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Biomedical subjects

D Frappaz

Publications and source records attributed to D Frappaz.

At least 109 records · Page 6Linked to original sources
PubMed1993

[Neuroblastoma in children: clinical and biological aspects. An experience of screening in France].

Neuroblastoma is the commonest solid tumour in children under the age of 5 years (50% of cases before 2 years, 90% before 5) and the second cause of death after accidents. Approximately one child in 10,000 develops neuroblastoma by the age of 15 years. The situation in other European and North American countries is similar to that in France. As neuroblastoma is derived from the sympathetic nervous tissues, it is associated with the production of large amounts of catecholamines and their metabolites which are excreted in the urine. Less than 5% of cases do not produce catecholamines. Vanillylmandelic acid (VMA), homovanillic acid (HVA) and dopamine (DA) are the most useful chemical markers for the diagnosis and clinical control of neuroblastoma. They are generally measured using the reliable and sensitive high pressure liquid chromatography (HPLC). Survival is related to stage (the Evan's staging protocol has been superseded by the INNS staging), and age at diagnosis. There is almost 100% survival for stages I and IIa before the age of 12 months, and less than 20% for stage IV when diagnosed after 2 years of age. Multiple copies of the N-myc oncogene, deletions of chromosome 1p, and diploidy in tumour cells are associated with poor prognosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1993

[Breaking the isolation. Telecommunication in the service of schooling of sick children].

We describe here a two-way teleconferencing system which links the sterile and pediatric units of the Center for Cancer Treatment in Lyon to a secondary school and the pediatric unit of the University Hospital Edouard-Herriot where primary teaching is given. Léon-Bérard Center and Edouard-Herriot Hospital are 300 m apart. Elie-Vignal secondary school is 6 km from the first site; all three institutes are connected through hyperfrequency aerials. With the help of this system, communication (voice and image) between children in hospital and pupils or teachers in school is instantaneous. Children in bed can listen to the lesson, break in on the conversation in school, ask questions or answer those of teachers. After school time, the system can also be used to organize games between children, or friendly contacts between children in each institute, children in the college, brothers, sisters or friends who may have access to one of those institutes. The system aims are: 1) to improve the education delivered to children and teenagers who spend long periods in hospital; 2) to establish a relationship between both worlds of healthy or sick children through teaching and playing in common; 3) finally, to facilitate the social insertion of sick children at leaving hospital.

Adolescent↗
PubMed1992

M4 protocol for cerebellar medulloblastoma: supratentorial radiotherapy may not be avoided.

The main goal of the M4 protocol was to evaluate the efficacy of treatment excluding supratentorial radiation in patients with newly diagnosed medulloblastoma. All patients underwent surgical resection and received postoperative chemotherapy. Chemotherapy was adapted to the initial staging and prognostic factors (Group A: good-risk; Group B: poor-risk). Chemotherapy was started early after surgery, and consisted of two courses of the "eight drug in one day" regimen and two courses of high dose methotrexate. Radiotherapy was delayed until 5 (Group B) to 7 (Group A) weeks after the first course of chemotherapy. Radiotherapy was administered only to the posterior fossa and the spinal axis. Only 3/16 patients (18%) are alive and disease-free with a mean follow up of 6 years. The site of progression was supratentorial in 9 out of 13 patients and three patients had spinal and/or cerebrospinal fluid relapses. Only one patient had isolated posterior fossa relapse. The mean time to relapse was 484 days. We conclude that the chemotherapy regimens used in the M4 protocol do not allow the reduction of irradiation fields in patients with cerebellar medulloblastoma. In spite of long-term side effects on neurocognitive functions, supratentorial radiotherapy should remain a major component of medulloblastoma treatment.

Adolescent↗
PubMed1992

Etoposide and carboplatin in neuroblastoma: a French Society of Pediatric Oncology phase II study.

PURPOSE: A phase II study of etoposide (VP 16) and carboplatin (CBDCA) was performed in patients with metastatic neuroblastoma (NB). The aim of the study was to find an alternative treatment for induction with different toxicities than the VP 16/cisplatin (CDDP) combination. PATIENTS AND METHODS: Forty-seven patients who were from 6 months to 16 years of age, with either relapsed (29) or primary resistant (18) NB, were included in a cooperative multicenter phase II study of the French Society of Pediatric Oncology (SFOP). The schedule consisted of 5 consecutive days of VP 16 100 mg/m2/d and CBDCA 160 mg/m2/d. RESULTS: The response rate for the 39 assessable patients was 43%; there were four complete remissions and 13 partial remissions. Neither the status of the patients nor the total dose of CDDP that was received previously influenced response. Hematologic toxicity was marked and caused considerable delay between courses (median interval, 39 days). In these heavily pretreated patients, 16% had a more than 50% decrease in creatinine clearance and a 22% World Health Organization (WHO) grade 2 ototoxicity. CONCLUSION: This VP 16/CBDCA combination deserves further evaluation for efficacy and toxicity in newly diagnosed patients, and the combination of both drugs should be considered for high-dose therapy with bone marrow transplantation.

