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J Ortega

Publications and source records attributed to J Ortega.

At least 19 recordsLinked to original sources

Clinical importance of myeloid-antigen expression in acute lymphoblastic leukemia of childhood.

BACKGROUND: Leukemic cells in 15 to 25 percent of patients with acute lymphoblastic leukemia (ALL) express myeloid antigens as well as lymphoid antigens (the latter reflecting B-cell or T-cell lineage). The relations of myeloid-antigen expression to other features of ALL and to prognosis have been controversial. METHODS: We analyzed clinical and laboratory features present at diagnosis in 236 consecutive cases of ALL in children. Immunophenotyping, including single- and dual-fluorescence analyses, was used to classify leukemic cells as B or T lymphoblasts and also to identify myeloid-antigen expression--the simultaneous expression of lymphoid-associated antigens and at least one of three myeloid-associated antigens (CD33, CD13, and CD14) on cells classified as L1 or L2 according to the French-American-British system. RESULTS: Forty-five of 185 patients with B-lineage ALL had myeloid-antigen expression, as did 8 of 41 patients with T-lineage ALL. In 10 patients, the lineage could not be determined. Myeloid-antigen expression was associated with L2 morphology (P less than 0.05), but it did not correlate with other prognostic features recognized previously. Multivariate analysis showed that myeloid-antigen expression was an important predictor of relapse in childhood ALL and the most significant prognostic factor statistically (P less than 0.0001). A white-cell count greater than or equal to 50 x 10(9) per liter at diagnosis was also an important and highly significant prognostic feature (P less than 0.001). After 40 months, the estimated disease-free survival for patients with ALL was 84 percent for those without myeloid-antigen expression and with a low white-cell count, 57 percent for those without myeloid-antigen expression and with a high white-cell count, 47 percent for those with myeloid-antigen expression and a low white-cell count, and 26 percent for those with myeloid-antigen expression and a high white-cell count (P less than 0.00001). CONCLUSIONS: Myeloid-antigen expression is an important independent predictor of a poor response to chemotherapy in childhood ALL.

Adolescent

The surgical management of children with incompletely resected hepatic cancer is facilitated by intensive chemotherapy.

This prospective study was undertaken to evaluate the efficacy of continuous-infusion doxorubicin and cisplatin (CI-DOX/CPPD) for the treatment of children with incompletely resected hepatic cancer. Of the 46 evaluable patients, 32 had hepatoblastoma (70%) and 14 had hepatocellular carcinoma. Ten children had stage II tumors (microscopic residual), 25 were defined as stage III (gross residual), and 11 had distant metastasis (stage IV). Twelve patients underwent initial incomplete resection of their hepatic lesions and in the 34 others tumor biopsy specimens were obtained. Chemotherapy was administered and the majority of the children (70%) had an excellent clinical response with a decrease in both alpha-fetoprotein levels and measured tumor dimensions. The combination of CI-DOX/CPDD clearly facilitated surgical management, allowing for delayed hepatic resections in 20 of the 34 patients (59%) whose tumors were initially biopsied and considered to be unresectable. Overall survival in this study demonstrates a significant improvement in comparison to the historical controls. Twenty-one patients (46%) remain in complete clinical remission an average of 30 months following diagnosis (range, 17 to 40 months). The outcome of the children with hepatoblastoma was much better than those with hepatocellular carcinoma (63% v 17% survival). Survival of the 20 children who underwent delayed hepatic resections was not statistically different from the 12 patients whose hepatic tumors were removed at the initial laparotomy (41% v 58% survival). Although no obvious survival advantage was observed in those patients who underwent initial hepatic resections, there did appear to be an increased risk of postoperative complications in children whose tumors were resected following chemotherapy (8% v 25%).

Adolescent

Influence of age, sex, and concurrent intrathecal methotrexate therapy on intellectual function after cranial irradiation during childhood: a report from the Children's Cancer Study Group.

Standard IQ tests were performed in 70 children and adolescents 3.5-10 years (median 5 yrs) after 24 Gy cranial irradiation, with or without concurrent intrathecal methotrexate, for central nervous system prophylaxis of acute lymphoblastic leukemia. Lower IQ scores correlated with younger age, female gender, and concomitant intrathecal methotrexate therapy. Multivariate analysis identified age as the most important determinant of the three factors. The lowest IQ scores were observed in girls who were less than 5 years old at the time of irradiation and who received concurrent intrathecal methotrexate. The observed adverse contribution of simultaneous intrathecal methotrexate therapy on IQ outcome may have significant implications for school performance, peer relationships, and occupational expectations.

Age Factors

A new technical approach for retrograde administration of cardioplegic solutions.

Myocardial protection via the coronary sinus is now currently used by several groups. Although it has generally provided satisfactory results, some of its problems are still not completely resolved. We present a new technique of cardioplegia delivery through the coronary sinus with a Pezzer catheter inserted into it and secured in place by a purse string suture. We believe that this method is safer and more reliable than others.

Cardiac Catheterization

The Intergroup Rhabdomyosarcoma Study-I. A final report.

