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Biomedical subjects

M Marletta

Publications and source records attributed to M Marletta.

15 recordsLinked to original sources

[Cardiac involvement in Kawasaki disease. Our experience].

BACKGROUND: Kawasaki disease is an acute vasculitis of unknown cause that affects children under 5 years of age and in 20-25% of cases can cause coronary artery anomalies. Early therapy with intravenous gamma globulin and aspirin greatly decreases the risk of developing coronary anomalies, but sometimes coronary disease develops even in timely diagnosed and treated cases. METHODS: Personal experience in 41 children with Kawasaki disease is reported. Age was within three months and 5 years. All patients had electrocardiography and echocardiography at diagnosis, after 1 week, at the third, sixth, and eighth week, after six months and after 1 year from diagnosis. Echocardiographic cardiac anomalies were found only in 5 cases (12.19%). Average age in these cases was lower. RESULTS: In these cases coronary involvement developed in spite of early diagnosis and therapy (intravenous gamma globulin 400 mg/kg die and aspirin) electrocardiographic findings were not relevant or specific to coronary arteries anomaly. In three cases coronary anomalies regressed during the first year from diagnosis, the other two are still in the first year of follow-up. Nine children had a long term follow-up (6 to 9 years) and they were all normal except one with renal arteritis and another with slight mitral incompetence. CONCLUSIONS: The authors underline the role of clinical and instrumental follow-up that should be extended to all patients affected by Kawasaki disease with coronary anomalies even if early regressed.

Age Factors↗

The activation of neuronal NO synthase is mediated by G-protein betagamma subunit and the tyrosine phosphatase SHP-2.

In CHO cells we had found that CCK positively regulated cell proliferation via the activation of a soluble guanylate cyclase. Here we demonstrate that CCK stimulated a nitric oxide synthase (NOS) activity. The production of NO was involved in the proliferative response elicited by CCK regarding the inhibitory effect of NOS inhibitors L-NAME and alpha-guanidinoglutaric acid. We identified the NOS activated by the peptide as the neuronal isoform: the expression of the C415A neuronal NOS mutant inhibited both CCK-induced stimulation of NOS activity and cell proliferation. These two effects were also inhibited after expression of the C459S tyrosine phosphatase SHP-2 mutant and the betaARK1 (495-689) sequestrant peptide, indicating the requirement of activated SHP-2 and G-betagamma subunit. Kinetic analysis (Western blot after coimmunoprecipitation and specific SHP-2 activity) revealed that in response to CCK-treatment, SHP-2 associated to G-beta1 subunit, became activated, and then dephosphorylated the neuronal NOS through a direct association. These data demonstrate that the neuronal NOS is implicated in proliferative effect evoked by CCK. A novel growth signaling pathway is described, involving the activation of neuronal NOS by dephosphorylation of tyrosyl residues.

Animals↗

Bioerodible polyanhydrides as drug-carrier matrices. II. Biocompatibility and chemical reactivity.

The biocompatibility of bioerodible polyanhydrides and toxicology of the polymer breakdown products were assessed. Poly-[bis (p-carboxy-phenoxy) propane anhydride] (PCPP), Poly(terephthalic acid anhydride) (PTA), and their copolymers with sebacic acid were tested. The polymers did not provoke inflammatory responses in the corneas of rabbits over a six week implantation period. Subcutaneous implantation studies of PCPP in rats over a six month period showed no evidence of inflammatory cells and only slight tissue encapsulation by layers of fibroblastic cells. The degradation products of the polymers were nonmutagenic, noncytotoxic, and had a low teratogenic potential. The in vitro growth of mammalian cells on the polymers was unaffected as measured by cell morphology and cell growth rate. The chemical reactivity of the polyanhydrides with reactive model drugs, para substituted anilines, was also examined. Amides were formed when the drugs were injection molded with the polyanhydrides at 120 degrees C. However, no reaction was observed using compression molding at room temperature. No reaction occurred between the polymer and the drug during the hydrolytic degradation of the matrix at 37 degrees C.

Anhydrides↗

[Physiopathologic findings and surgical treatment in transposition of great vessels: our experience].

Transposition of great vessels (TGV) is the most frequent neonatal cyanotic malformative cardiopathy. The Authors report their experience in surgical anatomical correction of transposition of the great arteries in 55 patients, 44 male and 11 female. Minimum age was 2 days, maximum 6 months. TGV was simple in 38 cases and associated with ventricular defect in 12 cases, with double outlet right ventricle in 3 cases, and with complex cardiopathy in 2 cases. There have been 11 deaths. The cause of death was: cardiac failure in 3 patients, myocardial infarct in 4 patients, respiratory insufficiency in 2 patients, and sepsis in 2 patients. There was one death by myocardial infarction and 1 asymptomatic ostial left coronary stenosis during follow-up (from 1 to 104 months). There were neither anastomotic pulmonary stenosis nor aortic valve incompetence. The authors review the literature on functional and anatomic correction of TGV and underline the importance of precocious anatomic together with early diagnosis, percutaneous atrioseptostomy and pharmacologic (PGE1) therapy in determining further reduction of mortality and to improve late outcome.

