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A Fiorillo

Publications and source records attributed to A Fiorillo.

At least 19 recordsLinked to original sources

Mortality from second tumour among long-term survivors of retinoblastoma: a retrospective analysis of the Italian retinoblastoma registry.

Survivors of retinoblastoma (Rb) are at high risk of dying from second malignant tumour. The occurrence of second malignant neoplasm (SMN) and related mortality in a cohort of 1111 cases from the Italian Retinoblastoma Registry was analysed, considering the possible role of both genetic and iatrogenic causes. Rb patients had a greater than 10-fold excess in overall mortality compared with the general population (standardized mortality ratio (SMR) 10.73, 95% CI 9.00-12.80). Their excess risk attributable to cancers other than Rb was 14.93 95% CI 10.38-21.49). Survivors of hereditary Rb had an SMR for all causes of 16.25 (95% CI 13.20-20.00), whereas their SMR for all cancers was 25.72 (95% CI 17.38-38.07). Survivors of unilateral sporadic Rb had an SMR of 4.12 from all cancers (95% CI 1.55-10.98) and a much higher excess for overall mortality (SMR 13.34, 95% CI 10.74-16.56). As expected, survivors of hereditary Rb had higher mortality from cancers of the bone (SMR 391.90, 95% CI 203.90-753.20) and soft tissue (SMR 453.00, 95% CI 203.50-1008.40), small intestine (SMR 1375.50, 95% CI 344.00-5499.70), nasal cavity (SMR 13.71, 95% CI 1.93-97.35) and cancers of the brain and central nervous system (SMR 41.14, 95% CI 13.2-127.55).

Cohort Studies↗

Family burden and social network in schizophrenia vs. physical diseases: preliminary results from an Italian national study.

OBJECTIVE: To compare burden and social network in families of patients with schizophrenia or physical diseases. METHOD: A total of 709 relatives of patients with schizophrenia and 646 relatives of patients with physical diseases were recruited in 169 specialized units located in 30 randomly selected Italian geographical areas. RESULTS: In both groups, the consequences of caregiving most frequently reported as present were constraints in social activities, negative effects on family life and a feeling of loss. Objective burden was similar in the two groups, while subjective burden was higher in schizophrenia. Social support was lower among relatives of patients with schizophrenia than among those of the other group. CONCLUSION: These results highlight the need to provide families of those with long-term diseases with supportive interventions, aiming to: i) manage relatives' psychological reactions to patient's illness; ii) provide information on patient's disease; iii) reinforce relatives' social network, especially in the case of schizophrenia.

Adolescent↗

The impact of professional and social network support on the burden of families of patients with schizophrenia in Italy.

OBJECTIVE: (a) To explore burden related to caregiving and support received from professionals and social network in relatives of patients with schizophrenia in Northern, Central and Southern Italy; and (b) to test whether a higher level of family burden is associated with a lower level of professional and social network support. METHOD: Seven hundred and nine patients with schizophrenia and their key-relatives were consecutively recruited in 30 Italian mental health departments. Data were collected on: (a) patients' clinical status and levels of disability; (b) relatives' burden, social and professional support; (c) interventions received by patients and their families. RESULTS: Family burden was found lower in Northern Italy. However, after controlling for psychosocial interventions, differences in family burden among the three geographical areas disappeared. Family burden was associated with patients' levels of disability and manic/hostility symptoms, and with professional and social network support received by the family. CONCLUSION: Professional and social network support represent crucial resources to reduce family burden in schizophrenia.

Adult↗

Shunt-related abdominal metastases in an infant with medulloblastoma: long-term remission by systemic chemotherapy and surgery.

