摘要:

In 96 cases of childhood acute lymphoblastic leukemia (ALL), handmirror cells (HMCs) were counted in cytospin preparations of washed lymphoblasts obtained at diagnosis. HMC counts were significantly lower in T-ALL as compared to C-ALL (p< 0.001). In T-ALL and pre-T-ALL 2/35 cases had more than 10% HMCs; in C-ALL 22/41 cases had more than 10% HMCs. In two cases of B-ALL 19% and 36% HMCs were found. Thus, HMC expression in ALL is related to the immunological phenotype, especially found in ALLs originating along the B-cell lineage.

ISSN:0192-8562    

出版商:OVID    

年代:1985

数据来源: OVID

摘要:

Plasma dopa and the catecholamines—dopamine, norepinephrine, and epinephrine—were assayed by a radioenzymatic method in 15 children with active neuroblastoma and in eight others without evidence of disease to assess the value of these determinations in the diagnosis and management of the tumor. Thirty-four children with solid tumors and hemopoietic malignancies served as our controls. Elevated plasma dopa levels were observed in 13 children with active neuroblastoma (87%); dopamine and norepinephrine were elevated in 1/4 of these patients. In the group of children with neuroblastoma without evidence of disease, dopa and catecholamine levels were within the range observed in the controls. Total urinary catecholamines, homovamillic acid (HVA) and/or vanil-mandelic acid (MVA) were elevated in 11 of the 15 (73%) neuroblastoma patients with active disease. While serial plasma dopa determinations correlated with the course of the disease in practically all patients and thus were useful in their follow-up, the catecholamines were of limited value in assessing tumor status. Our results suggest that plasma dopa, assayed by a radioenzymatic method, may be more reliable than the traditional urinary catecholamine determinations in the diagnosis of neuroblastoma, and it appears useful in the management of this disease.

ISSN:0192-8562    

出版商:OVID    

年代:1985

数据来源: OVID

摘要:

Necrosis of the bone marrow is rarely observed during life. Less than 50 cases have been reported, for the most part in patients with leukemia. The finding of bone marrow necrosis in children is even more rare. Of the seven cases reported, six patients had acute lymphoid leukemia and one patient had lymphocytic lymphoma. The occurrence of marrow necrosis diagnosed by aspirate and by biopsy in a child 18 months of age is presented.The necrosis was present several months before the appearance of acute lymphoid leukemia. The reasons for the presumed rarity of this pathological finding, its relationship with bone or joint pain and fever, hypotheses concerning pathogenesis, and its prognostic value are discussed.

ISSN:0192-8562    

出版商:OVID    

年代:1985

数据来源: OVID

ISSN:0192-8562    

出版商:OVID    

年代:1985

数据来源: OVID

摘要:

The Jamaican sickle cell cohort study, based on neonatal diagnosis of all cases of sickle cell disease among 100,000 consecutive births, has identified acute splenic sequestration (ASS) and pneumococcal disease as the most important complications in early life. The etiology of ASS is unknown and prophylaxis is therefore not possible. For first attacks, attention has been directed to parental education to achieve earlier diagnosis. Recurrent attacks may be prevented by prophylactic splenectomy.A controlled trial on the prevention of pneumococcal disease has indicated many pneumococcal septicemias in children given the 14 valent pneumococcal vaccine between the ages of 6 months and 3 years. No pneumococcal isolations occurred during the same period in children given monthly long-acting prophylactic penicillin.A controlled trial of folate supplementation for 1 year in children aged 6 months to 4 years indicated no difference between control and treatment groups in hemoglobin levels or weight and height velocity. The MCV was 4 fl less in the supplemented group.A controlled trial of feeder vessel photocoagulation in the therapy of proliferative retinopathy indicated significantly less vitreous hemorrhage in treated patients, but choroidal neovascularisation was a common complication of xenon arc therapy, and retinal tears commonly followed the use of the Argon laser. A new trial of scatter therapy is in progress.

