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Biomedical subjects

A Ragab

Publications and source records attributed to A Ragab.

53 records · Page 3Linked to original sources
PubMed1988

Pyridoxal 5'-phosphate levels in children with sickle cell disease.

Pyridoxal 5'-phosphate (PLP), the major coenzyme form of vitamin B6, is known to have antisickling properties in vitro. Recently, low plasma PLP levels were reported in a group of adults with sickle cell anemia. We measured the plasma PLP levels in a group of 55 asymptomatic nontransfused children with sickle cell diseases (SCD) to determine the prevalence of low plasma PLP levels in this population. Comparative studies were made with the measurement of PLP in three other groups serving as controls: Group A (black children, n = 36); Group B (white children, n = 37); and Group C (black adults, n = 13). PLP was measured directly in plasma by a radioenzymatic technique. The results of these comparisons showed that there was no statistically significant difference in plasma PLP of black children with SCD (10.7 +/- 10.0 ng/ml) as compared with black control children (group A, 9.0 +/- 12.3 ng/ml). The low plasma levels PLP in these two groups were significantly lower than that of the plasma PLP of white control children (group B, 15.85 +/- 15.92 ng/ml). This data suggest that a high prevalence of low PLP levels exists in black children seen at Grady Memorial Hospital, both with and without SCD.

Adolescent↗
PubMed1987

Intensive chemotherapy including cisplatin with or without etoposide for children with soft-tissue sarcomas.

Forty-two children, 6 months to 17 years of age with newly diagnosed soft-tissue sarcomas (gross residual or metastatic), were treated according to either of two pilot protocols that included intensive chemotherapy before irradiation. Vincristine, actinomycin D, cyclophosphamide, and doxorubicin were used in various combinations with cisplatin alone (regimen 35) or with cisplatin plus etoposide (regimen 36) in a 20-week induction treatment; irradiation (4,000 cGy) was delayed until week 6. Fourteen (82%) of the 17 patients on regimen 35 and 15 (60%) of the 25 on regimen 36 had a complete response. Although severe leukopenia was frequent in both groups (88% and 84% of patients), there were only two fatal infections and no early deaths. Other potentially serious toxicity included a greater than 10% weight loss in 52% of the patients and hypomagnesemia in 74%. An average of 75-100% of the prescribed drug doses were administered during the induction phase of therapy. We conclude that this intensified treatment is toxic but feasible to deliver. The higher overall response rate compared to that in the preceding Intergroup Rhabdomyosarcoma Study (69% vs 53%) suggests improved therapeutic efficacy that warrants further evaluation of both regimens.

Adolescent↗
PubMed1987

Localized orbital rhabdomyosarcoma. An interim report of the Intergroup Rhabdomyosarcoma Study Committee.

Children with rhabdomyosarcoma (RMS) (1461 total cases) were included in two prospective, randomized protocols conducted by the Intergroup Rhabdomyosarcoma Study (IRS) Committee from November 1972 to June 1983. Treatment assignment was determined by extent of tumor at diagnosis (clinicopathologic group) and not by primary site. Accordingly, 127 patients with RMS confined to the region of the eyelid and orbit were treated by a variety of regimens characterized by initial partial tumor resection followed by concurrent multiagent chemotherapy and orbital radiation therapy. Recurrent tumor developed in ten patients. Seven relapsed initially in the orbit (of whom 4 were salvaged) and three in regional nodes. In addition to the six patients who died of tumor, three died of other causes for a 93% 3-year actuarial survival rate. In 89 children who had subtotal resection or biopsy only, the local control rate with radiotherapy and chemotherapy was 94%.

Adolescent↗
PubMed1986

Late effects of therapy in orbital rhabdomyosarcoma in children. A report from the Intergroup Rhabdomyosarcoma Study.

Late effects of therapy were evaluated in 50 children surviving orbital rhabdomyosarcoma following treatment on Intergroup Rhabdomyosarcoma Study I. All patients had microscopic or gross tumor present following surgery and subsequently received radiotherapy and various combinations of chemotherapeutic agents. Problems in the eye included infections and functional and structural changes. Decreased vision in the treated eye was the most common functional problem and in most patients related to cataract formation, which occurred in 90% of eyes. Changes in the cornea and retina were also seen. Bony hypoplasia of the orbit and facial asymmetry were present in one half of the children. Gonadal development was normal. Statural growth was retarded in 61% of the patients. All children were in school, with five having learning or behavioral problems. One child developed acute myeloblastic leukemia. The excellent survival in patients with orbital rhabdomyosarcoma provides support for treatment programs that use multiple-agent chemotherapy and radiotherapy and do not include orbital exenteration initially.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1985

Sarcomas of the vagina and uterus: the Intergroup Rhabdomyosarcoma Study.