This is the first reported case of long remission of abdominal metastases spread through a ventriculo-peritoneal shunt in an infant diagnosed, four years ago, at age 1 year and 10 months, to have cerebral medulloblastoma. Two years later, while in second complete remission of his cerebral tumor, he showed abdominal metastases, successfully treated by platinum based chemotherapy and surgery. One year later, a second abdominal relapse and hepatic metastases were treated by doxorubicin administration and surgery. Since then the child remained in continuous complete remission. This unusual favorable outcome can be explained by an extreme responsiveness of the tumor, unprotected by the blood brain barrier, to systemic chemotherapy, particularly to doxorubicin administration. The need for careful surveillance of patients with ventriculo-peritoneal shunts is emphasized. Searching for new tools, such as entrapment of doxorubicin in liposomes, able to overcome the blood-brain barrier and to expose brain tumors to effective drugs, probably represents the best choice for future treatment strategies of CNS tumors.

Antineoplastic Combined Chemotherapy Protocols↗

Cardiac betaARK1 upregulation induced by chronic salt deprivation in rats.

The beta-adrenergic receptor (betaAR) kinase (betaARK1) is a G protein-coupled receptor kinase (GRK) that controls cardiac betaAR signaling via receptor phosphorylation, leading to desensitization. We have observed in mice that chronic isoproterenol administration results in increased myocardial levels of betaARK1 activity, suggesting that adrenergic activation can regulate cardiac betaARK1 expression. Thus, we evaluated left ventricular (LV) betaARK1 levels and activity in response to 3 weeks of a low-sodium (0.05%) diet, which is known to chronically activate the sympathetic nervous system. Wistar-Kyoto rats were subjected to either low or regular sodium (2%) intake. To prove the association of betaARK1 expression and low sodium-induced adrenergic activation, a group of rats was subjected to atenolol treatment (1 mg/kg per day) during the low-sodium diet. LV betaARK1 expression was assessed by protein immunoblotting and betaARK1 activity by in vitro GRK phosphorylation assays. We verified the LV protein levels of GRK5, which is abundantly expressed in the heart. A low-sodium diet reduced body weight and cardiac size so that the heart-to-body weight ratio did not change. On the contrary, low-sodium diet increased by 50% both LV betaARK1 protein (densitometry units: normal sodium, 26.5+/-0.9; low sodium, 35.7+/-1.6; P<0.05) and activity (fmol/mg per minute: normal sodium, 6.49+/-1.17; low sodium, 9.15+/-0.93; P<0.05). Atenolol treatment prevented the increase in both protein expression (low sodium plus atenolol, 27.6+/-5.33, P=NS versus normal sodium) and activity (6.54+/-1.19, P=NS versus normal sodium). GRK5 expression was not affected by a low-sodium diet (17.2+/-0.2 versus 18.4+/-0.4, P=NS). Our data indicate that cardiac betaARK1 is regulated by sympathetic action on betaARs as tested by reducing dietary salt and betaAR blockade.

Adrenergic beta-Antagonists↗

A multicenter Italian study of patients' relatives' beliefs about schizophrenia.

This study explored patients' relatives' beliefs about schizophrenia in Italy, a country with a long history of community-based psychiatry. The study was conducted among 709 key relatives of patients with schizophrenia who were recruited in 30 mental health departments. Relatives seemed to believe that mental hospitals are not appropriate places in which to treat their loved ones, even though most of them felt that they were alone in coping with the burden of their ill relative's disease. Most of the relatives agreed that people who have schizophrenia should be allowed to vote, but they appeared to have a stricter attitude toward affective rights, such as the patient's right to get married and have children, and tended to perceive their loved ones as being subject to social discrimination. The findings of this study may be useful for developing psychoeducational intervention and sensitization campaigns for the general population.

Adult↗

Longitudinal assessment of cardiac status by echocardiographic evaluation of left ventricular diastolic function in thalassaemic children.

Cardiac disease is the primary cause of death in patients affected by thalassaemia major. In most cases diastolic dysfunction precedes the onset of systolic impairment at a time when appropriate therapy can prevent progression of cardiac damage. We have assessed the pattern of left ventricular filling by Doppler echocardiography in six transfusion-dependent thalassaemic patients. They were re-evaluated 5 y after the first observation, after additional blood transfusions and consequent iron load. We registered a significantly impaired relaxation pattern in all of them. A state of cardiac anoxia has been hypothesized.

