An unusual intrathoracic tumor: giant lipoblastoma.
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Biomedical subjects
Publications and source records attributed to A Di Cataldo.
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The present trial was designed to test the effects of G-CSF on the duration of the second phase of induction chemotherapy in children with newly diagnosed acute lymphoblastic leukemia (ALL). A total of 32 patients were assigned randomly to a group that received (14 patients; group A) or a group that did not receive (18 patients; group B) G-CSF (10 g/kg/day subcutaneously and daily) throughout of the second phase of induction therapy. One of 14 (7.1%) patients in group A and 2 of 18 (11.1%) patients in group B completed the course of chemotherapy within the planned time. The median length of this phase was 37 days (range, 29 to 65; mean, 40; SD, 8.6) for patients in group A and 36 days (range, from 29 to 55; mean, 38; SD, 7.4) for those in group B, and the difference was not statistically significant. The number of days during which patients had granulocyte counts of less than 2 x 10(9)/l, the number of febrile episodes of unknown origin, the number of bacterial and fungal infections and the number of days of hospitalization did not differ in a statistically significant manner between the two groups. Our data suggest that G-CSF supportive therapy may be unnecessary in children with neutropenia of short duration, for whom the risk of infection is low.
PURPOSE: As part of a study on the pharmacokinetics associated with the administration of asparaginase (ASNase) from Erwinia to the CNS, we determined the levels of asparagine in the CSF of children with acute lymphoblastic leukemia (ALL). PATIENTS AND METHODS: Twenty children received eight standard doses of intramuscular ASNase (10,000 IU/m2) every 3 days as part of induction therapy. In the postremission phase of therapy, the children were randomized to receive either 20 courses of high-dose intramuscular ASNase (25,000 IU/m2) weekly (n = 8) or four courses of standard-dose intramuscular ASNase (10,000 IU/m2) every 3 days (n = 12). RESULTS: All patients had detectable levels of L-asparagine in the CSF at the time of diagnosis. The levels of L-asparagine in CSF were undetectable in 15 of 20 (75%) and in seven of 19 (36.8%) children 3 and 5 days, respectively, after administration of standard-dose ASNase. After administration of high-dose ASNase, the levels of L-asparagine in the CSF were undetectable in five (62.5%) and two (25%) of eight children after 3 and 5 days, respectively. CONCLUSION: In this study 60% to 70% and 25% to 35% of children had complete depletion of L-asparagine from the CSF after 3 and 5 days, respectively, after administration of both schedules of ASNase from Erwinia. In the remaining patients, administration of ASNase may have resulted in a suboptimal antileukemic effect at the CNS level.
OBJECTIVES: For several years, children in Sicily with acute lymphoblastic leukaemia have been treated locally at the University of Catania. We compared the results of locally treated children with the results obtained at other centres. METHODS: The diagnosis of acute lymphoblastic leukaemia (ALL) was made in 78 children in the haematology and oncology division of the University of Catania, Sicily, From January 1987 to December 1992. Patients diagnosed before December 1990 were treated with the protocol ALL 87 including prednisone, vincristine, daunorubicine, L-asparaginase and intrathecal methotrexate. Total duration of treatment was 25-26 months. For patients diagnosed after December 1990, the protocol 90-91 used the same drugs for induction and three intrathecal drugs (methotrexate, cytarabine and prednisone) as well as cyclophosphamide to prolong induction in intermediary risk patients. Only high risk patients received cerebral irradiation. Total duration of treatment was 2 years. Full follow-up data were available for 76 patients. RESULTS: Survival rate without relapse was 66% and the 5-year survival rate was 82.7%. These results were comparable with those published by other international groups. In addition, particular attention was given to psychological support to decrease the deleterious effects of both the disease and the treatment protocols. CONCLUSION: These results demonstrate that ALL children can be cared for locally in Sicily without risking poorer outcome. It would be preferable to treat these children as near as possible to their area of residence in order to diminish the psychological trauma resulting from long-term hospitalization far from their family.
