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Biomedical subjects

J Lemerle

Publications and source records attributed to J Lemerle.

At least 127 records · Page 7Linked to original sources
PubMed1989

[Malignant histiocytosis in children. Clinical and therapeutic aspects. Apropos of a series of 32 cases].

Thirty-two children with malignant histiocytosis were managed at the Department of Pediatrics of the Gustave-Roussy Institute from January 1975 through December 1987. Clinical presentation included enlarged lymph nodes in all cases, a decline in general health with fever in 74%, skin manifestations in 54%, and visceral involvement in 55%. Twenty-seven patients were treated with the COPAD protocol combining vincristine, cyclophosphamide, adriamycin and prednisone. Twenty-two patients achieved a complete remission, and one a partial remission; there was no remission in four patients. Eight patients had a recurrence and were given a combination of CCNU, vinblastine and bleomycin; seven achieved a second complete remission. Overall survival rate was 72.2% with a median follow up of five years. Survival rate free of disease was 54.5%. Poor prognostic factors included presence of constitutional symptoms upon diagnosis and age under ten years.

Adolescent↗
PubMed1988

Serum neuron-specific/nonneuronal enolase ratio in the diagnosis of neuroblastomas.

Pretreatment samples from 24 children with neuroectodermal tumors (two ganglioneuromas, 22 neuroblastomas) and from 106 others with various tumors were submitted to the enzymatic determination of the serum neuron-specific enolase (NSE). The enzymatic procedure employed in this study allows the systematic determination of the NSE and of the nonneuronal enolase (NNE), thus permitting the calculation of the ratio of the two enolase components. Like results obtained with other procedures, enzymatic determined serum NSE results were raised in a high proportion of Stage IV neuroblastoma (100%) but elevated values also were found in a considerable number of the other tumors (29.2%) like Wilms' tumor, lymphomas, and soft tissue sarcomas. The use of the NSE/NNE ratio which characterizes NSE elevations originating from relative poor or rich sources of NSE, represents an additional index for improving the specificity of the NSE results in the diagnosis of neuroblastomas. With a cutoff value fixed at 7.5%, the specificity of the test is 85.9%. When this limit is fixed at 15%, the specificity reaches 95.3% whereas 81.8% of the results of Stage IV neuroblastomas are still above this value.

Child↗
PubMed1988

[Hepatic complications after high-dose chemotherapy and bone marrow autograft in solid tumors in children].

Two-hundred and thirty-six courses of high-dose chemotherapy with bone marrow transplantation for solid tumors in children were reviewed in order to assess the incidence of hepatic abnormalities and their etiology. Bacterial and fungal hepatitis were excluded from this study. Hepatic complications occurred after 43 courses (20 per cent) in 39 patients. Four patients had hepatic abnormalities after two consecutive courses. Predisposing factors were found to be conditioning regimen including busulfan and previous high dose chemotherapy. Veno-occlusive disease occurred in 11 children and had a fatal outcome in 4. In 9 children, hepatitis was a symptom of diffuse viral infection. In the other 23 courses, hepatitis was isolated but it was difficult to determine whether its etiology was viral (cytomegalovirus, Epstein-Barr virus, adenovirus, HBs, non A non B) or toxic. In these latter forms, the outcome was always good.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1988

Epidemiological features of Wilms' tumor: results of studies by the International Society of Paediatric Oncology (SIOP).

This descriptive epidemiology study of 1,040 children with Wilms' tumor (WT) registered in the International Society of Paediatric Oncology (SIOP) clinical trials confirms the findings reported by the National Wilms' Tumor Study. The male:female rate was 0.89:1. The mean age at diagnosis of the 43 bilateral cases was significantly younger than children with unilateral renal involvement (32.4 vs 45 months). However, the mean ages of diagnosis for unilateral multicentric and for unicentric WT were very similar. On the other hand, the mean age at diagnosis of children with sporadic aniridia and hypospadias was younger than the mean age of patients with or without other congenital malformations. Thus aniridia as well as hypospadias could be indices of the first mutation, according to the Knudson and Stron hypothesis. WT was reported in two members of each of five families. However, these familial cases were comparable in terms of demographic and clinical features to the nonfamilial ones. These data suggest that the heritable fraction of WT is relatively small and that genetic and environmental factors interact in the development of WT.

