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Biomedical subjects

G Santoro

Publications and source records attributed to G Santoro.

At least 19 recordsLinked to original sources
PubMed1995

Hypertrophic cardiomyopathy in Tuscany: clinical course and outcome in an unselected regional population.

OBJECTIVES: Our aim was to study a population of patients with hypertrophic cardiomyopathy from the well defined geographic region of Tuscany in central Italy, a group virtually free of selective referral bias and therefore probably closely representative of the true patient population with this disease. BACKGROUND: Most available information on clinical course, natural history and prognosis of hypertrophic cardiomyopathy is based on data generated from tertiary referral centers and therefore constitutes a potentially biased perspective of the disease process in this complex and diverse condition. METHODS: The study group comprised 202 patients aged 1 to 74 years (mean +/- SD 41 +/- 17) at initial diagnosis and followed up for 1 to 30 years (mean 10 +/- 5). RESULTS: Largely with the use of single or multiple drug therapy, the vast majority of patients (n = 154 [76%]) were asymptomatic or mildly symptomatic and in stable or improved condition over the period of follow-up, whereas the remaining patients (n = 48 [24%]) experienced deterioration, had substantial functional impairment or died. Of the 13 patients (6%) who died of cardiovascular causes related to hypertrophic cardiomyopathy, 11 had progressive congestive heart failure (including 6 in the end-stage phase) and only 2 died suddenly. The annual mortality rate for cardiovascular disease was 0.6% and that due to sudden cardiac death was only 0.1%; the cumulative survival rate was 97%, 95% and 92%, respectively, at 5, 10 and 15 years of follow-up. Atrial fibrillation proved to be a relatively common (n = 57 [28%]) and particularly unfavorable clinical feature, with premature death occurring in 9 of the 57 patients. The cumulative survival rate after 15 years was 76% for patients with atrial fibrillation versus 97% for patients with sinus rhythm. Syncope occurred in 33 patients (16%) but did not appear to be of prognostic significance. CONCLUSIONS: In an unselected regional population, hypertrophic cardiomyopathy had a relatively benign prognosis inconsistent with its prior characterization as a generally progressive disorder, based primarily on the experience of selected referral institutions. Sudden unexpected cardiac death was distinctly uncommon, although a sizable proportion of patients (particularly the subset prone to atrial fibrillation), did experience clinical deterioration.

Adolescent↗
PubMed1995

Human delta opioid receptor: a stable cell line for functional studies of opioids.

A human delta opioid receptor cDNA clone (pREP10/hDOR) was transfected into Chinese hamster ovary (CHO) cells. The stable cell line expressed a high density of delta opioid receptors (137,000 +/- 21,600 receptors/cell). DPDPE inhibited 90% of the forskolin-stimulated cAMP accumulation in these cells with high potency (EC50 = 1.3 nM). This effect of DPDPE was antagonized by naltrindole. The pseudo-pA2 value (Ke) of 155 pM for naltrindole is consistent with that measured for delta receptor antagonism in the mouse vas deferens. This is the first detailed characterization of DPDPE activity on forskolin-stimulated cAMP accumulation mediated through a human delta opioid receptor. The data support the use of the recombinant cell line for functional studies of opioid drugs.

Animals↗
PubMed1995

Isolated atrial inversion in situs inversus: a rare anatomic arrangement.

Isolated atrial inversion in situs inversus is a rare congenital cardiac malformation. Its physiology resembles transposition of great vessels, and the best option for its surgical treatment is the atrial switch operation. In this article, we present a case of isolated atrial inversion in concordance with visceral situs inversus diagnosed at birth by echocardiography and cardiac catheterization, which was successfully treated at 8 months of age by a Senning procedure.

Coronary Circulation↗
PubMed1995

Origin of both coronary arteries from the pulmonary artery and aortic coarctation.

Single trunk anomalous origin of both coronary arteries from the pulmonary artery is a rare congenital cardiac anomaly. We report on 2 cases of its association with aortic coarctation, the diagnosis of which in living patients is very difficult. We think that the possibility of this anatomic arrangement should always be considered in patients with isolated aortic coarctation whose clinical condition seems impaired rather than improved after an apparently successful coarctectomy.

