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Biomedical subjects

S Giusti

Publications and source records attributed to S Giusti.

At least 19 recordsLinked to original sources
PubMed2001

Transcatheter closure of atrial septal defects with the STARFlex device: early results and follow-up.

BACKGROUND: The STARFlex (SF) device is a CardioSEAL (CS) double-umbrella device that has been modified by the addition of a self-centering mechanism comprised of nitinol springs connected between the two umbrellas and a flexible core wire with a pin-pivoting connection. This paper compares the results of atrial septal defect (ASD) closure with CardioSEAL and STARFlex devices. PATIENTS AND METHODS: Between December 1996 and March 2000, 117 patients underwent ASD closure with CardioSEAL (n = 79) and STARFlex (n = 38). The mean age (17 years), weight (49 kg), and ASD size (15 mm) were similar in the two groups. The procedures were performed under general anesthesia with both fluoroscopic and transesophageal echocardiographic monitoring. IMMEDIATE RESULTS: The devices were successfully implanted in all patients. Ten patients had multiple ASDs. A single device was used in four patients (CardioSEAL in three, STARFlex in one), while a simultaneous placement of two CardioSEAL (one patient) or two STARFlex (five patients) were performed in six patients. The results are summarized as follows: [table: see text] Follow-Up Results: On follow-up clinical exam, electrocardiograms, chest X-rays, and echocardiograms were obtained at 1, 6, and 12 months. [table: see text] During follow-up there were no deaths, endocarditis, rhythm disturbances, or other complications. Arm fractures were observed almost exclusively with large CardioSEAL devices (40 mm, less frequently with 33 mm), and only in one 33-mm STARFlex device. There were no clinical complications related to fractures. CONCLUSIONS: The STARFlex device seems to offer better results than the CardioSEAL, with significantly lower rates of residual shunts and arm fractures.

Adolescent↗
PubMed2001

Complex conotruncal heart defect, severe bleeding disorder and 22q11 deletion: a new case of Bernard-Soulier syndrome and of 22q11 deletion syndrome?

A patient with a deletion in the DiGeorge/velocardiofacial chromosomal region in 22q11, underwent cardiac repair for truncus arteriosus with a separate origin of the pulmonary arteries. This patient presented with a severe coagulation disorder similar to that described in the Bernard-Soulier syndrome. Additional features included minor facial anomalies, transient hypocalcemia and renal failure. To the best of our knowledge, this is the third case of a severe bleeding disorder associated with 22q 11 deletion reported in the literature.

Bernard-Soulier Syndrome↗
PubMed2000

Tyrosine phosphorylation of focal adhesion kinase by PDGF is dependent on ras in human hepatic stellate cells.

Focal adhesion kinase (FAK) is a widely expressed nonreceptor tyrosine kinase found in focal adhesions. FAK has been indicated as a point of convergence of other signaling pathways including platelet-derived growth factor (PDGF) receptors, and recently, FAK tyrosine phosphorylation has been shown to be stimulated by PDGF. In the present study we assessed the role of Ras as a possible intermediate protein regulating PDGF-induced FAK tyrosine phosphorylation in human hepatic stellate cells (HSCs), liver-specific pericytes primarily involved in the pathogenesis of liver fibrosis. For this purpose, cells were first subjected to retroviral-mediated gene transfer with a dominant-negative mutant of Ras (N17Ras). This resulted in a marked inhibition of PDGF-induced FAK tyrosine phosphorylation together with the expected reduction of PDGF-induced extracellular signal-regulated kinase activity (ERK). Afterward, the effects of pharmacological agents potentially affecting Ras isoprenylation were evaluated. PDGF-induced FAK tyrosine phosphorylation, ERK activity and intracellular calcium increase, as well as the biological effects of this growth factor, (i.e., mitogenesis and cell migration) were effectively blocked by GGTI-298, an inhibitor of geranylgeranyltransferase I. Inhibition of Ras processing obtained with FTI-277, an inhibitor of farnesyltransferase, resulted in detectable effects only at high doses. Taken together, these results establish that Ras operates as a protein-linking PDGF-beta receptor to FAK in human HSCs, and that signaling molecules requiring geranylgeranylation may also be involved in this process.

Alkyl and Aryl Transferases↗
PubMed2000

A European multicentric experience using the CardioSEal and Starflex double umbrella devices to close interatrial communications holes within the oval fossa.

