摘要:

PurposeThe goal of the study was to assess development in young children with sickle cell disease as part of the Cooperative Study of Sickle Cell Disease (CSSCD).Patients and MethodsThe Denver Developmental Screening Test (DDST) was administered to children younger than 6 years at 12 participating institutions of the CSSCD. Trained examiners administered tests to 344 children.ResultsTests were scored as normal in 90.4%, questionable in 6.4%, and abnormal in 1.5%; 1.7% of children were considered untestable. There was no relationship between DDST results and sickle cell genotype. Questionable and abnormal (Q/A) scores were more common in children ages 3–5 years than in younger children (12.6% versus 3.8%;P= 0.002).ConclusionsBecause the DDST is a screening test, it should be interpreted cautiously. However, the more numerous Q/A scores in our “older” group agree with the findings of recent reports of neuropsychological impairment in school-age children with sickle cell disease. Our data suggest that development is relatively normal before age 3 years; deficits seen in older children may reflect subsequent ischemic insults.

ISSN:0192-8562    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

PurposeThe study was performed to clarify in the hemophagocytic syndrome (HPS) how cytokinemia plays a role in its pathogenesis and if cytokinemia is of prognostic value.Patients and MethodsSerum concentrations of ferritin, interferon (IFN)-y, soluble interleukin-2 (IL-2) receptor, IL-6, and other cytokines were determined during the acute phase of the HPS in 29 children and three adults. Data comparing malignancy-associated (MAHS; n = 17) and infection-associated hemophagocytic syndrome (IAHS; n = 15) and those comparing surviving and fatal cases were assessed.ResultsHyperferritinemia and hypercytokinemia were present in all patients with HPS. Eleven of the 17 MAHS and three of the 15 IAHS cases were fatal (p < 0.05). No significant difference in cytokine concentrations was observed between MAHS and IAHS.ConclusionsIn terms of cytokine effect on patient outcome, serum concentrations of IL-6 >300 ng/L and IFN-γ concentrations >30 U/ml or soluble IL-2 receptor (sIL-2R) concentrations >10,000 U/ml were considered to reflect a poor prognosis in HPS patients.

ISSN:0192-8562    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

Patients and MethodsWe report patients who were treated for non-Hodgkin's lymphoma (NHL) or Ki-l antigen-positive (Ki-l) lymphoma with a T-8801 protocol that included etoposide (VP-16) and behenoylcytosine arabinoside.ResultsSecondary acute myeloid leukemia (AML) developed in 5 of 38 NHL and Ki-1 lymphoma patients, and the cumulative risk at 4 years was 18.4%. The median time from the initiation of the chemotherapy to the development of AML was 21 months (range, 13–30). Four patients had a FAB M5 morphology, and one had FAB M2. In four of five examined cases, chromosomal alterations involving the long arm of chromosome 11 were demonstrated at the time of development of AML. None of the 46 NHL patients who we treated with another protocol (B-8801), using significantly higher cumulative doses of VP-16 than in the case of the patients with T-8801 and a different schedule of VP-16 administration, developed secondary AML.ConclusionsThe risk of secondary AML possibly related to the use of VP-16 given twice weekly.

ISSN:0192-8562    

出版商:OVID    

年代:1993

数据来源: OVID

ISSN:0192-8562    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

PurposeThe management of oral bleeding in hemophilia A patients with high titer inhibitors can be challenging. Simultaneous administration of activated prothrombin complex concentrates and antifibrinolytic agents is potentially dangerous because both agents have thrombogenic properties. We report sustained control of life-threatening lingual hemorrhage in a hemophilic patient with a high titer inhibitor (100 Bethesda Units/ml) on continuous in-fusion of a monoclonal antibody-purified factor VIII concentrate (75 U/kg/h).Methods and ResultsIn vivo hemostasis was achieved without an initial increment in free plasma factor VIII:C. The biphasic nature of recovered factor VIII:C indicated initial antigen-antibody formation, a saturation point, then a rapid rise of free factor VIII in plasma. In vitro, rapid loss of factor VIII activity was noted in mixtures of patient's plasma and purified factor VIII during incubation at 37°C. When an excess of purified factor VIII was added to patient's plasma, a plateau of stable residual factor VIII activity followed the initial loss of factor VIII activity, suggesting a second-order reaction.ConclusionThis type I kinetic response is typical of alloantibodies induced by exposure to factor VIII concentrates.

ISSN:0192-8562    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

A previously healthy 11 year-old-white boy presented with persistent mild leukocytosis and a posterior mediastinal mass. Surgical excision of the mass showed it to be a ganglioneuroma. Postoperatively, the patient had marked leukocytosis (white blood cell count 57,000/mm3) and a low leukocyte alkaline phosphatase score. Bone marrow aspirate for cytogenetics performed in the evaluation of the posterior mediastinal mass showed the presence of the Philadelphia chromosome (Ph) in 19 of 20 metaphase spreads. The diagnosis of Ph+chronic myeloid leukemia (CML) was confirmed on repeat cytogenetics. Subsequent detailed skin examination showed six small cafe-au-lait spots but no other evidence of neurofibromatosis. This is the first report to describe an association between ganglioneuroma and Ph+CML.

ISSN:0192-8562    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

We report a 16-year-old Tanner IV male with homozygous hemoglobin S who presented with recurrent episodes of priapism unresponsive to standard therapy with hydration, analgesia, and exchange transfusion. He had a complete resolution of his symptoms with hydralazine therapy. We therefore suggest a trial of vasodilator therapy for recurrent sickle cell priapism before attempting surgical therapy.

ISSN:0192-8562    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

True extrarenal Wilms' tumor is a rare malignant neoplasm most frequently presenting in the retroperitoneal or inguinal regions. We report an unusual subcutaneous lumbosacral (LS) region extrarenal Wilms' tumor without associated teratomatous tumor elements or associated neural tube defect in a 2°-year-old girl. Pathologic review revealed features of true extrarenal Wilms' tumor, and the patient remains in complete remission following surgery and combination chemotherapy. This report illustrates the importance of early surgical intervention and pathologic examination of similar soft tissue masses in children.

ISSN:0192-8562    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

BackgroundIn adults—but not neonates—neutropenia has been reported to complicate treatment with intravenous immunoglobulin, but the mechanism is unknown.PurposeTo describe for the first time the case of a new-born infant who, after intravenous immunoglobulin, demonstrated serum antineutrophil antibodies and neutropenia.Patients and MethodsThe 1,425-g, 36-week-gestation boy was healthy except for intrauterine growth retardation. Intravenous immunoglobulin (1g/dose ± 3) was administered to treat alloimmune thrombocytopenia. Neutrophilspecific antibodies were detected by a granulocyte immunofluorescence assay.ResultsAfter the intravenous immunoglobulin, the plate-let count normalized but the neutrophil count declined to 450/mm3. Neutrophil-specific antibodies were detected in the serum of the infant but not in the maternal serum. Furthermore, cross-matching revealed that the maternal serum did not react with the infant's granulocytes. Two of three random lots of intravenous immunoglobulin contained detectible anti-neutrophil antibodies.ConclusionsAfter intravenous immunoglobulin, the infant's serum contained one or more antineutrophil anti-bodies that were not maternal in origin. We speculate that the neutropenia resulted from the administration of intravenous immunoglobulin containing antineutrophil antibodies.

ISSN:0192-8562    

出版商:OVID    

年代:1993

数据来源: OVID

ISSN:0192-8562    

出版商:OVID    

年代:1993

数据来源: OVID