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Biomedical subjects

G Leverger

Publications and source records attributed to G Leverger.

At least 37 records · Page 2Linked to original sources
PubMed2005

Hematopoietic stem cell transplantation for Shwachman-Diamond syndrome: experience of the French neutropenia registry.

Our objective was to study the outcome of allogeneic hematopoietic stem cell transplantation (HSCT) for Shwachman-Diamond Syndrome (SDS). Among 71 SDS patients included in the French Severe Chronic Neutropenia Registry, 10 received HSCT between 1987 and 2004 in five institutions. The indications were bone marrow failure in five cases, and myelodysplastic syndrome (MDS) or leukemia in five cases. The median follow-up of patients who survived without relapse is 6.9 years (3.1-16.8 years). The conditioning regimen consisted of a busulfan-cyclophosphamide combination (n=6) or total body irradiation plus chemotherapy (n=4). Six patients received stem cells from unrelated donors and four from identical siblings. Engraftment was complete in eight patients and unassessable in two patients. These latter two patients died of infections 32 and 36 days after HSCT, with grade IV graft-versus-host disease and multiorgan dysfunction. A third patient died from an acute respiratory distress syndrome 17 months after HSCT with progressive granulocytic sarcoma. One patient had an MDS relapse 4 months after HSCT and died 10 months later. The overall 5-year event-free survival rate is 60+/-15%. We conclude that HSCT is feasible for patients with SDS who develop bone marrow failure or malignant transformation.

Adolescent↗
PubMed2005

Treatment of childhood acute myeloblastic leukemia: dose intensification improves outcome and maintenance therapy is of no benefit--multicenter studies of the French LAME (Leucémie Aiguë Myéloblastique Enfant) Cooperative Group.

From 1989 to 1998, 341 children were included in the French multicentric LAME (Leucémie Aiguë Myéloblastique Enfant) trials. A total of 309 children were registered in the LAME 89/91 protocol. This intensive regimen included an induction phase (mitoxantrone plus cytarabine), two consolidation courses, one containing timed-sequential high-dose cytarabine, asparaginase and amsacrine; 276 (90%) achieved a CR. The 5-year overall survival (OS) and event-free survival (EFS) were 60+/-4 and 48+/-4%, respectively. From 1997, timed-sequencing of the LAME SP induction chemotherapy led to an unacceptable frequency of consolidation delay; future improvements are unlikely to come from further increases in intensity. The role of allogenic bone-marrow transplantation from an HLA-identical sibling in CR1 was examined. The disease-free survival (DFS) was 52+/-4% for non-allografted patients and 57+/-7% for allografted patients (P=NS); a better OS for allografted patients was shown and could be related either to allo-BMT early in CR1 or to a second allo-BMT in CR2. For the complete responders after consolidation therapy, the 5-year OS was significantly better in patients randomized for no maintenance therapy (MT-) than in patients randomized for MT (77.6+/-8 vs 59+/-8%; P=0.05), while the 5-year DFS was not significantly different. Exposure to low-dose MT might contribute to clinical drug resistance and treatment failure in relapsing patients.

Adolescent↗
PubMed2004

Outcome of childhood acute promyelocytic leukemia with all-trans-retinoic acid and chemotherapy.