In this review, we describe the experience from 13 European centres using the CardioSEAL and Starflex double umbrella devices to close interatrial communications within the oval fossa (so-called 'secundum' defects). Between October 1996 and April 1999, the procedure was attempted in 334 patients with a mean age of 12 years and a mean weight of 44kg. The mean measured stretched diameter of the defect was 15 mm. In the overall group, the defect was solitary in 245 patients (73%), multiple in 21 (6%), associated with an aneurysm of the flap valve in 15 (5 %), was represented by patency of the oval foramen in 44 (13%), and was a fenestration in a Fontan repair in 9 (3%). In all patients, the devices were inserted under general anesthesia, using fluoroscopic and transesophageal echocardiographic control. Implantation was achieved in 325 (97,3%). The device embolized within either a few minutes or a few hours in 13 patients (4%). Of these, uncomplicated surgical repair was undertaken in 10, while the device was retrieved in 3 using catheters and a second device was successfully implanted. Residual shunting was detected immediately after the procedure in 41% of the patients, with the incidence decreasing to 31% at discharge, 24% at 1 month, 21% at 6 months, and 20.5% at one year. During the period of follow-up, elective surgical repair became necessary in two patients, due to malposition of the device in one, and late embolization in the other. Fractures of arms were seen in 6.1 %, most commonly with the largest devices. All those with fractured arms of the device were asymptomatic, and no clinical complications related to the fractures were observed. There were no arrhythmias, endocarditis, valvar distortion, thromboembolic events, or other complications. After one year of follow-up, clinical success, defined as complete closure of the defect or presence of only a trivial leak, had been obtained in 92.5% of the patients. We conclude, therefore, that these devices produce excellent results when used to close defects of small to moderate size. Results are less than optimal, or else complications ensure, when attempts are made to close very large defects.

Adolescent↗
PubMed1999

Long-term follow-up of stents implanted to relieve peripheral pulmonary arterial stenosis: hemodynamic findings and results of lung perfusion scanning.

In recent years, percutaneous placement of stents has been used as an alternative to surgery or balloon angioplasty for the treatment of adults with peripheral pulmonary arterial stenosis. This therapy has also been proposed for children, but questions still remain about its indications in this group of patients. We describe here the results of intravascular placement of stents in a group of 29 patients, with a mean age of 12+/-7 (range 3-31) years and weighing 35+/-19 (range 11-74) kg. All were affected by postsurgical or congenital isolated pulmonary arterial stenosis, and have now been followed for 38+/-19 (range 6-65) months. The early hemodynamic results have been excellent, with a significant reduction of the pulmonary arterial systolic pressure, the systolic pressure gradient, and the ratio of systolic pressures in the pulmonary and systemic circuits, and with a significant increase of the diameter of the stented vessels in all the patients. Of the 29 patients, 24 have been recatheterized 18+/-10 months after the procedure, demonstrating the stability of the results, with a low incidence of late restenosis, this seen in only 1 patient (2%). Lung perfusion scanning, performed in 17 patients each year after the follow-up catheterization, has showed that the results are maintained at long-term follow-up (51+/-9 months).

Adolescent↗
PubMed1999

[Splenic artery aneurysm and portal hypertension. Report of a case].

Visceral arteries aneurysms are an uncommon pathology. Lienal artery (60%), hepatic (20%), superior mesenteric (5.9%) and tripod celiac (4%) are the most involved. Female predominance, portal hypertension, arteriosclerosis, pregnancy, traumatic and infective factors are the most remarkable etiopathogenetic factors. Portal hypertension with splenomegaly might be an important factor in the pathogenesis of intra- and extraparenchimal splenic artery aneurysms. Stress is laid on the importance of arteriography during portal hypertension in pregnancy as a preventive measure because the rupture of aneurysm of the splenic artery, despite laparotomy in emergency is often fatal, and of coeliac angiography prior to liver transplantation; if splenic artery aneurysm is found, ligation of the splenic artery should be performed at the time of transplantation to prevent possible rupture. The different roles of the imaging techniques used are examined.

Aged↗
PubMed1998

Multiple exposure to arsenic, antimony, and other elements in art glass manufacturing.