Adolescent↗

Radiation late effects in children treated for orbital rhabdomyosarcoma.

BACKGROUND AND PURPOSE: The experience resulting from large cooperative studies shows that correct radiation therapy at doses adequate to the tumor bulk are crucial for local control of rhabdomyosarcoma. The aim of the present study was to document the correlation between modalities and doses of radiotherapy and radiation side effects. PATIENTS AND METHODS: Between 1980 and 1997, 19 patients affected by primary orbital rhabdomyosarcoma have been followed at the University Federico II of Naples. All but three patients, who received 45, 54 and 55 Gy respectively, have been treated by immediate radiation at the dose of 60 Gy, delivered in 2 Gy fractions, five times per week, by cobalt 60 megavoltage equipment. Combined chemotherapy using vincristine and vincristine plus dactinomycin on alternate weeks was also administered as part of induction therapy. RESULTS: An overall survival rate of 94.7% was registered. In our patients the majority of radiation late effects were paid by orbit and ocular adnexa. Side effects to lens and ocular structures were fewer and of low grade. CONCLUSIONS: Radiation therapy is still essential for local control of orbital rhabdomyosarcoma, however radiation side effects have to be carefully considered together with the therapeutic goal to be obtained.

Child↗

Family burden and coping strategies in schizophrenia: are key relatives really different to other relatives?

Subjective and objective burden, psychiatric symptoms and coping strategies in a sample of 90 key relatives and other relatives of patients with schizophrenia, living in two European countries, were explored by means of well-validated questionnaires. The levels of burden on key relatives did not differ significantly from those on other relatives. Moreover, the risk of developing psychiatric symptoms was similar in the two subject groups at both centres. Significant correlations were found between key relatives and other relatives concerning the adoption of emotion-focused coping strategies. These data contrast with the current belief that family burden in schizophrenia is mainly a burden of key relatives, and they emphasize the need to provide supportive interventions for as many relatives as possible.

Adaptation, Psychological↗

Primary Ewing's sarcoma of the maxilla, a rare and curable localization: report of two new cases, successfully treated by radiotherapy and systemic chemotherapy.

Primary maxillary localization of Ewing's sarcoma is unusual. Involvement of facial bones is characterized by clinical and radiological features distinct from those commonly observed in other sites. Because of the above peculiarities a delay in diagnosis and thus in starting treatment is very probable in such cases. We report here two new cases of Ewing's sarcoma localized to facial bones, successfully treated by local high dosage radiotherapy and systemic chemotherapy. Our experience suggests that, especially for particular sites not suitable to radical surgery, radiation therapy can represent an effective tool to achieve local control of the tumor.

Adult↗

Interstitial 9q deletion is associated with CD7+ acute leukemia of myeloid and T lymphoid lineage.

We report the clinical, hematological and immunophenotypic characteristics from four cases of acute leukemia with interstitial deletion of chromosome 9, ie del(9)(q12-q22), as a single chromosomal abnormality. Three patients had acute myeloblastic leukemia (AML) and one T origin acute lymphoblastic leukemia (ALL). According to FAB classification, blasts were classified as M1 (two patients), M2 (one patient), and L2 (one patient). In two out of three AML cases a myelodysplastic syndrome, one AREB-t and one AREB diagnosed 6 and 11 months before respectively, preceded the onset of AML. Morphological examination showed dysgranulopoiesis, dyserythropoiesis and cytoplasmic vacuoles in two AML patients, while a strong positivity to myeloperoxidases was observed in all AML cases. As concerns immunophenotypic findings, blast cells from two of three AML patients expressed CD7 and CD34, while those from the T-ALL case displayed CD33 and CD34 along with CD7. These observations suggest that del (9q) is associated with CD7+ acute leukemia of myeloid or lymphoid lineage.

