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Biomedical subjects

F Schettini

Publications and source records attributed to F Schettini.

At least 19 recordsLinked to original sources
PubMed1995

I-131 MIBG scintigraphy of neuroectodermal tumors. Comparison between I-131 MIBG and In-111 DTPA-octreotide.

An account is given of the results observed with I-131 MIBG scintigraphy in four patients (1 bladder pheochromocytoma, 3 neuroblastomas) chosen on account of their particular clinical and diagnostic interest from a series of 41 apudoma patients examined by means of this technique. In the first patient, the unusual site of the tumor in the posterior wall of the bladder meant that its detection by I-131 MIBG was only possible after catheterization of the bladder. In the second patient, uptake in the metastasis was only evident after removal of the primary tumor. In the third patient, the scintiscan revealed several metastases (some in bone) not detected by CT. In the fourth patient (congenital neuroblastoma), enhanced uptake accompanied the appearance of high plasma catecholamine and urinary vanillylhandelic acid values, suggesting a functional switch from a nonsecreting to a secreting form. a supplementary In-111 DTPA-Octreotide (OCT) scintiscan of this patient demonstrated the presence of somatostatin receptors on the neuroblasts. Thus, this examination would seem particularly useful for the differentiation of nonsecreting neuroblastomas. Its employment in assessment of the therapeutic capacity of OCT itself is also suggested.

3-Iodobenzylguanidine↗
PubMed1994

A novel single-base mutation in the glucose 6-phosphate dehydrogenase gene is associated with chronic non-spherocytic haemolytic anaemia.

More than 80 variants of glucose-6-phosphate dehydrogenase (G6PD) are associated with chronic nonspherocytic haemolytic anaemia (CNSHA); however, the molecular basis of this association is not fully understood. We have used the polymerase chain reaction and nucleotide sequence analysis to characterize a new G6PD variant, which we designate as G6PD Bari, in a G6PD-deficient boy affected by CNSHA. A single mutation leading to an amino-acid substitution was detected in the G6PD coding region, viz. a C->T transition at position 1187 predicting leucine at residue 396 in the enzyme; proline is invariably present in evolutionary distant G6PD molecules at this position. Inheritance in the patient's family was demonstrated by the polymerase chain reaction followed by diagnostic restriction enzyme analysis. The C->T transition responsible for G6PD Bari maps close to several other mutations previously identified in G6PD variants associated with CNSHA.

Adolescent↗
PubMed1994

Rochalimaea henselae organisms possess an elevated capacity of binding to peripheral blood lymphocytes from patients with cat scratch disease.

Cat scratch disease (CSD) is a clinical condition whose aetiological agent, according to recent findings, is of bacterial origin. Two Gram-negative bacteria are invoked as causative agents of CSD, namely Afipia felis and Rochalimaea henselae. In this paper, five patients with suspected CSD were studied in terms of binding capacity of A. felis and R. henselae to their own peripheral blood lymphocytes (PBL). This parameter was correlated with serum antibody titres to both A. felis and R. henselae, as determined by an indirect fluorescence assay (IFA). Results demonstrate that in four out of five cases binding of R. henselae to PBL was higher than that observed with A. felis. In two cases serum antibody titres to both bacteria were lower or absent, while in the other two patients serum antibody titres to R. henselae were significantly high. In one case only, characterized by elevated titres of serum antibodies to A. felis, values of cytoadherence exhibited by this bacterium were similar to those observed with R. henselae. The results suggest that bacterial binding to lymphocytes may represent an additional parameter to support diagnosis of CSD.

Adolescent↗
PubMed1993

Association of congenital afibrinogenemia and K-dependent protein C deficiency--a case report.

The authors describe a rare case of congenital afibrinogenemia with concomitant K-dependent protein C deficit that was brought to our observation for ischemic lesions of the foot in association with fibrinogen concentrate infusions. These lesions can be attributed to the association of various factors: fibrinogen infusion without heparin coverage, microtrauma, and protein C (PC) deficit. In fact, thromboembolic complications during afibrinogenemia were previously reported usually in association with substitutive therapy, and it is also known that PC deficit predisposes to thrombotic complications. The the authors' knowledge, the case described by them is the first in which PC deficit is associated with afibrinogenemia. This association cannot be explained by a common genetic mechanism because the genes for fibrinogen and for protein C are located on different chromosomes (chromosomes 4 and 2 respectively).

Adolescent↗
PubMed1993

Effectiveness of thymostimulin and study of lymphocyte-dependent antibacterial activity in children with recurrent respiratory infections.