A group of 90 patients with acute lymphoblastic leukaemia (ALL) in first continuous complete remission (CCR), admitted in our hospital between January 1986 and September 1992, were tested for the presence of antibodies against hepatitis C virus (HCV), antibodies against hepatitis B virus and antibodies against HIV-1 during maintenance therapy or thereafter. They were compared with a group of 71 children with other malignancies in first CCR who had been diagnosed consecutively from January 1986 to September 1992. No patient with ALL or any other malignancy was found to be positive for hepatitis B surface antigen or HIV-1. HCV-specific antibodies were detected in 28 out of 87 children (32.1%) with ALL and in 4 out of 44 patients (9%) with malignancies other than ALL who had received at least one transfusion of blood or platelets (P < 0.01). HCV-specific antibodies were also detected in one out of three untransfused children with ALL but in none of the untransfused children with malignancies other than ALL. HCV-specific seropositivity influenced the management of children with ALL during maintenance therapy. In fact, as a result of abnormal liver function tests, maintenance therapy had to be suspended significantly more often in the case of HCV-seropositive patients with ALL than in HCV-seronegative ones. Despite the high morbidity during maintenance therapy, chronic liver disease (CLD) was uncommon in both groups: five children with ALL (17.2% of HCV-seropositive children) and one child with a malignancy other than ALL (25%) had CLD.(ABSTRACT TRUNCATED AT 250 WORDS)
The short bowel syndrome is a multifactorial disease that requires an intensive treatment especially if complication develop. The authors report their experience in a patient who, affected by acute renal failure after extensive intestinal resection, underwent temporary jejunostomy. The treatment of choice in this patient was initially a careful hydroelectrolytic balance, as he was in good nutritional status but underwent a dialytic treatment and jejunostomy. Then we started the nutritional support and initially we adopted enteral nutrition in order to facilitate small bowel functional integrity, avoid gastric uremic lesions and bacterial translocation which could prelude to multiple organ failure. At surgery we tried to preserve the distal ileum which is very important in order to absorb biliary salts and nutritional mixtures. Cholecystectomy was associated due to gangrenous cholecystitis probably caused both by short bowel syndrome and prolonged artificial nutrition. Six months after surgery the patient underwent a decrease of the body weight and then authors verified the stabilization of the weight and general conditions.
We evaluated the iron status of 50 Sicilian patients with G6PD deficiency under steady-state conditions and compared our results with those for 50 control patients. We studied haemolysis and iron indices to evaluate the iron balance. These patients could be considered to be at risk of iron overload as a result of increased bone marrow activity. Reticulocytosis and macrocytosis with reduced levels of haptoglobin were found in the G6PD-deficient subjects, both of which are evidence of a moderate haemolysis. Iron status within the normal range, without iron overload or iron deficiency, was found.
PURPOSE: To define factors that influence outcome in children with localized but unresectable neuroblastoma by retrospective investigation of response to therapy and outcome in 21 Italian institutions. PATIENTS AND METHODS: Of 145 assessable children diagnosed between 1979 and 1990, 77 were treated between 1979 and 1984 with three consecutive standard-dose (SD) protocols, and 68 between 1985 and 1990 with a high-dose (HD) protocol. All protocols included chemotherapy, followed by resection of primary tumor if feasible. If at least partial resection was achieved, consolidation therapy followed, except that from 1985 onward, patients considered disease-free following surgery received no further treatment. RESULTS: Ninety-four of 145 patients (65%) achieved a complete response (CR) or partial response (PR) with chemotherapy and 75 (52%) subsequently underwent complete resection of the primary tumor. Eighty-one patients are alive (73 without disease, eight with disease), 63 have died, and one is lost to follow-up. The 5-year overall survival (OS) rate is 55% and progression-free survival (PFS) rate 50%. Both OS and PFS correlated with response to chemotherapy, removal of primary tumor, HD therapy, and serum lactate dehydrogenase (LDH) levels. Infants (< 1 year), independent of primary tumor site, and children aged 1 to 15 years with a nonabdominal primary tumor, did better compared with children aged 1 to 15 years with an abdominal primary tumor (PFS, 72% and 64% v 30%; P < .001 and < .01, respectively). Outcome of this last group improved with the HD protocol (PFS, 40% v 23%; P = .01). CONCLUSION: In children with unresectable neuroblastoma, risk categories can be defined by age and primary tumor site. HD chemotherapy should be investigated for the poor-risk category age 1 to 15 years with an abdominal primary tumor.
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The authors report their experimental studies on early nutritional changes in 30 gastrectomized rats where intestinal continuity was restored by three different reconstruction methods, i.e., exclusion of the duodenum from alimentary transit (Sweet-Allen method), or duodenal recanalization (Longmire method) or double duodenal and jejunal recanalization (Moricca method). Ten sham operated rats were used as controls. Results showed that the group of rats which underwent Longmire's reconstruction presented better nutritional parameters (body weight gain, daily food intake, feeding efficiency, albuminemia) than the Moricca and Sweet-Allen reconstruction groups. These results became statistically significant when follow up was extended to 18 months by using an actuarial method. However, perioperative mortality rate was highest in the Longmire reconstruction group.
