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Biomedical subjects

E Bouffet

Publications and source records attributed to E Bouffet.

At least 145 records · Page 8Linked to original sources

Effective multiagent chemotherapy in children with advanced B-cell lymphoma: who remains the high risk patient?

Between 1981 and 1985, 50 patients, mainly children and adolescents, with advanced B-cell lymphoma were entered on a protocol comprising eight drugs: cyclophosphamide, vincristine, prednisolone, high dose methotrexate, adriamycin, BCNU, cytosine arabinoside and thioguanine. Treatment to the central nervous system consisted of intrathecal methotrexate and cytosine-arabinoside in association with high dose methotrexate without irradiation. Data was collected prospectively with regard to response rate, treatment related complications and survival. Histology was reviewed in all referred cases and in 21 there was supportive evidence from immunological and cytogenetic studies. The overall complete response rate was 86%: 31/36 stage III and 12/14 stage IV. There were four treatment related deaths. The overall disease-free survival is 75% with a median follow up of 32 months. In the group of stage IV patients 5/7 with only marrow involvement, 2/4 with isolated CNS involvement and 1/3 with combined CNS and marrow infiltration survive. All the patients with CNS involvement at presentation underwent consolidation treatment with high dose chemotherapy and bone marrow transplant. These results demonstrate the very high curability of B-cell lymphoma using intensive multiagent therapy even with advanced abdominal disease. Bone marrow infiltration does not appear to be an adverse prognostic factor in isolation from bulk disease or CNS involvement. There remain, however, two groups of patients in whom further intensification of therapy is indicated, namely, those with initial CNS involvement, especially in combination with marrow infiltration, and those with extensive multiorgan involvement at presentation who fail to achieve remission with initial therapy. For the other patients, the large majority, a reduction in the intensity and duration of therapy is currently under study.

Adolescent↗

[Detection of residual cells contaminating the bone marrow in neuroblastoma. Apropos of 80 bone marrow evaluations].

Eighty bone marrow studies (each including 4 aspirates and 4 trephine biopsies) were performed in 37 children with stage IV neuroblastoma to assess the most accurate means for detection of invasion by neuroblastoma cells. Among 38 abnormal results, only trephine biopsy(ies) were found positive in 24 cases (63%), only aspirate(s) in 5 cases (13%), and both in 9 cases (24%). In 37% of abnormal results, only 1 of the 8 tests performed was found positive. No benefit was obtained from either associated touch imprints of iliac biopsies (67 investigations), or exploration of extra-iliac sites (66 sternums, 53 tibias). Two monoclonal antibodies, claimed to be specific for detection of neuroblastoma cells in bone marrow, were used in 56 investigations; they could detect minimal residual disease in some cases, but they were unreliable when no staining was obtained if initial phenotype of neuroblastoma had not been assessed, or when few isolated cells were observed. Prospective studies using immunocytology and immunohistology are thus warranted.

Antibodies, Monoclonal↗

[Methotrexate poisoning: value of exchange transfusion].

The authors report on a case of methotrexate (MTX) intoxication occurring in a young boy treated for osteosarcoma. Exchange transfusion was shown to be an effective method. Respective indications of MTX removal methods are discussed: haemodialysis and charcoal haemoperfusion should be proposed for massive and acute intoxications; exchange-transfusion may be proposed as a treatment for prolonged and moderate intoxications.

Acute Kidney Injury↗

[Cardiotoxicity of anthracyclines in children. Contribution of anthracyclines in pediatric oncology].

Anthracyclines induced cardiotoxicity in children remains a major problem. Classical data are found: cumulative dose, way of injection, anterior cardiopathy, cardiac area irradiation, interaction with other cytotoxic drugs ad age. 2 kinds of disorders are recorded: 1) Acute rhythmic dysfunctions, early and often transitory; 2) congestive heart failure often not reversible and depending on total dose. Prognostic is still poor and intricate with prognostic of the tumor. Over all incidence is 2.7%. Very young children seem to be more sensitive. Continuous infusion is less toxic than IV bolus. Studies with new anthracyclines are yet uncommon but seems to be comparative with adult literature. Monitoring is identical to adults. EKG is of minor interest. Endomyocardial biopsies have been reported. There is no prognostic value of ultrasound tomogram, but exercise echocardiography can be performed. Nuclide fraction ejection is more reliable.

Adolescent↗

Metastatic interstitial pneumonitis after autologous bone marrow transplantation. A consequence of reinjection of malignant cells?

Two cases of fatal interstitial pneumonitis developing after an autologous bone marrow transplantation are described. In both cases the autopsy revealed diffuse malignant pulmonary involvement. The first case involved a 4-year-old boy who had a Burkitt's lymphoma; the second case involved a 4-year-old girl with a neuroblastoma. The authors postulate that in these cases, the clinical picture may have been related to reinfusion of malignant cells.

Abdominal Neoplasms↗

Autologous bone marrow transplantation in paediatric solid tumours.