Aortic Coarctation↗
PubMed1995

Biochemical and functional identification of GABA receptors in Hydra vulgaris.

GABA binding sites labelled in vitro with [3H]GABA are present in crude membrane preparations from Hydra vulgaris. [3H]GABA binding is specific (70%), saturable and it is completely displaced by the GABA mimetic muscimol but not by bicuculline or baclofen. Scatchard analysis of saturation data indicates the presence of only one population of binding sites with a Bmax of 4, 75 pmol/mg of protein and a KD of 76 nM. In the living animal GABA and benzodiazepines increase the duration of mouth opening during the glutathione-induced feeding response. Bicuculline prevents the GABA-induced increase of the feeding response. Diazepam activity is enhanced by simultaneous GABA administration and it is suppressed by the specific antagonist flumazenil. In contrast, no [3H]-flunitrazepam binding is detected in membrane preparations. We conclude that a population of GABA receptors is present in Hydra tissues, where they are involved in the modulation of response to chemical stimulation.

Animals↗
PubMed1995

Epidermolysis bullosa junctionalis associated with urinary bladder exstrophy: a case report.

We report the second infant of nonconsanguineous parents with epidermolysis bullosa junctionalis associated with urinary bladder exstrophy, epispadias, anteriorized anus, and bilateral inguinal hernias. The family history also included the death of a maternal cousin due to epidermolysis bullosa. Our diagnosis was based on electron microscopy and immunofluorescence evidence. This patient is reported because of the rarity of this constellation of findings.

Abnormalities, Multiple↗
PubMed1995

[Pulmonary valvuloplasty in childhood: the immediate results and long-term follow-up].

BACKGROUND: Pulmonary balloon valvuloplasty is the treatment of choice of pulmonary valve stenosis, but early results and long-term follow-up in a numerous and homogeneous pediatric population are poorly known. MATERIAL AND METHODS: From April 1984 to April 1994, 202 valvuloplastic procedures were performed in 197 patients (age 52.5 +/- 150 months). Hemodynamic data were analysed according with valvular morphology and patient's age at procedure. During a follow-up period of 50 +/- 38 months, clinical and instrumental data were collected. RESULTS: After pulmonary valvuloplasty RV pressure decreased from 86 +/- 28 to 41 +/- 21 mm Hg (p < 0.001), transvalvular pressure gradient from 67 +/- 27 to 24 +/- 14 mm Hg (p < 0.001) and RV/LV pressure ratio from 1.01 +/- 0.29 to 0.53 +/- 0.19 (p < 0.001). Overall success rate was 95% (187/197 patients). Pulmonary valve dysplasia and/or annulus hypoplasia significantly influenced the efficacy of the procedure and/or the recurrence of valvar stenosis (18.8% vs 2.7%, p < 0.01). During the follow-up period, clinical examination was unremarkable, and transvalvular pressure gradient did not change (23 +/- 9 vs 24 +/- 6 mm Hg at discharge, p = NS). Freedom from restenosis was 98% at 3 years and 96% at 5 and 10 years. CONCLUSIONS: Pulmonary balloon valvuloplasty is a safe, effective and possibly definitive treatment for isolated pulmonary valve stenosis in infants and children. Age at valvuloplasty does not influence the success of the procedure, as opposed to valvular dysplasia and/or annulus hypoplasia that positively relate to the need of surgical valvotomy and/or the recurrence of stenosis during a long-term follow-up. In conclusion, prognosis of the majority of infants and children after a successful valvuloplasty is excellent during a long-term follow-up period.

Adolescent↗
PubMed1995

[Oral anticoagulant therapy in dilated cardiomyopathy. Warfarin treatment results in subgroups of patients at risk of embolic complications].