Adolescent↗
PubMed1992

[Retinoblastoma].

Retinoblastoma (RB) is the most frequent ocular tumor in childhood. Due to recent advances in molecular biology, RB has become a study model for cancer suppressor genes, and antenatal diagnosis has now become feasible. The goals of therapy include an improved survival rate, decrease in iatrogenic sequelae (especially enucleation), and avoidance of radio-induced neoplasias. This review examines recent data from the literature.

Child↗
PubMed1992

[Multifocal tuberculosis with cerebellar tuberculoma].

A 10-year old child was admitted for vomiting and a high grade fever. He had been previously immunized twice against tuberculosis. A cerebellar syndrome appeared, and the brain CT scan showed an intracerebellar mass. Disseminated lesions were detected on abdominal (splenic abscess) and thoracic (mediastinal lymph nodes) CT scans. The bone scintigraphy also showed multiple localisations. A craniotomy was performed that revealed a cerebellar tuberculoma which was completely removed. The child received an antituberculous treatment over 18 months and is currently well with a 5 year follow-up.

Antitubercular Agents↗
PubMed1992

[Hematuric cystitis after bone marrow allograft: a case].

A 16-year-old boy experienced a severe macroscopic hemorrhagic cystitis 20 days after a partially mismatched allogeneic marrow transplantation for an acute lymphoblastic leukemia. The conditioning included cyclophosphamide and total body irradiation. After a conservative management, the hematuria spontaneously resolved after 3 months of gross hematuria. The authors review the literature, and stress the predisposing factors and clinical outcome of such complication. They advocate a conservative "watch-and-wait" approach including intensive bladder irrigation.

Adolescent↗
PubMed1992

[Blackfan-Diamond disease and malignancy: cause effect relationships?].

The authors report on a 24-year old patient with Blackfan-Diamond syndrome who developed a Hodgkin's disease. This patient became transfusion-dependent at the age of 10, after an initial period of corticosensitivity, and after failure of androgens. He developed hemochromatosis despite from parenteral chelation therapy. He died of infectious complications 4 months after the diagnosis of Hodgkin's lymphoma. A review of the literature shows an increased incidence of malignancies in Blackfan-Diamond syndrome (three cases of leukemia), and in similar disease (thalassemia and sickle cell disease), but not in other patients with hemosiderosis (primitive hemochromatosis, end-stage renal failure under dialysis). Etiopathogenic hypotheses are discussed.

Adult↗
PubMed1992

[Iron deficiency in infants and children].

A study of the incidence of iron deficiency and its risk factors was carried out in 90 hospitalized or outpatient children and infants, 4 to 48 months old. Iron deficiency (serum iron concentration less than 10 mumol/l) was found in 70% of them and microcytosis (mean corpuscular volume less than 70 fl) in 10%. A good correlation was observed between hemoglobin concentration or mean corpuscular volume, and serum iron or ferritin concentrations. When risk factors were studied, 20% of the children were found to have insufficient meat or vegetable intakes. Only 45% of 6 month-old and 20% of 1 year-old infants received iron fortified milk formulas. However no significant correlation was found between the nutritional factors and the hematological data. Anemia and microcytosis were significantly more frequent in children born from immigrant parents as compared with native children, but there was no difference between these two groups for isolated iron deficiency. These results indicate that there is a need for a better prevention of iron deficiency in French infants and young children; a larger use of iron fortified milk formula until the age of 12 months is to be recommended.

Anemia, Hypochromic↗
PubMed1992

In utero transplantation of stem cells in humans: immunological aspects and clinical follow-up of patients.

Four human fetuses were treated by transplantation of human fetal liver stem cells. Two of them had severe immunodeficiency disease and the two other ones had thalassemia major. Three of these in utero transplants were followed by engraftment. The three patients are now born: the first one is now very healthy thanks to the reconstitution of cell-mediated immunity associated with this transplant, and he lives normally at home; the two other ones, who have been more recently treated, have a significant improvement of their condition and they also live normally at home. This procedure, for the first time used in humans, has therefore demonstrated its feasibility and its efficacy: during early fetal development, foreign cells engraft readily and may result in cure or significant correction of a large variety of inherited diseases.

Blood Transfusion, Intrauterine↗
PubMed1992

[To break the isolation. Telecommunication to help the schooling of sick children].