The results of treatment of 686, previously untreated patients younger than 21 years with rhabdomyosarcoma or undifferentiated sarcoma, who were entered on Intergroup Rhabdomyosarcoma Study-I (IRS-I) were analyzed after a minimum potential follow-up time of 7 years. Patients in Clinical Group I (localized disease, completely resected) were randomized to receive either vincristine, dactinomycin, and cyclophosphamide (VAC) or VAC + radiation. At 5 years, approximately 80% of patients given either treatment were still disease-free and there was no significant difference between treatments in the overall percentages of patients surviving of 93% and 81%, respectively (P = 0.67). Patients in Clinical Group II (regional disease, grossly resected) were randomized to receive either vincristine and dactinomycin (VA) + radiation or VAC + radiation. At 5 years, 72% and 65% of the patients, respectively, were disease-free and there was no evidence of a difference between treatments (P = 0.46). The overall survival percentage at 5 years was approximately 72% for both treatments. Patients in Clinical Groups III (gross residual disease after surgery) and IV (metastatic disease) were randomized to receive either "pulse" VAC + radiation or "pulse" VAC + Adriamycin (doxorubicin) + radiation. The complete remission (CR) rate was 69% in Clinical Group III and 50% in IV, with no statistically significant difference in CR rates between treatments in either group. Those who achieved a CR had a nearly 60% chance of staying in remission for 5 years in Clinical Group III compared with approximately 30% in Clinical Group IV. The overall survival percentage at 5 years was 52% in Clinical Group III compared to 20% in Clinical Group IV (P less than 0.0001). The 5-year survival percentage for the entire cohort of 686 patients was 55%. Survival after relapse was poor, being 32% at 1 year and 17% at 2 years. The risk of distant metastasis was much greater than the risk of local recurrence within each clinical group, and there was no evidence of differing types of relapses between treatments. Primary tumors of the orbit and genitourinary tract carried the best prognosis, whereas tumors of the retroperitoneum had the worst prognosis. The authors conclude that for the therapeutic regimens evaluated there was no therapeutic advantage to including radiation in the treatment of Clinical Group I disease, or cyclophosphamide given as a daily low-dose oral regimen in the treatment of Clinical Group II disease or Adriamycin in the treatment of Clinical Groups III and IV diseases.

Adolescent

Danazol for children with immune thrombocytopenic purpura.

Ten patients with steroid-dependent or refractory immune thrombocytopenic purpura were treated with danazol in an attempt to improve their platelet counts. Nine of the ten children exhibited an excellent initial response to the drug, with five achieving a complete, unmaintained remission. Two others were able to have their prednisone doses tapered with a resultant disappearance of steroid-induced adverse effects. No significant adverse effects of danazol were noted. Danazol seems to be effective in the treatment of children with immune thrombocytopenic purpura and would warrant additional studies to determine its proper role in the management of this common pediatric ailment.

Adolescent

Primary liver tumors in children: comparison of CT and MR imaging.

A prospective study was performed at two institutions to determine the role of magnetic resonance (MR) imaging in the evaluation of primary pediatric liver tumors. The study compared the usefulness of MR imaging and computed tomography (CT) in providing a correct diagnosis, assessing resectability, and determining tumor recurrence in 23 children with liver tumors. The information obtained with both modalities was correlated with surgical data. The results indicate that the accuracy of MR imaging in helping to differentiate benign from malignant lesions is high and identical to that of CT, but not good enough to obviate surgical diagnosis. While all five hepatocellular carcinomas were correctly diagnosed preoperatively with both modalities, neither CT nor MR imaging could help differentiate these carcinomas from aggressive hepatoblastoma or primary hepatic lymphoma. MR imaging helped correctly predict tumor resectability in all 18 children with malignant hepatic tumors; in one, exact tumor extension was misjudged with CT. Postoperative MR studies suggested tumor recurrence in three children who had unremarkable CT examinations. Second-look surgery showed recurrent tumor in two and fibrosis with chronic inflammation at the surgical margin in the third child.

Adolescent

Bone marrow transplantation for constitutional pure red cell aplasia.

Constitutional pure red cell aplasia (CPRCA) is a syndrome of failed erythropoiesis usually diagnosed within the first year of life. Four patients with CPRCA received transplants with marrow from their HLA-identical, mixed lymphocyte culture-nonreactive siblings. All patients were resistant to corticosteroid therapy and were dependent on regular red cell transfusions for at least 5 years. Three patients were conditioned with procarbazine, antithymocyte globulin, cyclophosphamide, and busulfan, and one was conditioned with antithymocyte serum, cyclophosphamide, and busulfan. Three patients promptly had successful engraftments with establishment of donor hematopoiesis. One patient initially rejected his graft but received a successful retransplant. All patients are currently alive with Karnofsky performance scores of 100 and normal erythropoiesis of donor origin. Despite a history of multiple transfusions, bone marrow transplantation is a potentially curative therapy for patients with CPRCA.

Adolescent

Brain-stem tumors in childhood: a prospective randomized trial of irradiation with and without adjuvant CCNU, VCR, and prednisone. A report of the Childrens Cancer Study Group.

Seventy-four children with a brain-stem tumor diagnosed between 1977 and 1980 were entered into a prospective study in which exploration and assessment for resection were optional, radiation treatment using standard methods was required, and randomization occurred with regard to the use of adjuvant chemotherapy (1-(2-chloroethyl)-1-nitrosourea, vincristine, and prednisone) or no further treatment. The overall 5-year survival rate was 20% and was not improved by the adjuvant chemotherapy program. An increased risk of infection was associated with the adjuvant therapy.

Adolescent