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Biomedical subjects

G Nigro

Publications and source records attributed to G Nigro.

At least 19 recordsLinked to original sources
PubMed1998

Cytomegalovirus infection and schizencephaly: case reports.

Two children with left open-lip schizencephaly are reported. Cytomegalovirus (CMV) infection was demonstrated on the second day of life in Patient 1; in the other patient CMV infection was observed at the age of 4 years. Magnetic resonance imaging revealed polymicrogyric cortex lining the clefts. In Patient 2 polymicrogyria was also present in the corresponding area contralateral to the schizencephaly. Computed tomography revealed the presence of subependymal periventricular calcifications. Genetic analysis did not demonstrate the presence of EMX2 homeobox gene. The possible role of CMV infection in the complex multifactorial pathogenesis of schizencephaly is therefore suggested.

Brain↗
PubMed1998

The effect of glucocorticoid therapy on fetal lung maturity indices in hypertensive pregnancies.

OBJECTIVE: To assess fetal lung maturity tests in hypertensive pregnancies and to examine the effect of glucocorticoid therapy. METHODS: In a cohort study involving 68 pregnant women with hypertension, 34 received antenatal betamethasone before amniocentesis and 34 did not. Controls were 68 women with uncomplicated pregnancies, matched for gestational age at amniocentesis and fetal gender. Amniotic fluid (AF) samples were analyzed by lamellar body count, planimetric and stechiometric lecithin-sphingomyelin ratio (L/S), and presence of phosphatidylglycerol. RESULTS: Fetal lung maturity, as determined by lamellar body counts and by planimetric L/S, was lower in hypertensive pregnancies not treated with steroids than in controls (19,600 +/- 14,500 versus 39,800 +/- 22,700, P < .009, and 1.9 +/- 0.6 versus 3.9 +/- 1.8, P < .01, respectively). In the period of 24 to 33 weeks' gestation, the percentage of untreated pregnancies with mature lamellar body counts and mature L/S was significantly lower than that of controls (13% versus 33%, P < .001; 6% versus 40%, P < .002 and P < .003, respectively). In contrast, in patients treated with betamethasone, the percentage of cases with mature indices for both tests was not significantly different from that of controls, but was higher than that of untreated hypertensive patients (40% versus 13%, P < .001; 33% versus 6%, P < .001). Phosphatidylglycerol did not differ among groups. From 34 to 38 weeks, no difference was found in the percentage of mature cases for lamellar bodies in pregnant women with hypertension not treated with steroids in comparison with controls (68% versus 84%), nor between cases treated and controls (74% versus 84%). In the same period, no difference in L/S values was found among groups, and the percentage of cases positive for phosphatidylglycerol was lower in hypertensive pregnancies than in controls (47% versus 95%, P < .001) and was not affected by steroid treatment (37% versus 95%, P < .001). CONCLUSION: Fetal lung maturity, as reflected in AF tests, is delayed in hypertensive pregnant patients, and steroids increase all lung maturity indices except phosphatidylglycerol between 24 and 33 weeks' gestation.

Adult↗
PubMed1997

Autonomic nervous system imbalance and left ventricular systolic dysfunction as potential candidates for arrhythmogenesis in Becker muscular dystrophy.

We evaluated the arrhythmic profile in a population of 20 Becker muscular dystrophy (BMD) patients searching for possible correlations between the severity of the arrhythmic events, the cardiac autonomic balance (assessed by heart rate variability analysis in the time domain) and the degree of left ventricular systolic impairment. A population of 14 male healthy individuals served as the control group. BMD subjects exhibited lower values of SDNN (P=0.013), SDANN index (P=0.008) and 24-h mean heart rate (P=0.002). The total number of premature ventricular beats (totPVB) and the number of PVB out of 1000 heartbeats (PVB/1000) appeared also higher in BMD subjects (P=0.05 and P=0.046, respectively). No difference was found in terms of 24-h mean QTc and 24-h longest QT among the two groups. TotPVB and PVB/1000 were inversely related to both the ejection fraction (r= -0.620, P=0.004 and r= -0.517, P=0.019) and to the shortening fraction (r= -0.568, P=0.009 and r= -0.469, P=0.037). Twenty-four-h mean QTc was also inversely related to both the ejection fraction (r= -0.520, P=0.019) and the fractional shortening (r= -0.491, P=0.028). These data suggest that in BMD there is cardiac autonomic imbalance characterized by sympathetic predominance and an increased susceptibility to ventricular arrhythmias, even in the absence of overt cardiomyopathy. Furthermore, the severity of the arrhythmic profile in BMD appears closely related to the degree of left ventricular systolic dysfunction.