Massive therapy with ABMT is now an established treatment modality in paediatric oncology. The technical aspects and most treatment-related complications have been clarified and many phase II studies have shown encouraging results. In advanced neuroblastoma the poor outlook with conventional chemotherapy has stimulated extensive investigation of forms of massive therapy. Current results from several centres indicate that although the median survival is increased, long-term survival in an unselected group of stage IV patients is unlikely to exceed 30% with current regimens. In the future, management of this disease may involve the use of more intensive induction regimens to improve the quality of remission at the time of ABMT, which remains the single most important prognostic factor. Improved purging procedures involve the possible use of double massive therapy regimens and a combination of immunological and chemical treatments. In other paediatric tumours, the relative rarity and limited indications for ABMT make the evaluation of its role more difficult. Preliminary results in advanced rhabdomyosarcoma and Ewing's sarcoma are none the less encouraging and justify further investigation. The value of purging procedures remains controversial and their assessment has been hampered by the lack of sensitive clonogenic assays to detect residual tumour cells. However, neuroblastoma has provided a useful model for the investigation of physical, immunological and chemical procedures. Massive therapy is expensive, time consuming, and carries a high cost in patient morbidity and stress to the families involved. As with any new treatment, it must be adequately assessed in phase III, randomized studies. The ENSG and SIOP trials are a beginning and the future of massive therapy in the paediatric patient will, we hope, be based on a rigorous and scientific comparison with other treatment modalities.

Alkylating Agents↗

[Urinary sediment in acute renal insufficiency. Diagnostic and prognostic value of phase-contrast microscopic examination].

Living cells from the urinary tract can be examined by phase-contrast microscopy in a pellet obtained by centrifugation of 10 ml of freshly voided urine. Once these cells have been identified and classified according to their sources, their respective proportions can be evaluated, thus providing some information on the renal structures affected. Urine sediment examination was performed in 60 cases of acute renal failure in order to determine the relationship between the abnormalities encountered and the clinical or histological diagnosis. An abnormal sediment was always associated with parenchymal acute renal failure. Cellular debris and casts were abundant in acute tubular necrosis and less numerous in toxic acute renal failure than in failure resulting from shock. The finding of deformed erythrocytes was strongly suggestive of glomerular nephropathy, a diagnosis which was confirmed by renal biopsy in almost every case.

Acute Kidney Injury↗

Pituitary stalk thickening and primary central nervous system lymphoma.

We report a 14-year-old girl in whom a diagnosis of primary central nervous system lymphoma was confirmed while receiving growth hormone (GH) for GH deficiency, detected after presenting with short stature. MRI revealed an enhancing and thickened pituitary stalk with absence of the normal bright signal in the posterior pituitary. Regular MRI surveillance detected progression of the neurohypophyseal changes 13 months into GH treatment. Biopsy confirmed this to be B-cell large cell lymphoma. This case highlights the diagnostic and management challenges inherent in treating such children.

Central Nervous System Neoplasms↗

[Analysis of paediatric neuro-oncological information on the Internet in German language].

BACKGROUND: The fast growing internet offers easy access to medical information. So far there are limited data concerning the quality of this information. This study examined quality and readability of paediatric neuro-oncological information on the internet in german language. METHOD: Using the search terms "medulloblastoma", "ependymoma", "craniopharyngeoma", "brainstem glioma" and "low grade astrocytoma" in six different search engines, the first 30 universal/uniform resource locators (URLs) of each search engine were assessed. Appropriate Web sites were evaluated in regards to quality using DISCERN-Instrument and checklist rating system. Readability was rated by Flesch Reading Ease score. RESULTS: Out of 889,56 web sites remained evaluable. Most of the sites rated as poor to very poor (49 %), 30 % rated as fair and 21 % as good to very good. Readability was scored as very difficult with complex vocabulary content limiting the usefulness of good web sites. CONCLUSIONS: Search-ing for childhood brain tumours via internet is time consuming and most often ineffective. There is a lack of high-quality and comprehensible information on childhood brain tumours on german web sites. Cooperation of scientific medical societies and the Federal Ministry of Health is essential to provide comprehensible and high-quality information on internet as an effective and supportive resource for patients and their relatives.

Age Factors↗

Common brain tumours in children: diagnosis and treatment.

Dramatic advances have been made in the diagnosis of childhood brain tumours thanks to the development of modern imaging techniques. Advances in the management of these tumours have, however, been slow because of the limitations of an aggressive surgical approach and the risks associated with radiotherapy on the growing, and still immature, brain. The role of chemotherapy remains ill-defined in many patients with brain tumours and large variations in practice exist between groups and institutions. This article provides an overview of the most common paediatric brain tumours, mainly gliomas, medulloblastomas, ependymomas, germ-cell tumours and craniopharyngiomas. Considerations regarding the management of brain tumours in very young children are also examined. The long term outcome for children with brain tumours is discussed, stressing the need to focus on quality of life for survivors.

Brain Neoplasms↗