PubMed Health⌕ Search

Biomedical subjects

E Sariban

Publications and source records attributed to E Sariban.

At least 55 records · Page 3Linked to original sources

[Stomatococcus mucilaginosus meningitis in immunocompromised child].

BACKGROUND: Opportunistic infections are responsible for significant morbidity and mortality in patients with malignancy developing granulocytopenia as a result of therapy. A case of Stomatococcus mucilaginosus meningitis is reported. CASE REPORT: A 2 year-old boy was admitted because he had developed fever and neutropenia during chemotherapy given for neuroectodermal tumor. He was previously treated for a Stomatococcus mucilaginosus septicemia with vancomycin given for 15 days through an intravenous catheter which has subsequently been left in place. At admission, fever was associated with severe degree of aplasia (70 WBC/mm3). The patient was given IV ceftazidime plus amikacin. Two days later, the boy developed acute meningitis due to Stomatococcus mucilaginosus; he was then given IV vancomycin (40 mg/kg/d) and imipenem (100 mg/kg/d). Persistence of abnormal clinical and bacteriological findings required subsequent intrathecal administration of vancomycin (1.5 mg/d) for 5 days. CSF cultures were negative 2 days later, leading to stop IV antibiotics after 3 weeks. The catheter was removed 1 week later. CONCLUSION: This patient represents the third reported case of Stomatococcus mucilaginosus meningitis. Combined intrathecal and systemic administration of vancomycin seems to have been useful in our case.

Child, Preschool↗

Complete remission following donor leukocyte infusion in ALL relapsing after haploidentical bone marrow transplantation.

A 7-month-old boy with a high risk ALL harbouring the translocation (4;11) was grafted with an haploidentical bone marrow from paternal origin. At time of relapse, 11 months after BMT, he received donor leukocyte infusions (DLI) which put him in second CR. GVHD and pancytopenia occurred 2 weeks after DLI and were fully reversed with CsA + prednisolone. Six months later, the child continues to be in second CR, off steroid therapy, without any signs of GVHD. Our limited experience indicates that a second CR can be obtained with acceptable toxicity by DLI in very high risk ALL children who have been previously grafted with haploidentical bone marrow cells.

Bone Marrow Transplantation↗

Bone marrow transplantation corrects the splenic reticuloendothelial dysfunction in sickle cell anemia.

In sickle cell anemia (SCA), the loss of reticuloendothelial function is the result of vasoocclusive events occurring in the spleen. Such asplenia occurs early in the course of the disease and is considered to be permanent in late childhood. In this report, three patients 10, 11, and 14 years of age suffering from severe SCA and found to be asplenic were treated by bone marrow transplantation (BMT). Before transplantation, all three patients had loss of reticuloendothelial splenic function, as assessed by the presence of abundant Howell-Jolly bodies on blood smears and absence of technetium 99m (99mTc) splenic uptake. After BMT, Howell-Jolly bodies disappeared from blood smear, whereas 99mTc isotopic scan found normal isotope uptake. Our data indicate that BMT can correct "permanent asplenia" in SCA patients. However, it remains to be determined if such treatment can also correct other SCA-related organ dysfunctions.

Adolescent↗

T helper type 2-like cells and therapeutic effects of interferon-gamma in combined immunodeficiency with hypereosinophilia (Omenn's syndrome).

We characterized the defects of CD4+ cells in a 17-month-old girl suffering from combined immunodeficiency with hypereosinophilia (Omenn's syndrome). Because the vast majority of peripheral blood CD4+ cells expressed the CD45R0 isoform, we purified circulating CD4+ CD45R0+ cells from the patient and healthy individuals in order to compare their production of cytokines. The patient's CD4+ CD45R0+ cells spontaneously produced high levels of interleukin-5 (IL-5) in vitro (1600 pg/ml after 24 h of culture) and this was associated with the presence of IL-5 in serum (323 pg/ml). After stimulation with phorbol 12-myristate 13-acetate (PMA) and calcium ionophore A23187, they produced higher levels of IL-4 (306 vs. 55 +/- 4 pg/ml) and IL-5 (2900 vs. 213 +/- 72 pg/ml) and lower levels of IL-2 (17 vs. 63 +/- 17 IU/ml) and interferon-gamma (IFN-gamma) (16 vs. 299 +/- 70 IU/ml) than controls CD4+ CD45R0+ cells. This T helper type 2 (Th2) pattern was confirmed by the detection using reverse polymerase chain reaction of IL-4, IL-5 and IL-10 mRNA within peripheral blood mononuclear cells. During a therapeutic trial with human IFN-gamma (40 micrograms/day) which ameliorated the clinical status of the patient, we observed a down-regulation of the in vivo expression of IL-5 and IL-10, a normalization of the eosinophil count and an improvement of the T cell response to phytohemagglutinin. This observation indicates for the first time that Th2-like cells might be involved in certain forms of congenital immunodeficiency and that IFN-gamma might down-regulate their activities in vivo.

CD4 Antigens↗

MRI in seven cases of Rathke's cleft cyst in infants and children.

Seven MRI observations of presumed Rathke's cleft cysts in infants and children are reported. Age ranged between 6 months and 13 years. Size of the cysts ranged between 4 and 20 mm. While 3 small cysts were detected incidentally, 4 were large enough to cause symptoms secondary to compression of the pituitary gland and suprasellar components. The MRI appearance of the Rathke's cleft cysts varied according to their nature. MRI provides a major contribution to the diagnosis of these lesions; nevertheless, it often remains difficult to differentiate complex Rathke's cleft cysts from cystic craniopharyngiomas. The difficulties related to this differentiation are discussed.

Adolescent↗

[Granulocytic sarcoma with mediastinal involvement].