ISSN:0192-8562    

出版商:OVID    

年代:1985

数据来源: OVID

摘要:

Forty-five percent of the 121 million persons who live in Brazil have morphological indications of Black admixture, and 5–6% of them are carriers of the hemoglobin S gene. But even in persons identified as white, the hemoglobin AS genotype is present in about 1%. The total number of people with sickle cell disease is estimated as 45,000. The present analysis is based in 409 patients studied in Rio de Janeiro. The observed frequency of deformities of the vertebrae (18%), bone infarcts (12%), and gnathopathy (4%) was lower than those found in any other series. The proportion of adults with splenomegaly was also lower than that of other studies. Chest pains were more frequent in males, while bone or joint pains and splenic sequestration crises seemed to be more common in females. Effects of the disease in the viability and fertility of these patients were quantified. Fetal loss in 67 pregnancies was 48%. Hemoglobin F levels showed a clear age effect, and higher levels were associated with a more benign course of the disease. Information about the services for prevention and treatment of sickle cell disease that exist in Brazil and one place in Venezuela, is provided. Treatment is only symptomatic in 12 centers, while in Sao Paulo piracetam is being successfully used in the treatment of crises. A brief overview about the current Brazilian studies on this disease is also presented.

ISSN:0192-8562    

出版商:OVID    

年代:1985

数据来源: OVID

摘要:

Although the pathophysiologic manifestations of sickle cell disease have been assumed to result from micro-vascular occlusion consequent toin situsickling of erythrocytes, actual blood vessel obstruction have been rarely demonstratedin vivo.Recent observations utilizing sophisticated biophysical techniques to study the intracellular hemoglobin S polymerization process has led to major revisions in this previously held pathophysiologic paradigm, butin vivocorrelations are still lacking. With the development of new noninvasive imaging and para-imaging methods, it is now technically possible and feasible to characterize both regional organ perfusion and tissue biochemistry in quantitative terms. In addition, these modalities promise to clarify pathogenesis of the disease through definition of the events responsible for the progression from tissue ischemia and infarction through the resolution phase.Since these noninvasive techniques are amenable to sequential applications, they should facilitate objective evaluations of clinical trials of therapeutic agents designed to prevent or delay the vaso-occlusive manifestations of sickle cell disease.

ISSN:0192-8562    

出版商:OVID    

年代:1985

数据来源: OVID

摘要:

The possibility of using bone marrow transplantation to treat selected patients with sickle cell anemia has recently been raised by the effectiveness of this approach in an 8-year-old girl suffering from both acute myeloblastic leukemia and sickle cell anemia. The child's sickle cell anemia was converted to the donor's sickle cell trait and she remains in complete remission from her leukemia 22 months following transplantation. This paper considers the therapeutic implications of this child's progress and discusses the major immunological complications, particularly graft-vs.-host disease, which currently limit the more widespread use of marrow transplantation in the therapy of sickle cell anemia.

ISSN:0192-8562    

出版商:OVID    

年代:1985

数据来源: OVID

摘要:

The obvious beneficial effects of hemoglobin F on sick-ling have F motivated numerous investigators to increase this type of hemoglobin artificially in patients with sickle cell anemia. Various chemotherapeutic agents including 5-azacytidine, hydroxyurea, and cytosine arabinoside, have been used successfully in patients. All of these drugs can increase the level of hemoglobin F in sickle cell anemia (SS) patients, but the kinetics and magnitude of the responses are highly individual and variable. The mechanism or mechanisms responsible for the increased synthesis of hemoglobin F remain unknown. Further controlled studies in a limited number of patients with severe sickle cell disease will be necessary in order to work out a rational, safe treatment program suitable for wider use.

ISSN:0192-8562    

出版商:OVID    

年代:1985

数据来源: OVID

摘要:

Sickle cell anemia, like other chronic hemolytic states, predisposes to cholelithiasis. However, it seems that other factors, apart from the consequent hyperbilirubinemia, contribute to this predisposition. This concept is supported by the fact that while cholelithiasis is very common among North American sickle cell patients, it is not so common in African patients.It is now believed that African sickle cell patients are protected from developing gallstones because of their low dietary intake of cholesterol and low intake of refined fiber-depleted foods. A review of all the published work on cholelithiasis, in Nigerians in general and sickle cell patients in particular, is presented in this article. A comparison is made with similar studies carried out in North America.

ISSN:0192-8562    

出版商:OVID    

年代:1985

数据来源: OVID