Congenital Abnormalities↗
PubMed1988

Nonbacterial nonfungal interstitial pneumonitis following autologous bone marrow transplantation in children treated with high-dose chemotherapy without total-body irradiation.

Between February 1979 and May 1986, 165 children were treated with autologous bone marrow transplantation after high-dose chemotherapy without total-body irradiation for a hematologic malignancy or a solid tumor. Nonbacterial nonfungal interstitial pneumonitis was observed in 24 children and was the cause of death in 11 cases. Of the 24 cases of interstitial pneumonitis, 14 were considered to be idiopathic. Cytomegalovirus was the major pathogenic agent detected in the 10 other cases of interstitial pneumonitis (n = 5), followed by Herpes zoster (n = 2), Pneumocystis carinii (n = 1), tumor (n = 1), and adenovirus (n = 1). The only factor found to correlate significantly with the increased rate of interstitial pneumonitis was the use of high-dose 1-3 bis chloroethyl-1 nitrosourea (BCNU) (600 mg/m2), whereas BCNU at a dose of 300 mg/m2 did not affect this rate. These data, when compared with the literature, show a lower incidence of interstitial pneumonitis than in allogeneic transplantations, and an incidence similar to that observed in syngeneic transplantations, although there was no radiation toxicity in this series.

Adenovirus Infections, Human↗
PubMed1988

Ifosfamide is an active drug in Wilms' tumor: a phase II study conducted by the French Society of Pediatric Oncology.

Twenty-one patients with advanced Wilms' tumor entered a phase II study with high-dose ifosfamide (3 g/m2 over two days every 15 days). Mesna and hyperhydration were associated with minimal bladder toxicity. After two courses, five partial responses and six complete responses were observed. Ten patients did not respond. The median duration of response was 2 months (range, 1 to 7). Therapy was delayed because of leukopenia for 1 or 2 weeks in only three cases. Fever and infection were not observed. Seven patients presented with hematuria, three of whom were among the 17 patients coadministered mesna, which did not interfere with subsequent therapy.

Adolescent↗
PubMed1988

Juvenile granulosa cell tumor of the ovary in children: a clinical study of 15 cases.

Juvenile granulosa cell tumor (JGCT) in children accounted for 12% of all ovarian tumors treated in the Institut Gustave-Roussy (IGR) Pediatric Department from 1967 to 1985. The median age of the 15 girls was 8 years 7 months (range, 22 months to 15 years 7 months). Precocious pseudopuberty was present in six of the seven girls under 8 years. Of the other seven girls, one developed virilization symptoms. Surgery was the first treatment in each case. According to the Wollner classification, there were six stage I, one stage II, six stage III (including four ruptured tumors), and one stage IV JGCT cases. One patient was not available for staging. An adjuvant treatment (five chemotherapy and one radiotherapy combined with chemotherapy) was administered to six patients. Eleven girls are alive and free of disease, with a median follow-up of 6 years (range, 2 to 18 years). Four girls relapsed 6 to 17 months after surgery and died. Two of these relapses occurred in bone. The prognosis for JGCT in children is favorable for the lower stages when treated with surgery, but the best treatment for extensive and recurrent disease has yet to be determined.

Adolescent↗
PubMed1988

[Hodgkin's disease in children. Current therapeutic strategies].

The observed cure rates in childhood Hodgkin's disease (85 to 95%) and the knowledge of late complications of treatments, radiotherapy as well as chemotherapy, lead to studies aiming at decreasing the treatment burden without compromising these good results. An international workshop helped to make an update of the different presently used strategies. It appeared that the benefit and the risks of the staging laparotomy are still controversial, though most of the teams are now omitting this procedure. Different options have been selected in decreasing the treatments. If a few teams choose to treat by chemotherapy alone, the majority prefer to treat by combined modality therapy allowing to reduce at the same time the intensity of the radiation therapy (doses and fields) and of the chemotherapy (use of chemotherapy devoid of procarbazine and alkylating agents for the favorable cases). The presented french study shows the feasibility of such a strategy.