Adult↗
PubMed1997

Mother to infant transmission of coinfection by human immunodeficiency virus and hepatitis C virus: prevalence and clinical manifestations.

The prevalence and the clinical course of hepatitis C virus (HCV) infections were studied in 23 HIV-1-infected children, who were born to 22 mothers with HIV-1/HCV coinfection. During the follow-up only two children (8.7%) showed persistent anti-HCV antibodies and circulating HCV RNA. Both children, who were aged 10 and 10.6 years respectively at the end of follow-up, had chronically-evolving liver disease and autoimmune thrombocytopenia but no signs of progressive HIV disease. Based on our experience, vertically-acquired HIV-1/HCV coinfection is less frequent than is generally reported and may be associated with the development of chronic thrombocytopenia in addition to liver disease. Moreover, perinatal HIV-1/HCV coinfection appears to be associated with a slow progression of HIV disease.

AIDS-Related Opportunistic Infections↗
PubMed1997

Ganciclovir therapy for cytomegalovirus-associated liver disease in immunocompetent or immunocompromised children.

Ganciclovir therapy was given intravenously to 20 children with cytomegalovirus (CMV)-associated liver disease, of whom 6 were immunocompetent and 14 were immunocompromised (9 had AIDS and 5 had solid tumors). Immunocompetent children had isolated liver disease diagnosed at birth (4 children), or systemic congenital CMV infection including liver disease (2 children). Ganciclovir was used following two regimens: A) 5 mg/kg twice daily for 8 to 86 days (mean 21); B) 7.5 mg/kg twice daily for 14 days followed by 10 mg/kg three times weekly for three months. CMV infection was diagnosed by viral isolation, detection of viral antigens, and/or CMV DNA from blood and urine. All immunocompetent children had negative CMV culture and CMV DNA detection from blood and/or urine after 14 weeks of treatment. However, the three children who were treated with regimen B showed normal ALT levels at the end of the maintenance course, whereas the children who received ganciclovir with regimen A had normal ALT levels only after about 1 year. All children with tumors initiated regimen B, but only three, who had negative CMV detection and markedly decreased ALT levels, received full treatment; of the remaining two children, one recovered after only an initial course, and the other had therapy interrupted because of hepatic failure and died 9 days later. In contrast, the children with AIDS received several ganciclovir courses for different periods at the lower dosage: they generally improved during treatment but did not recover completely, and five children died with active CMV infections. Based on our study, CMV-associated liver disease can be efficiently treated with ganciclovir both in immunocompetent and immunodeficient children. However, a single ganciclovir course including a higher dosage and prolonged therapy appeared to be more effective than several courses with lower dosages.

AIDS-Related Opportunistic Infections↗
PubMed1997

Identification of the Syrian hamster cardiomyopathy gene.

The BIO14.6 hamster is a widely used model for autosomal recessive cardiomyopathy. These animals die prematurely from progressive myocardial necrosis and heart failure. The primary genetic defect leading to the cardiomyopathy is still unknown. Recently, a genetic linkage map localized the cardiomyopathy locus on hamster chromosome 9qa2.1-b1, excluding several candidate genes. We now demonstrate that the cardiomyopathy results from a mutation in the delta-sarcoglycan gene that maps to the disease locus. This mutation was completely coincident with the disease in backcross and F2 pedigrees. This constitutes the first animal model identified for human sarcoglycan disorders.

Amino Acid Sequence↗
PubMed1997

Left ventricular function and perfusion in Becker's muscular dystrophy.

UNLABELLED: The aim of this study was to evaluate left ventricular (LV) perfusion and function in patients with Becker muscular dystrophy (BMD). METHODS: Fourteen male patients (age range 14-40 yr) with BMD were evaluated by 201Tl SPECT and radionuclide angiography both at rest and after dipyridamole stress test. RESULTS: All patients showed uptake defect demonstrated by 201Tl SPECT (mean 4.1 +/- 2.2 uptake defect/patient). Significant relationships (p < 0.05) were found between the number of uptake defects and rest LV ejection fraction (LVEF) (r = -0.54); peak filling rate (PFR) (r = -0.57) and dipyridamole LVEF (r = -0.65). Dipyridamole induced reversible uptake defects were found in 7/14 (50%) patients with BMD. The 14 patients were divided into two groups on the basis of the presence (Group A, n = 6) or the absence (Group B, n = 8) of severe irreversible uptake defect (i.e., < 50% 201Tl uptake). Group A showed lower values of PFR and LVEF when compared to patients of Group B. CONCLUSIONS: In patients with BMD there is a relatively high incidence of uptake defects and LV function (both at rest and after dipyridamole) appears to be related to the number of uptake defects. Moreover, the presence of severe irreversible uptake defects identifies a subgroup of patients with BMD characterized by a severely depressed LV function.