PURPOSE: To determine the results of treatment combining all-trans-retinoic acid (ATRA) and chemotherapy (CT) in childhood acute promyelocytic leukemia (APL). PATIENTS AND METHODS: Children (< 18 years) with newly diagnosed APL were included in the APL93 trial, treated by ATRA followed or combined with daunorubicin-cytarabine, and then randomly assigned between no maintenance, intermittent ATRA, continuous CT, or both. RESULTS: Of the 576 patients included in APL93 trial, 31 (5%) were children, including 22 girls (71%) and nine boys (29%). Thirty of the children (97%) obtained complete remission (CR). ATRA syndrome occurred in four children (13%), who all achieved CR, and headaches occurred in 12 children (39%), with signs of pseudotumor cerebri in five children (16%). Seven patients (23%) relapsed. None of the eight patients who received both ATRA and CT for maintenance relapsed. All relapsing patients achieved a second CR. Twenty-two patients remained in first CR after 43+ to 96+ months, six remained in second CR after 17+ to 66+ months, and three patients had died. The 5-year event-free survival (EFS), relapse, and overall survival rates were 71%, 27%, and 90%, respectively. No difference between adults and children included in the APL93 trial was seen for CR rate, 5-year relapse rate, EFS, and overall survival, but significantly better survival was seen in children after adjustment on WBC counts (P =.02) and incidence of microgranular M3 variant (P =.04). CONCLUSION: ATRA combined with CT for induction and also probably for maintenance provides as favorable results in children with APL as in adults and currently constitutes the reference first-line treatment in both age groups.

Adolescent↗
PubMed2004

Carboplatin-epirubicin regimen for the treatment of hepatoblastoma.

BACKGROUND: In order to lower the long-term toxicity of chemotherapy for hepatoblastoma patients, a prospective study was designed based on pre-operative chemotherapy combining carboplatin and epirubicin (CE). PROCEDURES: Patients under 16 years of age with an epithelial hepatic tumor diagnosed by ultrasound or CT scan and a high serum alpha-foetoprotein (AFP) level were eligible. Patients were treated with a pre-operative chemotherapy regimen combining carboplatin 600 mg/m(2) and epirubicin 80 mg/m(2). Tumor resectability was assessed after four courses given at 3-week intervals. After surgery, patients were given two more courses of CE. Response was assessed based on a drop in serum AFP and tumor shrinkage. RESULTS: Between July 1988 and August 1995, 27 patients with a hepatoblastoma were included. The initial PRETEXT group according to the SIOPEL classification was: group 2 (5 pts), group 3 (15 pts), group 4 (5 pts), and 2 pts were not assessed. Six patients had lung metastases. Response was partial response (PR) in 20/27 (74%) patients, disease was stable in 3 and 4 had progressive disease (PD). A complete surgical resection was performed in 21 pts. Five-year overall and disease-free survival (DFS) were respectively 56% (95%CI: 37-72%) and 63% (95%CI: 44-78%). During the same time period, 7 pts with a hepatocellular carcinoma were treated according to this protocol. Only one achieved a PR. Toxicity was mostly hematologic with > or =grade 3 leukopenia in 23% of the courses, > or =grade 3 thrombocytopenia in 29% of the courses and anemia in 22%. CONCLUSION: The CE protocol is feasible and efficient in hepatoblastoma. However, only a randomized study will permit a valid comparison of the efficacy of cisplatin and carboplatin for the treatment of these patients.

Adolescent↗
PubMed2004

Modern diagnostics in chronic myeloproliferative diseases (CMPDs).

According to the new WHO classification a group of chronic myeloproliferative diseases (CMPDs) were defined: chronic myeloid leukemia (CML), chronic neutrophilic leukemia (CNL), chronic eosinophilic leukemia and hypereosinophilic syndrome (CEL/HES), polycythemia vera (PV), chronic idiopathic myelofibrosis (with extramedullary hematopoiesis, CIMF), essential thrombocythemia (ET), and so called CMPD/unclassifiable. As clinical features and laboratory findings differ widely between these diseases several diagnostic approaches are mandatory at diagnosis for classification and are needed also for follow up studies, especially for the measurement of minimal residual disease (MRD). We here outline the laboratory set up at diagnosis and during follow up in CMPDs with specific focus on the respective therapeutical consequences. Only by using a comprehensive diagnostic panel including cytomorphology, cytogenetics, and molecular genetic methods establishing the correct diagnosis, optimizing treatment as well as evaluating treatment response is possible in CMPDs today.

