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Biomedical subjects

L de Lumley

Publications and source records attributed to L de Lumley.

At least 37 records · Page 2Linked to original sources
PubMed1996

Myelodysplastic syndromes in childhood: report of 49 patients from a French multicentre study. French Society of Paediatric Haematology and Immunology.

We describe the clinical, cytological and cytogenetic features of 49 cases of myelodysplastic syndromes (MDS) in childhood. Three children had received prior cytotoxic treatment (group 1); all of these had cytogenetic abnormalities and died shortly after diagnosis. 22 children had constitutional anomalies (group 2). The remaining 24 MDS were considered as 'primary' (group 3). Hypoplastic marrow was found in nine cases, and only 53% of the MDS fitted the adult FAB classification. Transformation to AML occurred in 11 cases, development of aplastic anaemia in three cases, and spontaneous remission in one case each of RA and RAEB. Differences were observed between groups 2 and 3 in terms of mean age at diagnosis (11.1 months v 5 years), rate of cytogenetic anomalies (15% v 38%) and rate of progression towards acute leukaemia (13% v 29%). In group 2, all the fur girls studied exhibited a polyclonal pattern of X-inactivation, which suggests that MDS may be only the haematological expression of an embryological defect with different target tissues. This study suggests that some MDS in childhood can exhibit particular features such as congenital anomalies associated with MDS, bone marrow hypoplasia, polyclonality, and spontaneous remission. It emphasizes that the FAB classification is not adequate for children and addresses the question of whether these MDS are always malignant diseases.

Adolescent↗
PubMed1995

CD34+ cell immunoselection from G-CSF-alone-primed peripheral blood in children with low body mass.

We report the data of CD34+ cell immunoselection from peripheral blood after G-CSF-alone mobilization (10 micrograms/kg/d s.c.) in nine children with neuroblastoma (median age 4-5 years (2-8), median body weight 16 kg (10-20). Leukaphereses were carried out on a Cobe Spectra separator and two consecutive harvests (4 blood volumes processed) were used for immunoselection on a Ceprate column. The yield of CD34+ cells in the purified fraction was 50% (23-80), with a median number of 2.8 x 10(6) CD34+ cells/kg (1-9.4). All patients were reinfused with selected CD34+ cells after busulfan 600 mg/m2 +melphalan 180 mg/m2 and achieved successful haemopoietic recovery.

Antigens, CD34↗
PubMed1994

Use of G-CSF alone to mobilize peripheral blood stem cells for collection from children.

We report the data of 19 children with neuroblastoma (NB) or Ewing's sarcoma (EW) who had peripheral blood stem cells (PBSCs) harvested after mobilization by: (1) cyclophosphamide (CY) + etoposide + G-CSF, (2) CY + GM-CSF, or (3) G-CSF alone. There were no consistent differences in the number of PBSCs collected following these three different mobilization regimens as assessed by CFU-GM. 17 patients were reinfused with PBSCs after myeloablative therapy and had successful haemopoietic recovery. These results show that in children with solid tumours such as NB or EW a sufficient number of PBSCs can be collected after G-CSF alone, and that PBSCs collected following stimulation by G-CSF alone are as effective in reconstituting haemopoiesis as those collected after mobilizing chemotherapy + HGFs.

Child↗
PubMed1994

[Megaloblastic anemia in children].

Megaloblastic anemia in children is mainly due to folic acid or vitamin B12 deficiencies. However dosages of these two vitamins must be performed before any vitamin supplementation or blood transfusions are given in order to establish precisely the etiologic diagnosis. A disorder in the metabolism of these vitamins must be considered in any infants in whom the onset of neurological abnormalities is associated with the characteristic hematological abnormalities. Imerslund's syndrome is a specific vitamin B12 malabsorption defect that warrants consideration as it is easy to recognize on the basis of the association of megaloblastic anemia and proteinuria. A possible drug-induced etiology must also be looked for. In the absence of vitamin deficiency, several rare etiologies must be considered. A macrocytosis, occasionally with megaloblastosis reflecting an abnormal erythropoiesis, may accompany an hemopathy, eventually malignant.

Anemia, Megaloblastic↗
PubMed1993

Ewing's sarcoma of the mandible: a case report.

The authors report the case of an 8-year-old boy with a Ewing's sarcoma localized to the mandible. The therapeutic modalities consisting of induction chemotherapy, surgical removal of the involved portion of the mandible with reconstruction using a parascapular osteo-cutaneous free flap, and maintenance chemotherapy are described. Emphasis is placed on the importance of performing a wide resection and the use of a multidisciplinary team approach in the treatment of these unusual tumours.

Cell Nucleolus↗
PubMed1992

[24 cases of human parvovirus B19 infection in children].

