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Biomedical subjects

B Pautard

Publications and source records attributed to B Pautard.

At least 19 recordsLinked to original sources
PubMed2005

Long-term renal and hearing toxicity of carboplatin in infants treated for localized and unresectable neuroblastoma: results of the SFOP NBL90 study.

BACKGROUND: A secondary end point of the NBL90 protocol (Rubie H et al. Pediatr Oncol 2001;36:247-250) was the concern in this infant population for possible carboplatin-(CBDCA) induced late side effects including impaired renal and hearing functions. PROCEDURE: Glomerular filtration rate (GFR), tubular function (TF), pure tone audiometry (PTA), high-frequency, and transient evoked-otoacoustic emission were prospectively assessed in 30 children alive and disease-free 6 years after the end of the treatment. RESULTS: Median age at diagnosis and at assessment was 4.7 months and 7 years, respectively. Blood pressure was < or =97.5 centile in all children. The mean estimated GFR was 114 +/- 13 ml/min/1.73 m(2) by Schwartz formula [range 87-145]. TF assessment failed to demonstrate any impairment. 29/30 children had grade 0 ototoxicity and all transient evoked otoacoustic emission were normal. CONCLUSIONS: With a 6-year follow-up the combination of VP16 and carboplatin given at conventional doses is safe on renal and hearing functions in infants with unresectable neuroblastomas treated according to SFOP NB90.

Antineoplastic Agents↗
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

Infectious diseases in the first year of life, perinatal characteristics and childhood acute leukaemia.

The objective of the present study was to investigate the role of early common infections and perinatal characteristics in the aetiology of childhood common leukaemia. A case-control study was conducted from 1995 to 1998 in France, and included 473 incident cases of acute leukaemia (AL) (408 acute lymphoblastic leukaemia (ALL), 65 acute myeloid leukaemia (AML) age-, sex- and region-matched with 567 population-based controls. Data on the medical history of the child and his/her environment were collected using self-administered questionnaires. Analyses were conducted using nonconditional logistic regression. A slight negative association with early infections was observed (OR=0.8; 95% CI (0.6-1.0)). The association was stronger for early gastrointestinal infections. Early day-care was found to be associated with a decreased risk of AL (OR=0.6; 95% CI (0.4-0.8) and OR=0.8; 95% CI (0.5-1.2) for day-care starting before age 3 months and between 3 and 6 months, respectively). No association with breast-feeding was observed, irrespective of its duration. A birth order of 4 or more was associated with a significantly increased risk of AL (OR=2.0; 95% CI (1.1-3.7) with ALL). A history of asthma was associated with a decreased risk of ALL (OR 0.5; 95% CI (0.3-0.90). Although the results regarding birth order and breast-feeding do not fit with Greaves' hypothesis, the study supports the hypothesis that early common infections may play a protective role in the aetiology of childhood leukaemia, although this effect was not more marked for common ALL.

Acute Disease↗
PubMed2004

Epidemiological and quantitative study of GBV-C infection in french polytransfused children.

From 1999 to 2002, 246 serum samples taken from polytransfused children were tested for the presence of GB virus C (GBV-C) RNA using a real-time reverse transcription-polymerase chain reaction (RT-PCR) assay. This assay was based on the TaqMan technology and allowed viral load determination in infected children with a dynamic range from 10(3) to 10(7) genome equivalent (gEq) copies/ml. The limit of detection was estimated to 619 gEq copies/ml with a > or = 95% probability of a positive result. Thirty five sera were found to be GBV-C RNA positive, corresponding to a prevalence of GBV-C of 14.2%. The mean viral load was high, i.e., 6 +/- 1.4 log (range 3.22-7.42) gEq copies/ml, but low viral loads were also detected. Sequencing of the 5'-untranslated region (UTR) identified a majority of genotype 2 strains (82%) distributed into two subtypes, 88.5% genotype 2a and 11.5% genotype 2b. In conclusion, GBV-C active infection is very frequent in exposed populations such as polytransfused children. GBV-C RNA quantitation using real-time assay may be useful for diagnosis and follow-up of the natural history of GBV-C infection.

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↗
PubMed2003

[Intravenous immunoglobulin (Endobulin) clinical tolerance: prospective therapeutic follow-up of 142 adults and children].

PURPOSE: Prospective report of Endobulin clinical tolerance experience for 19 months over a large number of patients. METHOD: Collect diagnosis, age, gender, weight, dose regimen, infusion duration, and clinical tolerance of Endobulin. Treatment with this product was the only inclusion criteria in this follow-up. RESULTS: A hundred and forty-two patients, 85 children and 57 adults, mean age 23 (1-85 years) received 70 substitutive treatments for primary immunodeficiency, 36 substitutive treatments for secondary immunodeficiency and 36 immunomodulatory treatments. A thousand six hundred and sixty Endobulin infusions that led to 14, 061.5 g, from 52 different batches. Tolerance was judged as good for 135 patients even though side effects occurred in 2 of them. Thus, 133 out of 142 patients, that is 93.7% did not present any side effect and their tolerance to Endobulin infusions was defined as good. Tolerance was bad for 7 patients because of side effects occurrence. For a mean number of 11.7 infusions per patient (1-31), the 9 side effects observed led to a rate of 0.54% of collected infusions and 6.3% of patients included. CONCLUSION: This therapeutic follow-up of 142 patients confirms Endobulin clinical tolerance judged as good in 93.7% of patients (133/142) with a very low rate of side effects of 0.54% of infusions (9 side effects for 1660 infusions) for a mean number of 11.7 infusions per patient for an average of 10.9 months follow-up.