During a 12-year period (1972-1984), 43 patients with sarcomas of the female genital tract were admitted to the Intergroup Rhabdomyosarcoma Study (IRS), including 31 with primary tumors of the vagina; and 12 with tumors of the uterus, including the cervix. Thirty-four of these can be evaluated on the basis of periods of observation from 18 months to 12 years. Primary tumors of the uterus were a distinct group, distinguished from those arising in the vagina by patient age-range and probably by prognosis, as well as site. Patients with vaginal tumors, with a mean age of 1.8 years, responded to a multimodality approach employing combinations of chemotherapy (vincristine sulfate and actinomycin D, or the aforementioned two drugs with cyclophosphamide +/- doxorubicin hydrochloride), irradiation, and/or surgery, with only one tumor-related death, among 24 evaluable patients. In contrast, among the patients with primary uterine tumors, in which the mean age was greater than 14 years, four of ten evaluable patients died secondary to tumor relapse or progression.

Adolescent↗
PubMed1984

Pre-B cell leukemia responds poorly to treatment: a pediatric oncology group study.

Seventy-eight of 362 children with acute lymphocytic leukemia (ALL) had leukemic cells similar in phenotype to normal pre-B cells. When the clinical and laboratory features of patients with pre-B and "null" cell phenotypes of ALL were compared, no significant differences were noted, except that the pre-B cell ALL phenotype had a higher percentage of black children. In contrast, patients with T cell ALL had a higher median age at diagnosis, frequent thymic involvement, and higher WBC counts. Patients with pre-B and "null" cell ALL were treated identically and patients with T cell ALL differently. Although no difference in remission induction rates was noted between patient groups with pre-B and "null" cell ALL, the remissions were of shorter duration for patients with pre-B cell ALL (p = 0.004). Similarly, overt leukemic involvement of both the central nervous system (CNS) and bone marrow was noted sooner in the patient group with pre-B cell ALL. Univariate and multivariate Cox life table regression analyses demonstrate the independent prognostic significance of the pre-B phenotype and illustrate that the prognostic influence of potential relapse risk factors, such as WBC, sex, and age, are specific for leukemia phenotype. These findings may have importance for the design and tailoring of therapy for children with acute leukemia.

B-Lymphocytes↗
PubMed1983

Mortality among children with rhabdomyosarcomas of the alveolar histologic subtype.

Of 116 patients with rhabdomyosarcoma of the alveolar histologic subtype who entered the Intergroup Rhabdomyosarcoma Study (IRS) from 1972 to 1978, there were 72 deaths (63%), compared with a mortality of 39% among patients with all nonalveolar types combined. The subgroup with alveolar histology contributed greater than 29% of the total mortality, although it represented 20% of the total patients (p less than .001). This recurrence rate reflects increased local, regional, and distant relapse. Differences were most marked in patients with grossly excised tumors (clinical groups I and II) in which the mortality was 44% in patients with alveolar tumors (48 patients) versus 13.5% in those with embryonal histology (111 patients), and 16% in all patients with nonalveolar tumor types. When survival was influenced by primary tumor site this reflected, in most body areas, an increase in the proportion of patients with the alveolar histology in that site. Patients with unresected or disseminated tumors (clinical groups III and IV) of the alveolar subtype have initial response rates to VAC and radiotherapy which are similar to those of patients with tumors of other cell subtypes (70% for group III and 50% for group IV). In these clinical groups, the differences in survival related to histologic subtype are not significant. The presence of the alveolar histologic subtype represents a rational basis for employing more intensive therapy in the management of patients with rhabdomyosarcomas.

Adolescent↗
PubMed1982

Properdin factor B and acute lymphocytic leukemia (ALL).

One hundred-sixty-four ALL patients were compared to 545 controls for differences in phenotype and gene frequencies at the Properdin factor B locus. In addition, 90 of the ALL patients were immune phenotyped. A significant association with the Bf F allele and ALL was found, resulting in an estimated relative risk of 3.62. There was no difference in the Bf S phenotype between ALL patients and controls. However, those homozygous for the Bf S allele are at a significantly low risk of 0.30 for developing ALL. ALL patients with a non-B/T cell type were 2.5 times more likely to be Bf SS homozygotes; in contrast, those patients with the pre-B cell type were 2.5 more likely to be Bf FF homozygotes. These data suggest association of the Bf locus with an ALL protection and/or susceptibility gene(s).

