ISSN:0192-8562    

出版商:OVID    

年代:1980

数据来源: OVID

摘要:

The pretreatment characteristics of 936 children with previously untreated acute lymphoblastic leukemia diagnosed from 1972 to 1975 were analyzed to determine their ability to predict subsequent prognosis. The patients' treatment differed only in the type of CNS prophylaxis provided. Both univariate and multivariate analyses were performed to determine the relationship among patient characteristics and rate of remission induction, duration of initial bone marrow remission, and survival. The following patient characteristics were analyzed: initial white blood count (WBC), age at diagnosis, initial hemoglobin, initial platelet count, sex, race, pretreatment organomegaly, CNS leukemia at diagnosis, mediastinal mass, Down's syndrome, and the type of CNS prophylaxis provided. Down's syndrome and CNS leukemia at diagnosis were associated with a significantly lower remission induction rate. When analyzed in an univariate fashion, all factors studied, with the exception of Down's syndrome and type of CNS prophylaxis, were significant predictors (p< 0.05) of the length of initial bone marrow remission and survival. Utilizing multivariate techniques, factors found to independently and significantly predict length of marrow remission duration were (listed in order of importance) initial WBC, sex, age at diagnosis, type of CNS prophylaxis, pretreatment nodal enlargement, initial hemoglobin, and initial platelet count.

ISSN:0192-8562    

出版商:OVID    

年代:1980

数据来源: OVID

摘要:

In this study, we investigated the factors involved in the regulation of granulopoiesis in childhood aplastic anemia. Bone marrow, peripheral blood cells, and serum from nine children with aplastic anemia were added to normal bone marrow cells. All patients had decreased number of colony-forming units-granulocyte macrophage (CFU-GM). None of the patients had significant humoral inhibitors in their serum. Bone marrow cells from three patients were inhibitory to normalin vitrogranulopoiesis; peripheral blood cells from two of these patients were also inhibitory. Two of the patients showing inhibition had not received transfusions. It may be concluded that all children with aplastic anemia have decreased CFU-GM, and in only a minority of cases is this related to inhibitory cells. All patients with aplastic anemia should be investigated prior to bone marrow transplantation.

ISSN:0192-8562    

出版商:OVID    

年代:1980

数据来源: OVID

摘要:

Seventeen patients with Ewing's sarcoma were evaluated for long-term sequelae of CNS prophylaxis 2–12 years following cranial irradiation (2000 rads) and intrathecal methotrexate (12 mg/m2). The evaluation consisted of CT brain scans and hypothalamic-pituitary function testing utilizing provocative stimuli of several pituitary hormones. No abnormal CT scan findings or hormone levels were observed. These results contrast with the abnormal findings in children with acute lymphocytic leukemia, who received 2400 rads cranial irradiation and multiple doses of intrathecal chemotherapy as CNS prophylaxis, and suggest that a less intensive CNS prophylaxis regimen may not be associated with these sequelae.

ISSN:0192-8562    

出版商:OVID    

年代:1980

数据来源: OVID

摘要:

We recently described a test using epinephrine-induced aggregation of platelets pretreated with the metabolic inhibitor 2-deoxy-d-glucose to detect carriers of the Wiskott-Aldrich syndrome (WAS). We have evaluated this test further to include 22 obligate and 16 probable carriers of the WAS gene. The optimum concentration of epinephrine to discriminate between carriers and female controls was 5.5 μM. However, even at this level there were 18% false positive results in controls and 30% false negative results in obligate carriers. A significant trend existed for all obligate carriers within a family to be either positive or negative, suggesting the test may be useful but only in certain families. Based on the present results, and recent evidence linking monoamine oxidase activity and epinephrine aggregation, we present further suggestions for improving this stress test.

ISSN:0192-8562    

出版商:OVID    

年代:1980

数据来源: OVID

摘要:

The precise molecular defect in hereditary spherocytosis (HS) remains unknown. It is generally accepted, however, that the underlying membrane defect is the cause of the rigidity, shape, and permeability abnormalities of HS red cells. The spleen, with its unique microcirculation, plays the key role in the pathophysiology of hereditary spherocytosis. It is responsible for mechanical sequestration of spherocytes in the splenic cords, it is responsible for phagocytosis of spherocytes and their breakdown products, and it is responsible for the deleterious “conditioning” of those red cells which manage to escape back into the general circulation. The role of the spleen in the pathophysiology of the severe hemolytic form of hereditary elliptocytosis appears to be quite similar.

ISSN:0192-8562    

出版商:OVID    

年代:1980

数据来源: OVID

摘要:

Hematologic disorders are the most common indications for splenectomy in childhood. The increased incidence of severe, often fatal infections after splenectomy means that one must weigh the benefits to be gained from the operation against the risks. The most common hematologic indications are hereditary spherocytosis, chronic immune thrombocytopenic purpura, hemoglobinopathies, and hypersplenism. These and other disorders are discussed with emphasis on selection of patients for splenectomy, benefits to be expected, and care necessary after splenectomy, particularly the role of prophylactic antibiotics and pneumococcal vaccine immunization.

ISSN:0192-8562    

出版商:OVID    

年代:1980

数据来源: OVID

摘要:

Hodgkin's disease frequently involves the spleen. Careful histologic sections of the spleen are required to rule out involvement since the involved spleen may occasionally be of normal size. Vascular invasion may be the mode of splenic involvement. The spleen plays an important role in the body's immune function. Since splenectomized children with Hodgkin's disease are prone to fulminant infections, these patients should be kept under careful surveillance. The value of penicillin prophylaxis and the pneumococcal vaccine in these patients is still under consideration.

ISSN:0192-8562    

出版商:OVID    

年代:1980

数据来源: OVID

摘要:

The evidence that splenectomy produces a state of increased risk of late sepsis is now established. That risk is greatest in the child splenectomized in the first 2 years of life, for the initial 3 years after splenectomy, and in disease states previously recognized as producing diminished host defenses. Nevertheless, risk exists in all age groups and all primary disease states.Clinical practice has been appropriately altered. Where possible, elective splenectomy is delayed until risk is diminished. The need for splenectomy for trauma has been decreased markedly as nonoperative management and reparative techniques have emerged. All children should receive pneumococcal immunization and penicillin prophylaxis after splenectomy.

ISSN:0192-8562    

出版商:OVID    

年代:1980

数据来源: OVID

ISSN:0192-8562    

出版商:OVID    

年代:1980

数据来源: OVID