BACKGROUND: Granulocytic sarcoma is more frequent in adults; it can be a tumoral localization of acute non-lymphoblastic leukemia. This report describes an infantile case revealing an acute myeloid leukemia. CASE REPORT: A 14 month-old girl was admitted because of enlarged bilateral cervical lymph nodes. They had increased in volume over the past month and were accompanied by fever and anorexia. The liver and spleen were moderately enlarged. Hematologic data were: Hb = 7.5 g%; leucocytes = 14,900/mm3; platelets = 614,000/mm3. There were no abnormal cells. X-rays and MRI showed a mediastinal mass. Bone scintigraphy was normal but the myelogram showed a few atypical cells that were also seen in a biopsy of the cervical lymph node. Electron microscopic examination of these cells showed that they were myeloid in origin. This was confirmed by immunohistochemistry. The cytogenetic examination showed many chromosomal abnormalities in the lymph node and myelogram. The child is now recovered after nine months of chemotherapy. CONCLUSION: Diagnosis of acute myeloblastic leukemia in children is difficult when it is revealed by granulocytic sarcoma without major infiltration of bone marrow. The clinical presentation as a mediastinal mass is rare. The cytogenetic study was determinant for diagnosis.

Bone Marrow↗

Bone marrow transplantation in sickle cell disease: the Belgian experience.

Twenty eight patients underwent bone marrow transplantation (BMT) for sickle cell anemia in Belgium. The patients were originating from Central Africa, were young and symptomatic. Engraftment occurred in all patients and was sustained in 25. In 3 patients, a bone marrow rejection was observed. One of these patients underwent a second BMT and had an uneventful recovery. One patient died 3 months after BMT of graft-versus-host disease (GVHD). Twenty seven patients are alive and 25 are free of vaso-occlusive related manifestations with a follow-up ranging from 4 to 78 months. So far, eight patients went back to Africa and continue to do well.

Adolescent↗

Bone marrow transplantation for severe sickle cell anaemia.

Five children with sickle cell anaemia underwent bone marrow transplantation (BMT) for severe clinical disease. The conditioning regimen for BMT was in busulfan plus cyclophosphamide. The allograft contained more than 5 x 10(8) nucleated cells per kg recipient. Prophylaxis of GVHD consisted of methotrexate and cyclosporin A. Therapy was well tolerated. Duration of neutropenia (less than 0.5 x 10(9)/l) was short (14-25 d). Platelet recovery (greater than 50 x 10(9)/l) occurred between day 12 and 45. The patients have been followed up for 8-28 months. No major infections occurred and long-term BMT-related toxicity was limited to mild, chronic GVHD in one patient. Mean haemoglobin levels remained above 10 g/dl. Haemoglobin electrophoresis showed AS patterns in all grafted patients--all marrow donors having sickle cell trait. From our preliminary data, we conclude that BMT or sickle cell anaemia is curative, well tolerated and should be proposed for suitable patients.

Anemia, Sickle Cell↗

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↗

[Autologous bone marrow graft in solid tumors in childhood].

Curability in children suffering from malignant solid tumor is 50%. Thus, high dose chemotherapy with or without total body irradiation followed by autologous bone marrow transplantation (ABMT) has been proposed to patients suffering from cancer either at initial diagnosis (poor prognosis tumor) or at relapse. Thanks to these studies, drugs having dose effects properties have been selected. In some tumors, ABMT has significantly improved patients median survival. It remains to be determined if: 1. high dose chemotherapy protocols with ABMT are superior to new aggressive chemotherapeutic protocol without ABMT. 2. ABMT increases the curability of high risk patients.

Bone Marrow Transplantation↗

[Indications for bone marrow graft in severe hemoglobinopathies].

Allogenic bone marrow transplantation (ABMT) is the only curative approach for sickle cell anemia and major beta-thalassemia. In sickle cell anemia, ABMT can be proposed for severe clinical disease. In major beta-thalassemia, it must be proposed to young patients who have an HLA identical familial donor.

Anemia, Sickle Cell↗

[Medulloblastoma: analysis of 14 cases treated at the J. Bordet Institute and literature review].

Between 1966 and 1986, 14 patients with histologically verified cerebellar medulloblastomas were seen at J. Bordet Institute after total (4 cases) or partial (10 cases) tumor removal. Twelve received postoperative irradiation after some courses of chemotherapy (Vincristine, Procarbazine, Methotrexate) for 5. In 10 cases the X ray treatment was given to the whole cerebrospinal axis with approximately 50 Gy delivered to the primary tumor site, 25-35 Gy to the remaining brain and spinal cord. As in other series of the literature the long term prognosis is not too good, with approximately 50% of the patients surviving more than 5 years. Failure at the primary site happened in 4 cases. As a consequence of the small number of patients treated, no conclusion can be drawn from risk factor analysis. Yet it appears that younger patients fared better than older ones. A detailed description of the side effects of the treatment is therefore given for the five children treated at prepubertal age: some growth impairment seems to be the rule, a consequence of GH deficiency--if not compensated--and of the direct effect of ionizing radiation on the vertebral bodies. While discussing the progress of neurosurgery and radiotherapy for the treatment of medulloblastoma, the authors emphasize the need of more efficient adjuvant chemotherapy regimens and the interest of multicenter trial for evaluating them.

Adolescent↗

[Bone marrow graft in hereditary diseases].

Since 1968, bone marrow transplantation became the first line therapy for selected metabolic and immunological hereditary disorders. Actually, advances in the supportive care in bone marrow transplantation and a better knowledge of the immunology of BMT complications has been associated with a better disease correction and an increase in long term survival. New approaches are under investigation and include: hematopoietic growth factors, enzymatic replacement and gene therapy. However at the present time BMT is still the only curative treatment for selected hereditary disorders.

Bone Marrow Transplantation↗