Alleles↗
PubMed1981

Low-dose adriamycin remission maintenance for advanced acute lymphoblastic leukemia: a Southwest Oncology, Group study.

A low-dose maintenance schedule of adriamycin was evaluated in children with advanced acute lymphoblastic leukemia. Thirty-six evaluable patients who achieved two or more remissions were given adriamycin, 10 mg/m2/week, as a single maintenance agent. The median duration of second remission was 17 weeks, but only 5 weeks for third and fourth remission. Although the therapy was not found effective in more advanced disease, the median duration of second remission approached that obtained from various multi-agent treatment schedules. We conclude that the therapeutic efficacy of this treatment schedule makes it appealing for incorporation into multi-drug combination regimens.

Adolescent↗
PubMed1978

Granulopoiesis in chronic myeloproliferative disorders in children.

Four children with chronic myeloproliferative disorders (three with Philadelphia [Ph1] chromosome-positive chronic myelogenous leukemia [CML] were studied with soft agar culture at diagnosis (before therapy) in an attempt to define abnormalities in granulopoiesis. The three patients with CML had elevated peripheral blood golony-forming cells (CFCs) and/or normal or decreased bone marrow CFCs (in those studied). Colony-stimulating activity (CSA) was markedly decreased or absent at diagnosis in all three. Maturation of myeloid cells eithin the colonies in agar was normal, indicating that no block in myeloid maturation was present. These findings are in general agreement with results previously reported in untreated adults with Ph1 chromosome-positive CML and further define the similarity with the adult form of the disease. One Ph1 chromosome-negative patient with a clinically similar chronic myeloproliferative disorder was studied and had similarly elevated peripheral blood CFCs. She had normal CSA with a similarly high WBC count. This finding was unexpected and suggests that, unlike the patients with CML, her monocytes were capable of elaboration CSA. This difference might prove helpful in the classification of this type of disorder in cases where the Ph1 chromosome abnormality is not present.

Adolescent↗
PubMed1977

Effects of continuous or discontinuous maintenance therapy on subsequent remission maintenance in childhood leukemia.

This study was designed to determine if resistance to a standard drug during the second remission of children with acute leukemia was reduced by discontinuation of therapy during the initial remission. The initial maintenance therapy was either 6-mercaptopurine (6-MP), methotrexate (MTX), or cyclophosphamide (CYC) given continuously to relapse or discontinued (at random) at 2 or 6 months. Following the initial relapse and after induction of a second complete remission, 72 evaluable patients received (continuously to relapse) either 6-MP (53 patients) or CYC (19 patients) for the second remission maintenance. Resistance of 6-MP occurred during the second maintenance, regardless of the drug used during the initial maintenance, in that the length of second remissions was significantly shorter than the length of first remissions. However, this resistance was most pronounced in patients who initially relapsed while on continuous 6-MP maintenance (medium duration of remission [MDR] of 9 weeks). Patients whose initial relapse occurred after the discontinuation of 6-MP had a MDR of 23 weeks and patients whose second remission was maintained with CYC (after relapse from initial continuous remission on 6-MP) had a MDR of 25 weeks.

Acute Disease↗
PubMedDate not supplied

Adolescent cancer survivors. Psychosocial and familial adaptation.

Adolescent cancer survivors were compared with nondiseased control subjects on measures of adaptation, coping, body image, sexual adjustment, psychopathology, and family functioning. Cancer survivors reported no major difficulties in social competence, overall coping, and family communication. Although their school teachers reported no symptoms of psychopathology, the cancer survivors did report body image disturbances and adjustment difficulties. Further, the surviving adolescents were eager to present themselves favorably. Compared with nondiseased control families, families of survivors were characterized as somewhat inflexible. Implications for clinical practice include the careful monitoring of youth who have survived cancer as well as sensitivity to underlying concerns that the survivors and their families may avoid.

Adaptation, Psychological↗
PubMedDate not supplied

Cyclocytidine in the treatment of refractory acute childhood leukemia: a Southwest Oncology Group Phase I-II study.

Thirty-seven children and adolescents with acute leukemia in relapse were treated with cyclocytidine in a cooperative group setting. Only one of the 27 evaluable patients achieved complete remission. A significant decrease (greater than or equal to 20%) in circulating and/or bone marrow leukemia cells occurred in an additional five patients. Drug toxicity was evaluated in 35 patients and included ten cases of jaw pain, two cases of hypotension, one case of fever, and one case of severe vomiting.

Adolescent↗