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M Acampa

Publications and source records attributed to M Acampa.

13 recordsLinked to original sources
PubMed2006

Cardiac disease and Rett syndrome.

Rett syndrome (RS) is a neurodevelopmental disease,1 affecting approximately 1 in 10 000-15 000 females. Clinical severity of RS may vary with increasing age, following a four stage model.

Adolescent↗
PubMed2004

Cardiac dysautonomia and serotonin plasma levels in Rett syndrome.

BACKGROUND: In Rett syndrome the autonomic nervous system is abnormal at various levels, from the central to the peripheral nervous system. A role for serotoninergic dysfunction has been suggested. OBJECTIVES: The aim of our study was to evaluate the relation between cardiac dysautonomia (expressed by means of heart rate variability) and plasma serotonin levels in girls affected with Rett syndrome. Heart rate variability and plasma serotonin levels were evaluated in 28 Rett girls aged 1-14 years. A Pearson correlation was used to determine whether there was a relationship between plasma serotonin levels and each heart rate variability parameter. RESULTS: In untreated Rett girls the plasma serotonin levels correlated with the sympathovagal balance, as expressed by the low frequency (LF) to high frequency (HF) ratio (p<0.05). CONCLUSIONS: Our results suggest that cardiac dysautonomia could be linked to serotoninergic dysfunction and that treatment with a serotonin analogue could be useful in improving the sympathovagal balance.

Adolescent↗
PubMed2002

Acute effects of glibenclamide on reactive hyperaemia in the lower limbs in humans.

Three episodes of 1 min ischemia in the lower limbs in humans reduced the metabolic debt repayment (expressed as AUC of reactive hyperaemia) following more prolonged ischemia (666.6+/-86.6 vs 500.0+/-33.5 ml/100 ml). The administration of the ATP-dependent K(+) channel blocker glibenclamide was associated with a significant reduction in the AUC of reactive hyperaemia (666.6+/-86.6 vs 563.1+/-76.6 ml/100 ml), and with the removal of the protective effect produced by 3 episodes of 1 min ischemia (563.1+/-76.6 vs 551.8+/-71.3 ml/100 ml). Plasma level of glibenclamide reached the peak value of 1.295+/-0.15 micromol/l 2 h after drug administration, ranging around the 1 micromol/l concentration in the following 3 hours. Our findings produce indirect evidence that, similarly to the ischemic preconditioning of the heart, the protective effects towards ischemia of brief repeated episodes of sub-maximal occlusion in the peripheral circulation of the lower limbs in humans are mediated by ATP-dependent K(+) channels.

Adult↗
PubMed2001

Progressive cardiac dysautonomia observed in patients affected by classic Rett syndrome and not in the preserved speech variant.

Incidence of sudden death in Rett syndrome is greater than that of the general population, and cardiac electrical instability is a prime suspect cause. The objective of the present study was the evaluation of heart rate variability, a marker of autonomic activity, in females affected by classic Rett syndrome and atypical variants for a possible explanation of the higher risk for sudden death observed in these subjects. Our study showed that girls with classic Rett syndrome had significantly lower heart rate variability and longer corrected QT intervals than in atypical Rett syndrome and age-matched healthy girls. Reduction of heart rate variability progresses with age and with the clinical stage of the syndrome. These results suggest the possible role of the progressive cardiac dysfunction in the sudden death associated with Rett syndrome.

Child↗
PubMed2001

Effects of alprostadil on blood rheology and nucleoside metabolism in patients affected with lower limb chronic ischaemia.

The acute (0.57 microg/kg i.v. in 2 hours) and long-term (0.57 microg/kg i.v. in 2 hours for 5 days over 4 weeks) effects of the PGE1 analogue alprostadil were studied in patients affected with intermittent claudication. Whole Blood Viscosity (WBV), Whole Blood Filterability (WBF), haematocrit (Htc) and fibrinogen plasma concentration, were studied together with P50, 2,3-diphosphoglycerate, and adenosine plasma levels. Moreover, in the long-term study, pain-free (PFWD) and maximal walking distance (MWD) were measured. Single alprostadil infusion induced an improvement in WBV, WBF, and oxygen transport, and an increase in adenosine plasma levels. Long-term alprostadil administration produced a decrease in WBV only, without significant changes in WBF, Htc, fibrinogen, P50, 2,3-diphosphoglycerate, also inducing a significant prolongation of PFWD and MWD. The possibility is suggested that pulse rises in adenosine plasma levels play a role in the effects of chronic alprostadil administration, maybe in a way similar to that observed in the phenomenon of ischaemic preconditioning,

Alprostadil↗
PubMed1999

Reduced heart rate variability in patients affected with Rett syndrome. A possible explanation for sudden death.

Incidence of sudden death in Rett syndrome is greater than that of the general population and cardiac electrical instability is a prime suspect cause. Our study shows that girls with Rett syndrome had significantly lower heart rate variability (marker of autonomic disarray) and longer corrected QT intervals compared with an age-matched group of healthy girls. These abnormalities increased with advancing stages of the syndrome. These findings suggest a possible role of cardiac dysfunction in the sudden death associated with Rett syndrome.

Adolescent↗
PubMedDate not supplied

Nerve growth factor plasma levels and ventricular repolarization in Rett syndrome.

Rett syndrome is a severe neurological developmental disorder. In this syndrome, the high incidence of sudden death is correlated with an alteration of ventricular repolarization. The purpose of this study was to evaluate plasmatic levels of nerve growth factor (NGF) in Rett patients with prolonged corrected QT (QTc) interval in comparison with those of Rett patients with normal QTc. We observed 23 female Rett patients (9.9+/-4.7 years). NGF plasma levels and QTc interval were measured in all patients. Student t-test was performed for statistical analysis. NGF plasma levels were significantly lower in Rett patients with QTc interval prolongation (QTc > 0.44 sec) in comparison with Rett patients with a normal QTc interval (4.5+/-4.5 vs 11+/-8.3 pg/ml, p = 0.02). The alteration of NGF levels, observed in Rett patients with a long QTc interval, may explain the presence of an altered ventricular repolarization associated with a higher risk of cardiac arrhythmias.

Adolescent↗
PubMedDate not supplied

Effects of acetyl-L-carnitine on cardiac dysautonomia in Rett syndrome: prevention of sudden death?

There is a higher incidence of sudden death in patients with Rett syndrome than individuals in the general population. Previous studies have implicated cardiac dysautonomia and a long QT interval as causative factors. Because carnitine plays a critical role in cellular metabolism and may have beneficial effects on cardiac and nerve function, we investigated the effects of long-term treatment with acetyl-L-carnitine on heart rate variability and electrocardiographic abnormalities in 10 girls with Rett syndrome and compared the results with 12 control patients (girls with Rett syndrome who were not treated). The age range of the subjects was 2-21 years. The study design called for the evaluation of heart rate variability, corrected QT interval, and QTc dispersion. In the 10 Rett girls treated with acetyl-L-carnitine, a significant increase in heart rate variability was observed. To explain these results, we hypothesize that acetyl-L-carnitine has a neurotrophic action on the cardiac autonomic nervous system. This effect may reduce the risk of sudden death in patients with this syndrome.

Acetylcarnitine↗