Adolescent↗
PubMed2001

Use of recombinant factor VIIa (NovoSeven) in patients with Glanzmann thrombasthenia.

Recombinant factor VIIa (rFVIIa; NovoSeven, Novo Nordisk, Bagsvaerd, Denmark) appears effective and relatively safe for the treatment of bleeding and for surgical prophylaxis in patients with Glanzmann thrombasthenia as reported to the International Registry on rFVIIa and Congenital Platelet Disorders. One of the shortcomings of the Registry data is the heterogeneity of treatment protocol, including dosage, number of doses used, duration of treatment before declaration of failure, and mode of rFVIIa administration (bolus v continuous infusion). The data are not yet sufficient to define optimal regimens for various indications such as the type of bleeding or the type of procedures. The place of this drug compared to platelet transfusion in the overall management of patients with Glanzmann thrombasthenia will need to be determined in relationship to a number of challenges and unresolved issues in the clinical care of these patients. These issues include: how to improve local measures for patients with mucosal bleeds, optimal management of young women during menarche, optimal platelet transfusion regimens for various indications, the relationship between antiplatelet antibodies detected by monoclonal antibody-specific immobilization of platelet antigens (MAIPA) and effectiveness of platelet transfusion, whether there are other biological tests that may correlate with effectiveness of platelet transfusion, and management of pregnancy and delivery regarding antiplatelet immunization.

Coagulants↗
PubMed2000

Use of recombinant human granulocyte colony-stimulating factor to increase chemotherapy dose-intensity: a randomized trial in very high-risk childhood acute lymphoblastic leukemia.

PURPOSE: To determine whether the use of a recombinant human granulocyte colony-stimulating factor ([G-CSF] lenogastrim) can increase the chemotherapy dose-intensity (CDI) delivered during consolidation chemotherapy of childhood acute lymphoblastic leukemia (ALL). PATIENTS AND METHODS: Sixty-seven children with very high-risk ALL were randomized (slow early response to therapy, 55 patients; translocation t(9;22) or t(4;11), 12 patients). Consolidation consisted of six courses of chemotherapy; the first, third, and fifth courses were a combination of high-dose cytarabine, etoposide, and dexamethasone (R3), whereas the second, fourth, and sixth courses included vincristine, prednisone, cyclophosphamide, doxorubicin, and methotrexate (COPADM). G-CSF was given after each course, and the next scheduled course was started as soon as neutrophil count was > 1 x 10(9)/L and platelet count was > 100 x 10(9)/L. CDI was calculated using the interval from day 1 of the first course to hematologic recovery after the fifth course (100% CDI = 105-day interval). RESULTS: CDI was significantly increased in the G-CSF group compared with the non-G-CSF group (mean +/- 95% confidence interval, 105 +/- 5% v 91 +/- 4%; P <.001). This higher intensity was a result of shorter post-R3 intervals in the G-CSF group, whereas the post-COPADM intervals were not statistically reduced. After the R3 courses, the number of days with fever and intravenous antibiotics and duration of hospitalization were significantly decreased by G-CSF, whereas reductions observed after COPADM were not statistically significant. Duration of granulocytopenia was reduced in the G-CSF group, but thrombocytopenia was prolonged, and the number of platelet transfusions was increased. Finally, the 3-year probability of event-free survival was not different between the two groups. CONCLUSION: G-CSF can increase CDI in high-risk childhood ALL. Its effects depend on the chemotherapy regimen given before G-CSF administration. In our study, a higher CDI did not improve disease control.

Adolescent↗
PubMed1998

Human complement factor H deficiency associated with hemolytic uremic syndrome.

This study reports on six cases of deficiency in the human complement regulatory protein Factor H (FH) in the context of an acute renal disease. Five of the cases were observed in children presenting with idiopathic hemolytic uremic syndrome (HUS). Two of the children exhibited a homozygous deficiency characterized by the absence of the 150-kD form of Factor H and the presence, upon immunoblotting, of the 42-kD Factor H-like protein 1 (FHL-1) and other FH-related protein (FHR) bands. Southern blot and PCR analysis of DNA of one patient with homozygous deficiency ruled out the presence of a large deletion of the FH gene as the underlying defect for the deficiency. The other four children presented with heterozygous deficiency and exhibited a normal immunoblotting pattern of proteins of the FH family. Factor H deficiency is the only complement deficiency associated with HUS. These observations suggest a role for FH and/or FH receptors in the pathogenesis of idiopathic HUS.