Art glass manufacturing is one of the most interesting examples of exposure to complex mixtures. Among the raw materials used are silica sand, borax, carbonates, nitrates of Ca, Na, K, and a great number of compounds that are mainly oxides of As, Sb, Al, Zn, Cr, Ni, Sn, Se, Cd, Mn, Cu, Co, Fe, Nd, Er, Eu, and La. In six art glass factories that use As or Sb as fining agents, the exposure to these elements was investigated in 32 workers by means of environmental and biological monitoring. Analysis was conducted by atomic absorption spectrometry (AAS) and inductively coupled plasma mass spectrometry (ICP-MS). The results confirmed that As, which is the main carcinogen in glass production, reaches high air concentrations and is generally above the American Conference of Governmental Industrial Hygienists (ACGIH) threshold limit value-time-weighted average (TLV-TWA) of 10 micrograms/m3. When partly substituted by antimonial compounds, As air concentrations dropped dramatically, while the air levels of Sb (which is considered less toxic but is classified as a class 2B carcinogen by IARC) were relatively low and below the ACGIH TLV-TWA of 500 micrograms/m3. Exposure to As and Sb also differed in the three types of jobs investigated: As was high in oven chargers, Sb was higher in batch mixers, and both elements were low in makers-formers. Eleven to 18 elements were detected: arsenic, Al, Ba, Sb, Pb, and Zn were the elements most frequently measured (in tens, and in some cases hundreds, of micrograms/m3), followed by B, Li, Mn, Se, Sn, Sr, Ce, La, and Nd (in micrograms or in some cases tens of micrograms/m3. The results of biological monitoring for As, Sb, and other elements were in agreement with environmental monitoring data. We concluded that multiple detection of elements is a useful tool (or the evaluation of exposure to complex mixtures such as those used in the art glass industry and that such detection also allows a more accurate evaluation of related epidemiological data.

Antimony↗
PubMed1998

[20 years of biological monitoring (1976-1995) of occupational lead exposure in various Tuscan provinces].

We report the results of 24,475 blood lead determinations (PbB) performed by the Occupational Toxicology Laboratory of Florence between 1976 and 1995. Biological monitoring was carried out in 383 factories localized in the districts of Florence, Prato, Pistoia, Lucca and Pisa. The most represented production fields were colouring ceramic factories, artistic ceramics, transfer-pictures for ceramics, glass factories, typographies, glass decoration factories, chemical factories. In twenty years, the median values of PbB decreased from 48 to 17 micrograms/100 ml in males and from 40 to 8 micrograms/100 ml in females. The 95 degrees centile ranged from 84 to 46 micrograms/100 ml for males and from 63 to 42 micrograms/100 ml for females. In the 1988-95 period, we observed an increase of both the 95 degrees centile and the maximal values because of the inclusion of data from workers employed in factories where the "decorazione a scavo" technique, which is characterized by elevated environmental lead concentrations, was used. In the period 1991-93, the ranges of PbB observed in glass decoration factories were 13-160 micrograms/100 ml for males and 4-80 micrograms/100 ml for females, respectively.

Adolescent↗
PubMed1997

Surgical closure of muscular ventricular septal defects using double umbrella devices (intraoperative VSD device closure).

OBJECTIVES: Surgical closure of some muscular ventricular septal defects has been proven to be difficult. In order to simplify the surgical technique we have used intraoperatively Rashkind double umbrella devices to occlude muscular ventricular septal defects. METHODS: On the basis of haemodynamic and echocardiographic study five children aged 4, 6, 7, 14 and 41 months were considered suitable candidates for intraoperative closure of muscular ventricular septal defects (midmuscular in three cases, apical in two) by Rashkind devices. Three of them had previously undergone pulmonary artery banding at 10, 11 and 41 days, respectively. During hypothermic cardiopulmonary by pass a delivery system was introduced across the tricuspid valve into the right ventricle and then passed through the ventricular septal defect; the distal umbrella of a 17 mm device was opened in the left ventricular cavity; a traction was applied to the introducer and the proximal umbrella was opened on the right side straddling the interventricular septum; the device was then secured on the right side by few stitches. In one case because of the wide diameter of the ventricular septal defect two umbrellas were used. The surgical procedure was completed with debanding and/or closure of other defects close to the aortic or tricuspid valve. RESULTS: Immediate results, tested by epicardial or transesofageal echo, showed a minimal residual shunt in 4 patients and a moderate shunt in one. No early deaths occurred. A complete atrioventricular block developed in 1 patient who had an additional perimembranous defect closed with a prosthetic patch: a permanent pace maker was inserted 3 months after the operation. There was a late death for untractable right ventricular failure in 1 patient who had a large residual shunt erroneously considered moderate. In this patient, the size of the defect was underestimated both preoperatively then intraoperatively. The four survivors are doing well with no signs of hemodynamically significant residual shunts. CONCLUSIONS: The use of Rashkind umbrella devices for closing intraoperatively muscular defects can be helpful to standard surgical techniques when technical problems make patch closure difficult. Its use avoid the need of left ventriculotomy. Careful definition of the size of the defect is mandatory to select suitable candidates.