Recurrent respiratory infections (RRI) consist of more relapsing acute respiratory infections than the ones expected for the age [> 6 acute respiratory tract infections (RTI) per year if age is > 3 years, and > 8 acute RTI per year if age is < 3 years]. Concerning the pathogenesis of RRI, several investigations report the important role of environmental factors, early socialization and immunological dysfunctions, such as lymphocyte subpopulations alterations, IgG subclass deficiency and phagocytosis and/or opsonization deficit during acute infections. In this framework, we have studied the lymphocyte-dependent antibacterial activity (ABA) among 121 children affected by RRI. Results show a statistically significant alteration of this function in 38 children (31.4%): 19 of them exhibited an absent ABA (group 1), while in the others same function was reduced (group 2). A bovine thymic extract, thymostimulin, was administered to both groups by intramuscular injections (1 mg/kg) for a 3 month cycle. At the end of therapy we observed a statistical significant rise of ABA only in group 1 and among children aged > 3 years. Among the same patients, 33 children (86.8%) improved in terms of reduction of clinical score and better results were seen among children aged > 3 years. These data emphasize the beneficial role of thymostimulin in RRI-affected children, suggesting a transient immaturity of the immune system as one of the possible pathogenetic factor.

Adjuvants, Immunologic↗
PubMed1991

Thromboembolic risk in children with nephrotic syndrome.

The in vivo activation of the hemostatic system was evaluated in 14 children (4-13 years old) with nephrotic syndrome at different stages of the disease. The blood platelet count, beta-thromboglobulin (beta-TG), platelet factor 4 (PF4), fibrinogen, the coagulation inhibitors antithrombin III and protein C (ATIII:Ag and PC:Ag), and D-dimers were determined. Platelet number was significantly higher at the onset of the disease than in the next stages (p less than 0.05). beta-TG, PF4 and fibrinogen were significantly increased as compared with controls at the onset (p less than 0.001) and decreased progressively during the course of the disease without reaching the control values. Blood coagulation inhibitors behaved differently; PC was higher in patients than in controls at all stages (p less than 0.05) whereas ATIII values were significantly decreased at the onset (p less than 0.05), but increased during the course the disease (p less than 0.01). No changes were observed in the D-dimer plasma levels. These data suggest that the thrombotic risk in nephrotic syndrome is particularly evident at the onset of the disease, and appears to be due mainly to changes in platelet number and function, and to increased fibrinogen levels rather than to alterations of plasma anticoagulant factors.

Adolescent↗
PubMed1991

[Nuclear magnetic resonance and iron overload in thalassemia].

We assessed the iron load content in 36 beta-thalassemia patients by NMR correlating the results with serum ferritin levels. 22 of them were affected by beta-thalassemia major on hyper-transfusional regimen (Group A), 4 by beta-thalassemia intermedia (Group B) and 10 by beta-thalassemia major, who had been previously bone marrow transplanted (Group C). In A and C Groups the liver showed the lowest signal intensity on spin echo images (p less than 0.01; p less than 0.06, respectively). A significant correlation between the summation of signals obtained from all the examined organs and serum ferritin levels was observed by evaluating both all the patients globally (r = 0.78; p less than 0.001) and the A and C Group patients. This correlation was confirmed only in the liver both in all the patients (r = 0.77; p less than 0.001) and in A and C Group patients, when the signals obtained from each organ were evaluated.

Adolescent↗
PubMed1990

Unbalanced coagulation-fibrinolysis potential during L-asparaginase therapy in children with acute lymphoblastic leukaemia.

Treatment of acute lymphoblastic leukaemia (ALL) with L-asparaginase (L-asp) may be associated with thrombotic complications, but the pathogenetic mechanisms of thrombus formation and persistence remain unclear. We studied the procoagulant activity (PCA) of peripheral blood mononuclear cells and some components of the plasma fibrinolytic system in 10 children with ALL undergoing remission induction therapy which includes L-asp. Mononuclear cells obtained 14 days after starting L-asp treatment generated significantly higher amounts of PCA (identified as tissue factor) than cells isolated before the first dose of L-asp and 7 days after the cessation of L-asp administration (p less than 0.01). Augmented PCA coincided with an increase in the plasma D-dimer. The plasma levels of type 1 plasminogen activator inhibitor were found significantly elevated during L-asp therapy (p less than 0.05), whereas plasminogen levels were markedly decreased (p less than 0.05). These findings suggest that, during the course of L-asp treatment, the coagulation-fibrinolysis balance is shifted towards promotion of fibrin formation and deposition. Although it remains to be conclusively established whether L-asp per se or the concurrent administration of multiple chemotherapeutic agents is responsible for these changes, the latter could contribute to the thrombotic complications associated with remission induction therapy for ALL.