Acute Disease↗
PubMed1998

[Inflammatory pseudotumors of the liver. Apropos of a pediatric case with radiological and ultrasonographic features and anatomopathological correlations].

Inflammatory pseudo-tumor of the liver (IPT) are benign encapsulated masses. IPT or inflammatory myofibroblastic tumor is a myofibroblastic proliferation with chronic inflammatory cell infiltration of unknown origin. A four-year-old girl, with cutaneous flushing of the face and biological inflammatory syndrome was referred for abdominal investigation. Ultrasound examination showed a 5-cm mass located in the anterior segment of the right hepatic lobe. After abdominal CT showing slightly vascular feature of this right hepatic mass, diagnosis was made through percutaneous US-guided fine needle biopsy. Surgical resection was performed and pathologic examination of the mass confirmed preoperative diagnosis. Clinical outcome was good.

Biopsy↗
PubMed1993

[Pulmonary embolism in adolescents].

BACKGROUND: Pulmonary embolism is rare in children, but its incidence is probably underestimated. While its causes are the same as in adults, the role of clotting factor deficiency and oral contraceptive agents has recently been stressed. CASE REPORTS: Case n. 1: A 15 year-old girl was immobilized for an ankle strain. She developed thrombophlebitis of the deep veins of the right leg. The condition was confirmed by Doppler. A phlebogram showed a clot floating in the inferior vena cava. This required the insertion of a clip around it and further thrombectomy. Despite immediate intravenous heparin therapy, the adolescent died of a massive pulmonary embolization. The girl had a congenital antithrombin III deficiency; she was also taking an oral contraceptive. Case n. 2: A 15 year-old suffered from left thoracic pain radiating to the shoulder. Perfusion scintiphotography showed evidence of pulmonary embolism of the left inferior lobe and a phlebogram showed thrombosis of the hypogastric veins. This adolescent was 4 months pregnant. A filter was placed inside the inferior vena cava to permit therapeutic interruption of the pregnancy. The girl was treated with intravenous heparin, followed by antivitamin K with an uneventful course. She suffered from no clotting factor deficiency. CONCLUSION: Pulmonary embolism remains a severe complication of thrombi in the femoral and pelvic veins. It can occur in adolescents, especially those with a congenital clotting factor deficiency, those on oral contraceptives or those who are pregnant.

Adolescent↗
PubMed1988

Occurrence of hereditary spherocytosis and beta thalassaemia in the same family: globin chain synthesis and visco diffractometric studies.

Hereditary spherocytosis and beta thalassaemia are rarely inherited together. We have studied a large family of Caucasian extraction in whom these two diseases segregate independently over four generations. The diagnosis rested on specialized laboratory findings and in a number of subjects on the measurement of alpha and beta globin chain synthesis. In addition, a viscometric method (osmotic gradient ektacytometry) was used to evaluate the rheological function of the erythrocytes. In patients inheriting both diseases, the results indicate that the clinical and biological expression of hereditary spherocytosis is modulated by the degree of imbalanced globin chain synthesis. The opposite properties of spherocytes (decreased surface/volume ratio, increased haemoglobin concentration) and thalassaemic red cells (increased surface/volume ratio, decreased haemoglobin concentration) may explain the antagonistic influence of each genotype.

Adolescent↗
PubMed1988

[Embryonal rhabdomyosarcoma diagnosed prenatally. Apropos of a case].

A case report of congenital embryonal rhabdomyosarcoma of the superior extremity (right shoulder) is described. This patient was the first and only child of a 24 year-old woman who had been previously treated for sterility. The diagnosis was done by echography during the 36 th week of gestation. This full term white boy was born by cesarean section in reason of tumor size. First he was treated surgically. Then, a cyclic chemotherapy (VAC) was performed. After 4 courses, local recurrence led to a second surgical excision followed by a new cyclic chemotherapy (IVA). The treatment had an acceptable tolerance. Seven months later the child is free disease alive.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1986

Thyroid stimulating immunoglobulin (TSI) synthesis by pokeweed mitogen (PWM) stimulated peripheral blood lymphocytes (PBL) from Graves' disease (GD) patients.

Lymphocytes from freshly diagnosed untreated GD patients can be induced to produce in-vitro IgG, and more precisely TSI in the presence of PWM, a T cell dependent polyclonal activator of B cells. TSI were measured from days 8 to 41 of culture, using a functional cAMP production assay, after the deposition of culture supernatants on HTEC. Spontaneous or PWM induced IgG synthesis was optimal on day 28 of culture for GD MNC, with amounts of IgG in PWM stimulated GD culture supernatants twice those found in unstimulated GD MNC. Moreover only PWM stimulated GD MNC produce IgG which induce cAMP production by cultured HTEC, with a significant correlation between in-vitro IgG and cAMP levels.

Adult↗