Angiocardiography↗
PubMed1997

Transcatheter closure of patent arterial ducts using controlled-release coils.

OBJECTIVE: To determine the efficacy of transcatheter closure of patent arterial ducts using controlled-release coils. DESIGN: Transcatheter closure of patent arterial ducts was attempted in 52 patients using controlled-release coils. SETTING: For the study, four tertiary paediatric cardiology units were used, two of which were in the U.K., one in Italy and one in Turkey. PATIENTS: The 52 patients ranged in age between 3-5 months and 61 years (median 3-5 years), and weighted between 4.5 kg and 62 kg. The duct diameters were 1 mm to 6.5 mm. RESULTS: In four patients the ducts were too large for safe release of the coils. In the remaining 48, one coil was inserted in 33 patients, two coils in nine, three coils in four and four coils in two patients. Immediately at the end of the procedure, the duct was completely occluded in 26/47 (55%) patients. Haemolysis occurred in one patient, in whom the coil was removed by a snare catheter and a large umbrella device was implanted with resolution of the haemolysis. Coil embolization to the pulmonary artery occurred in five (10%) patients. All were easily retrieved and replaced by larger coils. At the latest follow-up by colour Doppler echocardiography, the duct was completely occluded in 44/47 (94%) patients. CONCLUSIONS: Transcatheter closure of patent arterial ducts by controlled-release coils is effective and safe. Even when more than one coil is inserted the technique is still less cumbersome and considerably cheaper than transcatheter umbrella closure.

Child, Preschool↗
PubMed1997

[Efficacy of recombinant erythropoietin on the quality of life in patients over 60 years of age undergoing hemodialysis].

We followed for a period of six months, 54 patients of over 60 years old, submitted to hemodialitic treatment. We gave human recombinant erythropoietin, average dosage 50 UI/Kg subcutaneously on alternative days, folic acid and iron supplements together with a proteic supply of 1.2 g/Kg/die (35 Kcal/Kg). The medullary response has been monitored with hematochemical tests; blood pressure and nutritional conditions have been evaluated. Furthermore, the patients were given a questionnaire to evaluate their quality of life. At the end of the follow up, 50 patients responded positively to therapy. These patients showed an increase of RBC (from 2,789,780 +/- 259,310 to 3,313,110 +/- 472,780 p < 0.001) of HCT (from 21.86% +/- 2.16% to 27.18 +/- 2.74% p < 0.0001) and of Hb (from 7.72 +/- 1.12 g/dl to 9.28 +/- 0.98 g/dl p < 0.006). Total protein and albumin increased too. Furthermore they showed a progressive increase of "performance status". Our results confirm efficacy of erythropoietin in the treatment of anemia in elderly hemodialized patients.

Age Factors↗
PubMed1996

Haemorheological changes in mixed cryoglobulinaemia during apheresis treatment.

Thirty-four patients affected by mixed cryoglobulinaemia have been submitted to treatment by apheresis (plasma exchange or double cascade filtration). The authors have monitored clinical, laboratory and, above all, haemorheological changes following therapy. The final results have shown mainly a reduction of plasma viscosity and consequently an improvement of the haemorheology of the affected organs. In conclusion, apheresis may be considered a successful therapy in patients with severe renal or neurological diseases due to mixed cryoglobulinaemia.

Blood Viscosity↗
PubMed1996

[Radiofrequency perforation in pulmonary valve atresia and intact ventricular septum].

METHODS: Four neonates with Pulmonary Atresia and intact Ventricular Septum underwent Radiofrequency Valvotomy in our institution from October 1994 to June 1995. All patients had "membranous" atresia with confluent and normal size pulmonary branches; one of them had abnormal tricuspid tensor apparatus and severe right ventricular outflow tract hypertrophy. The right ventricle was considered of sufficient size to support biventricular circulation in all cases. In three patients the pulmonary arteries were supplied by the arterial duct maintained open with intravenous infusion of Prostaglandins. The other patient had previously undergone a modified left Blalock Taussig shunt in another institution. The cardiac catheterization was performed by the femoral venous and arterial routes, under general anesthesia. After delineating the atretic valve with angiography, a 5 French right Judkins catheter was placed in the right ventricular outflow tract just beneath the membrane. A 2 french radiofrequency catheter (Cerablate PA 120 Osypka) was then passed through the right Judkins; with a mean energy of 5-10 watts over 3-5 seconds the valve was perforated. After a predilation with a 2,5 mm balloon catheter (New Probe USCI or Cobra SCIMED), the valve was dilated with balloon catheters 20 to 30% larger than the anulus (Cristal Balloon BALT). RESULTS: In all cases the procedure was successful without any complication; a significant decrease of right ventricular pressure and an excellent antegrade flow across the valve were achieved. Two patients were weaned from prostaglandins by sixth and eighth day after the procedure. The patient with previous Bialock Taussig shunt showed a complete recovery of the right ventricle, but four months later underwent surgery for shunt closure and enlargement of the left pulmonary branch. These three patients are well at latest follow-up (5-10 months), with normal biventricular circulation. The other patient remained duct-dependent and was operated on of outflow patch and shunt nine days after the procedure, with good result; he unfortunately died the tenth postoperative day for complications of hemopericardium related to epicardial wires removal. CONCLUSIONS: In our opinion radiofrequency valvotomy is the first choice procedure in cases suitable for biventricular repair. In selected patients it may represent a definitive treatment.

