ISSN:1040-8703    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

Neonatal screening for hemoglobinopathies, coupled with comprehensive medical care that includes parental education, prophylactic penicillin, and immunizations, has markedly reduced mortality from sickle hemoglobinopathies during infancy and early childhood. However, despite an increased knowledge of pathophysiology, current therapy does little to prevent acute, noninfectious complications such as anemic crises, pain events, or strokes; nor does it reliably prevent or delay the development of chronic organ damage. Recent publications have increased our understanding of factors that contribute to the clinical heterogeneity of sickle hemoglobinopathies, improved modestly our ability to identify and treat acute complications, and provided real hope for the development of potentially curative therapies. Prospective therapeutic trials of hydroxyurea and of bone marrow transplantation have recently begun.

ISSN:1040-8703    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

This review summarizes recent literature regarding the clinical and molecular features of nonmalignant leukocyte disorders in children. Leukocyte adhesion deficiency and chronic granulomatous disease, two inherited disorders of neutrophil function, continue to be the best-characterized disorders with respect to specific molecular defects. The underlying causes of severe congenital neutropenia and cyclic neutropenia remain elusive. Clinical features of immune-mediated neutropenias and their differential diagnoses are also reviewed.

ISSN:1040-8703    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

Many new developments have taken place in the field of coagulation during the past year. We focus on the interesting new controversies surrounding vitamin K prophylaxis of the newborn infant, on the increasing information regarding the growth factors responsible for thrombopoiesis, and on the role of protein C in the genesis of thrombosis.

ISSN:1040-8703    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

The human recombinant hematopoietic growth factors have attracted widespread interest because of their potential efficacy in a number of clinical settings. Recombinant human erythropoietin is now an established therapy to treat and prevent the anemia associated with chronic renal failure in children and adults. This agent also shows extraordinary promise to stimulate erythrocyte production and reduce transfusion requirements in premature infants. Granulocyte and granulocyte-macrophage colony-stimulating factors stimulate the production and function of myeloid cells and have provided major clinical benefit to children with congenital disorders of neutrophil production. The myeloid growth factors also show great promise in reducing the duration and severity of neutropenias associated with cytotoxic cancer treatment and in improving granulocyte production in patients with human immunodeficiency virus infections.

ISSN:1040-8703    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

Bone marrow transplantation has become well established in the treatment of malignant and nonmalignant disorders. Steady progress continues in improving survival after transplantation, but many problems remain to be solved. Relapsed disease is a major limitation, and new, more intensive preparative regimens are being evaluated in an effort to improve efficacy and reduce toxicity. The availability of unrelated donors has been improved with continued expansion of the National Marrow Donor Program. Additional alternative sources of marrow actively being investigated include partially matched family members and peripheral blood stem cells. Improved marrow purging techniques are also being studied to allow expanded use of autologous marrow for various malignancies. The use of cytokines to minimize regimen toxicity and as therapeutic agents is also promising. The late effects of bone marrow transplantation, including relapse, chronic graft-versus-host disease, secondary malignancies, and effects on growth and development, are important considerations in the design of new therapeutic strategies.

ISSN:1040-8703    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

This article highlights the surgeon's role in childhood cancer especially as it relates to local control of solid tumors, research, and surgical supportive care. There is a trend toward preresection chemotherapy, and rarely, radiotherapy. This may allow safer, less extensive, and function-preserving delayed resection in neuroblastoma, hepato-blastoma, bone tumors, and nephroblastoma in selected patients without negatively affecting outcome. Ultimately, complete resection for most tumors, even advanced neuroblastomas, significantly improves survival. Organ transplantation allows complete resection with good survival in children with otherwise nonresectable liver tumors. In non-Hodgkin's lymphoma, resection should not be attempted, except for localized disease and if complete resection is possible. Second-look procedures have limited value in lymphoma, but have an important role in germ cell tumors. The differentiation of typhlitis from appendicitis is critical in the neutropenic patient to avoid life-threatening complications. Studies of venous access devices show a clear benefit of totally implantable devices in preventing dislodgment and decreasing the rate of infection. Neuroblastoma models are fertile soil for research into tumor biology and novel treatment modalities. Surgeons continue to play crucial roles in childhood oncology.

ISSN:1040-8703    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

The perils of radiotherapy in pediatric oncology are well advertised and, justifiably, alternative treatment strategies have been sought. However, radiotherapy can be a highly effective antitumor agent and results with alternative therapies, in terms of survival and quality of life, need to be carefully scrutinized. In addition, biologic and technical innovations in radiotherapy are increasing its versatility and providing means of more adequately protecting adjacent normal tissues.

ISSN:1040-8703    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

The sustained improvements in pediatric cancer therapy are partially due to the large randomized and nonrandomized studies being conducted by multidisciplinary cooperative groups. Theoretic concepts of chemotherapy and better knowledge of the pharmacokinetics, pharmacodynamics, and drug resistance of current and new drugs are enabling a more rational approach to multiagent therapy. Biologic modifiers are adding to our current multimodal therapeutic approach.

ISSN:1040-8703    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

Cure of children with cancer is occurring in ever increasing numbers. Research in supportive care areas such as pain management, emesis control, and infection prevention and treatment, has contributed not only to improved survival, but a better quality of life for children undergoing cancer treatment. Additionally, long-term follow-up of childhood cancer survivors is yielding information that will decrease the morbidity of future treatment regimens.

ISSN:1040-8703    

出版商:OVID    

年代:1993

数据来源: OVID