摘要:

Glucocorticoids have been included in almost all treatment regimens for childhood acute lymphoblastic leukemia for decades. However, optimal agents, doses, and/or schedules have yet to be defined despite extensive clinical application. New data on the pharmacokinetics, pharmacodynamics, and molecular mechanisms of action of glucocorticoids have suggested alternative approaches in ALL. These suggest that prolonged, i.e. 28 day, glucocorticoid therapy may be unnecessary as exposure to glucocorticoid induces down-regulation of glucocorticoid receptors. Dexamethasone may be superior to prednisone in conventional equi-effective doses. Blast sensitivity to glucocorticoids correlates closely with sensitivity to other, putatively non-cross-resisting agents and with outcome after multi-agent therapy, suggesting overlapping mechanisms of action, and focusing attention on the determinants of the threshold for apoptosis. Increasing success in the treatment of childhood acute lymphoblastic leukemia has led to increasing awareness of avascular necrosis of bone as a potentially disabling sequela of glucocorticoid therapy, especially in adolescent and young adult patients.

ISSN:1077-4114    

出版商:OVID    

年代:1995

数据来源: OVID

摘要:

Increased understanding of the pathophysiology of anemia of prematurity (AOP) has emerged over the past years. It is apparent that low levels of endogenous erythropoietin (Epo) is the basis for this phenomenon. This has resulted in an effective therapeutic approach for this condition. Administration of Epo can ameliorate the signs and symptoms of AOP and lessen, and in many cases avoid, the necessity for blood transfusion.

ISSN:1077-4114    

出版商:OVID    

年代:1995

数据来源: OVID

摘要:

PurposeChildren with Down syndrome (DS) carry an elevated risk of developing clonal hematologic disorders. In this review, we summarize reported data describing the incidence of leukemia in children with DS and associated leukemic cell phenotypic and functional features.Patients and MethodsMajor leukemic subtypes, acute lymphoblastic leukemia (ALL), acute nonlymphoblastic leukemia (ANLL), myelodysplastic syndromes (MDS), and transient abnormal myelopoiesis (TAM) are evaluated in the context of patient age and treatment responsiveness.ResultsIt is apparent that although leukemia in children with DS is a relatively frequent event, prognosis with current conventional therapeutic strategies is excellent.ConclusionsTreatment options for TAM, a monoclonal cellular proliferation in neonates, are discussed.

ISSN:1077-4114    

出版商:OVID    

年代:1995

数据来源: OVID

摘要:

PurposeBecause there is little information on bleeding times (BTs) in children we initiated the following study.Materials and MethodsNormal children undergoing elective surgery and adult volunteers had their bleeding times measured with a disposable device (Simplate) with a vertical incision in the forearm. Results in children (four age groups) and adults, male and female, were compared.ResultsThe mean time in children was 270 s with a 95th percentile of 420 s compared with a mean time in adults of 320 s and a 95th percentile of 480 s (p = 0.001). Although the values in the various age groups and sexes were different, only sex had a statistically different value in adults.ConclusionsThe following reference values should be used for children: 0–4 years, 4 ± 1 min; boys >4 years, 5 ± 1 min; girls >4 years, 5.5 ± 1 min. We conclude that results obtained in children are significantly shorter than those obtained in normal adult subjects.

ISSN:1077-4114    

出版商:OVID    

年代:1995

数据来源: OVID

摘要:

PurposeWe evaluated the accuracy of transcranial Doppler (TCD) and magnetic resonance angiography (MRA) as a screening modality for cerebral infarct in patients with sickle cell disease.Patients and MethodsTwenty-four patients with sickle cell disease were assessed for a cerebral infarct. Each patient underwent neurologic examination, psychometric evaluation, magnetic resonance imaging (MRI), MRA, and TCD. Presence of a cerebral infarct was determined by an MRI and supported by neurologic and neuropsychometric evaluation. All investigators were unaware of the patient's status. Cerebral vasculature by MRA was categorized as being occluded, stenotic, or normal. The maximum TCD velocity of the right and left middle cerebral artery were assessed. Various cutoffs were used to determine the sensitivity and specificity of TCD.ResultsMRA had a sensitivity and specificity of 100% and 92%, respectively. At a maximum velocity cutoff of 180 cm/s, the sensitivity and specificity of TCD were 20% and 67%, respectively. No maximum velocity on TCD produced both sensitivity and specificity >50%.ConclusionMRA is more sensitive than TCD when the middle cerebral artery for maximum velocity is compared. Prospective evaluation is warranted to determine if TCD is useful as a screening technique for cerebral infarction in children with SCD.

