摘要:

Diabetes insipidus and growth retardation are the prominent endocrine manifestations of histiocytosis. Galactorrhea, hypogonadism, and panhypopituitarism are rarely associated with this disorder. To date, the vast majority of morphologic and functional studies of the pituitary and hypothalamus in affected patients indicate that histiocytic infiltration results in a hypothalamic dysfunction with a secondary partial or complete hypopituitarism. This hypopituitarism is due to deficient trophic stimulation or inhibition by hypothalamic releasing or inhibitory factors.

ISSN:0192-8562    

出版商:OVID    

年代:1981

数据来源: OVID

摘要:

Two previously healthy children, ages 8 months and 35 months, developed spontaneous inhibitors to Factor IX. Brief illnesses of presumed viral origin preceded hemorrhagic symptoms in both children. Cutaneous ecchymoses and traumatic soft tissue hemorrhage were the predominant clinical features in each case. Bleeding symptoms persisted for less than 3 days and laboratory evidence of Factor IX inhibition resolved within 3 weeks. One child required treatment with fresh frozen plasma and packed red blood cell transfusions. The other child received corticosteroid therapy. Given the transient nature of acquired Factor IX inhibitors in the nonhemophilic child, a conservative approach toward therapy is recommended unless life-threatening complications supervene.

ISSN:0192-8562    

出版商:OVID    

年代:1981

数据来源: OVID

摘要:

An 8-year-old girl was diagnosed as having idiopathic thrombocytopenic purpura which later became refractory to steroids, splenectomy, and immunosuppressive therapy. While she was being treated for pneumococcal meningitis, 7 1/2 years later, she was found to have Philadelphia-chromosome-positive chronic myelocytic leukemia. She died 2½ years after the diagnosis of leukemia. The association of autoimmune disorders and hematologic malignancies has been well-recognized. To our knowledge, there has been no previous report of chronic idiopathic thrombocytopenic purpura preceding the development of chronic myelocytic leukemia in the literature.

ISSN:0192-8562    

出版商:OVID    

年代:1981

数据来源: OVID

摘要:

The association betweenClostridium septicumsepsis in patients with malignant diseases has been frequently documented. A presentation with fever, neutropenia, vomiting, and an acute abdomen is characteristic of this anaerobic infection which has been uniformly fatal in children with acute leukemia. We report the unusual course and the successful treatment of an adolescent with an abdominal Burkitt's lymphoma with leukemic transformation andClostridium septicumsepsis and cellulitis.

ISSN:0192-8562    

出版商:OVID    

年代:1981

数据来源: OVID

摘要:

Techniques for the detection of neutrophil antibodies include leukoagglutination, cytotoxic assays, antiglobulin consumption tests, immunofluorescence, quantitation of cell-bound IgG, and opsonization assays. Employment of the various methodologies have led to the identification of neutropenic states associated with decreased survival of neutrophils in the circulation because of immune-mediated distribution of the neutrophils or excessive sequestration of their cells by reticuloendothelial cells.The immune clinical syndromes predisposing to selective neutropenia include isoimmune neonatal neutropenia, autoimmune neutropenia, drug-induced neutropenia, and complement-mediated neutropenia. Selective immune-mediated neutropenia occurs because the neutrophil contains unique antigens often not shared by other blood cells. Like incompatible red cell transfusions, incompatible neutrophil transfusions can result in clinical manifestations including fever and hypoxemia arising from pulmonary sequestration of neutrophils. Potentially therapeutic benefits derived from neutrophil transfusions and from bone marrow transplantations may be influenced by neutrophil antigen-antibody reactions. Newer techniques for detecting and identifying neutrophil antibodies may now provide a tool to answer these questions.

ISSN:0192-8562    

出版商:OVID    

年代:1981

数据来源: OVID

摘要:

Most of our current knowledge about the roles of blood vessels, platelets, and plasma factors in hemostasis has been gained in the last three decades. However, prior to this period a relatively limited but critical framework of ideas about hemostasis had slowly but steadily evolved, beginning in 1666 when Malpighi discovered fibrin in blood clots. By the turn of the 20th century aggregation of platelets at the site of blood vessel injury was recognized and four enduring coagulation factors emerged: fibrinogen, prothrombin, tissue extracts (thromboplastin), and calcium. Then progress exploded after World War II, leading us into a search for the molecular basis of hemostasis where we are today. Through it all, observations made at the bedside by astute clinicians have been a driving force in the advancement of basic knowledge.

ISSN:0192-8562    

出版商:OVID    

年代:1981

数据来源: OVID

摘要:

Autoimmune hemolytic anemia (AIHA) is produced by thein vivodestruction of red cells coated with auto-antibodies which are produced by naturally occurring B-lymphocytes. The auto-antibody-producing cells are under the control of suppressor T-lymphocytes whose activity may be deficient in patients with AIHA. The ultimate cure of AIHA will probably reside in the reestablishment of the normal control of the auto-antibody-producing cells and not in the elimination of the cells responsible for the destruction of the antibody-coated cells by splenectomy or drug therapy.The cells responsible for destruction of red cells are necessary for the removal of other antibody-coated particles (such as bacteria, etc.). The removal or inactivation of tissue macrophages predisposes the patient to other serious problems including the possibility of severe sepsis. The reestablishment of normal control of the auto-antibody-producing cells by the normalization of the patients' suppressor cell status may be a safer mode of therapy for autoimmune hemolytic anemia in the future.

ISSN:0192-8562    

出版商:OVID    

年代:1981

数据来源: OVID

ISSN:0192-8562    

出版商:OVID    

年代:1981

数据来源: OVID

ISSN:0192-8562    

出版商:OVID    

年代:1981

数据来源: OVID