ISSN:1040-8703    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

Idiopathic thrombocytopenic purpura (ITP) is the most common acquired bleeding disorder encountered by pediatricians. Most children with ITP have minimal bleeding and complete platelet count recovery within weeks to months. Therapy for ITP has ranged from close observation without medical intervention to aggressive management with corticosteroids, intravenous immunoglobulin G, or anti-D immune globulin. The topic of ITP has incited great debate among practitioners, and this debate prompted the development of ITP practice guidelines by the British Paediatric Haematology Group in 1992 and by the American Society of Hematology in 1996. A better understanding of the clinical course of, risk for significant bleeding in, and optimal evaluation and therapy of childhood ITP will require carefully designed, multicenter, clinical trials.

ISSN:1040-8703    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

Recombinant hematopoietic growth factors were introduced into clinical practice a decade ago: erythropoietin in 1989, granulocyte colony-stimulating factor (G-CSF) and granulocyte-macrophage colony-stimulating factor (GM-CSF) in 1991, and interleukin-11 in 1997. The role of these agents in supportive therapy for children with cancer is still under considerable evaluation. This pediatric-based review summarizes current clinical applications, practice guidelines, and practice patterns for hematopoietic growth factors in the supportive care of children with cancer. It also discusses ongoing controversies and unanswered questions.

ISSN:1040-8703    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

Recently, several groups have begun to administer granulocyte colony-stimulating factor (G-CSF), a hematopoietic growth factor, with or without dexamethasone to mobilize peripheral blood neutrophils. Granulocyte colony-stimulating factor (600 μg subcutaneously) and dexamethasone (8 mg orally) given 12 hours before standard leukapheresis routinely results in the collection of approximately 80 × 109granulocytes. This number of cells is sufficient to increase the neutrophil count of a severely neutropenic patient to normal and restore the recipients’ ability to develop a neutrophil response in tissues. Several trials are ongoing to establish the clinical benefit of this new approach to supportive care for neutropenic patients.

ISSN:1040-8703    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

Large and unusually large von Willebrand factor (vWf) multimers may be responsible for systemic platelet aggregation in thrombotic thrombocytopenic purpura (TTP). This possibility is supported by studies that show deficient vWf-cleaving metalloproteinase and increased platelet–vWf binding during TTP episodes. In acute idiopathic TTP, decreased vWf metalloproteinase is the result of autoantibodies against the enzyme. In familial and acquired hemolytic-uremic syndrome, vWf-cleaving metalloproteinase activity is normal. A deficiency or defect in factor H, which normally dampens the activation of C3 via the alternative complement pathway, has been seen in some patients with familial hemolytic-uremic syndrome. Ticlopidine therapy is an important risk factor for TTP.

ISSN:1040-8703    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

Absolute polycythemia is a condition with increased red blood cell mass. There are a number of primary and secondary polycythemic disorders leading to absolute polycythemia. Primary polycythemias are caused by a defect intrinsic to the erythroid progenitor cells. The best characterized primary polycythemia is the autosomal dominant primary familial and congenital polycythemia (PFCP). Familial or childhood occurrence of the myeloproliferative disorder polycythemia vera are also discussed, emphasizing the importance of distinction between polycythemia vera and PFCP. Congenital or familial secondary polycythemic conditions are characterized by increased red cell mass, which is caused by circulating serum factors, typically erythropoietin.

ISSN:1040-8703    

出版商:OVID    

年代:2000

数据来源: OVID

ISSN:1040-8703    

出版商:OVID    

年代:2000

数据来源: OVID

ISSN:1040-8703    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

Perinatal brachial plexus palsy (PBPP) has been traditionally classified into three types: upper plexus palsy (Erb’s) affecting the C5, C6, and ± C7 nerve roots, lower plexus palsy (Klumpke’s) affecting the C8 and T1 nerve roots, and total plexus palsy. Although most cases will resolve spontaneously, the natural history of the remaining cases is influenced by contractures of uninvolved muscle groups and subluxation or dislocation of the shoulder and elbow. Microsurgical nerve repair has demonstrated to provide improved outcomes compared to conservative treatment, while advancements in secondary reconstruction have offered significant improvements in the performance of activities of daily living for older children with unresolved plexus palsy.

ISSN:1040-8703    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

Transient synovitis of the hip is one of the most common causes of hip pain and limp in young children. Its cause is still largely unknown, but its natural history is that of a self-limiting disorder with no residual sequelae, although recurrences are possible. Because this benign condition is similar to more significant disorders, such as septic arthritis, the diagnosis should remain one of exclusion. Once transient synovitis is diagnosed, treatment consists of rest, anti-inflammatory agents, and a tincture of time.

ISSN:1040-8703    

出版商:OVID    

年代:2000

数据来源: OVID