Adolescent↗

A novel chromosomal abnormality detected in a case of acute myelomonocytic leukemia.

A not yet recorded translocation, t(3:17)(q21;q23), detected in a case of acute myelomonocytic leukemia, is reported. In spite of an aggressive cytotoxic chemotherapy, the disease showed a rapid fatal course, confirming the dismal prognostic significance of structural, 'primary' chromosomal abnormalities in acute leukemias.

Chromosomes, Human, Pair 17↗

Unusual seizures with a benign course in a case of acute lymphoblastic leukemia of childhood.

We report the unusual seizures in a patient with acute lymphoblastic leukemia. The convulsive disorder began acutely as partial somatomotor status epilepticus and with diffuse EEG slowing. The seizures then became myoclonic-atonic (drop attacks) and an EEG focus appeared on the left paramedian centro-parietal areas, activated by proprioceptive stimuli. Despite the severity of the clinical picture at onset, the seizures showed a benign course, and disappeared on carbamazepine therapy. We think that this epileptic syndrome may have been caused by diffuse iatrogenic encephalopathy, probably related to intrathecal methotrexate therapy.

Carbamazepine↗

Iron absorption and iron deficiency in infants and children with gastrointestinal diseases.

Iron status, iron absorption, and intestinal blood loss were studied in 199 children undergoing diagnostic evaluation for suspected malabsorption. Evaluation of iron status included hematological indices, serum ferritin, and transferrin saturation. Iron absorption was assessed by the increment of serum iron after an oral iron load. Iron deficiency was common among patients affected by malabsorptive states, such as celiac disease (84%), cow's milk intolerance (76%), Crohn's disease (72%), and giardiasis (64%), whereas it was less common among patients with postinfectious enteritis (41%) and chronic nonspecific diarrhea (11%). Intestinal blood loss was seen only in patients with Crohn's disease and cow's milk intolerance, irrespective of iron nutritional status. On the other hand, iron malabsorption was very common, affecting 85-95% of the iron-deficient patients in all diagnostic groups, except in chronic nonspecific diarrhea. Iron malabsorption was less common among patients with adequate iron nutritional status than in those with iron deficiency. Iron malabsorption appears to play a major role in the pathogenesis of iron deficiency in patients with malabsorption. The iron absorption test shows greater sensitivity as a screening test for upper intestinal malabsorption than the D-xylose absorption test.

Adolescent↗

Abnormal endogenous carbon monoxide production in children with ineffective erythropoiesis.

The aim of this study was to evaluate the extent of endogenous carbon monoxide production in subjects with ineffective erythropoiesis. Carboxyhaemoglobin concentrations were measured in eight transfused and eight nontransfused thalassemic patients. Measurements were made on venous blood collected under controlled conditions and were corrected by simultaneous measurement of HbF levels. Nontransfused thalassaemic patients with abnormal erythropoiesis were found to have elevated levels of carboxyhaemoglobin, significantly higher than those found in transfused subjects, as well as decreased cardiac performance. It is important to consider carboxyhaemoglobin levels in planning transfusion programmes.

Adolescent↗

Multidisciplinary treatment of primary orbital rhabdomyosarcoma. A single-institution experience.

Orbital rhabdomyosarcoma accounts for one-fourth of the primary tumors in the head and neck region. Modern treatment modalities have led to a 2-year survival rate of about 90% in these patients. However, new therapeutic trials are designed to reduce complications and salvage more than 90% of orbital cases. Between 1979 and 1990, 12 children affected by primary orbital rhabdomyosarcoma have been diagnosed and treated at the University of Naples. Ten of them have been uniformly treated by biopsy, followed by immediate radiation and combined chemotherapy. All 12 patients are alive and free of detectable disease, from a minimum of 7 months to a maximum of 123 months after diagnosis. In all children, ocular structures have been spared and the complications observed until now have been few. The above results suggest that the association of immediate radiation therapy and chemotherapy might represent an optimal tool for treatment of orbital rhabdomyosarcoma.

Adolescent↗