Blood Cell Count↗
PubMed2004

Piperacillin-tazobactam and netilmicin as a safe and efficacious empirical treatment of febrile neutropenic children.

GOALS OF WORK: We evaluated piperacillin-tazobactam in association with netilmicin (TN) in the early empirical treatment of neutropenic children, as data are limited in number. PATIENTS AND METHOD: In 1996, an observational study was initiated to assess the efficacy and safety of this association, with a glycopeptide (TNG) if needed. The impact on the bacterial ecology of our unit was also observed. Children were treated for hematological malignancy or solid tumor between September 1996 and December 1998 and presented a febrile neutropenia. RESULTS: There were 148 evaluable febrile neutropenic episodes in 104 patients. Median age was 7 years, 55% of the episodes were fever of unknown origin, 22% were clinically documented and 23% microbiologically documented (27 bacteriemia). A glycopeptide was added in 67 episodes. The initial unmodified treatment was successful in 114 episodes (77%): 75/81 episodes in the TN group and 39/67 in the TNG group. For successful episodes, median treatment duration was 6 days. There were 22 febrile recurrences. These patients, as well as initial failures, always responded to a second-line treatment. One child was considered a failure because he developed a skin rash probably due to piperacillin-tazobactam and required another beta-lactamase. CONCLUSION: This study suggests that piperacillin-tazobactam in association with netilmicin presents a satisfactory efficacy and a good tolerance as empirical therapy for febrile neutropenic children. It allowed us to maintain the bacterial ecology of our unit.

Adolescent↗
PubMed2004

[Vincristine treatment for function- and life-threatening infantile hemangioma].

AIM: To evaluate the efficacy of vincristine treatment for function- and life-threatening hemangiomas. PATIENTS AND METHOD: Nine infants, eight girls and one boy, received vincristine treatment (VCR) for endangering hemangiomas. In six cases, the hemangiomas involved head and neck in a segmental unilateral or bilateral distribution (3/6 also had laryngeal and 2/6 tracheal location causing respiratory distress, 5/6 had eyelid and orbital involvement); one infant had disseminated neonatal hemangiomatosis (skin, liver, kidney); two infants had liver hemangiomas with cardiac failure. VCR was prescribed after failure of high-dosage corticosteroid treatment in six, and of both corticosteroids and interferon alpha 2b (5 months) in one; two infants received VCR as first line treatment. RESULTS: A dosage of 1 mg/m(2) IV injection was delivered, with weekly injections first, and then tapering, increasing the interval between injections, depending on the clinical response. The nine infants received from 5 to 25 injections (average: 16), for a length of treatment of 1.5-8 months (average: 5.5 months). In seven patients a clear clinical response was observed at the end of the first month of treatment, while a slow protracted response was noted in two. Transient mild side effects were present in four patients. DISCUSSION: Corticosteroid treatment, although a worldwide recognized treatment of problematic hemangiomas, cannot always control the growth of alarming hemangiomas. Interferon alpha 2a and 2b have proven a 90% effectiveness: treatment for cortico-resistant, function- and life-threatening, hemangiomas.

Antineoplastic Agents, Phytogenic↗
PubMed2004

[4S neuroblastoma with bilateral adrenal tumors].

UNLABELLED: 4S neuroblastoma with bilateral adrenal involvement is defined by small primitive tumors (stage 1 or 2) with disseminated disease restricted to the liver, skin, and/or bone marrow. Children are less than one year old. These tumors are rare and of multicentric origin. PATIENTS AND METHODS: Our multicentric study analyzed four children less than four months old at diagnosis. RESULTS: All had a favourable histology, with normal MYC-N copy number, and one case had a diploid tumor. The four patients had first supportive care at the beginning, but three cases received chemotherapy because of progressive disease, with liver radiotherapy in two cases because of massive hepatomegaly; three cases had surgery (unilateral adrenal resection in two cases and bilateral in one case) and one had only a biopsy. Surgery was the only treatment in one case. One patient relapsed 17 months after initial treatment and was treated with intensive chemotherapy and stem cell rescue. The outcome is favorable for the four patients, without evidence of recurrent disease. CONCLUSION: Children with 4S neuroblastoma with bilateral adrenal tumors have a good prognosis. Treatment should be the less aggressive as possible. The group with favorable prognostic parameters should have supportive care if spontaneous regression occurs. But we have to treat with chemotherapy neonates with massive hepatomegaly and children with one or more unfavorable prognostic factors (unfavorable histology, high MYC-N copy number).

