Browsing by Author "Barriga, F"
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- ItemChildhood tumors(W B SAUNDERS CO-ELSEVIER INC, 2000) Herrera, JM; Krebs, A; Harris, P; Barriga, FPediatric solid tumors represent a distinct set of malignancies of embryonal origin whose incidence peaks in the first years of life. Specific genetic anomalies with pathogenic significance, which have helped to define the diagnosis better and to improve the prognosis of children with these tumors, recently have been discovered. Survival of children with solid tumors also has improved significantly because of effective multidisciplinary care, which, in this case, always involves chemotherapy and surgery. These favorable results require that children with these diseases are referred and treated at institutions that have multidisciplinary teams and the infrastructure and expertise for caring for these children. Diagnostic and therapeutic principles for the most common childhood solid tumors are discussed in this article, with an emphasis on surgical procedures.
- ItemHemolytic uremic syndrome in a child with leukemia and cytomegalovirus infection(2000) Cavagnaro, F; Barriga, FA 2-year-old boy had a severe cytomegalovirus (CMV) infection with multi-organ involvement, while on maintenance therapy for acute lymphoblastic leukemia. The patient was treated with intravenous gancyclovir, with a marked improvement in his clinical status, with the exception of a progressive deterioration of the renal function. He also developed hemolytic anemia and thrombocytopenia, suggesting a diagnosis of atypical hemolytic uremic syndrome (HUS). A percutaneous renal biopsy showed lesions consistent with HUS, but no evidence of CMV infection. The patient had a good clinical outcome with no evidence of renal sequelae. We report a rare association of CMV infection and HUS in the pediatric age-group, which suggests a possible cause-effect relationship that deserves further evaluation.
- ItemRapid intraclonal switch of lineage dominance in congenital leukaemia with a MLL gene rearrangement(1995) Ridge, SA; Cabrera, ME; Ford, AM; Tapia, S; Risueno, C; Labra, S; Barriga, F; Greaves, MFWe describe a case of neonatal mixed lineage leukaemia which presented with a dominant B progenitor lymphoblast population plus a minor monocytic component. Treatment of the patient with corticosteroid and Ara-C resulted in loss of lymphoblasts and a rapid (within 7 days) increase and dominance of the monocytic component. The common clonal origin of the two cell types was evident from the identical rearrangement in the MLL gene and a shared rearrangement of one IGH allele. In common with other neonatal or infant ALL with MLL gene rearrangements, this leukaemia may have originated in a common B-monocytic lineage stem cell during foetal haemopoiesis. The observations further suggest that the therapeutic impact of the MLL gene rearrangement is to some extent dependent on the cellular context in which it is expressed.
- ItemRefractory enteric amebiasis in pediatric patients with acute graft-versus-host disease after allogeneic bone marrow transplantation(LIPPINCOTT WILLIAMS & WILKINS, 2000) Perret, C; Harris, PR; Rivera, M; Vial, P; Duarte, I; Barriga, F
- ItemStem cell transplantation in patients with severe congenital neutropenia without evidence of leukemic transformation(2000) Zeidler, C; Welte, K; Barak, Y; Barriga, F; Bolyard, AA; Boxer, L; Cornu, G; Cowan, MJ; Dale, DC; Flood, T; Freedman, M; Gadner, H; Mandel, H; O'Reilly, RJ; Ramenghi, U; Reiter, A; Skinner, R; Vermylen, C; Levine, JESevere congenital neutropenia (CN) (Kostmann syndrome) is a hematologic disorder characterized by a maturation arrest of myelopoiesis at the promyelocyte/myelocyte stage of development, This arrest results in severe neutropenia leading to absolute neutrophil counts (ANC) below 0.2 x 10(9)/L associated with severe bacterial infections from early infancy. Data on over 300 patients with CN collected by the Severe Chronic Neutropenia International Registry (SCNIR) beginning in 1994 indicate that more than 90% of these patients respond to recombinant human granulocyte colony stimulating factor (r-HuG-CSF) treatment with an ANC greater than 1.0 x 10(9)/L. For patients who are refractory to r-HUG-CSF treatment and continue to have severe and often life-threatening bacterial infections, hematopoietic stem cell transplantation is the only currently available treatment. We report on a total of 11 patients with CN reported to the SCNIR who underwent transplantation for reasons other than malignant transformation between 1976 and 1998. Of these patients, 8 were nonresponders or showed only partial response to r-HuG-CSF treatment with ongoing infections. Results from these patients suggest that transplantation of stem cells from an HLA-identical sibling is beneficial for patients refractory to r-HuG-CSF. (Blood. 2000;95:1195-1198) (C) 2000 by The American Society of Hematology.
- Itemt(1;5)(q23;q33) in a patient with high-risk B-lineage acute lymphoblastic leukemia(1996) Barriga, F; Bertin, P; Legues, E; Risueno, C; Andrade, W; Cabrera, E; Grebe, GThe t(1;5)(q23;q33) is a rare genetic anomaly that was reported previously in two infants with a myeloproliferative disorder and eosinophilia and in one adult patient with acute nonlymphocytic leukemia (ANLL). A 13-year-old boy with high-risk early pre-B acute lymphoblastic leukemia (ALL) who presented to our institution carried the t(1;5)(q23;q33). He had an initial blast count of 230 x 10(9)/L and responded poorly to prednisone. Complete remission ICR) was achieved, and he had a bone marrow (BM) relapse 3 months after despite intensive consolidation therapy He underwent allogeneic BM transplantation (BMT) from a human leukocyte antigen (HLA)-identical sibling in early relapse with total body irradiation (TBI) and cyclophosphamide conditioning. He had a short second CR with a central nervous system (CNS) relapse on day +106 after BMT. Two of the previously reported patients also did not respond to chemotherapy. The t(1;5)(q23;q33) appears to be a rare lineage nonspecific anomaly related to hematologic malignancies that are resistant to current therapy.
- ItemTranexamic acid inhibits fibrinolysis, shortens the bleeding time and improves platelet function in patients with chronic renal failure(F K SCHATTAUER VERLAG GMBH, 1999) Mezzano, D; Panes, O; Munoz, B; Pais, E; Tagle, R; Gonzalez, F; Mezzano, S; Barriga, F; Pereira, JBackground: A defect in platelet function is the main determinant of the prolonged bleeding time in chronic renal failure (CRF). We previously reported a significant correlation between platelet abnormalities and elevated plasma markers of plasmin and thrombin generation. Our aim was to explore thr effect of inhibiting both plasmin action with tranexamic acid (TA) and thrombin production with low molecular weight heparin (LMWH), on the bleeding time (BT) and platelet function in patients with CRF. Methods: 37 patients with CRF (mean creatinine 8.6 +/- 4.4 mg/dl) under conservative treatment, with prolonged BT, entered this study and received TA during 6 days, with (n = 24) and without LMWH (n = 13). BT, platelet aggregation/secretion, platelet granule contents, von Willebrand factor and parameters of coagulation and fibrinolysis were recorded before and at the end of treatment. Results: The BT was shortened in 26/37 (67%) patients. This effect was associated with significant improvement of platelet aggregation and secretion, with decrease to a normal range of fibrin/fibrinogen degradation products, mild increase in plasmin-antiplasmin complexes and pronounced reduction of circulating plasminogen. No differences were seen among patients with or without LMWH. No serious side effects or complications were observed. Interpretation: These findings indicate that the activation of fibrinolysis plays a significant role in the defect of primary hemostasis in patients with CRF. inhibition of plasmin activity with TA shortens the BT and improves platelet function in the majority of patients with severe disease.