BACKGROUND: The aim of the study was to assess the role of oral anticoagulant therapy in patients with dilated cardiomyopathy at risk of embolic complications. METHODS: We studied retrospectively two hundred thirty-six patients with dilated cardiomyopathy; sixty-eight cases, with intracavitary thrombosis at cross-sectional echocardiography (17 patients), embolic episodes (22), N.Y.H.A. functional class IV (38), chronic atrial fibrillation (23) or with a combination of such conditions, were treated with warfarin. The cumulative period of exposure to embolic events during follow-up was 814 years in the whole population in the absence of anticoagulant treatment and 213 years during treatment. RESULTS: The rate of new embolic events was 1.6 and 0 for 100 patients-years for the two periods respectively. The difference was not statistically significant. No clinically relevant haemorrhagic complication was seen during treatment. CONCLUSIONS: Oral anticoagulant therapy may be safely given to subgroups of patients with dilated cardiomyopathy at risk of embolic episodes, following empirical guidelines, provided a careful clinical and laboratory monitoring is carried on, even if no definite conclusion may be obtained about the efficacy of this treatment from a non-randomized study with low rates of new embolic events.

Administration, Oral↗
PubMed1995

34-day total fast in an adult man.

The aim of this work was to investigate the changes of cardiac performance by both electrocardiography (ECG) and echocardiography (ECHOc), in addition to anthropometric and hormonal variables before, during and after prolonged total fasting (TF) and re-feeding in an overweight adult man. Physical examination, laboratory and hormonal measurements, ultrasonographic study of body fat distribution, ECG and ECHOc study were performed before during and after 34 days of TF and after 17 days of isocaloric re-feeding. The subject was a 52-year old Caucasian who was overweight with increased abdominal fat content (BMI: 28.6; W/H ratio: 0.95) and increased levels of arterial systolic and diastolic blood pressure (SBP, DBP). HPLC measurements of urinary catecholamine levels (HPLC), ECHOc study of cardiac performance, ultrasonographic study of body fat distribution were performed. The subject starved for 34 days losing 22kg, but after that time he was compelled to re-feed because of nausea and severe vomiting. A marked ketosis (ketonuria > 1200mg/day) was already present after 6 days of TF. After 17 days of TF norepinephrine (NE) and epinephrine (EPI) urinary levels showed a two-fold and nine-fold increase respectively, but they became undetectable at the end of TF. After 17 days of re-feeding catecholamine urinary levels were similar to those measured after 17 days of TF. After both TF and 17-day isocaloric re-feeding we found a decrease of visceral fat content and W/H ratio reached the normal values for age-matched subjects (W/H ratio after TF: 0.80, after re-feeding: 0.80).(ABSTRACT TRUNCATED AT 250 WORDS)

Adipose Tissue↗
PubMed1994

Coronary flow dynamics and reserve assessed by transesophageal echocardiography in obstructive hypertrophic cardiomyopathy.

Myocardial ischemia is frequently associated with left ventricular outflow obstruction. To assess coronary flow impairment in obstructive hypertrophic cardiomyopathy (HC), 10 patients with echo-Doppler-detected obstructive HC and normal coronary arteries underwent transesophageal echo-Doppler examination of both coronary flow velocity (CFV) at rest, recorded in the proximal left anterior descending coronary artery, and coronary flow reserve (CFR) evaluated by means of dipyridamole infusion response. Ten normal patients were similarly studied and served as a control group. Two relevant alterations in coronary flow dynamics were detected in patients with HC: (1) a significantly increased diastolic/systolic CFV ratio, and (2) a significantly reduced dipyridamole/baseline CFV ratio. Compared with normal subjects, the CFV pattern showed a significantly greater diastolic and a significantly lower systolic component at rest (in some patients it was reversed). Diastolic/systolic CFV ratio was significantly higher in patients with HC at baseline (3.1 +/- 1 vs 1.6 +/- 0.5; p < 0.01) and increased further after dipyridamole infusion (4.9 +/- 2 vs 2.2 +/- 0.7; p < 0.01). In addition, CFR was impaired in patients with HC (1.8 +/- 0.3 vs 3.1 +/- 0.5; p < 0.01). Furthermore, a significant correlation between CFR and intraventricular pressure gradient was found. Thus, transesophageal echo-Doppler examination is a useful tool for evaluating CFV dynamics and CFR as demonstrated in patients with obstructive HC.

Aged↗
PubMed1994

The cloning and expression of a human creatine transporter.