Adrenal Gland Neoplasms↗
PubMed2004

[Role of Abelcet in children with febrile neutropenia].

AIM OF STUDY: In order to optimise the use of new forms of Amphotericine B (Ampho B), a decisional tree was created at the end of 2001 in the paediatric hemato-oncology unit for the empirical antifungal treatment in febrile neutropenic children: the standard remained conventional Ampho B and Abelcet was proposed in case of antecedent or occurrence of a deterioration of the renal function (DRF). In order to validate the place of Abelcet we initiated a retrospective study over year 2002. RESULTS: 21 treatments were begun in 14 children for a median duration of 8 days (1-48 days). Three kind of indications were found: DRF antecedent (10 episodes: A group), DRF occurrence during a treatment with conventional Ampho B (7 episodes: B group), age lower than 1 year (3 episodes). 81% of the children were thus treated according to the decisional tree. The clinical tolerance was good in 90% of the cases, with a premedication in half of the cases. The study of the renal function showed a good renal tolerance for 6 episodes out of 9 evaluable in A group, 3 resolutions and 2 stabilisation of the renal failure for the 5 evaluable episodes of the B group. Seven to ten days of treatment by Abelcet were necessary to obtain the renal failure resolved. CONCLUSION: This study confirms the interest of Abelcet in the empirical antifungal treatment in febrile neutropenic children and specially in children having antecedents of DRF related or not to a treatment with conventional Ampho B.

Amphotericin B↗
PubMed2004

Incidence of childhood leukaemia and non-Hodgkin's lymphoma in France: National Registry of Childhood Leukaemia and Lymphoma, 1990-1999.

The French National Registry of Childhood Leukaemia and Lymphoma (NRCL) covers the whole French mainland population aged less than 15 years (approximately 11 million children) for all childhood haematopoietic tumours since 1 January 1990, except Hodgkin's disease, which has been registered since 1 January 1999. During the period from 1990 to 1999, 5757 cases of leukaemia, lymphoma and myelodysplastic syndrome were registered in the NRCL, with an average of 2.5 sources per case. The age-standardized incidence rates per million per year were 43.1 for leukaemia (34.3 for acute lymphoblastic leukaemia, 7.1 for acute myeloblastic leukaemia, 0.6 for chronic myeloid leukaemia and 0.5 for chronic myelomonocytic leukaemia), 8.9 for non-Hodgkin's lymphomas and 6.7 for Hodgkin's disease. Down's syndrome was present in 110 cases of acute leukaemia (2.5%) and three cases of non-Hodgkin's lymphoma (0.3%). The incidence of acute lymphoblastic leukaemia showed a typical peak at age 2 years for girls and 3 years for boys. The incidence rates of leukaemia and non-Hodgkin's lymphoma did not show any temporal trends over the 10 year period.

Adolescent↗
PubMed2004

Acute childhood leukaemia and environmental exposure to potential sources of benzene and other hydrocarbons; a case-control study.

