摘要:

Complete aortic occlusion at L2-L3occurred in an 11-day-old male with hypernatremic dehydration. Attempted thrombolytic therapy using intraarterial urokinase was unsuccessful in inducing either a systemic hyperfibrinolytic state or lysis of the clot. The infant's plasma plasminogen level was low (50–75% of adult levels) and neither fresh frozen plasma nor cryoprecipitate infusion provided adequate plasminogen replacement. Thrombolytic therapy failure was due in part to inadequate generation of plasmin. Alternative means of lytic therapy will need to be devised for this age group.

ISSN:0192-8562    

出版商:OVID    

年代:1982

数据来源: OVID

摘要:

A girl with clinical and laboratory findings of severe Von Willebrand's disease (VWD) characterized by a prolonged bleeding time, marked reduction of both Factor VIII procoagulant activity and Factor VIII related antigen with an abnormal crossed immunoelectrophoretic factor VIII molecule (CIEP) is presented. Persistent thrombocytopenia and abnormal platelet function manifested during platelet aggregation with epinephrine, ADP and collagen and abnormal C14serotonin and platelet factor 4 release were also noted. Family studies reveal both parents and a paternal aunt with low normal VWD parameters and normal immunoelectrophoretic factor VIII molecules. A sister has mild classical VWD. Both the father and paternal aunt have normal platelet counts but manifest a similar platelet functional defect. These findings suggest that our patient is homozygous for VWD and has inherited the platelet functional defect through the paternal side of the family. The addition of CIEP techniques may allow for further genetic clarification of the Von Willebrand syndromes; specifically, delineating the severe, homozygous Von Willebrand patient from the more common classical heterozygous patient and from the heterogeneous group of VWD patients with structural defects of the VWD factor. The genetic implications and the interaction of thrombocytopenia and abnormal platelet function in VWD are discussed.

ISSN:0192-8562    

出版商:OVID    

年代:1982

数据来源: OVID

摘要:

Six of 19 patients treated for medulloblastoma over an 8-year period developed extraneural metastases. The most common sites of metastatic disease were bone (Spatients), and bone marrow (4 patients). Radionucleotide bone scans and/or bone marrow aspirates detected disease in all such cases. Four patients also had recurrence within the central nervous system. Four of six patients relapsed within one year of initial treatment. All recurrences were treated with radiotherapy and/or chemotherapy with symptomatic improvement. The use of bone scans and bone marrow aspirations in the follow-up of patients with treated medulloblastoma would seem to be indicated.

ISSN:0192-8562    

出版商:OVID    

年代:1982

数据来源: OVID

摘要:

The medical records of 318 children and adolescents with acute lymphoblastic leukemia (ALL) were reviewed to determine the relationship between the occurrence of fever, a positive blood culture, and the absolute neutrophil count (ANC) during induction, remission maintenance, and bone marrow relapse. On the day of diagnosis of ALL, almost one-third of the patients had a temperature higher than 38.5°C, but only three (4.1%) had positive blood cultures. Following the initiation of induction chemotherapy, many patients had febrile episodes. Forty-six percent of the blood cultures obtained during this period were positive. Only patients with an absolute neutrophil count of 1000/mm3or less were at risk of a positive blood culture during induction. Thirty-four percent of the admissions during remission maintenance for fever were associated with pneumonia. One-half of the episodes of pneumonia occurred during the first 150 days following the diagnosis of ALL. No patient with pneumonia during remission maintenance had a positive blood culture. Eighteen percent of the blood cultures obtained from patients admitted during periods of bone marrow relapse were positive. Gram-positive organisms predominated during induction, whereas gram-negative organisms predominated during periods of bone marrow relapse.