Asparaginase↗
PubMed1990

Age-specific prevalence of hepatitis B virus infection among children in an endemic area in southern Italy.

In 1989 the prevalence of hepatitis B virus markers was studied by radioimmunoassay in a sample of 1,426 healthy children, 3 to 11 years old, attending kindergarten and the primary schools in a large urban area of the Apulia Region in Southern Italy, where the hepatitis B surface antigen (HBsAg) prevalence among pregnant women is 5.6%. The overall prevalence of any hepatitis B virus marker was 3.4%, increasing from 1.7% in 3- to 5-year-old children to 5% in 10- to 11-year-old children (P less than 0.002). Prevalence was not associated with the father's years of schooling (odds ratio, 1.98; confidence interval, 95% (0.9 to 4.6] or with the family size (odds ratio, 2.96; confidence interval, (0.7 to 11.8]. The overall HBsAg prevalence was 0.8, a rate that was lower than the 5.6% found in pregnant women. The finding of only 12 HBsAg-positive children of the 1,426 tested, despite 80 of them being born to HBsAg-positive carrier mothers (on the basis of the 5.6% HBsAg prevalence among pregnant women), is probably attributable to the low proportion (5%) of hepatitis B e antigen positivity among the HBsAg-positive carrier mothers in the study area. The observed low HBV infection rate in younger age groups, which confirms recent studies in other areas of Italy, appears to be the result of several factors: improved socioeconomic conditions; decreased family size; and increased use of disposable syringes in the last few years.

Age Factors↗
PubMed1990

[The clinico-immunological evaluation of a bivalent vaccine against measles and rubella].

Prevention of measles and rubella can be obtained by a single administration of bivalent vaccine which can be added to the trivalent vaccine already practice in the USA since 1970 and in the Scandinavian Countries since 1982. We evaluated the immunological response and the clinical reactions to a bivalent vaccine against measles and rubella by the study of a control group including 29 children (17 females and 12 males) aged from a minimum of 15 months to a maximum of 9 years and 4 months. All the children who resulted not to be immune to the 2 viruses by serologic tests showed a rise in the antibody titer with 100% seroconversion, whereas in those proving to be already immune to one of the 2 viruses, a persistent antibody titer or an increase was revealed in all except one. Neither general nor local clinical reactions were observed except for two children who showed feverish measles-like reactions 8 days after the vaccine administration. These results suggest that this bivalent vaccine is highly effective, without negative effects. Furthermore associated with trivalent vaccines, it offers larger immunization opportunity mainly in children who have already been affected by mumps.

Antibodies, Viral↗
PubMed1989

[Monosomy 5 (-5), deletion of the long arm of chromosome 6 (6q) and acquisition of a chromosome 21 (+21) in a boy with acute leukemia at high-risk].

The chromosomal abnormality 6q-, associated with acute lymphatic leukemia, is often found both in T cell form and in non T non B cell forms. The absence of chromosome -5, frequently associated with acute non-lymphatic leukemia of the adult, has been rarely found in the acute non-lymphatic leukemia of the child. Trisomy of chromosome 21 is the most associated with acute non-lymphatic leukemia of the adult, has been rarely found in the acute non-lymphatic leukemia of the child. Trisomy of chromosome 21 is the most frequent alteration found in children and adult with acute lymphatic leukemia. In a child (aged 7) affected by acute lymphatic leukemia the karyotype analysis showed simultaneously the presence of the 3 above mentioned abnormalities. It will be important to evaluate later on how the association of monosomy 5 with the deletion of chromosome's 6 long arm and with an acquired chromosome 21, the last two being indexes of a favourable prognosis, can influence the clinical course of the disease.

Child↗
PubMed1989

[Bone metabolism in thalassemic children with multiple transfusions. Compartmental analysis of 99mTc-Sn-MDP kinetics].

The kinetics of 99mTc(Sn) methylene diphosphonate [99mTc(Sn)MDP] were evaluated by means of compartmental analysis in 5 normal children and 17 thalassemic subjects. Blood and urine activity were analyzed with a computer program which generates intercompartmental rate constants by an iterative least-square method providing a best-fit to the input data. The amount of tracer in each compartment (corrected for decay) was determined as a function of time. The transfer rate of 99mTc(Sn)MDP from blood to extracellular fluid and lamellar bone (K1-3) was about 20% higher in thalassemic subjects than in normal children. The transfer rate of 99mTc(Sn)MDP from blood to woven bone (K1-4) was about 57% higher in thalassemic subjects. A faster rate of spread into woven bone appears to be the major cause of higher bone uptake of 99mTc(Sn)MDP in thalassemic subjects, which suggests that in these patients bone tissue reoccupies the space previously replaced by hyperplastic marrow. Moreover, the increased bone extraction rate revealed a correlation between anemia and bone metabolic activity.