AIM: To analyse the association between potential environmental exposure to hydrocarbons and the risk of acute childhood leukaemia. METHODS: A hospital based multicentre case control study, stratified on centre, age, and sex, with 280 leukaemia cases and 285 controls was carried out. Data were collected by a standardised interview of the mothers. RESULTS: No clear association was seen between maternal occupational exposure to hydrocarbons during pregnancy and leukaemia, or between residential traffic density and leukaemia. There was an association between dwellings neighbouring a petrol station or a repair garage during childhood and the risk of childhood leukaemia (OR 4.0, 95% CI 1.5 to 10.3), with a duration trend. The association, which appeared particularly strong for acute non-lymphocytic leukaemia (OR 7.7, 95% CI 1.7 to 34.3), was not altered by adjustment for potential confounding factors. CONCLUSIONS: Results showed an association between acute childhood leukaemia and dwellings neighbouring auto repair garages and petrol stations, which are benzene emitting sources. These findings could be due to chance, although the strength of the association and the duration trend are arguments for a causal association.

Benzene↗
PubMed2003

Lymphocyte-predominant Hodgkin's lymphoma in children: therapeutic abstention after initial lymph node resection--a Study of the French Society of Pediatric Oncology.

PURPOSE: To clarify treatment strategy for lymphocyte-predominant Hodgkin's lymphoma (LPHL), the French Society of Pediatric Oncology initiated a prospective, nonrandomized study in 1988. Patients received either standard treatment for Hodgkin's lymphoma or were not treated beyond initial adenectomy. PATIENTS AND METHODS: From 1988 to 1998, 27 patients were available for study. Twenty-four patients were male, and median age was 10 years (range, 4 to 16 years). Twenty-two, two, and three patients had stage I, II, and III disease, respectively. Thirteen patients (stage I, n = 11; stage III, n = 2) received no further treatment after initial surgical adenectomy (SA). Fourteen patients received combined treatment (CT; n = 10), involved-field radiotherapy alone (n = 1), or chemotherapy alone (n = 3). The two groups were comparable for clinical status, treatment, and follow-up. RESULTS: Twenty-three of 27 patients achieved complete remission (CR). With a median follow-up time of 70 months (range, 32 to 214 months), overall survival to date is 100%, and overall event-free survival (EFS) is 69% +/- 10% (SA, 42% +/- 16%; CT, 90% +/- 8.6%; P <.04). If we considered only the patients in CR after initial surgery (n = 12), EFS was no longer significantly different between the two groups. Patients with residual mass after initial surgery (n = 15) had worse EFS if they did not receive complementary treatment (P <.05). CONCLUSION: Although based on a small number of patients, our study showed that (1). no further therapy is a valid therapeutic approach in LPHL patient in CR after initial lymph node resection, and (2). complementary treatment diminishes relapse frequency but has no impact on survival.

Adolescent↗
PubMed2003

[Severe candidosis infections in neutropenic patient in onco-hematologic department].

Incidence of severe candidal infections is rapidly increasing since 15 years and is becoming a major concern in onco-hematology practice, especially due to its poor prognosis in neutropenic patients. Diagnosis of candidemia is suspected in case of persistent fever resistant to a large antibiotherapy and requires to search for secondary locations as cutaneous and hepatosplenic candidal infection. Improvement of yeasts detection in blood culture bottles with specific medium is now helpful but use of specific immunoserodiagnosis or PCR methods is at this point unuseful. Fluconazole and Amphotericine B remain the recommended treatments for candidemia. Indications for "new antifongal drugs" are still limited regarding their high cost and the limited clinical studies.

Candidiasis↗
PubMed2002

[(18)F]FDG in childhood lymphoma: clinical utility and impact on management.