Adolescent↗
PubMed1988

[A French genetic and epidemiologic study on nephroblastoma: preliminary results].

Preliminary results of genetic and epidemiological study of Wilms' tumor in France are given in this paper. The higher age at diagnosis in unilateral than in bilateral cases supports the bimutational theory of Knudson. The excess of cancer deaths found in relatives of patients might be due to genetic non specific factors of susceptibility to cancer.

Child↗
PubMed1988

Veno-occlusive disease of the liver following high-dose chemotherapy and autologous bone marrow transplantation in children with solid tumors: incidence, clinical course and outcome.

Two hundred and thirty-six consecutive courses of high-dose chemotherapy with autologous bone marrow transplantation in children with solid tumors were reviewed in order to assess the incidence, clinical presentation and outcome of veno-occlusive disease (VOD) of the liver. Patients conditioned with total body irradiation were excluded from this study. Eleven patients (4.6%) met the diagnostic criteria for VOD. The clinical course included sudden weight gain, jaundice, hepatomegaly and ascites. Renal dysfunction and refractoriness to platelet transfusions occurred in the most severe forms. Seven patients recovered within 7-29 days of onset and four patients died, all with renal failure and fluid overload. The time of onset appeared to determine two patterns of outcome: mild forms with early onset (before day 11) and more severe forms with onset after day 17. Analysis of pretransplant factors revealed no significant association with an increased risk of VOD. However, all the patients with severe VOD had received a conditioning regimen containing cyclophosphamide which might be involved in the pathogenesis of VOD.

Adolescent↗
PubMed1988

[Sensitivity and specificity of meta-iodobenzylguanidine (mIBG) scintigraphy in the evaluation of neuroblastoma: analysis of 115 cases].

Seventy children (3.7 +/- 3.3 y) with definitely confirmed diagnosis of neuroblastoma had 115 whole body scans carried out 24 h after injection of 3.7 MBq/kg of I-123 mIBG (83 scans) or 0.7 MBq/kg of I-131 mIBG (17 scans) or 0.9 to 4.5 GBq of I-131 mIBG (15 post-therapeutic scans). The scans were interpreted as positive in the presence of any non-physiological uptake area or of any bone uptake of the tracer, even at the level of the metaphyseal complex. For the primary tumour, the sensitivity of mIBG scans was 73%. Ten false negative patients had an overlap of the tumour with the bladder or heart images (4 cases) or with positive metastatic images (6 cases: liver, spine). Three false negative patients had neuroblastomas which did not secrete catecholamines. The specificity of mIBG was 94%. In our opinion, mIBG scans have a complementary role to assess the activity of post-therapeutic remnants. For the detection of hepatic and lymph node metastases, the sensitivity was about 50% and the specificity was 100%. The standard used for the detection of bone marrow metastases was the cytological and histological examination of 10 bone marrow aspirations and one or more biopsies (CHBMS). The sensitivity of mIBG scans was 90% and the specificity 68%. However, reviewing the data from the 16 false positive scans, we found 11 definitely proven bone metastases, 3 biological relapses and 2 cases of delayed abnormal CHBMS supporting the positivity of the mIBG scans, raising the specificity to 100%. Tc-99m diphosphonate bone scans had a sensitivity of 78% and a specificity of 51%. We suggest that positive mIBG scans may save other procedures since our data do not support false positive detection of bone or bone marrow metastases. In contrast, patients with negative mIBG findings should be further explored.

3-Iodobenzylguanidine↗
PubMed1987

Presence of circulating antibodies against cellular protein p53 in a notable proportion of children with B-cell lymphoma.