ISSN:0192-8562    

出版商:OVID    

年代:1982

数据来源: OVID

摘要:

Forty-eight cases of acquired aplastic anemia in children were analyzed in comparison to 26 cases of genetic aplastic anemia and 483 cases of aplastic anemia in adults. All were gathered from similar institutions and all were similarly followed and treated with androgens. The following conclusions were drawn: 1) Initial severity is greater in children than in adults, and is greater in acquired than in genetic aplastic anemia; 2) even in cases of similar initial severity, the early death rate is higher in children than in adults; 3) a multiparametric index allows the correct prediction of short-term evolution in 70 % of the cases and thus aids in providing an indication for bone marrow graft; its sensitivity is similar to that of the classical parameters proposed by Camitta, et al., but its specificity significantly higher; 4) most deaths occurred during the first 3–4 months and the chance for long-term improvement appears similar in the more severe than in the less severe cases if they survive this delay; 5) some data (relapse after androgen withdrawal and androgendependence and failure of corticoid therapy alone) suggest that androgen therapy in children is useful, as it is in adults, and that corticosteroids do not modify the course of the disease at its usual dosage (1 mg/kg/day); and 6) very few side effects, particularly concerning height, of androgens were noted in the survivors at adult age after long-term androgen therapy prescribed before puberty.

ISSN:0192-8562    

出版商:OVID    

年代:1982

数据来源: OVID

摘要:

A review of the literature indicates that black children in the United States have a lower overall incidence of cancer and are less prone to leukemia and certain solid tumors, including neuroblastoma, rhabdomyosarcoma, Ewing's sarcoma, testicular tumors, liver tumors, and malignant melanoma, than are white children. Black children with acute lymphoblastic leukemia and retino-blastoma, but not with neuroblastoma, Wilms' tumor, and rhabdomyosarcoma, have poorer survival rates than white children. Socioeconomic status appears to be an important reason for the discrepant outlook, but genetic differences may also play a role. Consideration of these issues will assist in planning appropriate treatment regimens.

ISSN:0192-8562    

出版商:OVID    

年代:1982

数据来源: OVID

摘要:

In this paper we describe thede novaappearance of azurophilic giant granules and rod-shaped inclusions in monocytes and macrophages grown from the peripheral blood and bone marrow of a patient with acute monocytic leukemia; no such inclusions were evident in direct smears of the patient's peripheral blood or bone marrow. The cytochemical profile of the inclusions, their origin in mononuclear phagocytes only, and their development exclusivelyin vitrosuggest that they are distinct from Auer rods, Chediak-Higashi-like giant granules, and other abnormal azuorophilic inclusions previously described in patients with acute non-lymphocytic leukemia.

ISSN:0192-8562    

出版商:OVID    

年代:1982

数据来源: OVID

ISSN:0192-8562    

出版商:OVID    

年代:1982

数据来源: OVID

摘要:

It is now possible to prevent effectively the occurrence of overwhelming pneumococcal infection from the majority of pneumococcal organisms in patients with increased susceptibility to infection. Immunization of patients with sickle cell disease and patients with splenectomy results in a significant increase in antibody amounts which persist for long periods of time. Patients under 2 years of age and patients with Hodgkin's disease and splenectomy respond less well. Most “vaccine failures” which have been described, have been in immunosuppressed patients, or a result of infection withS. pneumoniaetype 6A. Future directions in the development of a more effective vaccine for patients under 2 years of age and immunosuppressed patients will include research efforts to formulate a new immunogenic vaccine. Definitive recommendations regarding booster immunization must await more definitive studies, but current data suggests that booster immunization is not necessary for at least 5–6 years following primary immunization.

ISSN:0192-8562    

出版商:OVID    

年代:1982

数据来源: OVID

摘要:

The characteristics of irreversibly sickled cells (ISC) are reviewed in relationship to their potential significance in the initiation of sickle cell vaso-occlusion. Although ISC are implicated in the shortened red cell survival of sickle cell anemia, there is no direct correlation to the frequency or severity of sickle crisis. ISC are heterogeneous in their physical properties suggesting the possibility that a subpopulation may be disproportionately important in causing vaso-occlusion. Study of their deformability heterogeneity of ISC showed that filtration-separated hard ISC are similar in morphology and intracellular composition to soft ISC, and would not be distinguished on blood smear.

ISSN:0192-8562    

出版商:OVID    

年代:1982

数据来源: OVID