Adolescent↗
PubMed1997

Postpartum lupus erythematosus associated with parvovirus B19 infection.

We describe postpartum onset of systemic lupus erythematosus (SLE) associated with parvovirus B19 infection in a mother presenting with fever, polyarthritis, erythema, and multiorgan involvement. B19 infection was revealed by detection of B19 DNA and IgM antibodies. In addition, our patient showed low CD4+ (384 x 10(6)/l) and CD8+ (213 x 10(6)/l) T cells, high immunoglobulin values (23.77 g/l), hypocomplementemia, thrombocytopenia, leukopenia, and anemia. Her daughter had rash associated with increasingly high B19 IgG levels and transient antinuclear and anti-ds-DNA antibodies, suggesting that both development of SLE and active B19 infection occurred in pregnancy and B19 was transmitted prenatally. A 2 year followup showed persisting polyarthritis in the mother and atopy in the daughter.

Adult↗
PubMed1997

["Pediatric" forms of Creutzfeldt-Jakob disease (nvCJD)].

The authors propose the name of pediatric CJD disease (nvCJD) for those forms of this pathology which appear in childhood and adolescence (about twenty cases have been reported in Europe: 1996). The "pediatric form" differs both in terms of age ("teenagers" and also 16-year-old), but also owing to its long course (mean of 14 to 35 months) and the early onset of psychiatric and sensory symptoms. Until a few years ago, discussion of a new pediatric-adolescent form of the disease (new variant of CJD) would have been laughable.

Child↗
PubMed1996

Development of cardiomyopathy in female carriers of Duchenne and Becker muscular dystrophies.

OBJECTIVE: To characterize the presence and behavior of the dystrophinopathic myocardial damage in female carriers of a gene defect at the Xp21 locus of the X chromosome that causes Duchenne and Becker muscular dystrophies (DMD and BMD). DESIGN: Cohort study from April 1, 1985, to April 30, 1995, with cardiologic follow-up performed yearly for a minimum of 3 to a maximum of 10 years. SETTING: Counseling center for genetic muscular disorders. PATIENTS: A total of 197 women and girls aged 5 to 60 years ascertained to be carriers of the DMD (n = 152) or BMD (n = 45) gene. MAIN OUTCOME MEASURES: Cardiac status at yearly examinations as determined by 12-lead electrocardiogram (ECG), 24-hour ambulatory ECG, M-mode and 2-dimensional echocardiography, and carotid pulse tracing. Myocardial scintigram was performed on each individual at least twice during the study. Immunohistochemical analysis of dystrophin from myocardium and/or skeletal muscle biopsy was performed in 12 carriers. RESULTS: Preclinical or clinically evident myocardial involvement was found in 166 cases (84.3%), without significant differences in percentage and behavior between DMD and BMD carriers. Its occurrence increased significantly with age, from 54.5% (18 cases) in carriers aged between 5 and 16 years to 90.2% (148 cases) in carriers older than 16 years. Dystrophin anomalies were detected at the membrane level of the myocardial fibers in all endomyocardial biopsy specimens. CONCLUSIONS: Genetic anomalies can be considered the primary cause of myocardial damage in carriers of dystrophinopathic myopathies; myocardial damage shows the same behavior already described in DMD and BMD patients and progresses from preclinical to dilated cardiomyopathy, passing through stages of myocardial hypertrophy or dysrhythmias.

Adolescent↗
PubMed1996

Identification of a novel sarcoglycan gene at 5q33 encoding a sarcolemmal 35 kDa glycoprotein.

Mutations in any of the genes encoding the alpha, beta or gamma-sarcoglycan components of dystrophin-associated glycoproteins result in both sporadic and familial cases of either limb-girdle muscular dystrophy or severe childhood autosomal recessive muscular dystrophy. The collective name 'sarcoglycanopathies' has been proposed for these forms. We report the identification of a fourth member of the human sarcoglycan family. We named this novel cDNA delta-sarcoglycan. Its mRNA expression is abundant in striated and smooth muscles, with a main 8 kb transcript, encoding a predicted basic transmembrane glycoprotein of 290 amino acids. Antibodies specifically raised against this protein recognized a single band at 35 kDa on western blots of human and mouse muscle. Immunohistochemical staining revealed a unique sarcolemmal localization. FISH, radiation hybrid and YAC mapping concordantly linked the delta-sarcoglycan gene to 5q33, close to D5S487 and D5S1439. The gene spans at least 100 kb and is composed of eight exons. The identification of a novel sarcoglycan component modifies the current model of the dystrophin-glycoprotein complex.