Serum samples taken from children bearing a wide variety of tumors were screened for the presence of circulating antibodies against the cellular tumor antigen p53. There was a significant correlation (p less than 0.001, n = 119) between the presence of such antibodies and the occurrence of cancer; 12% of the sera tested were positive. These sera were derived from children with a wide range of tumor types. In particular, 21% of the sera obtained from children suffering from a B-cell lymphoma contained anti-p53 antibodies. We were not able to establish a correlation between the secretion of p53-reactive antibodies and any other parameters, such as the age or sex of the child, presence of metastasis, stage or prognosis of disease, or treatment regimen. These results therefore show that the development of p53 immunogenicity is associated with a wide range of neoplastic diseases in children, and in particular with the presence of a B-cell lymphoma.

Adolescent↗
PubMed1987

Therapeutic use of 131I-metaiodobenzylguanidine (MIBG) in neuroblastoma: a phase II study in nine patients.

Effects of high activities of I 131 meta-iodobenzylguanidine (mIBG) were evaluated in nine children with advanced neuroblastoma. All patients had been previously heavily treated and had either primarily refractory disease or resistant relapse. Twenty-two doses of mIBG labeled with 1.3 to 4 GBq (35-108 mCi) of iodine 131 were administered. Three subjective effects, especially relief of pain, and two objective effects were observed. Transient blood pressure increase was observed once and did not recur after prolongation of the infusion time to 6 hours. A major side effect was bone marrow toxicity, essentially marked by thrombopenia, particularly severe in previously bone-marrow-transplanted patients.

3-Iodobenzylguanidine↗
PubMed1987

Differentiated thyroid carcinoma in childhood: long term follow-up of 72 patients.

Seventy-two children with differentiated thyroid cancer who were 16 years old or younger at the time of initial treatment were followed for a median time of 13 yr. Initially, 18% had lung metastases, and 74% had palpable lymph nodes. Capsular invasion was found in 67%, and histological lymph node involvement in 90%. The recurrent laryngeal nerve chain and the jugulo-carotid chain were involved with the same frequency (greater than 80%). The anterior superior mediastinum was involved only in patients with involvement of the recurrent laryngeal nerve chain. Forty-three patients had a complete remission after initial treatment. In patients without distant metastases for whom surgery was macroscopically incomplete, relapses occurred 5 times more frequently than in patients whose surgery was complete. Six patients died from thyroid carcinoma at ages ranging from 19-44 yr, 12-33 yr after initial treatment, and 1 died from intercurrent disease. Despite favorable long term survival (90.3% at 20 yr), the standardized mortality ratio was equal to 8.1. This study underlines the need for complete surgical treatment and compulsive follow-up, which should be continued throughout the patient's life, in order to detect and effectively treat relapses of thyroid cancer.

Adenocarcinoma↗
PubMed1987

Incubation of autologous bone marrow graft with ASTA Z 7557: comparative studies of hematological reconstitution after purged or nonpurged bone marrow transplantation.

Thirty-three patients with advanced solid tumors were treated by high-dose chemotherapy (combined high-dose melphalan), followed by cryopreserved autologous bone marrow transplantation (ABMT). Thirteen of them had bone marrow (BM) tumor involvement at diagnosis, and BM harvest was purged with 50 micrograms/ml ASTA Z 7557 before cryopreservation. Following incubation, in vitro growth of granulomonocytic colony-forming cells (GM-CFC) was regularly inhibited (greater than 99%). Hemopoietic reconstitution after purged and nonpurged ABMT was studied. All patients experienced engraftment. However, peripheral leukocyte and granulocyte recoveries were delayed significantly in patients receiving purged BM (mean 27 and 26 days) compared with those observed in patients receiving nonpurged BM (mean 18 and 18 days). These results confirm that purged BM, despite GM-CFC depletion after in vitro treatment, ensures engraftment after high-dose chemotherapy, but prolonged pancytopenia is observed and postgraft hemopoietic recovery is delayed. A clinical trial is necessary to evaluate the efficiency of the purging technique in eradicating residual tumor cells.

Adult↗