ISSN:1077-4114    

出版商:OVID    

年代:1995

数据来源: OVID

摘要:

PurposeThe activity of the drug combination ifosfamide and etoposide (VP16) in refractory and relapsed childhood acute lymphoblastic leukemia (ALL) was assessed in a phase II study.Patients and MethodsTwenty children with ALL, all heavily pretreated and in bone marrow relapse, were entered on the study. Drugs were given i.v. each day for 5 days at the following doses: ifosfamide 1.8 g/m2/day, VP16 100 mg/m2/day, and MESNA 2,880 mg/m2/day (as a uroprotectant); cycles were repeated every 28 days. At study entry, eight patients were in first relapse (five of whom had failed intensive reinduction regimens), seven were in second relapse, and five were in third relapse. All patients had received cyclophosphamide in regimens before relapse.ResultsEight patients (40%; 95% confidence interval 19–64%) achieved complete bone marrow remission with ifosfamide/VP16. Three patients subsequently relapsed in the bone marrow while on ifosfamide/VP 16 therapy. Duration of remission ranged from 21 to 247 days. Treatment was generally well tolerated, with myelosuppression the most common toxicity; fever and neutropenia occurred in 18 of 31 evaluable cycles.ConclusionThe combination of ifosfamide/VP16 has significant activity in recurrent and refractory childhood ALL with tolerable toxicity.

ISSN:1077-4114    

出版商:OVID    

年代:1995

数据来源: OVID

摘要:

PurposeThis paper describes the prevalence of emotional and behavioral problems and the social competencies of forty children with cancer immediately after their diagnosis and one year post-diagnosis.Patients and MethodsAt both points of time the problems and competencies of the children with cancer were compared with the problems and competencies of a matched group of children living in the community.ResultsThe results suggest that early in their illnesses, younger children with cancer experience more internalising problems than children in the community. However, the younger children with cancer improved significantly in these areas during the year after their diagnosis. As a result, one year after their diagnosis there was little difference in the prevalence of problems amongst the younger children with cancer and the children in the community. The older children with cancer did not appear to have more problems than children in the community at either the first or the second assessment.ConclusionsThe results of the study draw attention to possible differences in the-prevalence of emotional and behavioral problems experienced by younger and older children with cancer. The results also suggest that amongst younger children with cancer the prevalence of problems declines during the year after their initial diagnoses.

ISSN:1077-4114    

出版商:OVID    

年代:1995

数据来源: OVID

摘要:

PurposeChildren with bladder rhabdomyosarcoma (RMS) are currently treated with primary chemotherapy and usually with local irradiation. More than 30% of this group ultimately require total cystectomy. The purpose of this study was to review the results of the use of partial as opposed to total cystectomy in 40 patients with bladder RMS during Intergroup Rhabdomyosarcoma Study (IRS)-I, -II, and -III (1972–1989).Patients and MethodsA total of 171 children with primary RMS of the bladder were enrolled in the IRS during this interval. Partial cystectomy was performed in 40, in 33 before any other therapy and in seven after 10–57 weeks of chemotherapy (primarily vincristine, actinomycin-D, and cyclophosphamide, i.e., VAC) radiotherapy. Surviving patients have been observed for 4–22 years.ResultsThirty-one of 40 patients (78.5%) have been disease free for 2–16 years. Survival among all other IRS cases with bladder RMS during the same interval was 79.5%. Of the 31 surviving patients, one required secondary total cystectomy and two required bladder augmentation procedures for benign bladder contracture. Three quarters of the total group of living patients who have undergone partial cystectomy are without bladder-related symptoms or demonstrable lower urinary tract disease. The remaining patients have a history of functional bladder problems related to contracture or incontinence.ConclusionsPartial cystectomy is an alternative to total cystectomy for bladder RMS when the tumor site makes it anatomically feasible. In such patients, it should be considered before total cystectomy and in patients with persistent areas of questionable residual tumor after otherwise apparently successful regimens of primary chemotherapy radiotherapy.

ISSN:1077-4114    

出版商:OVID    

年代:1995

数据来源: OVID

摘要:

PurposeA 12-year-old girl developed severe autoimmune thrombocytopenia after a bone marrow transplant for acute lymphoblastic leukemia.ResultsAlthough epsilon-aminocaproic acid helped to control her bleeding, it eventually caused a rare myopathy previously undescribed in a pediatric patient.ConclusionsThe myopathy resolved when the drug was discontinued and a different antifibrinolytic agent was used.

ISSN:1077-4114    

出版商:OVID    

年代:1995

数据来源: OVID

摘要:

PurposeI describe two siblings who have a novel congenital bone marrow failure syndrome with myelodysplastic features without progression to malignancy for 13 and 20 years, respectively.Patients and MethodsMale and female siblings were evaluated for chronic anemia and intermittent bruising.ResultsBoth patients had been anemic from birth, one carrying a diagnosis of Diamond-Blackfan anemia and the other an idiopathic marrow failure syndrome. Both patients had a platelet function defect, mild pancytopenia, and bone marrow examination showed hypoplasia with myelodysplasia that did not fit a French-American-British classification. Neither patient had Fanconi's anemia, and the family history was negative.ConclusionsThis is the first report of a novel syndrome of congenital bone marrow failure and myelodysplasia.

ISSN:1077-4114    

出版商:OVID    

年代:1995

数据来源: OVID