Angiography↗
PubMed1995

[Percutaneous occlusion of patent ductus arteriosus by the Rashkind double-umbrella device].

BACKGROUND: The transcatheter option consisting of implanting and releasing an occlusive device designed as a double-umbrella is an interesting alternative to surgery aimed to close persistently patent ductus arteriosus. POPULATION AND METHODS: Closure of a duct with the Rashkind device had been planned in 113 children. The procedure was abandoned in 12 with inadequately sized ducts (too large or too small). This study therefore included 101 attempts in patients aged 2.3 months to 18.5 years (m +/- 1 SD = 45.9 +/- 43.2 months) whose weights ranged from 3.3 to 87 kg (m +/- 1 SD = 15.7 +/- 11.7 kg). The narrowest dimension of the duct on the aortograms ranged from 1.2 to 6.2 mm (m +/- 1 SD = 2.9 +/- 0.9 mm). RESULTS: The procedure failed in seven patients because of a too large and/or tubular vessel, causing removal of the device prior to release in five patients, or surgical extraction after it had embolized into a pulmonary artery branch in two patients. An early acute hemolysis requiring again the surgical removal of an instable device in a tubular duct was seen in one case. Two patients had femoral artery occlusion successfully treated with thrombolytic agents. Complete occlusion was immediately proven in 32 (35%) of the 92 successful and stable implantations. These figures raised to 64% (59 cases) prior to discharge. At final follow-up (0.3-59 months, m +/- 1 SD = 13.8 +/- 14.4 months), another 16 total occlusions were observed and one patient was successfully managed by a second implantation. The final occlusion rate was 83% (76 cases). Of the 16 residual shunts, five were surgically suppressed and the remaining were minimal. CONCLUSION: Transcatheter occlusion of the patent ductus arteriosus is safe in children weighing more than 5 kg, having ducts with a narrowing ranging from 1 to 6 mm. It is efficient in five out of six cases and has less disadvantages than surgery.

Adolescent↗
PubMed1995

Balloon aortic valvuloplasty in the first year of life.

Between February 1988 and September 1993 balloon aortic valvuloplasty was attempted in 33 consecutive patients in the first year of life: 20 patients (61%) were younger than 1 month. Major associated anomalies such as mitral stenosis, coarctation, and hypoplastic left ventricle were found in 11 cases (33%). The balloon dilation of the aortic valve was accomplished through the right carotid cut-down approach in neonates and patients with body weight < 5 kg, through a percutaneous femoral approach in the others; the procedure was completed in all. The peak systolic gradient across the aortic valve measured at catheterization fell from 80 +/- 33 mmHg (range 25-165) before the dilation to 27 +/- 17 mmHg (range 0-65), afterwards (p < 0.0001). The left ventricular ejection fraction increased from 44% +/- 26% to 61% +/- 17%, 24-48 hours after the procedure (p < 0.0001). Aortic insufficiency developed in 17 cases, being moderate in 2, mild in 6, and trivial in 9. Seven patients (21%), all in the first month of life, died within 30 days from the valvuloplasty; major associated anomalies were present in six; the death was due to a procedure related complication in one. No mortality was observed among the patients undergoing valvuloplasty beyond the first month of life. On follow-up (6 months to 6 years) aortic restenosis occurred in 3 cases; 1 was treated by surgical valvotomy, 2 by repeat balloon valvatomy; in another 2 cases, a subvalvular aortic obstruction developed and was relieved by surgical resection. There was no late mortality. Thus, balloon valvuloplasty appears to be an effective palliation for critical aortic stenosis in infancy. Early mortality is mainly related to associated anomalies.

Aortic Valve Stenosis↗