A 2-way teleconferencing system has been described which links the sterile and pediatric units of the Center for Cancer Treatment in Lyon to a secondary school and the pediatric unit of the University Hospital Edouard Herriot where primary teaching is given. The Léon Bérard Center and Edouard Herriot Hospital are 300 m apart. Elie Vignal secondary school is 6 km from the first site; all three establishments are connected via hyperfrequency aerials. With the help of this system, communication (voice and image) between children in hospital and pupils or teachers in school is instantaneous. Children in bed can listen to the lesson, break in on the conversation in school, ask questions or answer those of teachers and play with children in the classroom. The aims of the system are: 1) to improve the education delivered to children and teenagers who have to spend long periods in hospital; 2) to establish a relationship between the worlds of healthy and sick children through teaching and playing in common; 3) to facilitate the social insertion of sick children after leaving the hospital.

Adolescent↗
PubMed1992

[Study of serum kinetics of vancomycin during the "antibiotic-lock" technique].

The serum kinetics of vancomycin was studied in two patients aged 3 and 15 years during antibiotic therapy for catheter related sepsis associated with Staphylococcus epidermidis. Vancomycin was administered, simultaneously, by parenteral conventional doses (30 mg/kg/day div q 8 h) and using the antibiotic-lock technique in the infected catheter at a high concentration (150 mg/ml) during one hour, 3 hours after each infusion. Pharmacokinetics data did not show any significant change in the serum kinetics of the antibiotic. The results suggest that delivering a high concentration of vancomycin in the infected catheter using the lock technique may be useful to sterilize infected catheter without toxic effect.

Adolescent↗
PubMed1991

Expression of integrin receptors on 45 clinical neuroblastoma specimens.

Immunohistological expression of integrins has been analyzed on 45 neuroblastoma specimens representative of the different clinical and histological forms of the tumor. None of the specimens expressed the alpha 5 chain of the integrins. The beta 1 chain was expressed on all specimens, the alpha 1 chain on 44 specimens and the alpha 3 chain on 42; the 4 specimens which lacked alpha 1 or alpha 3 were stage-4 neuroblastomas. The alpha 2 chain was expressed on 18 specimens, and the alpha 6 chain on 17; 15 reacted with both. Their reactivity was related to the maturation of the tumor rather than the stage of the disease: they were expressed on low-grade, well-differentiated specimens; stage 3-4 neuroblastoma specimens analyzed at diagnosis were negative, but usually expressed both chains when analyzed after in vivo differentiation by chemotherapy. alpha v reacted with 18 specimens and beta 3 with 12, without strict relation with the stage of the disease and/or its degree of differentiation; 9 well-differentiated specimens expressed the beta 4 chain; only 4 well-differentiated specimens expressed the alpha 4 chain. The 4 specimens which lacked alpha 1-beta 1 or alpha 3-beta 1 expression had n-myc amplification, whereas those which expressed either alpha 4, beta 4, beta 3 or alpha v had no amplification. Furthermore, the expression of the 3 heterodimers alpha 4-beta 1, alpha v-beta 3 and alpha 6-beta 4 was essentially observed on primary tumors which developed in the mediastinum. The expression of alpha 2-beta 1 and alpha 6-beta 1 was observed on both n-myc-positive and -negative specimens. beta 1 and alpha 3 were diffusely expressed on all counterparts of these tumors, from undifferentiated neuroblasts to ganglion and Schwann cells. The alpha 1 chain reacted with undifferentiated and intermediate neuroblasts as well as with Schwann cells, but ganglion cells were negative. alpha 2 and alpha 6 chains were negative on undifferentiated neuroblasts, variably expressed on intermediate neuroblasts, and restricted to Schwann cells in ganglioneuroma. The expression of alpha 4 and beta 4 was restricted to Schwann cells. alpha v and beta 3 occasionally reacted with undifferentiated and intermediate neuroblasts; alpha v was strongly positive on Schwann cells but negative on ganglion cells, whereas beta 3 was positive on both neuronal and non-neuronal populations.

Ganglioneuroma↗
PubMed1991

Burkitt's lymphoma: a model for clinical oncology.

Burkitt's lymphoma, a pathological entity initially described in Africa, is the most common childhood lymphoma in western countries and represents approximately 5% of all adults lymphomas. This high grade small non-cleaved diffuse lymphoma is a model with which to study the relations between cancer and viruses, the chromosomes and the genes. Burkitt's lymphoma is also a model for clinical research which allows evaluation of the dose effect concept with chemotherapy and the role of megatherapy with autologous bone marrow rescue.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1991

[Alexander's disease].

Alexander disease, or hypoproconvertinemia is a rare autosomic recessive coagulation disorder. The features include familial and/or personal history of bleeding, with an abnormal prothrombin period and a normal activated partial thromboplastin period. Coagulation and genetic studies allow subclassification with prognosis incidence for this disease. The authors report on a case of one family with Alexander disease.

Child↗