Amino Acid Sequence↗
PubMed1996

Coping strategies and anxiety in Italian adolescents.

203 Italian adolescents completed an Italian modified version of the Amirkhan's Coping Strategy Indicator and the Italian version of the Spielberger, et al. State-Trait Anxiety Inventory. Three brief stories were presented to the subjects who were requested to put themselves in the protagonist's place and to indicate the extent to which they would use specific coping behaviors to deal with one of the three stressful events. Analysis of variance 3 x 2 (stressor by sex) showed effects due to the sex of the respondent on Seeking Social Support and to stressor on the subscales Problem Solving and Avoidance. A negative correlation was observed between scores on Trait Anxiety and Problem Solving and a positive one between scores on Trait Anxiety and Avoidance. Results seem to confirm the hypothesis that both situational antecedents and anxiety affect the choice of coping strategies. Further implications of finding were discussed.

Adolescent↗
PubMed1996

Rapid progression of HIV disease in children with cytomegalovirus DNAemia.

OBJECTIVE: In HIV-1-infected children, active cytomegalovirus (CMV) infection can cause severe clinical manifestations and accelerate progression of HIV disease. However, sufficient quantities of blood samples may not be available either for culture or detection of CMV DNA or antigens in white blood cells. The aim of this study was to investigate the diagnostic and prognostic significance of detecting CMV DNA in serum samples from HIV-1-infected children. DESIGN: Sera from 55 children (18 boys), aged 2-130 months (mean, 49.8 months), with perinatal HIV-1 infection and clinical manifestations attributable to CMV infection were tested for CMV DNA by nested polymerase chain reaction and for class-specific CMV antibodies [immunoglobulin (Ig) G, IgA, IgM] by enzyme-linked immunosorbent assay. The children were followed up for 2 days to 59 months (mean, 25.5 months). RESULTS: CMV infection was demonstrated in 43 children (74.5%), 18 of whom (42%) were positive for CMV DNA. During the follow-up, 13 children with CMV infection (30.2%) died, including 11 (84.6%) who were positive for CMV DNAemia just before death. Of these children, seven died soon after hospitalization without antiviral treatment, and four died despite therapy with ganciclovir or foscarnet. Post-mortem CMV inclusions were revealed in seven out of eight children who underwent autopsy. The two other children who died also had progressive CMV disease and received ganciclovir until death. In comparison with CMV-seropositive children without CMV DNAemia, children with CMV DNAemia showed significantly shorter mean survival time (42.5 versus 60 months; P < 0.01), lower final CD4+ T-cell count (218 versus 499 x 10(6)/1; P < 0.01) and higher mortality rate (P < 0.0001). CONCLUSIONS: The detection of CMV DNA in serum is of value for diagnosis of active CMV infection in HIV-1-positive children, and CMV DNAemia is a good prognostic indicator of severe outcome of HIV disease.

Antibodies, Viral↗
PubMed1996

[Prions and infantile pathology].

The authors briefly review the problem of prions pathology (with particular emphasis on children). The outstanding interest of the subject justifies this review.

Animals↗
PubMed1996

[Psychiatry and dermatologic neoplasms: results of an experimental study].

Psycho-oncology has developed during the past decades in the area of medical oncology directly involved in the study of the psychological aspects of cancer. In this study the authors focused in particular on the psychopathological complications of dermatological cancer, the discovery of a possible history of psychiatric disorders prior to the diagnosis of the tumour, the predictive value of the latter concerning further psychiatric complications during cancer, and lastly the possible correlations between stress and the onset of cancer and between religious belief and adaptive strategies. A total of 30 patients suffering from dermatological neoplasia were examined within the setting of a Psychiatric Liaison Service. HDRS and STAI X-1 and 2 were used as psychodiagnostic aids to evaluate the level of depression and anxiety, together with an interview for the clinical evaluation of the patient ad possible life events of particular importance. It was found that 10% of patients conformed to the criteria for the diagnosis of dysthymia and 10% for adaption disorders, and that there was also a high rate of pathological values for status anxiety (66%). These results show that this type of disease is particularly subject to risk for psychiatric complications.

Female↗
PubMed1996

Prion-induced neuro-psychiatric syndromes.

Although further research is needed, the most recent thinking is that prions are tiny protein particles without DNA or RNA which have the ability to infect humans or animals. Prions cause slow infections which are fatal, experimentally transmissible, evoke no immune resistance from the infected host and are more resistant to disinfectants and chemical or physical agents than the other micro-organisms that proliferate in the infected host. Update no evidence of possible passage by mouth from cows to men with consequent illness.

Animals↗