A human creatine transporter (hCRT-BS2M) cDNA clone was isolated from a human brainstem/spinal cord using a PCR and phage plaque hybridization based technique. This clone included an open reading frame of 1,905 base pairs(bp) within a 2,283bp cDNA. Northern blot hybridization detected the expression of corresponding mRNAs most prominently in the skeletal muscle, heart and kidney. Peptide sequence analysis of the hCRT-BS2M protein product revealed 12 putative transmembrane domains. The predicted protein sequence further demonstrates that the hCRT-BS2M has highly conserved amino acid identity with the other members of the sodium dependent plasma membrane transporter family. Transient expression of the hCRT-BS2M in COS-7 cells demonstrates sodium dependent [14C]creatine uptake with a KM value of 14.9 +/- 3.0 microM (n = 5) that is attenuated by creatine and selective structural analogues of creatine.

Amino Acid Sequence↗
PubMed1994

Echocardiographically guided repair of tetralogy of Fallot.

Although 2-dimensional, Doppler, color flow echocardiography is accepted as a safe diagnostic method to guide the surgical treatment of certain heart defects, cardiac catheterization remains mandatory for patients with tetralogy of Fallot. Based on the excellent diagnostic correlation between echocardiography and angiocardiography, a policy of echo-guided primary repair of uncomplicated, selected cases of tetralogy of Fallot was introduced at Ospedale Bambino Gesù. In the last 5 years, of 139 patients who had surgery for tetralogy of Fallot, 105 underwent primary repair. In 56 patients (53.3%), surgery was guided only by echocardiography (group I). In the remaining 49 patients (46.7%) (group II), unclear imaging of cardiovascular anatomy (n = 23), or echo-suspected associated malformations (n = 26) prompted cardiac catheterization. The 2 groups did not differ in age and weight at surgery. Intraoperative examination did not show diagnostic errors in patients of group I; cardiac catheterization and surgery confirmed the suspected associated anomalies in 16 of 26 patients of group II (38.4% false-positive). Echocardiography showed an overall sensitivity of 100% and specificity of 85% for detection of associated malformations. In conclusion, echocardiography proved to be adequate for selection of patients with uncomplicated forms of tetralogy of Fallot for primary repair.

Cardiac Catheterization↗
PubMed1994

Effects of propofol, pentobarbital and alphaxalone on t-[35S]butylbicyclophosphorothionate binding in rat cerebral cortex.

The effects of propofol, pentobarbital, alphaxalone, etomidate and diazepam on t-[35S]butylbicyclophosphorothionate ([35S]TBPS) binding to membrane preparations from rat cerebral cortex were studied in the absence of gamma-aminobutyric acid (GABA). Addition of low concentrations (3-10 microM) of propofol to washed membrane preparations (devoid of GABA) markedly enhanced [35S]TBPS binding (maximal enhancement, 85%), whereas higher concentrations (50-100 microM) inhibited this parameter. Diazepam also enhanced [35S]TBPS binding in this preparation (maximal enhancement, 38%). In contrast, pentobarbital, alphaxalone and etomidate decreased [35S]TBPS binding in a concentration-dependent manner. The propofol-induced increase in [35S]TBPS binding in washed membranes was completely reversed by the addition of GABA at a concentration (0.3 microM) that alone did not modify [35S]TBPS binding (78% increase with 10 microM propofol alone, 33% decrease in the additional presence of GABA). The ability of GABA to reverse the effect of propofol on [35S]TBPS binding in washed membranes was shared by pentobarbital (200 microM) and alphaxalone (3 microM); etomidate (20 microM) only partially antagonized the effect of propofol. Diazepam at a concentration (30 microM) that alone had no effect on [35S]TBPS binding failed to modify the propofol-induced increase in [35S]TBPS binding, whereas at a concentration (3 microM) that alone increased [35S]TBPS binding the effect of diazepam was additive with that of propofol. The addition of bicuculline to washed membranes failed to abolish the increase in [35S]TBPS binding induced by propofol or diazepam, but completely antagonized the effects of pentobarbital, alphaxalone and etomidate.(ABSTRACT TRUNCATED AT 250 WORDS)

Anesthetics↗