Doppler), to evaluate the prevalence of pulmonary regurgitation. The authors, utilizing a triple method (diastolic turbulence above pulmonary valve detected by pulsed wave Doppler or diastolic flow detected by continuous wave Doppler, presence of regurgitant pulmonary color-jet, from short axis view, toward the right ventricular outflow tract, and presence of the same feature in the color m-multigate) to detect the presence or absence of pulmonary regurgitation found 73% positivity. There were no differences between the two sexes and the size of the pulmonary artery was in the normal range. The characteristics of regurgitation were: No holodiastolic. The regurgitant max velocity jet was not greater than 1.50 m/s. Beat to beat variability. Max length of color-jet was not more than 2 cm. Rapidly decreasing Doppler profile. We can conclude that pulmonary regurgitation is very frequent in children and is not significant if it has the above-named characteristics. This latter fact is further confirmed by other authors.