Age Factors↗

Vein of Galen aneurysmal malformation. Different clinical expressiveness. Three case reports.

The vein of Galen aneurysmal malformation (VGAM) is a rare cerebro-vascular disorder in neonates. It is characterized by an abnormal direct communication between one or several cerebral arteries and the vein of Galen. It may appear in the neonatal period or afterwards. Three cases of patients affected by VGAM with different clinical expression are presented. Two of them were treated successfully with endovascular embolization. It was not possible to provide the same treatment to the third patient for an intractable congestive heart failure already existing when VGAM was diagnosed.

Cerebral Angiography↗

[THe echographic diagnosis of diaphragmatic eventration in newborn infants. 2 cases].

The authors report two cases of neonatal partial diaphragmatic eventration. The diagnosis in both two cases was echographic more than radiographic. The authors underline the role of B-mode echography in the diagnosis and follow-up of diaphragmatic anomalies, especially eventration, and suggest that examination of diaphragm and of its motion should complete any abdominal and thoracic echography.

Diaphragm↗

[Heart involvement in progressive spinal muscular atrophy. A review of the literature and case histories in childhood].

There are few cardiological studies in progressive spinal muscular atrophy and mainly concern subjects affected by the juvenile form (Kugelberg-Welander disease). The presence of a cardiomyopathy has been reported in these patients but the cardiac involvement is often secondary to the chronic respiratory insufficiency typical of the disease. We performed a retrospective study in our Institute on 43 patients, age range 3 months to 3 years, 37 of which presented type I (Werdnig-Hoffmann disease) and 6 type II (intermediate form) of the disease. No clinical nor instrumental signs of cardiomyopathy were observed. However, ECG revealed signs of right ventricular overload in 37.3% of the patients, probably provoked by pulmonary hypertension due to respiration anomalies. The authors underline the importance of correct respiratory assistance to prevent onset of cardiological alterations.

Cardiomyopathies↗

[Realities and perspectives of perinatal care in Sicily. Experience at an outborn neonatal unit].

The Authors studied some mortality indices in the neonatal intensive and subintensive care unit of the University of Catania in order to assess the degree of efficiency of perinatal assistance. This Unit is the largest in southeastern Sicily and admits only outborn newborns from the city of Catania, the Catania province and other provinces. Comparison of the 1991-92 and the 1993-94 data revealed a marked reduction in the mortality rate, however this fall was only marginal in newborns of, or under 1,000 g. There was a marked decrease in the mortality rate from respiratory distress, especially in ventilated newborns receiving additional surfactant. Comparison of the data showed that in both study periods the mortality rate was much higher in newborns over 6 hours of life, in those presenting hematic pH values under 7.25 at admission and in those coming from other provinces. These results underline that it is essential for the political authorities to boost neonatal assistance in the delivery room and to ensure adequate transport of distressed newborns.

Birth Weight↗

[Indications for percutaneous atrioseptostomy. Comparison of echocardiographic and fluoroscopic monitoring].

Balloon atrial septostomy (BAS) is of established value in the management of several congenital heart diseases in the neonatal period. This procedure, which leads to creation of a tear in the membrane of the Foramen Ovalis using a balloon catheter, may be undertaken under fluoroscopic monitoring in catheter laboratory as well as under echographic control, even bedside. In this study we present our experience and discuss the indications to these two techniques. 91 neonates underwent to BAS; in 14 of them this was carried out under two-dimensional echocardiographic control in the intensive care unit. In all the patients BAS had good result, with clinical improvement in the majority of cases (97%) and a low rate (6%) of minor complications (such as transient supraventricular arrhythmias), without differences between fluoroscopic and ultrasound monitoring. The higher rate of mortality in the fluoroscopic monitoring group (2/91 = 2.2%) was thought to depend on an extremely critical presentation of the neonates. The echographic monitoring does not seem to offer any real advantage from the technical point of view, but the necessity of a prompt treatment of very ill patients emphasizes the advantages of a quickly and easily feasible procedure. Thus, we recommend the use two-dimensional echocardiographic imaging only in the very ill neonates in whom the septostomy can be more safely performed in intensive care unit bedside.

Catheterization↗

[Epidemiological and clinical findings in congenital heart diseases during the neonatal period. Report on personal cases].

Cardiovascular impairment is frequent in the neonatal period and can be linked to malformative and non malformative pathologies. The Authors performed a retrospective study on the incidence of these two types of pathologies in a population of 3326 newborns who underwent cardiological examination. Malformative cardiopathy was observed in 779 (23.4%) of them, while non malformative cardiopathy was present in 316 (9.5%). Acyanogen forms (86.7%) were more common than cyanogen forms (13.2%) in malformative, while cyanogen forms (64.8%) were more frequent than acyanogen ones (35.1%) in non malformative cardiopathies. The Authors reviewed the incidence of the single cardiac defects and report some clinical and physiopathological considerations that underline the need for prompt differential diagnosis and the validity of echocardiography in achieving this aim.

Cohort Studies↗