Splenic abscess is rare and may be present either as a localized area in the spleen or as part of a generalized sepsis. A 35 year old man presented with a two month history of anorexia, weight loss, fever, abdominal pain and arthralgia. Multiple abscesses localized in the spleen were diagnosed by CT and splenectomy was performed.
Differentiation of some abnormal haemoglobins, such as Hb C, O-Arab, Agenogi, E, O-Indonesia, C-Harlem, and Siriraj, is difficult and quantitation of the various fractions is impossible with cellulose acetate electrophoresis. The authors report 13 cases of Hb C, 10 of Hb O-Arab and 5 of Hb Agenogi whose haemoglobin fractions were quantitated by HPLC during a thalassaemia screening programme. Hb F was determined by both Betke's method and HPLC. Analysis of data by linear regression demonstrates that the methods furnish overlapping results. Our findings show that HPLC is a rapid and easily reproduced method which allows quantitative and qualitative discrimination of the various haemoglobin fractions, making it a valid tool in screening programmes for haemoglobinopathies.
The percentage of Leu M3+DR+ and of Leu M3+CD25+ cells was determined by means of immunofluorescence analysis in a group of patients with insulin dependent diabetes mellitus (IDDM). Our results show that an increased percentage of these cells may occur in the early stage of the disease. These data provide evidence for a "phenotypical" activation of Leu M3+ cells at the onset of the disease and warrant future studies to evaluate the potential role of these cells in the pathogenesis of IDDM.
The correlation between hyperammonemia, during porto-systemic encephalopathy, and brain's lesions in patients died for porto-systemic encephalopathy is not demonstrated. The aim of this study has been to try a demonstration. A histological study of the brain, cerebellum, brainstem, and spinal cord was made in 60 rats: in 40 rats 1, 3 and 6 months after portocaval shunt, and in 20 rats sham operated. The brain, cerebellum, brainsteam and spinal cord have been fixed with paraformaldehyde (4%) and then sectioned for optical and electronic microscopic study. Ammonemia was measured regularly in the 40 rats with portocaval shunt, all the rats have been hyperammonemia since 10 weeks, after this period in 4 rats ammonemia was normal. In 20 rats sham-operated ammonemia was always normal. One month after surgery electronic microscopy revealed changes in the astrocytes characterized by nuclear swelling and lobulation. Three months after surgery this lesion was increased. After six months most lesions were noted in the hyperammoniemic rats. No similar lesions were observed in control rats. These results suggest that hyperammonemia is responsible of nuclear changes in the astrocytes of the patients died from hepatic encephalopathy.
A sample population of 517 subjects was screened in order to evaluate the incidence of idiopathic varicocele. Subjects were examined clinically and underwent tests using a Doppler flow-meter. Following a brief introduction to the didymoepididymal disease, the findings of the study are reported. In conclusion, the important role of orthostasis as a causal factor leading to the onset of disease in subjects with a predisposition to diathesis is underlined.
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The onset of severe episodes of sepsis in splenectomized patients has induced surgeons to perform conservative operations in order to save at least partial functioning of the spleen. Recently, great interest has been focused on the non-operative treatment of traumatic lesions of the spleen, which involves careful and continual observation of patients with splenic nauma to monitor their course towards recovery. This method of treatment was recently adopted by our department for two patients whose case histories are presented herein. Non-operative treatment is suitable only in strictly selected subjects, who must be carefully monitored in an intensive care unit where constant examination and sophisticated diagnostic procedures are readily available. Although the non-operative treatment of splenic trauma is not new, it is only recently that its management, programming and role have been updated.
The authors pointed out that contamination may be exogenous, as a result of invasive diagnostic techniques, patient preparation, surgery, catheter insertion and wound dressing, or endogenous, especially in patients with specific risk factors (age, metabolic disorder, malnutrition, immunodeficiency) and aspecific risk factors (anesthesia, blood transfusion, surgery). Pharmacologic prophylaxis of infection may be unspecific (artificial nutrients, anticoagulants, immunomodulators) or specific (antibiotics). Prophylaxis is indicated in clean-contaminated and contaminated surgery; antibiotic chemoprophylaxis is also indicated in risk patients and permanent prosthesis surgery. The authors emphasized that antibiotics are no substitution for careful surgery.