Adolescent↗
PubMed1989

[Clinico-immunologic evaluation of a trivalent vaccine against measles, rubella and mumps].

Prevention of measles, mumps and rubella can be obtained by a single administration of a trivalent vaccine as it has already been practiced in the U.S.A. since 1970 in the Scandinavian Countries since 1982. The immunological response and the clinical reactions to a new trivalent vaccine (Triviraten-Berna) against measles, mumps, and rubella has been studied in a control group including 41 children (20 females and 21 males) aged from a minimum of 16 months to a maximum of 7 years and 4 months. All the children who resulted not to be immune to the 3 viruses by serologic tests showed a rise in the antibody titer with 100% seroconversion, whereas in those resulting to be already immune to one of the 3 viruses, a persistent antibody titer or an increase was revealed. Neither general nor local clinical reactions were observed. These results suggest that this trivalent vaccine is highly effective, without negative effects and helpful for a mass prevention of such viral diseases.

Child↗
PubMed1989

[Pediatric suspensions of amoxicillin and clavulanic acid in the treatment of bacterial infections of otorhinolaryngologic importance and the upper respiratory tract].

Thirty children, 20 males and 10 females, aged 6.57 +/- 0.78 on the average (range: from 13 to 6 months), weighing 21.60 +/- 2.25 kg with bacterial infections of the upper respiratory tract and ear were admitted to the present trial. A chemotherapeutic agent based on amoxycillin and clavulanic acid in the suspension for children of 312.5 mg/5 ml (4:1 ratio) at the dosage of about 17 mg/kg in two daily administrations according to the weight of the patients was used. The therapy was prolonged for 8.87 +/- 0.30 days on the average, from a minimum of 3 to a maximum of 11 days. In the course of the treatment the symptom parameters and the objective signs progressively reduced until disappearance; the body temperature came promptly back to normal values, confirming the definite improvement and the regression of the infectious pictures. The microbiological data also fully confirmed the successful activity of the chemoantibiotic drug used. No side effects were found and, as regards local and general tolerance, no signs of an even slight impairment related to the administration of the drug were found. The final judgement expressed on the efficacy, on the basis of the more or less quick and complete symptom regression, evolution of the infectious picture and good tolerance were the following: excellent in 22 cases, good in 6, fair in 1 and not able to be evaluated in 1 case (a female patient who left off the trial).

Adolescent↗
PubMed1989

Reconstitution of normal neutrophil function in chronic granulomatous disease by bone marrow transplantation.

Allogeneic bone marrow transplantation was carried out on an 11-year-old boy with chronic granulomatous disease and severe chronic pulmonary insufficiency of restrictive type. After preparative regimen with busulfan (13 mg/kg) and cyclophosphamide (200 mg/kg), the patient received marrow cells from his HLA-identical and MLC-nonreactive sister. Durable sustained engraftment of donor hematopoietic and lymphoid populations occurred, as documented by analysis of genetic markers and complete reversal of the neutrophil function defect. No episode of infection occurred in the post-transplant course and, currently, 40 months after transplantation the patient is in excellent health and growing normally and showing an increasing improvement of his respiratory capacity. The successful outcome in this patient demonstrates that marrow transplantation is at present the only curative approach for this congenital disorder of neutrophil function.

Adolescent↗
PubMed1988

Problems in prenatal diagnosis of beta-thalassaemia by fetal blood analysis: beta-chain variant comigrating with gamma chains.

This report describes a couple at risk for beta-thalassaemia in which one spouse was heterozygous for classical high Hb A2 beta-thalassaemia while the other had the compound heterozygous state for beta+-thalassaemia and a beta-chain variant. This variant comigrates on carboxymethyl-cellulose columns (CMC) with gamma-chains, indicating that globin separation on CMC columns could not have been used for fetal diagnosis. The beta-chain variant migrates separately from the other globin chains on HPLC and the respective abnormal haemoglobin can be separated by isoelectrofocusing. Oligonucleotide hybridization showed that both parents were carriers of the beta+ IVS-1, nt 6 mutation. Prenatal diagnosis was successfully accomplished by oligonucleotide analysis on trophoblast DNA. This case indicates that an Antenatal Service should have alternative methods to CMC columns so as to carry out prenatal diagnosis of beta-thalassaemia in uncommon cases.

Blood Protein Electrophoresis↗