From January 1, 1987 through December 31, 1990, twenty-four pediatric patients with human parvovirus B19 (HPV B19) infection were seen. In every case the diagnosis was established by a positive capture immunoassay for IgM antibodies against the HPV B19. Four patients had hematologic manifestations, including one case of transient bone marrow aplasia revealing hereditary spherocytosis, one case of autoimmune hemolytic anemia with beta-thalassemia, and two cases of peripheral thrombocytopenia. Eight patients had skin lesions, with a morbilliform rash in six cases, erythema nodosum in one case, and Gianotti-Crosti syndrome in one case. No patients had erythema infectiosum. Seven patients developed joint manifestations: Henoch-Schönlein purpura in two cases, arthralgia in four cases, and polyarticular disease progressing to severe rheumatoid arthritis in a thirteen-year-old girl. Unremarkable symptoms of viral disease were seen in three patients. A five-month-old infant developed severe acute myocarditis. One patient with hepatitis A had acute liver failure. This study confirms the broad spectrum of clinical manifestations of HPV B19 infection. There were a number of unusual findings, including the high rate of joint manifestations (29%) and the severe course of some hematologic and myocardial manifestations. These results raise the question of whether the HPV B19 may be involved in the genesis of chronic juvenile arthritis.

Adolescent↗
PubMed1988

[Treatment of malignant intracranial germ-cell tumors in children. 7 cases].

Seven children, aged from 9 to 16 years, with intracranial germ cell tumour received post-operative chemotherapy prior to radiotherapy. Treatment consisted of several courses of vinblastine, bleomycin and cisplatin alternating or not with courses of cyclophosphamide and actinomycin D. In the 6 children whose tumours secreted alphafoetoprotein or chorionic gonadotrophin, chemotherapy brought these markers down to normal. Tumoral regression at radiology exceeded 75 per cent in 6 patients. These results demonstrate the effectiveness of chemotherapy against intracranial germ cell tumours such as pure germinomas or secreting tumours. They encourage the systematic use of pre-irradiation chemotherapy in order to reduce the radiation doses delivered and to improve the prognosis.

Adolescent↗
PubMed1987

[Treatment of febrile episodes in neutropenic children by ceftazidime combined with netilmicin. Results of a multicenter study apropos of 88 cases].

Infection is the most important cause of mortality in leucopenic patients. A broad spectrum antibiotic therapy is imperative in febrile and neutropenic patients. In a multicentric study we have used ceftazidime (100 mg/kg/d) and netilmicin (6 mg/kg/d) in 88 children (fever greater than or equal to 38.5 degrees C, neutropenia less than 500/mm3) treated for acute leukemias (59), non Hodgkin lymphomas (13) or solid tumors (16). Median age was 7 years (2 months-16 years). In patients who continued to remain febrile, vancomycin (40 mg/kg/d) was added after 48 hours. The effective treatment was continued until a neutrophil count greater than 1,000/mm3. The first combination (ceftazidime + netilmicin) was effective in 64 children (73%) and the second combination (ceftazidime + netilmicin + vancomycin) in 11 patients. Bacteria were isolated in 39 children: Escherichia coli: 9, Staphylococcus epidermidis: 9, Staphylococcus aureus: 8, Streptococcus: 6, Pseudomonas aeruginosa: 3, Streptococcus pneumoniae: 1, Haemophilus: 1, Klebsiella pneumoniae: 1, Proteus: 1, Serratia: 1, Flavobacterium: 1. In these 39 patients, 30 became apyretic with ceftazidime and netilmicin and 6 after vancomycin. All blood culture were negative after the first combination. The median duration of antibiotic therapy was 14 days (5-9 days: 28, 10-20 days: 43, greater than 20 days: 17). There were no death, no superinfection. Tolerance was good without kidney or liver or biological perturbation. We conclude that the combination ceftazidime and netilmicin is effective in neutropenic children.

Adolescent↗
PubMed1982

Coexistence of minicores, cores, and rods in the same muscle biopsy. A new example of mixed congenital myopathy.

The authors report on the ultrastructural study of a muscle biopsy carried out in a child 6 years after the first biopsy which had led to diagnosis of "multicore disease". Clinical evolution following a few years of muscular involvement had been favorable. The lesions observed in the second biopsy were again characterized by the presence of multicores, but in addition to cores and rods. All of these abnormalities could sometimes be noted in a single muscle fiber. Although rare cases of an association of two types of lesions have been reported, no study has hitherto shown multicores, cores, and rods in the same biopsy. This observation might confirm the possibility of common pathogenic mechanisms producing these lesions.

Biopsy↗
PubMed1980

["Benign" intracranial hypertension due to bilateral thrombosis of the lateral sinuses treated with venous bypass (author's transl)].

The authors report on a case of "benign" intracranial hypertension due to bilateral thrombosis of the lateral sinuses and internal jugular veins of infectious origin (otitis). A venous by-pass was established between one of the lateral sinuses and an external jugular vein. As early as the first post-operative week, the intracranial pressure was reduced and the visual disorders regressed. Such venous by-passes, now made possible by micromicrosurgical techniques, can be suggested for treating occlusions of the intracranial sinuses due to thrombophlebitis, traumatisms or tumours.

Adolescent↗