Positron emission tomography (PET) with fluorine-18 fluorodeoxyglucose (FDG) is a very useful technique for the imaging of lymphomas in the adult population. It provides unique information about the behaviour of malignant cells and contributes to more accurate staging of the illness and better assessment of response to therapy. The purpose of this study was to evaluate the usefulness of FDG PET in childhood lymphoma compared with conventional imaging methods (CIMs) and clinical data. Between July 1998 and August 2001, 42 FDG PET examinations were performed using a dedicated PET system (27 examinations) or a hybrid coincidence PET system (15 examinations) for initial tumour staging ( n=7), restaging ( n=5) or assessment of response to therapy or residual masses ( n=30) in 27 children with Hodgkin's disease (HD) ( n=20) or non-Hodgkin's lymphoma (NHL) ( n=7). FDG PET results were compared with CIM findings and clinical data. Since 2000, a standardised questionnaire for evaluation of the clinical impact of FDG PET on both staging and therapy has been sent to the 16 referring physicians and 13 have replied. In all children, FDG PET was performed without any side-effects. FDG PET was found to be very sensitive (Se=12/12) for staging and restaging of the illness, showing more lesions than CIMs, with a 50% patient upstaging rate (6/12). It was very accurate for monitoring response to therapy and for characterisation of residual masses. False-positive results were observed in two NHL patients with thymic uptake and one false-negative result was obtained in a patient whose NHL relapsed 1 month after a negative FDG PET. The questionnaire emphasised the impact of FDG PET on clinical management, which was modified on the basis of the FDG PET results in 23% of patients. As previously demonstrated in the adult population, FDG PET appeared to be a very sensitive imaging technique for staging and restaging of lymphoma in children and was very useful for monitoring the response to therapy.

Adult↗
PubMed2002

Day-care, early common infections and childhood acute leukaemia: a multicentre French case-control study.

We conducted a case-control study to investigate the role of early infections in the aetiology of childhood acute leukaemias. The study included 280 incident cases (240 acute lymphoblastic leukaemia and 40 acute non-lymphoblastic leukaemia) and 288 hospital controls, frequency matched by age, gender, hospital, catchment area of the hospital and ethnic origin. Data were obtained from standardised face-to-face interviews of the mothers. The interviews included questions on early common infections, day-care attendance, breast-feeding, birth order and infantile diseases. Odds ratios were estimated using an unconditional regression model including the stratification variables, parental socio-economic status and perinatal characteristics. Birth order was not associated with childhood leukaemia (acute lymphoblastic or acute non-lymphoblastic). A statistically-significant inverse association was observed between childhood leukaemia and day-care attendance (odds ratio=0.6, 95% Confidence Interval=(0.4-1.0)), repeated early common infections (> or = 4 per year before age two, odds ratio=0.6 (0.4-1.0)), surgical procedures for ear-nose-throat infections before age two (odds ratio=0.5 (0.2-1.0)) and prolonged breast-feeding (> or = 6 months, odds ratio=0.5 (0.2-1.0)). In the multivariate model including day-care attendance, early common infections and breast-feeding, results concerning breast-feeding remained unchanged. A statistically significant interaction between day-care attendance and repeated early common infections was observed. When the interaction was taken into account, the simple effects of day-care and early common infections disappeared (odds ratio=1.1 (0.5-2.3) and odds ratio=0.8 (0.5-1.3), respectively) while the joint effect of day-care attendance and early common infections was negatively associated with childhood leukaemia (odds ratio=0.3 (0.1-0.8)). All the above associations were observed both for acute lymphoblastic leukaemia and acute non-lymphoblastic leukaemia. Our results support Greaves' hypothesis, even though they are not specific of common leukaemia.

Breast Feeding↗
PubMed2002

[Ethical dilemmas in the sharing of information in the care of HIV-infected children].

Ethical dilemmas are regularly encountered in the care of patients infected with HIV (Human Immunodeficiency Virus), especially in issues regarding professional confidentiality and shared information with the child and his/her family as well as among professional health workers. This communicable disease, which can be treated but not yet cured, leads to exclusion, due to the fear of infection, to its severity and induces stigmatisation of affected individuals. It interferes with life projects, sexuality, reproductive health, family life, projection into the future. In HIV paediatrics, practitioners regularly deal with complex situations and need to balance parental authority and children's needs/dependence, respect of patient's confidentiality and hazards to another person's health, potential negative impact of disclosing information and risks attributable to the lack of information.

Adolescent↗